Atlas of Retinal Detachment: Diagnosis and Differential Diagnosis [1st ed.] 978-981-10-8230-6, 978-981-10-8231-3

Table of contents :
Front Matter ....Pages i-xi
Introduction of Retinal Detachment (Kai Ma, Nan Zhou, Wenbin Wei)....Pages 1-11
Rhegmatogenous Retinal Detachment (Ruilin Zhu, Nan Zhou, Wenbin Wei)....Pages 13-32
Special Types of Rhegmatogenous Retinal Detachment (Kai Ma, Nan Zhou, Wenbin Wei)....Pages 33-66
Exudative Retinal Detachment (Haiying Zhou, Nan Zhou, Wenbin Wei)....Pages 67-115
Congenital Abnormalities of the Fundus and Retinal Detachment (Yao Huang, Nan Zhou, Wenbin Wei)....Pages 117-133
Tractional Retinal Detachment (Lei Shao, Nan Zhou, Wenbin Wei)....Pages 135-163
Traumatic Retinal Detachment (Jinqiong Zhou, Nan Zhou, Wenbin Wei)....Pages 165-176
Pathologic Myopia and Retinal Detachment (Liqin Gao, Nan Zhou, Wenbin Wei)....Pages 177-189
Infectious Uveitis and Retinal Detachment (Liqin Gao, Nan Zhou, Wenbin Wei)....Pages 191-204
Uveitis and Retinal Detachment (Yao Huang, Nan Zhou, Wenbin Wei)....Pages 205-220
Ocular Tumor and Retinal Detachment (Jinqiong Zhou, Nan Zhou, Wenbin Wei)....Pages 221-251
Surgical Techniques of Rhegmatogenous Retinal Detachment and Complications After Surgeries (Haicheng She, Nan Zhou, Wenbin Wei)....Pages 253-262

Citation preview

Atlas of Retinal Detachment Diagnosis and Differential Diagnosis Wenbin Wei Editor

123

Atlas of Retinal Detachment

Wenbin Wei Editor

Atlas of Retinal Detachment Diagnosis and Differential Diagnosis

Editor Wenbin Wei Beijing Tongren Eye Center Beijing Tongren Hospital, Capital Medical University Beijing China

ISBN 978-981-10-8230-6    ISBN 978-981-10-8231-3 (eBook) https://doi.org/10.1007/978-981-10-8231-3 Library of Congress Control Number: 2018955196 © Springer Nature Singapore Pte Ltd. and Beijing Science and Technology Press 2018 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Singapore Pte Ltd. The registered company address is: 152 Beach Road, #21-01/04 Gateway East, Singapore 189721, Singapore

Preface

We once heard retinal specialists, both clinicians and scientists, share a fascination with the retina, a unique tissue in our body. The retina forms the anatomic and physiologic basis for the gift of sight and accounts for over 35% of the neurons entering and exiting the human brain. It is helpful to recall that although there had long been a fascination with the eye and sight, it was not until the nineteenth century that modern ophthalmology and the study of the retina truly began. Everyone in our field points with pride to the revolution brought about by von Helmholtz in 1850 with his introduction of his innovative ophthalmoscope in Berlin. In recent years, great progress has been made in the treatment of retinal detachment, and we hope and expect this is a prelude to even greater progress in the near future. Despite these new treatments and the explosion of knowledge on the basic mechanisms of retinal biology, the detachment of the retina remains to date a major cause of blindness in all age groups. In revising The Atlas of Retinal Detachment: Diagnosis and Differential Diagnosis edition, we begin with many pictures and imagings, where there is a veritable revolution underway. The human eye cannot resolve what advanced imaging technologies including ocular coherence tomography (OCT) can detect. Human retina is a direct outgrowth of the forebrain and, therefore, is the most accessible part of the brain for neuroscience studies. Many of us consider the retina to be aesthetically the most beautiful tissue in nature. We hope the reader will enjoy and appreciate the major revisions to the figures and illustrations throughout the text. The editors gratefully acknowledge the support of the contributing authors who, in addition to their large clinical load and scientific research efforts, found the time to make such a large contribution to the completion of this project. Beijing, China November 2017

Wenbin Wei

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Contents

1 Introduction of Retinal Detachment�����������������������������������������������������������������������    1 Kai Ma, Nan Zhou, and Wenbin Wei 2 Rhegmatogenous Retinal Detachment �������������������������������������������������������������������   13 Ruilin Zhu, Nan Zhou, and Wenbin Wei 3 Special Types of Rhegmatogenous Retinal Detachment���������������������������������������   33 Kai Ma, Nan Zhou, and Wenbin Wei 4 Exudative Retinal Detachment �������������������������������������������������������������������������������   67 Haiying Zhou, Nan Zhou, and Wenbin Wei 5 Congenital Abnormalities of the Fundus and Retinal Detachment���������������������  117 Yao Huang, Nan Zhou, and Wenbin Wei 6 Tractional Retinal Detachment�������������������������������������������������������������������������������  135 Lei Shao, Nan Zhou, and Wenbin Wei 7 Traumatic Retinal Detachment�������������������������������������������������������������������������������  165 Jinqiong Zhou, Nan Zhou, and Wenbin Wei 8 Pathologic Myopia and Retinal Detachment ���������������������������������������������������������  177 Liqin Gao, Nan Zhou, and Wenbin Wei 9 Infectious Uveitis and Retinal Detachment �����������������������������������������������������������  191 Liqin Gao, Nan Zhou, and Wenbin Wei 10 Uveitis and Retinal Detachment �����������������������������������������������������������������������������  205 Yao Huang, Nan Zhou, and Wenbin Wei 11 Ocular Tumor and Retinal Detachment�����������������������������������������������������������������  221 Jinqiong Zhou, Nan Zhou, and Wenbin Wei 12 Surgical Techniques of Rhegmatogenous Retinal Detachment and Complications After Surgeries�������������������������������������������������������������������������  253 Haicheng She, Nan Zhou, and Wenbin Wei

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Contributors and Editors

Contributors Haicheng She, M.D., Ph.D.  Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China Haiying Zhou, M.D., Ph.D.  Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China Jinqiong Zhou, M.D., Ph.D.  Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China Kai Ma, M.D., Ph.D.  Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China Lei  Shao, M.D., Ph.D. Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China Liqin  Gao, M.D., Ph.D. Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China Ruilin Zhu, M.D., Ph.D.  Peking University First Hospital, Beijing, China Yao  Huang, M.D., Ph.D.  Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China

Editors Wenbin Wei, M.D., Ph.D.  Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China Nan  Zhou, M.D., Ph.D. Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China Yang Lihong, M.D.  Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China Shi  Xuehui, M.D., Ph.D.  Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China

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List of Editors

Editor-In-Chief Wenbin Wei, M.D., Ph.D.  Director, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China

Deputy Editor-In-Chief Nan  Zhou, M.D., Ph.D. Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China Haicheng She, M.D., Ph.D.  Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China Ruilin Zhu, M.D., Ph.D.  Peking University First Hospital, Beijing, China

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1

Introduction of Retinal Detachment Kai Ma, Nan Zhou, and Wenbin Wei

Abstract

Retinal detachment is a common disease; this chapter mainly introduces the anatomy, histology, and embryology of retinal detachment, classification of retinal detachment, and the development and natural history of rhegmatogenous retinal detachments.

1.1

 natomy, Histology, and Embryology A of Retinal Detachment

1.1.1 Embryology and Development of the Retina The development of bulbus oculi involves the development of neuroectoderm, surface ectoderm, and mesoderm constituting blood vessels. The primary optic vesicle forms the optic cup, whose outer wall develops into the cellular layer of retinal pigment epithelium. Apart from the metabolism of visual cells, monolayer cells also keep the retina pellucid, acting as an outer barrier of the retina. The inner wall of the optic cup is highly differentiated forming the sensory cell layer of retina, including the structure of tertiary neuron [1]. The potential space between retinal pigment epithelium and the neuroepithelial layer is a weak area, prone to separate to cause retinal detachment. According to histological changes, retinal detachment should be termed separation within the retina, instead of separation of the retina from the choroid. However, the term of “retinal detachment” is still being used (Figs. 1.1, 1.2, and 1.3).

K. Ma, M.D., Ph.D. · N. Zhou, M.D., Ph.D. · W. Wei, M.D., Ph.D. (*) Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China

© Springer Nature Singapore Pte Ltd. and Beijing Science and Technology Press 2018 W. Wei (ed.), Atlas of Retinal Detachment, https://doi.org/10.1007/978-981-10-8231-3_1

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Fig. 1.1  Cross-sectional histologic preparations of the retina (×200)

A B

C

D

Fig. 1.2  Construct relationship between photoreceptor cells’ segments and retinal pigment epithelium revealed by electron microscope (×500). A, Photoreceptor cells’ segments. B, The potential space between the photoreceptor cells’ segments and retinal pigment epithelium. C, The nucleus of a retinal pigment epithelium. D, Bruch membrane

Fig. 1.3  Detachment between neurosensory retina and retinal pigment epithelium (×200). A, Interspace between detached neurosensory retina and retinal pigment epithelium. B, Ganglion cell layer

1  Introduction of Retinal Detachment

1.1.2 Blood Supply of the Retina The central retinal artery supplies the five inner layers of the retina, while the uveal ciliary arteries supply the four outer layers of the neurosensory retina and the retinal pigment epi-

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thelial layer. These two circulations are both terminal branches, which are ended at the outer plexiform layer and the ora serrata. Due to poor blood flow, these two areas are prone to degeneration and then lead to retinal detachment (Figs. 1.4, 1.5, 1.6, and 1.7).

Fig. 1.6  Cystoid degeneration in retinal plexiform layer Fig. 1.4  A normal ocular fundus image by Optos Panoramic200 scanning laser ophthalmoscope

Fig. 1.5  Lattice degeneration and thin retina area in the right eye’s peripheral inferior temporal quadrant

Fig. 1.7  Retinal detachment associated with retinal dialysis (×200). A, The large subretinal space. B, The filamentous connection between neurosensory retina and the ora serrata. C, The ciliary body

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1.1.3 T  he Interrelation Between the Vitreous and Retina The normal vitreous is transparent and gelatinous and provides support for the eyeball. It adheres firmly to the retina at the vitreous base, optic nerve head, and macula. Aging and myopia or other diseases can cause the vitreous to degenerate and liquefy. After certain extent of liquefaction, the vitreous detaches, especially in the posterior and/or

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superior quadrants (Figs. 1.8 and 1.9) [2]. Following acute vitreous detachment, vitreous traction can cause a retinal break due to retinal adhesion [3]. The retinal breaks are usually macular holes (Fig.  1.10) and horseshoe tears (Figs. 1.11 and 1.12), with flap adherent or signs of vitreous traction [4]. The liquid vitreous passes into the potential space between the neurosensory retina and retinal pigment epithelium through the retinal breaks and leads to retinal detachment.

Fig. 1.8  Posterior vitreous detachment. Annular opacity floater in the vitreous is observed before the optic nerve, the Weiss ring

Fig. 1.10  Optical coherence tomography (OCT) showing vitreoretinal traction of the retina causing the macular hole

Fig. 1.9  B-scan ultrasounds showing posterior vitreous detachment

1  Introduction of Retinal Detachment

Fig. 1.11  Vitreoretinal traction of the retina causes the horseshoe tear, rolled edge of retinal break

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Fig. 1.12  Rolled posterior edge of giant retina tear indicating vitreoretinal traction

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1.2

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Classification of Retinal Detachment

Retinal detachment is classified based on pathology, and different ­ treatment strategies apply accordingly. Rhegmatogenous retinal detachment is caused by retinal breaks, while non-rhegmatogenous retinal detachment is caused by other ocular disease or systemic disease.

1.2.1 Rhegmatogenous Retinal Detachment Rhegmatogenous retinal detachments are associated with congenital or acquired disease and the interaction of the vitreous and retina that led to retinal break and retinal detachment. It is sometimes referred to as primary or idiopathic retinal detachment. The key to the successful treatment for this type of retinal detachment is to seal retinal breaks.

1.2.2 Non-rhegmatogenous Retinal Detachment The two general categories of non-rhegmatogenous retinal detachments are termed tractional and exudative. Tractional retinal detachments are more common. It occurs due to the vitreoretinal adhesions or membranes that mechanically pull

the retina away after vitreoretinal hemorrhage of retinal ­vascular disease or trauma. Exudative detachments occur when ocular disease or systemic disease causes retinal vessels to leak and produce subretinal fluid.

1.2.2.1 Tractional Retinal Detachments The causes of tractional retinal detachments include cellular proliferation resulting from retinal hemorrhage and vitreous hemorrhage of retinal vascular disease, cicatricial contraction in trauma, intraocular foreign body extraction, and incision of surgery [5]. Usually tractional retinal detachments are without retinal tears. However, in few cases, contraction may tear off the retina to cause secondary rhegmatogenous retinal detachments. The key to treatment is eliminating the vitreoretinal or epiretinal traction. For wide tractional retinal detachments, vitrectomy may be required (Figs. 1.13 and 1.14). 1.2.2.2 Exudative Retinal Detachments Exudative retinal detachment is associated with systemic disease or ocular dysemia, such as nephropathy, pregnancyinduced hypertension, intraocular inflammation, tumor, Vogt-Koyanagi-Harada disease, sympathetic ophthalmia, Coats disease, bullous detachment of the retina, idiopathic central serous chorioretinopathy, uveal effusion syndrome, etc. The exudative subretinal fluid elevates the retina, which causes retinal detachment without retinal tears (Figs. 1.15, 1.16, 1.17, 1.18, 1.19, 1.20, and 1.21).

1  Introduction of Retinal Detachment

Fig. 1.13  Tractional retinal detachments with intraocular foreign body

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Fig. 1.14 Proliferative diabetic retinopathy and tractional retinal detachment

a

Fig. 1.15  Secondary retinal detachment with nephropathy

b

Fig. 1.16  Secondary retinal detachment with Vogt-Koyanagi-Harada disease. (a) Secondary exudative retinal detachment with Vogt-Koyanagi-­ Harada disease. (b) Terminal stage of Vogt-Koyanagi-Harada disease, photograph shows sunset glow fundus

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Fig. 1.17  Secondary retinal detachment with Coats disease Fig. 1.20  Secondary retinal detachment with choroidal hemangioma

Fig. 1.18  Idiopathic central serous chorioretinopathy Fig. 1.21  Secondary retinal detachment with choroidal melanoma

Fig. 1.19  Secondary retinal detachment with retinoblastoma

1  Introduction of Retinal Detachment

1.3

 he Development and Natural History T of Rhegmatogenous Retinal Detachments

1.3.1 Early Retinal Detachments The common symptoms of early rhegmatogenous retinal detachments are floaters (“bugs”), flashes of light, blurred vision, and vision field loss. The detachments are bulbiform or flat elevation of the retina, with certain mobility of vitreous and soft and moist appearance. After careful examination of the entire retina, retinal breaks could be detected (Fig. 1.22).

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1.3.2 Long-Standing Retinal Detachments 1. The retina is thinner and translucent in long-standing retinal detachments. Due to proliferation, the retina could be with wrinkling of inner retinal surface, retinal stiffness, and/or subretinal proliferation (Figs. 1.23 and 1.24). 2. In some cases, inferior detachments develop slowly. It could not be noticed until it affected central vision. Usually one to three curved demarcation lines in detachment area could be detected which are matched with the borders of retinal detachment, which mark the trail of detachment progress (Fig. 1.25). 3. Translucent and spherical intraretinal macrocysts may develop in long-standing retinal dialysis, due to degeneration of peripheral retina (Fig. 1.26).

Fig. 1.22  Early retinal detachments

Fig. 1.24  A long-standing retinal detachment with subretinal strands

Fig. 1.23  A relatively early stage of retinal detachment

Fig. 1.25  Retinal detachment associated with inferior temporal retinal dialysis. As demonstrated, there are subretinal pigmented demarcation lines

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1.3.3 A  ppearance Following Reattachment of Retinal Detachments After the seal of retinal tears and resorption of subretinal fluid (Figs. 1.27, 1.28, 1.29, and 1.30), the reattachment area is dark and gray due to alteration of pigment granules, which is distinctly different with the normal retina.

Fig. 1.28  An annular crest of the buckle. After scleral buckling, the retina reattached on the posterior visible crest of the buckle

Fig. 1.26  An intraretinal cystoid in detached retina with retinal breaks

Fig. 1.29  An annular crest of scleral buckling in B-scan ultrasounds

Fig. 1.27  After scleral buckling, the retina reattached on the posterior visible crest of the buckle

Fig. 1.30  An annular crest of scleral buckling in B-scan ultrasounds

1  Introduction of Retinal Detachment

References 1. Graw J. Eye development. Curr Top Dev Biol. 2010;90:343–86. 2. Okun E. Histopathology of changes which precede retinal detachment. Bibl Ophthalmol. 1966;70:76–97. 3. Machemer R.  The importance of fluid absorption, traction, intraocular currents, and chorioretinal scars in the therapy of rhegmatog-

11 enous retinal detachments. XLI Edward Jackson memorial lecture. Am J Ophthalmol. 1984;98(6):681–93. 4. Foos RY.  Tears of the peripheral retina; pathogenesis, incidence and classification in autopsy eyes. Mod Probl Ophthalmol. 1975;15:68–81. 5. Machemer R, Aaberg TM, Freeman HM, et  al. An updated classification of retinal detachment with proliferative vitreoretinopathy. Am J Ophthalmol. 1991;112(2):159–65.

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Rhegmatogenous Retinal Detachment Ruilin Zhu, Nan Zhou, and Wenbin Wei

Abstract

Rhegmatogenous retinal detachment (RRD) is the most common type of retinal detachment. Different types of retinal degeneration may lead to retinal detachment. The shape and location of retinal breaks and detachment are various in different patients. In this chapter, we provide a large number of fundus photographs and diagrams of retinal degeneration, retinal breaks and detachment. With these pictures, the lesions are showed more clearly and distinctly.

2.1

Introduction

Retinal detachment (RD) is the separation of the neurosensory retina from the retinal pigment epithelium (RPE). Some detachments are secondary to ocular or systemic disease, while others do not have a distinct etiology. The most common type of RD is primary RD, in which there is no obvious original disease. The retina detaches with a full-thickness retinal break and vitreous degeneration, so it is usually called as rhegmatogenous retinal detachment (RRD). Previous studies have reported the incidence rate of RRD ranging from 9.7 to 20.7 per 100,000 persons per year [1–5]. The peak incidence is at 50–69  years of age, with a secondary peak at 20–29 years [3, 6]. Men are more likely to be affected [1–3]. Rates of bilateral RRD vary between 1% and 1.67%, increasing with study duration [4, 5, 7]. Risk factors for RRD include male gender, trauma, pseudophakic eye, and myopia [3, 8].

R. Zhu, M.D., Ph.D. Peking University First Hospital, Beijing, China N. Zhou, M.D., Ph.D. · W. Wei, M.D., Ph.D. (*) Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China

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2.2

 etinal Degeneration Related R to Retinal Detachment

Peripheral retinal degeneration includes lattice degeneration (Fig. 2.1), paving-stone degeneration, and retinal white without pressure (Fig. 2.2a, b). Lattice degeneration is the most significant peripheral retinal disorder predisposing to RD. Lattice degeneration is present in 5.2%–12.2% of the healthy population [9–12], in 30% of RD patients [13], and in 12.2%–13.6% of high myopic patients [12, 14]; 61.3% of the lesions occur in both eyes [9]. The temporal quadrant is the most affected area, especially the lower temporal quadrant, followed by the upper temporal quadrant, lower nasal quadrant, and upper nasal quadrant [15]. Lattice degeneration is most commonly located near the vertical meridian between 11 and 1 o’clock and between 5 and 7 o’clock [16], with its long axis parallel to the ora serrata. The width of the lesion has been reported to be 1/4 disc diameter (DD)–2/3 DD and the length to be 1/2 DD–12 DD [15]. The lesions are normally round-, oval-, or linear-shaped retinal thinning zone, with a distinct and slightly elevated border and rough surface. About 81.7%–92% of the degeneration shows pigment clumps underneath retinal blood vessels. White lines can be seen in 12% of the lesions [15], indicating the occlusion of blood vessels; these lines appear in the late stage of the disease, while in the early stage the vessels are con-

a

Fig. 2.2 (a, b) Retinal white without pressure

stricted (Fig. 2.3a, b). Vitreous degeneration associated with lattice degeneration includes vitreous liquefaction, changes in vitreous concentration, vitreous detachment, and vitreoretinal traction. Atrophic holes within lattice lesions have been found in 18.2%–24.9% of eyes, and tractional retinal tears have been found in 1.5%–2.4% of them [15] (Fig. 2.4a, b).

Fig. 2.1  Lattice degeneration in inferior temporal quadrant of right eye. Multiple atrophic holes reside in the degenerated lesion. Pigment change and yellowish dots can be seen in the lesion. Blood vessels constricted and appear as white lines

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Fig. 2.3 (a, b) Lattice degeneration. Blood vessels constricted and appear as white lines. A horse-shoe shaped retinal tear can be seen near the lesion with retinal detachment

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Fig. 2.4 (a, b) Lattice degeneration. (a) A horse-shoe shaped retinal tear can be seen on the right side of the lesion with retinal detachment. Pigment change and yellowish dots can be seen in the lesion. Blood

b

vessels constricted and appear as white lines. (b) Multiple atrophic holes reside in the degenerated lesion

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2.3

Retinal Breaks

With improvements in clinical practice, the pathogenesis of RRD is now better understood. The key point in the treatment of RRD is to close the retinal breaks and then to permanently seal them with retinopexy.

2.3.1 Shapes of Retinal Breaks 2.3.1.1 Round Hole Regional retinal tissue degeneration and thinning lead to atrophic retinal holes. The holes are usually round or oval,

a

with a distinct margin. The RPE and the red background of the uvea can be seen through the holes. In some cases, the retinal tissue is tilted up at the edge of the hole, and in some cases the retinal tissue on the surface of the hole is detached. The size and shape of the operculum coincides with the hole. Retinal holes are usually less than 1/4 DD and are seldom larger than 1 DD.  The holes can be solidary, multiple, or clustered. It has been reported that in 85% of patients who had round holes the fellow eye was affected [17]. About 95.4% of the atrophic holes are associated with lattice degeneration [18]. Round-hole retinal detachments accounted for 10% of primary retinal detachments [17, 19] (Figs.  2.5a–c and 2.6a–c).

b

c

Fig. 2.5 (a–c) Round holes. (a) Idiopathic macular hole in left eye. (b) Round retinal hole at peripheral retina of right eye. (c) Round retinal hole with subretinal proliferation and retinal detachment

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Fig. 2.6  Multiple round retinal holes. (a) Multiple round retinal holes with retinal detachment. (b) Multiple round retinal holes. (c) Laser photocoagulation was applied to seal the retinal holes

2.3.1.2 Horseshoe Tear Traction of a vitreoretinal adhesion leads to a retinal tear, with a horseshoe, crescent, or “U” shape. The shape of the tear is related to the magnitude and direction of the tractive force. Since the stress point resides at the peripheral retina, the basal part of a horseshoe tear always points toward the peripheral retina, and the acme points to the macula (Figs. 2.7a–c and 2.8a, b). Horseshoe tears account for 23.1% of the total number of breaks, and 60.2% of them are tears with attached flaps, while 39.8% are tears with free opercula [18]. The tears are usually larger than 1 DD, and are located at the equator or peripheral retina, near the junction of the diseased and healthy retina. Retinal horseshoe tears accounted for 70%–86.3% of the total number of RRDs [19, 20]. The retinal blood vessels can be involved and lead to vitreous hemorrhage (Fig. 2.9a–d). In cases of sudden vitreous hemorrhage in patients without hypertension, diabetes, or other ocular vessel disorders, one must investigate whether a retinal tear has caused the hemorrhage, and ultrasonography is recommended in such cases.

2.3.1.3 Retinal Dialysis Retinal dialysis, also known as ora serrata dialysis, is the disinsertion of the retina along the ora serrata. It has been reported that retinal dialysis was found in 5.9%–8.2% of RD cases [20–22]. Vitreous base backward traction results in retinal dialysis at the ora serrata. The anterior edge of the dialysis is at the ora serrata and is without a front edge, while the posterior edge is thickened and curved, and usually attached to the vitreous base. The extent of the dialysis varies from one quadrant up to 360° (Fig. 2.10a–c). Dialysis at the ora serrata usually occurs after severe blunt contusion trauma to the globe. Most of the cases are unilateral, while bilateral cases have been reported accounting for 5%–10.3% of the patients [21, 23]. As the result of a blunt blow to the eyeball, the retina detaches from the ora serrata, and avulsion of the nonpigmented ciliary epithelium or vitreous occasionally occurs.

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Fig. 2.7 (a–c) Horseshoe shaped retinal tear with retinal detachment

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Fig. 2.8 (a, b) Horseshoe shaped retinal tear treated by laser photocoagulation. Laser burns can be seen around the tear

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Fig. 2.9 (a–d) Retinal tear with retinal detachment. A blood vessel crosses over the edge of the retinal tear

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Fig. 2.10 (a–c) Ora serrata dialysis with retinal detachment. The posterior edge is thickened and curved

2.3.2 Sizes of Retinal Breaks Retinal breaks are of various sizes, from as small as a pinhole to all around the retina. Atrophic holes are usually small and multiple, and tractional tears are usually large and solitary. A break that extends circumferentially around the retina

for 90° or more is also called a giant retinal tear, and is usually located at the peripheral retina [24]. Giant retinal tears are usually caused by vitreous traction, and the posterior edge of the tears curl. Severe RD in the peripheral region often causes giant ora serrata dialysis. These two kinds of retinal breaks can both extend up to 360° (Fig. 2.11a–i).

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Fig. 2.11 (a–i) Retinal breaks with different sizes. (a) Retinal break with retinal detachment. (b) Large retinal break with pigmentation at the edge of the break and vitreous traction. (c) A large retinal break and a small one beside it. (d) A large horseshoe shaped retinal tear with retinal detachment. The edge of the tear was curved. (e) A large horseshoe

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b

d

f

shaped retinal tear with retinal detachment. (f) A large retinal break treated with laser photocoagulation. The laser burns can be seen around the tear. (g) Giant retinal break. The posterior edge of the break was curved. Retina was detached. (h) Giant retinal break. (i) Giant retinal break

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g

i

Fig 2.11 (continued)

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2  Rhegmatogenous Retinal Detachment

2.3.3 Distribution of Retinal Breaks The common sites of retinal breaks include the equator of the eyeball and the temporal quadrant. The superotemporal quadrant is the most frequent location of retinal breaks. It has been reported that 69% of retinal breaks were found in the superotemporal quadrant, 40% in the superonasal quadrant, 32% in the inferotemporal quadrant, and 17% in the ­inferonasal quadrant [19]. Solitary breaks were found in 38.9% of cases of retinal breaks, and 58.8% of cases had breaks in more than one quadrant [19]. In retinal dialysis cases, the inferotemporal quadrant was the most likely involved quadrant (82%). The superotemporal and superonasal quadrants each accounted for 7%, and the inferonasal quadrant accounted for 3% [25].

2.4

Retinal Detachment

The normal retina is a transparent membranous tissue. The appearance of a detached retina varies according to the extent, location, and duration of detachment, as well as the amount of subretinal fluid. In patients whose retinal detachment is shallow, with clear subretinal fluid, the pinkish pigment epithelium can be seen through the retina, but the structure of the choroid cannot be seen clearly, and a vascular shadow can sometimes be seen. In the acute phase, the detached retina loses transparency and turns gray, and is often recognized by a dune-like appearance and slight movement with eye movement. The reflection of the retinal ves-

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sels disappear, and the dark-red vessels on the detached retina are tortuous. With the progression of the disease, the retinal degeneration and vitreoretinal proliferation result in retinal wrinkling and stiffness (Fig. 2.12a–f). The shape of the detachment depends on the location of the retinal breaks. The retinal detachment usually starts at the break site and extends to the ora serrata and optic nerve disc. When RD is confined to one superior quadrant, the retinal break could be in the same area. Detachment involves two superior quadrants prompts breaks in the peripheral retina at 11:00–1:00 o’clock. Inferior RD with equal heights on the two sides of the disc indicates a break at 6:00 o’clock. When the detachment height of the two sides is not equal, the breaks usually correspond with the higher side of the detachment. When an inferior RD is bullous, the breaks are usually in the superior quadrant, with shallow detachment [26, 27]. Detachment on the nasal or temporal side indicates breaks at 1–2 clock hours inferior to the edge of the upper detachment. Posterior pole or inferior detachment may show a break at the macula. Peripheral detachment with a retinal cyst often indicates small dialysis of the ora serrata or breaks in an adjacent area. In some slowly progressive RDs of long duration, a black line parallel to the edge of the detachment is determined as a demarcation line or high-water mark; retinal breaks may reside within the detachment area. In some chronic forms of RD, retinal cysts of one or more papillary diameter (PD) in size could be detected (Figs. 2.13a–d and 2.14a–f). In some cases, RDs are combined with ciliary body or choroidal detachment.

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b

a

c

d

f e

Fig. 2.12 (a–f) The various appearance of detached retina. (a) Retinal detachment. The detached retina shaped like a dome. (b) Retinal detachment. The macula was involved. (c) Retinal detachment. The

retina was wrinkle. (d) Retinal detachment. The macula was involved. (e) Horseshoe retinal tear with retinal detachment. The retina was wrinkle and stiff. (f) Retinal detachment with full-thickness retinal folds

2  Rhegmatogenous Retinal Detachment

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Fig. 2.13 (a–d) The relationship of retinal detachment and location of retinal breaks

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a

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Fig. 2.14 (a–f) Distribution of retinal breaks. (a) Superior retinal detachment with a retinal tear at the superior temporal quadrant. (b) Superior retinal detachment with a retinal tear at the superior temporal quadrant. (c) Superior nasal quadrant retinal detachment with a retinal

break within the detached area. (d) Temporal retinal detachment with a retinal break at the peripheral temporal retina. (e) Inferior retinal detachment with multiple retinal breaks at the inferior retina. (f) Inferior nasal retinal detachment with a retinal tear in the detached area

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2.5

Proliferative Vitreoretinopathy

Proliferative vitreoretinopathy (PVR) is a complication of RRD.  Fibrous membranes proliferate on the surface of the detached retina, in the subretinal space, and on the posterior surface of the detached vitreous. Contraction of the membranes causes retinal distortion and fixed retinal folds. The fixed folds make it difficult to close the retinal breaks and lead to failure of RD surgery. PVR is estimated to occur in 5%–10% of all RD cases [28]. Risk factors for PVR include uveitis, large tears, long duration of detachment, vitreous hemorrhage, choroidal detachments, aphakia, multiple previous surgeries, and extensive RD [29, 30]. The surgical outcome is good in mild PVR cases, while vitrectomy is often necessary in severe cases. Therefore, classification of the PVR is crucial for ophthalmologists to determine the severity of the disease, select treatment strategies, and assess surgical prognosis.

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2.5.1 Classification 2.5.1.1 The Retinal Society Terminology Committee PVR Classification (1983) [31] Grade A B

Name Minimal Moderate

C CCCD DD-

Marked 1 2 3 Massive 1 2

D-3

3

Clinical signs Vitreous haze, vitreous pigment clumps Wrinkling of the inner retinal surface, rolled edge of retinal break, retinal stiffness, vessel tortuosity Full-thickness retinal folds One quadrant Two quadrants Three quadrants Fixed retinal folds in four quadrants Wide funnel shape Narrow funnel shape (anterior end of the funnel can be seen by indirect ophthalmoscopy within the 45° field of a +20 diopter (D) condensing lens) Closed funnel (optic nerve head not visible)

With improvements in the understanding of PVR and with the achievement of therapeutic advances, the Retina Society updated the classification of PVR in 1991. The new classification retained grades A and B, modified grade C, and eliminated grade D.

2.5.1.2 Updated PVR Classification (1991) [32] Grade A B

CP 1–12 CA 1–12

Features Vitreous haze, vitreous pigment clumps, pigment clusters on inferior retina Wrinkling of the inner retinal surface, retinal stiffness, vessel tortuosity, rolled and irregular edge of retinal break, decreased mobility of vitreous Posterior to equator: focal, diffuse, or circumferential full-thickness foldsa, subretinal strandsa Anterior to equator: focal, diffuse, or circumferential full-thickness foldsa, subretinal strandsa, anterior displacementa, condensed vitreous with strands

Expressed in the number of clock hours involved

a

See Figs. 2.15, 2.16, 2.17a, b, and 2.18.

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Fig. 2.15  PVR C1. Focal full-thickness retinal folds posterior to equator Fig. 2.16  PVR D2. Diffuse full-thickness retinal folds posterior to equator

a

b

Fig. 2.17 (a, b) PVR D1. (a) Subretinal napkin-ring proliferated membrane. (b) Linear subretinal proliferated membrane

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2.5.2 Subretinal Proliferation In some RD cases, RPE cells, retinal neovascular adventitial cells, ciliary horizontal cells, and choroidal and episcleral connective tissue cells proliferate, contract, and form a cellular membrane. The formation and development of the membrane leads to retinal and vitreous fibrosis. The membranes generated by astrocytes and RPE cells through a defect of the inner limiting membrane are termed preretinal membranes. Contraction of these membranes causes deformation of the retinal inner layer, macular folds, retinal breaks with rolled edges, star folds, and irregular fixed folds. The subretinal membrane formed by retinal glial cells and RPE cells appear as a thick white rigid band, patch, or dendritic structure. Fixed retina presents a linear or napkin-ring appearance. Detached deep retinal yellowish granules are seen; these are depigmented RPE cells (Fig. 2.19a–f).

Fig. 2.18  PVR D. The retina circularly restrained and formed radial retinal folds

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a

c

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f e

Fig. 2.19 (a–f) Long-lasting retinal detachment with subretinal fibrosis

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2.6

Retinal Cysts

Intraretinal cysts are usually associated with long-standing RD due to the nutrition disorder of the detached retina (Fig. 2.20a). Retinal cysts mainly occur secondarily as pseudocysts without an epithelium lining. The cysts are normally a

c

Fig. 2.20 (a–c) Retinal detachment with retinal cyst

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oval or spherical, 1–8 DD in size, and may be solitary or multiple; they are independently located in the peripheral retina, are not involved in the ora serrata, and are mostly seen in the inferior temporal quadrant [33, 34]. The cysts are immobile. The walls of the cysts are thin and transparent, without breaks (Fig. 2.20a–c). b

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References 1. Poulsen CD, Peto T, Grauslund J, et al. Epidemiologic characteristics of retinal detachment surgery at a specialized unit in Denmark. Acta Ophthalmol. 2016;94(6):548–55. 2. Howie AR, Darian-Smith E, Allen PL, et  al. Whole population incidences of patients presenting with rhegmatogenous retinal detachments within Tasmania, Australia. Clin Exp Ophthalmol. 2016;44(2):144–6. 3. Chen SN, Lian Ie B, Wei YJ.  Epidemiology and clinical characteristics of rhegmatogenous retinal detachment in Taiwan. Br J Ophthalmol. 2016;100(9):1216–20. 4. Hajari JN, Bjerrum SS, Christensen U, et  al. A nationwide study on the incidence of rhegmatogenous retinal detachment in Denmark, with emphasis on the risk of the fellow eye. Retina. 2014;34(8):1658–65. 5. Van de Put MA, Hooymans JM, Los LI.  The incidence of rhegmatogenous retinal detachment in The Netherlands. Ophthalmology. 2013;120(3):616–22. 6. Park SJ, Choi NK, Park KH, et al. Five year nationwide incidence of rhegmatogenous retinal detachment requiring surgery in Korea. PLoS One. 2013;8(11):e80174. 7. Zhou JQ, You QS, Tu Y, et al. Clinical features of bilateral rhegmatogenous retinal detachments. Acta Ophthalmol. 2013;91(8):e654–5. 8. Feltgen N, Walter P. Rhegmatogenous retinal detachment—an ophthalmologic emergency. Dtsch Arztebl Int. 2014;111(1–2):12–21; quiz 22. 9. Semes LP, Holland WC, Likens EG.  Prevalence and laterality of lattice retinal degeneration within a primary eye care population. Optometry. 2001;72(4):247–50. 10. Yura T. The relationship between the types of axial elongation and the prevalence of lattice degeneration of the retina. Acta Ophthalmol Scand. 1998;76(1):90–5. 11. Wilkinson CP.  Interventions for asymptomatic retinal breaks and lattice degeneration for preventing retinal detachment. Cochrane Database Syst Rev. 2014;(9):Cd003170. 12. Lam DS, Fan DS, Chan WM, et  al. Prevalence and characteristics of peripheral retinal degeneration in Chinese adults with high myopia: a cross-sectional prevalence survey. Optom Vis Sci. 2005;82(4):235–8. 13. Dumas J, Schepens CL. Chorioretinal lesions predisposing to retinal breaks. Am J Ophthalmol. 1966;61(4):620–30. 14. Lai TY, Fan DS, Lai WW, et al. Peripheral and posterior pole retinal lesions in association with high myopia: a cross-sectional community-based study in Hong Kong. Eye (Lond). 2008;22(2):209–13. 15. Byer NE.  Lattice degeneration of the retina. Surv Ophthalmol. 1979;23(4):213–48.

R. Zhu et al. 16. Lewis H.  Peripheral retinal degenerations and the risk of retinal detachment. Am J Ophthalmol. 2003;136(1):155–60. 17. Ung T, Comer MB, Ang AJ, et  al. Clinical features and surgical management of retinal detachment secondary to round retinal holes. Eye (Lond). 2005;19(6):665–9. 18. Byer NE.  The natural history of asymptomatic retinal breaks. Ophthalmology. 1982;89(9):1033–9. 19. Shunmugam M, Shah AN, Hysi PG, et al. The pattern and distribution of retinal breaks in eyes with rhegmatogenous retinal detachment. Am J Ophthalmol. 2014;157(1):221–6.e221. 20. Mitry D, Singh J, Yorston D, et al. The predisposing pathology and clinical characteristics in the Scottish retinal detachment study. Ophthalmology. 2011;118(7):1429–34. 21. Vote BJ, Casswell AG. Retinal dialysis: are we missing diagnostic opportunities? Eye (Lond). 2004;18(7):709–13. 22. Jan S, Hussain Z, Khan U, et  al. Retinal detachment due to retinal dialysis: surgical outcome after scleral buckling. Asia Pac J Ophthalmol (Phila). 2015;4(5):259–62. 23. Stoffelns BM, Richard G. Is buckle surgery still the state of the art for retinal detachments due to retinal dialysis? J Pediatr Ophthalmol Strabismus. 2010;47(5):281–7. 24. Shunmugam M, Ang GS, Lois N.  Giant retinal tears. Surv Ophthalmol. 2014;59(2):192–216. 25. Kennedy CJ, Parker CE, McAllister IL. Retinal detachment caused by retinal dialysis. Aust N Z J Ophthalmol. 1997;25(1):25–30. 26. Saxena S, Lincoff H. Finding the retinal break in rhegmatogenous retinal detachment. Indian J Ophthalmol. 2001;49(3):199–202. 27. Lincoff H, Gieser R.  Finding the retinal hole. Arch Ophthalmol. 1971;85(5):565–9. 28. Pastor JC, Rojas J, Pastor-Idoate S, et  al. Proliferative vitreoretinopathy: a new concept of disease pathogenesis and practical consequences. Prog Retin Eye Res. 2016;51:125–55. 29. Khan MA, Brady CJ, Kaiser RS. Clinical management of proliferative vitreoretinopathy: an update. Retina. 2015;35(2):165–75. 30. Nagasaki H, Shinagawa K, Mochizuki M.  Risk factors for proliferative vitreoretinopathy. Prog Retin Eye Res. 1998;17(1):77–98. 31. The Retina Society Terminology Committee. The classifica tion of retinal detachment with proliferative vitreoretinopathy. Ophthalmology. 1983;90(2):121–5. 32. Machemer R, Aaberg TM, Freeman HM, et  al. An updated classification of retinal detachment with proliferative vitreoretinopathy. Am J Ophthalmol. 1991;112(2):159–65. 33. Kumar V, Vivek K, Chandra P, et  al. Ultrawide field imaging of multiple intraretinal cysts in old rhegmatogenous retinal detachment. Oman J Ophthalmol. 2016;9(3):191–2. 34. Verdaguer P, Nadal J.  Intraretinal cyst secondary to longstanding retinal detachment. Eur J Ophthalmol. 2012;22(3):506–8.

3

Special Types of Rhegmatogenous Retinal Detachment Kai Ma, Nan Zhou, and Wenbin Wei

Abstract

This chapter introduces several special types of rhegmatogenous retinal detachment; namely, retinal detachments with dialyses of the ora serrata, retinal detachment with giant retinal tear, retinal detachments with severe proliferative vitreoretinopathy, retinal detachment with choroidal detachment, congenital coloboma of the choroid with retinal detachment, retinal detachment with Marfan syndrome, and retinal detachment after vitreoretinal surgery. Particulars of the etiology, differential diagnosis, and surgery of each type are outlined.

3.1

 etinal Detachments with Dialyses R of the Ora Serrata

3.1.1 Definition The ora serrata is the junction between the retina-choroid and the ciliary epithelium, and is also known as the vitreous symphysis. Retinal breaks that occur along the posterior edge of the ora serrata are called retinal dialyses of the ora serrata [1, 2].

3.1.2 Etiology and Pathogenesis 3.1.2.1 Ocular Trauma Etiology The most common cause of retinal detachments with dialyses of the ora serrata is blunt ocular trauma [3, 4]. According to research reports, about one third of retinal detachments are associated with trauma factors, 74%–86% of which are blunt trauma, which causes dialyses of the ora serrata. Pathogenesis Retinal dialyses are thought to result from the avulsion of the vitreous base on the retinal ora serrata, when the globe expands equatorially under longitudinal external compression of the eyeball. Within a few days or months after the injury, the liquefied vitreous accumulates under the retina through the dialyses, causing acute or chronic retinal detachments [1, 5]. K. Ma, M.D., Ph.D. · N. Zhou, M.D., Ph.D. W. Wei, M.D., Ph.D. (*) Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China

3.1.2.2 Other Factors A positive family history and bilaterality of the retinal detachments imply that the dialyses of the ora serrata are associated with developmental or genetic factors.

© Springer Nature Singapore Pte Ltd. and Beijing Science and Technology Press 2018 W. Wei (ed.), Atlas of Retinal Detachment, https://doi.org/10.1007/978-981-10-8231-3_3

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3.1.3 Diagnosis 3.1.3.1 Clinical Presentation General Situation Retinal dialyses of the ora serrata are most common in the young. The most common location is in the inferior temporal quadrant, followed by the nasal or temporal superior quadrant. Inferior retinal detachments develop slowly [5]. Most cases are not diagnosed until the detachment involves the fovea and affects central vision. Fundus Retinal detachments can be partial or total. One or several slitlike or flat oval retinal holes can be observed in the corresponding retinal detachments. The anterior edges of the retinal holes are located at the ora serrata (Fig.  3.1). Demarcation lines and intraretinal macrocysts often occur in long-term (over 3 months) retinal detachments (Fig. 3.2). The demarcation lines are gray or pigmented, concave to parts of the ora serrata, and can be multiple. Intraretinal ­macrocysts often develop around the ora serrata. Pigment clumps may be observed at the front of the vitreous in long-term cases. Giant retinal tears rarely happen in this type of retinal detachment; when they do occur, they are mostly seen in high myopia, and are usually associated with avulsion of the vitreous base and detachment of the ciliary epithelium.

Fig. 3.1  Retinal detachments with dialyses of the ora serrata. This case is an inferior retinal detachment with dialyses of the ora serrata. At first, there were three adjacent holes with an asymptomatic subclinical retinal detachment (left). Subsequently, the retinal holes merged into a typical sign of ora serrata with inferior retinal detachment (right)

K. Ma et al.

Classification Schepens divided it into two types according to clinical prognosis [6]. 1. Benign type: The retinal dialysis is less than 90°; it is located in the inferior temporal quadrant, and is normally without posterior vitreous detachment. The retinal detachments are limited and progress slowly, with the vitreous cortex covering the ora serrata. 2. Malignant type: The retinal dialysis is more than 90°, mainly in the superior temporal quadrant, and it can be associated with liquefaction and traction of the v itreous, as well as with the concentration of the ­ ­v itreous [6]. Points of Diagnosis The anterior edge of the retinal dialysis is located at the ora serrata, while the posterior edge without or slightly with rolled edges. A fundus examination with binocular indirect ophthalmoscopy under scleral depression is essential to identify the ora serrata.

3.1.3.2 Medical Imaging B-type ultrasonic examination helps to identify localized retinal detachment and dialyses of the ora serrata, by showing discontinuity of the retinal image.

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Fig. 3.2  The 30-year-old male whose images are shown here has an inferior temporal retinal detachment in the left eye, with shallow detachments of the macular fovea. A large retinal cyst, with a gray translucent appearance, can be observed at the peripheral retina in the inferior temporal quadrant,. Such patients, if they have no pupillary dilation, can easily be misdiagnosed as having central serous chorioretinopathy

3.1.4 Differential Diagnosis 3.1.4.1 Peripheral Retinal Breaks Some retinal breaks occur near the ora serrata, without a specific tendency in any quadrant. The retinal breaks are in various forms, such as horseshoe with flap, irregular shape with or without flap, and round with or without free opercula (round retinal holes are commonly located in areas of retinal atrophy and degeneration) (Fig. 3.3).

3.1.4.2 Giant Retinal Tears Giant retinal tears are most common in myopia, especially in high myopia [7]; they may be caused by blunt ocular trauma, but generally occur spontaneously, and are occasionally associated with previous choroidal and retinal lesions (degeneration or excessive cryotherapy and laser photocoagulation). A giant retinal tear is in excess of 90° around the peripheral retina (Fig. 3.4). The anterior edge of the retinal tear is located at the posterior vitreous base, while the posterior edge is usually rolled back to the posterior pole of the fundus; this rolling back is most likely to occur with proliferative vitreoretinopathy (PVR). Giant retinal tears are rarely seen with avulsion of the vitreous base and detachment of the ciliary epithelium, except in severe ocular trauma.

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Fig. 3.3  Peripheral retinal breaks. This 14-year-old male has the secondary RRD from FEVR, an oval-shaped break of the peripheral superior temporal retina. The posterior edge of the retinal detachment is located between the papilla and fovea

Fig. 3.4  A 15-year-old male has an inferior temporal retinal detachment from a giant retinal tear in the right eye. The posterior edge of the retinal tear is rolled back, while the anterior edge is still at the peripheral retina

3  Special Types of Rhegmatogenous Retinal Detachment

3.2

 etinal Detachment with R Giant Retinal Tear

3.2.1 Definition A retinal break in excess of 90° around the retinal circumference is considered to be a giant retinal tear. Owing to the extent of the retinal break, the rolled posterior edge of the retinal tear, and the pronounced tendency to show PVR, a giant retinal tear is regarded as a complex rhegmatogenous retinal detachment (Fig. 3.5).

3.2.2 Etiology and Pathogenesis Primary giant retinal breaks with the appearance of avulsion are most likely to occur at the posterior vitreous base, and are associated with marked vitreous degeneration and constriction of the vitreous base; on histology, they are also related to a firm attachment between the vitreous base and the retina [6, 7].

Fig. 3.5  Primary giant retinal breaks. A giant retinal tear is most likely to occur in the posterior area of the vitreous base, while both ends of the hole are often combined with a radial tear. As the posterior edge is rolled back, a large area of retinal pigment epithelium is exposed

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3.2.3 Diagnosis 3.2.3.1 Clinical Presentation General Situation Primary giant retinal breaks mostly occur in young adults. There is a significant gender difference, with approximately 80% of the detachments occurring in males. These breaks are common in myopia, with an incidence of 80%, especially in high myopia (40%). Patients with a giant retinal tear can ultimately develop detachment in the fellow eye. Fundus Continuous marked contraction of the vitreous base occurs while the posterior vitreous is liquefied and collapsed, forming a giant zone of vitreous liquefaction. A giant retinal tear is most likely to occur in the posterior area of the vitreous base, and both ends of the hole are often combined with a radial tear. As the posterior edge is rolled back, a large area of retinal pigment epithelium is exposed. As the vitreous constantly contracts, it subsequently detaches the retina. A giant retinal tear with PVR shows the extensive presence of preretinal or subretinal membranes (retinal folds). An obvious preretinal membrane is most likely to be located at the posterior edge of the retinal break, but these membranes can also be located at the inferior and superior ends of the retinal break. These retinal folds may be circumferential or meridional (Figs. 3.6, 3.7, and 3.8).

3.2.3.2 Surgery According to the clinical characteristics of a giant retinal tear, the goal of surgery is to unfold and flatten the posterior flap of the tear. Before the 1980s, there were several treatments, such as retinal insertion, suture fixation through the sclera or vitreous, and fixation of retinal nails [6]. However, these treatments were rarely successful. Modern surgical procedures for retinal detachment include vitrectomy and a stripping operation, using perfluorooctane to unfold the posterior edge of the retinal tear; laser photocoagulation; expanding gas; and silicone oil tamponade, the last of which is now regarded as the best treatment for giant retinal detachment, with surgical success rates of over 95% (Figs. 3.9, 3.10, and 3.11).

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a

Fig. 3.6  Giant retinal tear in excess of 180°. In cases of superior or giant retinal tears, the peripheral retina can be torn and folded over itself, with the posterior edge of the retinal break now draped over the papilla, fovea, and the inferior retina

b

Fig. 3.7  Giant retinal tear in excess of 270°. The edge of the retinal tear is completely torn and folded over, covering the inferior retina. In this patient, implantation of four titanium nails was performed in the 1980s. In the surgery, the retina was reattached after the detached retina had been completely expanded. However, the loss of a superior titanium nail affected the complete reattachment of the retina. (a) Before the surgery. (b) After the surgery

3  Special Types of Rhegmatogenous Retinal Detachment Fig. 3.8  Giant retinal tear in a patient with severe peripheral vascular disease. (a) The giant retinal tear with proliferative vitreoretinopathy (PVR) shows the extensive presence of preretinal and subretinal membranes. Preretinal membranes are obvious, located at the posterior edge of the retinal break and at the inferior and superior ends of the retinal break. These retinal folds are circumferential and meridional. (b) Retinal reattachment following vitrectomy. (c) After the removal of silicone oil, the retina maintained reattachment, with visual acuity (VA) of 0.7

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a

b

c

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a

b

c

d

Fig. 3.9  Bilateral retinal detachments with giant retinal tears. The patient was an 11-year-old with severe PVR. Vitrectomy and silicone oil tamponade were performed in both eyes. A cataract occurred in the left eye. Four months after the surgery, the silicone oil was removed. The retina was reattached, and phacoemulsification and intraocular lens implantation were performed. VA: right eye, 0.3; left eye, 0.05. (a) Fundus image of left eye before the surgery. (b) Fundus image of right eye before the surgery. (c) Fundus image of right eye

before the surgery. Proliferation can be seen around the edge of the retinal tear. (d) Fundus image of left eye after vitrectomy and silicone oil tamponade. The retina was reattached. (e) Fundus image of right eye after vitrectomy and silicone oil tamponade. The retina was reattached. (f) Fundus image of left eye after removal of the silicone oil; owing to the cataract, the image was blurred. The retina was reattached. (g) Fundus image of right eye after removal of the silicone oil. The retina was reattached

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e

g

Fig. 3.9   (continued)

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f

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a

a

b

b

c

Fig. 3.10  Traumatic retinal detachments with giant retinal tears. (a) The left eye of this patient was subjected to blunt ocular trauma. Dialyses of the ora serrata of 180° caused the superior retina to roll over. (b) In the image of the inferior fundus, the dislocated crystalline lens can be observed above the detached retina. (c) After surgery, the retina was reattached, with VA of 0.5

Fig. 3.11  Retinal detachments with giant retinal tears. (a) Before surgery, the posterior edge of the retinal break had rolled over. (b) After the surgery, the retina was reattached, with mild macular translocation. The patient’s VA was 0.3

3  Special Types of Rhegmatogenous Retinal Detachment

3.3

 etinal Detachments with Severe R Proliferative Vitreoretinopathy (PVR)

Retinal detachments with severe PVR are very complex, and this complexity is the most common cause of surgical failure [8]. These detachments can be located in any part of the retina. Limited or general contraction of membranes may be located on the inner or outer retinal surface, or at the vitreous base, anterior vitreous, ciliary body, and the posterior surface of the lens and iris. This contraction of fibrous proliferative membranes is a major barrier to retinal reattachment [9]. According to differences in clinical features, surgical treatment, and prognosis, PVR is categorized as anterior (aPVR) and posterior (pPVR) (Figs.  3.12, 3.13, 3.14, 3.15, and 3.16) [10]. In severe cases of peripheral vascular disease, only vitrectomy surgery can be effective. Since the 1970s, the surgical treatment of pPVR has had satisfactory results owing to the increased understanding of PVR and the development of vitrectomy. However, because of the particular anatomical site of aPVR, the surgery of aPVR is one of the most difficult modern vitreoretinal surgeries for complex retinal detachment.

Fig. 3.12  Proliferative vitreoretinopathy (PVR) grade B. Rolled-over posterior edge of retinal break

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The basic surgical goals in eyes with retinal detachment complicated by PVR are to relieve as much vitreoretinal traction as possible, to completely restore the mobility of the retina with minimal surgical trauma, and to reduce the postoperative recurrence of PVR.  Surgery of retinal detachment complicated by PVR is complex. However, intraocular surgery with the procedures of vitrectomy, the removal of epiretinal membranes, retinotomy, retinectomy, and laser photocoagulation, as well as the use of intraocular filling materials, will damage the blood -ocular barrier to various degrees, causing reactions by retinal cells and growth factors. To reduce the postoperative recurrence of PVR, alleviation of the surgical stress response is very important. Although the characteristic of PVR is hyperplastic lesions, sealing the breaks cannot be ignored, because closing the retinal breaks is still the key to the success of rhegmatogenous retinal detachment surgery (Figs. 3.17, 3.18, 3.19, 3.20, 3.21, 3.22, 3.23, 3.24, 3.25, 3.26, 3.27, 3.28, 3.29, 3.30, 3.31, 3.32, 3.33, 3.34, 3.35, 3.36, 3.37, 3.38, and 3.39).

Fig. 3.13  PVR grade C-1. One quadrant of full-thickness retinal folds

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Fig. 3.14  PVR grade C-2. Two quadrants of full-thickness retinal folds

Fig. 3.15  PVR grade C-2. Two quadrants of full-thickness retinal folds in the fovea area

Fig. 3.16  PVR grade C-3. Three quadrants of full-thickness retinal folds; part of the papilla can be observed

Fig. 3.17  Retinal detachment with macular hole in the left eye complicated by PVR grade C-3

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Fig. 3.18  Rhegmatogenous retinal detachment with pathological myopia complicated by PVR grade C-3

Fig. 3.19  Focal PVR.  The posterior edge of the horseshoe tear was rolled over, with focal subretinal hyperplasia

Fig. 3.20  Rhegmatogenous retinal detachment with PVR grade C-2. The posterior edge of the horseshoe tear was rolled over, with vitreous hyperplasia at the surface of the retinal tear and two quadrants of full-­ thickness retinal folds

Fig. 3.21  Retinal detachment after scleral buckling with PVR grade C-3

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Fig. 3.22  Rhegmatogenous retinal detachment with PVR grades D-1 to D-2

Fig. 3.23  Rhegmatogenous retinal detachment with PVR grade D-3

Fig. 3.24  Rhegmatogenous retinal detachment with PVR grade D-3

Fig. 3.25  Rhegmatogenous retinal detachment with PVR grade D-3

Fig. 3.26  Retinal detachment after vitrectomy surgery with PVR grade D-3

Fig. 3.27  Rhegmatogenous retinal detachment with PVR grades D-2 to D-3

3  Special Types of Rhegmatogenous Retinal Detachment

Fig. 3.28  Rhegmatogenous retinal detachment with PVR grade D-3

Fig. 3.30  Retinal detachment after vitrectomy surgery with PVR grade D-3

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Fig. 3.29  Old retinal detachment with PVR grade D-3

Fig. 3.31  Retinal detachment after vitrectomy surgery. Although the papilla cannot observed, as the retina remains mobile, this is not a case with PVR grade D-3

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Fig. 3.32  Retinal detachment after vitrectomy surgery. Although the papilla can be partially observed, as the retina remains mobile, this is not a case with PVR grade D-3

Fig. 3.33  Image of anterior PVR with retinal anterior displacement and vitreous hyperplasia on ultrasound biomicroscopy (UBM)

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Fig. 3.34  Image of anterior PVR with forward retinal shifting on UBM

Fig. 3.35  Retinal detachment with PVR grade C.  Severe subretinal fibrosis with tight collarette

Fig. 3.36  Retinal detachment. Full-thickness retinal folds occur at the posterior and anterior of the retinal equator

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Fig. 3.37  Retinal detachment with PVR grade C-3. Retinal hyperplasia occurs with annular contraction at the anterior of the retinal equator

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Fig. 3.38  Old retinal detachment with subretinal hyperplasia

b

Fig. 3.39  Fundus photographs of rhegmatogenous retinal detachment with PVR grade C-3 before and after surgery. (a) The fundus image of the retina before the surgery. (b) After vitrectomy and silicone oil tamponade. There are laser burns surrounding the retinal tear

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3.4

 etinal Detachment with Choroidal R Detachment

Rhegmatogenous retinal detachment with cyclodialysis and choroidal detachment, a special type of complex retinal detachment, is characterized by rapid onset and development [11]. Severe uveitis and ocular hypotension cause cyclodialysis and choroidal detachment, which, without prompt treatment, can gradually cause proliferation around the vitreous body and retina, with a poor prognosis.

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3.4.1 Clinical Presentation 1. The disease commonly occurs in the elderly and in patients with high myopia and aphakic eyes, with no sex differences seen. 2. The extent of the retinal detachment is generally large, and often involves more than three quadrants. However, the detachment is shallow, with small folds, in which a slit pore is often hidden, making it hard to find. Glucocorticoid hormone treatment can alleviate the choroidal detachment, elevating the retinal detachment and raising the bulge of the retina. In the advanced stage of the disease, a wide range of findings, such as fixed wrinkles and stiffness, can be observed in the retina. The retinal tears are mostly located in the posterior pole of the retina, being mainly macular holes and horseshoe-shaped tears (Figs. 3.40, 3.41, and 3.42). 3. Uveitis is one of the important features of this disease. Patients usually complain about pain in the eyes. During examination, ocular touch pain and ciliary injection can be noticed. The Tyndall sign is strongly positive. Wide posterior synechiae of the pupil can be observed. The vitreous body shows opacity and membrane formation. 4. Ocular hypotension is also a major feature of this disease. Intraocular pressure is often below 3 mmHg. The ocular bulb becomes soft. Anterior chamber deepening can be identified. The iris presents concentric circle-like folds, iridodonesis, and lens oscillation (Fig. 3.43). 5. Mainly owing to the lack of early diagnosis and timely treatment, proliferative changes occur in the retina and vitreous body, with opacity, vitreous concentration, and membrane formation displayed in the vitreous body. The widespread proliferation around the retina causes changes such as the formation of a large number of fixed folds (Fig. 3.44). 6. Cyclodialysis and choroid detachment can occur 1–3 weeks or later after retinal detachment, but they do not necessarily happen at the same time. Generally cyclodialysis can be observed when the pupil is fully dilated. Sometimes, the ora serrata is elevated without compression of the sclera. The shape and extent of choroidal detachment varies and can be manifested as a peripheral flat detachment or a globular-shaped bulge occupying multiple quadrants. If the choroidal detachment develops in a backward fashion, it can reach the optic disc, or if it develops in a forward fashion it can cause cyclodialysis. The choroidal detachment shows a brown and gray bulge with a solid feel, with an appearance like that of choroidal melanoma. Lamellar division can be observed when the vasa vorticosa or long ciliary nerves pass by (Figs. 3.45, 3.46, and 3.47).

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Fig. 3.40  Retinal detachment with choroidal detachment in left eye

Fig. 3.42  Retinal detachment with choroidal detachment showing small retinal folds

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Fig. 3.41  Retinal detachment with choroidal detachment combined with posterior PVR

Fig. 3.43  Clinical presentation of anterior segment in retinal detachment with choroidal detachment. The iris presents concentric circle-like folds with a deep anterior chamber

Fig. 3.44  Retinal detachment with choroidal detachment in the left eye, with general full-thickness retinal folds

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Fig. 3.45  Spherical and annular choroidal detachment with shallow retinal detachment

Fig. 3.47  UBM image of annular choroidal detachment with supraciliaris effusion

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Fig. 3.46  Annular choroidal detachment

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3.4.2 Differential Diagnosis 3.4.2.1 Uveitis In typical uveitis, there are vitreous opacities and changes of the fundus, as well as ocular pain, hyperemia, positive Tyndall sign, miosis, and white-gray keratic precipitates (KP). In this case the white-gray KP are rare, although with an obvious clinical presentation of uveitis, there is no presentation of choroiditis and retinitis. 3.4.2.2 Idiopathic Uveal Effusion Syndrome In this syndrome, the subretinal fluid is clear and shifts with changes in the position of the head. Retinal detachment and choroidal detachment may occur without retinal breaks. There are minor cellular infiltrations in the vitreous, without PVR. Intraocular pressure is normal, without uveitis or Fig. 3.48  Color Doppler image of hemorrhagic choroidal detachment

Fig. 3.49  Fundus of suprachoroidal hemorrhage

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with only slight uveitis. Most cases are binocular, and cerebrospinal fluid pressure and protein content can be increased.

3.4.2.3 Choroidal Melanoma Choroidal melanoma generally occurs in the posterior pole of the retina, and it can occur in other quadrants; it shows localized solid surface lesions, with pigment hyperplasia. Diaphanoscopy examination results are positive. The condition can cause secondary retinal detachment. There are no clinical presentations of uveitis or low intraocular pressure. 3.4.2.4 Hemorrhagic Choroidal Detachment Hemorrhagic choroidal detachment is normally caused by suprachoroidal hemorrhage following ocular trauma or intraocular surgery (Figs. 3.48 and 3.49).

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3.5

 ongenital Coloboma of Choroid C with Retinal Detachment

Congenital coloboma of choroid results from a dysplastic neuroectoderm in the choroidal fissure during the closure of this fissure [12]. The inner layer of the optic cup fails to develop into a normal retina, and the pigment epithelium in the outer layer of the optic cup shows maldevelopment, which then affects the development of the choroid, especially that of the choriocapillary layer, resulting in ­maldevelopment of the sclera in this part. Retinal detachment occurs in 40% of patients.

Fig. 3.50  Retinal detachment in the choroid defect area Fig. 3.52 B-ultrasound image showing coloboma of choroid

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3.5.1 Clinical Presentation Defects of the inferior iris, ciliary body, choroid, some parts of the retinal sensory layer, and the pigmented retinal epithelium can be observed. The sclera becomes thin and bulges outward with a lengthening axis oculi, which causes myopia of different degrees. This disease is often associated with other ocular tissue dysplasias, such as microcornea, microphthalmus, cataract, and various dysplasias of the optic disc. B-mode ultrasonography can help to diagnose the condition (Figs. 3.50, 3.51, 3.52, and 3.53). Retinal detachment in the defect area occurs in the preclinical phase, and expansion of the retinal detachment out of the defect area occurs in the clinical phase (Figs. 3.54 and 3.55).

Fig. 3.51  Coloboma of choroid

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Fig. 3.53 B-ultrasound image showing coloboma of choroid with retinal detachment

Fig. 3.54  Coloboma of choroid with retinal detachment beyond the choroid defect area

Fig. 3.55  Coloboma of choroid with retinal detachment. The optic disc and macula are in the choroid defect area, while retinal detachment occurs beyond the choroid defect area

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3.5.2 Features of Retinal Detachment 3.5.2.1 Range of Retinal Detachment in the Defect Area In the intramarginal part of the defect area, the detachment involves a width of only about 2 disc diameter (DD). And retinal detachment is not observed in the center of the defect area; this can be shown by B-mode ultrasonography and an optical coherence tomography (OCT) test. An OCT test can also show retinal thinning in the defect area (Figs. 3.56, 3.57, 3.58, and 3.59). The retina shows multilayer tissue in the defect area, and this gradually thins into a single layer, and finally fuses with the sclera at one point in the center of the defect area. As the thinnest part of the retina is then at the center of the defect area, retinal detachment does not occur in this area. 3.5.2.2 Retinal Tears in the Defect Area A retinal tear often appears at the border of the retina in the defect area. The retinal tears are of three types:

Fig. 3.56 B-ultrasound image of coloboma of choroid with retinal detachment, showing axial length of 28.9 mm with back-curved sclera. The retina is detached beyond the choroid defect area

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1. Oval slit-shaped tears. In these tears the major axis is along the boundary line of the retinal detachment; the margin near the non-defect area curls up, and the margin near the defect area is attached to the sclera. Owing to differences in the retinal structure between the margin near the defect area and the central side, a single-layer retina near the central margin fuses with the sclera in the basal part of the defect concavity, resulting in one side of the margin of the tear being higher than the other side. 2. Macular hole in the defect area. The lower part of its arch ring has not developed, so a macular hole is defined according to the appearance of yellow lutein during an operation. 3. Atrophic hole. An atrophic hole is round in shape with a floating margin, so it is difficult for the margin to become attached to the deep concavity of the scleral layer in the defect area. After silicone oil injection, small silicone oil droplets can easily be concealed under the detached retina in the defect area.

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Fig. 3.57 B-ultrasound image of coloboma of choroid with retinal detachment. The retina was attached at the central part of the choroid defect area

Fig. 3.58  Optical coherence tomography (OCT) image of coloboma of choroid with retinal detachment, showing thinning of multi-layer ­structures at the edge of the choroid defect area, while part of the central retina is firmly attached to the sclera

Fig. 3.59  OCT image of coloboma of choroid with retinal detachment. Retinal thinning is seen in the defect area

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3.5.2.3 Features of Retinal Detachment Beyond the Defect Area Retinal detachment beyond the defect area is often shallow and without posterior vitreous detachment. Prolonged detachment can lead to severe PVR (Figs. 3.60, 3.61, 3.62, 3.63, and 3.64).

Higher success rates of retinal detachment surgery are found in patients in whom the optic disc is located out of the defect area of the choroid, while success rates are lower in patients whose optic disc is located in the defect area. Vitrectomy combined with intraocular silicone oil tamponade is the best operation method.

Fig. 3.60  Coloboma of choroid with retinal detachment. The retinal detachment, with general elevation, and the optical disc, are not in the choroid comoboma area

Fig. 3.61  The edge of the choroid coloboma is associated with retinal detachment, while the central retina in the area of the choroid coloboma is not detached

Fig. 3.62  Coloboma of choroid with retinal detachment. The optical disc and the sclera show bulging

Fig. 3.63  Coloboma of choroid with retinal detachment showing fixed macular folds

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3.6

 etinal Detachment with R Marfan Syndrome

Marfan syndrome is an autosomal dominant disorder with mesodermal dystrophy characterized by binocular high myopia, lens subluxation, retinal detachment, and skeletal abnormalities, caused by widespread systemic dysplasia of mesodermal tissue. Owing to the marked characteristics of Marfan syndrome, diagnosis may be confirmed in early childhood. There are no gender differences.

3.6.1 Retinal Detachment

Fig. 3.64  Coloboma of choroid with retinal detachment showing PVR

Retinal detachment is only one manifestation of Marfan syndrome, occurring in 5%–10% of patients with the syndrome, most likely causing peripheral retinal breaks and complete retinal detachment. Clinical analysis found that 75% of cases of retinal detachment showed complete retinal detachment [13]. Small round holes in the lattice degeneration area are most common, followed by various dialyses of the ora serrata. The clinical features of the retinal breaks in dialyses are similar to aphakic retinal detachment. The age of onset of retinal detachment in patients with Marfan syndrome is earlier than the general age of onset of retinal detachment. Common causes of retinal detachment in Marfan syndrome include: 1. Increased ocular axial length. A clinical study found that the average axial length was 24.90 mm in normal control groups, while it was 28.47 mm in patients with Marfan syndrome with retinal detachment. 2. Lens subluxation. The constant traction of the dislocated lens to the ora serrata usually causes retinal detachment. As reported [14], retinal detachment in Marfan syndrome occurs in 9% of patients with phakic eyes, and in 19% of patients with aphakic eyes. 3. Heredity. Retinal detachment shows a hereditary predisposition in patients with a positive family history of Marfan syndrome. 4. Liquefaction, incrassation, and traction of the vitreous may increase the possibility of retinal detachment. 5. Attenuation of the choroid and retina in Marfan syndrome easily causes peripheral rhegmatogenous retinal detachment.

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3.6.2 Other Common Ocular Features

3.6.3 Imaging Features

Other common ocular features in Marfan syndrome include dislocated lens, abnormal iris, abnormal pupil, ametropia, abnormal cornea and sclera, and secondary glaucoma (Figs. 3.65, 3.66, 3.67, and 3.68).

Imaging examination is essential for the treatment of retinal detachment in Marfan syndrome. B-type ultrasonic examination and UBM help to identify the location and range of retinal detachment in patients with clouding lens or patients with a small pupil.

Fig. 3.65  Upper shifting lens (lens subluxation) in Marfan syndrome under slit lamp biomicroscope

Fig. 3.67  Subluxation of lens in Marfan syndrome under slit lamp biomicroscope. The edge of the dislocated lens can be seen in the center of the pupil

Fig. 3.66  Subluxation of lens in Marfan syndrome under slit lamp biomicroscope. The suspensory ligament is observed below the equator of the lens

Fig. 3.68  Transparent luxated lens in Marfan syndrome has fallen back into the vitreous cavity

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3.7

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 etinal Detachment After R Vitreoretinal Surgery

3.7.1 R  etinal Detachment After Scleral Buckling 3.7.1.1 Reasons for recurrent retinal detachment The reasons for recurrent retinal detachment are open retinal breaks (with an inadequate buckling effect or a new tear) or PVR. 3.7.1.2 Clinical Presentation 1. Recurrent or persistent retinal detachment occurs days or several months after vitreoretinal surgery. The subretinal fluid accumulates around the retinal breaks. 2. Open retinal breaks can lie on the side of the crest of the buckle, causing failure of adhesion with the retinal pigment epithelium. In other cases, although the treated retinal breaks lie on the crest of the buckle, the retinal breaks are still open because of vitreoretinal traction or because of a proliferative vitreous force upon the edge of the retinal breaks and persistent opening of the posterior edge of the retinal breaks (called “fish-mouth” retinal breaks) (Figs. 3.69, 3.70, and 3.71).

Fig. 3.70  Fundus of retinal detachment after scleral buckling. A 48-year-old male with retinal detachment combined with choroidal detachment has preretinal proliferative membranes at the posterior pole in the right eye, causing recurrent retinal detachment after scleral buckling. The peripheral retinal break is not shown in this image

Fig. 3.69  Fundus of retinal detachment after scleral buckling. A 43-year-old female has a small break on the macular fovea with inferior retinal detachment in her right eye. A missed macular hole is the cause of recurrent retinal detachment

Fig. 3.71  Fundus of retinal detachment after scleral buckling. A 45-year-old female has inferior retinal detachment, after scleral buckling and pneumatic retinopexy. The superior retina is reattached, while a macular break is located at the edge of the inferior retinal detachment

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3.7.1.3 Differential Diagnosis 1. Exudative retinal detachment. This usually occurs 2 or 3 days after the surgery, and may be associated with the obstruction of vortex venous reflux circulation or effects on the blood-ocular barrier caused by cryotherapy. In some cases, exudative retinal detachment is combined with choroidal detachment. The subretinal fluid may be turbid and may shift with changes in eye position. The most important clinical feature is that the subretinal fluid is not associated with retinal breaks, while retinal breaks lie on the crest of the buckle. Sometimes rapid increase of the subretinal exudate may lead to complete retinal detachment, although partial retinal detachment had occurred before the surgery. Normally, the subretinal exudate would be absorbed in 2  weeks; in some cases systemic glucocorticoids can promote this process. 2. Vitreous opacities. Severe vitreous opacities may obstruct the examination of retinal detachment. B-type ultrasonic examination is helpful to diagnose these opacities.

3.7.2 Retinal Detachments After Vitrectomy 3.7.2.1 Reasons for the retinal detachment The retinal detachment is mainly caused by new retinal breaks or vitreous traction. Retinal breaks may be caused by accidental injury with surgical instruments during vitrectomy surgery; by traction of the peripheral vitreous base caused by instruments repeatedly passing through the eyeball; and by extreme pulling of vitreoretinal strands during the surgical procedure. 3.7.2.2 Clinical Presentation and Diagnosis 1. The retinal breaks are mainly located at the peripheral retina, especially at the sites of adherence of vitreous opacities. The retinal breaks are mainly annular strands or triangular linear strands, which are usually associated with the traction of the peripheral vitreous base, caused by surgical instruments repeatedly passing through the eyeball, and other accidental injuries caused by surgical instruments. In some cases, giant retinal breaks can be observed. 2. The retinal breaks may be irregular or somewhat rotund, with hemorrhage on the edge of the breaks, caused by direct injury. 3. The retina is taut owing to the traction of proliferative vitreous strands, which are usually located in the primary lesions of the retina.

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4. These types of retinal detachments are mostly seen as soft undulating bullae and they develop rapidly owing to the lack of vitreous support, which can also occur rapidly (Figs. 3.72 and 3.73).

Fig. 3.72  Fundus of retinal detachment after vitrectomy surgery. A 26-year-old male has complete retinal detachment in his right eye. Because the retina has no vitreous support, the detachments are seen as soft undulating bullae. Inferior retinal detachments are more severe owing to the effect of gravity. Retinal breaks are not displayed in this image

Fig. 3.73  Fundus of retinal detachment after vitrectomy surgery. A 12-year-old male has nasal retinal detachment in his left eye. The remaining vitreous base is pulled by proliferative tissue at the peripheral retina, causing traction of multiple full-thickness folds

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3.7.3 R  etinal Detachments After Silicone Oil Tamponade 3.7.3.1 Reasons for the Retinal Detachments 1. Early onset after surgery. In this case, retinal detachments are caused by inadequate elimination of epiretinal or subretinal traction, inadequate silicone oil filling or improper posture that leads to the reopening of retinal breaks, and missed or unclosed retinal breaks. 2. Late onset after surgery. In this case, retinal detachments are caused by atrophy of the retinal pigment epithelium, or by PVR that leads to the relapse of retinal breaks, the formation of new retinal breaks, or tractional retinal detachment. These retinal detachments occur in cases of PVR, proliferative diabetic nephropathy, traumatic tractional retinal detachment, and retinal detachment with macular hole. 3.7.3.2 Clinical Presentation and Diagnosis 1. After intraocular silicone oil tamponade. There are two clinical presentations after silicone oil ­tamponade—silicone oil emulsification and silicone oil displacement, which can help to ensure that the silicone oil is placed intraocularly. Displacement of the silicone oil can occur under the retina through the retinal breaks, or through the pupil or ciliary zonule into the anterior

Fig. 3.74  Fundus of retinal detachment after silicone oil injection. A 12-year-old female has major retinal detachment with a marked boundary between the detached and reattached retina. Owing to the pressure of the silicone oil, the superotemporal retina remains reattached. There are several retinal folds caused by various proliferative membranes on the surface of the retina

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chamber as transparent oil drops. When the silicone oil does not completely fill the vitreous cavity, slow separation of the silicone oil and the retinal surface can be observed under indirect bio-ophthalmoscopy, as the boundary of silicone oil at the inferior retina. 2. Retinal detachment and proliferative membranes. Retinal detachment often develops slowly in the inferior retina, because of the pressure of the silicone oil. In cases of early onset after the surgery, retinal breaks or retinal incisions are often reopened, or they may be combined with remaining or new retinal hyperplasia, which is usually located at the anterior side of the crest or at the bottom of the posterior side of the crest caused by the efficient pressure of the silicone oil. In cases of late onset after the surgery, retinal breaks may be unclear or irregular with full-­thickness folds and retinal hyperplasia traction. The most common and most serious clinical presentation is aPVR, which can be newly formed or may be present as it was before the surgery. Recurrent peripheral retinal detachment may be caused by traction and thickening of anterior loop proliferation at the peripheral retina and vitreous base. The duration of the peripheral detachment can be several hours, with lower duration of detachment occurring at the posterior pole (Figs. 3.74, 3.75, 3.76, 3.77, and 3.78).

Fig. 3.75  Fundus of retinal detachment after silicone oil injection. A 65-year-old female has peripheral inferior retinal detachment, with the site of subretinal fluid drainage at the posterior edge of the detachment (depigmentation lesion). Non-closure of the drainage site was confirmed, causing shallow peripheral retinal detachment

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Fig. 3.76  Fundus of retinal detachment after silicone oil injection. A 48-year-old male has recurrent retinal detachment at the posterior pole of his right eye. There are several retinal folds caused by traction of the epiretinal proliferative membrane on the macular fovea

Fig. 3.78  Fundus of retinal detachment after silicone oil injection. A 68-year-old male has nasal retinal detachment with the retinal breaks showing fixed and folded superior edges and vascular tortuosity at the peripheral inferior nasal retina. The horizontal black ribbon at the inferior edge of the retinal break shows the dislocation of silicone oil

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Fig. 3.77  Fundus of retinal detachment after silicone oil injection. A 48-year-old male has peripheral inferior temporal retinal detachment, with the posterior edge in the depigmentation area and the anterior edge at the crest of the scleral buckle. The boundary of the silicone oil is observed at the posterior edge of the scleral buckle crest, because of the inadequate filling of silicone oil

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References 1. Smiddy WE, Green WR. Retinal dialysis: pathology and pathogenesis. Retina. 1982;2(2):94–116. 2. Hamrick KE, Helgeson MK.  Retinal dialysis. Optom Clin. 1992; 2(3):93–112. 3. Kennedy CJ, Parker CE, McAllister IL.  Retinal detachment caused by retinal dialysis. Aust N Z J Ophthalmol. 1997;25(1): 25–30. 4. Hollander DA, Irvine AR, Poothullil AM, Bhisitkul RB. Distinguishing features of nontraumatic and traumatic retinal dialyses. Retina. 2004; 24(5):669–75. 5. Ross WH. Traumatic retinal dialyses. Arch Ophthalmol. 1981;99(8): 1371–4. 6. Howard RO, Gaasterland DE.  Giant retinal dialysis and tear. Surgical repair. Arch Ophthalmol. 1970;84(3):312–5. 7. Nacef L, Daghfous F, Chaabini M, et  al. [Ocular contusions and giant retinal tears]. J Fr Ophtalmol. 1997;20(3):170–4. 8. Charteris DG, Sethi CS, Lewis GP, Fisher SK.  Proliferative vitreoretinopathy-developments in adjunctive treatment and retinal pathology. Eye (Lond). 2002;16(4):369–74.

K. Ma et al. 9. Pastor JC, de la Rua ER, Martin F.  Proliferative vitreoretinopathy: risk factors and pathobiology. Prog Retin Eye Res. 2002; 21(1):127–44. 10. Machemer R, Aaberg TM, Freeman HM, et  al. An updated classification of retinal detachment with proliferative vitreoretinopathy. Am J Ophthalmol. 1991;112(2):159–65. 11. Seelenfreund MH, Kraushar MF, Schepens CL, Freilich DB. Choroidal detachment associated with primary retinal detachment. Arch Ophthalmol. 1974;91(4):254–8. 12. Weng N, Wei W. [The clinical features of retinal detachment with congenital choroidal coloboma]. Zhonghua Yan Ke Za Zhi. 1998; 34(4):250–2. 13. Chandra A, Ekwalla V, Child A, et al. Prevalence of ectopia lentis and retinal detachment in Marfan syndrome. Acta Ophthalmol. 2014;92(1):e82-3. https://doi.org/10.1111/aos.12175. 14. Rahmani S, Lyon AT, Fawzi AA, et al. Retinal Disease in Marfan Syndrome: From the Marfan Eye Consortium of Chicago. Ophthalmic Surg Lasers Imaging Retina. 2015;46(9):936–41. https://doi.org/10.3928/23258160-20151008-06.

4

Exudative Retinal Detachment Haiying Zhou, Nan Zhou, and Wenbin Wei

Abstract

Systemic diseases and exudative retinal detachment include hypertensive retinopathy, retinopathy of nephropathy, and exudative retinal detachment related to eye diseases, such as Vogt-Koyanagi-Harada syndrome, central exudative chorioretinopathy, bullous retinal detachment, uveal effusion syndrome, posterior scleritis, sympathetic ophthalmia, acute posterior multifocal placoid pigment epitheliopathy, and exudative retinal detachment secondary to central retinal vein occlusion (CRVO).

4.1

 ystemic Diseases and Exudative S Retinal Detachment

4.1.1 Hypertensive Retinopathy Hypertensive retinopathy is a complication of hypertension. The prevalence of hypertension in the population is 3–5%, and about 95% of adults with high blood pressure have primary hypertension (sometimes called essential hypertension) [1]. Hypertension was diagnosed when a person’s systolic blood pressure (SBP) is ≥140  mmHg and/or their diastolic blood pressure (DBP) is ≥90 mmHg [1]. It divides

into essential hypertension, which causes remain unknown, and secondary hypertension which is caused by systemic disease including chronic kidney disease, renal artery stenosis, pregnancy-induced hypertension, excessive aldosterone secretion, pheochromocytoma, and sleep apnea. Essential hypertension is divided into benign hypertension and accelerated hypertension according to the course of the disease.

4.1.1.1 Fundus Changes of Benign Hypertension Benign hypertension has a dormant onset and a familial hereditary tendency. The incidence of female is twice higher than that of male. In general, complications of various organs may occur at 10–15 years after the onset, especially in the eyes, brain, and kidney, where small arteriosclerosis and the related diseases may be found. Symptoms: There may no obvious symptoms in the early stage, but with the progression of the disease, there may have varying degrees of vision loss. Fundus changes: According to the progress of the diseases, it is divided into arterial stenosis stage and atherosclerosis stage. On atherosclerosis stage, arteriolar changes including “copper” or “silver” wiring and arteriovenous nicking are seen. As these changes progressed, an extensive hypertensive retinopathy occurs, with hemorrhages, hard exudates, and edema of the optic disc (Figs. 4.1, 4.2, 4.3 and 4.4).

H. Zhou, M.D., Ph.D. · N. Zhou, M.D., Ph.D. · W. Wei, M.D., Ph.D. (*) Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China

© Springer Nature Singapore Pte Ltd. and Beijing Science and Technology Press 2018 W. Wei (ed.), Atlas of Retinal Detachment, https://doi.org/10.1007/978-981-10-8231-3_4

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Fig. 4.1  Fundus image of hypertensive retinopathy. Note retinal artery narrowing especially in the inferior temporal artery, arteriovenous nicking, and flame-shaped hemorrhages beside the optic nerve head

a

Fig. 4.2  Fundus image of hypertensive retinopathy. Optic disc edema, hyperemia, and blurred disc margins. There are also cotton-wool spots, flame-shaped hemorrhages, and star-shaped hard exudates in the macula

b

Fig. 4.3  Fundus images of hypertensive retinopathy. Bilateral retinal artery narrowing, multiple cotton-wool spots, and a few dot hemorrhages in posterior pole. There is also AV nicking at upper vascular arcade in the left eye. (a) Fundus image of the right eye. (b) Fundus image of the left eye

a

b

Fig. 4.4  Fundus fluorescein angiography images corresponding to Fig. 4.3. Both figures (a, b) show retinal cotton-wool spots, corresponding to capillary non-perfusion area, surrounded by leaking dilated small vessels and a few microaneurysms

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4.1.1.2 Fundus Changes of Accelerated Hypertension About 1% of essential hypertension patients develop accelerated hypertension [2], which occurs more often in young patients. A rapid rise in blood pressure, often with diastolic blood pressure higher than 130 mmHg, can cause optic neuropathy, retinopathy, renal hypofunction, heart failure, and hypertensive encephalopathy. Symptoms: A sudden or gradual decrease of vision, often accompanied by headache, vomiting, and coma. Fundus changes: An extensive retinopathy is seen, with extensive or focal arteriostenosis, flame-shaped hemorrhage, cotton-wool spots, hard exudates, and edema of the retina and the optic disc. Hard exudates, which represent the deposition of lipid from chronic leakage of plasma from incompetent vessels, can have a star appearance. These exudates are typically located in the Henle’s layer of the retina. A cottonwool spot is a focus of retinal opacification developed after retinal arteriole occlusion with secondary axoplasmic damming. Retinal edema and exudation are caused by fibrinoid

a

necrosis within the peripapillary arteriole. Disc edema is the result of the obstruction and leakage of the small arteries and veins. In severe cases, exudative retinal detachment is present. The main causes of it are ischemic change of the choriocapillaris, including acute occlusion of choroidal arterioles and patchy non-perfusion of choriocapillaris, leading to leakage and accumulation of fluid under the retina, and the formation of exudative retinal detachment and pigment epithelial detachment (Figs. 4.5, 4.6 and 4.7). Prognosis: With the control of hypertension, vasospasm rapidly relieves. Resolution of the hemorrhage, retinal edema, and cotton-wool spots occurs within a few weeks, while regression of hard exudates may take a few months. However, arteriosclerosis is irreversible. Retinal pigment epithelium changes secondary to accelerated hypertension may been seen, including Elschnig spots (central hyperpigmented and peripheral hypopigmented lesion of RPE changes corresponding with patchy non-perfusion area of choriocapillaris) or Siegrist spots (spots of pigmentary changes arranged linearly along the sclerosal choroidal vessels) [3].

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Fig. 4.5  Fundus images of malignant hypertensive retinopathy. Male patient, 20 years old, claimed sudden vision loss for 2 weeks, with visual acuity counting fingers and blood pressure 240/180 mmHg on first visit. Note the binocular severe optic disc edema and hyperemia, retinal artery

narrowing, arteriovenous nicking, and sausage-shaped tortuous distended retinal veins. There are also multiple cotton-wool spots at posterior pole and exudative retinal detachment in the inferior peripheral retina. (a) Fundus image of the right eye. (b) Fundus image of the left eye

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Fig. 4.6  Bilateral hypertensive retinopathy. Photograph shows multiple cotton-wool spots and intraretinal hemorrhage at posterior pole, starshaped hard exudates in macula, and pale optic disc. (a) Fundus image of the right eye. (b) Fundus image of the left eye

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Fig. 4.7  Bilateral hypertensive retinopathy. Note the severe optic disc edema and hyperemia with hemorrhage in both the right and left eye, accompanied with macular edema and hard exudates. (a) Fundus image of the right eye. (b) Fundus image of the left eye

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4.1.2 F  undus Changes in Secondary Hypertension Secondary hypertension can be caused by kidney or endocrine diseases. It generally occurs in young patients with an acute onset. The characteristics of the arterial changes are arteriospasm, rather than arteriosclerosis. Retinopathy of nephropathy and pregnancy-induced hypertension are special types of secondary hypertensive retinopathy.

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4.1.2.1 Retinopathy of Nephropathy In patients with acute, chronic glomerulonephritis or adrenomedullary tumors, retinopathy is secondary to changes of arterioles but not renal disease itself. Therefore, only when the secondary hypertension caused by renal disease occurs can different levels of hypertensive retinopathy be seen according to the severity of the hypertension (Figs. 4.8, 4.9, 4.10, 4.11 and 4.12).

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Fig. 4.8  Fundus images of retinopathy caused by renal disease. Photograph shows enhanced central light reflex of retinal artery, scattered cottonwool spots and dot hemorrhages, and star-shaped hard exudates in the macula. (a) Fundus image of the right eye. (b) Fundus image of the left eye

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Fig. 4.9  Fundus images of retinopathy caused by renal disease. Note bilateral massive hard exudates at posterior pole. (a) Fundus image of the right eye. (b) Fundus image of the left eye

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Fig. 4.10  Fundus image of patients with renal failure. Note massive retinal exudates post-renal transplant

Fig. 4.11  Fundus image of patients with renal failure. Exudative retinal detachment can be seen in inferior retina

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Fig. 4.12  Fundus image of patients with renal failure. Exudative retinal detachment can be seen at posterior pole

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4.1.2.2 Retinopathy of Pregnancy-Induced Hypertension Hypertension is the most common medical disorder during pregnancy, affecting 6–8% of all pregnancies [4]. The changes of retinal blood vessels are related to the increase of blood pressure. Local constriction of the retinal artery can be seen in the early stage, and extensive small artery stenosis occurs subsequently. These changes are restored if blood pressure is reduced with caution or pregnancy is terminated.

Fig. 4.13  Fundus image of retinopathy caused by pregnancy-induced hypertension syndrome (PIH). Bullous exudative retinal detachment can be seen in inferior retina of left eye

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Fig. 4.15  Fundus images of retinopathy caused by PIH during convalescent period. A female patient, 27 years old, who was diagnosed as having PIH at 8 months of pregnancy and got recovery after labor, claimed bilateral blurred vision for 2 months. Her best-

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If development of hypertension is not being controlled, hypertension retinopathy including hemorrhages, edema, and exudation occurs. About 1–2% of patients develop exudative retinal detachment. With termination of pregnancy and control of hypertension, exudative detachment rapidly subsides and other retinal changes gradually regress, but RPE changes (depigmentation and hyperpigmentation) and visual impairment remains (Figs. 4.13, 4.14, 4.15, 4.16, 4.17, 4.18 and 4.19).

Fig. 4.14  Fundus image of retinopathy caused by PIH.  Exudative retinal detachment can be seen in inferior retina

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corrected visual acuity was 20/200  in the right eye and 20/500  in the left eye on first visit. Note the pigmentation and depigmentation at posterior pole. (a) Fundus image of the right eye. (b) Fundus image of the left eye

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Fig. 4.16  FFA images corresponding to Fig. 4.15. Note multiple Elschnig spots (pigmented lesions surrounded by a pale halo), leaking small vessels in the periphery, and exudative retinal detachment in the inferior retina. (a) FFA images of the right eye. (b) FFA images of the left eye

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Fig. 4.17  Fundus images of retinopathy caused by PIH during convalescent period. A female patient, 27 years old, who was diagnosed having PIH at 8 months of pregnancy without corresponding treatment after labor, claimed bilateral sudden blurred vision for 9 months. The blood pressure was normal on first visit. The visual acuity was counting

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fingers in both eyes. The photograph shows bilateral optic nerve atrophy, retinal arteriole narrowing like silver wire, remnant cotton-wool spots and hard exudates, a few scattered dot hemorrhages, and generalized depigmentation of the whole fundus. (a) Fundus image of the right eye. (b) Fundus image of the left eye

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Fig. 4.18  FFA images corresponding to Fig. 4.17. Note bilateral multiple Elschnig spots at posterior pole. There are small capillary non-perfusion areas surrounded by capillary dilation and leaking. (a) FFA image of the right eye. (b) FFA image of the left eye

Fig. 4.19  Fundus images of retinopathy caused by PIH. Photograph shows enhanced light reflex on retinal artery, scattered cotton-wool spots and dot hemorrhages at posterior pole, and star-shaped hard exudates in macular area

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4.1.2.3 FFA of Hypertensive Retinopathy In benign hypertension, a narrowing of the retinal arteries and capillary is seen, but the microcirculation changes are not obvious. As the hypertension progressed, microangiopathy occurs. FA may show a delay of retinal circulation time and narrowing of retinal arteries. In the areas of cotton-wool spots, hypofluorescence is observed for capillary non-perfusion. Retinal capillaries that are located adjacent to non-perfusion areas are dilated and leaky, and microaneurysms which appear as small, hyperfluorescent dots can be seen. When papilledema appears, capillaries on the surface of optic papilla and in peripapillary area dilate and curl into a loop, with fluorescence leaking (Fig.  4.17). Elschnig spots appear as central hyperpigmented (blockage of the fluorescence) and peripheral hypopigmented (window defect) lesion of RPE changes (Figs. 4.16 and 4.18).

4.1.3 F  undus Changes in Hematologic Diseases Fundus changes in hematologic diseases include those seen in diseases caused by abnormal leukocytes, erythrocytes, coagulation factors, or plasma proteins. Disseminated intravascular coagulation can cause exudative retinal detachment, while anemia, leukemia, and plasma cell disease can also present with associated serous retinal detachment occasionally. Disseminated intravascular coagulation (DIC) is a pathological condition in the stages of many disease progressions. Extensive intravascular coagulation occurs on the effect of some pathogenic factors, causing embolism and microcirculation. Meanwhile, large number of platelets and clotting factors being consumed may lead to massive hemorrhage and ischemic necrosis. A common finding is exudative retinal detachment, which is thought to be caused by extensive obstruction of choroidal capillaries and disruption of the pigment epithelial. Retinal hemorrhage and vitreous hemorrhage also can be seen in DIC. Anemia is a disorder in which erythrocyte count, hemoglobin concentration, or hematocrit of the circulation decreased, lower than normal. The main causes of the disease are deficiency of erythrocyte, increase of erythrocyte damage, or bleeding. No change is found in fundus in mild anemia. When the hemoglobin or erythrocyte fell to 30% of normal, anemic fundus changes may occur. On fundus examination, anemic show optic and retinal edema, retinal

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h­ emorrhage, retinal hemorrhage spot center contains white spots which due to agglutination of platelet and cellulose (Figs.  4.20, 4.21 and 4.22). Occasionally vitreous hemorrhage can be seen. In severe cases, exudative retinal detachment may be present. Due to the reduction of reduced hemoglobin, the color of retinal vein becomes pale, close to that of the arterial. Meanwhile the whole fundus color may become pale because of reduction of choroidal reduced hemoglobin. Hemorrhagic anemia is also accompanied by ischemic optic neuropathy. Leukemia is a malignant disease of hematopoietic system, which is characterized by hyperplasia of a large number of uncontrolled leukocytes. Its etiology remains unknown. The incidence of leukemia ranks first children and adolescents and seventh in adults [5]. Because of abnormal leukocyte infiltration of organs, as well as decrease of normal blood cells, a series of clinical manifestations may occur. The clinical sign of the eye is divided into two types: one is caused by the infiltration of leukocytes, such as preretinal white patches due to infiltration, which is relatively rare (3%), and the other is secondary to anemia and thrombocytopenia, which is more common (39%), including conjunctival and retinal hemorrhage, retinal cotton-wool spots, and optic and retinal edema [6]. Retinal hemorrhage can contain a white center, white center can be caused by leukocyte infiltration, but in most cases it is associated with anemia (Figs. 4.23, 4.24, 4.25 and 4.26). Histopathological examination showed that there is choroidal infiltration of leukocytes but seldom found on fundus examination. Occasionally ­retinal pigment epitheliopathy and exudative retinal detachment were observed. Polycythemia is a syndrome caused by significant high hemoglobin concentration, erythrocyte count, and hematocrit in the peripheral blood, which is divided into primary polycythemia and secondary polycythemia. The fundus changes include papilla hyperemia, edema or atrophy, retinal vascular tortuosity, venous dilation, normal artery, or thin and retinal vascular occlusion (Figs. 4.27 and 4.28). Plasma cell dyscrasia is a disease caused by overhyperplasia of plasma cells or lymphocyte producing immunoglobulin, including primary Waldenstrom’s macroglobulinemia and myeloma. The change of fundus is induced by tissue hypoxia that is secondary to increase of blood viscosity and decrease of blood circulation. Retinal vein dilation, retinal hemorrhage, edema, and exudation can be seen in the fundus.

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Fig. 4.20  Fundus image of anemic retinopathy. Female patient, 39 years old, suffered from hemolytic anemia and thrombocytopenic purpura. Photographs show bilateral multiple Roth’s spots, which are reti-

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nal hemorrhages with white or pale centers, and hard exudates in macular area. (a) Fundus image of the right eye. (b) Fundus image of the left eye

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Fig. 4.21  Fundus image of anemic retinopathy. (a) Fundus image of the right eye. (b) Fundus image of the left eye

Fig. 4.22  Fundus image of anemic retinopathy. The photographs, taken from a 75-year-old male patient with multiple myeloma and secondary anemia, whose hemoglobin was 5.7g%, show bilateral scattered hemorrhage spots, some of which with white centers

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Fig. 4.23  Fundus image of anemic retinopathy. Note the bilateral scattered Roth’s spots and a few cotton-wool spots. (a) Fundus image of the right eye. (b) Fundus image of the left eye

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Fig. 4.25  Fundus image of anemic retinopathy. (a) Image of superior retina. (b) Image of retinal periphery. (c) Image of retinal posterior pole; note the gray lesion and abnormal vessels Fig. 4.24  Fundus image of anemic retinopathy. Photograph shows Roth’s spots

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Fig. 4.26  Fundus image of anemic retinopathy. (a) Fundus image of the right eye. (b) Fundus image of the left eye. Note the bilateral elevated gray lesion on optic nerve head

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Fig. 4.27  Fundus image of polycythemia. Photographs show bilateral swelling and pale optic nerve head, tortuous retinal vessels, dilated retinal vein, and narrowing arteriole. (a) Fundus image of the right eye. (b) Fundus image of the left eye

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Fig. 4.28  FFA images corresponding to Fig. 4.27. (a–c) show the early phase of the left eye; note the slow dye filling in the retinal artery. (d) shows the late phase of the left eye; note the hyperfluorescence and dye leakage of optic nerve head

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4.2

 xudative Retinal Detachment E Related to Eye Diseases

4.2.1 Vogt-Koyanagi-Harada Syndrome Vogt-Koyanagi-Harada (VKH) syndrome is a multisystemic inflammatory disorder, characterized by bilateral uveitis, meningeal irritation signs, auditory dysfunction, and integumentary manifestations such as vitiligo, alopecia, and ­poliosis [7]. The pathogenesis remains uncertain. The onset of VKH syndrome is frequently in young and middle-aged patients and shows a slight female predilection.

4.2.1.1 Clinical Manifestations VKH syndrome can be divided into four different phases [8]: Prodromal phase: The prodromal phase occurs several days ahead of the ocular symptoms, which is characterized by slight fever, headache, orbital pain, nausea, etc. Uveitic phase: Patients get sudden bilateral vision loss in this phase. The majority of patients show posterior uveitis, while the minority show anterior uveitis; but both can develop to panuveitis in the end. The changes of fundus include optic nerve hyperemia and edema, thickening of posterior choroid and retina, and multiple subretinal fluids, which can be exudative bullous serous retinal detachment in severe cases (Figs. 4.29, 4.30, 4.31 and 4.32). Convalescent phase: The active inflammation subsides about 8 weeks later, followed by depigmentation of the skin and choroid, resulting in a classic orange-red discoloring fundus which is known as “sunset glow fundus”. DalenFuchs nodules (Fig.  4.33), representing as yellowish-white lesions located in retinal pigment epithelium layer, also can be seen in this phase. Those nodules result from inflammatory infiltrations in fundus and consist of macrophages, epithelioid cells, lymphocytes, and retinal pigment epithelial cells. Recurrent phase: Ocular complications are relatively common in this phase, such as synechia of the iris, glaucoma, cataract, and subretinal neovascularization. Fundus Fluorescein Angiography (FFA) Examination: In acute uveitic phase, multiple hyperfluorescent dots which leak with time can be seen in FFA, and dye pooling into subretinal space and dye leakage beyond the optic disc can be seen at the late phase of FFA (Figs. 4.34, 4.35 and 4.36). In convalescent phase, salt-and-pepper-like changes and atrophic spots of retinal pigment epithelium and choroid, which

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is due to diffuse pigment epithelial lesions, can be seen in FFA (Figs. 4.37, 4.38, 4.39 and 4.40). Natural course: The prognosis of VKH syndrome is usually bad, especially for patients into the fourth phase, among whom 35% of patients develop synechia of the iris and glaucoma [9–11]. Treatment: VKH syndrome can be successfully treated with systemic corticosteroids. The majority of patients preserve good vision after proper treatment. Differential diagnosis: Other types of uveitis should be considered in the differential diagnosis of VKH syndrome, for example, sympathetic ophthalmia, acute posterior multifocal placoid pigment epitheliopathy (APMPPE), uveal effusion syndrome, posterior scleritis, primary ocular B-cell lymphoma, and Lyme disease. Primary ocular B-cell lymphoma, used to be called reticulum cell sarcoma, is non-Hodgkin’s lymphoma. It occurs mainly in middle-aged patients, representing as bilateral asymmetrical lesions (Figs. 4.41 and 4.42). Posterior uveitis and vitreous inflammation are common signs, accompanied with neurological symptoms.

Fig. 4.29 Fundus image of Vogt-Koyanagi-Harada syndrome. A 37-year-old female patient claimed sudden vision loss for a few days, with visual acuity 20/100 in the right eye and 20/640 in the left eye on first visit. Photograph shows multiple bullous retinal detachment at posterior pole and hyperemic optic disc in the left eye

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Fig. 4.30  Fundus image of Vogt-Koyanagi-Harada syndrome. Photograph shows macular edema and radial chorioretinal folds at posterior pole (a, b)

Fig. 4.31  Fundus image of Vogt-Koyanagi-Harada syndrome. Note the macular edema and retinal fold

Fig. 4.32 Fundus image of Vogt-Koyanagi-Harada syndrome. A 48-year-old male patient claimed bilateral vision loss for 3 months. He had been treated with cortical steroid, but the symptoms aggravated after cessation of treatment. The visual acuity was counting fingers on first visit. Photographs show bilateral retinal edema at posterior pole, macular radial folds, and exudative retinal detachment in inferior retina, in which the subretinal fluid can move when changing position. (a) Fundus image of the right eye. (b) Fundus image of the left eye

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Fig. 4.34  FFA images of the patient in Fig. 4.29. (a) The early phase of FFA; note the multiple pinpoint hyperfluorescence. (b) The late phase of FFA; note the pinpoint leakage, dye pooling in the area of bullous retinal detachment, and optic disc hyperfluorescence Fig. 4.33  Fundus image of Vogt-Koyanagi-Harada syndrome in convalescent period. A 45-year-old female patient claimed bilateral vision loss for 3 months, with visual acuity 20/100 in right eye and 20/40 in left eye on first visit. Fundus images (a, b) show bilateral sunset glow fundus. Note the scattered yellow nodules known as Dalen-Fuchs nodules both at posterior pole

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Fig. 4.35  FFA images of the patient in Fig. 4.30. Photograph shows dye leakage on and around the optic disc and dye pooling in the area of retinal detachment in the macula and inferior retina (a, b)

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Fig. 4.36  FFA images of the patient in Fig. 4.31. Note bilateral multiple leaking point and dye pooling in the area of bullous retinal detachment. (a) FFA image of the right eye. (b) FFA image of the left eye

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Fig. 4.37  FFA images of the patient in Fig. 4.33. (a) FFA image of right eye; note the massive dye leakage, especially in macular area and optic disc, with cystoid macular edema. (b) FFA image of the left eye; note the hypofluorescence dark dots and transmitted fluorescence spots

Fig. 4.38  Fundus image post-steroid treatment of the patient in Fig.  4.32. Photograph shows sunset glow fundus, with irregular pigmentation and depigmentation

Fig. 4.39  Fundus image of late-stage Vogt-Koyanagi-Harada syndrome. Photograph shows sunset glow fundus with massive pigment epithelium atrophy at posterior pole in the left eye

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Fig. 4.40  Fundus image of late-stage Vogt-Koyanagi-Harada syndrome. Photograph shows sunset glow fundus

Fig. 4.41  Fundus image of retinopathy caused by primary intraocular B-cell lymphoma. Photograph shows white lesions of retinal infiltration inferior to the optic disc and in the lower vascular arcade

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Fig. 4.42  Fundus image of the patient in Fig. 4.41 2 months after chemotherapy. Photograph shows the white lesions in Fig.  4.41 totally vanished

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4.2.2 Central Serous Chorioretinopathy Central serous chorioretinopathy (CSC) mostly affects patients in the age group 20–50, and males are more commonly affected than females. About 20% of patients have bilateral lesions. The pathogenesis of CSC remains uncertain. Clinical symptoms are blurred vision, distorted vision (metamorphopsia), and objects seeming smaller than normal (micropsia). The vision decreases mildly or moderately, usually better than 20/40. Fundus examination: Localized round serous retinal detachment can be visualized in the macula (Figs. 4.43, 4.44, 4.45 and 4.46). A few weeks after onset, small yellow dots appear in subretinal space. Retinal pigment epithelium detachment (RPED), which appears as small gray or grayishyellow lesion, can be seen within or without the area of serous retinal detachment (Figs.  4.47, 4.48 and 4.49). In severe recurrent cases, retinal detachment appears in inferior periphery, resulting in extensive epithelial depigmentation after a longtime pileup of subretinal fluid. Optical coherence tomography (OCT) helps to detect tiny RPED and retinal detachment (Figs. 4.44, 4.50 and 4.51). Fundus fluorescein angiography (FFA) examination: FFA demonstrates discrete hyperfluorescent points with leakage, among which some appear in classic smokestack or ink stain shape. Dye pooling in retinal detachment area can be ­visualized at the late phase of FFA. The leakage point, which

Fig. 4.43  Fundus image of CSC.  Photograph shows serous retinal detachment in the macula

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can be single or multiple, usually locates within 1 mm range from central fovea of the macula (Figs.  4.52, 4.53, 4.54, 4.55, 4.56 and 4.57). Long depigmented zones extending from posterior pole to periphery can be detected in some cases. Natural course: CSC is self-limited disease with good prognosis for vision. Subretinal fluid will be absorbed during 3–6 months in most patients, followed by visual acuity recovery. Incomplete vision recovery occurs in a few patients, accompanied with continuous metamorphopsia or micropsia. Twenty to 50% of patients relapse. Severe vision decrease occurs in patients who have undergone long-term clinical course or repeated relapse. Differential diagnosis: Serous pigment epithelial detachment is one of the clinical manifestations in CSC, but it also can be seen in other macular diseases (Figs. 4.48, 4.49, 4.50 and 4.51). Other diseases like rhegmatogenous retinal detachment, choroidal neoplasms, central exudative chorioretinopathy, age-related macular degeneration, uveitis, and retinal vasculopathy, which cause macular edema, can show the same clinical symptoms as CSC, leading to misdiagnosis. Treatment: For leakages at a safe distance from the fovea, direct focal laser photocoagulation with low intensity is ­beneficial to visual acuity recovery and shortening of clinical course. Low-fluence photodynamic therapy (PDT) is also effective for treatment of patients with both acute and chronic CSC [12, 13].

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Fig. 4.44  OCT image of CSC of the patient in Fig. 4.43. The image shows retinal detachment in the macula

Fig. 4.45  Fundus image of CSC. Photograph shows a round area of serous retinal detachment, whose diameter is about 2.5 DD

Fig. 4.46  Fundus image of CSC. A 43-year-old male patient claimed vision loss and visual distortion in the right eye, with the visual acuity counting fingers. Photograph shows bullous retinal detachment in the macula, the diameter of which is about 4DD

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Fig. 4.48  Fundus image of RPED. Photograph shows a 2–3 DD-sized RPED with clear margin in the temporal inferior macular zone of the right eye

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Fig. 4.49  Fundus image of RPED. Photograph shows a 2–3 DD-sized RPED with clear margin in the inferior temporal macular zone of the left eye

Fig. 4.47  Fundus image of CSC combined with RPED. A 45-year-old female patient claimed visual distortion in the right eye for 1 year, with visual acuity 20/200. Photograph shows bullous retinal detachment both in the macula and lower vascular arcade. Temporal to the foveola, there is a 1 DD-sized retinal pigment epithelium detachment (RPED), representing as an orange round elevated lesion with clear margin (a). (b, c) FFA images of the patient of the CSC

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Fig. 4.50  OCT image of RPED. Note those two RPED adjacent to each other. One of them is located inside the retinal detachment area

Fig. 4.51  OCT image of RPED. Note the small RPED inside the area of retinal detachment

Fig. 4.52  Fundus image of CSC. A 40-year-old male patient claimed vision loss in right eye, with visual acuity 20/200. Photograph shows retinal detachment in the macula, the lower margin of which exits gray depigmentation area extending to the peripheral retina

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Fig. 4.53  FFA images of the patient in Fig. 4.47. (a) The early phase of FFA shows a hyperfluorescent spot with clear margin according to the orange lesion (RPED) temporal to the foveola in Fig. 4.47. There are small hyperfluorescent dots 1 DD superior temporal and inferior to

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Fig. 4.54  FFA images of the patient in Fig. 4.43. (a) Early phase of FFA shows tiny hyperfluorescent dots superior temporal to the foveola, which are the leaking points.(b) Middle phase of FFA shows the leak-

that spot. (b) The late phase of FFA, the hyperfluorescent spot remains unchanged, while the hyperfluorescent dots enlarge. There is also bullous dye pooling in the lower vascular arcade

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ing points enlarge. (c) Late phase of FFA shows the leaking points enlarge further accompanied by dye pooling around the macula

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Fig. 4.55  FFA images of the patient in Fig. 4.45. (a) Early phase of FFA shows tiny hyperfluorescent dots in central fovea. (b, c) Late phase of FFA shows those hyperfluorescent dots enlarge as “mushroom” in shape

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Fig. 4.56  FFA images of the patient in Fig. 4.52. (a) Early phase of FFA shows hyperfluorescent dots in macular zone. (b) Late phase of FFA shows most of the hyperfluorescence dots fade, while three of them enlarge, with diffuse hyperfluorescence in the lower vascular arcade

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Fig. 4.57  FFA images of the patient in Fig. 4.46. (a) Early phase of FFA shows tiny hyperfluorescent dots temporal to the foveola. (b) Late phase of FFA shows hyperfluorescent dots leaking with dye pooling

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4.2.3 Central Exudative Chorioretinopathy Central exudative chorioretinopathy (CEC) is characterized by subretinal neovascularization in the macula and usually appears in young and middle-aged adults. It is similar to idiopathic choroidal neovascularization (ICNV) with a difference that the former is very common in China and causes of infections such as tuberculosis and toxoplasmosis can be found in parts of the patients, while the latter has no definite causes. The symptoms and manifestations in fundus are similar to that of age-related macular degeneration, but the macular lesion is smaller without any drusen around. Fluorescein angiography

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shows the choroid neovascular with dye leakage clearly. The neovascular membrane, when active, is often accompanied by crescent-like or ringlike hemorrhage (Figs. 4.58 and 4.59) and is much more quiet with absorption of hemorrhage when lasts long (Fig. 4.60). Central exudative chorioretinopathy should be differentiated from central serous chorioretinopathy as both can manifest macular subretinal fluid. Treatment and prognosis: Anti-VEGF therapy and antiinfection therapy to those with definite infectious cause are effective [14]. Since the lesion is smaller than that of agerelated macular degeneration, the prognosis of visual acuity is much better.

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Fig. 4.58  Fundus images of central exudative chorioretinopathy. A 23-year-old male patient claimed vision loss with metamorphopsia and micropsia in the left eye for 4 months. (a) Color fundus photograph

shows gray lesion (CNV) adjacent to focal retinal detachment. (b) Early phase of FFA shows hyperfluorescence CNV in the macula. (c) Late phase of FFA shows mild leakage of CNV

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Fig. 4.59  Fundus images of central exudative chorioretinopathy. A 25-year-old female patient claimed visual distortion in the left eye for 2 months. (a) Color fundus photograph shows macular gray lesion (CNV)

surrounded by hemorrhage and focal retinal detachment. (b, c) Late phase of FFA shows CNV leakage

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Fig. 4.60  Fundus images of central exudative chorioretinopathy. A 36-year-old male patient claimed visual distortion in the left eye for half a month, with visual acuity 20/50 in right eye and 20/200 in left eye. (a) Color fundus photograph shows macular gray lesion (CNV) surrounded by hemorrhage. (b) Early phase of FFA shows well-marginated hyper-

fluorescent lesion (CNV) that is surrounded by hypofluorescence corresponding to the hemorrhage. (c) Late phase of FFA shows mild leakage of CNV. (d) OCT shows high reflective lesion with interruption of RPE (CNV) (white arrow), subretinal hemorrhage, and serous retinal detachment

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4.2.4 Bullous Retinal Detachment Bullous retinal detachment is an exaggerated form of CSC characterized by multiple leaking points of RPE with massive bullous serofibrinous retinal detachment [15]. So it is also known as multifocal posterior pigment epitheliopathy (MPPE) [16]. Clinical features: It is similar to CSC; usually occurs in healthy young or middle-aged adults, predominantly males with or without history of CSC; and can be unilateral or bilateral without any inflammation in ocular refracting media. The disease has a tendency of self-healing. Corticosteroid therapy doesn’t help but aggravates the condition [14]. Funduscopic examination can show multiple bullous RD and retinal folds at posterior pole, with yellow grayish subretinal exudation and pigmentation and with nonmigrating non-rhegmatogenous retinal detachment in inferior retina (Figs. 4.61, 4.62 and 4.63).

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FFA images show multiple leaking points of RPE layer at posterior pole and dye leakage with time as shapes of smokestack, ink stain, or other shapes (Fig.  4.64). Indocyanine green angiography (ICGA) shows that in the early phase, dye filling delay lasting about 2  min can be seen at or in the vicinity of the leaking points, with choroidal vascular dilation at posterior pole or equator; in the mid and late phase, the leaking points, which are corresponding with those in FFA, appear in ICGA, with diffuse hyperfluorescence around and even outside the temporal superior and inferior arcade. Natural course: The disease may last a year or more. Long-term RD can cause subretinal proliferation and massive RPE atrophy at posterior pole, leading to bad visual acuity. Differential diagnosis: It should be differentiated from rhegmatogenous retinal detachment and exudative retinal detachment identification caused by Vogt-Koyanagi-Harada (VKH) syndrome, choroidal tumor, and retinal vascular disease. Treatment: The treatment is similar to that of CSC. Laser photocoagulation and photodynamic therapy are effective and may improve the prognosis [17, 18]. If the subretinal fluid is too much for a patient to undergo laser therapy, drainage via the sclera can be done in advance (Figs. 4.65 and 4.66).

Fig. 4.61  Fundus image of bullous retinal detachment. Note bullous retinal detachment in the macula of the left eye. The subretinal fluid is transparent

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Fig. 4.62  Fundus image of bilateral bullous retinal detachment. Photograph shows scattered retinal epitheliopathy combined with retinal detachment at posterior pole. Some laser spots can be detected. (a) Image of the right eye. (b) Image of the left eye

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Fig. 4.63  Fundus image of bullous retinal detachment. A 40-year-old female patient claimed vision loss for nearly 1 year with visual acuity light perception on first visit. The diagnosis was multiple posterior pigment epitheliopathy with bullous retinal detachment. Subretinal fluid was totally absorbed after direct laser photocoagulation on leaking

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Fig. 4.64  FFA images of the patient in Fig.  4.63. (a, b) are images before treatment. (a) Early phase of FFA shows massive hyperfluorescence at posterior pole. (b) Late phase of FFA shows hyperfluorescent

points twice. (a) Fundus image before laser treatment shows exudates in the vicinity of lower vascular arcade and temporal to the foveola, with retinal detachment in inferior retina which can move when changing position. (b) Fundus image 1.5 months after first laser treatment; subretinal fluid was mostly absorbed with visual acuity back to 20/200

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lesions partially fade while partially leak (temporal to the foveola). (c, d) are images 1.5 months after treatment. Note the leaking points reduced a lot

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Fig. 4.64­ (continued)

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Fig. 4.65  Fundus images of bullous retinal detachment. (a, b) are images before treatment; note retinal detachment in posterior pole, radial folds in the macula, yellowish-white exudates in the lower vascular arcade, and

exudative retinal detachment in inferior retina which can move when changing position. (c) is the fundus image 6 weeks after treatment. Note multiple depigmented lesions after total absorption of subretinal fluid

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Fig. 4.66  FFA images of the patient in Fig.  4.65. (a, b) are images before treatment. (a) Early phase of FFA shows multiple hyperfluorescent dots. (b) Late phase of FFA shows leakage of those hyperfluores-

cent dots. (c, d) are images after treatment. (c) Early phase of FFA shows diffuse hyperfluorescence at posterior pole. (d) Late phase of FFA shows the hyperfluorescence partially fade without any leakage

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4.2.5 Uveal Effusion Syndrome Uveal effusion syndrome (UES) is a special type of secondary retinal detachment, which can be divided into idiopathic versus one associated with nanophthalmos. UES was first reported in the year 1963 by Schepens and Brockhurst [19]. It may be caused by congenital tissue abnormalities of the sclera and compressed cortex vein, resulting in hindered outflow of intraocular fluid and fluid retention in suprachoroidal and supraciliary space and then causing retinal detachment. UES occurs mostly in healthy male middle-aged adults. It is bilateral and can affect one eye after the other. Clinical features: Dilation of episcleral vessels and congestion of Schlemm’s canal can be noticed (Fig. 4.67). There is no obvious inflammation in the anterior segment, and the intraocular pressure can remain normal. Funduscopic examination shows annular peripheral choroidal detachment and choroidal thickening at posterior pole. It can also show serous retinal detachment shifting with head positons, for example, the subretinal fluid accumulates in inferior retina when seated (Fig. 4.68a). Chronic a

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Fig. 4.67  External photograph of idiopathic uveal effusion syndrome. Note dilated and hyperemic episcleral vessels

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Fig. 4.68  Fundus images of idiopathic uveal effusion syndrome. (a) Color fundus photograph of the left eye shows serous retinal detachment shifting with head positions. The subretinal fluid accumulates in

inferior retina when seated. (b, c) are FFA images; note the leopard spots and hyperfluorescence on optic disc. (d) Color fundus photograph of right eye shows pigmentation deposits at posterior pole

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disease may lead to secondary retinal pigment epithelial changes (leopard spot). Subretinal white yellowish deposits may appear at posterior pole in the later stage of UES, and papillary edema may appear in some of the patients. FFA images reveal dye filling delay of the choroid in the early phase, and leopard spots (pigmentation alternating with depigmentation) without leakage are noted (Fig. 4.68).

Fig. 4.69  Ultrasonography image of idiopathic uveal effusion syndrome. B-scan ultrasonography image shows annular choroidal detachment

Fig. 4.70  UBM image of idiopathic uveal effusion syndrome. Note detached ciliary body detachment and thickened sclera

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Optic nerve head capillary dilation with dye leakage appears in some cases. B-scan ultrasonography shows annular choroidal detachment and thickening of the choroid at posterior pole (Fig. 4.69). UBM shows a thickened sclera and supraciliary effusion (Fig. 4.70).

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4.2.6 Pathological Changes The lamellar sclera removed from patient with UES can be detected 1.5–3 times thicker than the full-thickness sclera of normal corpse after H-E staining, with fibrous structural disorder, interlaced or curled; fiber thickening; and scleral cell atrophy but without inflammatory cell infiltration [20] (Figs. 4.71, 4.72 and 4.73). Histochemical staining shows ingredient anomaly of the sclera. Alcian blue (0.1%) staining, colloidal iron staining, or alcian blue plus PAS staining is positive, especially inside the sclera, which proves the existence of glycoprotein and acid mucopolysaccharide in the abnormal sclera (Figs. 4.74, 4.75 and 4.76). Under electron microscope, lots of granular deposits like glycosaminoglycan (mucopolysaccharide) or mucoprotein can be detected in collagen fiber (Figs. 4.77 and 4.78). Shape anomaly of scleral cells with karyopyknosis, reduction or loss of cytoplasm, and disappearance of organelles can also be detected (Fig. 4.79). Pathogenesis: Under normal circumstances, there are two ways for the protein outside the intraocular vessels to drain out of the eye: first, it drains into Schlemm’s canal and then via aqueous vein into vortex vein, and second, it diffuses through the sclera. The scleral thickening or structural anom-

Fig. 4.71  H-E staining of normal full-thickness sclera observed under light microscope (×100). Note neatly arranged collagen fiber and fusiform or rod-shaped dark-stained scleral nucleus

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aly, plus the increased glycosaminoglycan with high hydrophilicity which can cause scleral swelling, will both result in the impairment of permeability of the sclera [17, 21]. The vortex was compressed by swollen sclera, narrowing or congenital missing, which hinders the outflow of protein. Thus, the hypertonic fluid with high protein accumulates in the choroid and suprachoroidal space, causing choroidal thickening and choroidal detachment and then retinal detachment. The normal drainage of the fluid is hindered; that’s why the intraocular pressure is normal or higher than normal and even the choroidal detachment exists [18, 22]. Long-term subretinal fluid impairs the function of RPE, causing proliferation and migration of RPE cells, resulting in typical changes of “leopard spot.” Protein deposits at the posterior pole can be observed in the later stages of UES.  It is supposed that the permeability of the sclera degenerates with age, so UES occurs in middle-aged adults even though the scleral anomaly is congenital. Treatment and prognosis: Lamellar sclerectomy with a smaller scleral excision to expose the underlying choroid can effectively improve the permeability of the sclera. Subretinal fluid can be absorbed, and reattachment of the retina can be obtained gradually after surgery. The prognosis of visual acuity depends on the length of the course (Figs. 4.80 and 4.81).

Fig. 4.72  H-E staining of full-thickness sclera of UES patient observed under light microscope (×100). The sclera showed obvious thickening, and the resected lamina sclera was more than three times thicker than the normal full-thickness sclera. The arrangement of collagen fibers was disordered, interlaced, or curled. The fibers were thickened, and fibrous structures were obscure

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Fig. 4.73  H-E staining of full-thickness sclera of UES patient observed under light microscope (×100). Note scleral cell atrophy and degeneration (lightly stained) without inflammatory cell infiltration

Fig. 4.76  Histochemical staining of pathologic sclera (×100). Alcian blue (0.1%) staining is positive

Fig. 4.74 Histochemical staining of pathologic sclera (×100). Colloidal iron staining is positive

Fig. 4.77  Normal sclera observed under electron microscope (×1500). Note neatly arranged collagen fiber. Distinct gray nodes are visible

Fig. 4.75  Histochemical staining of pathologic sclera (×100). Alcian blue plus PAS staining is positive

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Fig. 4.78  Pathologic sclera observed under electron microscope. (a) Note vacuolar degeneration and swelling of scleral fiber with fuzzy structure (×2000). (b) Note swelling collagen fibers with fuzzy structure gathering and granular glycogen-liked deposits in different sizes (×1000)

Fig. 4.79 Pathologic sclera observed under electron microscope (×2000). The image shows scleral cells with karyopyknosis, severe reduction of cytoplasm, and residual organelles with vague structure. The space between collagen fibers enlarges

Fig. 4.80  Fundus image of patient with uveal effusion syndrome associated with nanophthalmos. A 46-year-old female patient with ocular axis 14 mm. Note retinal detachment in inferior retina

Fig. 4.81  Fundus image of the patient in Fig. 4.80 after surgery. This image shows reattached retina with leopard spots 2 years after lamellar sclerectomy

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4.2.7 Posterior Scleritis Posterior scleritis is a rare autoimmune disease targeting the sclera. The disease, which can be idiopathic or associated with connective tissue diseases, occurs mainly in female, usually accompanied with anterior scleritis. It is hard to diagnose since the lesion locates at the posterior of the eyeball. Symptoms: The symptoms of posterior scleritis include ocular pain, blurred vision, refractive error, double vision (diplopia), bulging eyes (proptosis), etc. Physical examination: Retinal edema and folds, even exudative retinal detachment, and signs of choroiditis can be observed at posterior pole. Ultrasonography and computed tomography disclose sclerochoroidal thickening (Fig. 4.82). Treatment: Systemic cortical steroid is effective to treat posterior scleritis.

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Fig. 4.82  Fundus image and ultrasonography image of posterior scleritis. (a) Fundus image shows retinal detachment with turbid subretinal fluid. (b) Ultrasonography image of posterior scleritis shows thickening of the ocular wall and T-sign

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4.2.8 Sympathetic Ophthalmia Sympathetic ophthalmia (SO) is bilateral granulomatous panuveitis that usually occurs after an open globe injury or intraocular surgery. It is a rare disease, with an estimated incidence of 0.19–0.7% after injury and 0.007–0.015% after intraocular surgery [23, 24]. Clinical features: SO is characterized by an insidious onset and generally occurs 4–8 weeks after trauma or surgery, out of which about 70% occurs within 3 months, while 90% occurs within 1 year [25, 26]. The ocular findings are similar to VKH (Figs. 4.83, 4.84, 4.85 and 4.86).

Fig. 4.83  Fundus image of sympathetic ophthalmia. A 45-year-old male patient claimed vision loss in the left eye for 2 months, whose right eye had been enucleated for 3 months after trauma. Visual acuity was 0.5 on first visit. Photograph shows massive depigmented lesions and a few atrophic spot at posterior pole

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Prognosis: The prognosis of visual acuity is related to the level of inflammation and when the diagnosis is made. Generally speaking, the lighter the inflammation is and the earlier the diagnosis and treatment are given, the better the prognosis will be. Differential diagnosis: SO should be differentiated with uveitis of other kind. Positive history of ocular trauma without systemic diseases or infection is significant for the diagnosis of SO. Treatment: The preferred treatment strategy for SO is systemic cortical steroid. The majority of patients can obtain better visual acuity after treatment.

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Fig. 4.84  FFA images of the patient in Fig. 4.83. Photograph shows mild dye leakage on and around optic disc and multiple hyperfluorescent spots (choriocapillary atrophy) with mild leakage of small vessels in periphery

Fig. 4.85  Fundus image of sympathetic ophthalmia. A 30-year-old male patient claimed vision loss in the left eye for a few days, whose right eye had been enucleated for 8 years after trauma. Photograph shows multiple bullous retinal detachment at posterior pole Fig. 4.86  FFA images of the patient in Fig. 4.85. Note multiple leaking point and dye pooling inside retinal detachment area

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4.2.9 A  cute Posterior Multifocal Placoid Pigment Epitheliopathy Acute posterior multifocal placoid pigment epitheliopathy (APMPPE), also known as acute multifocal ischemic choroidopathy (AMIC), is a rare inflammatory disease. It is first reported by Gass [27], who considered the RPE layer as the target. It predominantly affects young patients, bilateral or unilateral, and occurs alone or accompanied with systemic inflammatory diseases. Symptoms of respiratory or gastrointestinal viral infection can be the precursor of APMPPE. Symptoms: APMPPE presents with varying degrees of visual blurring, metamorphopsia, and scotomas. Fundus examination: The lesions are characteristically at the posterior pole or near the equator. They represent as multiple creamy colored or gray lesions with indistinct margins in the active phase. The lesions can merge together and turn to be brown ones with distinct margins a few days later, showing various degrees of pigmentation and depigmentation of RPE. As time goes by, the lesions become flat with choriocapillary and RPE atrophy. New lesions can be concurrent with old scars in the same eye. Some cases may involve papillary edema, intraretinal hemorrhage, retinal edema, and even serous retinal detachment in a minority of patients.

FFA images show lobular absence of early background choroidal fluorescence underneath the active lesions, and in the late phase, dye leakage and staining [28]. As for old scars, RPE pigmentation causes fluorescein blockage, and RPE depigmentation causes transmission defects. In cases concurrent with papillary edema, FFA images show hyperfluorescence in optic disc, with distinct or indistinct margins. ICGA images show absent dye filling of choroidal lobule underneath the active lesion, which lasts till the late phase. In some cases, larger choroidal vessels can be observed within or around those dark areas. That explains the main cause of APMPPE: inflammation causes multiple choroidal vasculitis and precapillary arteriole blockage, leading to ischemic choroidal lobule, resulting in the secondary changes of RPE which is swelling at first then atrophy and pigmentation later (Figs.  4.87, 4.88, 4.89, 4.90, 4.91 and 4.92). Treatment principle: Cortical steroid is recommended in the active phase, and treatment aiming at the underlying causes is helpful in some cases. Prognosis: The visual outcome differs greatly due to the difference of lesion locality, and the visual acuity is better in patients without central fovea involved.

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Fig. 4.87  Color fundus photography, FFA and ICGA images for acute posterior multifocal placoid pigment epitheliopathy. (a) Color fundus photograph shows acute lesions, presented as creamy colored lobular lesions around the macula, which can turn into gray ones with time. (b) Early phase of FFA image shows lobular hyperfluorescent spots around

the macular. (c) Late phase of FFA image shows lobular hyperfluorescent spots leak gradually over time. (d) Early phase of ICGA shows lobular blockage of choroidal fluorescence from the acute lesions. (e) Late phase of ICGA shows lobular absence of choroidal fluorescence still exists

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Fig. 4.87 (continued)

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Fig. 4.88  Color fundus photography, FFA and ICGA images for acute posterior multifocal placoid pigment epitheliopathy. (a) Color fundus photograph shows widespread distribution of gray lobular lesions. (b) Early phase of FFA image shows lobular hyperfluorescent spots accom-

pany with some hypofluorescent lesions. (c) Late phase of FFA image shows dye staining of the lobular lesions without leakage. (d) ICGA shows some hypofluorescence which documents the choriocapillaris and pigment epithelial atrophic changes

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Fig. 4.88 (continued)

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Fig. 4.89  Fundus image of acute posterior multifocal placoid pigment epitheliopathy. Photograph shows multiple small lesions located in pigment epithelium layer at posterior pole, some of which merge together (a, b, c, d)

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Fig. 4.90  FFA images of the patient in Fig. 4.89 (a, b, c, d). Note the transmitted fluorescence of those lesions in Fig. 4.89

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Fig. 4.91  Fundus image of acute posterior multifocal placoid pigment epitheliopathy. (a, b, c, d) Photograph shows multiple small pigmented lesions in posterior pole and midperipheral retina of the right eye, some of which merge together

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Fig. 4.92  FFA images of the patient in Fig. 4.91 (a, b, c, d). Note those multiple lesions in posterior pole and midperipheral retina, presented as hypofluorescence in the center with hyperfluorescence at the margin, some of which merge together

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4.2.10 Exudative Retinal Detachment Secondary to Central Retinal Vein Occlusion (CRVO) Exudative retinal detachment secondary to CRVO is rare and generally caused by increased permeability of impaired retinal vein (Figs.  4.93, 4.94, 4.95 and 4.96). It progresses quickly, resulting in bad outcomes. Subretinal fluid and retinal edema coexist with intraretinal hemorrhage in those cases, so it should be differentiated from central retinal artery occlusion (Figs. 4.97, 4.98, 4.99 and 4.100).

Fig. 4.95  Fundus image of CRVO. Note massive intraretinal and subretinal exudates and hemorrhages

Fig. 4.93  Fundus image of CRVO. Photograph shows optic disc with indistinct margins and multiple retinal hemorrhage at posterior pole

Fig. 4.96  Color fundus photograph of the inferior retina of the patient in Fig. 4.95. Photograph shows massive exudates and hemorrhages and retinal detachment in inferior retina with turbid subretinal fluid

Fig. 4.94  Color fundus photograph of the inferior retina of the patient in Fig. 4.93. Photograph shows massive exudates and hemorrhages and retinal detachment in inferior retina with turbid subretinal fluid

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Fig. 4.97  Fundus image of CRAO. Note the swollen retina at posterior pole and dark red central macula

Fig. 4.99  Fundus image of BRAO. Note the gray swollen inferior retina at posterior pole

Fig. 4.98  Fundus image of CRAO. Note severe swollen retina, gray in color, at posterior pole, with cherry-red spot in macular region

Fig. 4.100  Fundus image of BRAO. Note the gray swollen superior retina at posterior pole

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References 1. Weber MA, Schiffrin EL, White WB, et al. Clinical practice guidelines for the Management of Hypertension in the Community: a statement by the American Society of Hypertension and the International Society of Hypertension. J Hypertens. 2014;32(1):3–15. 2. Stropes LL, Luft FC. Hypertensive crisis with bilateral bullous retinal detachment. JAMA. 1977;238:1948–9. 3. Sohan SH, Gray ES, Prem SV. Macular lesions in malignant arterial hypertension. Ophthalmologica. 1989;198(4):230–46. 4. Report of the National High Blood Pressure Education Program. Working group report on high blood pressure in pregnancy. Am J Obstet Gynecol. 2000;183:S1–S22. 5. Torre LA, Siegel RL, Ward EM, Jemal A. Global cancer incidence and mortality rates and trends—an update. Cancer Epidemiol Biomarkers Prev. 2016;25(1):16–27. 6. Schachat AP, Markowitz JA, Guyer DR. Ophthalmic manifestations of leukemia. Arch Ophthalmol. 1989;107:697–700. 7. Damico FM, Kiss S, Young LH.  Vogt-Koyanagi-Harada disease. Semin Ophthalmol. 2005;20:183–90. 8. Rajendram R, Evans M, Rao NA. Vogt-Koyanagi-Harada disease. Int Ophthalmol Clin. 2005;45(2):115–34. 9. Chee SP, Jap A, Bacsal K. Spectrum of Vogt-Koyanagi-Harada disease in Singapore. Int Ophthalmol. 2007;27(2–3):137–42. 10. Yang P, Ren Y, Li B, Fang W, Meng Q, Kijlstra A. Clinical characteristics of Vogt-Koyanagi-Harada syndrome in Chinese patients. Ophthalmology. 2007;114(3):606–14. 11. Read RW, Rechodouni A, Butani N, Johnston R, LaBree LD, Smith RE, et al. Complications and prognostic factors in Vogt-KoyanagiHarada disease. Am J Ophthalmol. 2001;131(5):599–606. 12. Naseripour M, Falavarjani KG, Sedaghat A, et al. Half-dose photodynamic therapy for chronic central serous chorioretinopathy. J Ophthalmic Vis Res. 2016;11(1):66–9. 13. Liu HY, Yang CH, Yang CM, et  al. Half-dose versus half-time photodynamic therapy for central serous chorioretinopathy. Am J Ophthalmol. 2016;167:57–64. 14. Zhang C, Liu DN, Li XU. Intravitreal injection of lucentis for central exudative chorioretinopathy. Rec Adv Ophthalmol. 2014;34(9): 864–7.

115 15. Gass GDM.  Bullous retinal detachment: an unusual manifestation of idiopathic centre serous choroidopathy. Am J Ophthalmol. 1973;75:810–2. 16. Mitsunaga H, Nishimura T, Yuyama M.  Multifocal posterior pigment epitheliopathy: recently experienced cases. Jpn J Clin Ophthalmol. 1992;46:729–33. 17. Kuroyanagi K, Sakai T, Kasai K, Tsuneoka H.  Spectral domain optical coherence tomography and angiographic findings in multifocal posterior pigment epitheliopathy treated with low-fluence photodynamic therapy. Clin Exp Optom. 2012;11(10):1111–4. 18. Ng WW, Wu ZH, Lai TY. Half-dose verteporfin photodynamic therapy for bullous variant of central serous chorioretinopathy: a case report. J Med Case Rep. 2011;5:208. 19. Schepens CL, Brockhurst RJ.  Uveal effusion. 1. Clinical picture. Arch Ophthalmol. 1963;70:189–20. 20. Uyama M, Takahashi K, Kozaki J, et al. Uveal effusion syndrome: clinical features, surgical treatment, histologic examination of the sclera, and pathophysiology. Ophthalmology. 2000;107: 441–9. 21. Forrester JV, Lee WR, Kerr PR, et al. The uveal effusion syndrome and trans-scleral flow. Eye (Lond). 1990;4:354–65. 22. Liew SC, MeCluskey PJ, Parker G, et  al. Bilateral uveal effusion associated with sclera thickening due to amyloidosis. Arch Ophthalmol. 2000;118(9):1293–5. 23. Du Toit N, Motala MI, Richards J, et al. The risk of sympathetic ophthalmia following evisceration for penetrating eye injuries at Groote Schuur Hospital. Br J Ophthalmol. 2008;92:61–3. 24. Makley TA Jr, Azar A. Sympathetic ophthalmia. A long-term follow-up. Arch Ophthalmol. 1978;96(2):257–62. 25. Goto H, Rao NA.  Sympathetic Ophthalmia and Vogt-KoyanagiHarada syndrome. Int Ophthalmol Clin. 1990;30:279–85. 26. Lubin JR, Albert DM, Weisntein M. Sixty-five years of sympathetic ophthalmia. A clinicopathologic review of 105 cases (1913–1978). Ophthalmology. 1980;87:109–12. 27. Gass JDM. Acute pesterior multifocal placoid pigment epitheliopathy. Arch Ophthalmol. 1968;80:177–85. 28. Gendy MG, Fawzi AA, Wendel RT, et al. Multimodal imaging in persistent placoid maculopathy. JAMA Ophthalmol. 2014;132(1): 38–49.

5

Congenital Abnormalities of the Fundus and Retinal Detachment Yao Huang, Nan Zhou, and Wenbin Wei

Abstract

This chapter deals with congenital abnormalities of the fundus and retinal detachment. Congenital abnormalities of the fundus include morning glory syndrome, a specific congenital papillary dysplasia. Congenital optic disc pit is a type of congenital optic disc abnormality. Congenital coloboma of the optic disc can occur secondary to serous retinal detachment in the macular area or elsewhere. Large areas of myelinated nerve fibers can be manifested as “white pupils” and have been misdiagnosed as retinal detachment. Sometimes retinal detachment can occur in a choroid coloboma, and the hole is often located at the edge of the sclera. The main feature of Coats Disease, also known as external exudative retinopathy, is idiopathic retinal telangiectasia. Retinoschisis is the separation of layers within the retinal neuroepithelium, and it should be differentiated from rhegmatogenous retinal detachment.

5.1

Morning Glory Syndrome

Morning glory syndrome is a specific congenital papillary dysplasia, which, because its appearance is similar to that of a blooming morning glory, was named “morning glory syndrome” (by Kindler [1] in 1970). The exact pathogenesis of the syndrome is not clear, but it has been related to poor development of the posterior sclera and lamina cribrosa during gestation [2]. Some investigators believe that morning glory disc anomalies are caused by defects in the closure of the top of the embryonic fissure [3]. The prevalence of morning glory disc anomaly has been reported to be 2.6/100,000. Coexisting retinal peripapillary or macular edema was com-

Y. Huang, M.D., Ph.D. · N. Zhou, M.D., Ph.D. W. Wei, M.D., Ph.D. (*) Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China

mon, as were cerebral abnormalities and/or cutaneous vascular malformations [4]. 1. Clinical features Most morning glory disc anomalies are monocular. Generally visual acuity is poor from childhood. There are also extreme cases with visual acuity of 1.0, or no light perception. Fundus findings: The optic disc expands to two to five times the normal size and is pink or orange, with an opaque white or translucent glial tissue filling in its center. The optic disc is funnel-shaped, and there is a gray-­ white or pigmented annular bulge around it. Some cases have an irregular circular retina or zone of choroid atrophy outside this annular bulge. The central retinal blood vessels are distributed radially from the optic disc to the peripheral retina. The number of vascular branches can reach 20 or more. The blood vessels are thin and straight, and often bend suddenly when they originate from the optic disc. When the vessels reach the peripheral retina, they become straight, and it is difficult to distinguish arteries and veins. The macula can be involved and there are no normal macular structures in some cases (Figs. 5.1, 5.2, and 5.3). Fundus fluorescein angiography (FFA): In the early stage, in most cases, choroidal blood vessels can be seen through the atrophied pigment epithelium around the optic disc; the retinal artery and veins fill slowly, the vascular network is distorted by leakage and the central optic disc shows hypofluorescence as a result of being filled with collagen tissue. In the late stage, the optic disc shows diffuse hyperfluorescence. Morning glory syndrome may be associated with other congenital eye abnormalities, such as microphthalmia, optic disc coloboma, persistent hyperplastic primary vitreous, anterior chamber angle schisis syndrome, pupil remnant, lens opacification, and strabismus. Morning glory syndrome can also be associated with other systemic abnormalities.

© Springer Nature Singapore Pte Ltd. and Beijing Science and Technology Press 2018 W. Wei (ed.), Atlas of Retinal Detachment, https://doi.org/10.1007/978-981-10-8231-3_5

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2. Morning Glory Syndrome with Retinal Detachment About 26%–38% of patients with morning glory syndrome will develop retinal detachment. The typical retinal detachment originates from the optic disc, and extends to the posterior pole of the fundus and may even develop into complete retinal detachment. The subretinal proliferation is the same as that in rhegmatogenous retinal detachment (RRD). Retinal breaks are very rare (Figs. 5.4, 5.5, 5.6 and 5.7). The following causes of retinal detachment have been reported:

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traffic between the vitreous cavity and the subarachnoid space in an abnormal optic disc. 3. Abnormal retinal blood vessels around the optic papilla cause fluid to enter the subretinal space. Kindler et al. [1] suggested that retinal detachment at the posterior pole may be related to abnormal retinal vascular leakage. Akiyama et al. [8] reported that abnormal vascular leakage could cause peripheral exudative retinal detachment. They noted that the multiple vascular stenoses around the papilla and the retinal arteriovenous transit in morning glory syndrome caused peripheral retinal ischemia and neovascularization.

1. The liquefied vitreous enters the subretinal space through the optic cup. Bartz [5] reported that octafluoropropane (C3F8) injected into the vitreous cavity was found to enter the subretinal space. This suggests that retinal breaks in the abnormal optic discs provide a link between the vitreous and the subretinal space and cause retinal detachment. 2. Abnormal traffic in the subarachnoid space causes cerebrospinal fluid to enter the subretinal space. Ho and Wei [6] reported a case of morning glory syndrome with retinal detachment. On computed tomography brain pool angiography, the contrast agent was found to enter the subretinal space, while no contrast agent appeared in the vitreous cavity. This indicated that the subretinal fluid was related to subarachnoid traffic abnormalities. Irvine et al. [7] also reported a case of morning glory syndrome with retinal detachment. Their patient received an intravitreal gas injection combined with optic nerve fenestration. The bubble was discharged from the dura during the surgery. This also confirmed the presence of abnormal

Fig. 5.2  Fundus image of morning glory syndrome. The optic disc is abnormal with many vascular branches and an atrophic ring around it

Fig. 5.1  Fundus image of morning glory syndrome. The best corrected visual acuity in the left eye of this 6-year-old boy is 0.1 (−8.0D). The optic disc is big, the optic cup is deep, and retinal vessels are abnormal, with many branches

Fig. 5.3  Fundus image of morning glory syndrome. The optic disc is abnormally enlarged, with a very deep optic cup. The retinal blood vessels are knee-like crawling with many branches and abnormal travelling

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Fig. 5.4  Morning glory syndrome with retinal detachment in the right eye in a 4-year-old girl. The optic disc is abnormally enlarged, with retinal detachment (RD). Central retinal blood vessels with numerous branches originate from the optic disc and are distributed radially to peripherally. It is difficult to distinguish arterial and venous vessels. Different from conventional RD, the detached retina does not originate from the edge of the optic disc

Fig. 5.6  Morning glory syndrome with RD in the right eye before surgery in a 4-year-old boy. The optic disc was pink, was four times the normal size, and was filled with white glial tissue in the center. There was a circular elevation around the optic disc. More than 20 central retinal vessels originated from the optic disc and were distributed radially to peripherally. The blood vessels were straight, and suddenly became curved when they originated from the optic disc. It was difficult to distinguish arteries and veins. The RD was not new and there was extensive subretinal pigment and a proliferative membrane. There was no retinal hole

Fig. 5.5  Morning glory syndrome with RD in the right eye in a 10-year-old boy. The inferior optic disc was filled with opaque white or translucent glial tissue

Fig. 5.7  Morning glory syndrome with RD in the right eye after surgery in a 4-year-old boy. The retina was reattached postoperatively. The lesion located at the superior nasal disc of the optic disc was closed after having been cut during surgery to drain subretinal fluid

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Congenital Optic Disc Pit

Congenital optic disc pit is a type of congenital optic disc abnormality. Because it is a congenital dysplasia, it is associated with other eye abnormalities, such as an arc below the optic disc, a membrane above the optic disc, a residual vitreous artery, and abnormal pupil. If there is no secondary serous macular detachment, there is no visual impairment and the pit is not easily found. The condition has monocular or binocular onset, with no gender difference. The pit is present at birth. In about half of these patients macular edema or retinal detachment occurs at 20 to 40 years of age. This will present as metamorphopsia or micropsia [9]. 1. Fundus characteristics An optic disc with a pit is slightly larger than a normal optic disc. The pit is always located at the temporal region of the optic disc; the pits are mostly single, their width is about 1/8–1/2 pupillary diameter (PD), and their depth is variable. Some pits are filled with translucent or pigmented tissue. Retinal detachment occurs between the vascular arch in the posterior pole connecting with the optic disc. There is no retinal hole. If the course of the disease is long, there may be secondary cystoid macular degeneration, or even the formation of macular holes, which leads to further reduction of visual acuity (Fig. 5.8). If there is no retinal detachment, the visual field can show expansion of the blind spot owing to the large optic disc. If secondary retinal detachment has occurred, there may be large visual field defects between the central part and the blind spot (Fig. 5.9). Optical coherence tomography is valuable for the diagnosis of congenital optic disc pit (Fig. 5.10).

2. Pathogenesis Congenital optic disc pit is a congenital malformation caused by the incomplete closure of the top of the embryonic fissure. The pit is filled with embryonic neuroectodermal, neural epithelial, pigment epithelial, and glial cells. There are different views about the origin of the subretinal fluid in cases with retinal detachment. Brown et  al. [10] injected 10% fluorescein sodium and ink into the subarachnoid space of a German shepherd dog, and found no traffic between the subarachnoid space and the optic pit. When they injected ink into the vitreous cavity, there were ink particles under the retina. In normal optic discs, the tissue fluid drains through the vitreous-optic pass. When a pit exists, part of the liquid flows from the pit to the submacular area. There is no need to treat cases of optic disc pit without complications. The associated retinal detachment has a self-­healing tendency. If necessary, laser treatment or vitrectomy can be employed.

Fig. 5.9  Optic disc pit with maculopathy in the left eye

Fig. 5.8  Congenital optic disc pit located in the temporal area of the disc

Fig. 5.10  Optical coherence tomography (OCT) of optic disc pit with maculopathy; this in the patient whose images are shown in Figs. 5.6, 5.7, 5.8, and 5.9. There is macular retinoschisis

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5.3

Congenital Coloboma of Optic Disc

Congenital coloboma of the optic disc can be divided into two types: optic disc coloboma only, or optic disc coloboma with choroid and retinal coloboma (Fig. 5.11). Most patients have severe impairment of visual acuity, disuse strabismus, nystagmus, or even blindness. At the entrance of the optic nerve, the scleral canal expands downward or to the side and forms a depression (the coloboma). The pit has an irregular funnel shape. The sizes and depths of the pits vary. Small and shallow pits are similar to enlarged and deepened physiological depressions that are limited to the optic nerve sheath. The majority of optic disc colobomas are four to five times or even greater than the size of a normal optic disc. The coloboma often includes the retina and choroid around the optic disc. The colobomas are round, vertical oval, triangular, or irregular, and they are white or gray. The optic nerve fibers are pushed to upward or beside, and are slightly pink. The diameters of the retinal arteriovenous vessels are normal and their distribution is variable. A coloboma of the optic disc can occur secondary to serous retinal detachment in the macular area or elsewhere [11].

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may be distributed near the upper and lower vascular arches (Figs. 5.12 and 5.13). The presence of medullated retinal nerve fibers is often associated with high refractive errors, especially myopia. Sometimes this abnormality is associated with other congenital fundus abnormalities, such as choroidal coloboma, optic disc hypoplasia, and persistent vitreous artery. Large areas of myelinated nerve fibers can also be manifested as “white pupils”, and this phenomenon may be misdiagnosed as retinal detachment. Clinically, medullated retinal nerve fibers should be differentiated from both ­retinoblastoma and retinal white lesions caused by inflammation and degeneration.

Medullated Retinal Nerve Fibers

Medullated retinal nerve fibers are white and opaque, showing a silk-like myelin luster under an ophthalmoscope [12]. The surfaces and edges of these medullated optic nerve fibers show a feather-like texture, and retinal vessels are obscured in the dense area. The locations and the sizes of medullated retinal nerve fibers are variable. Most of these fibers are distributed superior or inferior to the optic disc. From the optic disc the medullated nerve fibers extend in the direction of the nerve fibers. The entire optic disc and its surroundings are covered by myelin patches. Occasionally, the medullated nerve fibers are far from the optic disc and isolated fibers

Fig. 5.11  Congenital optic disc and choroidal coloboma

Fig. 5.12  Medullated retinal nerve fibers in the left eye. As well as the macular area, the posterior fundus was covered with white opaque myelin patches

Fig. 5.13  Medullated retinal nerve fibers in the right eye. The area around the lower vascular arch is covered with white opaque feather-­ like myelin patches

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Ocular Albinism

Albinism is a congenital deficiency of pigment. Ocular albinism is often part of systemic albinism. It can also exist alone, and even be limited to the fundus, and it is then called albinism fundus [13]. Skin, hair, and eyes lack pigment. The eyebrows, eyelashes, and eyelid skin are white or light yellow. The iris is light gray, the pupil has a red fundus reflex, the fundus is orange, the retinal and choroidal vessels. The red optic disc

is difficult to distinguish from the surrounding orange retina. Under an ophthalmoscope, the macula and macular foveola are not seen. Patients have poor visual acuity, photophobia, and a high incidence of refractive errors that cannot be corrected. There is horizontal or rotational nystagmus owing to macular hypoplasia. The visual field is concentric and narrow, with central scotoma. Color vision, light perception, dark adaptation, and electroretinography may be normal. Ocular albinism can be combined with RRD, but the hole is difficult to find (Figs. 5.14 and 5.15).

Fig. 5.14  Left ocular albinism

Fig. 5.15  Left ocular albinism combined with RD

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5.6

Congenital Choroidal Coloboma

Choroidal coloboma is a defect of the choroid and the retinal pigment epithelium. The gray-white sclera can be seen through the thin retinal nerve epithelial layer, owing to lack of the choroid and the retinal pigment epithelial layer. Colobomas are located superior lateral of the optic disc. The ranges and shapes of colobomas are very variable; a coloboma can be larger than one quadrant, and the top of a coloboma can comprise the entire optic disc or the lower part of the disc. A coloboma can also be located immediately below the lower edge of the optic disc or at a certain distance from the optic disc. The edge of a coloboma is located in the periphery of the fundus (Fig. 5.16). The margin of a coloboma is clear with irregular pigmentation. The coloboma is gray and white and the surface is smooth, with scattered brown spots on it, showing that the brown layer of the sclera remains. In most cases, a coloboma is slightly depressed compared with the surrounding area, with this being caused by scleral thinning. There is still retinal vascular distribution in a coloboma and most of the distribution is normal. Sometimes the vascular distribution is interrupted or surrounded by the edge of the coloboma (Fig. 5.17). Atypical choroidal colobomas are rare, mostly monocular, and often isolated in any area of the fundus; these colobomas show scleral exposure, mild depression, and pigmentation. Choroidal coloboma may be associated with microphthalmus, iris defect, or other congenital ocular abnormalities, leading to severe visual impairment. Although these colobomas do not involve the macula, they can also lead to poor vision, owing to dysplasia [14].

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Sometimes retinal detachment can occur in a choroid coloboma. The hole is often located at the edge of the sclera. Macular coloboma is rare, is mostly monocular, and occasionally binocular. There is severe impairment of visual acuity at an early age. The morphology of the fundus varies. The shape of the coloboma is round or elliptical, and it is located in the macular area or near the macula. The size ranges from 1 to 5 PD. A very small number of macular colobomas can develop into retinal detachment (Fig. 5.18).

Fig. 5.17  Wide-angle retinal photography of congenital choroidal coloboma

Fig. 5.18  Macular coloboma of the left eye Fig. 5.16  Congenital choroidal coloboma combined with optic disc coloboma

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Coats Disease

Coats Disease is also known as external exudative retinopathy. The etiology is not clear. The main feature is idiopathic retinal telangiectasia, a great deal of white retinal exudation, and exudative retinal detachment. It often occurs in young people (76%), is more common in men, and the vast majority of cases show monocular onset (95%) [15]. Clinical features: Lesions in the early stage are located in the periphery of the retina. Patients often do not have symptoms, and visual acuity can decrease after macular involvement. Most pediatric patients come to see a doctor because of the appearance of leukocoria or disuse strabismus. The anterior segment of the eye is normal. Abnormal retinal blood vessels can be seen in the fundus, and these are located in the lower or temporal quadrant and are mostly distributed between the equator and ora Fig. 5.20  Fundus image of Coats Disease, showing peripheral retinal vasserrata (Figs.  5.19 and 5.20). Abnormalities in blood vessels cular dilatation and microaneurysm, combined with annular exudation include telangiectasia, microaneurysms, macroaneurysms, and arteriovenous short circuits (Figs.  5.21, 5.22, 5.23 and 5.24). Severe cases also show angiomatous proliferation and hemorrhage (Figs.  5.25 and 5.26). Large numbers of yellow-white exudative lesions can be seen in the subretina of the posterior fundus and in the abnormal blood vessels (Figs. 5.27 and 5.28). In the vicinity of the exudative lesions cholesterol crystal bodies, in the form of dots, can often be seen (Fig. 5.29). In severe cases, exudative retinal detachment can develop (Figs.  5.30, 5.31, and 5.32). With further progress of the disease, secondary glaucoma and atrophy of the eyeball can occur [16]. FFA showed tortuous expansion of small retinal arteries and veins and significant expansion of capillaries, with a fishnet-like appearance, and microaneurysm formation. Some cases develop capillary occlusion, with a large non-­ perfused area. Arteriovenous short circuits or neovascularization was often seen around the lesion. In the late phase of FFA, there were obvious fluorescence leakages and tissue Fig. 5.21 Fundus image of Coats Disease. The retinal vascular staining (Figs. 5.33, 5.34, 5.35, 5.36, and 5.37). network is abnormal

Fig. 5.19  Fundus image of Coats Disease. Retinal blood vessels showed sausage-like expansion with a large amount of subretinal exudation

Fig. 5.22  Fundus image of Coats Disease with typical retinal vascular dilatation, microaneurysm, hemorrhage, and annular exudation in the right eye

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Fig. 5.23  Fundus image of Coats Disease. There are abnormal blood vessels and extensive exudation in the temporal area of the macula Fig. 5.26  Fundus image of Coats Disease. There is angiomatous proliferation and hemorrhage

Fig. 5.24  Fundus image of Coats Disease, showing extensive abnormal vascularity and arteriovenous shunt Fig. 5.27  Fundus image of Coats Disease with a large amount of yellow-­white subretinal exudation

Fig. 5.25  Fundus image of Coats Disease. There is angiomatous proliferation and a large amount of subretinal exudation and hemorrhage around this proliferation

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Fig. 5.28  Fundus image of Coats Disease with macular exudation

Fig. 5.29  Fundus image of Coats Disease with macular exudation. There are abnormal retinal blood vessels and exudation with cholesterol crystals

Fig. 5.30  Fundus image of Coats Disease of the left eye. There is a large amount of subretinal yellow-white exudation in the posterior fundus and bulbar exudative RD inferiorly

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Fig. 5.31  Fundus image of Coats Disease of left eye. There are extensive abnormal vessels and exudation in the temporal fundus

Fig. 5.32  Fundus image of Coats Disease. There is peripheral retinal telangiectasia in the inferior fundus, with scattered microaneurysms and secondary RD

Fig. 5.33  Fundus image of Coats Disease with peripheral retinal telangiectasia and microaneurysm

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Fig. 5.34  Fundus fluorescein angiography (FFA) image of Coats Disease. There is fishnet-like peripheral retinal telangiectasia in the inferior fundus, with microaneurysm leakage

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Fig. 5.36  FFA image of patient 5–35. The capillaries have expanded in a fishnet-­like fashion and there is a hyperfluorescent aneurysm

Fig. 5.37  FFA image of patient 6–35. The capillaries have expanded in a fishnet-like fashion with fluorescent leakage Fig. 5.35  Fundus image of Coats Disease in a 17-year-old male. There is a strip of yellow-white subretinal exudation above the optic disc. The capillaries have expanded in a fishnet-like fashion. An aneurysm and some scattered microaneurysms can be seen

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 ersistent Hyperplastic Primary P Vitreous

Persistent hyperplastic primary vitreous is caused by a primary vitreous body that did not regress normally during the embryonic period. It is usually found because of leukocoria after birth. The vast majority of cases are monocular. Other than leukocoria, the condition is associated with a small eye, a small cornea, and a shallow anterior chamber. Gray membrane-­like tissue covers the posterior capsule of the lens

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and the central part of the lens is thick. Persistent vitreous artery is also occasionally associated with the condition. Ciliary bodies can be seen around the lens. Once the posterior capsule is broken, the lens cortex becomes swollen and opaque. The swollen cortex blocks the aqueous outflow pathway and causes secondary glaucoma. If the fundus can be seen, then intravitreal fibro-strips, the epi-optic disc membrane, and traction folds can be seen in the peripheral retina [17] (Fig. 5.38).

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Fig. 5.38  Fundus of persistent hyperplastic primary vitreous. (a) Preoperative fundus. (b) Postoperative fundus

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5.9

Congenital Retinal Fold

Congenital retinal fold is a rare congenital retinal dysplasia, that is also called congenital sickle-cell retinal detachment, and it is usually secondary to the FEVR. Presentation shows a bundle of folds in the retina, with parallel vessels. The fold originates from the optic disc, travels through the macula, extends to the peripheral retina, and reaches the ora serrata or even the lens equator. If the fold ends at the macula, it is called incomplete type [18]. The locations of the fold are variable; most lie temporal or slightly inferior temporal to the optic disc. There may be one or multiple folds, which are white or yellowish white. The width

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is about 1 PD, and the width increases when the folds extend to the peripheral retina. Both edges of the fold are steep. The fold may be bilateral or unilateral. Visual acuity is poor from childhood. Even if the structure of the macula is normal, visual acuity is still poor. This suggests that the entire retinal neuroepithelial layer, including the macula, has incomplete tissue differentiation and structural disorder (Figs. 5.39 and 5.40). A small number of congenital retinal folds are associated with conditions including macular degeneration, congenital coloboma of the retina and choroid, small eyeball, persistent pupillary membranes, cataracts, strabismus, and nystagmus. Congenital retinal folds may also be associated with retinal detachment.

Fig. 5.40  Fundus of congenital retinal fold in the right eye Fig. 5.39  Fundus of congenital retinal fold in the left eye

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5.10 R  etinoschisis Associated with Retinal Detachment Retinoschisis is the separation of layers within the retinal neuroepithelium [19]; the condition includes congenital, senile, and tractional retinoschisis of various causes. Congenital retinoschisis is mostly a sex-linked recessive genetic disease [20]. Congenital retinoschisis occurs in the optic nerve fiber layer, whereas senile retinoschisis occurs in the inner plexiform layer. Because of the further degeneration of the inside and outside layers, the layer becomes thinner and a hole forms. If the hole occurs only in the inner layer of the cleft, there will be no retinal detachment. Localized retinal detachment occurs in only 16% of isolated outer retinal holes. Retinal detachment occurs when both the internal and external cleft layers have holes (Figs.  5.41, 5.42, and 5.43). Schisis of the peripheral retina is very common in the lower fundus, especially the inferior temporal fundus. From the equator to the periphery of the retina, the clefted inner retina is large, cystic, and almost transparent, and the retinal vessels crawl on it. The vessels are white or have white sheaths (Figs. 5.44 and 5.45). In the early stage, retinoschisis appears in the macula, and then it appears in radial folds from the macula fovea. Gradually the radial folds fuse with each other and form

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inner layer schisis of the retinal neuroepithelial layer (Figs. 5.46 and 5.47). Retinoschisis should be differentiated from RRD (Figs. 5.48, 5.49, 5.50, and 5.51).

Fig. 5.42  Fundus of congenital retinoschisis. There is an inferior inner retinal break that is similar to ora serrata dialysis

Fig. 5.41  Fundus of congenital retinoschisis in the right eye Fig. 5.43  Fundus of congenital retinoschisis. There is a circular hole in the outer layer of the retina

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Fig. 5.44  Fundus of retinoschisis in a 30-year-old male. There is a boundary of retinal pigment at the inferior temporal retina. The retinal vessels have white sheaths

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Fig. 5.46  Fundus of macular schisis in the right eye

Fig. 5.47  OCT image of macular schisis Fig. 5.45  Fundus of retinoschisis, with a large hole in the inner layer of the retina, with vascular traction

Fig. 5.48  Fundus of retinoschisis with a large hole in the inner retina

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Fig. 5.49  Fundus of retinoschisis with a large hole in the inner retina. There is a circular hole in the outer retina, with pigmentation around it

Fig. 5.50  Fundus of retinoschisis with a circular hole in the inner retina

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Fig. 5.51  Fundus of retinoschisis with a hole in the inner retina, associated with retinal detachment

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References 1. Kindler P. Morning glory syndrome: unusual congenital optic disk anomaly. Am J Ophthalmol. 1970;69(3):376–84. 2. Lee BJ, Traboulsi EI. Update on the morning glory disc anomaly. Ophthalmic Genet. 2008;29:47–52. 3. Golnik KC. Cavitary anomalies of the optic disc: neurologic significance. Curr Neurol Neurosci Rep. 2008;8(5):409–13. 4. Ceynowa DJ, Wickström R, Olsson M, Ek U, Eriksson U, Wiberg MK, Fahnehjelm KT. Morning glory disc anomaly in childhood—a population-based study. Acta Ophthalmol. 2015;93(7):626–34. 5. Bartz-Schmidt KU, Heimann K.  Pathogenesis of retinal detachment associated with morning glory disc. Int Ophthalmol. 1995;19:35–8. 6. Ho CL, Wei LC. Rhegmatogenous retinal detachment in morning glory syndrome pathogenesis and treatment. Int Ophthalmol. 2001;24(1):21–4. 7. Irvine AR, Crawford JB, Sullivan JH.  The pathogenesis of retinal detachment with morning glory disc and optic pit. Retina. 1986;6(3):146–50. 8. Akiyama K, Azuma N, Hida T, Uemura Y. Retinal detachment in morning glory syndrome. Ophthalmic Surg. 1984;15:841–3. 9. Georgalas I, Ladas I, Georgopoulos G, Petrou P. Optic disc pit: a review. Graefes Arch Clin Exp Ophthalmol. 2011;249(8): 1113–22. 10. Brown GC, Shields JA, Goldberg RE.  Congenital pits of the optic nerve head. II.  Clinical studies in humans. Ophthalmology. 1980;87(1):51–65.

133 11. Ari S, Keklíkçí U, Caça I, Unlü K, Alakuş F. Congenital isolate and total optic disc coloboma: case report and review of the literature. Ann Ophthalmol (Skokie). 2007;39(1):75–7. 12. Sakai T, Sano K, Tsuzuki K, Ueno M, Kawamura Y.  Temporal raphe of the retinal nerve fiber layer revealed by medullated fibers. Jpn J Ophthalmol. 1987;31(4):655–8. 13. McCafferty BK, Wilk MA, McAllister JT, Stepien KE, Dubis AM, Brilliant MH, Anderson JL, Carroll J, Summers CG. Clinical insights into foveal morphology in albinism. J Pediatr Ophthalmol Strabismus. 2015;52(3):167–72. 14. Vuković D, Pajić SP, Paović P. Retinal detachment in the eye with the choroidal coloboma. Srp Arh Celok Lek. 2014;142(11–12):717–20. 15. Nuzzi R, Lavia C, Spinetta R.  Paediatric retinal detachment: a review. Int J Ophthalmol. 2017;10(10):1592–603. 16. Yang Q, Wei W, Shi X, Yang L. Successful use of intravitreal ranibizumab injection and combined treatment in the management of Coats’ disease. Acta Ophthalmol. 2016;94(4):401–6. 17. Zhu X, Du Y, He W, Sun T, Zhang Y, Chang R, Zhang K, Lu Y. Clinical features of congenital and developmental cataract in east China: a five-year retrospective review. Sci Rep. 2017;7(1):4254. 18. Nishina S, Suzuki Y, Yokoi T, Kobayashi Y, Noda E, Azuma N. Clinical features of congenital retinal folds. Am J Ophthalmol. 2012;153(1):81–7. 19. Droms RJ, Liang MC, Duker JS.  Retinoschisis and outer retinal hole formation in a patient with papillorenal syndrome. Ophthalmic Surg Lasers Imaging Retina. 2015;46(4):477–80. 20. Fahim AT, Ali N, Blachley T, Michaelides M.  Peripheral fun dus findings in X-linked retinoschisis. Br J Ophthalmol. 2017;101(11):1555–9.

6

Tractional Retinal Detachment Lei Shao, Nan Zhou, and Wenbin Wei

Abstract

Tractional retinal detachment can be caused by vitreous hemorrhage, proliferative diabetic retinopathy (PDR), retinal vein occlusion (RVO), retinal vasculitis, familial exudative vitreoretinopathy (FEVR), and retinopathy of prematurity (ROP). For most of them, intravitreal anti-­ vascular endothelial growth factor (anti-VEGF) drugs tend to block the growth of neovascularization and would reach to an optimistic gain of visual acuity. Vitrectomy can remove the vitreous hemorrhage and proliferative membrane.

L. Shao, M.D., Ph.D. · N. Zhou, M.D., Ph.D. W. Wei, M.D., Ph.D. (*) Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China

6.1

Vitreous Hemorrhage

Vitreous hemorrhage is the fibrovascular proliferation in the vitreous potential spaces or the leakage of blood into the vitreous body, which results from vascular injury of the retina or uvea. The common cause involves: 1. Retinal vascular disease, such as diabetic retinopathy, retinal vein occlusion, and retinal vasculitis 2. Ocular penetrating injury, intraocular foreign body, ocular contusion, and various intraocular surgeries 3. Retinal vascular hemorrhage caused by acute retinal tears or posterior vitreous detachment (Fig. 6.1a–c) 4. Some systemic diseases, as Terson syndrome, which is the occurrence of a vitreous hemorrhage in association with subarachnoid hemorrhage (Fig. 6.2a–d) 5. Exudative Age-related macular degeneration, uveitis, congenital retinal fold, retinal angioma, choroidal melanoma, etc. Slight vitreous hemorrhage often presents with painless diminution of vision or new multiple floaters [1]. Ophthalmoscopic examination reveals dusty or flocculent turbidness. Massive hemorrhage may lead to dense vitreous disturbance and tends to significantly decrease vision with a decreased or no red reflex in ophthalmoscopic examination. Slit-lamp examination shows blood or dark-red coagulation clot within the anterohyaloid spaces, while obsolete hemorrhage reveals as brown dusty turbidity. Hemorrhage can destroy the gel structure of the vitreous body and then results in tractional retinal detachment. Slight hemorrhage may be absorbed. Massive hemorrhage could undergo organization in several weeks and cause tractional retinal detachment. Exceptions also include cases of neovascular glaucoma (NVG), acute ocular hypertension secondary to ghost cell glaucoma (Fig. 6.3a, b).

© Springer Nature Singapore Pte Ltd. and Beijing Science and Technology Press 2018 W. Wei (ed.), Atlas of Retinal Detachment, https://doi.org/10.1007/978-981-10-8231-3_6

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Fig. 6.1 (a–c) Retinal vascular hemorrhage caused by acute retinal tears or posterior vitreous detachment

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Fig. 6.2 (a–d) Terson syndrome, which is the occurrence of a vitreous hemorrhage in association with subarachnoid hemorrhage

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Fig. 6.3 (a, b) Massive hemorrhage could undergo organization in several weeks and cause tractional retinal detachment

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In general, morphological characteristics of vitreous hemorrhage, which are related to retinal vasculitis, proliferative diabetic retinopathy, and retinal vein occlusion, include the following:

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1. Complete posterior vitreous detachment. The type has better prognosis, usually without neovascularization (Fig. 6.4). 2. “V”- and “L”-shaped posterior vitreous detachment. The type has uniform morphology, but with various adhesion. The vitreous cortex in proliferative diabetic retinopathy (PDR) usually attaches with optic disk and vascular arch. For retinal vein occlusion, the adhesion is often between the optic nerve head and the sites of vas-

cular occlusion, while the attachment of retinal vasculitis is mostly in the vascular lesion area. Usually, the adhesive points above are a critical area of neovascularization (Fig. 6.5). 3. Vitreoschisis. The vitreous cortex frequently splits into double layers. The neovascular membrane attached to the posterior wall, and the unconsolidated blood fully deposited in the splitting cavity (Fig. 6.6). 4. Incomplete posterior vitreous detachment. The type is most likely to lead to tractional retinal detachment. This type of retinal detachment usually has no tears and shapes as a tent with different attached positions and extent. Certainly, it also can cause rhegmatogenous retinal detachment.

Fig. 6.4  Complete posterior vitreous detachment. The type has better prognosis, usually without neovascularization

Fig. 6.6 Vitreoschisis

Fig. 6.5  “V”- and “L”-shaped posterior vitreous detachment

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 roliferative Diabetic Retinopathy P (PDR)

or presence of retinal neovascularization [2] (Fig. 6.7a–w). In more than 42% of patients with type 1 diabetes and nearly 30% of those with type 2 diabetes, potentially vision-­ threatening retinal lesions develop over time, but early retinal changes are not noticed by the patients [3, 4].

Diabetic retinopathy (DR) is one of the severe complications of diabetes, which involved two different forms: non-­proliferative and proliferative, named for the absence a

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Fig. 6.7 (a–w) Diabetic retinopathy (DR), which involved two different forms: non-proliferative and proliferative, named for the absence or presence of retinal neovascularization

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Fig. 6.7 (continued)

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Fig. 6.7 (continued)

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Table 6.1  International Clinical Diabetic Retinopathy Disease Severity Scale [5] Proposed disease severity level No apparent retinopathy Mild non-proliferative diabetic retinopathy (NPDR) Moderate non-proliferative diabetic retinopathy(NPDR) Severe non-proliferative diabetic retinopathy (NPDR)

Proliferative diabetic retinopathy (PDR)

Findings observable upon dilated ophthalmoscopy No diabetic fundus changes Microaneurysms only More than just microaneurysms but less than severe NPDR Any of the following:  • More than 20 intraretinal hemorrhages in each of 4 quadrants  • Define venous beading in two or more quadrants Prominent IRMA in one or more quadrants One or both of the following:  • Neovascularization  • Vitreous/preretinal hemorrhage

Clinically, the international classification system of DR was widely used (AAO) classification in 2003 [5]. According to this system, NPDR is the earliest stage of DR, which includes microaneurysms, intraretinal hemorrhages, and cotton wool spots. NPDR is further classified as mild, moderate, and severe depending on the degree of severity (Table  6.1). As DR progresses, retinal ischemia occurred, which results in retinal venous abnormalities such as loops, venous beadings, intraretinal microvascular abnormalities (IRMA), increased intraretinal hemorrhage, and exudation leading to severe leakage. Patients with severe NPDR should consider scatter laser photocoagulation as treatment for their case. PDR is characterized by neovascularization of the optic disk, the iris, or elsewhere in the retina, which may lead to severe complications such as vitreous hemorrhage, tractional

retinal detachment, or NVG. If the macula edema is present, the central vision would be lost. For NPDR patients, retinal detachment commonly results in the accumulation of fluid between the retinal pigment epithelium and neural retina which is caused by a break in the retina (rhegmatogenous retinal detachment). When the lesions develop to the proliferative stage, tractional retinal detachment may occur that results from condensation and contraction of the vitreous hemorrhage and fibrosis; fibroblast proliferation and membrane formation make the continuous traction on the retina. Then, the retinal breaks lead to tractional retinal detachment [3, 4]. With the development of molecular biotechnology, recent studies reveal that the levels of IL-6, TGFβ-1, and VEGF correlate with the severity of PDR [6]. So in the future, the severity of diabetic retinopathy may be determined by serological testing.

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Retinal Vein Occlusion

Retinal vein occlusion (RVO) is one of the most common causes of vision loss. Specifically, it is the second most common cause of blindness from retinal vascular disease after diabetic retinopathy. The prevalence varies from 0.7 to 2% in persons older than 40 years of age. The 10-year incidence of RVO in a population-based cohort was 1.6% [7]. RVO is more frequent in older age, 90% patients with RVO are older than 50 years, and the age of onset ranges from 9 months to 90 years. Most cases are unilateral, with approximately 6–14% of cases bilateral. Sex ratio for the disease is 6:4. Classification: RVO is essentially a blockage of the venous circulation that drains the retina. The occlusion of retinal veins may cause congestion, stasis, hemorrhage, edema, and exudation. According to the locations of RVO, two types were proposed: central retinal vein occlusion (CRVO) and branch retinal vein occlusion (BRVO). Based on the severity of blockage (the manifestations of FFA), RVO was further divided into ischemic RVO and nonischemic RVO. The ischemic RVO is characterized by marked decreased vision (Fig. 6.8a–f), as ischemic CRVO predisposes to extensive capillary non-perfusion and cell death. Even if the perfusion was recovered, the visual function could not be improved.

The nonischemic RVO may maintain relative better blood flow to the retina through collaterals. The majority of people diagnosed with CRVO is nonischemic (54–78%), and capillary non-perfusion zone was rare in the early time (Fig. 6.9a–i). Its pathogenesis is multifactorial and involves vessel damage, stasis, and hypercoagulability, which could occur in conditions such as atherosclerosis, stroke, or inflammation. The tractional retinal detachment usually occurred in ischemic cases. Retinal hypoxia causes the elevation of IL-6, TGFβ-1, and VEGF and then neovascularization. Proliferative membrane was formed and tracts the retina, which may result in retinal tear or detachment [8]. The serous retinal detachment may be detected in some cases, which Müller cell cone traction plays an important role in the formation of pointed or dome-shaped ones. The Müller cell cones bind the photoreceptor cells to the foveola, extend outward, and form the internal limiting membrane at the foveal area. When RVO happens, leakage from the affected retinal capillaries travels preferentially through the outer plexiform layer and causes retinal edema mainly within Henle’s layer. Occasionally, serous retinal detachment may regress spontaneously, but focal laser photocoagulation often results in regression immediately.

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Fig. 6.8 (a–f) The ischemic CRVO predisposes to extensive capillary non-perfusion and cell death

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CRVO usually occurs in scleral lamina cribrosa, which associated with hypertension, hyperlipidemia, arteriosclerosis, and inflammation. Symptom: a rule typically presents with sudden painless monocular reduction in central vision. In some cases, this loss of vision is subtle and depends primarily on the severity of macular edema and retinal hemorrhage. Fundus manifestations: papilledema, thin arteries, and dilated tortuous retinal veins, intraretinal hemorrhages, and edema through all four quadrants of the fundus, especially at the posterior pole. Superficial hemorrhage was located in the nerve fiber layer and presents as “flare” or “line.” Deep hemorrhage exists on inner nuclear layer and inner plexiform layer and shows as “dot.” Cotton wool spots and exudations would appear after hemorrhage in some patients. Macular edema is often obvious. The changes above are related to the severity of vein occlusion (Figs.  6.8a, c, e, 6.9b–d and 6.10). Fluorescein angiography: a nonischemic type, with delayed filling and dilation of retinal vessels, blocked fluorescence by hemorrhage, and petal-like hyperfluorescence of edema in macula, which is cystoid macular edema (CME) (Fig.  6.11), while an ischemic type (Fig.  6.8b, d, e), with large areas of capillary non-perfusion or evidence of retinal neovascularization, compensatory enlargement of surrounding capillaries, arteriovenous shunt, and collateral circulation in some case.

Natural pathogenesis: retinal edema and hemorrhage disappeared gradually; the vein was wrapped with white scabbard or even changed into white line. Macular edema lasts for a long time and may develop to cystoid edema or macular hole. Pigmentations were remained after edema absorption. Although the prognosis of nonischemic type is better than the ischemic one, final acuity of 50% patients with nonischemic CRVO are under 0.1; and 10–20% cases will develop to ischemic type. For nonischemic CRVO, recovering circulation may regain the visual function to different degrees, but visual acuity of only 15% cases could improve more than two lines. For ischemic CRVO, final acuity of 93% patients are under 0.1; 60% cases occur neovascularization of anterior segment (iris or anterior chamber angle); 30% patients develop to retinal neovascularization; and also company with tractional or exudative retinal detachment, neovascular glaucoma, which make the prognosis even worse (Figs. 6.12, 6.13, 6.14 and 6.15). Treatment: there is no specific treatment for RVO.  Treatment of the RVO itself may be directed at the etiology or the sequelae of the occlusion. It is important to manage associated diseases, such as diabetes, systemic hypertension, hyperlipidemia, and blood hyperviscosity. Taking aspirin or other blood thinners has no obvious effect but increases the risk of hemorrhagic complications. Common treatment is taking laser treatment to prevent the macula edema or neovascularization; and treatment modalities for the complications of RVO include laser photocoagulation and intravitreal injection of anti-vascular endothelial growth factor (anti-VEGF) drugs into the vitreous cavity.

Fig. 6.10 The retinal edema and hemorrhage were absorbed completely

Fig. 6.11  Fluorescein angiography: a nonischemic type, with delayed filling and dilation of retinal vessels; blocked fluorescence by hemorrhage; petal-like hyperfluorescence of edema in macula, which is cystoid macular edema (CME)

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Fig. 6.12  The long-standing CRVO

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Fig. 6.14  The CRVO with exudative retinal detachment and hemorrhage

Fig. 6.15  RVO company with tractional retinal detachment, which make the prognosis even worse

Fig. 6.13  The FFA of Fig. 6.12

These drugs can block the growth of neovascularization that may cause the NVG.  Moreover, there are some other ­therapies to be reported (Figs. 6.9c, d and 6.10), including arteriovenous sheathotomy, intravitreal injection of antiinflammatory, radial optic neurotomy, and chorioretinal anastomosis. These treatments are still being studied.

6.3.2 Branch Retinal Vein Occlusion BRVO usually obstruct in one or more branch vein, which pathogenic factor is similar to CRVO [9]. BRVO usually occurs in arteriovenous crossings where the artery and the vein of the retina share a common adventitial sheath. It has

been hypothesized that increased arterial stiffness may be a mechanical factor in the pathogenesis of BRVO. So if someone suffered from hypertension or arteriolosclerosis, external compression of the vein leads to vascular turbulence and stasis, and the turbulent flow in combination with the preexisting vessel wall damage from the different conditions ­creates a local environment favorable to intravascular thrombus formation, which results in BRVO. Increased intraluminal pressure with reduced blood flow in the macular capillaries and the production of VEGF from the ischemic retina lead to dysfunction of the endothelial blood-retinal barrier and result in severe macular edema; macular edema results in visual loss. In addition, it is reported that serious macular edema associated with BRVO is often accompanied by serous retinal detachment (SRD) beneath the macular fovea and leakages from macular capillaries may migrate into the subretinal space.

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Symptom: related to the location of vein occlusion and sudden loss of visual field and visual acuity, but milder than CRVO. Fundus manifestations and fluorescein angiography: similar to CRVO, but the lesions were only limited to the area of

the retina supplied by the occluded vessel, which is triangular in shape, and the tip points to the occluded position (Fig. 6.16a–f). Natural pathogenesis: prognosis of BRVO is relatively good. Final acuity of 53–60% patients are better than 0.5.

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Fig. 6.16 (a–f) Fundus manifestations and fluorescein angiography: similar to CRVO, but the lesions were only limited to the area of retina supplied by the occluded vessel, which is triangular in shape and the tip point to the occluded position

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Retinal edema and hemorrhage usually disappeared in 3–6 months after the vein occlusion; but in some cases, it would last for a long time (Fig. 6.17a, b). The vein was white scabbard or even presents as white line (Fig. 6.18a). Neovascular glaucoma was rare, with 1% of prevalence rate. Disk neovascularization seems common, and the rate was nearly 10% (Fig. 6.18b), while the incidence rate of retinal neovascularization was 20%, which may cause vitreous hemorrhage and retinal traction and even rhegmatogenous retinal detachment. Furthermore, macular pigmentation, edema, and CME would persist; macular hole may occur occasionally (Fig.  6.19a–h). Although it may be an uncommon feature associated with SRD, some cases could not detect any apparent breaks of the retina. Small breaks on the external surface of the retina may be a possible mechanism for causing focal serous retinal detachment beneath the macular fovea. Simple diffusion of intraretinal fluid into the subretinal space may represent a more common cause of foveal serous retinal detachment associated with BRVO.

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Medical treatment of BRVO is not effective. Laser photocoagulation was long considered the gold standard care for the BRVO. Intravitreal injection of triamcinolone has been used to treat macular edema because of its potent antipermeability and anti-inflammatory properties. Over the past decades, the use of anti-VEGF agents has revolutionized the treatment of retinal vascular disease, including age-related macular degeneration (AMD), PDR, and RVO.  Intravitreal anti-vascular endothelial growth factor (anti-VEGF) drugs tend to block the growth of neovascularization and reduce macular edema. Anti-VEGF agents would reach to an optimistic gain of visual acuity but require a high injection frequency. Triamcinolone might be an alternative, but comparison is impaired, for the effect is temporary and the ocular risk profile seems to be favorable for anti-VEGF agents in comparison to steroids [10]. Besides, focal laser treatment is effective both in developing the visual acuity and preventing neovascularization.

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Fig. 6.18 (a, b) The vein was white scabbard or even present as white, and the retinal neovascularization cause vitreous hemorrhage and ­retinal tractional retinal detachment

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Fig. 6.19 (a–h) Macular pigmentation, edema, CME and tactional detachment would be persist; macular hole may occur occasionally

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6.4

Retinal Vasculitis

Retinal vasculitis can be an isolated condition or a complication of local or systemic inflammatory disorders characterized by inflammation of the retinal vessels [11, 12]. It is a sight-threatening condition associated with various pathogenesis, but the vast majority of patients with retinal vasculitis do not have a systemic vasculitis. Eales’ disease is a type of idiopathic inflammatory vasculopathy without etiology, also known as retinal periphlebitis, and is an ocular disease characterized by inflammation and possible blockage of retinal blood vessels, abnormal growth of neovascularization, and recurrent retinal and vitreous hemorrhages [13]. Such disorders mostly strike the binoculus of healthy young men. Although believed to affect ­primarily the retinal veins, others have reported the same prevalence of both venules and arterioles. Eales’ disease is an idiopathic obliterative vasculopathy that primarily affects the peripheral retina of young adult men. The predominant age group of onset of symptoms is 20–30 years. Retinal changes include periphlebitis, vascular sheathing, peripheral non-perfusion, and retinal neovascularization. Visual loss is characteristically caused by recurrent vitreous hemorrhage and its sequelae. The clinical manifestation of Eales’ disease is categorized by three basic pathological changes: inflammation, ischemia, and neovascularization and its sequelae. Initially, it presents as active retinal periphlebitis with perivascular exudates around the retinal veins accompanied by superficial

retinal hemorrhages and small-sized retinal infarctions. The retinal periphlebitis may lead to non-perfusion of a substantial portion of the retina that could result in proliferative vascular retinopathy. It has been proved that the VEGF expression in the retinal neovascular membranes may be due to retinal ischemia and chronic mild inflammation in the inflammatory stage and lead to tractional retinal detachment finally in the proliferative stage. Although many patients complain of symptoms in only one eye, a detailed fundus examination of the fellow eye will often show the early changes such as periphlebitis, vascular sheathing, or peripheral non-perfusion. Often one to be detected using fluorescein angiography, about 50–90% of patients develop bilateral involvement eventually. Symptom: decreased vision or floaters, specks, cobwebs, and blurring. Fundus manifestations: the three hallmark signs of Eales’ disease are retinal phlebitis, peripheral non-perfusion, and retinal neovascularization. Retinal lesion often from periphery to posterior pole; small vessels of peripheral retina were tortuous and dilate; superficial retinal hemorrhages; vascular sheathing ranges from thin white lines limiting the blood column on both sides to segmental heavy exudative sheathing (Fig.  6.20a–g). With the development of the disease, large branch of vessels would be involved and even to the BRVO. Neovascularization occurs in up to 80% of patients with Eales’ disease, which causes repeated episodes of vitreous hemorrhage, organization, and tractional or rhegmatogenous retinal detachment (Fig. 6.21a–c).

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Fig. 6.20 (a–g) Fundus manifestations: the three hallmark signs of the Eales’ disease are retinal phlebitis, peripheral non-perfusion, and retinal neovascularization. Retinal lesion often from periphery to posterior pole; small vessels of peripheral retina were tortuous and dilate;

superficial retinal hemorrhages; vascular sheathing ranges from thin white lines limiting the blood column on both sides to segmental heavy exudative sheathing

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Fig. 6.21 (a–c) Neovascularization occurs in patients with Eales’ disease, which causes repeated episodes of vitreous hemorrhage, organization, and tractional or rhegmatogenous retinal detachment

Fluorescein angiography: the vessels of lesion area were irregularly thin, dilated, and tortuous; areas of vascular sheathing often leak dye or stained with fluorescein. Local capillaries were dilated; microaneurysms and patchy capillary non-perfusion zone in the area. Neovascularization was obvious in the late stage and cystoid macular edema in some case (Fig. 6.22a–c). Natural pathogenesis: prognosis of visual acuity, which depends on the degree of macular lesion and vitreous hemor-

rhage, or whether complications happen, is relatively good. The main reason of visual acuity decreasing is vitreous hemorrhage. Some cases may go blind for neovascularization-­ associated complications, such as proliferative vitreoretinopathy, secondary retinal detachment, and secondary glaucoma. Laser therapy is the main method to control neovascularization of retinal vasculitis. Vitrectomy can remove the vitreous hemorrhage and proliferative membrane (Fig. 6.23a, b).

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Fig. 6.22 (a–c) Fluorescein angiography: the vessels of lesion area were irregularly thin, dilated, and tortuous; areas of vascular sheathing often leak dye or stained with fluorescein. Local capillaries were

dilated; microaneurysms and patchy capillary non-perfusion zone in the area. Neovascularization was obvious in the late stage; cystoid macular edema in some case

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Fig. 6.23 (a, b) The vitreous hemorrhage and proliferative membrane. Figure b is after surgery

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Familial Exudative Vitreoretinopathy

Familial exudative vitreoretinopathy (FEVR) is a hereditary fundus disease associated with visual loss particularly in the pediatric group, in which the main pattern is autosomal dominant inheritance with up to 100% of penetrance, and has been reported to have X-linked autosomal recessive inheritances. Mutations in the Norrin Disease Protein (NDP), a

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Fig. 6.24 (a–g) FEVR, the branches of vessels increase and elongate, which present as a gray “V”-shaped area at temporal retina pointing fovea; in serious cases, the temporal vessels were abnormally dilated with exudation, edema, and retinoschisis

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Severe cases with FEVR present at tractional and exudative retinal detachment in the earlier age, but in less severe ones, it presents later with rhegmatogenous retinal detachment due to globe enlargement frequently from myopia and vitreous liquefaction, which both occur later in life. The disease does not necessarily follow the stages sequentially, and the fundus does not always present with symmetrical changes. A few features markedly suggested FEVR, which include the avascular peripheral retina and a knife-like radial retinal fold extending from the optic nerve to even the ciliary body. Besides, retinoschisis is not rare. Moreover, tractional retinal detachment and rhegmatogenous retinal detachment could also complicate the clinical presentation. FEVR may cause exudative or tractional retinal detachment. While it also can result in rhegmatogenous retinal detachment, for the temporally avascular area, it is easy to form retinal tears. It is important to consider whether there is abnormal exudation or retinoschisis at the temporal area for retinal detachment of juvenile. Besides, vessels of the lateral eye must be examined; FFA can ensure the patterns of retinal detachments in necessity. Because the disease is similar to the retinopathy of prematurity, history of premature delivery and oxygen inhalation seems pivotal. Positive findings of other family members are also indispensable.

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6.6

Retinopathy of Prematurity

Retinopathy of prematurity (ROP) is a proliferative retinopathy that affects preterm infants, which belong to retinal vascular disease, and characterized by neovascularization. Nearly 10% of births occur preterm (before gestational age 37 full weeks) all over the world, and the incidence of severe retinopathy ranges from 16 to 35%. Comparisons of the prevalence rate of ROP from population-based studies are difficult because of substantial variability in study designs, gestational ages of included infants, survival rates, and treatments used. [15, 16] The fundus manifestations are similar to diabetic retinopathy or sickle cell retinopathy, which also presents as vasoproliferative retinopathy. ROP usually affects the babies before 32 weeks of gestation with a birth weight less than 1600g and with the history of excessive oxygen inhalation. ROP can be viewed as an arrest of normal retinal neuronal and vascular development in the premature infants, with ultimately pathological compensatory mechanisms that cause aberrant vascularization of the retina. For pathogenesis, suppression of growth factors due to hyperoxia and loss of the maternal–fetal interaction result in an arrest of retinal vascularization. Then, the increasingly metabolically active, yet poorly vascularized, retina becomes hypoxic, stimulating growth factor-induced vasoproliferation, which leads to retinal detachment.

6.6.1 Clinical Manifestations 1. Acute stage: firstly struck peripheral retina, especially the temporal area, which is characterized by termination of peripheral vessels and formation of demarcation line that parallels to the ora serrata. Then, the demarcation line was thickened and widened to form an elevated ridge. The vessels terminated at the ridge were dilated and tortuous, which lack capillaries and result in arteriovenous shunt. Neovascularization was on the anterior and posterior sides of the ridge. 2. Regression stage: most infants enter into regression stage naturally. The color of the retina of demarcation line is from gray to pink and peripheral retina from opacity to transparent. 3. Scar stage: the lesion in 20–25% patients would develop to neovascular hemorrhages and exudations, which cause proliferative vitreoretinopathy and tractional retinal detachment. The mild case often involved temporal retina, while the serious case may lead to total retinal detachment, whose vitreous body and post-lens were full of organized tissue, presenting as leukocoria.

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International classification: according to the system used for describing the findings of active ROP in 1984, which used a number of parameters to describe the disease, the ROP was classified by location (zones 1, 2, and 3), extent (clock hours 1–12), severity (stages 1–5), and the presence or absence of “plus disease” (Fig. 6.25a–d). Each aspect of the classification has a technical definition. This classification was used for the major clinical trials and was revised in 2005. 1. Zones of theZ retina in ROP

Zone II Zone III

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The retina is divided into three zones: Zone 1 is the posterior zone of the retina, defined as the circle with a radius extending from the optic nerve to double the distance to the macula. Zone 2 is an annulus with the inner border defined by zone 1 and the outer border defined by the radius defined as the distance from the optic nerve to the nasal ora serrata. Zone 3 is the residual temporal crescent of the retina. 2. The extent of the disease is described in segments as if the top of the eye were 12 on the face of a clock. 3. The stages describe the ophthalmoscopic findings at the junction between the vascularized and avascular retina: Stage 1: a thin demarcation line Stage 2: a ridge Stage 3: extraretinal fibrovascular proliferation Stage 4: part retinal detachment    A. retinal detachment not involving the fovea    B. retinal detachment involving the fovea Stage 5: total retinal detachment Plus disease: presents as a major complicating factor at any stage. It is characterized by a significant level of vascular dilation and increased arterial tortuosity observed at the posterior retinal vessels. Aggressive posterior ROP: refers to an uncommon, rapidly progressive form of ROP previously referred to as “rush

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Fig. 6.25 (a–d) International classification: according to the system used for describing the findings of active ROP in 1984, which used a number of parameters to describe the disease, the ROP was classified by

location (zones 1, 2, and 3), extent (clock hours 1–12), severity (stage 1–5), and the presence or absence of “Plus Disease”

disease.” It is characterized by a posterior location, severe plus disease, and flat intraretinal neovascularization. It can progress very fast to stage 5 ROP and blindness, if not intervened early. The flat neovascularization can be quite subtle and can easily confuse less-experienced examiners. Stages 1 and 2 are mild and would regress spontaneously. In stage 3, gradual extraretinal neovascularization would result to total retinal detachment (stage 5) finally, which results in blindness. The presence of increased dilation and tortuosity of posterior vessels, which showed as plus disease, is an ominous sign of progressive disease.

References 1. Sharma P, Sridhar J, Mehta S.  Flashes and floaters. Prim Care. 2015;42(3):425–35. 2. Nentwich MM, Ulbig MW. Diabetic retinopathy—ocular complications of diabetes mellitus. World J Diabetes. 2015;6(3): 489–99. 3. Kollias AN, Ulbig MW. Diabetic retinopathy: early diagnosis and effective treatment. Dtsch Arztebl Int. 2010;107(5):75–83. 4. Morello CM. Etiology and natural history of diabetic retinopathy: an overview. Am J Health Syst Pharm. 2007;64(17 Suppl 12):S3–7. 5. [AAO] American Academy of Ophthalmology. Diabetic retinopathy preferred practice pattern. 2003. Accessed 24 Nov 2006.

6  Tractional Retinal Detachment 6. Rusnak S, Vrzalova J, Sobotova M, Hecova L, Ricarova R, Topolcan O. The measurement of intraocular biomarkers in various stages of proliferative diabetic retinopathy using multiplex xMAP technology. J Ophthalmol. 2015;2015:424783. 7. Zhou JQ, Xu L, Wang S, et al. The 10-year incidence and risk factors of retinal vein occlusion: the Beijing eye study. Ophthalmology. 2013;120(4):803–8. 8. Chatziralli IP, Jaulim A, Peponis VG, Mitropoulos PG, Moschos MM. Branch retinal vein occlusion: treatment modalities: an update of the literature. Semin Ophthalmol. 2014;29(2):85–107. 9. Jaulim A, Ahmed B, Khanam T, Chatziralli IP. Branch retinal vein occlusion: epidemiology, pathogenesis, risk factors, clinical features, diagnosis, and complications. An update of the literature. Retina. 2013;33(5):901–10. 10. Pielen A, Feltgen N, Isserstedt C, Callizo J, Junker B, Schmucker C. Efficacy and safety of intravitreal therapy in macular edema due to branch and central retinal vein occlusion: a systematic review. PLoS One. 2013;8(10):e78538. 11. Sy A, Khalidi N, Dehghan N, Barra L, Carette S, Cuthbertson D, Hoffman GS, Koening CL, Langford CA, McAlear C, Moreland L, Monach PA, Seo P, Specks U, Sreih A, Ytterberg SR, Van Assche G, Merkel PA, Pagnoux C, Vasculitis Clinical Research Consortium (VCRC) and the Canadian Vasculitis Network (CanVasc). Vasculitis  in

163 patients with inflammatory bowel diseases: a study of 32 patients and systematic review of the literature. Semin Arthritis Rheum. 2016;45(4):475–82. Author manuscript; available in PMC 2016 Aug 12. Published in final edited form as: Semin Arthritis Rheum. 12. Sanders E, Graham M.  Retinal vasculitis. Postgrad Med J. 1988;64(753):488–96. 13. Biswas J, Ravi RK, Naryanasamy A, Kulandai LT, Madhavan HN.  Eales’ disease—current concepts in diagnosis and management. J Ophthalmic Inflamm Infect. 2013;3:11. https://doi. org/10.1186/1869-5760-3-11. Published online 2013 Jan. 14. Fei P, Zhu X, Jiang Z, Ma S, Li J, Zhang Q, Zhou Y, Xu Y, Tai Z, Lin Z, Huang L, Yang Z, Zhao P, Zhu X. Identification and functional analysis of novel FZD4 mutations in Han Chinese with familial exudative vitreoretinopathy. Sci Rep. 2015;5:16120. 15. Ochiai M, Matsushita Y, Inoue H, Kusuda T, Kang D, Ichihara K, Nakashima N, Ihara K, Ohga S, Hara T, Kyushu University High-­ Risk Neonatal Clinical Research Network, Japan. Blood reference intervals for preterm low-birth-weight infants: a multicenter cohort study in Japan. PLoS One. 2016;11(8):e0161439. 16. Gunay M, Celik G, Tuten A, Karatekin G, Bardak H, Ovali F.  Characteristics of severe  retinopathy of prematurity  in infants with birth weight above 1500 grams at a Referral Center in Turkey. PLoS One. 2016;11(8):e0161692.

7

Traumatic Retinal Detachment Jinqiong Zhou, Nan Zhou, and Wenbin Wei

Abstract

Traumatic retinal detachment is caused by blunt contusion, and most of them starts from ora serrata dialysis. Intraocular foreign body usually associates with openglobe injury, for eyes with intraocular foreign body, the nature of the foreign body determines the clinical behavior. Tractional retinal detachment caused by trauma is one of the main causes of blindness. Endophthalmitis is a rare but serious complication, but Retinal detachment associates with endophthalmitis is very common.

7.1

Ocular Blunt Contusion

Blunt contusion is the most common ocular trauma in clinic, caused by several kinds of blunt objects, such as fist, injury during sports, explosion, hits or falls, etc. Young males are found to run a higher risk of ocular contusion [1]. Ocular blunt contusion usually associates with various complications in multiple parts of the eye, such as cornea rupture, angle recession, lens opacity or dislocation, vitreous hemorrhage, choroidal rupture, intraocular foreign body, Berlin’s edema, peripheral retinal tears, retinal hemorrhage, choroidal rupture, subretinal bleeding, and macular holes. Most traumatic retinal detachment is caused by blunt contusion, and most of them start from ora serrata dialysis [1, 2].

J. Zhou, M.D., Ph.D. · N. Zhou, M.D., Ph.D. · W. Wei, M.D., Ph.D. (*) Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China

© Springer Nature Singapore Pte Ltd. and Beijing Science and Technology Press 2018 W. Wei (ed.), Atlas of Retinal Detachment, https://doi.org/10.1007/978-981-10-8231-3_7

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7.1.1 Traumatic Ora Serrata Dialysis

7.1.2 Dissection of the Anterior Vitreous Base

Ora serrata dialysis can occur shortly after injury or after a few years. However, half of those patients cannot tell any traumatic history. Ora serrata dialysis mostly situated at the lower temporal quadrant or superior nasal associates with the direction of external force on the eye. Patients may be asymptomatic after injury because inferior ora serrate dialysis leads to a slow accumulation of subretinal fluid. Hence, peripheral retinal examination after ocular blunt contusion is very important. Some patients don’t come to the clinic until the macular detaches. Clinical examination shows the demarcation line of retinal detachment and retinal cysts (Fig. 7.1).

When blunt force comes from the front, the anterior vitreous cortex can separate with the ciliary body from the ora serrata [3]. The tear will be parallel to the ora serrata, with brownred base. The anterior edge can be low and flat, but the rear edge can tilt up or roll, forming a white floating vitreous strip.

7.1.3 Vitreous Base Avulsion Sometimes the anterior and posterior edges can dissect from the vitreous base at the same time, called vitreous base avulsion. Vitreous base avulsion mostly suggests strength external force on the eye, resulting in a vitreous band suspending in the vitreous [4] (Fig. 7.2a, b).

7.1.4 Irregular Retinal Tear

Fig. 7.1  The retinal detachment and retinal cysts

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Early in 1966, Cox and associates [5] divided retinal tears by ocular contusion into three groups: (1) retinal tears at the border of vitreous, (2) retinal tears in areas with no apparent vitreoretinal attachment, and (3) retinal tears that developed at the site of abnormal vitreoretinal attachment. Groups 1 and 3 may be the result of traction by the vitreous, due to deformation of the globe in the process of injury [6]. And Group 2 could be a consequence of direct damage to the retina or the choroid, which can result in choroidal necrosis, followed by retinal tear on the corresponding spot. Retinal break by ocular contusion mostly occurred in the inferior temporal (Fig. 7.3a–c).

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Fig. 7.2 (a, b) Vitreous base avulsion results in a vitreous band suspending in the vitreous. Figure b show the macular edema and the retinal folds

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Fig. 7.3 (a) Commotio retinae (b, c) Ocular contusion and retinal break, retinal hemorrhage, mostly occurred in the inferior temporal

7.1.5 Traumatic Macular Hole

7.1.7 Ciliary Epithelial Detachment

Traumatic macular hole (TMH) is a well-known complication of ocular contusion injury. According to relevant literature, the frequency of TMH is between 1 and 9% [7]. It can be caused by the traction of vitreous or secondary to severe injury such as retinal concussion or choroidal rupture (Fig.  7.4a, b). Retinal detachment arising from TMH occurs rarely, surgical intervention is controversial due to possible spontaneous resolution of the RD along with MH closure [8, 9]. Extensive longterm ophthalmological follow-up is required.

Ciliary epithelial detachment is an important kind of traumatic retinal detachment. Retina combines tightly with the ciliary epithelium at ora serrata. This combination could be loosed by trauma. In such cases, fluid can come through the gap and result in retinal detachment (Fig. 7.5a–d). Blunt contusion of the ocular can cause tears of the front vitreous base avulsion, resulting in ciliary epithelial tear. In eyes with traumatic corneal and/or sclera rupture, vitreous could adhere to the wounds and proliferation. Hyperplasia would tract the ciliary epithelium; ciliary body cyst will be formed. Cyst break will cause the ciliary epithelial tear. Similarly, other unexplained ciliary body nonpigmented epithelial cyst broken can also form the tear, and then ciliary epithelial detachment will be presented, followed with the retinal detachment.

7.1.6 Retinal Tear near the Equator Retinal tear near the equator is not so common in ocular trauma. But it can cause retinal detachment and threats the visual acuity severely.

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Fig. 7.4 (a) Traumatic macular hemorrhage (b) Traumatic macular hole (TMH)

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Fig. 7.5 (a, b) Ciliary epithelial detachment and retinal detachment. Figure c, d show the lication of ciliary epithelial detachment in the gonioscopy

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7.1.8 Others Blunt contusion of the eye may also result in the pupillary margin damage, lens dislocation, secondary glaucoma, hyphema, traumatic hypotony syndrome (Fig. 7.6), choroidal rupture (Fig.  7.7), traumatic optic nerve retinopathy (Fig. 7.8), and so on.

Fig. 7.8  Traumatic optic nerve retinopathy Fig. 7.6  Traumatic hypotony syndrome

Fig. 7.7  Choroidal rupture

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Open-Globe Injury

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Open-globe injuries, defined as full-thickness penetration of the eye wall, are devastating due to the sudden and wide-ranging nature of the trauma. As the leading cause of visual loss for working-aged individuals, the cost of open-globe injuries to the patient and society is considerable, not only because of medical suffering and expenditures but also due to lifelong loss of productivity. According to the shape of the wounds, open-globe injuries can be classified into two types, ocular perforation and ocular penetration. In open-globe injuries, a poor preoperative best-corrected visual acuity is associated with poorer visual acuity outcome, more additional surgeries, and a higher rate of enucleation [10].

7.2.1 Ocular Perforating Injury Ocular perforating injury is one of the reasons of monocular vision loss, mostly occurring in sharp instrument hurts, with or without intraocular foreign body. Inevitably, ocular perforating injury often combines with series of injuries of different parts, such as the cornea, sclera, lens, and so on. Vision prognosis depends on a variety of factors, such as the relationship between injury sites to the macula or optic nerve, whether combine with endophthalmitis, vitreous hemorrhage, intraocular foreign body, its toxicity, etc. Vitreous incarceration or prolapse can occur in scleral perforation injury, especially from the corneal limbus to the ora serrata. After debridement, incarcerated vitreous become hyperplasia and fibrosis, which will cause tractional retinal detachment (Fig.  7.9a, b). Severe ora serrata or vitreous base avulsion can cause adhesion of the retina with the scleral perforation wound (Fig.  7.10). Tractional retinal detachment resulting from the intraocular fibrosis hyperplasia is the most severe complication of the ocular perforation injury.

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Fig. 7.9 (a, b) After debridement, incarcerated vitreous become hyperplasia and fibrosis, which will cause tractional retinal detachment

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7.2.2 Ocular Penetrating Injury

7.3

Ocular penetrating injury has both the entrance and exit wound on the ocular wall, mostly caused by high-speed sharp metals, such as iron, copper fragments, or gunshot. Although ocular penetrating is not so common, it can cause great damage to the eye, with poor prognostic vision. Studies showed that after injury, the vitreous between the entrance and exit would concentrate, forming a fibrosis band. In eyes with ocular penetrating injury, fibroblasts and pigment epithelium mixed together, and hyperplasia, followed with contraction and traction, tractional retinal detachment may occur. Experimental modeling and clinical experience showed that vitrectomy can prevent the process of proliferation, reduce the occurrence of retinal detachment, and improve the visual prognosis.

Intraocular foreign body usually associates with open-globe injury, accounting for 18–41% [11]. For eyes with intraocular foreign body, the nature of the foreign body determines the clinical behavior [12]. Inert objects such as steel and glass may remain asymptomatic and not cause significant inflammation to warrant their removal. However, organic foreign bodies may second with serious morbidities such as cellulitis, optic neuropathy, and ocular dismotility [13]. Removal of them is mandatory. Preoperative and postoperative retinal detachment is a serious complication of injury associated with intraocular foreign body, with a poor visual prognosis [11]. Foreign bodyrelated retinal detachment mostly occurs in eyes with posterior injury. The occurrence of retinal detachment was associated with a lot of factors, such as the characteristic of foreign body, whether combined with intraocular inflammation, vitreous hemorrhage and surgical timing as well. Removal of a foreign body in the presence of a detached retina is associated with an increased risk of iatrogenic retinal breaks, which in turn can lead to increased risk of postoperative retinal detachment. When patients with a history of trauma, doctors should pay attention to examine whether there is a perforation injury carefully, in particular, the scleral wound. In addition, the details about the injury, such as the characteristic of foreign body, the angle, and the distance between eye and the foreign body, should also be inquired. When intraocular foreign body was suspected, X-ray examination would be performed to determine the location of foreign bodies. If the refractive media is clear, the fundus should be examined carefully with a direct or indirect ophthalmoscope. Foreign body or metal foreign body with fibrous clumps can often be discovered in the posterior ­vitreous, retina, or optic disk. If the fundus could not be examined, ultrasound B scan is valuable for the assessment of the location of foreign body, vitreous, and retinal conditions. For those nonmetallic foreign body that can’t be determined in X-ray, ultrasound, MRI, or CT would be needed.

Fig. 7.10  Severe ora serrata or vitreous base avulsion can cause adhesion of the retina with the scleral perforation wound

Intraocular Foreign Body

7.3.1 L  ooking for the Channel of the Foreign Body If the foreign body can not be detected directly, the channel which foreign body passed by can provide us some helpful information to determine the lesions. 1. Fresh corneal wound or healing scar often suggests the size and character of foreign bodies. For example, a neat wound gives us the tips of the metal debris, while an irregular wound may suggest a nonmetallic foreign body. Corneal perforation injuries often combine with iris or lens damage; the vitreous or retina also can be involved.

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2. Vitreous channel: If the cornea is ruptured but the lens is still transparent, or the entrance of the foreign body is on the sclera, examine the vitreous channel carefully. This channel can be detected as a transparent or translucent cord, or white bundle may be associated with pigment particles or bleeding. The anterior segment can be checked by slit lamp, and indirect ophthalmoscopy could be used to examine the posterior segment. Along the channel, foreign body can be detected at the end of the channel. 3. If the channel of foreign body is discovered, X-ray, CT, or other auxiliary examination would be needed to confirm and locate the foreign body.

7.3.2 Determine the Retinal Damage When the foreign body is deep, the retina can be damaged, resulting in retinal tear, vitreous hemorrhage, or retinal

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detachment. The foreign body can also reach the subretinal space or incarcerated in the sclera. 1. Newly occurred retinal foreign bodies: Along the vitreous channel, retinal foreign body often can be found at the point of retinal hemorrhage. If the hemorrhage is not very dense, the foreign body can be detected under the ophthalmoscope. Or else the foreign body would be blocked by the hemorrhage. Sometimes, foreign body can hit the retina firstly and then bounce back and stay in other parts. Foreign bodies often stay in the inferior part due to the gravity. 2. If the foreign body stays in the eye for a long time, it can be wrapped by fiber clumps. Fiber proliferation may extend to the peripheral retina, resulting in epiretinal membrane or leading to retinal detachment (Fig. 7.11a–d).

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Fig. 7.11 (a–d) The foreign body stays in the eye and the fiber proliferation may extend to the peripheral retina, resulting in epiretinal membrane, or leading to retinal detachment

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Asymptomatic inorganic foreign body may be followedup without surgical removal. When deciding to perform surgery, potential complications of surgical removal should also be considered, as well as composition and possible effects of intraocular foreign body. For most of the eyes with intraocular foreign body, the retention of foreign body can cause a series of complications. Intraocular infection is one of the serious complications, which can cause retinal detachment. Foreign body in the vitreous can cause fiber proliferation and proliferative membrane. Those foreign bodies close to the retina can cause the proliferative vitreoretinopathy (PVR). Severe PVR and pre-PVR can lead to tractional retinal detachment and ultimately cause blindness. Because of an uncertain traumatic history or missed foreign body during the examination, long-term retention of the intraocular foreign body can cause a lot of complications, such as retinal detachment, ocular siderosis, or ocular chalcosis. Typical signs can be found in anterior segment, accompanied by vitreous opacification and tractional retinal detachment (Fig. 7.12a, b).

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Traumatic Tractional Retinal Detachment

7.4.1 Pathogenesis Tractional retinal detachment caused by trauma is one of the main causes of blindness. The process of the traumatic healing of the eye is the basis for the occurrence of tractional retinal detachment. That means, tractional retinal detachment occurs in the final wound healing stage. This wound healing reaction is benefit for recovery if it occurs in other parts of the body but can result in tractional retinal detachment in the eye. The Process of Tractive Retinal Detachment 1. First stage: a certain pathological changes or irritation can cause inflammation of the posterior segment of the eye. 2. Second stage: in blood-retinal barrier damage, a series of factors that come out from the blood can stimulate wound healing reaction in the vitreous.

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Fig. 7.12 (a) The ocular chalcosis (b) The ocular siderosis and secondary retinitis pigmentosa

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3. Third stage: cell migration and proliferation in the posterior segment plays the most important role in occurrence of tractional retinal detachment. 4. Fourth stage: after proliferation, the contractile force of the aggregation of cells can break the adhesion of the retinal sensory layers, and tractional retinal detachment would occur. 5. Fifth stage: tractional retinal detachment may play a terrible role on the blood-retinal barrier, and inflammation would become exacerbate, leading to further retinal detachment (Fig. 7.13).

7.4.2 Clinical Features Tractional retinal detachment commonly occurs in perforated ocular trauma with vitreous hemorrhage, with or without intraocular foreign body.

7.4.2.1 Tractional Retinal Detachment Combined with Vitreous Hemorrhage For eyes with tractional retinal detachment combined with vitreous hemorrhage, if there is a serious pre-PVR, the retinal detachment often presents as concentric annular. It can develop to funnel-shaped retinal detachment in the late stage. For eyes with local vitreous hemorrhage, the hemorrhage often occurs in the inferior. Retinal detachment can be found in the inferior or the superior funds, with tractive retinal tears in the peripheral part or ora serrata dialysis.

Fig. 7.13  Tractional retinal detachment

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7.4.2.2 Tractional Retinal Detachment Combined with Intraocular Foreign Body and Vitreous Hemorrhage For those eyes with proliferation of the intraocular foreign body pathways, tractional retinal detachment can be caused. Tractive retinal tear can be found in front of the equator of its opposite. 7.4.2.3 Tractional Retinal Detachment After the Surgery for Intraocular Foreign Body Removal After the surgery for intraocular foreign body removal, secondary tractional retinal detachment often associates with the channel or the export of foreign body, especially for those eyes with pars plana vitrectomy; retinal detachment would be caused by retinal incarceration or retinal hemorrhage.

7.5

Endophthalmitis

Endophthalmitis is referred to as the inflammation of the vitreous, retina, and uvea and caused by the bacteria, fungi, or other pathogenic microorganisms. Bacterial infection is more common, especially Gram-positive aerobic bacteria, such as staphylococcus epidermidis. Fungal infection accounts for about 1/4  in exogenous endophthalmitis, of which Aspergillus and Candida albicans-based are the most common pathogens. Traumatic endophthalmitis could occur in the injuries with retained intraocular foreign body, openglobe injury, and also iatrogenic endophthalmitis. Iatrogenic endophthalmitis most commonly occurred in ocular perforating injury, followed by intraocular surgery. Postcataract bacterial endophthalmitis is another reason for iatrogenic endophthalmitis. The clinical features of the endophthalmitis include the acute onset, rapidly vision reduction. Most patients complain with associated signs and symptoms including photophobia, tearing, eye pain, conjunctival congestion and edema, corneal edema, anterior chamber opacity or empyema, pupil narrowing, exudative membrane of the pupil, iris swelling, blurred iris texture, lens opacity, yellow-white reflection from behind the pupil, and low intraocular pressure. For some patients, those symptoms may be absent in most cases at initial presentation, likely because they are masked in the presence of severe ocular injury, and the red light reflection can’t be even seen because of vague refractive media [14]. Endophthalmitis is a rare but serious complication. If not treated promptly, it can develop to corneal or scleral ulceration, rupture, cataract, glaucoma, vitreous opacity, trac-

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tional retinal detachment, and finally the eye atrophy, leading to anatomical and/or functional loss of the eye (Fig. 7.14a–e). Retinal detachment associated with endophthalmitis is very common, even after pars plana vitrectomy is performed,

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especially those eyes with retained intraocular foreign body [15, 16]. It could be rhegmatogenous, tractional, or exudative retinal detachment. Pars plana vitrectomy would be performed immediately, with poor prognosis.

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Fig. 7.14 (a–e) Endophthalmitis

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References 1. Ersanli D, Sonmez M, Unal M, Gulecek O. Management of retinal detachment due to closed globe injury by pars plana vitrectomy with and without scleral buckling. Retina. 2006;26(1):32–6. 2. Hoogewoud F, Chronopoulos A, Varga Z, Souteyrand G, Thumann G, Schutz JS.  Traumatic retinal detachment—the difficulty and importance of correct diagnosis. Surv Ophthalmol. 2016;61(2):156–63. 3. Boscher C, Kuhn F.  Endoscopic evaluation and dissection of the anterior vitreous base. Ophthalmic Res. 2015;53(2):90–9. 4. Gonzales CA, Scott IU, Chaudry NA, Oster AS, Hess DJ, Murray TG. Bilateral rhegmatogenous retinal detachments with unilateral vitreous base avulsion as the presenting signs of child abuse. Am J Ophthalmol. 1999;127(4):475–7. 5. Cox MS, Schepens CL, Freeman HM. Retinal detachment due to ocular contusion. Arch Ophthalmol. 1966;76(5):678–85. 6. Kimura M, Nishimura A, Sugiyama K.  Localized vitreous adhesion to the retina after ocular contusion with a baseball. Clin Ophthalmol. 2012;6:879–84. 7. Querques G, Barone A, Forte R, Prascina F, Iaculli C, Delle Noci N. Optical coherence tomography and fundus-related perimetry in spontaneous closure of a traumatic macular hole. J Fr Ophtalmol. 2008;31:710–3. 8. Nasr MB, Symeonidis C, Tsinopoulos I, Androudi S, Rotsos T, Dimitrakos SA. Spontaneous traumatic macular hole closure in a 50-year-old woman: a case report. J Med Case Rep. 2011;5:290.

J. Zhou et al. 9. Aalok L, Azad R, Sharma YR, Phuljhele S.  Microperimetry and optical coherence tomography in a case of traumatic macular hole and associated macular detachment with spontaneous resolution. Indian J Ophthalmol. 2012;60(1):66–8. 10. Page RD, Gupta SK, Jenkins TL, Karcioglu ZA.  Risk factors for poor outcomes in patients with open-globe injuries. Clin Ophthalmol. 2016;10:1461–6. 11. Loporchio D, Mukkamala L, Gorukanti K, Zarbin M, Langer P, Bhagat N. Intraocular foreign bodies: a review. Surv Ophthalmol. 2016;61(5):582–96. 12. Wasfi E, Kendrick B, Yasen T, Varma P, Abd-Elsayed AA. Penetrating eyelid injury: a case report and review of literature. Head Face Med. 2009;5:2. 13. Dolar Bilge A, Yılmaz H, Yazıcı B, Naqadan F. Intraorbital foreign bodies: clinical features and outcomes of surgical removal. Ulus Travma Acil Cerrahi Derg. 2016;22(5):432–6. 14. Palioura S, Eliott D.  Traumatic endophthalmitis, retinal detachment, and metallosis after intraocular foreign body injuries. Int Ophthalmol Clin. 2013;53(4):93–104. 15. Chiquet C, Aptel F, Combey-de Lambert A, Bron AM, Campolmi N, Palombi K, Thuret G, Rouberol F, Cornut PL, Creuzot-Garcher C, FRIENDS (French Institutional Endophthalmitis Study) group. Occurrence and risk factors for retinal detachment after pars plana vitrectomy in acute postcataract bacterial endophthalmitis. Br J Ophthalmol. 2016;100(10):1388–92. 16. Parke DW 3rd, Pathengay A, Flynn HW Jr, Albini T, Schwartz SG.  Risk factors for endophthalmitis and retinal detachment with retained intraocular foreign bodies. J Ophthalmol. 2012;2012:758526.

8

Pathologic Myopia and Retinal Detachment Liqin Gao, Nan Zhou, and Wenbin Wei

Abstract

Pathologic myopia (PM) is described as myopia accompanied by excessive axial elongation of the globe and characteristic degenerative changes in the posterior segment, with posterior staphyloma as an important hallmark lesion. Myopia is a complex disease and the etiology of myopia is not clearly understood. The pathogenesis of pathologic myopia is possibly related with biomechanics. It is believed that the excessive elongation of the globe and sclera staphyloma are the most important factors in the development of these degenerative changes in pathologic myopia. Rhegmatogenous retinal detachment is a commom complication of pathologic myopia.

L. Gao, M.D., Ph.D. · N. Zhou, M.D., Ph.D. · W. Wei, M.D., Ph.D. (*) Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China e-mail: [email protected]

8.1

Introduction

Pathologic myopia (PM) is described as myopia accompanied by excessive axial elongation of the globe and characteristic degenerative changes in the posterior segment, with posterior staphyloma as an important hallmark lesion [1]. Although the definition of pathologic myopia is not standardized, it is usually defined by a refractive error of greater than −6.00 diopters (D) and an axial length more than 26.5 mm, along with the degenerative changes involving the sclera, optic disc, choroid, Bruch’s membrane, retinal pigment epithelium (RPE), and neural retina. The terms “malignant myopia” and “degenerative myopia” have also been used. The prevalence of myopia varies greatly between different populations and ethnic groups. Myopia is highly more frequent in Chinese, especially among school-age children. Recent studies on schoolchildren in East China revealed the prevalence of myopia increased from 1.7% in the 4-year-olds to 84.6% in 17-year-olds, and 14% of the 17-year-olds were highly myopic [2]. Complications from pathologic myopia become one of the leading causes of visual impairment and legal blindness in the elderly population worldwide both from populations based studies and from blind registry data. Myopic retinopathy has been reported to be the second common cause of visual impairment and blindness in Chinese populations [3–5]. Myopia is a complex disease, and the etiology of myopia is not clearly understood. It is well documented as a multifactorial etiology, both genetic and environmental influences. The modes of inheritance of myopia include X-linked recessive inheritance, autosomal recessive inheritance, and autosomal dominant pattern. It is speculated that the near work, accommodation, education level, and urbanization may be the environmental factors for the onset and progression of myopia [2, 3]. The pathogenesis of pathologic myopia is possibly related with biomechanics. It is believed that the excessive elongation of the globe and sclera staphyloma are

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the most important factors in the development of these degenerative changes in pathologic myopia [6], including the features of chorioretinal atrophy, central pigment spot (“Fuchs spot”), lacquer cracks, posterior staphyloma, and optic disc changes.

8.2

Changes of Myopic Chorioretinopathy

8.2.1 Tessellated Fundus The tessellated fundus appearance may result from a thinning of the RPE and choriocapillaris following the progressive elongation of the globe [7]. The reduced density of the retinal pigments allows the choroidal vessels easily to be seen (Fig. 8.1).

8.2.2 T  ilted Disc, Myopic Conus, and Tractional Crescent A tilted optic disc is a relatively common finding in eyes with myopia and represents the oblique insertion of the optic nerve into the globe. With the continued elongation of the globe, there are three possible axes of rotation for the optic disc: horizontal axis, vertical axis, and torsional axis. Usually, the temporal portion of the nerve is more posterior than the nasal portion [8], and the myopic conus, peripapillary areas of retinal pigment epithelium atrophy, occurs from the temporal side of the optic disc. Myopic conus varies greatly. As the axial length develops, the retinal pigment epithelium and choroid around the optic nerve head come to atrophy, and the exposed sclera looks white. In some cases, the retina and choroid are pulled at the nasal side of optic disc and seemingly grow into optic nerve head, which forms the red or brown tractional crescent and makes the nasal boundary vague (Figs. 8.1 and 8.2).

Fig. 8.1  The tessellated fundus Fig. 8.2  The tessellated fundus, tilted optic disc and tractional crescent

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8.2.3 Chorioretinal Atrophy Chorioretinal atrophy has been described diffuse and patchy types, respectively. Diffuse chorioretinal atrophy can be observed as yellowish-white appearance of the posterior pole, which usually first appears around the optic disc [9]. The extent of the diffuse atrophy may vary from a restricted area around the optic disc and a part of the macula to the entire posterior pole. Patchy chorioretinal atrophy appears as well-defined, grayish-white lesion(s) in the macular area or around the optic disc, sometimes with pigment clumping within and

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underlined by a pigmented line. Patchy chorioretinal atrophy is characterized by a complete loss of choriocapillaris and can progress to an absence of the outer retina and retinal pigment epithelium (Fig. 8.3a, b). The patchy chorioretinal atrophy is most likely to occur in the macula and to enlarge in all directions. With progressive atrophy, the choroidal vessels appear yellowish or whitish in the atrophic zone; the term “sclerosis” is used. Three different types of patchy atrophy have been reported: one progressing from lacquer cracks, one developing within the area of severe diffuse atrophy, and one developing along the edge of a staphyloma [7].

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Fig. 8.3 (a) The tessellated fundus and local subretinal hemorrhage. (b) The tessellated fundus and patchy chorioretinal atrophy

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8.2.4 Posterior Staphylomas Posterior staphyloma is a protrusion of the posterior shell of the eye, which is considered to be a hallmark lesion of pathologic myopia. A posterior staphyloma is presented as a local bulging of the sclera at the posterior pole of the globe that has a radius of less than the surrounding curvature of the wall of the eye [7]. The sclera of pathologic myopia has increased elasticity and a tendency to expand gradually and to thin. By binocular indirect ophthalmoscope, the edge of the staphyloma is inclined or steep, with retinal vessels crawl geniculately above the margins and pigment abnormalities along it. Staphylomas are various in their localization, shape, and a

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Fig. 8.4 (a, b) Posterior staphyloma

depth. The common methods to detect and classify the staphylomas include color fundus photography and ultrasonography or a combination of both, while OCT and three-dimensional MRI are also used to analyze and classify the staphyloma recently. Huang et  al. [10] reported that staphylomas were present in 90% of a group of 209 eyes with high myopia (myopic refractive error  >  8 D or AXL ≥ 26.5 mm). The progression of staphylomas is highly correlated with age and axial length. Children and young individuals do not generally have staphylomas even though they are highly myopic [11]. It was found that the worse vision in highly myopic eyes with staphyloma than those without (Fig. 8.4a, b).

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8.2.5 Lacquer Cracks Lacquer cracks are mechanical breaks of the Bruch’s membrane and are identified by yellowish linear lesions in the macula. They present as linear horizontal or vertical cracks or exhibit a crisscrossing (stellate) pattern, and radiate from the disc, at the papillomacular bundle, through the macula and around the macula. Most of the lacquer cracks do not run in parallel to the large choroidal vessels, and they often crisscross over the underlying choroidal vessels (Fig. 8.5a, b). The prevalence of lacquer crack in

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Fig. 8.5 (a, b) The tessellated fundus and lacquer cracks

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highly myopic eyes has been reported from 4.3 to 15.7% in several studies using different methods [7, 9]. Lacquer cracks show irregularly and discretely linear or stellate hyperfluorescence in the early phase of fluorescein angiography. The fluorescence increases moderately, and in the late phase, lacquer cracks show faintly hyperfluorescence (Fig. 8.6a–c). It is widely accepted that ICGA is the best method for detecting lacquer cracks, which typically shows linear hypofluorescence in the late-phase ICGA.  Subretinal hemorrhage may be observed at the onset of lacquer cracks without CNV.

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Fig. 8.6 (a–c) The fluorescence increases moderately, and in the late phase, lacquer cracks show faintly hyperfluorescent

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8.2.6 Myopic Choroidal Neovascularization Myopic choroidal neovascularization (CNV) is a most common vision-threatening complication of pathologic myopia, and it develops in about 5–11% of highly myopic patients [7, 9, 12]. An active CNV appears as a flat, small, light gray, round, or elliptic subretinal lesion beneath or in close proximity to the fovea with or without hemorrhage.

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Fig. 8.7 (a–c) Myopic choroidal neovascularization (CNV)

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There are usually mild subretinal fluid or exudative changes associated with the myopic CNV (Fig. 8.7a–c). Most myopic CNVs may be developed from lacquer creaks or an atrophic area. As the CNV progressed finally further to develop macular atrophy or Fuchs spot, the natural history of eyes with myopic CNV is poor. Intravitreal anti-VEGF therapy has been proved an effective and safe method for myopic CNV.

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8.2.7 Macular Hemorrhage Macular hemorrhage may be observed in highly myopic eyes with or without CNV, and the latter one is termed simple hemorrhage. Simple hemorrhage appears as round, welldefined, and dark-red subretinal bleeding, which is due to mechanical stretching and rupture of the Bruch’s membrane leading to new lacquer crack formation [6] (Fig. 8.8a, b). It is a

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reported in 77% eyes with subretinal hemorrhage without CNV, lacquer cracks appeared at the corresponding site of the previous bleeding [13]. The simple hemorrhage will resolve spontaneously, and the visual prognosis is generally more favorable compared with myopic CNV. FFA and ICGA are useful methods to differentiate macular hemorrhage.

8.2.8 Fuchs Spot Fuchs spot appears as a black ring area with clear boundary in the posterior pole, usually slightly smaller than the disc [7]. It is a pigmented spot representing the scarring phase of myopic CNV (Fig.  8.9). The fibrovascular membrane becomes reduced in size and flattens and then forms a grayish-white scar sometimes along with pigmentation (Fig. 8.10a, b).

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Fig. 8.9  Fuchs spot

Fig. 8.8 (a, b) Macular hemorrhage

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Fig. 8.10 (a, b) Fuchs spot, and the fibrovascular membrane forms a grayish white scar sometimes along with pigmentation

8.2.9 Macular Hole The formation of macular hole may be associated with vitreomacular traction due to posterior staphyloma or longer axial length, epiretinal membrane, and reduced chorioretinal adhesion due to RPE atrophy. And the focal chorioretinal atrophy or scar and cystoid macular changes may also contribute to the hole formation. Myopic macular hole appears as round or oval defect of macular tissue, without flap. It often looks pale due to the chorioretinal atrophy in the back-

ground. Myopic macular hole is often accompanied with foveoschisis and may develop macular hole retinal detachment [14, 15] (Fig. 8.11a, b).

8.2.10 Peripheral Retinal Degeneration Peripheral retinal degeneration includes lattice degeneration, paving stone degeneration, pigmentary degeneration, and white without pressure. The prevalence of peripheral retinal

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degeneration is associated with high myopia and increased axial length. Lattice degeneration is the most important peripheral retinal degeneration that may develop tears, breaks, or holes and further progress to rhegmatogenous retinal detachment. It is atrophy of peripheral retina characterized by oval or linear patches of retinal thinning with branching white lines, running in parallel to ora serrata. Atrophy holes may be found in the lesion, while tractional tear may be found at the ends or posterior margins with exaggerated vitreoretinal attachments. Lattice degeneration is sometimes combined with pigmentation, white with pressure, and white without pressure.

8.2.11 Changes of Vitreous Degeneration of vitreous occurs earlier in pathologic myopic eyes and progresses with age. The major changes include the

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Fig. 8.11 (a, b) Myopic macular hole

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breakdown of collagen fiber network, vitreous gel collapse, and vitreous liquefaction. The collagen fibrils harden sometimes leading to perception of the mobile dots and threads known as muscae volitantes or “floaters.” The degeneration of vitreous eventually results in separation of the vitreous from the retina, which is described as posterior vitreous detachment. In the course of posterior vitreous detachment, the vitreous traction may stimulate the retina and lead to flashes. Weiss ring, a prepapillary ringlike floater, is considered to be the sign of complete PVD since vitreoretinal adhesion is strongest at the optic nerve head. In high myopia, PVD develops increasingly with age and degree of myopia. It is suggested that PVD may develop nearly 10 years earlier in highly myopic than in emmetropic eyes [16]. Excessive vitreous retinal adherent and vitreoschisis may also be found in pathologic myopic eyes.

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8.3

Complications

8.3.1 Rhegmatogenous Retinal Detachment Rhegmatogenous retinal detachment is a common complication of pathologic myopia. The prevalence of rhegmatogenous retinal detachment is significant association of increasing axial length. It is reported that almost 55% of all patients with rhegmatogenous retinal detachment were attributable to myopia [17]. The vitreous degeneration and posterior vitreous detachment, holes and tears with peripheral retinal degeneration, and vitreoretinal attachment result in retinal detachment (Fig.  8.12a, b). Macular hole, horseshoe tear and atrophy hole with lattice degeneration, and giant retinal tear are the common types. High myopia is one of the risk factors for giant retinal tears, especially following trauma [18]. The giant tear is associated with the peripheral retinal degeneration of white with pressure and extensive vitreous liquefaction with condensation of the vitreous base. Macular hole-related retinal detachment occurs mostly in older women with pathologic myopia. The retinal detachment resulting from full-thickness macular hole may be limited in the central retina within the arcade or posterior staphyloma, and it may progress to extensive peripheral detachment of the retina. When macular hole-related retinal detachment with peripheral detachment of the retina ­combined with peripheral retinal breaks, a secondary macular hole should be considered (Fig. 8.13a–h). Horseshoe tear-associated retinal detachment is related to development of horseshoe tear and abnormal vitreoretinal

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traction around it. Most of the horseshoe tear appears with a flap and often occurs along the retinal blood vessels. Vitreous hemorrhage, more or less, happens with it. This type of retinal detachment is more susceptible to proliferative vitreoretinopathy.

8.3.2 Glaucoma Individuals with myopia have an increased risk of developing open-angle glaucoma and increased risk of developing the disease approximately by two- to threefolds [19]. Eyes with increased axial length appear to have higher cup-disc ratios (CDRs), increased optic nerve fiber layer defects, and possibly greater deformability of the lamina cribrosa, leading to higher susceptibility to glaucomatous optic disc changes [20]. Myopia adds significant complexity to the diagnosis, monitoring, and treatment of open-angle glaucoma.

8.3.3 Cataract Cataract is the first leading cause of blindness worldwide. The Australian Blue Mountains Eye Study of adults aged over 49  years reported that posterior subcapsular cataract (PSC) was associated with myopia, while high myopia was associated with all three types of cataract: PSC, cortical cataract, and nuclear cataract [21].

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Fig. 8.12 (a, b) Pathologic myopia and rhegmatogenous retinal detachment

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Fig. 8.13 (a–h) Macular hole-related retinal detachment

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References 1. Curtin BJ. The posterior staphyloma of pathologic myopia. Trans Am Ophthalmol Soc. 1977;75:67–86. 2. Wu JF, Bi HS, Wang SM, et al. Refractive error, visual acuity and causes of vision loss in children in Shandong, China. The Shandong Children Eye Study. PLoS One. 2013;8(12):82763. 3. Xu L, Li Y, Wang S, Wang Y, et al. Characteristics of highly myopic eyes: the Beijing Eye Study. Ophthalmology. 2007;114(1):121–6. 4. Kempen JH, Mitchell P, Lee KE, et al. The prevalence of refractive errors among adults in the United States, Western Europe, and Australia. Arch Ophthalmol. 2004;122(4):495–505. 5. Liang YB, Friedman DS, Wong TY, et al. Prevalence and causes of low vision and blindness in a rural Chinese adult population: the Handan Eye Study. Ophthalmology. 2008;115(11):1965–72. 6. Moriyama M, Ohno-Matsui K, Hayashi K, et al. Topographic analyses of shape of eyes with pathologic myopia by high-resolution three-dimensional magnetic resonance imaging. Ophthalmology. 2011;118(8):1626–37. 7. Ohno-Matsui K, Kawasaki R, Jonas JB, et al. International photographic classification and grading system for myopic maculopathy. Am J Ophthalmol. 2015;159(5):877–83. 8. Hyung SM, Kim DM, Hong C, et al. Optic disc of the myopic eye: relationship between refractive errors and morphometric characteristics. Korean J Ophthalmol. 1992;6(1):32–5. 9. Ohno-Matsui K, Lai TYY, Lai CC, et  al. Updates of pathologic myopia. Prog Retin Eye Res. 2016;52:156–87. 10. Huang WH, Ohno-Matsui K, Shimada N, et al. Clinical characteristics of posterior staphyloma in eyes with pathologic myopia. Am J Ophthalmol. 2008;146(1):102–10.

11. Kobayashi K, Ohno-Matsui K, Kojima A, et  al. Fundus char acteristics of high myopia in children. Jpn J Ophthalmol. 2005;49(4):306–11. 12. Ohno-Matsui K, Yoshida T.  Myopic choroidal neovascular ization: natural course and treatment. Curr Opin Ophthalmol. 2004;15(3):197–202. 13. Klein RM, Green S. The development of lacquer cracks in pathologic myopia. Am J Ophthalmol. 1988;106(3):282–5. 14. Wang S, Xu L, Jonas JB, et al. Prevalence of full-thickness macular holes in urban and rural adult Chinese the Beijing Eye Study. Am J Ophthalmol. 2006;141(3):589–91. 15. Benhamou N, Massin P, Haouchine B, et al. Macular retinoschisis in highly myopic eyes. Am J Ophthalmol. 2002;133(6):794–800. 16. Akiba J. Prevalence of posterior vitreous detachment in high myopia. Ophthalmology. 1993;100(9):1384–8. 17. The Eye Disease Case Control Study Group. Risk factors for idiopathic rhegmatogenous retinal detachment. Am J Epidemiol. 1993;137(7):749–57. 18. Stirpe M, Heimann K. Vitreous changes and retinal detachment in highly myopic eyes. Eur J Ophthalmol. 1996;6(1):50–8. 19. Marcus MW, de Vries MM, Junoy Montolio FG, et al. Myopia as a risk factor for open-angle glaucoma: a systematic review and metaanalysis. Ophthalmology. 2011;118(10):1989–94. 20. Saw SM, Gazzard G, Shih-Yen EC, et  al. Myopia and asso ciated pathological complications. Ophthalmic Physiol Opt. 2005;25(5):381–91. 21. Lim R, Mitchell P, Cumming RG.  Refractive associations with cataract: the Blue Mountains eye study. Invest Ophthalmol Vis Sci. 1999;40(12):3021–6.

9

Infectious Uveitis and Retinal Detachment Liqin Gao, Nan Zhou, and Wenbin Wei

Abstract

Ocular cysticercosis is a parasitic infection of the larval form of the pork tapeworm (cysticerci), Taenia solium, migrates into the eye or its adnexal tissues and encysts, leading to severe vitreous opacity and secondary retinal detachment. Acute retinal necrosis (ARN) is a rare infectious viral uveitis syndrome that is caused by members of the herpes family including varicella zoster virus (VZV), herpes simplex virus (HSV), cytomegalovirus (CMV), and less frequently Epstein-Barr virus (EBV). Choroid is most susceptibly involved in ocular infections of mycobacterium tuberculosis. For the solitary choroidal tuberculoma, careful differential diagnosis should be made with choroidal tumors.

9.1

Intraocular Cysticercosis

Ocular cysticercosis is a parasitic infection of the larval form of the pork tapeworm (cysticerci), Taenia solium, which migrates into the eye or its adnexal tissues and encysts. It is endemic in China, India, Indonesia, Pakistan, Eastern Europe, and Central America [1]. It is reported that 35% of cysts were in subretinal space, 22% in vitreous cavity, 22% in subconjunctival space, 5% in the anterior segment, and only 1% in the ocular orbit [2]. In the natural life cycle of Taenia solium, humans act as the definitive host, and pig is the intermediate host. Cysticercosis in man occurs when man accidentally becomes the intermediate host, by ingestion of eggs through

L. Gao, M.D., Ph.D. · N. Zhou, M.D., Ph.D. · W. Wei, M.D., Ph.D. (*) Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China e-mail: [email protected] © Springer Nature Singapore Pte Ltd. and Beijing Science and Technology Press 2018 W. Wei (ed.), Atlas of Retinal Detachment, https://doi.org/10.1007/978-981-10-8231-3_9

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contaminated water or food, which in turn is related to poor hygiene and poverty. T. solium eggs liberate from gravid proglottids that can infect humans and hatch out in the small intestine followed by parasitizing the subcutaneous tissues, muscles, brain (Fig. 9.1a–c), eyes, and heart, which lead to cysticercosis [3]. They travel via the hematogenous route, entering the central retinal artery system directly into the vitreous body, or reaching the subretinal space through the short posterior ciliary artery, and then entering the vitreous body [4]. After intraocular invasion, toxins and heterologous proteins in the vesicle liquid are released, which a

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leads to local granulomatous inflammation with fibrous tissue proliferation. When the parasite is untreated, it will eventually die after 2–4  years. Secondary severe uveitis, retinal detachment, and proliferative vitreoretinopathy may result in destruction of the eye, with the inflammatory reaction due to the release of toxins. The characteristics of intraocular cysticercosis differ in the location, size, and inflammatory reactions. In the vitreous, it is visualized as a translucent cyst, 3–8 mm diameters in size, with a white and condensing scolex pouch attached to the vesicle by an ophthalmoscope. Upon light stimulation, b

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Fig. 9.1 (a–c) T. solium eggs liberate from gravid proglottids can infect humans and hatch out in the small intestine followed by parasitizing the subcutaneous tissues, muscles, and brain

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spontaneous peristalsis of the vesicle could be observed, which caused scolex location changes as well as vesicle ­surface fluctuation. While in the subretinal space, a translucent cyst with a central dense white spot in the subretinal space [5], the feature of mobile under bright illumination indicates the location of the larva inside the cyst. We have found that the scolex shifted its location from subretinal space to the vitreous body through a retinal break, with the rest of cyst still in the subretinal space.

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The viable cysts cause mild inflammatory reactions, while the degenerating cyst rapidly increases in size due to osmotic regulation and caused compression of the surrounding tissues and release of antigens into the surrounding tissue leading to severe vitreous opacity and secondary retinal detachment (Fig. 9.2a–d). The most effective treatment of preserving function in an eye with intraocular cysticercosis is surgical removal of the larva by vitrectomy [6].

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Fig. 9.2 (a–d) The viable cysts leading to severe vitreous opacity and secondary retinal detachment

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Acute Retinal Necrosis

Acute retinal necrosis (ARN) is a rare infectious viral uveitis syndrome that is caused by members of the herpes family including varicella zoster virus (VZV), herpes simplex virus (HSV), cytomegalovirus (CMV), and less frequently EpsteinBarr virus (EBV). The vast majority (over 50%) of ARN is due to VZV, followed by HSV-2 (5.1%) and HSV-1 (3.5%) [7]. It can occur in patients of either sex and at any age, classically immunocompetent. Patients with HSV-2 appear to be much younger (mean 21 years) than those with either HSV-1 or varicella zoster (mean age 40 years) [8]. Most cases begin with one eye affected, and about one third of patients present with bilateral ARN within 6  weeks, ranging from several weeks to years. Some cases have a history of preceding or concurrent associated skin or systemic herpes infection. ARN may progress rapidly and lead to extensive peripheral retinal necrosis, retinal detachment, and multiple retinal breaks. ARN is characterized by acute uveitis, vitritis, and fullthickness necrotizing retinitis with occlusive vasculopathy. The diagnosis of ARN is based on clinical features. The Executive Committee of the American Uveitis Society established the diagnostic criteria for ARN: (1) one or more foci of retinal necrosis with discrete borders in the peripheral retina, (2) rapid progression of disease without treatment, (3) circumferential disease spread, (4) occlusive vasculopathy with arteriolar involvement, and (5) vitreous and anterior chamber inflammatory reaction [9].

1. At an early stage of the disease, most patients have complaints of blurred vision and floater, usually with pain and redness. The signs of an anterior uveitis, such as keratic precipitates, plasmoid aqueous are the most symptoms. Mild to moderate vitritis occurs with the optic disk swelling, veins dilating, and macular edema. The appearance of retinal vasculitis includes retinal arteritis, periphlebitis, and venous occlusion, with perivascular hemorrhages and sheathing. The necrotizing retinitis appears as multifocal small, patchy, white-yellow lesions, typically beginning in the midperiphery and then spreading and coalescing to the posterior pole (Fig. 9.3a–f). 2. The acute intraocular inflammation tends to resolve over 3–4 weeks with or without therapy. The peripheral lesions become pigmented, starting at their posterior margins. Some patients may have a vision improvement at this stage (Fig. 9.4a–d). 3. Late in the disease, up to 85% of cases develop tractional and rhegmatogenous retinal detachments generally within 1–2  months of the onset of disease [10]. Many retinal breaks, varying in numbers and shapes, occur in the areas of thinned necrotic retina with a clearing edge at the junction of normal and necrotic retina. Vitreous inflammation causes proliferative vitreoretinopathy, forming a traction force to the retinal detachment. A further sudden vision decline may happen (Fig. 9.5a–c).

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Fig. 9.3 (a–f) The necrotizing retinitis appears as multifocal small, patchy, white-yellow lesions, typically beginning in the midperiphery, then spreading and coalescing to the posterior pole

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Fig. 9.4 (a–d) The acute intraocular inflammation has resolved and the peripheral lesions become pigmented, starting at their posterior margins

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Fig. 9.5 (a–c) The extensive peripheral retinal necrosis, retinal detachment, and multiple retinal breaks

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Acquired Immunodeficiency Syndrome (AIDS)

1. The retinal manifestations of AIDS patients include noninfectious retinal vasculopathy and infectious retinopathy due to opportunistic infections. The retinal vasculopathy of HIV occurs in 25–92% of patients and includes cotton wool spots, retinal hemorrhages, and microvascular abnormalities such as microaneurysms, telangiectatic vascular changes, and focal areas of nonperfusion and capillary loss. Cotton wool spots, the microinfarction of nerve fiber layer, are the earliest and the most common lesions in HIV retinopathy and appear as superficial white fluffy lesions. Although individual lesions will disappear over a period of several months, new lesions often appear [11, 12]. Retinal hemorrhages associated with HIV infection appear as small dot, flame-shaped, or blot hemorrhages. Roth’s spot may sometimes be found, which is characterized by whitecentered retinal hemorrhage (Fig. 9.6a, b). 2. Cytomegalovirus (CMV) retinitis is the most common opportunistic infection of the eye in patients with AIDS.  CMV retinitis appears as multiple perivascular

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granular yellow-white dots or fluffy white lesions with varying amounts of hemorrhage, originating peripherally (Fig.  9.7a–d). The lesions tend to enlarge and coalesce over time and produce full-thickness retinal necrosis that may result in retinal detachment. Retinal detachment develops in approximately 20% of patients with retinitis, with 50% of these patients develop detachment in the second eye if it is involved with retinitis. It is reported that nearly 11% of patients with retinitis will experience a rhegmatogenous retinal detachment over 6 months after diagnosis and 24% will have detachment over the first year after diagnosis in the first eye [13]. Detachments were characterized by multiple, poorly visualized, fullthickness breaks within areas of necrotic retina and at the border of necrotic and healthy retina with the vitreous traction on it [14]. 3. Toxoplasmosis retinochoroiditis. The manifestations of toxoplasmosis retinochoroiditis secondary to opportunistic infections of AIDS patients are similar to those in the healthy, immunocompetent patients. Moreover, the inflammation is much more drastic, and the retina often cannot be detected clearly due to the dense opacity of aqueous and vitreous.

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Fig. 9.6 (a) Cotton wool spots with HIV infection. (b) Retinal hemorrhages associated with HIV infection appears as small dot, flame-shaped, or blot hemorrhages. Roth’s spot may be found, which is characterized by white-centered retinal hemorrhage

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Fig. 9.7 (a–d) CMV retinitis appears as multiple perivascular granular yellow-white dots or fluffy white lesions with varying amounts of hemorrhage, originating peripherally

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Purulent Chorioretinitis

Endogenous purulent chorioretinitis results from hematogenous spread of microorganisms from distant infectious foci, for example, cellulitis, endocarditis, and skin or pulmonary bacterial or fungal infections. Exogenous chorioretinitis also refers to infections resulting from breach of the globe exterior through surgery or trauma [15]. The progress of purulent chorioretinitis varies accordingly with the bacteria quantity, toxicity, and the whole and local organism immunity ability and whether timely and effective treatment is given. Lesion is relatively circumscribed in mild cases, while vitreous abscess soon occurs in severe cases. Mostly endogenous purulent chorioretinitis begins in the posterior pole. Exudative lesions with indistinct boundary are visible in the early state by ophthalmoscope through the mild vitreous opacity. Retina edema and thickening happen around the lesions with yellowish and even gray-brownish opacification. There are hemorrhage spots with different

sizes, exudations, and radial folds on the surface and/or on the surrounding area. Retinal vessels may go tortuous and considerably dilated, and some are covered by edema opacification and exudation. Vision is severely poor then, even only light perception. Inflammation soon spreads to the vitreous and develops vitreous cyst and retinal detachment, if bacterial amount and virulence are great and strong, while with weak immunity and untimely treatment. With the progression of inflammation, anterior uvea is involved, and only yellow reflex is found by ophthalmoscope because of the hazy media. Thus endophthalmitis occurs with severer pain of the affected eye, eyelid hyperemia and swelling, intensified bulbar conjunctiva hyperemia, and edema. Swelled bulbar conjunctiva may extrude beyond palpebral fissure with corneal opacity and hypopyon. Complete loss of vision happens, combined with headache, high fever, nausea, and vomiting. It is developed to panophthalmitis if sclera and Tenon capsule get involved, and then protopsis and restriction of ocular movement occur because of the swelling of orbital tissues (Fig. 9.8a–e).

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Fig. 9.8 (a–e) Endogenous purulent chorioretinitis

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Lyme Disease

Lyme disease, also known as erythema chronicum migrans, is an epidemic infectious disease caused by Borrelia burgdorferi and transmitted to humans by the bite of infected ticks of the Ixodes genus. It was firstly found in Sweden in the early twentieth century and named after the fact that the epidemic disease was prevailed in 1975 in Lyme, Connecticut, USA [16]. Lyme disease usually outbreaks in summer and mostly occurs among forestry workers or population in forest area. The prevalence goes up because of growing awareness of a

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Fig. 9.9 (a, b) Lyme disease

the disease by both physicians and patients. It has been reported throughout the world, and in China, it was firstly reported in Hailin county, Heilongjiang province, in 1986, and the pathogen was extracted from the blood of the patient in 1988 [17]. It is a multiple-system human immune disease caused by the Borrelia infection, which mainly damages the skin, joint, nerve system, and cardiovascular system. Its incubating period varies from 3 to 32 days, mostly 7 to 9 days. It is classified into three stages: localized infection, disseminated infection, and persistent infection (Fig. 9.9a, b).

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9.6

Choroidal Tuberculosis

Choroid is most susceptibly involved in ocular infections of Mycobacterium tuberculosis. The lesions of choroid vary according to the different quantity and virulence of bacterium and the local immunoreaction. The choroidal tuberculosis lesions may appear as multifocal miliary tuberculosis lesions of different sizes or choroidal granulomas; the latter may cause exudative retinal detachment. Granulomatous tuberculoma of choroid is usually in the posterior pole and uneven surface and is yellowish white. Small nodules and a

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annular exudation may appear in the surrounding area. With the increase of the subretinal fluid of the lesions, exudative retinal detachment occurs around the lesion, and the complete retinal detachment may occur in severe cases [12]. For the solitary choroidal tuberculoma, careful differential diagnosis should be made with choroidal tumors. It is differentiated from choroidal melanoma in color and shape, and its hemorrhage on surface and uneven status is different from choroidal hemangioma. Although choroidal osteoma has uneven surface, it is flat and not globular and rarely associated with retinal detachment (Fig. 9.10a–e). b

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Fig. 9.10 (a–c) Granulomatous tuberculoma of choroid is usually in the posterior pole and uneven surface and is yellowish white. (d–e) The choroidal tuberculosis before and after treatment

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References 1. Ito A, Nakao M, Wandra T, et  al. Taeniasis and cysticercosis in Asia and the Pacific: present state of knowledge and perspectives. Southeast Asian J Trop Med Public Health. 2005;36(Suppl 4):123–30. 2. Kruger-Leite E, Jalkh AE, Quiroz H, et al. Intraocular cysticercosis. Am J Ophthalmol. 1985;99(3):252–7. 3. Ament CS, Young LH. Ocular manifestations of helminthic infections: onchocersiasis, cysticercosis, toxocariasis, and diffuse unilateral subacute neuroretinitis. Int Ophthalmol Clin. 2006;46(2):1–10. 4. Li JJ, Zhang LW, Li H, et al. Clinical and pathological characteristics of intraocular cysticercosis. Korean J Parasitol. 2013;51(2):223–9. 5. Luger MH, Stilma JS, Ringens PJ, et  al. In-toto removal of a subretinal Cysticercus cellulosae by pars plana vitrectomy. Br J Ophthalmol. 1991;75(9):561–3. 6. Madigubba S, Vishwanath K, Reddy G, et al. Changing trends in ocular cysticercosis over two decades: an analysis of 118 surgically excised cysts. Indian J Med Microbiol. 2007;25(3):214–9. 7. Wong RW, Jumper JM, McDonald HR, et  al. Emerging concepts in the management of acute retinal necrosis. Br J Ophthalmol. 2013;97(5):545–52. 8. Ganatra JB, Chandler D, Santos C, et  al. Emerging concepts in the management of acute retinal necrosis. Br J Ophthalmol. 2013;97(5):545–52.

L. Gao et al. 9. Holland GN.  Standard diagnostic criteria for the acute retinal necrosis syndrome. Executive Committee of the American Uveitis Society. Am J Ophthalmol. 1994;117(5):663–7. 10. Blumenkranz MS, Culbertson WW, Clarkson JG, et al. Treatment of the acute retinal necrosis syndrome with intravenous acyclovir. Ophthalmology. 1986;93(3):296–300. 11. Ryan SJ.  Retina. 4th ed. St. Philadelphia: Elsevier Mosby; 2006. p. 1625–72. 12. Nussenblatt RB, Whitcup SM. Uveitis: fundamentals and clinical practice. 4th ed. St. Louis, MI: Elsevier Mosby; 2010. p. 161–75. 13. Freeman WR, Friedberg DN, Berry C, et  al. Risk factors for development of rhegmatogenous retinal detachment in patients with cytomegalovirus retinitis. Am J Ophthalmol. 1993;116(6):713–20. 14. Goldberg DE, Smithen LM, Angelilli A, et al. HIV-associated retinopathy in the HAART era. Retina. 2005;25(5):633–49. 15. Safneck JR.  Endophthalmitis: a review of recent trends. Saudi J Ophthalmol. 2012;26(2):181–9. 16. Steere AC, Malawista SE, Snydman DR, et al. Lyme arthritis: an epidemic of oligoarticular arthritis in children and adults in three connecticut communities. Arthritis Rheum. 1977;20(1):7–17. 17. Ai CH, Wen YX, Zhang YG, et  al. Epidemiological study on Lyme disease in Hailin of Heilongjiang. China Pub Health. 1987;6(2):82–5.

Uveitis and Retinal Detachment

10

Yao Huang, Nan Zhou, and Wenbin Wei

Abstract

Intermediate uveitis is an inflammatory and proliferative disease involving the pars plana, basal of vitreous body, peripheral retina, and choroid, which account for 4–15% of the total number of uveitis. Traction of the fibrous membrane can cause retinal detachment. Posterior uveitis is an inflammation of the choroid, retina, and vitreous body. It can be caused by a variety of causes, including trauma, systemic disease, infection, immune abnormalities, tumors, and some unknown factors. Corticosteroid therapy is effective in most patients.

10.1 Part 1: Intermediate Uveitis Intermediate uveitis is an inflammatory and proliferative disease involving pars plana, basal of vitreous body, peripheral retinal, and choroid, which accounts for 4–15% of the total number of uveitis. It is more common in men and adolescents under 25 years of age, and 70–80% is with binocular disease [1–3].

10.1.1 Clinical Manifestations The anterior segment manifestations are mild in patients with intermediate uveitis. There may have a few tiny gray keratic precipitates. Tyndall sign is positive. In the posterior lens space, there is a yellow “crispy” reticular membrane. The anterior vitreous body presents dusty, gray, or brownish opacity. Severe cases can present with vitreous hemorrhage or a large amount of white granular or patchy turbidity. Using binocular indirect ophthalmoscope with scleral indentation to exam the fundus. The surface of ora serrata

presents snowbank exudation. Cellular aggregates float predominantly in the inferior vitreous (snowballs). There is a tiny whitish exudate or vascular sheath near the peripheral vessels. In severe cases, there may be grayish-white fibrous membrane originating from pars plana and extending into the vitreous cavity, which may have new blood vessels. There are fractures in the fibrous membrane, which is often mistaken for retinal hole against the red fundus background. Traction of the fibrous membrane can cause retinal detachment. The main UBM image features of intermediate uveitis include (1) membranous vitreous opacities of different shapes and ranges in the pars plana and peripheral, (2) signs of vitreoretinal adhesions and traction, and (3) combination of mild ciliary body edema and/or ciliary body detachment (Figs. 10.1, 10.2, 10.3, 10.4, 10.5, and 10.6).

10.1.2 Diagnosis The diagnosis was mainly based on the examination of the peripheral fundus. With the application of three-mirror contact lens and binocular indirect ophthalmoscope with scleral indentation to exam peripheral retina, it presents snowball or snowbank exudation, membranous hyperplasia, and retinal vasculopathy. Other signs such as peripheral retinal vascular sheathing, peripheral neovascularization, mild anterior chamber inflammation, CME, posterior subcapsular cataract, band keratopathy, secondary glaucoma, epiretinal membrane, and exudative retinal detachment are included. UBM examination can early detect vitreoretinal adhesions and traction and can indicate the risk of retinal complications at early stage of the disease, especially in the case of refractive media opacity.

Y. Huang, M.D., Ph.D. · N. Zhou, M.D., Ph.D. · W. Wei, M.D., Ph.D. (*) Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China © Springer Nature Singapore Pte Ltd. and Beijing Science and Technology Press 2018 W. Wei (ed.), Atlas of Retinal Detachment, https://doi.org/10.1007/978-981-10-8231-3_10

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Fig. 10.1  The figure of UBM show the punctate opacities of vitreous base

a Fig. 10.2  Figure a and b show the mass opacities of vitreous base

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10  Uveitis and Retinal Detachment Fig. 10.3  The UBM image show the membranous opacities of vitreous base

Fig. 10.4  The UBM image of punctate combined with membranous opacities of vitreous base

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208 Fig. 10.5  UBM features of intermediate uveitis: vitreous and retinal traction and adhesion at ora serrata

Fig. 10.6  The UBM image show the mild ciliary effusion

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10.2 Part 2: Posterior Uveitis 10.2.1 Outlines Posterior uveitis is an inflammation of the choroid, retina, and vitreous body. It can be caused by a variety of causes, including trauma, systemic disease, infection, immune abnormalities, tumors, and some unknown factors [4, 5]. It can be a monocular or binocular attack. Specific types of posterior uveitis include acute posterior multifocal placoid pigment epitheliopathy (APMPPE), multiple evanescent white dot syndrome, toxoplasmosis, ocular histoplasmosis, birdshot chorioretinopathy, multifocal choroiditis, and serpiginous choroiditis. Occasionally, APMPPE can cause exudative retinal detachment. Symptoms: Sudden loss of visual acuity. Fundus signs: Depending on the severity of the inflammation, the patient has varying degrees of vitreous opacity. The choroid lesions of different sizes can appear in different parts of the fundus. Early lesions are white or gray white, and the boundaries are not clear. Sometimes they are slightly elevated (Figs. 10.7, 10.8, 10.9, and 10.10). Severe posterior uveitis can even cause exudative retinal detachment (Figs.  10.11, 10.12, and 10.13). In the recovery stage, secondary pigmented epithelium changes occur in the lesion area, and then an atrophic focus is formed. Long-term chronic inflammation can develop cystoid macular edema (Fig. 10.14). FFA signs: In acute stage, extensive leakage of fluorescein in the retinal veins and capillaries and fluorescein leakage of the optic disc (Figs. 10.15, 10.16, 10.17, and 10.18).

Fig. 10.8  Fundus of posterior uveitis. Male, 25 years old. The visual acuity decreased for 2 months. He is being treated for tuberculous pleurisy. The vitreous body of the right eye has opacity. There are yellowish-white exudations. The nasal peripheral fundus, nasal surrounding yellowish-white exudates

Fig. 10.7  Fundus of posterior uveitis. The optic disc bulge, the boundary is not clear, and there is subretinal yellowish-white nodule temporal to macula

In chronic stage, cystoid macular edema is manifested by petallike high fluorescence (Fig. 10.19). Pigment epithelial dissociation and change produce mottled or salt-and-pepperlike hyper- and hypofluorescence changes. Corticosteroid therapy is effective in most patients. If there are infectious factors, anti-infective treatment should be given at the same time. Immunosuppressive therapy may be considered for those who are not sensitive to corticosteroid therapy.

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Fig. 10.9  Fundus image of the patient of Fig. 10.8 after 1 month treatment. With the combination of corticosteroids and antituberculous therapy for 1 month, tuberculous pleurisy was not completely controlled, but the lesion in the nasal peripheral retina subsided, while new lesions appeared in the superior and superior temporal retina

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Fig. 10.10  Fundus image of posterior uveitis combining with retinal vasculitis and branch retinal vein occlusion

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Fig. 10.11  Fundus image of posterior uveitis. Optic disc edema and posterior choroid edema and exudation

Fig. 10.13  FFA image of posterior uveitis. Fluorescein leak present throughout the fundus. The posterior pole is the most significant. There is exudative retinal detachment inferiorly with laser spots around it

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Fig. 10.12  Fundus image of posterior uveitis combining with exudative retinal detachment in the right eye

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Fig. 10.14  Fundus image of posterior uveitis with cystoid macular edema

Fig. 10.16  FFA of posterior uveitis. The small vessels of the whole fundus are dilated and leaked. The peripheral fundus is most obvious and the choroid is focal hyperfluorescent

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Fig. 10.15  FFA of posterior uveitis. The optic disc is in high fluorescence and small retinal vessels around it present fluorescent leakage. The macular edema show fluorescence leakage

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Fig. 10.17  FFA of posterior uveitis of the right eye (venous phase). Significant fluorescence leakage at the posterior fundus

Fig. 10.19  FFA of posterior uveitis of the right eye (venous phase). Macular present petallike fluorescence accumulation

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Fig. 10.18  FFA of posterior uveitis of the left eye (venous phase). Significant fluorescence leakage at the posterior fundus

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10.2.2 Specific Types of Uveitis 1. Multiple evanescent white dot syndrome (MEWDS) is a self-limiting disease which occurs in healthy young people. It is often unilateral. In the initial stage of disease, the visual acuity mildly and moderately decreased. There are a large quantity of gray spots in the deep layer of the retina (Fig. 10.20). It was gradually absorbed after a week (Fig. 10.21). Vision acuity gradually returned to normal without treatment. 2. Punctate inner choroidopathy (PIC) disease usually occurs in healthy, young, and myopic women. It is often bilateral. There are scattered round yellow-white lesions in the retinal pigment epithelium and choroid. They are located in the posterior fundus, and the size is about 100– 500 μm. After the lesion subsided, it often left an obvious atrophic scar. It is often combined with choroidal ­neovascularization (Figs.  10.22 and 10.23). PIC has no vitreous inflammation and it rarely recurred. If there is no choroidal neovascularization, the prognosis is generally good. 3. Serpiginous choroiditis is a rare progressive choroidal inflammation, involving retinal pigment epithelium and choroidal capillary. The etiology is unknown. It occurs in healthy people, usually at 40–50 years of age. The lesions were not detected until they were involved in the macula. It is more common in Caucasians. Both eyes can be attacked successively. The fundus showed a geographic yellowish or gray lesion around the disc, with a clear

Fig. 10.20  Fundus of multiple evanescent white dot syndrome. During the active period, there are some gray spots in the deep layer of the retina

Fig. 10.21  Fundus of recovery period of the patient of Fig. 10.20. The white dots completely disappeared

boundary. Gray scar formed after healing, accompanied by varying degrees of fibrosis and with pigmentation on margin. New lesions usually occur on the margins of old lesions (Figs.  10.24 and 10.25). It can be complicated with optic papillitis, retinal vasculitis, and macular choroidal neovascularization membrane, etc. 4. Toxoplasmosis is caused by toxoplasma infection. The fundus showed inflammation of the retina and choroid. Inflammatory lesions can be large or small, round or oval, and may be multiple or single lesion. The local retina showed gray edema. The lesion subsided, and the atrophic spots remained. The lesion usually occurs in the posterior fundus and cause visual impairment. Neuropapillitis may also occur. 5. Ocular histoplasmosis syndrome is caused by infection with Histoplasma capsulatum. It occurs mostly in 20–50-year-old Caucasians. The clinical findings were similar to those of recurrent multifocal choroiditis. However, the vitreous body is clear, and the positive skin test of Histoplasma can be differentiated from the recurrent multifocal choroiditis. 6. Birdshot retinochoroidopathy frequently occurs in middle-aged and elderly women. It is often a binocular attack. The fundus shows cream-like lesions, with variable size, located in the deep retina or RPE layers, showing a radial distribution with binocular symmetry. Both the optic disc and macular are edema. Vitreous inflammation is obvious. The lesions in the recovery stage are depigmentation. Secondary optic atrophy and subretinal neovascularization can occur.

10  Uveitis and Retinal Detachment Fig. 10.22  Fundus image of punctate inner choroidopathy combining with macular neovascularization of the left eye. (a) Fundus image of the left eye. Scattered atrophic spots in the posterior fundus, subretinal gray membrane in the macular area with retinal hemorrhage around it. (b, c) FFA images of the left eye. In early phase (b), the macular choroidal neovascularization showed hyperfluorescence. The hyperfluorescence of macular choroidal neovascularization showed fluorescence leakage, and the atrophy spots showed fluorescence staining

Fig. 10.23  Fundus image of the right eye of the same patient of Fig. 10.22. (a) Fundus image of the right eye. Subretinal fibrous membrane and local retinal detachment in macular region. (b–d) Atrophic spots inferior and nasal to optic disc showed fluorescein staining, and the macular choroidal neovascularization showed fluorescence leakage

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10.3 Part 3: Panuveitis Panuveitis involves anterior and posterior uvea, including Vogt-Koyanagi-Harada syndrome, sympathetic ophthalmia, Behcet’s disease, sarcoidosis, and Lyme disease. The first two can cause exudative retinal detachment, and the latter two can cause traction retinal detachment.

10.3.1 Behcet’s Disease

Fig. 10.24  Fundus of serpiginous choroiditis. Geographic yellowish or gray lesion around the disc, with a clear boundary

Behcet’s disease is a multisystem occlusive vasculitis, involving uveitis, oral ulcers, and genital ulcers. Its pathogenesis is related to infection, autoimmunity, and genetic factors, but the etiology is still not clear. It occurs more commonly in the young and middle ages, mostly binocular [6]. Clinical manifestations: About 97.7% patients developed oral ulcers and healed spontaneously after 7–10 days, but recurrent attacks occurred. About 90.4% patients develop skin lesions including erythema, acne-like lesions, folliculitis, embolism phlebitis, and skin hypersensitivity. About 79.8% patients develop genital ulcers. About 78.6% patients have eye involvement [7]. Ocular manifestations: It usually occurs after 2–3 years of other lesions, presenting with bilateral sudden panuveitis, which is relieved within 2–4 weeks but occurs repeatedly. About 1/3 present hypopyon (Fig. 10.26). The fundus findings included vitritis, retinitis, retinal vasculitis, and retinal vascular occlusion (Figs. 10.27, 10.28, and 10.29). Natural history: Recurrent episodes of inflammation often lead to complications such as secondary glaucoma, complicated cataracts, atrophy of the retina and optic nerve (Figs.  10.30 and 10.31), retinal neovascularization, and related complications (tractional retinal detachment). It often leads to blindness.

Fig. 10.25  FFA image of patient of Fig.  10.24. The lesions showed transmitted fluorescence (window defect), fluorescence leakage was seen at the margin of the lesion inferior and superior of optic disc, and the optic disc was hyperfluorescent

Fig. 10.26  The anterior segment image of Behcet’s disease. Hypopyon and iris adhesion

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Diagnostic criteria: 1 . Recurrent oral ulcer 2. Two of the following four items: (a) Recurrent genital ulcer (b) Typical eye manifestations (c) Skin lesions (d) Positive skin allergy Corticosteroids and immunosuppressive agents are e­ ffective. The effect of early treatment is good (Figs. 10.32 and 10.33).

10.3.2 Sarcoidosis Fig. 10.27  Fundus image of Behcet’s disease. The color of the optic disc is pale, the artery is thin, and the inferior branch of the retinal artery is white-line like, accompanied by hard exudates and small macular hemorrhage

Fig. 10.28  The FFA image of the patient in Fig. 10.27. There is fluorescence leakage of the capillary throughout the fundus, peripheral small vascular occlusion, and a large area of capillary nonperfusion

Sarcoidosis is a multisystem granulomatous disease. The peak of incidence is 20-50 years, more women than men. There are racial differences, and the incidence of African Americans is highest [8].

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Fig. 10.29  Fundus image of Behcet’s disease of the left eye. The retinal vessels show a segmental white sheath

Fig. 10.30  Fundus image of old Behcet’s disease. The optic disc is pale, the retinal blood vessels are mostly white lines, and the macular area is atrophic, accompanied by pigmentation

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Fig. 10.31  The FFA image of the patient in Fig.  10.30. The retinal vessels around optic disc are very thin, the distal vessels are occluded, and there is large area of window defect hyperfluorescence at the posterior fundus

Clinical manifestation: Multisystem lesions include hilar lymph node enlargement, interstitial lung disease, and generalized lymphadenopathy. The incidence of ocular lesions was 25%, in which 2/3 was anterior uveitis, and the incidence of posterior uveitis was 14–30% [9, 10]. Fundus changes: These include vitreous snowball lesions, venous white sheath, wax droplet-like changes, deep yellow choroidal lesions and granulomas, serous retinal detachment, CNV, optic disc edema, optic disc neovascularization, and retrobulbar neuritis. Natural history: Acute sarcoidosis has an acute onset and is spontaneously relieved within 2 years. Chronic sarcoidosis is insidious and has disease duration of more than 2 years. Complications often occur, such as glaucoma, cataract, macular edema, and optic nerve atrophy. Vision prognosis is poor. Diagnosis: In addition to typical clinical presentation, biopsy is required to confirm the diagnosis. The histopathological features were non caseous necrotic granulomas. Glucocorticoid therapy is the main treatment. The treatment effect of acute sarcoidosis is good, and the treatment effect of chronic sarcoidosis is poor.

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Fig. 10.33  Fundus image after treatment of patient in Fig. 10.31. After a few days with glucocorticoid treatment, the yellow lesion gradually subsided, and the vision gradually recovered

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References 1. Abaño JM, Galvante PR, Siopongco P, Dans K, Lopez J. Review of epidemiology of uveitis in Asia: pattern of uveitis in a tertiary hospital in the Philippines. Ocul Immunol Inflamm. 2017;25(Suppl 1):S75–80. 2. Ayena KD, Vonor K, Santos MAK, Sounouvou I, OdoulamiYehouessi L, Diallo JW, Monteiro S, Nagbe YE, Balo K.  Epidemiological profile of patients with uveitis in Boko and Parakou, in northern Bénin. Med Sante Trop. 2017;27(3):315–8. 3. Annamalai R, Biswas J. Patterns of intermediate uveitis in children presenting at a tertiary Eye Care Center in South India. Middle East Afr J Ophthalmol. 2017;24(2):94–9. 4. Sève P, Cacoub P, Bodaghi B, Trad S, Sellam J, Bellocq D, Bielefeld P, Sène D, Kaplanski G, Monnet D, Brézin A, Weber M, Saadoun D, Chiquet C, Kodjikian L. Uveitis: diagnostic work-up. A literature review and recommendations from an expert committee. Autoimmun Rev. 2017. pii: S1568-9972(17)30260-4.

Y. Huang et al. 5. Lee JH, Mi H, Lim R, Ho SL, Lim WK, Teoh SC, Agrawal R. Ocular autoimmune systemic inflammatory infectious study  – report 3: posterior and panuveitis. Ocul Immunol Inflamm. 2017;9:1–10. 6. Merashli M, Eid RE. Uthman I, vol. 30. Curr Opin Rheumatol: A review of current management of vasculo-Behcet’s; 2018. p. 50–6. 7. Ishido T, Horita N, Takeuchi M, Kawagoe T, Shibuya E, Yamane T, Hayashi T, Meguro A, Ishido M, Minegishi K, Yoshimi R, Kirino Y, Kato S, Arimoto J, Ishigatsubo Y, Takeno M, Kurosawa M, Kaneko T, Mizuki N. Clinical manifestations of Behçet’s disease depending on sex and age: results from Japanese nationwide registration. Rheumatology (Oxford). 2017;56(11):1918–27. 8. Baughman RP, Field S, Costabel U, Crystal RG, Culver DA, Drent M, Judson MA, Wolff G. Sarcoidosis in America. Analysis based on health care use. Ann Am Thorac Soc. 2016;13(8):1244–52. 9. Kansal V, Dollin M.  Ocular involvement in sarcoidosis. CMAJ. 2017;189(16):609. 10. Pasadhika S, Rosenbaum JT. Ocular sarcoidosis. Clin Chest Med. 2015;36(4):669–83.

Ocular Tumor and Retinal Detachment

11

Jinqiong Zhou, Nan Zhou, and Wenbin Wei

Abstract

Optic disc melanocytoma is a rare, benign, dark brown- to black-colored tumor that usually occurs on or adjacent to the optic nerve head. Papillary capillary hemangioma is one possible manifestation of ocular capillary hemangioma and may occur sporadically or as a single or first manifestation of von Hippel-Lindau disease. Retinal hemangioma includes three kinds of diseases: retinal capillary hemangioma, retinal cavernous hemangioma, and retinal plexiform hemangioma. Retinoblastoma is the most common malignant tumor in eyes of infants. Choroidal hemangioma is a benign, relatively rare hamartomatous tumor. Choroidal melanoma is the most common primary malignant tumor in adults. Uveal tumors primarily which occur in the iris can also involve the ciliary body, such as iris melanoma, iris neurofibroma, iris nerve sheath tumor, iris cyst, etc. Choroidal melanoma can also involve the ciliary body. Metastatic carcinoma to the choroid is common, with the breast and the lung as frequent primary sites; secondary exudative retinal detachment could occur in the surface of the tumor.

11.1 Optic Disc Tumor 11.1.1 Optic Disc Melanocytoma Optic disc melanocytoma is a rare, benign, dark brown to black colored tumor that usually occurs on or adjacent to the optic nerve head. It is unilateral and mostly occurs in middleaged patients, without gender difference. Most tumors are asymptomatic and are found occasionally on physical examination. A few patients come to the office because of the visual symptoms related to neural or vascular compression or tumor necrosis. Melanocytoma is mostly located in temporal or inferior portion of the optic disc. Occasionally it can involve the entire optic disc and the adjacent retina. Severe vision loss can be present in a few patients with parts or hemiretinal vascular obstruction associated with optic disc neovascularization [1]. In that case, secondary retinal detachment may present (Fig. 11.1a–j). Clinical diagnosis can be given according to the fundus examination. For those patients with obvious visual dysfunction, detailed examination could be used for differential diagnosis, in case it is choroidal melanoma spread from the adjacent. Standard fundus photography has been used for follow-up to document any changes of the tumor. Optical coherence tomography (OCT) has been utilized to characterize optic disc melanocytoma, showing a gradual transition from normal retina to nodular tumor, and the mass displays a

J. Zhou, M.D., Ph.D. · N. Zhou, M.D., Ph.D. · W. Wei, M.D., Ph.D. (*) Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China © Springer Nature Singapore Pte Ltd. and Beijing Science and Technology Press 2018 W. Wei (ed.), Atlas of Retinal Detachment, https://doi.org/10.1007/978-981-10-8231-3_11

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Fig. 11.1 (a–j) Optic disc melanocytoma is a rare, benign, dark brown- to black-colored tumor that usually occurs on or adjacent to the optic nerve head

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bright anterior border layer with optically empty internal details. Visual field examination shows the expansion of the physiological blind spot, corresponding to the tumor. There are a few eyes which can be detected with quadrant visual field defect connected to the physiological blind spot. Color Doppler ultrasound can be used to detect whether there is a blood flow signal in the tumor. For FFA, no fluorescence leakage helps to identify the melanocytoma [2] (Fig. 11.2a, b). This intraocular tumor must be clinically differentiated from juxtapapillary uveal melanoma, juxtapapillary choroi-

dal nevus, hyperplasia or hypertrophy of the RPE, combined hamartoma of the retina and RPE, and adenoma of the RPE [3, 4]. In addition, it has been estimated that 1–2% of optic disc melanocytoma can undergo malignant transformation [3]. Except for those eyes with tumor involving the entire optic disc, or with secondary neovascularization, exudative retinal detachment seldom occurs. If it happens, choroidal melanoma or malignant transformation would be noticed.

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Fig. 11.2 (a, b) For FFA, no fluorescence leakage helps to identify the optic disc melanocytoma

11.1.2 Papillary Capillary Hemangioma Papillary capillary hemangioma is one possible manifestation of ocular capillary hemangioma, which may occur sporadically or as a single or first manifestation of von Hippel-Lindau disease [5]. Most patients with papillary capillary hemangioma are asymptomatic and are found only during routine physical examinations. Patients who come to doctors complaining with visual decrease are 15–40 years old mostly. Papillary capillary hemangioma of the optic disc can be divided into two types: endogenous and exogenous. Endogenous capillary hemangioma is relatively common, red or orange, with clear realm. It usually uplifts to the vitreous cavity, involving part or all of the optic disc. Sometimes it can also immerse the adjacent retina. Exogenous capillary hemangioma is often located in the deep of optic disc, without clear realm. It can go across the edge of the optic nerve and deep into the subretina, acting like the subretinal neovascularization (Fig. 11.3a–e). For fluorescein fundus angiography, the mass can be filled in the early artery stage, showing that the blood supply of hemangioma comes from not only the ciliary vascular system in front of the sclera sieve but also the central retinal artery branch at the surface of the optic nerve. At the late stage of angiography, strong fluorescence can be present in the mass and its surroundings, due to fluorescein leakage (Fig.  11.4a–e). Sometimes, capillary hemangioma of the optic disc also needs to be differentiated with other optic disc tumors (Fig. 11.5).

The course of the disease is usually progressive, and the visual prognosis is unfavorable. Loss of central vision is a substantial threat in patients with disc hemangioma, particularly those with a location on the temporal side. Central vision could be damaged by macular cystic edema secondary to circulatory disorder or exudative retinal detachment. As the tumors persist or enlarge, the vascular walls become incompetent, and leakage of blood components leads to macular edema, accumulation of lipid exudates, and serous retinal detachment at the posterior pole or inferior part. The unsatisfactory prognosis is influenced by difficulties in the early diagnosis of the lesion, an anatomic location of a lesion embedded within the retinal and nerve fiber layers, and the limited accessibility to therapeutic intervention. Treatment of papillary capillary hemangioma is one of the most hazardous conditions to manage. Techniques easily applicable to peripheral lesions, such as feeder vessel coagulation with a yellow dye laser, cryotherapy, and ruthenium-106 brachytherapy, are not an option for a papillary lesion. Argon laser coagulation is the most widely used approach for retinal angiomas, but the results are generally disappointing. Photodynamic therapy (PDT) with verteporfin is conformed to be with potential benefit to induce regression of the lesion by complete thrombosis of the neovascular compartment.

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Fig. 11.3 (a–e) Papillary capillary hemangioma

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Fig. 11.4 (a–e) For fluorescein fundus angiography, at the late stage of angiography, strong fluorescence can be present in the mass and its surroundings, due to fluorescein leakage

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11.2 Retinal Tumor 11.2.1 Retinal Hemangioma

Fig. 11.5  The optic disc tumors

Retinal hemangioma includes three kinds of diseases: retinal capillary hemangioma, retinal cavernous hemangioma, and retinal plexiform hemangioma. Among those three hemangiomas, retinal capillary hemangioma is the main cause of exudative retinal detachment. And retinal plexiform hemangioma is the congenital anomaly of retinal vascular without solid tumor, and seldom cause secondary retinal detachment, so does retinal cavernous hemangioma. Retinal capillary hemangioma is a benign capillary angiomatous hamartoma that occurs on the peripheral or juxtapapillary retina [6]. It can be an independent disease, called retinal angiomatosis (Von-Hippel disease). Or it can also be a part of the brain-retinal angiomatosis (von-Hippel-Lindau disease). Those two diseases cannot be distinguished with the retinal hemangioma. Brain MRI would be needed. In the early stage, hemangioma can be as small as the size of retinal microangioma, without leakage around the tumor. At this time, there are no significant nourishing blood vessels around the mass. Medical history or family history can help us to give the diagnosis. The typical hemangioma presents with tortuous and expanded nourishing blood vessels, with more or less exudation. Orange-red mass, tortuous and expanded blood vessels, and the consistency of tumor size and the degree of expansion of the nourishing blood vessels are the key signs for diagnosis. The secondary retinal detachment of retinal hemangioma often presents as exudative retinal detachment. As the destruction of the blood-retinal barrier at the site of the mass, membranous hyperplasia is common. Hence, exudative combined tractional retinal detachment can also present in some cases. Rhegmatogenous retinal detachment is rare (Fig. 11.6a–h). The treatment of retinal hemangioma is variable and depends on the size, location, and secondary effects. Laser photocoagulation or cryotherapy is the standard treatment for small- and medium-sized hemangioma (Fig.  11.7a–d). PDT for the treatment of peripheral retinal capillary hemangioma has shown effective results in terms of decreasing regional exudation from large lesions.

11.2.2 Retinoblastoma Retinoblastoma is the most common malignant tumor in the eyes of infants. It is a hereditary disease, occurs binocularly or monocularly, without gender differences.

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Fig. 11.6 (a–h) The secondary retinal detachment of retinal hemangioma often presents as exudative retinal detachment

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Fig. 11.7 (a–d) The small and medium-sized hemangioma

The most common signs of patients with retinoblastoma are yellow-white pupils (Fig. 11.8) or strabismus. Detailed examination of the fundus after the pupil dilation is strongly recommended. If the tumor fills up the vitreous cavity, or with secondary glaucoma, patients may complain with pain. Tumor can occur in any part of the fundus, which presents as white or slightly yellow-white round lesion. It is located in the retina, with retinal blood vessels at the surface. The border of the mass would be a little fuzzy but can be easily distinguished with the normal retina. The mass gradually grows, which presents as different sizes. Most eyes with retinoblastoma present as a single mass. But in a few eyes, more than one mass can also be detected, which is commonly present in binocular patients (Fig. 11.9a, b). Because of lack of tight adhesion between retinoblastoma cells, cells would shed and scatter in the vitreous as the mass grows, acting as endophthalmitis (Fig. 11.10). Tumor cells can also enter the anterior chamber, deposit in the bottom of the anterior chamber, and act as empyema. The sizes of the tumor cells were inconsistent but larger than the inflammatory substances. In some eyes, vitreous and ante-

Fig. 11.8  The most common signs of patients with retinoblastoma are yellow-white pupils

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Fig. 11.9 (a) The pathological section of retinoblastoma. (b) In a few eyes, more than one mass can also be detected, which commonly present in binocular patients

rior chamber hemorrhage can come out at the same time. In a few cases, the tumor grows along the subretinal space, called exogenous retinoblastoma. Through the retina on the surface of the mass, we can see the white or yellow-white uplift. For infants or young children, such performance reminds us the retinal detachment second to the retinoblastoma. For eyes suspected with retinoblastoma, patchy or granular calcification foci in obit X-ray may contribute to the diagnosis. B-scan ultrasound may also show positive findings.

Fig. 11.10  Retinoblastoma act as endophthalmitis

As a universally fatal cancer prior to the 1900s, retinoblastoma is now highly curable with 5-year patient survival rates that reach 97% in the western world [7]. Management of retinoblastoma includes intra-arterial chemotherapy and local treatments such as enucleation, ­external beam radiotherapy, laser photocoagulation, thermotherapy, and cryotherapy. However, most of these therapies include certain limitations and unwanted complications, such as, long-term toxicities, nephrotoxicity, or secondary cancers [8]. Retinal detachment second to retinoblastoma could be exudative and commonly occurs in those eyes which have massive tumor with invasion of nearly the entire retina. And rhegmatogenous retinal detachment could also be found in eyes after treatment of retinoblastoma, with the rate of 6% [9]. The etiology of rhegmatogenous retinal detachment could be atrophic hole because of rapid tumor and choroid necrosis or horseshoe tear because of vitreoretinal traction. Even in those eyes after successful treatment, some could develop severe proliferative vitreoretinopathy, associated with vitreous hemorrhage and tractional or rhegmatogenous retinal detachment. The management of secondary retinal detachment is challenging. Complete tumor control is the first concern. Intravitreal chemotherapy to ensure the regression of vitreous seed could be performed and followed with subretinal fluid drainage or pars plana vitrectomy. Enucleation may be required due to poor vision and inability to adequately monitor for tumor recurrence [10].

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11.3 Uveal Tumor 11.3.1 Ciliary Body Tumor Melanoma is the most common primary tumor in the ciliary body, followed by medulloepithelioma, benign or malignant epithelioma, cyst, astrocytoma, cycloplegia of the ciliary and retina, leiomyoma, and so on. Tumors primarily occur in the iris and can also involve the ciliary body, such as iris melanoma, iris neurofibroma, iris nerve sheath tumor, iris cyst, etc. Choroidal melanoma can also involve the ciliary body. Ciliary melanoma accounts for about 5–8% of uveal melanoma, with a little bit lower degree of malignancy than choroidal melanoma (85–90%) [11]. It presents as spherical or nodular lesion. If the lesion is located in the rear of the ciliary body, it may rupture into the vitreous cavity or extend to the choroid. The ciliary body can become hypertrophy because of tumor growth. If the lesion is located in the anterior part of the ciliary body, it can push the iris forward and block the anterior chamber angle, and the intraocular pressure would increase. As the tumor grows, the lens suspensory ligament

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can be involved, followed by refractive state change. In some eyes, it can also cause lens opacity, shifting or dislocation, ciliary congestion, secondary retinal detachment, and choroidal detachment (Fig. 11.11a, b). Because of the special location and usually no clinical symptoms when tumor is small, it is difficult to give a diagnosis at an early stage. In addition to ultrasound and MRI (Fig.  11.12a–c), ultrasound biomicroscopy (UBM) is an important method in the early diagnosis of ciliary body or iris tumor. UBM can be used to measure the thickness of the lesion and determine the range of the tumor. And it can also be used to observe the changes of the lesion in follow-up. The tumor can be detected by UBM in the early stage, before any other conventional examination. It is also important for the differential diagnosis of other anterior segment tumors, such as, to distinguish the iris neoplasms from primary ciliary body tumors involving the anterior chamber (Fig. 11.13a–c). For local ciliary body tumor, local excision is recommended. If the lesion is extensive and difficult to resect completely, enucleation would be an appropriate choice (Fig. 11.14a, b).

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Fig. 11.11 (a, b) The local ciliary body tumor, it cause lens opacity, shifting or dislocation

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Fig. 11.12 (a–c) In MRI, the mass presents as spherical, nodular, or mushroom-like lesion, hyperintense on T1WI, and hypointense on T2WI

Fig. 11.13 (a, b) The ciliary body tumor can be detected by UBM. (c) UBM image after local tumor resection

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Fig. 11.14 (a, b) Pathological specimens (a) and pathological sections (b) after local excision of tumor

11.3.2 Uveal Melanoma Choroidal melanoma is the most common primary malignant tumor in adults. It is a monocular disease, without significant difference between genders. But it occurs more in white than the other colors [12]. Small choroidal melanoma presents as uplifted nodular tumor with clear border. As the tumor grows, it can break through the Bruch’s membrane, acting as a characteristic mushroom-like mass (Fig. 11.15a–e). There is seldom secondary retinal detachment in the early stage or when the tumor has not yet penetrated the Bruch’s membrane (Fig. 11.16a, b). Once the Bruch’s membrane is broken, a large lump can be formed under the retina, followed with exudative retinal detachment (Fig.  11.17a, b). There is another type of choroidal melanoma which presents as diffuse growth, with intact Bruch’s membrane. And the retina is rarely involved. Fluorescein fundus angiography shows that at the beginning of the arterial phase, there are multiple fluorescent dots appearing on the surface of the tumor (multiple pinpoint leaks). These dots would immediately enhance and diffuse and present as irregularly scattered strong fluorescent spots. Sometimes the tortuous vascular can be showed in the center of the tumor (double circulation). In some parts of the tumor, it presents with fluorescence shading because of pigment hyperplasia or non-perfusion because of tumor necrosis. But

in contrast, the fluorescence can stay still in some parts of the tumor after more than 30–50  min, acting like the map of many lakes (Fig. 11.18a, b). Indocyanine green angiography (ICGA) is another important imaging method for the diagnosis of choroidal melanoma. The ICGA result varies according to the amount of pigment, tumor thickness, and blood vessels. Usually, high fluorescence would be present in those tumors with less pigment and more blood vessels. Normally, thicker tumors present higher fluorescence because of more blood vessels within the mass. Sometimes, the lesions also present as normal fluorescence or low fluorescence. When the pigmented melanoma is thick or with internal vessels, the appearance of ICGA would be similar with nonpigmented melanoma (Fig. 11.19). Ultrasound and MRI can contribute to the diagnosis (Figs.  11.20a, b and 11.21a, b). B-scan ultrasonography shows inhomogeneous echo, with choroidal depression and acoustic attenuation in the lesion would support choroidal melanoma. In MRI, the mass presents as spherical, nodular or mushroom-like lesion, hyperintense on T1WI, and hypointense on T2WI. Differential diagnosis with other choroidal tumors is important, such as nevus, hemangioma, metastases, etc. (Fig. 11.22a–c). Treatment options include enucleation, radiation therapy, laser, proton beam therapy, episcleral brachytherapy, transpupillary thermotherapy, local excision, etc. (Fig. 11.23a–e).

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Fig. 11.17 (a, b) Once the Bruch membrane is broken, a large lump can be formed under the retina, followed with exudative retinal detachment

11  Ocular Tumor and Retinal Detachment Fig. 11.18 (a, b) Color fundus images of choroidal melanoma and FFA images at different stages. The fluorescence can stay still after more than 9 min, acting like the map of many lakes

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Fig. 11.19  ICGA images of choroidal melanoma. The ICGA result varies according to the amount of pigment, tumor thickness, and blood vessels

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Fig. 11.20 (a, b) B-scan ultrasonography shows inhomogeneous echo, with choroidal depression and acoustic attenuation in the lesion would support choroidal melanoma

11  Ocular Tumor and Retinal Detachment Fig. 11.21 (a, b) In MRI, the mass presents as spherical, nodular or mushroom-like lesion, hyperintense on T1WI, and hypointense on T2WI

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Fig. 11.22 (a) Choroidal nevus. (b, c) RPE Hypertrophy

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Fig. 11.23 (a–e) Choroidal melanoma and after treatment with local resection

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11.3.3 Choroidal Hemangioma Choroidal hemangioma is a benign, relatively rare hamartomatous tumor. Typical hemangioma occurs in two forms: more frequently as a circumscribed isolated lesion and less commonly as a diffuse form associated with Sturge-Weber syndrome. Circumscribed choroidal hemangioma usually presents as a discrete, smooth, round or slightly oval, and orange-red mass. It mostly locates posterior to the equator, mainly in the macular and peripapillary region, with rather indistinct margins (Fig. 11.24a–c). Diffuse choroidal hemangiomas are usually a manifestation of Sturge-Weber syndrome, a congenital condition characterized by angiomatous malformations that t­ ypically involve the leptomeninges, the choroid and/or episclera in the eye, and the facial skin in the ipsilateral distribution of the ophthalmic and maxillary branches of the trigeminal nerve. Those patients with diffuse choroidal hemangioma are most likely to

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develop secondary retinal detachment with shifting of the subretinal fluid, stimulated by vascular endothelial growth factors and neovascularization [13]. Due to the fluid leakage secondary to the damage of the retinal pigment epithelium, anatomic and functional symptoms vary considerably and range from an asymptomatic lesion found incidentally to cases of severe vision loss. The associated chronic exudation strongly compromises visual acuity in some patients [14]. Usually, fluid leakage caused by hemangioma firstly appeared in the tumor surface. If the tumor is in the superior of the macular or with numerous liquid, it can affect the macular area quickly, or even present with secondary serous retinal detachment. When the liquid in the macular is not so much, it is easy to be misdiagnosed as central serous chorioretinopathy. Binocular indirect binocular ophthalmoscopy would help to distinguish those two diseases (Fig. 11.25a–h).

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Fig. 11.24 (a–c) Choroidal hemangioma mostly locates posterior to the equator, mainly in the macular and peripapillary region, with rather indistinct margins

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Fig. 11.25 (a–h) Choroidal hemangioma present with secondary serous retinal detachment

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Fluorescein fundus angiography and ultrasonography would be useful for identification. Differential diagnosis is critical when the hemangioma was large and does not appear with the typical orange-red color in the surface. Fluorescein angiography revealed abnormal vascular structure and rapid enhancement and leakage at the early stage (Fig.  11.26a). ICGA plays a very important role in the diagnosis of choroidal hemangiomas. In ICGA, the blood vessels of the hemangioma would be showed at the early stage and become clearer over time. It would fade gradually at the late stage

(Fig. 11.26b). B-scan showed uniform echo, without choroidal depression and acoustic attenuation in the lesion, would support hemangioma (Fig. 11.27). Argon laser photocoagulation, transpupillary thermotherapy, radiotherapy, anti-vascular endothelial growth factor, and photodynamic therapy can be used to treat choroidal hemangioma with localized retinal detachment. All these techniques have limited efficacy, recurrences are not uncommon, and the scar affects the foveola in central lesions [15].

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Fig. 11.26 (a) Fluorescein angiography revealed abnormal vascular structure and high fluorescence and leakages would gradually appear in the site of the lesion at the early stage. (b) In ICGA, the blood vessels

Fig. 11.27  B-scan showed uniform echo, without choroidal depression and acoustic attenuation in the lesion, would support hemangioma

of the hemangioma would be showed at the early stage and become clearer over time. It would fade gradually at the late stage

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11.3.4 Choroidal Metastases Metastatic carcinoma to the choroid is common, with the breast (47–49%) and the lung (14–21%) as frequent primary sites [16]. Cancer cell embolus is transferred along the blood. Because of the characteristics of the anatomy of the supplying arteries, short posterior ciliary arteries, choroid metastases account for the vast majority of metastases and less in the iris and ciliary body. In addition, the left carotid artery connects with the aortic arch directly, while the right carotid artery branch comes from innominate artery, and there are more chances for cancer cell embolus to go into the left eye; therefore, the rate of choroidal metastases of the left eye is much higher than the right eye. For eyes with choroidal metastases, yellow or yellowish white, flat-shaped uplifted plaques could be detected in the posterior pole. Those plaques present as different sizes, with blur border. There are yellow-white exudation, pigment spots, or hemorrhages at the edge of the lesion occasionally. Secondary exudative retinal detachment could occur in the

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surface of the tumor. The detached retina usually presents transparent, without moving (Fig. 11.28a–d). Fluorescein fundus angiography: in the arterial phase and early venous phase, the lesion presents as low fluorescence. After that, mottled high fluorescence would gradually appear in the site of the lesion. Brown patches on the surface of the tumor show low fluorescence during the whole process. If the tumor is secondary with exudative retinal detachment, moderate intensity of high fluorescence would be presented at the late stage around the lesion. Sometimes the performance of FFA can be atypical. High fluorescence would present as early as the arterial phase in some eyes. And for some eyes, FFA can be normal. ICGA: In the early stage (within 5 min), the lesion usually presents as diffuse low fluorescence, with the same size of the mass. Through the tumor, normal choroidal blood vessels could be detected. In the late stage (about 30  min), tumor blood vessels may be stained or leak mildly (Fig. 11.29a, b). Ocular ultrasonography and MRI are helpful in the diagnosis and differential diagnosis (Fig. 11.30a, b).

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Fig. 11.28 (a–c) For eyes with choroidal metastases, yellow or yellowish white, flat-shaped uplifted plaques could be detected in the posterior pole. Those plaques present as different sizes, with blur border. (d) The pathological section of metastatic carcinoma of choroid

11  Ocular Tumor and Retinal Detachment Fig. 11.29 (a, b) FFA: in the arterial phase and early venous phase, the lesion presents as low fluorescence. After that, mottled high fluorescence would gradually appear in the site of the lesion. Brown patches on the surface of the tumor show low fluorescence during the whole process

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Fig. 11.30 (a, b) B-scan ultrasonography shows no choroidal depression and acoustic attenuation in the lesion

11.3.5 Choroidal Osteoma Choroidal osteoma, also known as choroidal osteodystrophy, is a rare benign tumor. Of all those patients, 80–90% are young women without any other medical history19. About 75% cases present as unilateral disease. It occurs more common in Caucasian, less in colors. Until now, the etiology and pathogenesis of this disease is unclear. There are several hypotheses, including inflammation, trauma, endocrine abnormality, environmental affection, hereditary factors, bone sprouting, etc. Of all those hypotheses, the theory of bone sprouting is more commonly recognized. Choroidal osteomata are seen in the peripapillary and macular areas, which present as yellow-white mass. The striking features of a choroidal osteoma are well-defined mass, slightly elevated, and white-to-cream or orange lesion as observed by ophthalmoscopy. The surface of the mass is uneven, with varying degrees of pigmentation. High tissue density due to calcification can be observed by ultrasonography and computed tomography (CT). At the early stage, there is an absence of any symptoms. Choroidal osteomata usually grow slowly, but some patients can complain an acute decrease in visual acuity. The main causes of this sudden decrease in vision are a serous macular detachment (Fig. 11.31a, b) or a subretinal hemorrhage, with or without choroidal neovascularization (CNV).

In the early stage of fluorescein fundus angiography, the lesion presents as granular, plaque-like high fluorescence. Then the intensity of fluorescence would be increased gradually with unchanged morphology. This performance of angiography suggests the vascular plexus in the tumor combined with the atrophy of retinal pigment epithelial, vascular plexus comes from the choroidal capillary. They are different with the neovascular, without fluorescence leakage. When there is neovascularization on the tumor or the subretinal space, lobulated high fluorescence would be present in the early stage of angiography. In that case, the tissue would be stained in the late stage. The fluorescence can be covered if there is subretinal hemorrhage or pigmentation. In the early stage of indocyanine green angiography, the choroid at the site of the lesion shows low fluorescence due to the coverage of the mass. The surrounding choroidal vessels present as tortuosity as snakes. The lesion can maintain this low fluorescence until the late stage. But there are exceptions sometimes (Fig. 11.32). Ultrasonography and CT can help for the diagnosis (Fig. 11.33a–c). A definitive treatment for the retinal detachment associated with a choroidal osteoma has not been established. Photocoagulation, surgical removal of the CNV, photodynamic therapy, and transpupillary thermotherapy have been tried, with long-term vision preservation.

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Fig. 11.31 (a, b) Choroidal osteoma

Fig. 11.32  In the early stage of indocyanine green angiography, the choroid at the site of the lesion shows low fluorescence due to the coverage of the mass

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Fig. 11.33 (a–c) Ultrasonography and CT images of choroidal osteoma

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11.4 Orbital Tumor

References

Retinal detachment secondary to the orbital tumor is rare. However, sometimes the tumor behind the ocular can give some pressure and squeeze the rear wall of the eyeball. In that case, we can detect a bulb lifted into the vitreous cavity. Sometimes the retinal folds can lead us to misdiagnose it as retinal detachment. Ultrasound and other imaging examinations can help to confirm the diagnosis (Fig. 11.34).

1. Thanos A, Gilbert AL, Gragoudas ES.  Severe vision loss with optic disc neovascularization after hemiretinal vascular obstruction associated with optic disc melanocytoma. JAMA Ophthalmol. 2015;133(10):e151502. 2. Salvanos P, Utheim TP, MC M, Eide N, Bragadόttir R. Autofluorescence imaging in the differential diagnosis of optic disc melanocytoma. Acta Ophthalmol. 2015;93(5):476–80. 3. Shields JA, Demirci H, Mashayekhi A, Eagle RC Jr, Shields CL. Melanocytoma of the optic disk: a review. Surv Ophthalmol. 2006;51:93–104. 4. Krohn J, Kjersem B. Stereo fundus photography in the diagnosis of optic disc melanocytoma. Acta Ophthalmol. 2011;89:e533–4. 5. Schmidt-Erfurth UM, Kusserow C, Barbazetto IA, Laqua H. Benefits and complications of photodynamic therapy of papillary capillary hemangiomas. Ophthalmology. 2002;109(7):1256–66. 6. Kim HM, Park KH, Woo SJ. Massive exudative retinal detachment following photodynamic therapy and intravitreal bevacizumab injection in retinal capillary hemangioma. Korean J Ophthalmol. 2015;29(2):143–5. 7. Palioura S, Gobin YP, Brodie SE, Marr BP, Dunkel IJ, Abramson DH.  Ophthalmic artery chemosurgery for the management of retinoblastoma in eyes with extensive (>50%) retinal detachment. Pediatr Blood Cancer. 2012;59(5):859–64. 8. Ong SJ, Chao AN, Wong HF, Liou KL, Kao LY. Selective ophthalmic arterial injection of melphalan for intraocular retinoblastoma: a 4-year review. Jpn J Ophthalmol. 2015;59(2):109–17. 9. Lindner T, Langner S, Falke K, Walter U, Krüger PC, Pohlmann A, Zimpfer A, Stahnke T, Hadlich S, Guthoff R, Erbersdobler A, Niendorf T, Stachs O. Anatomic and pathological characterization of choroidal melanoma using multimodal imaging: what is practical, what is needed? Melanoma Res. 2015;25(3):252–8. 10. Anaya-Pava EJ, Saenz-Bocanegra CH, Flores-Trejo A, Castro Santana NA.  Diffuse choroidal hemangioma associated with exudative retinal detachment in a Sturge-Weber syndrome case: photodynamic therapy and intravitreous bevacizumab. Photodiagnosis Photodyn Ther. 2015;12(1):136–9. 11. Boixadera A, García-Arumí J, Martínez-Castillo V, Encinas JL, Elizalde J, Blanco-Mateos G, Caminal J, Capeans C, Armada F, Navea A, Olea JL. Prospective clinical trial evaluating the efficacy of photodynamic therapy for symptomatic circumscribed choroidal hemangioma. Ophthalmology. 2009;116(1):100–5. 12. Nugent R, Lee L, Kwan A.  Photodynamic therapy for diffuse choroidal hemangioma in a child with Sturge-Weber syndrome. J AAPOS. 2015;19(2):181–3. 13. Shields CL, Shields JA, Gross NE, Schwartz GP, Lally SE.  Survey of 520 eyes with uveal metastases. Ophthalmology. 1997;104:1265–76. 14. Freedman MI, Folk JC.  Metastatic tumors to the eye and orbit. Patient survival and clinical characteristics. Arch Ophthalmol. 1987;105:1215–9. 15. Inoue K, Numaga J, Kaji Y, Toda J, Kato S, Sakurai M, Ikeda M, Motoi N, Murakami T, Fujino Y. Bilateral choroidal metastases secondary to uterocervical carcinoma of the squamous cell type. Am J Ophthalmol. 2000;130(5):682–4. 16. Kubota-Taniai M, Oshitari T, Handa M, Baba T, Yotsukura J, Yamamoto S.  Long-term success of intravitreal bevacizumab for choroidal neovascularization associated with choroidal osteoma. Clin Ophthalmol. 2011;5:1051–5.

Fig. 11.34  Retinal folds secondary to the orbital tumor

Surgical Techniques of Rhegmatogenous Retinal Detachment and Complications After Surgeries

12

Haicheng She, Nan Zhou, and Wenbin Wei

Abstract

The main surgical procedures for the reattachment of rhegmatogenous retinal detachment (RRD) are scleral buckling and vitrectomy. Pneumatic retinopexy and Lincoff balloon are not widely used today. Cryopexy and photocoagulation may sometime be selected for very localized, peripheral RRD.  No matter which method is chosen, the key procedures are sealing retinal breaks and relief of vitreous traction. Therefore, a thorough evaluation of the location of the retinal breaks and vitreous status is crucial before the surgery.

The main surgical procedures for the reattachment of rhegmatogenous retinal detachment (RRD) are scleral buckling and vitrectomy. Pneumatic retinopexy and Lincoff balloon are not widely used today. Cryopexy and photocoagulation may sometime be selected for very localized,

peripheral RRD. No matter which method is chosen, the key procedures are sealing retinal breaks and relief of vitreous traction. Therefore, a thorough evaluation of the location of the retinal breaks and vitreous status is crucial before the surgery.

12.1 Evaluation Before the Surgery Most RRD cases may be treated by scleral buckling at early stages when PVR is not prominent. The most important factor is the location of retinal breaks. Therefore, finding all the retinal breaks is a crucial procedure to decide the choice of surgery and to ensure the success of it. One can follow the Lincoff rule [1, 2] (Fig. 12.1) to find retinal breaks. Some times when the breaks are not found, it is even possible to buckle the areas that are most likely to have retinal breaks and still have a good chance of success in retinal reattachment.

H. She, M.D., Ph.D. · N. Zhou, M.D., Ph.D. · W. Wei, M.D., Ph.D. (*) Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, China © Springer Nature Singapore Pte Ltd. and Beijing Science and Technology Press 2018 W. Wei (ed.), Atlas of Retinal Detachment, https://doi.org/10.1007/978-981-10-8231-3_12

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Fig. 12.1 (a) When the retinal detachment is superior nasal or temporal, the breaks usually (98%) lie close to the highest border of the retinal detachment, higher than the meridian of the highest border of the opposite side. (b) In superior or total retinal detachment, breaks are usually (93%) within 1.5 clock hours of the 12 o’clock. (c) In asymmetrical

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inferior retinal detachment, breaks are usually (95%) in the higher side of the detachment. (d) When the inferior detachment is bullous, the break is more likely to be superior, possibly connected with the detached area through a shallow peripheral pathway

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12.2 S  urgical Techniques of Scleral Buckle and Complications During the Surgical Procedure

surgery. For a small retinal break, marking one point on the sclera is enough. For a comparatively bigger retinal tear, three points may be marked on the sclera (Fig. 12.2).

12.2.1 Marking the Exact Location of Retinal Breaks

12.2.2 Cryopexy

Finding the exact location of the retinal breaks and marking it on the sclera properly are essential to the success of the

Proper cryopexy (Fig.  12.2f) may ensure adequate adhesion of the neural retina to RPE. Under binocular indirect

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Fig. 12.2 (a) Scleral depressor may leave a temporary mark on the sclera. (b) For a small retinal break, marking one point on the sclera is enough (left). For a comparatively bigger retinal tear, three points may be marked on the sclera (right). (c) Marking retinal dialysis. (d) In bullous retinal detachment, it is more difficult to mark the correct location of a retinal break. (e) The tip of a scleral depressor should be used to leave an indentation on the sclera, which marks the correct location of the retinal break (right). In the left image the indentation by the scleral depressor tip is posterior than the real location of the retinal break. (f) Illustration of treatment of retinal tears with cryotherapy. When retinal break is small, one freezing spot may cover the break. When retinal break is comparatively large, several freezing spots should be placed around it and confluent to each other. Avoid freezing the exposed RPE in large retinal tears because it may cause releasing of pigment from RPE. (g) A sclerotomy of 3 mm is

made at the drainage site. A forceps is used to separate the incision and at the same time gentle pressure is applied beside the incision, so that the choroid is herniated. (h) The choroidotomy is done using a 28G needle. Note the needle is almost parallel to or just has a very small acute angle with the sclera surface. (i) Circumferential scleral buckle. (j) Radial scleral buckle. (k) Figure a shows an adequately placed scleral buckle with firm support of the tear. In figure b, the buckle is placed posterior than the tear. Vitreous traction to the anterior of the tear is not released, and liquid from vitreous may still go into the subretinal space through the tear. In figure c, the buckle is placed anterior to the tear. Again, there is fluid communication between the subretinal space and the vitreous. (l) Scleral buckle may be combined with encircling. In the left image, a circumferential buckle leaves a fishmouth phenomenon, with subretinal fluid posterior to the buckle. In the right image, a radial buckle properly closes the tear

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ophthalmoscope, we may see the choroid and the sclera pressed closer to the detached retina by the cryoprobe. During cryopexy, the choroid changes from orange to yellow first, then white. Then the retina starts to change to white. Freezing should be stopped as soon as the retina changes its color. Excessive treatment may result in RPE dispersion, which may lead to PVR. When the retinal break is relatively big and the amount of freezing is large than for normal retinal breaks, epiretinal membrane may form after even in successful reattached cases. It also may result in excessive scar, at which margin, new retinal breaks may form.

12.2.3 Subretinal Fluid Drainage The following factors should be considered while choosing the drainage site: (1) To avoid retina damage, the ideal site for subretinal fluid drainage should be at the place where the retina is prominently elevated. (2) To avoid hemorrhage, avoid vortex vein and the area that has been frozen. (3) The incision is preferable to be at the inferior quadrant so that even when there is hemorrhage, the macula is less likely to be involved with sitting position. (4) Avoid drainage near large retinal tears to avoid vitreous incarceration. (5) Easy sclera access, that is, between lateral rectus and inferior rectus or between medial rectus and inferior rectus [2, 3]. The procedures are described in Fig. 12.2g, h.

12.2.4 Scleral Buckle The most commonly used buckling materials are hard silicone elements and soft silicone sponges. Different types of scleral buckle should be chosen according to the size, shape, and distribution of retinal breaks. The buckle can be sutured radially, circumferentially, or in combination with scleral encircling (Fig. 12.2i–l). Circumferential buckle is more suitable for multiple small holes that are located close to each other on the same latitude, retinal dialyses, or when the latitudinal length of a tear is longer than the longitudinal length. When the exact location of possible small retina holes is not sure, a circumferential buckle may be placed in the suspected 1 or 2 quadrants [3]. Radial buckle is more suitable for single horseshoe tears, especially when there is longitudinal retinal fold. A radial buckle may nicely release the traction to the anterior margin of the tear and reduce fishmouth phenomenon [3] (Fig. 12.2l).

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12.3 Surgical Techniques of Vitrectomy 12.3.1 Location of the Three Ports The scleral incision should be at 3.5–4 mm posterior to the corneal margin. The infusion port should be at the inferior temporal. While the other two ports are at about 2 and 10 o’clock. The angle between the upper two ports should be at least 120° but less than 170° to facilitate surgical procedures [4] (Fig. 12.3a).

12.3.2 Posterior Vitreous Detachment (PVD) If there is no PVD, after cutting the core vitreous and shaving off the vitreous before the disc, PVD should be induced using the cutter (Fig. 12.3b). When it is not sure whether the PVD is complete, or when it is difficult to induce PVD, triamcinolone may be used to stain the vitreous. It is easier to remove the remaining vitreous when it is visualized. When the posterior hyaloid is detached to the anterior of equator, it is better to remove the remaining vitreous by shaving to avoid traction to the vitreous base. Perfluorocarbon liquid maybe used to splint the retina while shaving the peripheral vitreous. After a complete vitrectomy, gas-fluid exchange is performed to drain the subretinal fluid. Retinal breaks and lattice degeneration are sealed using endo-photocoagulation, sometime with cryotherapy, either before or after gas-fluid exchange, depending on the location of retinal breaks. Then long-acting gas or silicone oil is chosen as endo-tamponade agent.

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Fig. 12.3 (a) The location of the three ports for vitrectomy. (b) While inducing PVD or peeling membranes, note to peel parallel to the retinal surface, not perpendicular. (c) Gas-fluid exchange. Perfluorocarbon liquid squeezes the subretinal fluid from posterior to the retinal tear, while

gas squeezes the subretinal fluid from anterior to the retinal tear. When all the subretinal fluid is drained out, the perfluorocarbon liquid is also drained through the flute needle

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12.4 C  omplications After Retinal Reattachment Surgeries 12.4.1 Proliferative Vitreo-Retinopathy (PVR) PVR is the most common cause of retinal re-detachment at late stage. It may occur after both scleral buckle and vitrectomy. In some cases, even when the primary retinal breaks are closed and retina is reattached, PVR still may occur, which may result in epi-macular membrane, new retinal breaks or reopening of primary breaks, and recurrence of retinal detachment.

cases if observed for long enough time. The interfacial surface tension between silicone oil and intraocular fluid is the main factor that sustains the integrity of silicone oil bubble and prevents emulsification. The factors that cause emulsification are:

12.4.2 Silicone Oil Emulsification

(a) Intraocular bleeding and inflammation that may change the physical property of intraocular fluid, which may decrease the interfacial surface tension. (b) Eye movements. (c) Viscosity of silicone oil may influence the threshold of emulsification. Silicone oil with higher viscosity tends to have a higher threshold for emulsification. (d) Pureness of silicone oil.

Silicone oil emulsification refers to the phenomenon that small droplets of silicone oil separate from silicone oil bubble after some time of tamponade. It may occur in 100% of

Emulsified silicone oil droplets may be at the interface of intraocular fluid or attached to the surface of intraocular structures (Fig. 12.4a–d).

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Fig. 12.4 (a–c) Show emulsified silicone oil droplets before the retina. (d) Shows large amount of emulsified silicone oil droplets in the anterior chamber

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12.4.3 Extrusion and Infection of Scleral Buckle Extrusion and infection are comparatively common complications after scleral buckle. The extrusion rates for silicone sponges are reported to be 3.4–24.4% [5–7] and for solid silicone bands are 0.0–1.2% [5, 8, 9]. The clinical manifestations are foreign-body sensation, conjunctiva congestion, purulent discharge, and scleral buckle extrusion (Fig.  12.5a, b). a

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The only treatment for this situation is removal of scleral buckle. There is a chance of recurrence of retinal detachment after scleral buckle removal [10].

12.4.4 Vitreo-Retinal Incarceration After Subretinal Fluid Drainage Subretinal fluid drainage during scleral buckle surgery may help reattachment of the retina. But it sometime may cause vitreo-retinal incarceration with new retinal breaks. Incarcerated retina and vitreous form radial folds, observed from indirect ophthalmoscope (Fig. 12.5c).

12.4.5 Anterior Segment Ischemia

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Fig. 12.5 (a, b) Infection of scleral buckle with conjunctival congestion and subconjunctival granuloma. (c) Incarceration of vitreous and retina. Fibrosis of the incarcerated vitreous (arrow) is surrounded by radial fold of detached retina

Anterior segment ischemia is a serious but rare complication of scleral buckle. It is supposed to be due to reduced perfusion from anterior ciliary artery and posterior ciliary artery. It usually presents 2–5 days after surgery, with anterior chamber inflammation and ischemia of the iris. In mild cases, there are minor cornea edema, Tyndall phenomenon, cells in the anterior chamber, abnormal pupillary reflex, and segmented iris atrophy (Fig.  12.6a). In serious cases, there are conjunctiva edema, cornea edema with cornea bulla, serious anterior chamber inflammation with hyphema, synechia, and iris atrophy (Fig.  12.6b). The intraocular pressure may be high in early stage but lower than normal in late stage and finally results in phthisis bulbi (Fig. 12.6c).

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12.4.6 Anterior Migration of Scleral Buckle Anterior migration of scleral buckle after the surgery without disinsertion of rectus muscles during the operation is a rare complication. The reasons for anterior migration of scleral buckle may be related to tight scleral encircling, improper position, and sutures that cause erosion of the insertion of rectus muscle (Fig. 12.7).

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Fig 12.7  Anterior migration and extrusion of a silicone band

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Fig. 12.6 (a) Anterior segment ischemia with cornea edema, rubeosis, synechia, cataract, and pigment before the anterior capsule. (b) Anterior segment ischemia with mixed congestion, cornea edema, cornea opacity, and rubeosis. (c) Anterior segment ischemia leads to phthisis bulbi with cornea edema and opacity

1. Lincoff H, Gieser R.  Finding the retinal hole. Arch Ophthalmol. 1971;85:565–9. 2. Lois N, Wang D, Wenbin W.  Complications of vitreo-retinal surgery. Beijing: People’s Military Surgeon Publishing House; 2015. 3. Wenbin W.  Tongren indirect ophthalmoscope. Beijing: People’s Medical Publishing House; 2014. 4. Wenbin W.  Manual of vitreoretinal surgery. Beijing: People’s Medical Publishing House; 2014. 5. Hilton GF, Wallyn RH.  The removal of scleral buckles. Arch Ophthalmol. 1978;96:2061–3. 6. Russo CE, Ruiz RS.  Silicone sponge rejection. Early and late complications in retinal detachment surgery. Arch Ophthalmol. 1971;85:647–50. 7. Theodossiadis G, Chatzoulis D, Patelis J, et  al. Extraocular observations in episcleral sponge implants. Ophthalmologica. 1975;171:439–50. 8. Roldan-Pallares M, del Castillo Sanz JL, Awad-El Susi S, et  al. Long-term complications of silicone and hydrogel explants in retinal reattachment surgery. Arch Ophthalmol. 1999;117:197–201. 9. Wiznia RA. Removal of solid silicone rubber exoplants after retinal detachment surgery. Am J Ophthalmol. 1983;95:495–7. 10. Wenbin W. The atlas and textbook of retinal detachment diagnosis and differential diagnosis. Beijing: Beijing Science & Technology Press; 2006.