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Atlas of Pediatric Surgery: Principles and Treatment
9783030292102, 9783030292119, 303029210X

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Ahmed H. Al-Salem

Table of contents :
Preface
Acknowledgments
Contents
1: Intravenous Fluids, Electrolytes, Blood, and Blood Products
1.1 Introduction
1.2 Indications for Intravenous Fluids
1.3 Maintenance Fluid Requirements
1.4 Fluid Deficit
1.5 Ongoing Fluid Losses
1.6 Fluid Therapy During the First Week of Life
1.7 Electrolytes Disturbances (Table 1.7)
1.7.1 Sodium
1.7.2 Potassium
1.7.3 Calcium
1.8 Blood and Blood Products Transfusion
1.9 Platelets Transfusion
1.9.1 Types of Platelets
1.9.1.1 Platelets Apheresis Pediatric Leucocyte Depleted (PAPLD)
1.9.1.2 Platelets Pooled in T-Sol Leucocyte Depleted
1.9.2 Dose of Platelets Transfusion
1.10 Fresh Frozen Plasma
1.11 Cryoprecipitate Transfusion
1.12 Complications of Blood and Platelets Transfusion
1.13 Albumin Transfusion
1.14 Factor VIII Transfusion
1.15 Granulocytes Transfusion
Further Reading
2: Nutrition for Infants and Children
2.1 Introduction
2.2 Important Terms to Be Remembered
2.3 Indications for Total Parenteral Nutrition
2.4 Goals and Characteristics of Parenteral Nutrition for Infants and Children
2.5 Caloric Requirements for Infants and Children
2.5.1 Carbohydrate (Dextrose)
2.5.2 Protein (Amino Acids)
2.5.3 Intralipids
2.5.4 Electrolytes, Vitamins and Minerals
2.5.5 Vitamins
2.6 Complications Associated with the Use of Parenteral Nutrition
Further Reading
3: Venous Access for Infants and Children
3.1 Introduction
3.2 Types of Catheters
3.3 Indications for Central Venous Catheters
3.4 Peripheral Catheters (Percutaneous Peripheral Catheters)
3.5 Umbilical Vascular Access
3.6 Peripheral Venous Cutdown Catheters
3.6.1 Intraosseous Catheters
3.6.2 Peripherally Inserted Central Catheters (PICC)
3.7 Central Venous Catheters (CVCs)
3.8 Implantable Vascular-Access Devices
3.9 Complications for Central Vascular Access
3.9.1 Long-Term Catheter Complications
Further Reading
4: Dermoid Cysts
4.1 Introduction
4.2 Head and Neck Dermoid Cysts
Further Reading
5: Sternocleidomastoid Tumor and Torticollis
5.1 Introduction
5.2 Etiology
5.3 Clinical Features
5.4 Treatment
Further Reading
6: Thyroglossal Cyst
6.1 Introduction
6.2 Embryology (Fig. 6.2)
6.3 Histology
6.4 Clinical Features
6.5 Investigations
6.6 Treatment
Further Reading
7: Branchial Cysts, Sinuses, and Fistulae
7.1 Introduction
7.2 Embryology
7.3 Branchial Grooves (Clefts) and Membranes
7.4 Branchial Remnants
7.5 Clinical Features
7.5.1 First Branchial Remnants
7.5.2 Second Branchial Remnants
7.5.3 Third and Fourth Branchial Remnants
7.5.4 Branchial Cystic Remnants
7.6 Branchial Fistulae Remnants
7.6.1 Treatment
7.6.2 Pathology
7.7 Congenital Midline Cervical Cleft
Further Reading
8: Infantile Fibromatosis
8.1 Introduction
8.2 Infantile Fibromatoses
8.3 Treatment
8.4 The Prognosis
8.5 Fibromatosis Colli
Further Reading
9: Epulis
9.1 Introduction
9.2 Treatment
Further Reading
10: Ranula
10.1 Introduction
10.2 Treatment
Further Reading
11: Abdominal Wall Defects
11.1 Introduction
11.2 Embryology (Figs. 11.1, 11.2, and 11.3)
11.3 Pathogenesis
11.4 Omphalocele (Fig. 11.14)
11.5 Gastroschisis (Fig. 11.15)
11.6 Hernia of the Umbilical Cord (Fig. 11.16)
11.7 Urachal Remnants and Omphalomesenteric Duct Malformations (Figs. 11.17, 11.18, and 11.19)
11.8 Bladder Exstrophy (Figs. 11.20, 11.21, and 11.22)
11.9 Prune-Belly Syndrome (Fig. 11.23)
11.10 Cloacal Exstrophy (Fig. 11.24)
Further Reading
12: Abdominal Wall Hernias and Hydroceles
12.1 Introduction: Abdominal Wall Hernias
12.2 Inguinal Hernia
12.3 Etiology
12.4 Clinical Features
12.5 Variants of Inguinal Hernia
12.5.1 Indirect Inguinal Hernia
12.5.2 Direct Inguinal Hernia
12.5.3 Littre’s Hernia
12.5.4 Sliding Inguinal Hernia
12.5.5 Richter’s Hernia
12.5.6 Busse’s Hernia
12.5.7 Maydl’s Hernia
12.5.8 Amyand’s Hernia
12.6 Complications of Inguinal Hernias
12.6.1 Incarceration
12.6.2 Strangulation
12.7 Treatment
12.8 Complications of Inguinal Herniotomy
12.9 Hydrocele
12.10 Embryology
12.11 Classification of Hydroceles (Fig. 12.20)
12.12 Treatment
12.13 Femoral Hernia
12.14 Etiology
12.15 Diagnosis
12.16 Treatment
12.17 Umbilical Hernia
12.18 Paraumbilical Hernia
12.19 Epigastric Hernias
12.20 Diastasis Recti
12.21 Congenital Lumbar Hernia
12.22 Spigelian Hernia
12.23 Incisional Hernias
12.24 Other Rare Abdominal Wall Hernias
Further Reading
13: Umbilical Abnormalities in Infants and Children
13.1 Introduction
13.2 Embryology
13.3 Abnormalities of the Umbilicus
13.4 Umbilical Granuloma
13.4.1 Treatment
13.5 Umbilical Polyp
13.6 Umbilical Infection (Omphalitis)
13.6.1 Treatment
13.7 Umbilical Hernia
13.7.1 Treatment
13.8 Omphalomesenteric Remnants
13.9 Urachal Remnants
Further Reading
14: Hemangiomas and Vascular Malformations
14.1 Introduction
14.2 Classification
14.3 Vascular Malformations
14.4 Hemangiomas
14.4.1 Clinical Features
14.5 Pathological and Developmental Changes in Hemangiomas
14.6 Complications
14.7 Investigations
14.8 Hepatic Hemangiomas
14.9 Treatment
14.10 Prognosis
Further Reading
15: Lymphangiomas
15.1 Introduction
15.2 Classifications
15.3 Lymphangioma Circumscriptum
15.4 Cavernous Lymphangioma
15.5 Cystic Hygroma
15.6 Sites
15.7 Etiology
15.8 Complications
15.9 Diagnosis
15.10 Treatment
Further Reading
16: Undescended Testes (Cryptorchidism)
16.1 Introduction
16.2 Embryology and Etiology
16.3 Classification of Abnormal Testes
16.4 Effects of Undescended Testes
16.5 Investigations
16.6 Treatment
16.7 Hormonal Treatment
16.8 Surgical Treatment
16.9 Nonpalpable Testis
16.10 Complications of Orchidopexy
Further Reading
17: Acute Scrotum
17.1 Introduction
17.2 Torsion of Testes
17.2.1 Introduction
17.2.2 Classification
17.2.3 Etiology
17.2.3.1 Intravaginal Torsion
17.2.3.2 Extravaginal Torsion
17.2.4 Effects of Torsion of Testes
17.2.5 Clinical Features
17.2.5.1 Intravaginal Torsion
17.2.5.2 Extravaginal Torsion
17.2.6 Investigations and Treatment
17.3 Torsion of the Testicular or Epididymal Appendage
17.3.1 Introduction
17.3.2 Embryology
17.3.3 Clinical Features
17.3.4 Investigations and Treatment
17.4 Epididymitis, Orchitis, and Epididymo-orchitis
17.4.1 Introduction
17.4.2 Etiology
17.4.3 Clinical Features
17.4.4 Investigations and Treatment
17.5 Idiopathic Scrotal Edema
17.6 Testicular Trauma
17.7 Other Causes of Acute Scrotum
17.8 Algorithm
Further Reading
18: Varicocele
18.1 Introduction
18.2 Etiology
18.3 Classification
18.4 Grading of Varicocele
18.5 Clinical Features
18.6 Investigations
18.7 Treatment
18.8 Complications of Varicocelectomy
Further Reading
19: Pediatric Abdominal and Thoracic Trauma
19.1 Introduction
19.2 Abdominal Trauma in Children
19.3 Thoracic Trauma in Children
19.4 Airway Injuries
19.5 Rib Fractures, Hemothorax, and Pneumothorax
19.6 Pulmonary Contusion (Figs. 19.17 and 19.18)
19.7 Diaphragm Rupture
Further Reading
20: Omphalocele
20.1 Introduction
20.2 Etiology
20.3 Classification
20.4 Associated Anomalies
20.5 Management and Outcome
20.6 Hernia of the Umbilical Cord
Further Reading
21: Gastroschisis
21.1 Introduction
21.2 Etiology
21.3 Diagnosis
21.4 Clinical Features
21.5 Associated Anomalies
21.6 Management and Outcome
Further Reading
22: Biliary Atresia
22.1 Introduction
22.2 Etiology
22.3 Associated Congenital Anomalies
22.4 Classification
22.5 Clinical Features
22.6 Investigations
22.7 Biliary Hypoplasia
22.8 Treatment
22.9 Complications and Outcome
Further Reading
23: Choledochal Cysts
23.1 Introduction
23.2 Etiology
23.3 Classification
23.4 Forme Fruste Choledochal Cyst
23.5 Clinical Features
23.6 Investigations
23.7 Treatment
Further Reading
24: Congenital Pancreatic Cyst
24.1 Introduction
24.2 Classification
24.3 Etiology
24.4 Clinical Features
24.5 Diagnosis
24.6 Treatment
Further Reading
25: Pancreatitis and Pancreatic Pseudocyst
25.1 Introduction
25.2 Etiology
25.3 Pancreatic Divisum
25.4 Pathophysiology
25.5 Clinical Features
25.6 Acute Hemorrhagic Pancreatitis
25.7 Investigations
25.8 Management
25.8.1 Medical Management
25.8.2 Surgical Management
25.9 Pancreatic Pseudocysts
25.9.1 Management of Pancreatic Pseudocysts
25.10 Prognosis of Acute Pancreatitis
Further Reading
26: Cholelithiasis and Choledocholithiasis
26.1 Introduction
26.2 Types of Gallstones
26.3 Etiology of Cholelithiasis
26.4 Complications of Cholelithiasis
26.5 Clinical Features
26.6 Investigations
26.7 Acute Cholecystitis (Fig. 26.17)
26.8 Cholangitis
26.9 Choledocholithiasis (Figs. 26.18 and 26.19)
26.10 Treatment
26.11 Postcholecystectomy Syndrome
Further Reading
27: Nesidioblastosis: Persistent Hyperinsulinemic Hypoglycemia of Infancy (PHHI)
27.1 Introduction
27.2 Etiology and Pathogenesis
27.3 Clinical Features
27.4 Investigations
27.5 Pathology
27.6 Histologically, congenital hyperinsulinism is divided into two types:
27.7 Management
Further Reading
28: The Spleen and Splenic Disorders
28.1 Introduction
28.2 Embryology
28.3 Functions of the Spleen
28.4 Disorders of the Spleen
28.4.1 Splenomegaly
28.4.2 Massive Splenomegaly
28.4.3 Splenic Rupture
28.4.4 Splenosis
28.4.5 Accessory Spleen
28.4.6 Asplenia
28.4.7 Hepatolienal Fusion
28.4.8 Polysplenia
28.4.9 Splenogonadal Fusion
28.4.9.1 Introduction
28.4.9.2 Classification
28.4.9.3 Etiology
28.4.9.4 Clinical Features
28.4.9.5 Associated Anomalies
28.4.9.6 Treatment
28.4.10 Splenorenal Fusion
28.4.11 Wandering Spleen
28.4.12 Splenic Cysts
28.4.13 Splenic Abscess
28.4.14 Massive Splenic Infarction
28.4.15 Congestive Splenomegaly
28.5 Immunizations and Splenectomy
28.6 Partial Splenectomy
Further Reading
29: Acute Appendicitis
29.1 Introduction
29.2 Pathophysiology
29.3 Classification and Staging of Acute Appendicitis
29.4 Clinical Features
29.5 Diagnosis
29.6 Management
29.6.1 Appendectomy
29.7 Complications of Acute Appendicitis
Further Reading
30: Minimal Invasive Surgery in the Pediatric Age Group
Further Reading
31: Congenital Lobar Emphysema
31.1 Introduction
31.2 Etiology
31.3 Pathophysiology
31.4 Sites
31.5 Clinical Features
31.6 Diagnosis
31.7 Treatment
Further Reading
32: Congenital Cystic Adenomatoid Malformation
32.1 Introduction
32.2 Etiology and Pathophysiological Changes
32.3 Classification
32.4 Clinical Features
32.5 Prognosis
32.6 Investigations
32.7 Treatment
Further Reading
33: Bronchopulmonary Sequestration
33.1 Introduction
33.2 Etiology and Pathophysiology
33.3 Classification
33.4 Diagnosis
33.5 Complications
33.6 Pathophysiology
33.7 Treatment and Prognosis
Further Reading
34: Bronchogenic Cyst
34.1 Introduction
34.2 Etiology
34.3 Sites
34.4 Clinical Features
34.5 Cyst-Related Complications
34.6 Diagnosis
34.7 Histopathological Features
34.8 Treatment
Further Reading
35: Meckel’s Diverticulum
35.1 Introduction
35.2 Embryology
35.3 Symptoms
35.4 Diagnosis
35.5 Treatment
Further Reading
36: Intussusception
36.1 Introduction
36.2 Etiology
36.3 Classification
36.4 Clinical Features
36.5 Diagnosis
36.6 Treatment
Further Reading
37: Congenital Diaphragmatic Hernia
37.1 Introduction
37.2 Etiology and Pathophysiology
37.3 Associated Anomalies
37.4 Clinical Features
37.5 Investigations
37.6 Treatment
37.7 Long-Term Outcomes and Prognosis
37.8 Agenesis of Diaphragm
37.9 Traumatic Diaphragmatic Hernia
Further Reading
38: Congenital Morgagni Hernia
38.1 Introduction
38.2 Embryology
38.3 Site
38.4 Herniated Viscera
38.5 Associated Anomalies
38.6 Clinical Features
38.7 Investigations
38.8 Treatment
Further Reading
39: Congenital Paraesophageal Hernia
39.1 Introduction
39.2 Anatomy and Pathophysiology
39.3 Etiology
39.4 Classification
39.5 Clinical Features
39.6 Diagnosis
39.7 Treatment
Further Reading
40: Eventration of Diaphragm
40.1 Introduction
40.2 Etiology and Classification
40.3 Clinical Features
40.4 Diagnosis
40.5 Management
Further Reading
41: Esophageal Atresia and Tracheoesophageal Fistula
41.1 Introduction
41.2 Classification
41.3 Associated Anomalies
41.4 Clinical Features
41.5 Diagnosis
41.6 Management
41.7 Esophageal Atresia Without Tracheoesophageal Fistula
41.8 Prognosis
41.9 Postoperative Complications
41.10 Esophageal Replacement
41.11 Congenital Short Esophagus
41.12 Congenital Esophageal Diverticulum
41.13 Foregut Duplications
41.14 Congenital Bronchopulmonary Foregut Malformations
41.15 Esophageal Duplications (Figs. 41.66, 41.67, and 41.68)
41.16 Laryngotracheoesophageal Cleft
Further Reading
42: Congenital Esophageal Stenosis
42.1 Introduction
42.2 Etiology and Embryogenesis
42.3 Classification
42.4 Clinical Features
42.5 Diagnosis
42.6 Treatment
Further Reading
43: H-Type Tracheoesophageal Fistula
43.1 Introduction
43.2 Embryology
43.3 Classification
43.4 Clinical Features
43.5 Diagnosis
43.6 Treatment
Further Reading
44: Achalasia
44.1 Introduction
44.2 Etiology
44.3 Clinical Features
44.4 Diagnosis
44.5 Management
44.6 Allgrove (AAA) Syndrome
Further Reading
45: Infantile Hypertrophic Pyloric Stenosis
45.1 Introduction
45.2 Etiology
45.3 Clinical Features
45.4 Investigations and Diagnosis
45.5 Management
Further Reading
46: Gastric Volvulus
46.1 Introduction
46.2 Etiology
46.3 Classification
46.4 Clinical Features
46.5 Diagnosis
46.6 Treatment
Further Reading
47: Congenital Gastric Outlet Obstruction
47.1 Introduction
47.2 Etiology
47.3 Classification
47.4 Clinical Features
47.5 Diagnosis
47.6 Treatment
47.7 Prognosis
Further Reading
48: Congenital Duodenal Obstruction
48.1 Introduction
48.2 Embryology and Etiology
48.3 Classification
48.4 Associated Anomalies
48.5 Clinical Features
48.6 Investigations and Diagnosis
48.7 Management
48.8 Postoperative Early and Long-Term Complications
Further Reading
49: Congenital Intestinal Atresia and Stenosis
49.1 Introduction
49.2 Jejunoileal Atresia and Stenosis
49.2.1 Introduction
49.2.2 Etiology and Pathogenesis
49.2.3 Classification
49.2.4 Associated Anomalies
49.2.5 Clinical Features
49.2.6 Investigations and Diagnosis
49.2.7 Treatment and Outcome
49.2.8 Short-Bowel Syndrome
49.3 Colonic Atresia and Stenosis
49.3.1 Introduction
49.3.2 Etiology
49.3.3 Classification
49.3.4 Associated Anomalies
49.3.5 Clinical Features
49.3.6 Diagnosis
49.3.7 Treatment
49.4 Chilaiditi Syndrome
Further Reading
50: Intestinal Malrotation
50.1 Introduction
50.2 Embryology
50.3 Associated Anomalies
50.4 Clinical Features
50.5 Investigations
50.6 Management and Prognosis
50.7 Complications
Further Reading
51: Gastrointestinal Duplications
51.1 Introduction
51.2 Etiology
51.3 Clinical Features
51.4 Investigations
51.5 Treatment
Further Reading
52: Necrotizing Enterocolitis
52.1 Introduction
52.2 Incidence of NEC
52.3 Pathology of NEC
52.4 Etiology and Pathogenesis of NEC
52.5 Clinical Features
52.6 Spontaneous Intestinal Perforation (SIP)
52.7 Stages of NEC
52.8 Diagnosis of NEC
52.9 Treatment
52.10 Operative Considerations
52.11 Outcome
Further Reading
53: Meconium Ileus
53.1 Introduction
53.2 Etiology
53.3 Classification
53.4 Clinical Features
53.5 Diagnosis
53.6 Management
53.7 Meconium Peritonitis
53.8 Meconium Ileus Equivalent
53.9 Complications Associated with Cystic Fibrosis and Meconium Ileus
Further Reading
54: Hirschsprung’s Disease
54.1 Introduction
54.2 Etiology and Pathogenesis
54.3 Associated Conditions
54.4 Clinical Features
54.5 Investigations and Diagnosis (Table 54.1)
54.6 Management
54.7 Hirschsprung’s Enterocolitis
54.8 Postoperative Complications and Outcome
Further Reading
55: Congenital Rectal Stenosis and Atresia
55.1 Introduction
55.2 Classification
55.3 Clinical Features
55.4 Diagnosis
55.5 Treatment
Further Reading
56: Congenital Segmental Dilatation of the Intestines
56.1 Introduction
56.2 Clinical Features
56.3 Associated Anomalies
56.4 Etiology
56.5 Sites
56.6 Diagnosis
56.7 Treatment
Further Reading
57: Meconium Plug Syndrome
57.1 Introduction
57.2 Clinical Features
57.3 Diagnosis
57.4 Management
Further Reading
58: Neonatal Small Left Colon Syndrome
58.1 Introduction
58.2 Etiology and Pathogenesis
58.3 Clinical Features
58.4 Investigations
58.5 Treatment and Outcome
Further Reading
59: Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (Berdon Syndrome)
59.1 Introduction
59.2 Etiology
59.3 Clinical Features
59.4 Associated Anomalies
59.5 Diagnosis
59.6 Treatment and Prognosis
Further Reading
60: Intestinal Polyps and Polyposis Syndromes
60.1 Introduction
60.2 Clinical Features
60.3 Classification of Intestinal Polyps and Polyposis Syndromes
60.4 Juvenile Polyps
60.5 Juvenile Polyposis Syndrome (JPS)
60.6 Peutz-Jeghers Syndrome (PJS)
60.7 Familial Adenomatous Polyposis (FAP)
60.8 Attenuated Familial Adenomatous Polyposis (AFAP)
60.9 mutY Homologue (MYH)-Associated Polyposis (MAP)
60.10 Gardner Syndrome
60.11 Turcot Syndrome
60.12 Cowden Syndrome
60.13 Bannayan-Zonana Syndrome (BRR Syndrome)
60.14 Cronkhite-Canada Syndrome
60.15 Hereditary-Mixed Polyposis Syndrome
60.16 Gorlin Syndrome (GS)
60.17 Lymphoid Polyposis
60.18 Ruvalcaba-Myhre-Smith Syndrome
Further Reading
61: Anorectal Malformations
61.1 Introduction
61.2 Anatomy and Embryology
61.3 Classifications
61.4 Krickenbeck Classification (2005)
61.5 The Most Common Malformations
61.6 Associated Malformations
61.7 Clinical Features
61.8 Investigations
61.9 Management
61.10 Outcome
61.11 The Pouch Colon Syndrome
Further Reading
62: Perianal Abscess and Fistula-in-Ano
62.1 Introduction
62.2 Anatomy
62.3 Classification
62.4 Etiology
62.5 Clinical Features
62.6 Treatment
Further Reading
63: Cloacal Anomalies
63.1 Introduction
63.2 Associated Anomalies
63.3 Classification
63.4 Clinical Features
63.5 Investigations
Further Reading
64: Cloacal Exstrophy
64.1 Introduction
64.2 Etiology and Pathogenesis
64.3 Associated Anomalies
64.4 Clinical Features and Management
Further Reading
65: Renal Tumors: Wilms Tumor (Nephroblastoma)
65.1 Introduction
65.2 Pathology
65.3 Renal Blastema and/or Nephroblastomatosis (Nephrogenic Rests)
65.4 Clinical Features
65.5 Investigations
65.6 Staging
65.7 Treatment (Table 65.1)
65.7.1 Management of Lung Metastasis
65.8 Surgical Considerations
65.8.1 Radical Nephrectomy
65.8.2 Partial Nephrectomy
65.8.3 Bilateral Wilms’ Tumor (Fig. 65.47)
65.9 Surgical Complications
65.10 Prognosis and Outcome (Table 65.2)
65.11 Extrarenal Wilms’ Tumors
65.12 Clear Cell Sarcoma of the Kidney
65.12.1 Treatment
65.12.2 Prognosis
65.13 Malignant Rhabdoid Tumor of the Kidney
65.13.1 Histology
65.13.2 Treatment
65.14 Mesoblastic Nephroma
65.14.1 Pathology
65.14.2 Treatment
Further Reading
66: Neuroblastoma
66.1 Introduction
66.2 Embryology and Anatomy
66.3 Cystic Neuroblastoma
66.4 Perinatal Neuroblastoma
66.5 Etiology, Chromosomal and Molecular Markers
66.6 Clinical Features
66.7 Diagnosis
66.8 Histology
66.9 Shimada Histopathologic Classification System
66.10 Staging
66.11 Screening
66.12 Treatment
66.12.1 Intermediate-Risk Group Treatment
66.12.2 High-Risk Group Treatment
66.13 Refractory and Relapsed Neuroblastoma
66.14 Complications and Prognosis
Further Reading
67: Liver Tumors
67.1 Introduction
67.2 Hepatoblastoma
67.2.1 Introduction
67.2.2 Etiology and Associated Anomalies
67.2.3 Histologic Classification of Hepatoblastoma
67.2.4 Clinical Features
67.2.5 Diagnosis
67.2.6 Staging
67.2.7 Management
67.2.8 Liver Transplantation
67.2.9 Surgical Considerations
67.3 Hepatocellular Carcinoma (HCC)
67.4 Hepatic Metastases
67.5 Other Primary Malignant Tumors of the Liver
67.6 Undifferentiated Embryonal Sarcoma
67.7 Infantile Choriocarcinoma of the Liver
67.8 Benign Liver Tumors
67.9 Hepatic Hemangiomas
67.9.1 Management
67.10 Hemangioendothelioma
67.11 Mesenchymal Hamartomas
67.12 Focal Nodular Hyperplasia
67.13 Hepatic Adenoma
Further Reading
68: Rhabdomyosarcoma
68.1 Introduction
68.2 Etiology
68.3 Histology
68.4 Classification
68.5 Staging
68.6 Clinical Features
68.7 Investigations
68.8 Risk Classification
68.9 Treatment
Further Reading
69: Lymphomas in Children
69.1 Introduction
69.2 Classifications
69.3 Hodgkin’s Lymphoma
69.3.1 Etiology and Pathophysiology
69.3.2 Clinical Features
69.3.3 Staging
69.3.4 Investigations
69.3.5 Histologic Classification of Hodgkin’s Disease
69.3.6 Management
69.4 Non-Hodgkin’s Lymphoma
69.4.1 Etiology
69.4.2 Burkitt Lymphoma
69.4.2.1 Clinical Features
69.4.3 Investigations
69.4.4 Management
69.4.5 Prognosis and Outcome
Further Reading
70: Ovarian Cysts and Tumors
70.1 Introduction
70.2 Classification
70.3 Ovarian Cysts in the Fetus
70.4 Diagnosis
70.5 Management and Outcome
70.6 Ovarian Cysts in Neonates
70.7 Management
70.8 Ovarian Cysts in Infants and Prepubertal Girls
70.9 Investigations
70.10 Management and Outcome (Fig. 70.12)
70.11 Ovarian Cysts in Adolescents
70.12 Management and Outcome
70.12.1 Follicular Cysts
70.12.2 Corpus Luteum Cysts
70.13 Ovarian Tumors
70.13.1 Introduction
70.13.2 Fibromas
70.13.3 Thecoma
70.13.4 Ovarian Cystadenoma
70.13.5 Germ Cell Tumors
70.13.6 Ovarian Teratoma
70.13.7 Epithelial Ovarian Tumors
70.13.8 Dysgerminoma
70.13.9 Clinical Manifestations of Ovarian Tumors
70.13.10 Investigations
70.13.11 Staging
70.13.12 Treatment
Further Reading
71: Thyroid Tumors in Children
71.1 Introduction
71.2 Etiology
71.3 Histopathology
71.4 Classification of Thyroid Tumors
71.5 Clinical Features
71.6 Diagnosis
71.7 Staging
71.8 Treatment
71.9 Differentiated Thyroid Carcinoma
71.10 Medullary Thyroid Carcinoma (MTC)
71.11 Prognosis
Further Reading
72: Testicular Tumors
72.1 Introduction
72.2 Classification
72.3 Histologic Classification of Seminomas
72.4 Etiology
72.5 Clinical Features
72.6 Investigations
72.7 Staging
72.8 Treatment
72.9 Yolk Sac Tumor
72.10 Teratoma
72.11 Mixed Germ Cell Tumor
72.12 Stromal Tumors
72.13 Testicular Cyst
72.14 Paratesticular Rhabdomyosarcoma
72.15 Prognosis and Outcome
Further Reading
73: Teratomas
73.1 Introduction
73.2 Sacrococcygeal Teratoma
73.2.1 Introduction
73.2.2 Pathogenesis
73.2.3 Classification
73.2.4 Histology
73.2.5 Clinical Features
73.2.6 Investigations
73.2.7 Treatment
73.2.8 Surgical Consideration
73.2.9 Fetal Intervention
73.2.10 Outcomes
73.2.11 Complications of Sacrococcygeal Teratoma
73.3 Gastric Teratoma
73.3.1 Clinical Features
73.3.2 Treatment
73.4 Ovarian Teratomas
73.4.1 Introduction
73.4.2 Staging of Malignant Teratoma
73.4.3 Clinical Features
73.4.4 Investigations
73.4.5 Histopathology
73.4.6 Treatment
73.5 Testicular Teratoma
73.5.1 Introduction
73.5.2 Clinical Features
73.5.3 Treatment
73.6 Intra-Abdominal Teratoma
73.7 Mediastinal Teratoma
73.7.1 Clinical Features
73.7.2 Investigations
73.7.3 Treatment
73.8 Cervical Teratoma
73.8.1 Introduction
73.8.2 Primary Thyroid Teratoma
73.8.3 Management
Further Reading
74: Persistent Müllerian Duct Syndrome (Hernia Uteri Inguinalis)
74.1 Introduction
74.2 Embryology and Etiology
74.3 Clinical Features
74.4 Management
74.5 Follow-Up
Further Reading
75: Hypospadias
75.1 Introduction
75.2 Embryology
75.3 Classification
75.4 Associated Anomalies
75.5 Etiology
75.6 Treatment
Further Reading
76: Epispadias and the Exstrophy-Epispadias Complex
76.1 Introduction
76.2 Embryology
76.3 Epispadias
76.3.1 Introduction
76.3.2 Etiology
76.3.3 Classification
76.3.4 Treatment
76.3.5 Female Epispadias
76.4 Bladder Exstrophy
76.4.1 Introduction
76.4.2 Radiological Evaluation
76.4.3 Management
Further Reading
77: Hydrocolpos, Vaginal Agenesis, and Atresia
77.1 Introduction
77.2 Vaginal Atresia
77.3 Classification
77.4 Associated Anomalies
77.5 Embryology
77.6 Clinical Features
77.7 Investigations
77.8 Management
Further Reading
78: Pelviureteric Junction Obstruction
78.1 Introduction
78.2 Embryology and Etiology
78.3 Clinical Features
78.4 Associated Anomalies
78.5 Investigations and Diagnosis
78.6 Treatment
Further Reading
79: Hydronephrosis in Infants and Children
79.1 Introduction
79.2 Classification
79.3 Pathophysiology
79.4 Etiology
79.5 Clinical Features
79.6 Investigations and Diagnosis
79.7 Treatment
Further Reading
80: Vesicoureteric Reflux
80.1 Introduction
80.2 Classification
80.3 Clinical Features
80.4 Diagnosis
80.5 Management
Further Reading
81: Posterior Urethral Valve
81.1 Introduction
81.2 Etiology and Pathophysiology
81.3 Classification
81.4 Diagnosis
81.5 Clinical Features
81.6 Management
Further Reading
82: Disorders of Sexual Development
82.1 Introduction
82.2 Embryology and Physiology of Sex Development
82.3 Classification
82.4 Classification of Disorders of Sexual Development (Fig. 82.9)
82.5 Evaluation of a Newborn with DSD
82.6 Diagnosis and Investigations
82.7 Management of Patients with DSD
82.8 Congenital Adrenal Hyperplasia (CAH)
82.9 Androgen Insensitivity Syndrome (Testicular Feminization Syndrome)
82.10 Partial (Incomplete) Androgen Insensitivity Syndrome
82.11 Deficiency of MIS (Persistent Müllerian Duct Syndrome)
82.12 Alpha-Reductase Deficiency
82.13 Gonadal Dysgenesis
82.14 Deficient Testosterone Biosynthesis
82.15 Ovotestis Disorders of Sexual Development
82.15.1 Presentation
82.15.2 Investigations
82.15.3 Management
82.16 Other Rare Disorders of Sexual Development
82.16.1 17β-Hydroxysteroid Dehydrogenase Deficiency
82.16.2 Aromatase Deficiency
82.16.3 Turner Syndrome
82.16.4 Klinefelter Syndrome
82.16.5 Triple X Syndrome
82.16.6 Aphallia
82.16.7 Diphallia
82.16.8 Micropenis
82.16.9 Uterus Didelphys
82.17 Algorithms
Further Reading
Index

Citation preview

Ahmed H. Al-Salem

Atlas of Pediatric Surgery Principles and Treatment

123

Atlas of Pediatric Surgery

Ahmed H. Al-Salem

Atlas of Pediatric Surgery Principles and Treatment

Ahmed H. Al-Salem Pediatric Surgery AlSadik Hospital Qatif Saudi Arabia

ISBN 978-3-030-29210-2 ISBN 978-3-030-29211-9 (eBook) https://doi.org/10.1007/978-3-030-29211-9 © Springer Nature Switzerland AG 2020 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, expressed 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 Switzerland AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland

Preface

Pediatric surgery is a rapidly growing specialty that has witnessed several advancements in the past 20 years such as minimally invasive surgery. This atlas, written in a simple, easy-to-read way, covers most areas in pediatric surgery and pediatric urology, with emphasis on the most important areas regarding patient presentation, diagnosis, and management. With clinical, operative, pathological, radiological, and hand-drawn images, this book should be useful to consultant pediatric surgeons, specialists, fellows, and residents, as well as general surgeons, accident and emergency doctors, pediatricians, neonatologists, general practitioners, trainees, medical students, and nurses. Qatif, Saudi Arabia

Ahmed H. Al-Salem

v

Acknowledgments

I would like to express special thanks and gratitude to my family who supported me and made this project possible. I would also like to thank all my patients and their families, as well as my colleagues and friends who supported and encouraged me.

vii

Contents

1 Intravenous Fluids, Electrolytes, Blood, and Blood Products �� 1 1.1 Introduction�� 1 1.2 Indications for Intravenous Fluids�� 1 1.3 Maintenance Fluid Requirements �� 1 1.4 Fluid Deficit�� 2 1.5 Ongoing Fluid Losses �� 2 1.6 Fluid Therapy During the First Week of Life �� 2 1.7 Electrolytes Disturbances�� 2 1.7.1 Sodium�� 2 1.7.2 Potassium�� 2 1.7.3 Calcium �� 3 1.8 Blood and Blood Products Transfusion�� 3 1.9 Platelets Transfusion �� 4 1.9.1 Types of Platelets�� 4 1.9.2 Dose of Platelets Transfusion �� 4 1.10 Fresh Frozen Plasma �� 4 1.11 Cryoprecipitate Transfusion�� 5 1.12 Complications of Blood and Platelets Transfusion�� 5 1.13 Albumin Transfusion�� 5 1.14 Factor VIII Transfusion�� 5 1.15 Granulocytes Transfusion �� 5 Further Reading �� 6 2 Nutrition for Infants and Children�� 7 2.1 Introduction�� 7 2.2 Important Terms to Be Remembered�� 7 2.3 Indications for Total Parenteral Nutrition �� 7 2.4 Goals and Characteristics of Parenteral Nutrition for Infants and Children �� 7 2.5 Caloric Requirements for Infants and Children�� 8 2.5.1 Carbohydrate (Dextrose)�� 8 2.5.2 Protein (Amino Acids)�� 8 2.5.3 Intralipids�� 8 2.5.4 Electrolytes, Vitamins and Minerals �� 8 2.5.5 Vitamins�� 9 2.6 Complications Associated with the Use of Parenteral Nutrition�� 9 Further Reading �� 9 3 Venous Access for Infants and Children�� 11 3.1 Introduction�� 11 3.2 Types of Catheters�� 11 3.3 Indications for Central Venous Catheters�� 11 3.4 Peripheral Catheters (Percutaneous Peripheral Catheters) �� 11 3.5 Umbilical Vascular Access�� 12 ix

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3.6 Peripheral Venous Cutdown Catheters�� 12 3.6.1 Intraosseous Catheters�� 12 3.6.2 Peripherally Inserted Central Catheters (PICC)�� 13 3.7 Central Venous Catheters (CVCs)�� 13 3.8 Implantable Vascular-Access Devices�� 15 3.9 Complications for Central Vascular Access�� 16 3.9.1 Long-Term Catheter Complications �� 17 Further Reading �� 17 4 Dermoid Cysts�� 19 4.1 Introduction�� 19 4.2 Head and Neck Dermoid Cysts�� 20 Further Reading �� 24 5 Sternocleidomastoid Tumor and Torticollis�� 25 5.1 Introduction�� 25 5.2 Etiology�� 26 5.3 Clinical Features �� 26 5.4 Treatment�� 28 Further Reading �� 28 6 Thyroglossal Cyst�� 29 6.1 Introduction�� 29 6.2 Embryology�� 29 6.3 Histology�� 30 6.4 Clinical Features �� 30 6.5 Investigations�� 31 6.6 Treatment�� 31 Further Reading �� 35 7 Branchial Cysts, Sinuses, and Fistulae�� 37 7.1 Introduction�� 37 7.2 Embryology�� 39 7.3 Branchial Grooves (Clefts) and Membranes�� 40 7.4 Branchial Remnants�� 40 7.5 Clinical Features �� 41 7.5.1 First Branchial Remnants�� 42 7.5.2 Second Branchial Remnants �� 42 7.5.3 Third and Fourth Branchial Remnants�� 43 7.5.4 Branchial Cystic Remnants�� 44 7.6 Branchial Fistulae Remnants�� 46 7.6.1 Treatment�� 46 7.6.2 Pathology�� 49 7.7 Congenital Midline Cervical Cleft�� 49 Further Reading �� 51 8 Infantile Fibromatosis�� 53 8.1 Introduction�� 53 8.2 Infantile Fibromatoses�� 54 8.3 Treatment�� 56 8.4 The Prognosis�� 58 8.5 Fibromatosis Colli�� 58 Further Reading �� 59

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9 Epulis�� 61 9.1 Introduction�� 61 9.2 Treatment�� 62 Further Reading �� 63 10 Ranula �� 65 10.1 Introduction�� 65 10.2 Treatment�� 66 Further Reading �� 67 11 Abdominal Wall Defects�� 69 11.1 Introduction�� 69 11.2 Embryology�� 69 11.3 Pathogenesis�� 73 11.4 Omphalocele �� 73 11.5 Gastroschisis �� 73 11.6 Hernia of the Umbilical Cord �� 74 11.7 Urachal Remnants and Omphalomesenteric Duct Malformations �� 74 11.8 Bladder Exstrophy�� 74 11.9 Prune-Belly Syndrome�� 76 11.10 Cloacal Exstrophy�� 76 Further Reading �� 77 12 Abdominal Wall Hernias and Hydroceles�� 79 12.1 Introduction: Abdominal Wall Hernias �� 79 12.2 Inguinal Hernia �� 79 12.3 Etiology�� 80 12.4 Clinical Features �� 81 12.5 Variants of Inguinal Hernia�� 82 12.5.1 Indirect Inguinal Hernia�� 82 12.5.2 Direct Inguinal Hernia�� 82 12.5.3 Littre’s Hernia�� 82 12.5.4 Sliding Inguinal Hernia �� 82 12.5.5 Richter’s Hernia�� 82 12.5.6 Busse’s Hernia �� 82 12.5.7 Maydl’s Hernia�� 82 12.5.8 Amyand’s Hernia�� 83 12.6 Complications of Inguinal Hernias �� 83 12.6.1 Incarceration�� 83 12.6.2 Strangulation�� 84 12.7 Treatment�� 84 12.8 Complications of Inguinal Herniotomy�� 85 12.9 Hydrocele�� 85 12.10 Embryology�� 85 12.11 Classification of Hydroceles �� 86 12.12 Treatment�� 87 12.13 Femoral Hernia �� 88 12.14 Etiology�� 89 12.15 Diagnosis�� 89 12.16 Treatment�� 89 12.17 Umbilical Hernia�� 89 12.18 Paraumbilical Hernia�� 91

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12.19 Epigastric Hernias�� 92 12.20 Diastasis Recti�� 92 12.21 Congenital Lumbar Hernia �� 92 12.22 Spigelian Hernia�� 94 12.23 Incisional Hernias �� 96 12.24 Other Rare Abdominal Wall Hernias�� 96 Further Reading �� 98 13 Umbilical Abnormalities in Infants and Children�� 99 13.1 Introduction�� 99 13.2 Embryology�� 99 13.3 Abnormalities of the Umbilicus�� 100 13.4 Umbilical Granuloma�� 100 13.4.1 Treatment�� 100 13.5 Umbilical Polyp�� 101 13.6 Umbilical Infection (Omphalitis)�� 101 13.6.1 Treatment�� 101 13.7 Umbilical Hernia�� 101 13.7.1 Treatment�� 102 13.8 Omphalomesenteric Remnants �� 103 13.9 Urachal Remnants�� 104 Further Reading �� 107 14 Hemangiomas and Vascular Malformations �� 109 14.1 Introduction�� 109 14.2 Classification�� 110 14.3 Vascular Malformations�� 112 14.4 Hemangiomas �� 113 14.4.1 Clinical Features�� 113 14.5 Pathological and Developmental Changes in Hemangiomas�� 115 14.6 Complications �� 115 14.7 Investigations�� 119 14.8 Hepatic Hemangiomas�� 119 14.9 Treatment�� 119 14.10 Prognosis�� 123 Further Reading �� 123 15 Lymphangiomas �� 125 15.1 Introduction�� 125 15.2 Classifications �� 125 15.3 Lymphangioma Circumscriptum�� 128 15.4 Cavernous Lymphangioma �� 128 15.5 Cystic Hygroma�� 129 15.6 Sites�� 129 15.7 Etiology�� 130 15.8 Complications �� 131 15.9 Diagnosis�� 131 15.10 Treatment�� 132 Further Reading �� 135 16 Undescended Testes (Cryptorchidism)�� 137 16.1 Introduction�� 137 16.2 Embryology and Etiology �� 138 16.3 Classification of Abnormal Testes�� 139

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16.4 Effects of Undescended Testes�� 140 16.5 Investigations�� 141 16.6 Treatment�� 142 16.7 Hormonal Treatment �� 142 16.8 Surgical Treatment�� 142 16.9 Nonpalpable Testis�� 143 16.10 Complications of Orchidopexy �� 144 Further Reading �� 145 17 Acute Scrotum�� 147 17.1 Introduction�� 147 17.2 Torsion of Testes �� 148 17.2.1 Introduction �� 148 17.2.2 Classification �� 148 17.2.3 Etiology �� 148 17.2.4 Effects of Torsion of Testes �� 149 17.2.5 Clinical Features�� 150 17.2.6 Investigations and Treatment�� 151 17.3 Torsion of the Testicular or Epididymal Appendage�� 153 17.3.1 Introduction �� 153 17.3.2 Embryology �� 154 17.3.3 Clinical Features�� 154 17.3.4 Investigations and Treatment�� 155 17.4 Epididymitis, Orchitis, and Epididymo-orchitis �� 156 17.4.1 Introduction �� 156 17.4.2 Etiology �� 156 17.4.3 Clinical Features�� 157 17.4.4 Investigations and Treatment�� 157 17.5 Idiopathic Scrotal Edema�� 158 17.6 Testicular Trauma�� 158 17.7 Other Causes of Acute Scrotum�� 158 17.8 Algorithm�� 159 Further Reading �� 160 18 Varicocele�� 161 18.1 Introduction�� 161 18.2 Etiology�� 161 18.3 Classification�� 162 18.4 Grading of Varicocele �� 162 18.5 Clinical Features �� 162 18.6 Investigations�� 162 18.7 Treatment�� 162 18.8 Complications of Varicocelectomy �� 163 Further Reading �� 163 19 Pediatric Abdominal and Thoracic Trauma�� 165 19.1 Introduction�� 165 19.2 Abdominal Trauma in Children�� 165 19.3 Thoracic Trauma in Children�� 169 19.4 Airway Injuries �� 170 19.5 Rib Fractures, Hemothorax, and Pneumothorax�� 170 19.6 Pulmonary Contusion�� 171 19.7 Diaphragm Rupture�� 172 Further Reading �� 176

xiv

20 Omphalocele �� 177 20.1 Introduction�� 177 20.2 Etiology�� 178 20.3 Classification�� 178 20.4 Associated Anomalies�� 179 20.5 Management and Outcome �� 181 20.6 Hernia of the Umbilical Cord �� 183 Further Reading �� 185 21 Gastroschisis �� 187 21.1 Introduction�� 187 21.2 Etiology�� 187 21.3 Diagnosis�� 188 21.4 Clinical Features �� 188 21.5 Associated Anomalies�� 188 21.6 Management and Outcome �� 189 Further Reading �� 192 22 Biliary Atresia�� 193 22.1 Introduction�� 193 22.2 Etiology�� 193 22.3 Associated Congenital Anomalies�� 194 22.4 Classification�� 195 22.5 Clinical Features �� 196 22.6 Investigations�� 197 22.7 Biliary Hypoplasia�� 200 22.8 Treatment�� 200 22.9 Complications and Outcome�� 203 Further Reading �� 204 23 Choledochal Cysts�� 205 23.1 Introduction�� 205 23.2 Etiology�� 205 23.3 Classification�� 205 23.4 Forme Fruste Choledochal Cyst�� 207 23.5 Clinical Features �� 208 23.6 Investigations�� 208 23.7 Treatment�� 209 Further Reading �� 214 24 Congenital Pancreatic Cyst �� 215 24.1 Introduction�� 215 24.2 Classification�� 215 24.3 Etiology�� 215 24.4 Clinical Features �� 216 24.5 Diagnosis�� 216 24.6 Treatment�� 217 Further Reading �� 220 25 Pancreatitis and Pancreatic Pseudocyst�� 221 25.1 Introduction�� 221 25.2 Etiology�� 221 25.3 Pancreatic Divisum �� 222 25.4 Pathophysiology�� 223 25.5 Clinical Features �� 223

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Contents

xv

25.6 Acute Hemorrhagic Pancreatitis �� 223 25.7 Investigations�� 224 25.8 Management�� 225 25.8.1 Medical Management�� 225 25.8.2 Surgical Management�� 225 25.9 Pancreatic Pseudocysts �� 225 25.9.1 Management of Pancreatic Pseudocysts�� 225 25.10 Prognosis of Acute Pancreatitis�� 227 Further Reading �� 227 26 Cholelithiasis and Choledocholithiasis�� 229 26.1 Introduction�� 229 26.2 Types of Gallstones�� 230 26.3 Etiology of Cholelithiasis �� 231 26.4 Complications of Cholelithiasis�� 231 26.5 Clinical Features �� 231 26.6 Investigations�� 231 26.7 Acute Cholecystitis �� 234 26.8 Cholangitis�� 234 26.9 Choledocholithiasis�� 235 26.10 Treatment�� 235 26.11 Postcholecystectomy Syndrome �� 236 Further Reading �� 236 27 Nesidioblastosis: Persistent Hyperinsulinemic Hypoglycemia of Infancy (PHHI)�� 237 27.1 Introduction�� 237 27.2 Etiology and Pathogenesis�� 237 27.3 Clinical Features �� 238 27.4 Investigations�� 238 27.5 Pathology�� 238 27.6 Histologically, congenital hyperinsulinism is divided into two types:�� 238 27.7 Management�� 239 Further Reading �� 240 28 The Spleen and Splenic Disorders�� 241 28.1 Introduction�� 241 28.2 Embryology�� 241 28.3 Functions of the Spleen�� 242 28.4 Disorders of the Spleen�� 242 28.4.1 Splenomegaly�� 242 28.4.2 Massive Splenomegaly�� 243 28.4.3 Splenic Rupture �� 243 28.4.4 Splenosis�� 243 28.4.5 Accessory Spleen�� 244 28.4.6 Asplenia�� 245 28.4.7 Hepatolienal Fusion�� 246 28.4.8 Polysplenia�� 246 28.4.9 Splenogonadal Fusion �� 246 28.4.10 Splenorenal Fusion�� 248 28.4.11 Wandering Spleen�� 248 28.4.12 Splenic Cysts �� 248 28.4.13 Splenic Abscess �� 250

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Contents

28.4.14 Massive Splenic Infarction�� 250 28.4.15 Congestive Splenomegaly �� 254 28.5 Immunizations and Splenectomy�� 254 28.6 Partial Splenectomy�� 254 Further Reading �� 256 29 Acute Appendicitis �� 257 29.1 Introduction�� 257 29.2 Pathophysiology�� 258 29.3 Classification and Staging of Acute Appendicitis�� 259 29.4 Clinical Features �� 259 29.5 Diagnosis�� 260 29.6 Management�� 261 29.6.1 Appendectomy�� 262 29.7 Complications of Acute Appendicitis �� 262 Further Reading �� 262 30 Minimal Invasive Surgery in the Pediatric Age Group�� 263 Further Reading �� 265 31 Congenital Lobar Emphysema �� 267 31.1 Introduction�� 267 31.2 Etiology�� 267 31.3 Pathophysiology�� 268 31.4 Sites�� 268 31.5 Clinical Features �� 268 31.6 Diagnosis�� 269 31.7 Treatment�� 271 Further Reading �� 272 32 Congenital Cystic Adenomatoid Malformation�� 273 32.1 Introduction�� 273 32.2 Etiology and Pathophysiological Changes�� 273 32.3 Classification�� 274 32.4 Clinical Features �� 276 32.5 Prognosis�� 277 32.6 Investigations�� 278 32.7 Treatment�� 279 Further Reading �� 281 33 Bronchopulmonary Sequestration �� 283 33.1 Introduction�� 283 33.2 Etiology and Pathophysiology�� 283 33.3 Classification�� 284 33.4 Diagnosis�� 286 33.5 Complications �� 286 33.6 Pathophysiology�� 286 33.7 Treatment and Prognosis�� 287 Further Reading �� 289 34 Bronchogenic Cyst �� 291 34.1 Introduction�� 291 34.2 Etiology�� 291 34.3 Sites�� 291 34.4 Clinical Features �� 293

Contents

xvii

34.5 Cyst-Related Complications �� 293 34.6 Diagnosis�� 294 34.7 Histopathological Features�� 295 34.8 Treatment�� 296 Further Reading �� 297 35 Meckel’s Diverticulum�� 299 35.1 Introduction�� 299 35.2 Embryology�� 299 35.3 Symptoms �� 301 35.4 Diagnosis�� 304 35.5 Treatment�� 304 Further Reading �� 305 36 Intussusception�� 307 36.1 Introduction�� 307 36.2 Etiology�� 307 36.3 Classification�� 308 36.4 Clinical Features �� 308 36.5 Diagnosis�� 309 36.6 Treatment�� 310 Further Reading �� 314 37 Congenital Diaphragmatic Hernia �� 315 37.1 Introduction�� 315 37.2 Etiology and Pathophysiology�� 317 37.3 Associated Anomalies�� 319 37.4 Clinical Features �� 319 37.5 Investigations�� 320 37.6 Treatment�� 321 37.7 Long-Term Outcomes and Prognosis�� 324 37.8 Agenesis of Diaphragm�� 325 37.9 Traumatic Diaphragmatic Hernia�� 327 Further Reading �� 330 38 Congenital Morgagni Hernia�� 331 38.1 Introduction�� 331 38.2 Embryology�� 331 38.3 Site�� 331 38.4 Herniated Viscera�� 332 38.5 Associated Anomalies�� 332 38.6 Clinical Features �� 333 38.7 Investigations�� 334 38.8 Treatment�� 335 Further Reading �� 339 39 Congenital Paraesophageal Hernia�� 341 39.1 Introduction�� 341 39.2 Anatomy and Pathophysiology �� 342 39.3 Etiology�� 345 39.4 Classification�� 345 39.5 Clinical Features �� 347 39.6 Diagnosis�� 348 39.7 Treatment�� 349 Further Reading �� 354

xviii

40 Eventration of Diaphragm�� 355 40.1 Introduction�� 355 40.2 Etiology and Classification �� 355 40.3 Clinical Features �� 356 40.4 Diagnosis�� 356 40.5 Management�� 357 Further Reading �� 359 41 Esophageal Atresia and Tracheoesophageal Fistula �� 361 41.1 Introduction�� 361 41.2 Classification�� 361 41.3 Associated Anomalies�� 361 41.4 Clinical Features �� 363 41.5 Diagnosis�� 364 41.6 Management�� 365 41.7 Esophageal Atresia Without Tracheoesophageal Fistula�� 368 41.8 Prognosis�� 371 41.9 Postoperative Complications�� 371 41.10 Esophageal Replacement�� 378 41.11 Congenital Short Esophagus �� 380 41.12 Congenital Esophageal Diverticulum �� 380 41.13 Foregut Duplications�� 382 41.14 Congenital Bronchopulmonary Foregut Malformations�� 385 41.15 Esophageal Duplications�� 387 41.16 Laryngotracheoesophageal Cleft�� 388 Further Reading �� 389 42 Congenital Esophageal Stenosis�� 391 42.1 Introduction�� 391 42.2 Etiology and Embryogenesis�� 392 42.3 Classification�� 392 42.4 Clinical Features �� 392 42.5 Diagnosis�� 394 42.6 Treatment�� 395 Further Reading �� 396 43 H-Type Tracheoesophageal Fistula�� 397 43.1 Introduction�� 397 43.2 Embryology�� 397 43.3 Classification�� 397 43.4 Clinical Features �� 398 43.5 Diagnosis�� 398 43.6 Treatment�� 399 Further Reading �� 400 44 Achalasia�� 401 44.1 Introduction�� 401 44.2 Etiology�� 401 44.3 Clinical Features �� 401 44.4 Diagnosis�� 403 44.5 Management�� 403 44.6 Allgrove (AAA) Syndrome�� 407 Further Reading �� 407

Contents

Contents

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45 Infantile Hypertrophic Pyloric Stenosis�� 409 45.1 Introduction�� 409 45.2 Etiology�� 409 45.3 Clinical Features �� 409 45.4 Investigations and Diagnosis�� 411 45.5 Management�� 413 Further Reading �� 416 46 Gastric Volvulus �� 417 46.1 Introduction�� 417 46.2 Etiology�� 417 46.3 Classification�� 417 46.4 Clinical Features �� 421 46.5 Diagnosis�� 421 46.6 Treatment�� 423 Further Reading �� 424 47 Congenital Gastric Outlet Obstruction�� 425 47.1 Introduction�� 425 47.2 Etiology�� 425 47.3 Classification�� 425 47.4 Clinical Features �� 426 47.5 Diagnosis�� 427 47.6 Treatment�� 430 47.7 Prognosis�� 431 Further Reading �� 432 48 Congenital Duodenal Obstruction�� 433 48.1 Introduction�� 433 48.2 Embryology and Etiology �� 433 48.3 Classification�� 434 48.4 Associated Anomalies�� 435 48.5 Clinical Features �� 438 48.6 Investigations and Diagnosis�� 438 48.7 Management�� 439 48.8 Postoperative Early and Long-Term Complications �� 441 Further Reading �� 443 49 Congenital Intestinal Atresia and Stenosis�� 445 49.1 Introduction�� 445 49.2 Jejunoileal Atresia and Stenosis�� 445 49.2.1 Introduction �� 445 49.2.2 Etiology and Pathogenesis�� 446 49.2.3 Classification �� 446 49.2.4 Associated Anomalies �� 448 49.2.5 Clinical Features�� 449 49.2.6 Investigations and Diagnosis �� 450 49.2.7 Treatment and Outcome�� 450 49.2.8 Short-Bowel Syndrome �� 456 49.3 Colonic Atresia and Stenosis�� 460 49.3.1 Introduction �� 460 49.3.2 Etiology �� 460 49.3.3 Classification �� 461

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49.3.4 Associated Anomalies �� 461 49.3.5 Clinical Features�� 461 49.3.6 Diagnosis �� 461 49.3.7 Treatment�� 462 49.4 Chilaiditi Syndrome�� 463 Further Reading �� 466 50 Intestinal Malrotation�� 467 50.1 Introduction�� 467 50.2 Embryology�� 468 50.3 Associated Anomalies�� 469 50.4 Clinical Features �� 470 50.5 Investigations�� 472 50.6 Management and Prognosis�� 476 50.7 Complications �� 478 Further Reading �� 480 51 Gastrointestinal Duplications �� 481 51.1 Introduction�� 481 51.2 Etiology�� 482 51.3 Clinical Features �� 482 51.4 Investigations�� 482 51.5 Treatment�� 482 Further Reading �� 484 52 Necrotizing Enterocolitis �� 485 52.1 Introduction�� 485 52.2 Incidence of NEC�� 485 52.3 Pathology of NEC �� 486 52.4 Etiology and Pathogenesis of NEC�� 487 52.5 Clinical Features �� 488 52.6 Spontaneous Intestinal Perforation (SIP)�� 489 52.7 Stages of NEC�� 489 52.8 Diagnosis of NEC �� 490 52.9 Treatment�� 493 52.10 Operative Considerations�� 496 52.11 Outcome�� 498 Further Reading �� 502 53 Meconium Ileus�� 503 53.1 Introduction�� 503 53.2 Etiology�� 504 53.3 Classification�� 505 53.4 Clinical Features �� 505 53.5 Diagnosis�� 506 53.6 Management�� 507 53.7 Meconium Peritonitis�� 512 53.8 Meconium Ileus Equivalent�� 513 53.9 Complications Associated with Cystic Fibrosis and Meconium Ileus�� 514 Further Reading �� 514 54 Hirschsprung’s Disease�� 515 54.1 Introduction�� 515 54.2 Etiology and Pathogenesis�� 516 54.3 Associated Conditions�� 517

Contents

xxi

54.4 Clinical Features �� 518 54.5 Investigations and Diagnosis�� 518 54.6 Management�� 521 54.7 Hirschsprung’s Enterocolitis�� 524 54.8 Postoperative Complications and Outcome�� 524 Further Reading �� 527 55 Congenital Rectal Stenosis and Atresia�� 529 55.1 Introduction�� 529 55.2 Classification�� 529 55.3 Clinical Features �� 530 55.4 Diagnosis�� 530 55.5 Treatment�� 531 Further Reading �� 534 56 Congenital Segmental Dilatation of the Intestines�� 535 56.1 Introduction�� 535 56.2 Clinical Features �� 535 56.3 Associated Anomalies�� 535 56.4 Etiology�� 536 56.5 Sites�� 537 56.6 Diagnosis�� 538 56.7 Treatment�� 540 Further Reading �� 540 57 Meconium Plug Syndrome�� 541 57.1 Introduction�� 541 57.2 Clinical Features �� 542 57.3 Diagnosis�� 542 57.4 Management�� 542 Further Reading �� 545 58 Neonatal Small Left Colon Syndrome�� 547 58.1 Introduction�� 547 58.2 Etiology and Pathogenesis�� 547 58.3 Clinical Features �� 548 58.4 Investigations�� 548 58.5 Treatment and Outcome�� 550 Further Reading �� 552 59 Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (Berdon Syndrome)�� 553 59.1 Introduction�� 553 59.2 Etiology�� 553 59.3 Clinical Features �� 554 59.4 Associated Anomalies�� 554 59.5 Diagnosis�� 554 59.6 Treatment and Prognosis�� 555 Further Reading �� 557 60 Intestinal Polyps and Polyposis Syndromes�� 559 60.1 Introduction�� 559 60.2 Clinical Features �� 560 60.3 Classification of Intestinal Polyps and Polyposis Syndromes�� 561 60.4 Juvenile Polyps �� 561

xxii

Contents

60.5 Juvenile Polyposis Syndrome (JPS)�� 563 60.6 Peutz-Jeghers Syndrome (PJS) �� 566 60.7 Familial Adenomatous Polyposis (FAP) �� 568 60.8 Attenuated Familial Adenomatous Polyposis (AFAP)�� 569 60.9 mutY Homologue (MYH)-Associated Polyposis (MAP)�� 569 60.10 Gardner Syndrome�� 569 60.11 Turcot Syndrome�� 570 60.12 Cowden Syndrome�� 570 60.13 Bannayan-Zonana Syndrome (BRR Syndrome)�� 570 60.14 Cronkhite-Canada Syndrome�� 570 60.15 Hereditary-Mixed Polyposis Syndrome�� 571 60.16 Gorlin Syndrome (GS)�� 571 60.17 Lymphoid Polyposis�� 571 60.18 Ruvalcaba-Myhre-Smith Syndrome �� 571 Further Reading �� 571 61 Anorectal Malformations�� 573 61.1 Introduction�� 573 61.2 Anatomy and Embryology�� 574 61.3 Classifications �� 574 61.4 Krickenbeck Classification (2005)�� 576 61.5 The Most Common Malformations�� 576 61.6 Associated Malformations�� 581 61.7 Clinical Features �� 583 61.8 Investigations�� 585 61.9 Management�� 589 61.10 Outcome�� 595 61.11 The Pouch Colon Syndrome �� 595 Further Reading �� 596 62 Perianal Abscess and Fistula-in-Ano�� 597 62.1 Introduction�� 597 62.2 Anatomy�� 597 62.3 Classification�� 598 62.4 Etiology�� 599 62.5 Clinical Features �� 599 62.6 Treatment�� 600 Further Reading �� 603 63 Cloacal Anomalies�� 605 63.1 Introduction�� 605 63.2 Associated Anomalies�� 606 63.3 Classification�� 606 63.4 Clinical Features �� 606 63.5 Investigations�� 607 Further Reading �� 610 64 Cloacal Exstrophy�� 611 64.1 Introduction�� 611 64.2 Etiology and Pathogenesis�� 611 64.3 Associated Anomalies�� 612 64.4 Clinical Features and Management�� 613 Further Reading �� 615

Contents

xxiii

65 Renal Tumors: Wilms Tumor (Nephroblastoma)�� 617 65.1 Introduction�� 617 65.2 Pathology�� 619 65.3 Renal Blastema and/or Nephroblastomatosis (Nephrogenic Rests) �� 620 65.4 Clinical Features �� 621 65.5 Investigations�� 624 65.6 Staging�� 628 65.7 Treatment�� 630 65.7.1 Management of Lung Metastasis�� 632 65.8 Surgical Considerations�� 633 65.8.1 Radical Nephrectomy�� 633 65.8.2 Partial Nephrectomy�� 633 65.8.3 Bilateral Wilms’ Tumor �� 633 65.9 Surgical Complications �� 634 65.10 Prognosis and Outcome�� 634 65.11 Extrarenal Wilms’ Tumors�� 635 65.12 Clear Cell Sarcoma of the Kidney�� 635 65.12.1 Treatment�� 636 65.12.2 Prognosis �� 636 65.13 Malignant Rhabdoid Tumor of the Kidney �� 636 65.13.1 Histology �� 637 65.13.2 Treatment�� 637 65.14 Mesoblastic Nephroma �� 638 65.14.1 Pathology �� 640 65.14.2 Treatment�� 641 Further Reading �� 642 66 Neuroblastoma �� 643 66.1 Introduction�� 643 66.2 Embryology and Anatomy�� 644 66.3 Cystic Neuroblastoma�� 645 66.4 Perinatal Neuroblastoma�� 645 66.5 Etiology, Chromosomal and Molecular Markers�� 646 66.6 Clinical Features �� 647 66.7 Diagnosis�� 649 66.8 Histology�� 651 66.9 Shimada Histopathologic Classification System�� 652 66.10 Staging�� 652 66.11 Screening�� 653 66.12 Treatment�� 653 66.12.1 Intermediate-Risk Group Treatment�� 655 66.12.2 High-Risk Group Treatment�� 655 66.13 Refractory and Relapsed Neuroblastoma�� 656 66.14 Complications and Prognosis�� 656 Further Reading �� 657 67 Liver Tumors�� 659 67.1 Introduction�� 659 67.2 Hepatoblastoma�� 659 67.2.1 Introduction �� 659 67.2.2 Etiology and Associated Anomalies�� 660 67.2.3 Histologic Classification of Hepatoblastoma�� 661

xxiv

Contents

67.2.4 Clinical Features�� 662 67.2.5 Diagnosis �� 663 67.2.6 Staging�� 666 67.2.7 Management�� 668 67.2.8 Liver Transplantation�� 673 67.2.9 Surgical Considerations�� 673 67.3 Hepatocellular Carcinoma (HCC)�� 674 67.4 Hepatic Metastases�� 675 67.5 Other Primary Malignant Tumors of the Liver �� 675 67.6 Undifferentiated Embryonal Sarcoma�� 675 67.7 Infantile Choriocarcinoma of the Liver�� 675 67.8 Benign Liver Tumors�� 675 67.9 Hepatic Hemangiomas�� 676 67.9.1 Management�� 676 67.10 Hemangioendothelioma�� 676 67.11 Mesenchymal Hamartomas�� 678 67.12 Focal Nodular Hyperplasia �� 680 67.13 Hepatic Adenoma�� 682 Further Reading �� 682 68 Rhabdomyosarcoma�� 683 68.1 Introduction�� 683 68.2 Etiology�� 684 68.3 Histology�� 685 68.4 Classification�� 687 68.5 Staging�� 687 68.6 Clinical Features �� 688 68.7 Investigations�� 688 68.8 Risk Classification�� 690 68.9 Treatment�� 690 Further Reading �� 692 69 Lymphomas in Children�� 693 69.1 Introduction�� 693 69.2 Classifications �� 693 69.3 Hodgkin’s Lymphoma�� 695 69.3.1 Etiology and Pathophysiology�� 695 69.3.2 Clinical Features�� 696 69.3.3 Staging�� 697 69.3.4 Investigations�� 699 69.3.5 Histologic Classification of Hodgkin’s Disease�� 699 69.3.6 Management�� 700 69.4 Non-Hodgkin’s Lymphoma�� 702 69.4.1 Etiology �� 702 69.4.2 Burkitt Lymphoma�� 703 69.4.3 Investigations�� 706 69.4.4 Management�� 708 69.4.5 Prognosis and Outcome�� 709 Further Reading �� 709 70 Ovarian Cysts and Tumors�� 711 70.1 Introduction�� 711 70.2 Classification�� 711 70.3 Ovarian Cysts in the Fetus�� 712

Contents

xxv

70.4 Diagnosis�� 713 70.5 Management and Outcome �� 713 70.6 Ovarian Cysts in Neonates�� 714 70.7 Management�� 714 70.8 Ovarian Cysts in Infants and Prepubertal Girls�� 714 70.9 Investigations�� 716 70.10 Management and Outcome �� 716 70.11 Ovarian Cysts in Adolescents �� 717 70.12 Management and Outcome �� 718 70.12.1 Follicular Cysts �� 718 70.12.2 Corpus Luteum Cysts �� 720 70.13 Ovarian Tumors�� 720 70.13.1 Introduction�� 720 70.13.2 Fibromas �� 721 70.13.3 Thecoma �� 721 70.13.4 Ovarian Cystadenoma�� 721 70.13.5 Germ Cell Tumors�� 722 70.13.6 Ovarian Teratoma�� 722 70.13.7 Epithelial Ovarian Tumors�� 723 70.13.8 Dysgerminoma�� 725 70.13.9 Clinical Manifestations of Ovarian Tumors�� 725 70.13.10 Investigations�� 725 70.13.11 Staging�� 726 70.13.12 Treatment�� 726 Further Reading �� 727 71 Thyroid Tumors in Children �� 729 71.1 Introduction�� 729 71.2 Etiology�� 730 71.3 Histopathology�� 731 71.4 Classification of Thyroid Tumors �� 732 71.5 Clinical Features �� 732 71.6 Diagnosis�� 733 71.7 Staging�� 733 71.8 Treatment�� 734 71.9 Differentiated Thyroid Carcinoma�� 734 71.10 Medullary Thyroid Carcinoma (MTC) �� 735 71.11 Prognosis�� 735 Further Reading �� 736 72 Testicular Tumors�� 737 72.1 Introduction�� 737 72.2 Classification�� 738 72.3 Histologic Classification of Seminomas �� 740 72.4 Etiology�� 740 72.5 Clinical Features �� 741 72.6 Investigations�� 742 72.7 Staging�� 742 72.8 Treatment�� 743 72.9 Yolk Sac Tumor�� 744 72.10 Teratoma �� 745 72.11 Mixed Germ Cell Tumor�� 745 72.12 Stromal Tumors�� 745 72.13 Testicular Cyst�� 746

xxvi

Contents

72.14 Paratesticular Rhabdomyosarcoma �� 747 72.15 Prognosis and Outcome�� 747 Further Reading �� 747 73 Teratomas �� 749 73.1 Introduction�� 749 73.2 Sacrococcygeal Teratoma�� 752 73.2.1 Introduction�� 752 73.2.2 Pathogenesis�� 753 73.2.3 Classification�� 753 73.2.4 Histology�� 754 73.2.5 Clinical Features �� 754 73.2.6 Investigations�� 756 73.2.7 Treatment�� 757 73.2.8 Surgical Consideration�� 757 73.2.9 Fetal Intervention�� 758 73.2.10 Outcomes�� 760 73.2.11 Complications of Sacrococcygeal Teratoma�� 760 73.3 Gastric Teratoma �� 760 73.3.1 Clinical Features �� 761 73.3.2 Treatment�� 761 73.4 Ovarian Teratomas�� 761 73.4.1 Introduction�� 761 73.4.2 Staging of Malignant Teratoma�� 763 73.4.3 Clinical Features �� 764 73.4.4 Investigations�� 766 73.4.5 Histopathology�� 766 73.4.6 Treatment�� 767 73.5 Testicular Teratoma�� 767 73.5.1 Introduction�� 767 73.5.2 Clinical Features �� 767 73.5.3 Treatment�� 767 73.6 Intra-Abdominal Teratoma�� 768 73.7 Mediastinal Teratoma�� 768 73.7.1 Clinical Features �� 768 73.7.2 Investigations�� 769 73.7.3 Treatment�� 769 73.8 Cervical Teratoma �� 769 73.8.1 Introduction�� 769 73.8.2 Primary Thyroid Teratoma�� 771 73.8.3 Management �� 771 Further Reading �� 772 74 Persistent Müllerian Duct Syndrome (Hernia Uteri Inguinalis) �� 773 74.1 Introduction�� 773 74.2 Embryology and Etiology �� 774 74.3 Clinical Features �� 774 74.4 Management�� 775 74.5 Follow-Up �� 775 Further Reading �� 776 75 Hypospadias�� 777 75.1 Introduction�� 777 75.2 Embryology�� 778

Contents

xxvii

75.3 Classification�� 780 75.4 Associated Anomalies�� 783 75.5 Etiology�� 783 75.6 Treatment�� 785 Further Reading �� 794 76 Epispadias and the Exstrophy-Epispadias Complex�� 795 76.1 Introduction�� 795 76.2 Embryology�� 795 76.3 Epispadias �� 795 76.3.1 Introduction �� 795 76.3.2 Etiology �� 796 76.3.3 Classification �� 796 76.3.4 Treatment�� 797 76.3.5 Female Epispadias �� 798 76.4 Bladder Exstrophy�� 799 76.4.1 Introduction �� 799 76.4.2 Radiological Evaluation�� 801 76.4.3 Management�� 801 Further Reading �� 803 77 Hydrocolpos, Vaginal Agenesis, and Atresia�� 805 77.1 Introduction�� 805 77.2 Vaginal Atresia�� 805 77.3 Classification�� 807 77.4 Associated Anomalies�� 807 77.5 Embryology�� 807 77.6 Clinical Features �� 808 77.7 Investigations�� 810 77.8 Management�� 812 Further Reading �� 817 78 Pelviureteric Junction Obstruction�� 819 78.1 Introduction�� 819 78.2 Embryology and Etiology �� 819 78.3 Clinical Features �� 821 78.4 Associated Anomalies�� 821 78.5 Investigations and Diagnosis�� 821 78.6 Treatment�� 823 Further Reading �� 827 79 Hydronephrosis in Infants and Children�� 829 79.1 Introduction�� 829 79.2 Classification�� 829 79.3 Pathophysiology�� 829 79.4 Etiology�� 831 79.5 Clinical Features �� 833 79.6 Investigations and Diagnosis�� 833 79.7 Treatment�� 837 Further Reading �� 840 80 Vesicoureteric Reflux �� 841 80.1 Introduction�� 841 80.2 Classification�� 841 80.3 Clinical Features �� 845

xxviii

Contents

80.4 Diagnosis�� 847 80.5 Management�� 848 Further Reading �� 853 81 Posterior Urethral Valve�� 855 81.1 Introduction�� 855 81.2 Etiology and Pathophysiology�� 855 81.3 Classification�� 857 81.4 Diagnosis�� 857 81.5 Clinical Features �� 858 81.6 Management�� 859 Further Reading �� 861 82 Disorders of Sexual Development�� 863 82.1 Introduction�� 863 82.2 Embryology and Physiology of Sex Development �� 865 82.3 Classification�� 866 82.4 Classification of Disorders of Sexual Development �� 869 82.5 Evaluation of a Newborn with DSD �� 870 82.6 Diagnosis and Investigations�� 872 82.7 Management of Patients with DSD�� 873 82.8 Congenital Adrenal Hyperplasia (CAH)�� 874 82.9 Androgen Insensitivity Syndrome (Testicular Feminization Syndrome) �� 876 82.10 Partial (Incomplete) Androgen Insensitivity Syndrome�� 878 82.11 Deficiency of MIS (Persistent Müllerian Duct Syndrome)�� 879 82.12 Alpha-Reductase Deficiency�� 880 82.13 Gonadal Dysgenesis�� 881 82.14 Deficient Testosterone Biosynthesis �� 882 82.15 Ovotestis Disorders of Sexual Development�� 882 82.15.1 Presentation �� 882 82.15.2 Investigations�� 883 82.15.3 Management�� 884 82.16 Other Rare Disorders of Sexual Development�� 885 82.16.1 17β-Hydroxysteroid Dehydrogenase Deficiency�� 885 82.16.2 Aromatase Deficiency�� 885 82.16.3 Turner Syndrome �� 885 82.16.4 Klinefelter Syndrome�� 885 82.16.5 Triple X Syndrome�� 885 82.16.6 Aphallia �� 886 82.16.7 Diphallia�� 886 82.16.8 Micropenis�� 886 82.16.9 Uterus Didelphys �� 886 82.17 Algorithms�� 887 Further Reading �� 887 Index�� 889

1

Intravenous Fluids, Electrolytes, Blood, and Blood Products

1.1

Introduction

• The enteral route should be used whenever possible to give fluids and nutrients for infants and children. • The use of intravenous fluids, however, becomes necessary to correct fluid- and electrolyte-imbalance and in those who are kept nil by mouth. • Fluid- and electrolyte-management of pediatric surgical patients represents an important aspect of their medical care. • An understanding of the physiology of fluids and electrolytes requirements is essential for the care of infants and children. • It is also important to keep infants and children on intravenous fluids under close monitoring, as they are liable to develop serious complications. • One of these serious complications is iatrogenic hyponatremia. • It is also important to keep a fluid balance chart documenting total fluid intake, total output and deficit, and ongoing losses as well as body weight.

1.2

Indications for Intravenous Fluids

• Infants and children require intravenous fluids for the following reasons: –– To correct hypovolemia or dehydration. –– To provide maintenance fluids and electrolytes in those kept nil by mouth. –– To replace fluid losses from drains, nasogastric tube, gastrointestinal losses as a result of vomiting or diarrhea, bleeding, fever, and third-space losses.

1.3

Maintenance Fluid Requirements

• Fluid maintenance for infants and children is based on the body weight as follows (Table 1.1): • Or as follows (Table 1.2): • Another simpler and quick method of calculating fluid requirements in infants and children in milliliter per hour is the 4:2:1 formula which is based on body weight (Table 1.3). • The daily fluid requirements may be given using dextrose 5% in half-normal saline solution. • For patients with significant hyponatremia, it is preferable to use dextrose 5% in normal saline. • The daily Sodium and Potassium requirements must also be taken in consideration when calculating fluids and electrolytes requirements.

Table 1.1 Daily fluid requirements 0–10 kg 10– 20 kg 20– 70 kg >70 kg

100 per kg 100 per kg + 50 ml per kg above 10 kg (1000 ml + 50 ml per kg above 10 kg) 100 ml per kg + 50 ml per kg for the next 10 kg + 20 ml per each kg above 20 kg (1500 ml + 20 ml per kg above 20 kg) 2500 ml

Table 1.2 Daily fluid requirements, second method for calculation Weight First 10 kg weight Second 10 kg weight Additional kg

© Springer Nature Switzerland AG 2020 A. H. Al-Salem, Atlas of Pediatric Surgery, https://doi.org/10.1007/978-3-030-29211-9_1

ml/kg 100 ml/kg 50 ml/kg 20 ml/kg

ml/kg/h 4 ml/kg/h 2 ml/kg/h

1

2

1 Intravenous Fluids, Electrolytes, Blood, and Blood Products

Table 1.3 The 4:2:1 formula 4: 2: 1:

4 ml/kg/h for the first 10 kg plus 2 ml/kg/h for the second 10 kg plus 1 ml/kg/h for each kg over 20 kg

Sodium : 2 - 3mEq / day Potassium : 2 - 3mEq / day

• It is important to monitor the rate of intravenous fluids as raid infusion can lead to complications including: –– Hyponatremia –– Edema –– Pulmonary edema –– Persistent PDA (patent ductus arteriosus) –– Increased risk of IVH (intraventricular hemorrhage)

1.4

Fluid Deficit

• The fluid deficit must be taken in consideration when calculating daily fluid requirements. • The fluid deficit is usually the result of dehydration and the degree of this is estimated clinically. • This is not an accurate measurement, and the deficit is usually replaced gradually over 24 h. The fluid deficit is estimated as follows: Fluid deficit in ml = %dehydration ´ weight ( kg ) ´ 10 • The degree of fluid deficit (dehydration) is expressed as a percentage of body weight. • This is usually replaced using 0.9% normal saline or 5% albumin if sufficient protein is being lost.

1.5

Ongoing Fluid Losses

• Fluid losses are calculated and assessed every 4 h. • These fluids are replaced milliliter for milliliter, and the type of fluid being used depends on the likely electrolytes composition of the fluid lost as shown in Table 1.4. • Normally 0.9% normal saline or Ringer’s lactate with or without additional potassium is used to replace ongoing fluid losses.

1.6

luid Therapy During the First Week F of Life

• One of the most important points is fluid therapy during the first week of life. • This varies on daily basis and depends on the body weight as well.

Table 1.4 Electrolyte composition of body and replacement fluids Gastric Pancreatic Bile Diarrhea Ileostomy Ringer’s lactate Normal Saline (0.9% Na Cl) 0.45% Na Cl

Na+ 70 140 130 50 130 130 154 7

K+ 5–15 5 5 35 15–20 4 0 0

Cl− 120 50–100 100 40 120 109 154 77

HCO3 0 100 40 50 25–30 28 0 0

• The fluid requirements are calculated in ml/kg/day as follows (Table 1.5): • The fluid replacement in these patients is usually by Dextrose 10%. • Sodium and Potassium replacement should be started after 48 h as follows: Sodium : 2 - 3mEq / kg / day Potassium : 2 - 3mEq / kg / day Calcium : Calcium supplements may be added in certain situations in a dose of 4ml / kg / day ( 40mg / kg / day ) ascalcium gluconate

• In these patients it is also important to calculate the insensible water loss. This is also calculated according to birth weight as follows (Table 1.6).

1.7

Electrolytes Disturbances (Table 1.7)

1.7.1 Sodium • Normal sodium level: 135–145 mmol/L. • Hyponatremia: Plasma sodium level less than 135 mmol/L. • It is considered severe when the plasma sodium level is less than 130 mmol/L. • Acute dilutional hyponatremia is a medical emergency. • Symptomatic Hyponatremia is treated with 3% sodium chloride solution using the following formula: mmol of sodium required = (130 - Reported Serum sodium level ) ´ 0.6 ´ weight ( kg ) 1ml / kgof 3% sodium chloride solution will raise the serum sodiumby1mmol / L.

• Asymptomatic hyponatremia will not require active treatment.

1.7.2 Potassium • Normal potassium level (Table 1.8): • Hypokalemia: Plasma potassium level less than 3.4 mmol/L.

1.8 Blood and Blood Products Transfusion

3

Table 1.5 First week of life, fluid requirements Birth weight 1500 g

Day 1 80 80 60

Day 2 100 95 75

Day 3 120 110 90

Day 4 130 120 105

Insensible water loss (ml/kg/day) 60–80 40–60 20

Table 1.7 Electrolytes disturbances Parameter Normal serum sodium level Hyponatremia Hypernatremia Moderate hypernatremia Severe hypernatremia Normal serum potassium level Hypokalemia Severe hypokalemia Hypokalemia Normal serum calcium level Hypocalcemia

Serum level 135–145 mmol/L 150 mmol/L 150–170 mmol/L >170 mmol/L 3.4–4.7 mmol/L