Atlas of Pediatric Surgery: Principles and Treatment

Preface\nAcknowledgments\nContents\n1: Intravenous Fluids, Electrolytes, Blood, and Blood Products\n	1.1	 Introduction\n	1.2	 Indications for Intravenous Fluids\n	1.3	 Maintenance Fluid Requirements\n	1.4	 Fluid Deficit\n	1.5	 Ongoing Fluid Losses\n	1.6	 Fluid Therapy During the First Week of Life\n	1.7	 Electrolytes Disturbances (Table 1.7)\n		1.7.1	 Sodium\n		1.7.2	 Potassium\n		1.7.3	 Calcium\n	1.8	 Blood and Blood Products Transfusion\n	1.9	 Platelets Transfusion\n		1.9.1	 Types of Platelets\n			1.9.1.1	 Platelets Apheresis Pediatric Leucocyte Depleted (PAPLD)\n			1.9.1.2	 Platelets Pooled in T-Sol Leucocyte Depleted\n		1.9.2	 Dose of Platelets Transfusion\n	1.10	 Fresh Frozen Plasma\n	1.11	 Cryoprecipitate Transfusion\n	1.12	 Complications of Blood and Platelets Transfusion\n	1.13	 Albumin Transfusion\n	1.14	 Factor VIII Transfusion\n	1.15	 Granulocytes Transfusion\n	Further Reading\n2: Nutrition for Infants and Children\n	2.1	 Introduction\n	2.2	 Important Terms to Be Remembered\n	2.3	 Indications for Total Parenteral Nutrition\n	2.4	 Goals and Characteristics of Parenteral Nutrition for Infants and Children\n	2.5	 Caloric Requirements for Infants and Children\n		2.5.1	 Carbohydrate (Dextrose)\n		2.5.2	 Protein (Amino Acids)\n		2.5.3	 Intralipids\n		2.5.4	 Electrolytes, Vitamins and Minerals\n		2.5.5	 Vitamins\n	2.6	 Complications Associated with the Use of Parenteral Nutrition\n	Further Reading\n3: Venous Access for Infants and Children\n	3.1	 Introduction\n	3.2	 Types of Catheters\n	3.3	 Indications for Central Venous Catheters\n	3.4	 Peripheral Catheters (Percutaneous Peripheral Catheters)\n	3.5	 Umbilical Vascular Access\n	3.6	 Peripheral Venous Cutdown Catheters\n		3.6.1	 Intraosseous Catheters\n		3.6.2	 Peripherally Inserted Central Catheters (PICC)\n	3.7	 Central Venous Catheters (CVCs)\n	3.8	 Implantable Vascular-Access Devices\n	3.9	 Complications for Central Vascular Access\n		3.9.1	 Long-Term Catheter Complications\n	Further Reading\n4: Dermoid Cysts\n	4.1	 Introduction\n	4.2	 Head and Neck Dermoid Cysts\n	Further Reading\n5: Sternocleidomastoid Tumor and Torticollis\n	5.1	 Introduction\n	5.2	 Etiology\n	5.3	 Clinical Features\n	5.4	 Treatment\n	Further Reading\n6: Thyroglossal Cyst\n	6.1	 Introduction\n	6.2	 Embryology (Fig. 6.2)\n	6.3	 Histology\n	6.4	 Clinical Features\n	6.5	 Investigations\n	6.6	 Treatment\n	Further Reading\n7: Branchial Cysts, Sinuses, and Fistulae\n	7.1	 Introduction\n	7.2	 Embryology\n	7.3	 Branchial Grooves (Clefts) and Membranes\n	7.4	 Branchial Remnants\n	7.5	 Clinical Features\n		7.5.1	 First Branchial Remnants\n		7.5.2	 Second Branchial Remnants\n		7.5.3	 Third and Fourth Branchial Remnants\n		7.5.4	 Branchial Cystic Remnants\n	7.6	 Branchial Fistulae Remnants\n		7.6.1	 Treatment\n		7.6.2	 Pathology\n	7.7	 Congenital Midline Cervical Cleft\n	Further Reading\n8: Infantile Fibromatosis\n	8.1	 Introduction\n	8.2	 Infantile Fibromatoses\n	8.3	 Treatment\n	8.4	 The Prognosis\n	8.5	 Fibromatosis Colli\n	Further Reading\n9: Epulis\n	9.1	 Introduction\n	9.2	 Treatment\n	Further Reading\n10: Ranula\n	10.1	 Introduction\n	10.2	 Treatment\n	Further Reading\n11: Abdominal Wall Defects\n	11.1	 Introduction\n	11.2	 Embryology (Figs. 11.1, 11.2, and 11.3)\n	11.3	 Pathogenesis\n	11.4	 Omphalocele (Fig. 11.14)\n	11.5	 Gastroschisis (Fig. 11.15)\n	11.6	 Hernia of the Umbilical Cord (Fig. 11.16)\n	11.7	 Urachal Remnants and Omphalomesenteric Duct Malformations (Figs. 11.17, 11.18, and 11.19)\n	11.8	 Bladder Exstrophy (Figs. 11.20, 11.21, and 11.22)\n	11.9	 Prune-Belly Syndrome (Fig. 11.23)\n	11.10	 Cloacal Exstrophy (Fig. 11.24)\n	Further Reading\n12: Abdominal Wall Hernias and Hydroceles\n	12.1	 Introduction: Abdominal Wall Hernias\n	12.2	 Inguinal Hernia\n	12.3	 Etiology\n	12.4	 Clinical Features\n	12.5	 Variants of Inguinal Hernia\n		12.5.1	 Indirect Inguinal Hernia\n		12.5.2	 Direct Inguinal Hernia\n		12.5.3	 Littre’s Hernia\n		12.5.4	 Sliding Inguinal Hernia\n		12.5.5	 Richter’s Hernia\n		12.5.6	 Busse’s Hernia\n		12.5.7	 Maydl’s Hernia\n		12.5.8	 Amyand’s Hernia\n	12.6	 Complications of Inguinal Hernias\n		12.6.1	 Incarceration\n		12.6.2	 Strangulation\n	12.7	 Treatment\n	12.8	 Complications of Inguinal Herniotomy\n	12.9	 Hydrocele\n	12.10	 Embryology\n	12.11	 Classification of Hydroceles (Fig. 12.20)\n	12.12	 Treatment\n	12.13	 Femoral Hernia\n	12.14	 Etiology\n	12.15	 Diagnosis\n	12.16	 Treatment\n	12.17	 Umbilical Hernia\n	12.18	 Paraumbilical Hernia\n	12.19	 Epigastric Hernias\n	12.20	 Diastasis Recti\n	12.21	 Congenital Lumbar Hernia\n	12.22	 Spigelian Hernia\n	12.23	 Incisional Hernias\n	12.24	 Other Rare Abdominal Wall Hernias\n	Further Reading\n13: Umbilical Abnormalities in Infants and Children\n	13.1	 Introduction\n	13.2	 Embryology\n	13.3	 Abnormalities of the Umbilicus\n	13.4	 Umbilical Granuloma\n		13.4.1	 Treatment\n	13.5	 Umbilical Polyp\n	13.6	 Umbilical Infection (Omphalitis)\n		13.6.1	 Treatment\n	13.7	 Umbilical Hernia\n		13.7.1	 Treatment\n	13.8	 Omphalomesenteric Remnants\n	13.9	 Urachal Remnants\n	Further Reading\n14: Hemangiomas and Vascular Malformations\n	14.1	 Introduction\n	14.2	 Classification\n	14.3	 Vascular Malformations\n	14.4	 Hemangiomas\n		14.4.1	 Clinical Features\n	14.5	 Pathological and Developmental Changes in Hemangiomas\n	14.6	 Complications\n	14.7	 Investigations\n	14.8	 Hepatic Hemangiomas\n	14.9	 Treatment\n	14.10	 Prognosis\n	Further Reading\n15: Lymphangiomas\n	15.1	 Introduction\n	15.2	 Classifications\n	15.3	 Lymphangioma Circumscriptum\n	15.4	 Cavernous Lymphangioma\n	15.5	 Cystic Hygroma\n	15.6	 Sites\n	15.7	 Etiology\n	15.8	 Complications\n	15.9	 Diagnosis\n	15.10	 Treatment\n	Further Reading\n16: Undescended Testes (Cryptorchidism)\n	16.1	 Introduction\n	16.2	 Embryology and Etiology\n	16.3	 Classification of Abnormal Testes\n	16.4	 Effects of Undescended Testes\n	16.5	 Investigations\n	16.6	 Treatment\n	16.7	 Hormonal Treatment\n	16.8	 Surgical Treatment\n	16.9	 Nonpalpable Testis\n	16.10	 Complications of Orchidopexy\n	Further Reading\n17: Acute Scrotum\n	17.1	 Introduction\n	17.2	 Torsion of Testes\n		17.2.1	 Introduction\n		17.2.2	 Classification\n		17.2.3	 Etiology\n			17.2.3.1	 Intravaginal Torsion\n			17.2.3.2	 Extravaginal Torsion\n		17.2.4	 Effects of Torsion of Testes\n		17.2.5	 Clinical Features\n			17.2.5.1	 Intravaginal Torsion\n			17.2.5.2	 Extravaginal Torsion\n		17.2.6	 Investigations and Treatment\n	17.3	 Torsion of the Testicular or Epididymal Appendage\n		17.3.1	 Introduction\n		17.3.2	 Embryology\n		17.3.3	 Clinical Features\n		17.3.4	 Investigations and Treatment\n	17.4	 Epididymitis, Orchitis, and Epididymo-orchitis\n		17.4.1	 Introduction\n		17.4.2	 Etiology\n		17.4.3	 Clinical Features\n		17.4.4	 Investigations and Treatment\n	17.5	 Idiopathic Scrotal Edema\n	17.6	 Testicular Trauma\n	17.7	 Other Causes of Acute Scrotum\n	17.8	 Algorithm\n	Further Reading\n18: Varicocele\n	18.1	 Introduction\n	18.2	 Etiology\n	18.3	 Classification\n	18.4	 Grading of Varicocele\n	18.5	 Clinical Features\n	18.6	 Investigations\n	18.7	 Treatment\n	18.8	 Complications of Varicocelectomy\n	Further Reading\n19: Pediatric Abdominal and Thoracic Trauma\n	19.1	 Introduction\n	19.2	 Abdominal Trauma in Children\n	19.3	 Thoracic Trauma in Children\n	19.4	 Airway Injuries\n	19.5	 Rib Fractures, Hemothorax, and Pneumothorax\n	19.6	 Pulmonary Contusion (Figs. 19.17 and 19.18)\n	19.7	 Diaphragm Rupture\n	Further Reading\n20: Omphalocele\n	20.1	 Introduction\n	20.2	 Etiology\n	20.3	 Classification\n	20.4	 Associated Anomalies\n	20.5	 Management and Outcome\n	20.6	 Hernia of the Umbilical Cord\n	Further Reading\n21: Gastroschisis\n	21.1	 Introduction\n	21.2	 Etiology\n	21.3	 Diagnosis\n	21.4	 Clinical Features\n	21.5	 Associated Anomalies\n	21.6	 Management and Outcome\n	Further Reading\n22: Biliary Atresia\n	22.1	 Introduction\n	22.2	 Etiology\n	22.3	 Associated Congenital Anomalies\n	22.4	 Classification\n	22.5	 Clinical Features\n	22.6	 Investigations\n	22.7	 Biliary Hypoplasia\n	22.8	 Treatment\n	22.9	 Complications and Outcome\n	Further Reading\n23: Choledochal Cysts\n	23.1	 Introduction\n	23.2	 Etiology\n	23.3	 Classification\n	23.4	 Forme Fruste Choledochal Cyst\n	23.5	 Clinical Features\n	23.6	 Investigations\n	23.7	 Treatment\n	Further Reading\n24: Congenital Pancreatic Cyst\n	24.1	 Introduction\n	24.2	 Classification\n	24.3	 Etiology\n	24.4	 Clinical Features\n	24.5	 Diagnosis\n	24.6	 Treatment\n	Further Reading\n25: Pancreatitis and Pancreatic Pseudocyst\n	25.1	 Introduction\n	25.2	 Etiology\n	25.3	 Pancreatic Divisum\n	25.4	 Pathophysiology\n	25.5	 Clinical Features\n	25.6	 Acute Hemorrhagic Pancreatitis\n	25.7	 Investigations\n	25.8	 Management\n		25.8.1	 Medical Management\n		25.8.2	 Surgical Management\n	25.9	 Pancreatic Pseudocysts\n		25.9.1	 Management of Pancreatic Pseudocysts\n	25.10	 Prognosis of Acute Pancreatitis\n	Further Reading\n26: Cholelithiasis and Choledocholithiasis\n	26.1	 Introduction\n	26.2	 Types of Gallstones\n	26.3	 Etiology of Cholelithiasis\n	26.4	 Complications of Cholelithiasis\n	26.5	 Clinical Features\n	26.6	 Investigations\n	26.7	 Acute Cholecystitis (Fig. 26.17)\n	26.8	 Cholangitis\n	26.9	 Choledocholithiasis (Figs. 26.18 and 26.19)\n	26.10	 Treatment\n	26.11	 Postcholecystectomy Syndrome\n	Further Reading\n27: Nesidioblastosis: Persistent Hyperinsulinemic Hypoglycemia of Infancy (PHHI)\n	27.1	 Introduction\n	27.2	 Etiology and Pathogenesis\n	27.3	 Clinical Features\n	27.4	 Investigations\n	27.5	 Pathology\n	27.6	 Histologically, congenital hyperinsulinism is divided into two types:\n	27.7	 Management\n	Further Reading\n28: The Spleen and Splenic Disorders\n	28.1	 Introduction\n	28.2	 Embryology\n	28.3	 Functions of the Spleen\n	28.4	 Disorders of the Spleen\n		28.4.1 Splenomegaly\n		28.4.2 Massive Splenomegaly\n		28.4.3 Splenic Rupture\n		28.4.4 Splenosis\n		28.4.5 Accessory Spleen\n		28.4.6 Asplenia\n		28.4.7 Hepatolienal Fusion\n		28.4.8 Polysplenia\n		28.4.9 Splenogonadal Fusion\n			28.4.9.1	 Introduction\n			28.4.9.2	 Classification\n			28.4.9.3	 Etiology\n			28.4.9.4	 Clinical Features\n			28.4.9.5	 Associated Anomalies\n			28.4.9.6	 Treatment\n		28.4.10 Splenorenal Fusion\n		28.4.11 Wandering Spleen\n		28.4.12 Splenic Cysts\n		28.4.13 Splenic Abscess\n		28.4.14 Massive Splenic Infarction\n		28.4.15 Congestive Splenomegaly\n	28.5	 Immunizations and Splenectomy\n	28.6	 Partial Splenectomy\n	Further Reading\n29: Acute Appendicitis\n	29.1	 Introduction\n	29.2	 Pathophysiology\n	29.3	 Classification and Staging of Acute Appendicitis\n	29.4	 Clinical Features\n	29.5	 Diagnosis\n	29.6	 Management\n		29.6.1	 Appendectomy\n	29.7	 Complications of Acute Appendicitis\n	Further Reading\n30: Minimal Invasive Surgery in the Pediatric Age Group\n	Further Reading\n31: Congenital Lobar Emphysema\n	31.1	 Introduction\n	31.2	 Etiology\n	31.3	 Pathophysiology\n	31.4	 Sites\n	31.5	 Clinical Features\n	31.6	 Diagnosis\n	31.7	 Treatment\n	Further Reading\n32: Congenital Cystic Adenomatoid Malformation\n	32.1	 Introduction\n	32.2	 Etiology and Pathophysiological Changes\n	32.3	 Classification\n	32.4	 Clinical Features\n	32.5	 Prognosis\n	32.6	 Investigations\n	32.7	 Treatment\n	Further Reading\n33: Bronchopulmonary Sequestration\n	33.1	 Introduction\n	33.2	 Etiology and Pathophysiology\n	33.3	 Classification\n	33.4	 Diagnosis\n	33.5	 Complications\n	33.6	 Pathophysiology\n	33.7	 Treatment and Prognosis\n	Further Reading\n34: Bronchogenic Cyst\n	34.1	 Introduction\n	34.2	 Etiology\n	34.3	 Sites\n	34.4	 Clinical Features\n	34.5	 Cyst-Related Complications\n	34.6	 Diagnosis\n	34.7	 Histopathological Features\n	34.8	 Treatment\n	Further Reading\n35: Meckel’s Diverticulum\n	35.1	 Introduction\n	35.2	 Embryology\n	35.3	 Symptoms\n	35.4	 Diagnosis\n	35.5	 Treatment\n	Further Reading\n36: Intussusception\n	36.1	 Introduction\n	36.2	 Etiology\n	36.3	 Classification\n	36.4	 Clinical Features\n	36.5	 Diagnosis\n	36.6	 Treatment\n	Further Reading\n37: Congenital Diaphragmatic Hernia\n	37.1	 Introduction\n	37.2	 Etiology and Pathophysiology\n	37.3	 Associated Anomalies\n	37.4	 Clinical Features\n	37.5	 Investigations\n	37.6	 Treatment\n	37.7	 Long-Term Outcomes and Prognosis\n	37.8	 Agenesis of Diaphragm\n	37.9	 Traumatic Diaphragmatic Hernia\n	Further Reading\n38: Congenital Morgagni Hernia\n	38.1	 Introduction\n	38.2	 Embryology\n	38.3	 Site\n	38.4	 Herniated Viscera\n	38.5	 Associated Anomalies\n	38.6	 Clinical Features\n	38.7	 Investigations\n	38.8	 Treatment\n	Further Reading\n39: Congenital Paraesophageal Hernia\n	39.1	 Introduction\n	39.2	 Anatomy and Pathophysiology\n	39.3	 Etiology\n	39.4	 Classification\n	39.5	 Clinical Features\n	39.6	 Diagnosis\n	39.7	 Treatment\n	Further Reading\n40: Eventration of Diaphragm\n	40.1	 Introduction\n	40.2	 Etiology and Classification\n	40.3	 Clinical Features\n	40.4	 Diagnosis\n	40.5	 Management\n	Further Reading\n41: Esophageal Atresia and Tracheoesophageal Fistula\n	41.1	 Introduction\n	41.2	 Classification\n	41.3	 Associated Anomalies\n	41.4	 Clinical Features\n	41.5	 Diagnosis\n	41.6	 Management\n	41.7	 Esophageal Atresia Without Tracheoesophageal Fistula\n	41.8	 Prognosis\n	41.9	 Postoperative Complications\n	41.10	 Esophageal Replacement\n	41.11	 Congenital Short Esophagus\n	41.12	 Congenital Esophageal Diverticulum\n	41.13	 Foregut Duplications\n	41.14	 Congenital Bronchopulmonary Foregut Malformations\n	41.15	 Esophageal Duplications (Figs. 41.66, 41.67, and 41.68)\n	41.16	 Laryngotracheoesophageal Cleft\n	Further Reading\n42: Congenital Esophageal Stenosis\n	42.1	 Introduction\n	42.2	 Etiology and Embryogenesis\n	42.3	 Classification\n	42.4	 Clinical Features\n	42.5	 Diagnosis\n	42.6	 Treatment\n	Further Reading\n43: H-Type Tracheoesophageal Fistula\n	43.1	 Introduction\n	43.2	 Embryology\n	43.3	 Classification\n	43.4	 Clinical Features\n	43.5	 Diagnosis\n	43.6	 Treatment\n	Further Reading\n44: Achalasia\n	44.1	 Introduction\n	44.2	 Etiology\n	44.3	 Clinical Features\n	44.4	 Diagnosis\n	44.5	 Management\n	44.6	 Allgrove (AAA) Syndrome\n	Further Reading\n45: Infantile Hypertrophic Pyloric Stenosis\n	45.1	 Introduction\n	45.2	 Etiology\n	45.3	 Clinical Features\n	45.4	 Investigations and Diagnosis\n	45.5	 Management\n	Further Reading\n46: Gastric Volvulus\n	46.1	 Introduction\n	46.2	 Etiology\n	46.3	 Classification\n	46.4	 Clinical Features\n	46.5	 Diagnosis\n	46.6	 Treatment\n	Further Reading\n47: Congenital Gastric Outlet Obstruction\n	47.1	 Introduction\n	47.2	 Etiology\n	47.3	 Classification\n	47.4	 Clinical Features\n	47.5	 Diagnosis\n	47.6	 Treatment\n	47.7	 Prognosis\n	Further Reading\n48: Congenital Duodenal Obstruction\n	48.1	 Introduction\n	48.2	 Embryology and Etiology\n	48.3	 Classification\n	48.4	 Associated Anomalies\n	48.5	 Clinical Features\n	48.6	 Investigations and Diagnosis\n	48.7	 Management\n	48.8	 Postoperative Early and Long-Term Complications\n	Further Reading\n49: Congenital Intestinal Atresia and Stenosis\n	49.1	 Introduction\n	49.2	 Jejunoileal Atresia and Stenosis\n		49.2.1	 Introduction\n		49.2.2	 Etiology and Pathogenesis\n		49.2.3	 Classification\n		49.2.4	 Associated Anomalies\n		49.2.5	 Clinical Features\n		49.2.6	 Investigations and Diagnosis\n		49.2.7	 Treatment and Outcome\n		49.2.8	 Short-Bowel Syndrome\n	49.3	 Colonic Atresia and Stenosis\n		49.3.1	 Introduction\n		49.3.2	 Etiology\n		49.3.3	 Classification\n		49.3.4	 Associated Anomalies\n		49.3.5	 Clinical Features\n		49.3.6	 Diagnosis\n		49.3.7	 Treatment\n	49.4	 Chilaiditi Syndrome\n	Further Reading\n50: Intestinal Malrotation\n	50.1	 Introduction\n	50.2	 Embryology\n	50.3	 Associated Anomalies\n	50.4	 Clinical Features\n	50.5	 Investigations\n	50.6	 Management and Prognosis\n	50.7	 Complications\n	Further Reading\n51: Gastrointestinal Duplications\n	51.1	 Introduction\n	51.2	 Etiology\n	51.3	 Clinical Features\n	51.4	 Investigations\n	51.5	 Treatment\n	Further Reading\n52: Necrotizing Enterocolitis\n	52.1	 Introduction\n	52.2	 Incidence of NEC\n	52.3	 Pathology of NEC\n	52.4	 Etiology and Pathogenesis of NEC\n	52.5	 Clinical Features\n	52.6	 Spontaneous Intestinal Perforation (SIP)\n	52.7	 Stages of NEC\n	52.8	 Diagnosis of NEC\n	52.9	 Treatment\n	52.10	 Operative Considerations\n	52.11	 Outcome\n	Further Reading\n53: Meconium Ileus\n	53.1	 Introduction\n	53.2	 Etiology\n	53.3	 Classification\n	53.4	 Clinical Features\n	53.5	 Diagnosis\n	53.6	 Management\n	53.7	 Meconium Peritonitis\n	53.8	 Meconium Ileus Equivalent\n	53.9	 Complications Associated with Cystic Fibrosis and Meconium Ileus\n	Further Reading\n54: Hirschsprung’s Disease\n	54.1	 Introduction\n	54.2	 Etiology and Pathogenesis\n	54.3	 Associated Conditions\n	54.4	 Clinical Features\n	54.5	 Investigations and Diagnosis (Table 54.1)\n	54.6	 Management\n	54.7	 Hirschsprung’s Enterocolitis\n	54.8	 Postoperative Complications and Outcome\n	Further Reading\n55: Congenital Rectal Stenosis and Atresia\n	55.1	 Introduction\n	55.2	 Classification\n	55.3	 Clinical Features\n	55.4	 Diagnosis\n	55.5	 Treatment\n	Further Reading\n56: Congenital Segmental Dilatation of the Intestines\n	56.1	 Introduction\n	56.2	 Clinical Features\n	56.3	 Associated Anomalies\n	56.4	 Etiology\n	56.5	 Sites\n	56.6	 Diagnosis\n	56.7	 Treatment\n	Further Reading\n57: Meconium Plug Syndrome\n	57.1	 Introduction\n	57.2	 Clinical Features\n	57.3	 Diagnosis\n	57.4	 Management\n	Further Reading\n58: Neonatal Small Left Colon Syndrome\n	58.1	 Introduction\n	58.2	 Etiology and Pathogenesis\n	58.3	 Clinical Features\n	58.4	 Investigations\n	58.5	 Treatment and Outcome\n	Further Reading\n59: Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (Berdon Syndrome)\n	59.1	 Introduction\n	59.2	 Etiology\n	59.3	 Clinical Features\n	59.4	 Associated Anomalies\n	59.5	 Diagnosis\n	59.6	 Treatment and Prognosis\n	Further Reading\n60: Intestinal Polyps and Polyposis Syndromes\n	60.1	 Introduction\n	60.2	 Clinical Features\n	60.3	 Classification of Intestinal Polyps and Polyposis Syndromes\n	60.4	 Juvenile Polyps\n	60.5	 Juvenile Polyposis Syndrome (JPS)\n	60.6	 Peutz-Jeghers Syndrome (PJS)\n	60.7	 Familial Adenomatous Polyposis (FAP)\n	60.8	 Attenuated Familial Adenomatous Polyposis (AFAP)\n	60.9	 mutY Homologue (MYH)-Associated Polyposis (MAP)\n	60.10	 Gardner Syndrome\n	60.11	 Turcot Syndrome\n	60.12	 Cowden Syndrome\n	60.13	 Bannayan-Zonana Syndrome (BRR Syndrome)\n	60.14	 Cronkhite-Canada Syndrome\n	60.15	 Hereditary-Mixed Polyposis Syndrome\n	60.16	 Gorlin Syndrome (GS)\n	60.17	 Lymphoid Polyposis\n	60.18	 Ruvalcaba-Myhre-Smith Syndrome\n	Further Reading\n61: Anorectal Malformations\n	61.1	 Introduction\n	61.2	 Anatomy and Embryology\n	61.3	 Classifications\n	61.4	 Krickenbeck Classification (2005)\n	61.5	 The Most Common Malformations\n	61.6	 Associated Malformations\n	61.7	 Clinical Features\n	61.8	 Investigations\n	61.9	 Management\n	61.10	 Outcome\n	61.11	 The Pouch Colon Syndrome\n	Further Reading\n62: Perianal Abscess and Fistula-in-Ano\n	62.1	 Introduction\n	62.2	 Anatomy\n	62.3	 Classification\n	62.4	 Etiology\n	62.5	 Clinical Features\n	62.6	 Treatment\n	Further Reading\n63: Cloacal Anomalies\n	63.1	 Introduction\n	63.2	 Associated Anomalies\n	63.3	 Classification\n	63.4	 Clinical Features\n	63.5	 Investigations\n	Further Reading\n64: Cloacal Exstrophy\n	64.1	 Introduction\n	64.2	 Etiology and Pathogenesis\n	64.3	 Associated Anomalies\n	64.4	 Clinical Features and Management\n	Further Reading\n65: Renal Tumors: Wilms Tumor (Nephroblastoma)\n	65.1	 Introduction\n	65.2	 Pathology\n	65.3	 Renal Blastema and/or Nephroblastomatosis (Nephrogenic Rests)\n	65.4	 Clinical Features\n	65.5	 Investigations\n	65.6	 Staging\n	65.7	 Treatment (Table 65.1)\n		65.7.1 Management of Lung Metastasis\n	65.8	 Surgical Considerations\n		65.8.1 Radical Nephrectomy\n		65.8.2 Partial Nephrectomy\n		65.8.3 Bilateral Wilms’ Tumor (Fig. 65.47)\n	65.9	 Surgical Complications\n	65.10	 Prognosis and Outcome (Table 65.2)\n	65.11	 Extrarenal Wilms’ Tumors\n	65.12	 Clear Cell Sarcoma of the Kidney\n		65.12.1 Treatment\n		65.12.2 Prognosis\n	65.13	 Malignant Rhabdoid Tumor of the Kidney\n		65.13.1 Histology\n		65.13.2 Treatment\n	65.14	 Mesoblastic Nephroma\n		65.14.1 Pathology\n		65.14.2 Treatment\n	Further Reading\n66: Neuroblastoma\n	66.1	 Introduction\n	66.2	 Embryology and Anatomy\n	66.3	 Cystic Neuroblastoma\n	66.4	 Perinatal Neuroblastoma\n	66.5	 Etiology, Chromosomal and Molecular Markers\n	66.6	 Clinical Features\n	66.7	 Diagnosis\n	66.8	 Histology\n	66.9	 Shimada Histopathologic Classification System\n	66.10	 Staging\n	66.11	 Screening\n	66.12	 Treatment\n		66.12.1 Intermediate-Risk Group Treatment\n		66.12.2 High-Risk Group Treatment\n	66.13	 Refractory and Relapsed Neuroblastoma\n	66.14	 Complications and Prognosis\n	Further Reading\n67: Liver Tumors\n	67.1	 Introduction\n	67.2	 Hepatoblastoma\n		67.2.1	 Introduction\n		67.2.2	 Etiology and Associated Anomalies\n		67.2.3	 Histologic Classification of Hepatoblastoma\n		67.2.4	 Clinical Features\n		67.2.5	 Diagnosis\n		67.2.6	 Staging\n		67.2.7	 Management\n		67.2.8	 Liver Transplantation\n		67.2.9	 Surgical Considerations\n	67.3	 Hepatocellular Carcinoma (HCC)\n	67.4	 Hepatic Metastases\n	67.5	 Other Primary Malignant Tumors of the Liver\n	67.6	 Undifferentiated Embryonal Sarcoma\n	67.7	 Infantile Choriocarcinoma of the Liver\n	67.8	 Benign Liver Tumors\n	67.9	 Hepatic Hemangiomas\n		67.9.1	 Management\n	67.10	 Hemangioendothelioma\n	67.11	 Mesenchymal Hamartomas\n	67.12	 Focal Nodular Hyperplasia\n	67.13	 Hepatic Adenoma\n	Further Reading\n68: Rhabdomyosarcoma\n	68.1	 Introduction\n	68.2	 Etiology\n	68.3	 Histology\n	68.4	 Classification\n	68.5	 Staging\n	68.6	 Clinical Features\n	68.7	 Investigations\n	68.8	 Risk Classification\n	68.9	 Treatment\n	Further Reading\n69: Lymphomas in Children\n	69.1	 Introduction\n	69.2	 Classifications\n	69.3	 Hodgkin’s Lymphoma\n		69.3.1	 Etiology and Pathophysiology\n		69.3.2	 Clinical Features\n		69.3.3	 Staging\n		69.3.4	 Investigations\n		69.3.5	 Histologic Classification of Hodgkin’s Disease\n		69.3.6	 Management\n	69.4	 Non-Hodgkin’s Lymphoma\n		69.4.1	 Etiology\n		69.4.2	 Burkitt Lymphoma\n			69.4.2.1	 Clinical Features\n		69.4.3	 Investigations\n		69.4.4	 Management\n		69.4.5	 Prognosis and Outcome\n	Further Reading\n70: Ovarian Cysts and Tumors\n	70.1	 Introduction\n	70.2	 Classification\n	70.3	 Ovarian Cysts in the Fetus\n	70.4	 Diagnosis\n	70.5	 Management and Outcome\n	70.6	 Ovarian Cysts in Neonates\n	70.7	 Management\n	70.8	 Ovarian Cysts in Infants and Prepubertal Girls\n	70.9	 Investigations\n	70.10	 Management and Outcome (Fig. 70.12)\n	70.11	 Ovarian Cysts in Adolescents\n	70.12	 Management and Outcome\n		70.12.1 Follicular Cysts\n		70.12.2 Corpus Luteum Cysts\n	70.13	 Ovarian Tumors\n		70.13.1 Introduction\n		70.13.2 Fibromas\n		70.13.3 Thecoma\n		70.13.4 Ovarian Cystadenoma\n		70.13.5 Germ Cell Tumors\n		70.13.6 Ovarian Teratoma\n		70.13.7 Epithelial Ovarian Tumors\n		70.13.8 Dysgerminoma\n		70.13.9 Clinical Manifestations of Ovarian Tumors\n		70.13.10 Investigations\n		70.13.11 Staging\n		70.13.12 Treatment\n	Further Reading\n71: Thyroid Tumors in Children\n	71.1	 Introduction\n	71.2	 Etiology\n	71.3	 Histopathology\n	71.4	 Classification of Thyroid Tumors\n	71.5	 Clinical Features\n	71.6	 Diagnosis\n	71.7	 Staging\n	71.8	 Treatment\n	71.9	 Differentiated Thyroid Carcinoma\n	71.10	 Medullary Thyroid Carcinoma (MTC)\n	71.11	 Prognosis\n	Further Reading\n72: Testicular Tumors\n	72.1	 Introduction\n	72.2	 Classification\n	72.3	 Histologic Classification of Seminomas\n	72.4	 Etiology\n	72.5	 Clinical Features\n	72.6	 Investigations\n	72.7	 Staging\n	72.8	 Treatment\n	72.9	 Yolk Sac Tumor\n	72.10	 Teratoma\n	72.11	 Mixed Germ Cell Tumor\n	72.12	 Stromal Tumors\n	72.13	 Testicular Cyst\n	72.14	 Paratesticular Rhabdomyosarcoma\n	72.15	 Prognosis and Outcome\n	Further Reading\n73: Teratomas\n	73.1	 Introduction\n	73.2	 Sacrococcygeal Teratoma\n		73.2.1 Introduction\n		73.2.2 Pathogenesis\n		73.2.3 Classification\n		73.2.4 Histology\n		73.2.5 Clinical Features\n		73.2.6 Investigations\n		73.2.7 Treatment\n		73.2.8 Surgical Consideration\n		73.2.9 Fetal Intervention\n		73.2.10 Outcomes\n		73.2.11 Complications of Sacrococcygeal Teratoma\n	73.3	 Gastric Teratoma\n		73.3.1 Clinical Features\n		73.3.2 Treatment\n	73.4	 Ovarian Teratomas\n		73.4.1 Introduction\n		73.4.2 Staging of Malignant Teratoma\n		73.4.3 Clinical Features\n		73.4.4 Investigations\n		73.4.5 Histopathology\n		73.4.6 Treatment\n	73.5	 Testicular Teratoma\n		73.5.1 Introduction\n		73.5.2 Clinical Features\n		73.5.3 Treatment\n	73.6	 Intra-Abdominal Teratoma\n	73.7	 Mediastinal Teratoma\n		73.7.1 Clinical Features\n		73.7.2 Investigations\n		73.7.3 Treatment\n	73.8	 Cervical Teratoma\n		73.8.1 Introduction\n		73.8.2 Primary Thyroid Teratoma\n		73.8.3 Management\n	Further Reading\n74: Persistent Müllerian Duct Syndrome (Hernia Uteri Inguinalis)\n	74.1	 Introduction\n	74.2	 Embryology and Etiology\n	74.3	 Clinical Features\n	74.4	 Management\n	74.5	 Follow-Up\n	Further Reading\n75: Hypospadias\n	75.1	 Introduction\n	75.2	 Embryology\n	75.3	 Classification\n	75.4	 Associated Anomalies\n	75.5	 Etiology\n	75.6	 Treatment\n	Further Reading\n76: Epispadias and the Exstrophy-Epispadias Complex\n	76.1	 Introduction\n	76.2	 Embryology\n	76.3	 Epispadias\n		76.3.1	 Introduction\n		76.3.2	 Etiology\n		76.3.3	 Classification\n		76.3.4	 Treatment\n		76.3.5	 Female Epispadias\n	76.4	 Bladder Exstrophy\n		76.4.1	 Introduction\n		76.4.2	 Radiological Evaluation\n		76.4.3	 Management\n	Further Reading\n77: Hydrocolpos, Vaginal Agenesis, and Atresia\n	77.1	 Introduction\n	77.2	 Vaginal Atresia\n	77.3	 Classification\n	77.4	 Associated Anomalies\n	77.5	 Embryology\n	77.6	 Clinical Features\n	77.7	 Investigations\n	77.8	 Management\n	Further Reading\n78: Pelviureteric Junction Obstruction\n	78.1	 Introduction\n	78.2	 Embryology and Etiology\n	78.3	 Clinical Features\n	78.4	 Associated Anomalies\n	78.5	 Investigations and Diagnosis\n	78.6	 Treatment\n	Further Reading\n79: Hydronephrosis in Infants and Children\n	79.1	 Introduction\n	79.2	 Classification\n	79.3	 Pathophysiology\n	79.4	 Etiology\n	79.5	 Clinical Features\n	79.6	 Investigations and Diagnosis\n	79.7	 Treatment\n	Further Reading\n80: Vesicoureteric Reflux\n	80.1	 Introduction\n	80.2	 Classification\n	80.3	 Clinical Features\n	80.4	 Diagnosis\n	80.5	 Management\n	Further Reading\n81: Posterior Urethral Valve\n	81.1	 Introduction\n	81.2	 Etiology and Pathophysiology\n	81.3	 Classification\n	81.4	 Diagnosis\n	81.5	 Clinical Features\n	81.6	 Management\n	Further Reading\n82: Disorders of Sexual Development\n	82.1	 Introduction\n	82.2	 Embryology and Physiology of Sex Development\n	82.3	 Classification\n	82.4	 Classification of Disorders of Sexual Development (Fig. 82.9)\n	82.5	 Evaluation of a Newborn with DSD\n	82.6	 Diagnosis and Investigations\n	82.7	 Management of Patients with DSD\n	82.8	 Congenital Adrenal Hyperplasia (CAH)\n	82.9	 Androgen Insensitivity Syndrome (Testicular Feminization Syndrome)\n	82.10	 Partial (Incomplete) Androgen Insensitivity Syndrome\n	82.11	 Deficiency of MIS (Persistent Müllerian Duct Syndrome)\n	82.12	 Alpha-Reductase Deficiency\n	82.13	 Gonadal Dysgenesis\n	82.14	 Deficient Testosterone Biosynthesis\n	82.15	 Ovotestis Disorders of Sexual Development\n		82.15.1 Presentation\n		82.15.2 Investigations\n		82.15.3 Management\n	82.16	 Other Rare Disorders of Sexual Development\n		82.16.1 17β-Hydroxysteroid Dehydrogenase Deficiency\n		82.16.2 Aromatase Deficiency\n		82.16.3 Turner Syndrome\n		82.16.4 Klinefelter Syndrome\n		82.16.5 Triple X Syndrome\n		82.16.6 Aphallia\n		82.16.7 Diphallia\n		82.16.8 Micropenis\n		82.16.9 Uterus Didelphys\n	82.17	 Algorithms\n	Further Reading\nIndex