Rickham’s Neonatal Surgery

Foreword
Editors’ Preface: Rickham’s Neonatal Surgery
Contents
Part I: General
	1: Medical Law as Applied to Neonatal Surgery
		1.1	 Wrongful Birth
		1.2	 Proposed Guidelines for Instituting Intensive Care at Birth
		1.3	 Parental Responsibility
		1.4	 The ‘Baby Cases’
			1.4.1	 Baby Alexandra, and the Question of Life’s Sanctity
			1.4.2	 Dr. Arthur
			1.4.3	 Re C, and the Emergence of the Best Interests of the Child
			1.4.4	 Re J, and ‘Substituted Judgements’
			1.4.5	 Re C, and the Reassertion of Parental Rights?
			1.4.6	 Re A; Conjoined Twins, and the Impact on the Influence of Parents
		1.5	 Statutory Guidance
		1.6	 Practical Application
		References
	2: Embryology of Surgical Birth Defects
		2.1	 General Remarks on Embryology and The Embryology of Malformations
		2.2	 Animal Models Used for Applied Embyology
		2.3	 Scanning Electron Microscopic Atlas of Normal and Abnormal Development in Embryos
			2.3.1	 Normal Foregut Development
			2.3.2	 The Formation of Esophageal Atresia
			2.3.3	 Normal Diaphragmatic Development
			2.3.4	 Abnormal Diaphragmatic Development
			2.3.5	 Animal Model
			2.3.6	 Normal Hindgut Development
			2.3.7	 Abnormal Hindgut Development
			2.3.8	 Normal Development of the External Genitalia
			2.3.9	 Abnormal Development of the External Genitalia (Hypospadia Formation [80, 81]
			2.3.10	 Normal Midgut Development
				2.3.10.1	 Traditional Theories
				2.3.10.2	 Own Observations
			2.3.11	 Normal Testicular Descent
			2.3.12	 The Role of the Gubernaculum
		References
	3: Research in Pediatric Surgery
		3.1	 Introduction
		3.2	 Animal Models
			3.2.1	 Abdominal Wall Defects
			3.2.2	 Biliary Atresia
			3.2.3	 Congenital Diaphragmatic Hernia
			3.2.4	 Hirschsprung’s Disease
			3.2.5	 Necrotizing Enterocolitis
			3.2.6	 Short Bowel Syndrome
			3.2.7	 Parenteral Nutrition
			3.2.8	 Vacter and Other Models
		3.3	 Cell-Based Research
		3.4	 Clinical Research
		3.5	 Final Considerations
		References
	4: Antenatal Diagnosis: Current Status for Paediatric Surgeons
		4.1	 Introducing the Fetal Patient
		4.2	 Lower Urinary Tract Obstruction
			4.2.1	 Definition and Epidemiology
			4.2.2	 Genetics
			4.2.3	 Pathophysiology and Natural History
			4.2.4	 Antenatal Diagnosis
			4.2.5	 Antenatal Prediction of Prognosis
			4.2.6	 Obstetric Management
		4.3	 Sacrococcygeal Teratoma
			4.3.1	 Definition and Epidemiology
			4.3.2	 Genetics
			4.3.3	 Pathophysiology and Natural History
			4.3.4	 Antenatal Diagnosis
			4.3.5	 Antenatal Prediction of Prognosis
			4.3.6	 Obstetric Management
		4.4	 Fetal Congenital Thoracic Malformations
			4.4.1	 Definition and Epidemiology
			4.4.2	 Genetics
			4.4.3	 Pathophysiology and Natural History
			4.4.4	 Antenatal Diagnosis
			4.4.5	 Antenatal Prediction of Prognosis
			4.4.6	 Obstetric Management
		4.5	 Fetal Hydrothorax/Pleural Effusion
			4.5.1	 Definition and Epidemiology
			4.5.2	 Genetics
			4.5.3	 Pathophysiology and Natural History
			4.5.4	 Antenatal Diagnosis
			4.5.5	 Antenatal Prediction of Prognosis
			4.5.6	 Obstetric Management
		4.6	 Congenital Diaphragmatic Hernia
			4.6.1	 Definition and Epidemiology
			4.6.2	 Genetics
			4.6.3	 Pathophysiology and Natural History
			4.6.4	 Antenatal Diagnosis
			4.6.5	 Antenatal Prediction of Prognosis
			4.6.6	 Antenatal Management
			4.6.7	 Trials on FETO Versus Expectant Management
		4.7	 Fetal Cardiac Interventions
			4.7.1	 Definition and Epidemiology
			4.7.2	 Genetics
			4.7.3	 Pathophysiology and Natural History
			4.7.4	 Antenatal Diagnosis
			4.7.5	 Antenatal Prediction of Prognosis
			4.7.6	 Obstetric Management
		4.8	 Amniotic Band Syndrome
			4.8.1	 Definition and Epidemiology
			4.8.2	 Genetics
			4.8.3	 Pathophysiology and Natural History
			4.8.4	 Antenatal Diagnosis
			4.8.5	 Antenatal Prediction of Prognosis
			4.8.6	 Obstetric Management
		4.9	 In Conclusion: From Tinkering to Translation
		References
	5: How Pathology Helps the Neonatal Surgeon
		5.1	 Introduction
			5.1.1	 Biopsy Handling
			5.1.2	 Frozen Sections
			5.1.3	 Histochemistry
			5.1.4	 Immunohistochemistry
			5.1.5	 Molecular Techniques
			5.1.6	 Team Work
		5.2	 Developmental Disorders of the Gastrointestinal Tract
			5.2.1	 Oesophagus
				5.2.1.1	 Esophageal Atresia and Tracheoesophageal Fistula
				5.2.1.2	 Esophageal Diverticulum
				5.2.1.3	 Esophageal Stenosis and Webs
				5.2.1.4	 Duplication Cyst
			5.2.2	 Stomach
				5.2.2.1	 Gastric Duplication Cysts
				5.2.2.2	 Heterotopias
				5.2.2.3	 Hypertrophic Pyloric Stenosis
			5.2.3	 Small Intestine
				5.2.3.1	 Intestinal Atresia
				5.2.3.2	 Meckel’s Diverticulum and Related Vitello-Intestinal Remnants
				5.2.3.3	 Omphalomesenteric Cysts and Sinus
				5.2.3.4	 Duplication Cysts
			5.2.4	 Large Bowel
				5.2.4.1	 Anorectal Anomalies
				5.2.4.2	 Currarino Triad
		5.3	 Intestinal Obstruction
			5.3.1	 Intussusception
			5.3.2	 Volvulus
			5.3.3	 Meconium Ileus
		5.4	 Hisrchsprung Disease
			5.4.1	 Primary Diagnosis on Rectal Suction Biopsy
			5.4.2	 Assessment of Rectal Suction Biopsy Specimens for Hirschsprung Disease and Its Pitfalls
			5.4.3	 Short Segment Hirschsprung Disease
			5.4.4	 Ultra-short Segment Hirschsprung Disease
			5.4.5	 Intestinal Neuronal Dysplasia (IND)
				5.4.5.1	 Reporting on Frozen Sections of Intra-operative Biopsies
				5.4.5.2	 Evaluation of Resected Bowel
			5.4.6	 Procedures in Other Institutions
			5.4.7	 Assessment of Biopsies from Patients with Failed Pull-through Procedure for Hirschsprung’s Disease
			5.4.8	 Hirschsprung’s-Associated Enterocolitis (HAEC)
			5.4.9	 Hypoganglionosis
			5.4.10	 Megacystis Microcolon Intestinal Hypoperistalsis Syndrome
		5.5	 Necrotising Enterocolitis
		5.6	 Biliary Atresia and Choledochal Cyst
		5.7	 Congenital Neck Cysts in Infants
			5.7.1	 Thyroglossal Duct Cyst
			5.7.2	 Branchial Cleft Cysts
			5.7.3	 Dermoid Cyst
			5.7.4	 Bronchogenic Cyst
			5.7.5	 Cervical Thymic Cyst
		5.8	 Cystic Hygroma
		5.9	 Neonatal Lung Pathology
			5.9.1	 Congenital Lung Cysts
				5.9.1.1	 Congenital Pulmonary Airway Malformation
					Type 0 (Acinar Dysplasia)
				5.9.1.2	 Bronchial Atresia
				5.9.1.3	 Pulmonary Sequestration
				5.9.1.4	 Congenital Lobar Overinflation
				5.9.1.5	 Interstitial Emphysema
			5.9.2	 Alveolar Capillary Dysplasia
		5.10	 Genito-urinary Abnormalities
			5.10.1	 Autosomal Recessive Polycystic Kidney Disease
			5.10.2	 Nephronophthisis
			5.10.3	 Renal Dysplasia
			5.10.4	 Reflux Nephropathy
			5.10.5	 Testicular Torsion
			5.10.6	 Cryptorchid Testis
		5.11	 Neonatal Tumours
			5.11.1	 Teratoma
			5.11.2	 Neuroblastoma
			5.11.3	 Liver Tumours
			5.11.4	 Renal Tumours
			5.11.5	 Brain Tumours
			5.11.6	 Tumours of the Chest Wall and Lung
				5.11.6.1	 Congenital Peribronchial Myofibroblastic Tumor
				5.11.6.2	 Fetal Lung Interstitial Tumor
				5.11.6.3	 Pleuropulmonary Blastoma
				5.11.6.4	 Infantile Chest Wall Mesenchymal Hamartoma
			5.11.7	 Vascular Tumours
			5.11.8	 Rhabdomyosarcoma
				5.11.8.1	 Infantile Fibrosarcoma
				5.11.8.2	 Infantile Myofibromatosis
		5.12	 Autopsy
		References
	6: Developmental Physiology and Pharmacotherapy in Pediatric Surgical Newborns
		6.1	 Introduction
		6.2	 Drug Absorption
		6.3	 Drug Distribution
		6.4	 Drug Metabolism
		6.5	 Phase I Enzymes
			6.5.1	 CYP3A
			6.5.2	 CYP1A2
			6.5.3	 CYP2D6
			6.5.4	 CYP2C9/CYP2C19
			6.5.5	 CYP2E1
		6.6	 Drug Excretion
		6.7	 Other Factors Influencing the Absorption, Distribution, Metabolism and Excretion of Drugs in Neonates and Young Infants
		6.8	 Pharmacogenomics: Impact for Pediatric Populations
		6.9	 Developmental Pharmacodynamics
		References
	7: Transfer of the Surgical Neonate
		7.1	 Introduction
		7.2	 In Utero Transfer
		7.3	 Pre-Transfer Stabilisation
		7.4	 General Principles of Transfer
		7.5	 Mode of Transfer
		7.6	 Parents
		7.7	 Consent
		7.8	 Condition Specific Considerations
			7.8.1	 Gastroschisis
			7.8.2	 Oesophageal Atresia and Tracheo-Oesophageal Atresia
			7.8.3	 Congenital Diaphragmatic Hernia
			7.8.4	 Myelomeningocele
			7.8.5	 Intestinal Obstruction
		7.9	 Back Transfer of the Post-Operative Neonate
		References
	8: Fluid, Electrolyte and Nutritional Support of the Surgical Neonate
		8.1	 Introduction
		8.2	 Perinatal Changes in Body Composition
		8.3	 Energy Requirements of the Neonate
		8.4	 Energy Requirements of the Surgical and Septic Neonate
			8.4.1	 Perinatal Changes in Fluid Balance and Renal Function
			8.4.2	 Fluid and Electrolyte Requirements of the Neonate
			8.4.3	 Common Fluid and Electrolyte Disturbances and Their Treatment
				8.4.3.1	 Sodium
				8.4.3.2	 Potassium
				8.4.3.3	 Calcium
				8.4.3.4	 Magnesium
		8.5	 Acid-Base Balance
		8.6	 Parenteral Nutrition
			8.6.1	 Indications
			8.6.2	 Route of Administration
			8.6.3	 Components of Parenteral Nutrition
				8.6.3.1	 Fluid Requirements
				8.6.3.2	 Glucose
				8.6.3.3	 Lipids
				8.6.3.4	 Amino Acids
				8.6.3.5	 Minerals, Vitamins and Trace Elements
			8.6.4	 Complications of Parenteral Nutrition
				8.6.4.1	 Infectious Complications
				8.6.4.2	 Mechanical Complications
				8.6.4.3	 Hepatic Complications
		8.7	 Enteral Nutrition
			8.7.1	 Feeding Routes
			8.7.2	 Selection of Enteral Feeds
			8.7.3	 Administration of Enteral Feeds
			8.7.4	 Complications of Enteral Tube Feeding
		References
	9: Neonatal Vascular Access
		9.1	 Introduction
		9.2	 Historical Considerations
		9.3	 Commonplace Neonatal Vascular Access Procedures
			9.3.1	 Umbilical Venous and Arterial Cannulation
			9.3.2	 Percutaneous Central Venous Cannulation
			9.3.3	 Peripheral Arterial Catheterization
			9.3.4	 Surgical Venous Access
				9.3.4.1	 Pre-Operative Planning
				9.3.4.2	 Surgical Procedure: Open Technique
			9.3.5	 Percutaneous Versus Open Central Access
			9.3.6	 Complications of Venous Access
				9.3.6.1	 Catheter Infection
				9.3.6.2	 Occlusive Catheter Complications
				9.3.6.3	 Mechanical Complications
				9.3.6.4	 Anatomical Variations
				9.3.6.5	 Catheter Migration
				9.3.6.6	 Extravasation
		9.4	 Concluding Remarks
		References
	10: Radiology of Surgical Conditions in the Newborn
		10.1	 Care of Neonates Undergoing Radiological Investigations
		10.2	 Radiation Dose
		10.3	 Imaging Modalities
			10.3.1	 Ultrasound
			10.3.2	 Plain Radiography
			10.3.3	 Fluoroscopy
			10.3.4	 Computed Tomography (CT)
			10.3.5	 Magnetic Resonance Imaging (MRI)
			10.3.6	 Radio-Isotopes
			10.3.7	 Interventional Radiology
		10.4	 Positioning of Lines, Tubes and Catheters
			10.4.1	 Nasogastric Tubes
			10.4.2	 Endotracheal Tubes
		10.5	 The Neonate with Jaundice
			10.5.1	 Biliary Atresia and Neonatal Hepatitis
			10.5.2	 Choledochal Cysts
			10.5.3	 Inspissated Bile and Cholelithiasis
			10.5.4	 Spontaneous Perforation of the Bile Ducts
		10.6	 The Neonate with Failure to Feed/Inability to Pass NG Tube
			10.6.1	 Choanal Atresia
			10.6.2	 Oesophageal Atresia and Tracheo-Oesophageal Fistula
		10.7	 The Neonate with Vomiting or Suspected Gastro-­Oesophageal Reflux
			10.7.1	 Malrotation
			10.7.2	 Gastro-Oesophageal Reflux
			10.7.3	 Atresias and Stenoses of the Foregut and Midgut
			10.7.4	 Small Bowel Atresia
		10.8	 The Neonate with Abdominal Distention/Failure to Pass Meconium
			10.8.1	 Hirschsprung Disease
			10.8.2	 Unused Colon
			10.8.3	 Distal Small Bowel Atresia
			10.8.4	 Meconium Plug Syndrome
			10.8.5	 Meconium Ileus
			10.8.6	 Colonic Atresia
			10.8.7	 Anorectal Malformations
			10.8.8	 Necrotising Enterocolitis (NEC)
		10.9	 The Neonate with an  Abdominal or Pelvic Mass
			10.9.1	 Renal Masses in the Neonate
				10.9.1.1	 Hydronephrosis
				10.9.1.2	 Multicystic Dysplastic Kidney (MCDK)
				10.9.1.3	 Congenital Mesoblastic Nephroma (CMN)
				10.9.1.4	 Renal Vein Thrombosis
			10.9.2	 Ovarian Cyst
			10.9.3	 Duplication Cysts
			10.9.4	 Meconium Pseudocyst
			10.9.5	 Adrenal Masses
		10.10	 Neoplasms in the Neonate
			10.10.1	 Neuroblastoma
			10.10.2	 Liver Tumours
				10.10.2.1	 Hepatoblastoma
				10.10.2.2	 Hepatic Vascular Tumours
				10.10.2.3	 Mesenchymal Hamartoma
			10.10.3	 Intra-Abdominal Lymphatic Malformation
			10.10.4	 Presacral Masses
			10.10.5	 Sacrococcygeal Teratoma
			10.10.6	 Anterior Meningocoele
			10.10.7	 Enteric Cysts
		10.11	 The Neonate with Difficulty in Breathing/Chest Mass
			10.11.1	 Congenital Diaphragmatic Hernia
			10.11.2	 Congenital Airway Malformations
				10.11.2.1	 Pulmonary Sequestration
				10.11.2.2	 Congenital Lobar Emphysema (CLE)
				10.11.2.3	 Bronchogenic Cysts
				10.11.2.4	 Anomalous Pulmonary Venous Drainage
				10.11.2.5	 Pulmonary Underdevelopment
				10.11.2.6	 Pulmonary Artery Sling
				10.11.2.7	 Bronchial Atresia
				10.11.2.8	 Tracheo-Bronchomalacia
			10.11.3	 Chest Wall Masses
		10.12	 The Neonate with a Urinary Tract Infection
			10.12.1	 Vesico-Ureteric Reflux
			10.12.2	 Obstructive Uropathy/Bladder Outlet Obstruction
		10.13	 The Neonate with a Scrotal Swelling
			10.13.1	 Hydrocoeles
			10.13.2	 Hernia
			10.13.3	 Testicular Torsion
			10.13.4	 Infection
		10.14	 Urogenital Anomalies
			10.14.1	 Congenital Obstruction of the Genital Tract and Cloacal Anomalies
			10.14.2	 Disorders of Sexual Differentiation (DSD)/Ambiguous Genitalia
		10.15	 Increasing Head Circumference, Suspected Hydrocephalus and Intracranial Haemorrhage
		10.16	 Suspected Spinal Dysraphism/Sacral Dimple
		10.17	 The Neonate with a Neck Swelling
			10.17.1	 Congenital Cystic Lesions of the Neck
				10.17.1.1	 Thyroglossal Duct Cysts
				10.17.1.2	 Lymphatic Malformations
				10.17.1.3	 Branchial Cysts
				10.17.1.4	 Dermoid Cysts
				10.17.1.5	 Thymic Cysts
			10.17.2	 Jugular Varix
			10.17.3	 Benign Sternomastoid Tumour of Infancy (Fibromatosis Colli)
		10.18	 The Neonate with a Superficial Vascular Birthmark or Soft Tissue Swelling
			10.18.1	 Proliferative Haemangiomas (Common Infantile Haemangiomas)
			10.18.2	 Vascular Malformations
		References
	11: Anaesthesia for Neonatal Surgery
		11.1	 Introduction
		11.2	 History
		11.3	 Pharmacology in the Neonate
		11.4	 General and Developmental Pharmacology
		11.5	 Allometric Scaling
		11.6	 Pharmacogenetics and Pharmacogenomics
		11.7	 Inhalational Agents
		11.8	 Nitrous Oxide
		11.9	 Intravenous Agents
			11.9.1	 Thiopentone
			11.9.2	 Propofol
			11.9.3	 The Propofol Infusion Syndrome
			11.9.4	 Ketamine
			11.9.5	 Midazolam
			11.9.6	 Alpha-2-Adrenergic Agonists
			11.9.7	 Muscle Relaxants
			11.9.8	 Atracurium
			11.9.9	 Vecuronium and Rocuronium
		11.10	 Reversal Agents
			11.10.1	 Neostigmine
			11.10.2	 Sugammadex
		11.11	 Opioids and Other Analgesics
			11.11.1	 Morphine
			11.11.2	 Fentanyl
			11.11.3	 Remifentanil
			11.11.4	 Codeine and Tramadol
			11.11.5	 Paracetamol
			11.11.6	 Non-Steroidal Anti-
			11.11.7	 Local Anaesthetics
			11.11.8	 Topical Local Analgesia
		11.12	 Regional Analgesia Techniques
			11.12.1	 Caudal Blockade
			11.12.2	 Epidural Catheters Inserted Via the Caudal Route
			11.12.3	 Epidural Blockade
			11.12.4	 Transversus Abdominis Plane Block
			11.12.5	 Rectus Sheath Block
			11.12.6	 Ilio-Inguinal Block
			11.12.7	 Spinals
			11.12.8	 Paravertebral Block (PVB)
		11.13	 Vascular Access
			11.13.1	 Pre-Operative Assessment and Preparation
			11.13.2	 Intra-Operative Management
				11.13.2.1	 Induction of Anaesthesia
				11.13.2.2	 Intubation
				11.13.2.3	 The Role of Rapid Sequence Induction
				11.13.2.4	 Maintenance of  Anaesthesia and Analgesia
				11.13.2.5	 Ventilation
				11.13.2.6	 Temperature
				11.13.2.7	 Humidification
				11.13.2.8	 Fluids and Electrolytes
				11.13.2.9	 Blood Products
				11.13.2.10	 Glucose and Nutrition
				11.13.2.11	 Monitoring
				11.13.2.12	 Unexpected Events
			11.13.3	 Post-Operative Care
		11.14	 Specific Conditions
			11.14.1	 Congenital Diaphragmatic Hernia (CDH)
			11.14.2	 Oesophageal Atresia and Tracheo-­Oesophageal Fistula (OA/TOF)
			11.14.3	 Airway, Thoracic and Respiratory Problems
			11.14.4	 Abdominal Wall Defects
			11.14.5	 Bowel Obstructions
			11.14.6	 Coexistent Congenital Heart Defects
			11.14.7	 Persistent Pulmonary Hypertension of the Newborn (PPHN)
			11.14.8	 Prematurity and the NICU Graduate
			11.14.9	 Necrotising Enterocolitis (NEC)
		11.15	 Minimally Invasive Surgery
		11.16	 Controversies and Ethics
			11.16.1	 Neonatal Anaesthetic Neurotoxocity
			11.16.2	 Evidence
			11.16.3	 Treatment of Multiple Congenital Defects
		References
	12: Intensive Care and the Surgical Neonate
		12.1	 High Frequency Oscillatory Ventilation (HFOV)
		12.2	 Extra-Corporeal Membrane Oxygenation (ECMO)
		12.3	 Control of the Airway
		12.4	 Support for the  Cardiovascular System
		12.5	 Renal Support
			12.5.1	 Peritoneal Dialysis
			12.5.2	 Haemofiltration
		12.6	 Nutritional Support
		12.7	 Sedation
		12.8	 Some Aspects of Intensive Care for Particular Conditions in Neonatal Surgery
			12.8.1	 Necrotizing Enterocolitis (NEC)
			12.8.2	 Congenital Diaphragmatic Hernia (CDH)
			12.8.3	 Oesophageal Atresia/Tracheo-
			12.8.4	 Abdominal Wall Defects (Gastroschisis and Exomphalos)
		References
	13: Infections and Antibiotic Therapy in Surgical Newborn Infants
		13.1	 Pathogenesis of Infections: Gut Overgrowth
			13.1.1	 Introduction: Flora Development in the Neonate
			13.1.2	 Definition
			13.1.3	 Four Harmful Side-Effects
			13.1.4	 Risk Factors
				13.1.4.1	 Diagnosis
		13.2	 Prevention
			13.2.1	 Surgical Prophylaxis
			13.2.2	 Early Enteral Feeding
			13.2.3	 Enteral Antimicrobials: SDD
		13.3	 Treatment
		13.4	 Septicaemia
		13.5	 Wound Infection
		13.6	 Control of Antibiotic Resistance
		References
	14: Fetal Surgery
		14.1	 Rationale and Foundation for Fetal Surgery
		14.2	 Basic Considerations that are Common to all Open Fetal Surgery
			14.2.1	 Ethical Considerations and Preoperative Management
			14.2.2	 General Principles of Open Fetal Surgery
				14.2.2.1	 Personnel and Equipment
				14.2.2.2	 Anesthesia
				14.2.2.3	 Positioning and Draping
				14.2.2.4	 Incision and Exposure
				14.2.2.5	 Opening the Gravid Uterus
				14.2.2.6	 Closure of the Gravid Uterus
				14.2.2.7	 Tocolysis and Postoperative Care
		14.3	 Anatomic Anomalies Currently Treated by Open Fetal Surgery
			14.3.1	 Fetal Lung Lesions
				14.3.1.1	 Pathophysiology and Natural History
				14.3.1.2	 Fetal Intervention for Lung Lesions
			14.3.2	 Sacrococcygeal Teratoma
				14.3.2.1	 Pathophysiology and Natural History
				14.3.2.2	 Fetal Intervention for SCT
			14.3.3	 Fetal Myelomeningocele
				14.3.3.1	 Pathophysiology and Natural History
				14.3.3.2	 Fetal Intervention for MMC
			14.3.4	 Congenital Diaphragmatic Hernia
				14.3.4.1	 Pathophysiology and Natural History
				14.3.4.2	 Fetal Intervention for CDH
		14.4	 Other Invasive Maternal/Fetal Procedures
		14.5	 Efficacy and Future of Fetal Surgery
		References
	15: Minimal Access Neonatal Surgery
		15.1	 Physiological Considerations in Neonates Undergoing Minimal Access Surgery
		15.2	 Thoracic Procedures
			15.2.1	 Diaphragmatic Hernia
			15.2.2	 Oesophageal Atresia
			15.2.3	 Aortopexy
			15.2.4	 Lung Resection
			15.2.5	 Cyst Excision
			15.2.6	 Chylothorax
			15.2.7	 Mediastinal Masses
		15.3	 Abdominal Procedures
			15.3.1	 Pyloromyotomy
			15.3.2	 Fundoplication
			15.3.3	 Gastrostomy
			15.3.4	 Duodenal Atresia
			15.3.5	 Malrotation
			15.3.6	 Small Bowel Atresias and Duplications
			15.3.7	 Hirschsprung Disease
			15.3.8	 High Ano-Rectal Malformations
			15.3.9	 Necrotising Enterocolitis
			15.3.10	 Biliary Tract
			15.3.11	 Pancreas
			15.3.12	 Ovarian Cyst
			15.3.13	 Intersex
		15.4	 Tumour Surgery
			15.4.1	 Renal Tumours
			15.4.2	 Hepatic Tumours
			15.4.3	 Sacrococcygeal Teratoma
		15.5	 Inguinal Hernia
		References
	16: The Genetics of Neonatal Surgical Conditions
		16.1	 Introduction
		16.2	 DNA Structure and Genetic Function
		16.3	 The Objectives of Clinical Genetics
		16.4	 Syndromes, Sequences and Malformations
		16.5	 Developmental Genes and Malformations
		16.6	 Hox Homeobox Genes
		16.7	 SOX Genes
		16.8	 PAX Genes
		16.9	 Multi-factorial Conditions and Polygenic Inheritance
		16.10	 The Genetics of Hirschprung Disease
		16.11	 Investigation of Malformations and Syndrome Recognition
		16.12	 Methods of Genetic Investigation
		16.13	 Microarray Analysis
		16.14	 Molecular Genetic (DNA) Testing
		16.15	 Non-genetic Investigation of Malformations
		16.16	 Approaches to Genetic Counselling
		16.17	 Methods of Ante-natal Diagnosis
		16.18	 Approaches to and the Ethics of Pre-natal Diagnosis
		References
Part II: Trauma, Pierre Robin Sequence, and Twins
	17: Birth Trauma
		17.1	 Head Injuries
			17.1.1	 Superficial Injuries
			17.1.2	 Caput Succedaneum
			17.1.3	 Cephalohaematoma
			17.1.4	 Subgaleal Haemorrhage
			17.1.5	 Intracranial Haemorrhage
			17.1.6	 Nasal Injuries
			17.1.7	 Eye Injuries
		17.2	 Fractures
			17.2.1	 Skull Fractures
			17.2.2	 Clavicular Fracture
			17.2.3	 Long Bone Fractures
		17.3	 Nerve Injuries
			17.3.1	 Brachial Plexus Injury
			17.3.2	 Phrenic Nerve Injury
			17.3.3	 Facial Nerve Injury
			17.3.4	 Laryngeal Nerve Injury
		17.4	 Anoxic Injuries
		References
	18: Pierre Robin Sequence
		18.1	 What is Pierre Robin Sequence?
		18.2	 Aetiology
		18.3	 Genetics
		18.4	 Incidence
		18.5	 Antenatal Diagnosis
		18.6	 Clinical Presentation
		18.7	 Investigations
		18.8	 Management
		18.9	 Surgical Therapies
			18.9.1	 Tongue Lip Adhesion
			18.9.2	 Subperiosteal Release of the Floor of the Mouth
			18.9.3	 Mandibular Distraction Osteogenesis
			18.9.4	 Tracheostomy
		18.10	 Feeding and Growth
		18.11	 Natural History of PRS
		References
	19: Conjoined Twins
		19.1	 Symmetrical Conjoined Twins
			19.1.1	 History
			19.1.2	 Etiology and Embryology
			19.1.3	 Incidence
			19.1.4	 Classification
			19.1.5	 Diagnosis
				19.1.5.1	 Antenatal Diagnosis and Imaging
				19.1.5.2	 Fetal Ultrasound
				19.1.5.3	 Magnetic Resonance Imaging (MRI)
			19.1.6	 Postnatal Imaging
				19.1.6.1	 Ultrasound
				19.1.6.2	 Echocardiography (ECHO)
				19.1.6.3	 Computed Tomography (CT)
				19.1.6.4	 MRI
				19.1.6.5	 Contrast Studies of the Gastrointestinal and Genitourinary Tracts
				19.1.6.6	 Nuclear Medicine
			19.1.7	 Obstetric Management
			19.1.8	 Anaesthetic Management
			19.1.9	 Separation Procedure
			19.1.10 Postoperative Management
			19.1.11 Follow-Up
		19.2	 Ethics
		19.3	 Heteropagus (Parasitic) Twins
			19.3.1	 Definition
			19.3.2	 Incidence
			19.3.3	 Embryological Considerations
			19.3.4	 Anatomical Types
			19.3.5	 Diagnosis
			19.3.6	 Obstetric Care
			19.3.7	 Investigation
			19.3.8	 Surgery
			19.3.9	 Outcome
		References
Part III: Thorax and Cardiac Surgery
	20: Congenital Malformations of the Airway and Chest Wall
		20.1	 Introduction
		20.2	 Congenital Malformations of the Airway
			20.2.1	 History
			20.2.2	 Airway Embryology
			20.2.3	 Nomenclature
			20.2.4	 Congenital High Airway Obstruction Syndrome
			20.2.5	 Laryngeal Clefts
				20.2.5.1	 Associated Malformations
				20.2.5.2	 Classification
				20.2.5.3	 Management
			20.2.6	 Laryngeal Atresia
			20.2.7	 Tracheal Agenesis
			20.2.8	 Pulmonary Agenesis
			20.2.9	 Abnormalities of Bronchial Branching
			20.2.10 Bronchial Atresia
			20.2.11 Congenital Tracheal Stenosis
				20.2.11.1	 Classification
				20.2.11.2	 Associated Malformations
				20.2.11.3	 Assessment
				20.2.11.4	 Management
			20.2.12 Tracheomalacia
				20.2.12.1	 Classification and Aetiology
				20.2.12.2	 Associated Conditions
				20.2.12.3	 Clinical Features
				20.2.12.4	 Diagnosis and Assessment
				20.2.12.5	 Treatment
		20.3	 Congenital Malformations of the Chest Wall
			20.3.1	 Embryology
			20.3.2	 Sternal Cleft
			20.3.3	 Pentalogy of Cantrell
			20.3.4	 Pectus Excavatum
			20.3.5	 Pectus Carinatum
			20.3.6	 Jeune’s Syndrome (Asphyxiating Thoracic Dystrophy)
			20.3.7	 Ellis-van Creveld Syndrome
			20.3.8	 Jarcho-Levin Syndrome
			20.3.9	 Poland’s Syndrome
		20.4	 Acquired Chest Wall Deformity Secondary to Neonatal Thoracic Surgery
		References
	21: Extracorporeal Membrane Oxygenation
		21.1	 Introduction
			21.1.1	 History of ECMO
			21.1.2	 ECMO Outcomes
		21.2	 Neonatal Patient Selection Criteria
			21.2.1	 Reversible Disease Process
			21.2.2	 Gestastional Age
			21.2.3	 Birth Weight
			21.2.4	 Hemorrhage and Coagulopathy Complications
			21.2.5	 Intracranial Hemorrhage
			21.2.6	 Coexisting Anomalies
			21.2.7	 Bridge to Diagnosis
			21.2.8	 Failure of Medical Management and Cardiopulmonary Criteria
			21.2.9	 Congenital Diaphragmatic Hernia
		21.3	 Methods of Extracorporeal Support
			21.3.1	 Veno-Venous ECMO
			21.3.2	 Veno-Arterial ECMO
		21.4	 ECMO Circuit and Cannulation
			21.4.1	 ECMO Circuit
			21.4.2	 Cannulation and Anesthesia
			21.4.3	 Patient Position and Incision
			21.4.4	 Dissection and Vessel Exposure
			21.4.5	 Cannulation
		21.5	 Clinical Management of Extracorporeal Support
			21.5.1	 Cannula Management
			21.5.2	 Prime Management
			21.5.3	 Pump Management
			21.5.4	 Oxygenator Management
			21.5.5	 Volume Management
			21.5.6	 Ventilator Management
			21.5.7	 Medical Management
			21.5.8	 Coagulation Management
			21.5.9	 Hemodynamic Management
			21.5.10 Temperature Management
			21.5.11 Pharmacology Management
			21.5.12 Surgical Procedures on ECMO
			21.5.13 ECMO Weaning and Decannulation
		21.6	 Complications on ECMO
			21.6.1	 Hemorrhagic and Thrombotic Complications
			21.6.2	 Mechanical Complications
			21.6.3	 Neurologic Complications
			21.6.4	 Renal Complications
		21.7	 ECMO and Congenital Diaphragmatic Hernia
			21.7.1	 Surgical Repair CDH
			21.7.2	 CDH and ECMO Selection
		21.8	 Results and Outcomes
			21.8.1	 Survival
			21.8.2	 Feeding and Growth
			21.8.3	 Respiratory
			21.8.4	 Neurologic
		21.9	 Summary
		References
	22: Congenital Lung Malformations
		22.1	 Introduction
		22.2	 Embryology
		22.3	 Etiology and Animal Models of Cystic Lung Lesions
		22.4	 Prenatal and Neonatal Diagnosis and Imaging
		22.5	 Congenital Pulmonary Airway Malformations
			22.5.1	 Prenatal Management and Fetal Surgical Considerations
			22.5.2	 Postnatal Management and Surgical Considerations
			22.5.3	 Surgical Approach
		22.6	 Bronchopulmonary Sequestration
		22.7	 Congenital Lobar Emphysema
		22.8	 Bronchogenic Cysts and Foregut Duplication
		References
	23: Esophageal Atresia and Tracheo-­Esophageal Fistula
		23.1	 Introduction
		23.2	 History
		23.3	 Classification
		23.4	 Risk Categorization
		23.5	 Epidemiology
		23.6	 Genetics
		23.7	 Animal Models
		23.8	 Embryology
		23.9	 Associated Anomalies
		23.10	 Antenatal Diagnosis
		23.11	 Clinical Presentation
		23.12	 Surgical Management
		23.13	 Upper Pouch Fistula
		23.14	 Long-Gap EA with Distal TEF
		23.15	 Right-Sided Aortic Arch
		23.16	 Premature Infant with RDS
		23.17	 Postoperative Care
		23.18	 Surgical Management of Isolated (‘Pure’) Esophageal Atresia
		23.19	 H-Type TEF
		23.20	 EA TEF—Complications and Special Considerations
			23.20.1 Anastomotic Leak
			23.20.2 Gastroesophageal Reflux Disease—GERD
			23.20.3 Anastomotic Stricture
			23.20.4 Tracheomalacia
			23.20.5 Recurrent TEF
		23.21	 EA TEF Advances
		23.22	 Quality of Life and Long-­Term Outcome
		References
	24: Congenital Esophageal Pathology
		24.1	 Congenital Esophageal Stenosis
			24.1.1	 Introduction
			24.1.2	 Presentation
			24.1.3	 Diagnosis
			24.1.4	 Treatment
			24.1.5	 Outcome
		24.2	 Esophageal Duplication Cysts
			24.2.1	 Introduction
			24.2.2	 Presentation
			24.2.3	 Diagnosis
			24.2.4	 Treatment
			24.2.5	 Outcome
		24.3	 Esophageal Perforation
			24.3.1	 Introduction
			24.3.2	 Presentation
			24.3.3	 Diagnosis
			24.3.4	 Treatment
			24.3.5	 Outcome
		24.4	 Achalasia
			24.4.1	 Introduction
			24.4.2	 Presentation
			24.4.3	 Diagnosis
			24.4.4	 Treatment
			24.4.5	 Outcome
		References
	25: Gastroesophageal Reflux in Newborns and Premature Infants
		25.1	 Introduction
		25.2	 History
		25.3	 Embryology
		25.4	 Functional Anatomy of the Anti-Reflux Barrier
		25.5	 Epidemiology
		25.6	 Genetics
		25.7	 Clinical Presentation and Diagnosis
		25.8	 When to Perform Diagnostic Tests
		25.9	 Co-Morbidities in Newborns and Prematures
			25.9.1	 Severe Respiratory Tract Disease and GERD
			25.9.2	 GERD in Neurologically Impaired Babies
			25.9.3	 GERD After Treatment of Esophageal Atresia and Tracheo-Esophageal Fistula
			25.9.4	 GERD After Treatment of Congenital Diaphragmatic Hernia (CDH)
			25.9.5	 GERD After Treatment of Anterior Abdominal Wall Defects (AAWD)
		25.10	 Treatment
		References
	26: Congenital Diaphragmatic Hernia and Eventration
		26.1	 Pathology
		26.2	 Antenatal Diagnosis
		26.3	 Associated Anomalies
		26.4	 Predicting Outcome
		26.5	 Fetal Intervention
		26.6	 Newborn Management
			26.6.1	 Delivery
			26.6.2	 Postnatal Diagnosis—‘Late Presenting CDH’
			26.6.3	 Stabilisation
				26.6.3.1	 Permissive Hypercapnea/‘Gentle’ Ventilation Strategy
				26.6.3.2	 ECMO, Nitric Oxide, Sildenafil
			26.6.4	 Surgery
			26.6.5	 Morbidity
		26.7	 Outcome(s) and Long Term Follow Up
			26.7.1	 Respiratory Function
			26.7.2	 Gastroesophageal Reflux
			26.7.3	 Neurodevelopmental
		26.8	 Lesser Variants—Morgagni Hernia, Pentalogy of Cantrell
		26.9	 Diaphragm Eventration
		References
	27: Chylothorax and Other Pleural Effusions
		27.1	 Introduction
		27.2	 Epidemiology
		27.3	 Anatomy and Physiology
		27.4	 Aetiology
		27.5	 Antenatal Presentation
		27.6	 Postnatal Clinical Presentation
		27.7	 Diagnosis
		27.8	 Management
		27.9	 Prognosis
		References
	28: Congenital Cardio Thoracic Surgery
		28.1	 Critical Congenital Heart Disease
		28.2	 Introduction to Embryology and Functional Classification of Cardiac Lesions
			28.2.1	 Cardiac Septation
		28.3	 Cardio Pulmonary Bypass in Congenital Patients
			28.3.1	 Technology
			28.3.2	 Technique
		28.4	 Pre and Peri Operative Management of Congenital Heart Disease Patients
			28.4.1	 Pre Operative Multi Disciplinary Team (MDT)
			28.4.2	 Unique Features for Management of Congenital Cardiac Surgical Patients in ICU
		28.5	 Pathophysiology of Congenital Heart Surgery Patients
			28.5.1	 Physiology of Congenital Heart Disease
		28.6	 Classification of Congenital Heart Disease
		28.7	 Atrial Septal Defects (ASD)
			28.7.1	 Introduction
			28.7.2	 Embryology and Genetics
			28.7.3	 Types
			28.7.4	 Natural History and Pathophysiology
			28.7.5	 Clinical Features and Diagnosis
			28.7.6	 Diagnosis
			28.7.7	 Management
			28.7.8	 Outcome and Follow Up
		28.8	 Ventricular Septal Defect
			28.8.1	 Introduction
			28.8.2	 Classification (Fig. 28.1)
			28.8.3	 Pathophysiology
			28.8.4	 Natural History
			28.8.5	 Clinical Features and Diagnosis
			28.8.6	 Surgical Management
		28.9	 Tetralogy of Fallot
			28.9.1	 Introduction
			28.9.2	 Embryology and Genetics
			28.9.3	 Anatomy and Pathophysiology
			28.9.4	 Natural History
			28.9.5	 Clinical Features and Diagnosis
			28.9.6	 Management Including Surgical Principles
			28.9.7	 Follow Up and Outcomes
		28.10	 Atrio Ventricular Septal Defects
			28.10.1	 Introduction
			28.10.2	 Embryology and Genetics
			28.10.3	 Classification
			28.10.4	 Pathophysiology
			28.10.5	 Natural History
			28.10.6	 Clinical Features and Diagnosis
			28.10.7	 Management Including Surgical Principles
			28.10.8	 Follow Up and Outcome
		28.11	 Transposition of Great Arteries (TGA)
			28.11.1	 Introduction
			28.11.2	 Embryology and Genetics
			28.11.3	 Pathophysiology
			28.11.4	 Natural History
			28.11.5	 Clinical Features and Diagnosis
			28.11.6	 Management Including Surgical Principles
			28.11.7	 Follow Up and Outcome
		28.12	 Hypoplastic Left Heart Syndrome
			28.12.1	 Introduction
			28.12.2	 Embryology and Genetics
			28.12.3	 Pathophysiology
			28.12.4	 Clinical Features and Diagnosis
			28.12.5	 Natural History
			28.12.6	 Management Including Surgical Principles
			28.12.7	 Follow Up and Outcome
		28.13	 Total Anomalous Pulmonary Venous Connection (TAPVC)
		28.14	 Truncus Arteriosus
		References
Part IV: Gastrointestinal System
	29: Inguinal Hernia
		29.1	 Introduction
		29.2	 Etiology
		29.3	 Epidemiology
		29.4	 Associated Conditions with an Increased Incidence of Inguinal Hernia
		29.5	 Clinical Presentation
		29.6	 Management
		29.7	 Anaesthesia
		29.8	 Operative Technique
			29.8.1	 Males
			29.8.2	 Females
			29.8.3	 Laparoscopic Repair
		29.9	 Incarcerated Inguinal Hernia
		29.10	 Management of an Incarcerated Inguinal Hernia
		29.11	 Sliding Hernia and Atypical Herniated Organs
		29.12	 Direct Hernia and Femoral Hernia
		29.13	 Contralateral Exploration and Metachronous Hernia
		29.14	 Differential Diagnostics
			29.14.1 Hydrocele
			29.14.2 Testicular Torsion
			29.14.3 Inguinal Lymphadenitis
		29.15	 Complications
			29.15.1 Iatrogenic Ascent of the Testis
			29.15.2 Injury to Reproductive Organs
		References
	30: Gastric Outlet Obstruction
		30.1	 Introduction
		30.2	 Infantile Hypertrophic Pyloric Stenosis (IHPS)
		30.3	 Incidence
		30.4	 Aetiology
			30.4.1	 General Considerations
			30.4.2	 Genetic Factors
			30.4.3	 Environmental Factors
			30.4.4	 Hormonal Factors
			30.4.5	 Histological Anomalies
			30.4.6	 Pyloric Innervation
		30.5	 Clinical Features
		30.6	 Diagnosis
			30.6.1	 Clinical Examination
			30.6.2	 Biochemistry
			30.6.3	 Radiology
			30.6.4	 Other Diagnostic Testing
		30.7	 Management
			30.7.1	 Pre-operative
			30.7.2	 Operative Considerations
			30.7.3	 Postoperative Management
			30.7.4	 Outcomes and Complications
			30.7.5	 Intra-operative Complications
			30.7.6	 Post-operative
		30.8	 Other Treatment Options
		30.9	 Other Causes of Outlet Obstruction
		References
	31: Duodenal Atresia and Stenosis
		31.1	 Duodenal Atresia and Stenosis
		31.2	 Etiology
		31.3	 Classification
		31.4	 Postnatal Presentation
		31.5	 Prenatal Diagnosis
		31.6	 Postnatal Management
		31.7	 Outcomes
		References
	32: Malrotation and Volvulus
		32.1	 Introduction
			32.1.1	 Definition
			32.1.2	 Significance
		32.2	 Embryology and Pathology
			32.2.1	 Normal Rotation and Fixation of the Midgut
				32.2.1.1	 Development of Dorsal Mesentery
				32.2.1.2	 Herniation
				32.2.1.3	 Rotation
				32.2.1.4	 Return to the Abdominal Cavity
				32.2.1.5	 Fixation
			32.2.2	 Abnormal Embryology
			32.2.3	 Non Rotation
			32.2.4	 Failure of Rotation of Pre-­arterial Segment of Midgut
			32.2.5	 Reverse Rotation
			32.2.6	 Failure of Rotation of the Post-­arterial Segment of Midgut
		32.3	 Associated Abnormalities
		32.4	 Incidence
		32.5	 Age At Presentation
		32.6	 Presentation (Table 32.4)
			32.6.1	 Antenatal Diagnosis
			32.6.2	 Presentation in the Neonatal Period
			32.6.3	 Presentation in Older Children
			32.6.4	 Approach to Clinical Assessment
		32.7	 Investigation/Imaging
			32.7.1	 Plain Radiology
				32.7.1.1	 Diagnostic Features
				32.7.1.2	 Limitations
				32.7.1.3	 Role
			32.7.2	 Upper Gastrointestinal Contrast Study
				32.7.2.1	 Diagnostic Features
				32.7.2.2	 Limitations
				32.7.2.3	 Role
			32.7.3	 Distal Gastrointestinal Contrast Study
				32.7.3.1	 Diagnostic Features
				32.7.3.2	 Limitations
				32.7.3.3	 Role
			32.7.4	 Ultrasonography
				32.7.4.1	 Diagnostic Features
				32.7.4.2	 Limitations
				32.7.4.3	 Role/Indications
			32.7.5	 CT Scan
				32.7.5.1	 Diagnostic Features
				32.7.5.2	 Limitations
				32.7.5.3	 Role
			32.7.6	 MRI
		32.8	 Management
			32.8.1	 Initial Resuscitation (Table 32.7)
			32.8.2	 Indications for Surgery
			32.8.3	 Surgical Approach
				32.8.3.1	 Open Approach
				32.8.3.2	 Laparoscopic Approach
			32.8.4	 Initial Operative Assessment
				32.8.4.1	 Deliver Bowel
				32.8.4.2	 Untwist the Small Bowel Mesentery
				32.8.4.3	 Assess Bowel Viability
				32.8.4.4	 Broaden the Mesentery
				32.8.4.5	 “en passant” Appendicectomy
		32.9	 Complications and Results (Table 32.8)
			32.9.1	 Early Complications
			32.9.2	 Late Results and Complications
			32.9.3	 Mortality
		32.10	 The Future
			32.10.1 Genetics of Malrotation
			32.10.2 Antenatal Intervention
			32.10.3 Management of Short Gut Syndrome
		References
	33: Jejuno-Ileal Atresia and Stenosis
		33.1	 Introduction
		33.2	 History
		33.3	 Classification
			33.3.1 Prognosis
		33.4	 Epidemiology
		33.5	 Aetiology and Genetics
		33.6	 Associated Anomalies
		33.7	 Antenatal Presentation
		33.8	 Clinical Presentation and Diagnosis
		33.9	 Surgical Management
		33.10	 Complications and Special Considerations
			33.10.1 Outcome
		33.11	 Quality of Life and Long-­Term Outcome
		References
	34: Duplications of the Alimentary Tract
		34.1	 Introduction
		34.2	 Definition, Etiology, Epidemiology and Anatomical Characteristics
		34.3	 Clinical Presentation
		34.4	 Diagnosis
			34.4.1 Duplications of Oropharynx
			34.4.2 Cervical Oesophageal Duplications
			34.4.3 Thoracic and Thoracoabdominal Duplications
			34.4.4 Thoracoabdominal Duplications
			34.4.5 Gastric Duplications
			34.4.6 Duplications of the Duodenum and the Pancreas
			34.4.7 Duplicated Liver, Common Bile Duct and Gallbladder
			34.4.8 Duplications of the Small Bowel
			34.4.9 Duplications of the Colon and Rectum
			34.4.10 Duplication of the Anal Canal
		34.5	 Complications and Long-­Term Results
		References
	35: Meconium Ileus
		35.1	 History
		35.2	 Definition and Classification
		35.3	 Epidemiology
		35.4	 Etiology and Pathophysiology
		35.5	 Diagnosis
			35.5.1	 Prenatal Detection
			35.5.2	 Postnatal Presentation
			35.5.3	 Imaging
			35.5.4	 Laboratory Test
		35.6	 Differential Diagnosis
			35.6.1	 Intestinal Atresia
			35.6.2	 Midgut Volvulus
			35.6.3	 Hirschsprung Disease
			35.6.4	 Meconium Plug Syndrome and Neonatal Small Left Colon
			35.6.5	 Hypothyroidism
			35.6.6	 Prematurity
		35.7	 Treatment
			35.7.1	 Nonoperative Management (Simple MI)
			35.7.2	 Operative Management 1 (Unresponsive to Medical Treatment)
			35.7.3	 Operative Management 2 (Complicated MI)
		35.8	 Outcomes
		References
	36: Ascites in the Newborn
		36.1	 Overview
			36.1.1	 Background
			36.1.2	 Clinical Presentation
			36.1.3	 Investigations
		36.2	 Fetal Ascites
		36.3	 Neonatal Ascites
			36.3.1	 Chylous Ascites
				36.3.1.1	 Aetiology
				36.3.1.2	 Diagnosis
				36.3.1.3	 Management
			36.3.2	 Biliary Ascites
				36.3.2.1	 Aetiology
				36.3.2.2	 Diagnosis
				36.3.2.3	 Management
			36.3.3	 Urinary Ascites
				36.3.3.1	 Aetiology
				36.3.3.2	 Diagnosis
				36.3.3.3	 Management
		References
	37: Neonatal Bowel Obstruction
		37.1	 Introduction
		37.2	 Key Features
			37.2.1	 Vomiting
			37.2.2	 Abdominal Tenderness and Distension
			37.2.3	 Constipation
			37.2.4	 Abdominal Radiology
		37.3	 Management
		37.4	 Summary
		References
	38: Necrotising Enterocolitis
		38.1	 Introduction
		38.2	 Pathogenesis and Risk Factors
			38.2.1	 Peripartum Events
			38.2.2	 Feeding Regimen
			38.2.3	 Altered Blood Supply
			38.2.4	 Bacterial Involvement
		38.3	 Prevention
		38.4	 Clinical Features and Diagnosis
		38.5	 Staging
		38.6	 Clinical Management
			38.6.1	 Medical Management
			38.6.2	 Surgical Management
		38.7	 Outcome
		References
	39: Neonatal Intestinal Failure and Transplantation
		39.1	 Introduction
		39.2	 Epidemiology and Mortality
		39.3	 Definition
		39.4	 Causes of Intestinal Failure
		39.5	 Pathophysiology
			39.5.1	 Adaptation
			39.5.2	 Bacterial Overgrowth
			39.5.3	 Intestinal Failure Associated Liver Disease
		39.6	 Management of Intestinal Failure
			39.6.1	 Parenteral Nutrition
			39.6.2	 Catheter-Related Sepsis and Thrombosis
			39.6.3	 Enteral Nutrition and Medical Treatment
			39.6.4	 Surgical Principles and Initial Operative Management
			39.6.5	 Autologous Intestinal Reconstruction
		39.7	 Transplantation
			39.7.1	 Indications and Timing
			39.7.2	 Type of Transplant and Technical Considerations
			39.7.3	 Outcomes
		References
	40: Hirschsprung’s Disease
		40.1	 Introduction
			40.1.1	 Historical Background
			40.1.2	 Classification
			40.1.3	 Epidemiology
		40.2	 Etiology
			40.2.1	 Failure of Neural Crest Cell Migration
			40.2.2	 Genetic Factors
		40.3	 Pathophysiology
			40.3.1	 Cholinergic Hyperinnervation
			40.3.2	 Adrenergic Innervation
			40.3.3	 Nitrergic Innervation
			40.3.4	 Interstitial Cells of Cajal
			40.3.5	 Enteroendocrine Cells
			40.3.6	 Smooth Muscle Cells
			40.3.7	 Extracellular Matrix
		40.4	 Pathology
		40.5	 Diagnosis
			40.5.1	 Clinical Features
			40.5.2	 Radiological Evaluation
			40.5.3	 Anorectal Manometry
			40.5.4	 Rectal Biopsy
			40.5.5	 Differential Diagnosis
		40.6	 Surgical Management
			40.6.1	 Preoperative Management
			40.6.2	 Transanal One-Stage Endorectal Pull-through Operation
				40.6.2.1	 Operative Technique
			40.6.3	 Postoperative Complications
				40.6.3.1	 Anastomotic Leakage
				40.6.3.2	 Retraction of Pull-through
				40.6.3.3	 Perianal Excoriation
				40.6.3.4	 Constipation
				40.6.3.5	 Enterocolitis
				40.6.3.6	 Fecal Soiling
		40.7	 Long-Term Outcome
		References
	41: Anorectal Malformations
		41.1	 Males
			41.1.1	 Rectoperineal Fistula
			41.1.2	 Rectobulbar and Rectoprostatic Urethral Fistulae
			41.1.3	 Rectobladderneck Fistula
			41.1.4	 Imperforate Anus with No Fistula
		41.2	 Females
			41.2.1	 Rectoperineal Fistula
			41.2.2	 Rectovestibular Fistula
			41.2.3	 Cloaca
			41.2.4	 Imperforate Anus with No Fistula
			41.2.5	 Rectal Atresia and Stenosis
		41.3	 Colostomy
		41.4	 Primary Repair for Females
		41.5	 Primary Repair in Males
		References
Part V: Liver, Biliary Tract, Pancreas
	42: Biliary Atresia
		42.1	 History
		42.2	 Introduction
		42.3	 Pathophysiology
			42.3.1	 Etiological Heterogeneity
			42.3.2	 Biliary Atresia Splenic Malformation (BASM) Syndrome
			42.3.3	 Pathology
			42.3.4	 Viruses and Biliary Atresia
			42.3.5	 (Auto)immune Response and Inflammation in Biliary Atresia
			42.3.6	 Biliatresone
		42.4	 Clinical Features
			42.4.1	 Diagnostic Workup
			42.4.2	 Screening for Biliary Atresia
		42.5	 Surgery: Kasai Portoenterostomy
			42.5.1	 Options and Alternatives
			42.5.2	 Adjuvant Therapy for Biliary Atresia
				42.5.2.1	 Corticosteroids
				42.5.2.2	 Ursodeoxycholic Acid (UDCA)
			42.5.3	 Post-operative Complications
				42.5.3.1	 Cholangitis
				42.5.3.2	 Portal Hypertension and Oesophageal Varices
				42.5.3.3	 Ascites
		42.6	 Outcome and Results
			42.6.1	 Prognostic Factors
				42.6.1.1	 Age at Kasai Portoenterostomy
				42.6.1.2	 Surgical Experience
				42.6.1.3	 Liver Histology and Biliary Remnant
		References
	43: Choledochal Cyst
		43.1	 Introduction
		43.2	 Classification
		43.3	 Prognosis
		43.4	 Prenatal Diagnosis
		43.5	 Clinical Presentation
		43.6	 Diagnostic Images
			43.6.1	 Ultrasonography
			43.6.2	 CT
			43.6.3	 ERCP
			43.6.4	 MRCP
		43.7	 Surgical Management
		43.8	 Special Considerations
			43.8.1	 Carcinogenesis
			43.8.2	 Pancreaticobiliary Maljunction Without Choledochal Cyst
		43.9	 Long Term Results
		43.10	 Laparoscopic Surgery
		References
	44: Spontaneous Biliary Perforation, Liver Cysts, and Abscesses
		44.1	 Spontaneous Perforation of the Bile Duct
			44.1.1	 History
			44.1.2	 Anatomy and Pathogenesis
			44.1.3	 Clinical Features
			44.1.4	 Investigations
			44.1.5	 Management
			44.1.6	 Complications
		44.2	 Parenchymal Liver Cysts
			44.2.1	 Management
		44.3	 Parenchymal Liver Abscess
			44.3.1	 Management
		References
	45: Surgery for Congenital Hyperinsulinism
		45.1	 Introduction
		45.2	 History
		45.3	 Pathophysiology
		45.4	 Diagnosis
		45.5	 Classification
			45.5.1	 Histological Classification
			45.5.2	 Therapeutic Classification
		45.6	 Genetics
		45.7	 Prenatal Diagnosis and Counseling
		45.8	 Medical Management
		45.9	 Preoperative Management
		45.10	 Imaging Studies
		45.11	 Surgical Management
		45.12	 Postoperative Management
		45.13	 Postoperative Complications
		45.14	 Long-Term Outcomes After Surgery
		References
Part VI: Abdominal Wall Defects
	46: Gastroschisis and Exomphalos
		46.1	 History
		46.2	 Epidemiology
		46.3	 Embryology
		46.4	 Prenatal Diagnosis
		46.5	 Newborn Management
		46.6	 Surgical Management
		46.7	 Long Term Outcome(s)
		References
	47: Omphalomesenteric Duct and Urachal Remnants
		47.1	 Introduction
		47.2	 Omphalomesenteric Duct Remnant
			47.2.1	 History
			47.2.2	 Epidemiology
			47.2.3	 Embryology
			47.2.4	 Anatomy
			47.2.5	 Clinical Presentation
			47.2.6	 Management
			47.2.7	 Incidental Finding of Meckel’s Diverticulum
		47.3	 Urachal Abnormalities
			47.3.1	 Epidemiology
			47.3.2	 Embryology
			47.3.3	 Anatomy
			47.3.4	 Clinical Presentation
			47.3.5	 Management
		References
	48: The Exstrophy Complex: Bladder and Cloacal Exstrophy
		48.1	 Introduction
		48.2	 History
		48.3	 Classification
		48.4	 Prognosis
		48.5	 Epidemiology
		48.6	 Genetics
		48.7	 Animal Models
		48.8	 Embryology
		48.9	 Associated Anomalies
			48.9.1 Bladder Exstrophy
			48.9.2 Cloacal Exstrophy
		48.10	 Antenatal Presentation
			48.10.1 Bladder Exstrophy
			48.10.2 Cloacal Exstrophy
		48.11	 Clinical Presentation, Diagnosis, and Postnatal Care
		48.12	 Surgical Management of Bladder Exstrophy
			48.12.1 Primary Bladder Closure
			48.12.2 Pelvic Osteotomy
			48.12.3 Bladder, Posterior Urethral, and Abdominal Wall Closure
			48.12.4 Combined Bladder Closure and Epispadias Repair
			48.12.5 Radical Soft Tissue Mobilization (The Kelly Repair)
		48.13	 Surgical Management of Cloacal Exstrophy
		48.14	 Complications and Special Considerations
			48.14.1 Primary Bladder Closure Failure
			48.14.2 Genitourinary Soft Tissue Loss
			48.14.3 Bowel Complications in Cloacal Exstrophy Reconstruction
		48.15	 Quality of Life and Long-­Term Outcomes
		References
Part VII: Nervous System
	49: Hydrocephalus
		49.1	 Introduction
			49.1.1	 CSF Physiology
			49.1.2	 Classification
			49.1.3	 New Concepts
		49.2	 Causes of Hydrocephalus
			49.2.1	 Post-haemorrhagic
			49.2.2	 Post-infectious
			49.2.3	 Chiari Malformation and Spina Bifida
			49.2.4	 Aqueduct Stenosis
			49.2.5	 Dandy Walker Complex
			49.2.6	 Craniosynostosis
			49.2.7	 Uncommon Congenital Malformations
				49.2.7.1	 Encephalocoele
				49.2.7.2	 Agenesis of Corpus Callosum
				49.2.7.3	 Hydrancephaly
				49.2.7.4	 Holoprosencephaly
			49.2.8 Neoplasms
			49.2.9 External Hydrocephalus
			49.2.10 Overproduction
		49.3	 Clinical Presentation of Hydrocephalus in Neonates
		49.4	 Imaging and Investigations
		49.5	 Management Options
			49.5.1	 External Ventricular Drainage
			49.5.2	 Subcutaneous Reservoir Ventricular Catheter Placement
			49.5.3	 Ventriculoperitoneal Shunt
				49.5.3.1	 Overview
				49.5.3.2	 Surgical Technique
				49.5.3.3	 Image Guided Placement of Ventricular Catheter
				49.5.3.4	 Complications
			49.5.4	 Ventriculoatrial Shunt
			49.5.5	 Ventriculopleural Shunt
			49.5.6	 Post Operative Care of Shunted Patients
			49.5.7	 Endoscopic Third Ventriculostomy
				49.5.7.1	 Indications
				49.5.7.2	 Surgical Technique
			49.5.8	 Fetal Surgical Therapy
		49.6	 Outcome
		49.7	 Ongoing Research
		References
	50: Neural Tube Defects
		50.1	 Introduction
		50.2	 Embryology and Pathogenesis
		50.3	 Aetiology
			50.3.1	 Nutritional Factors
			50.3.2	 Genetics
			50.3.3	 Teratogens
		50.4	 Historical
		50.5	 Classification and Types
		50.6	 Antenatal Diagnosis
		50.7	 Initial Management
		50.8	 Operative Details of Closure (As per Myelomeningocoele)
		50.9	 Post-Operatively
		50.10	 Other Considerations
		50.11	 Outcome
		50.12	 Summary
		References
	51: Neonatal Brain Tumours
		51.1	 Epidemiology and Aetiology
		51.2	 Causative Factors
		51.3	 Clinical Presentation
		51.4	 Neuroimaging
		51.5	 Surgical Management
		51.6	 Adjuvant Therapies
		51.7	 Chemotherapy
		51.8	 Multidisciplinary Management
		51.9	 Genetic and Biological Characterisation of Tumours and Risk Stratification
		51.10	 Prognosis/Survival/Outcomes
		51.11	 Low Grade Glioma (LGG) and High Grade Glioma (HGG)
		51.12	 High Grade Glioma (HGG)
		51.13	 Medulloblastoma
		51.14	 Central Nervous System Primitive Neuroectodermal Tumours (CNS-PNET)
		51.15	 Atypical Teratoid/Rhabdoid Tumour (AT/RT)
		51.16	 Teratoma
		51.17	 Ependymoma
		51.18	 Choroid Plexus Tumours (CPT)
		51.19	 Desmoplastic Infantile Astroglial Tumours
		51.20	 Discussion
		References
Part VIII: Oncology
	52: Epidemiology and Genetics of Neonatal Tumours
		52.1	 Incidence
		52.2	 Genetic and Familial Associations
		52.3	 Other Birth Characteristics
		52.4	 Exogenous Risk Factors
		52.5	 Survival
		References
	53: Vascular Anomalies
		53.1	 Introduction
		53.2	 Vascular Tumors
			53.2.1	 Infantile Hemangioma
				53.2.1.1	 Etiology and Pathogenesis
				53.2.1.2	 Associated Structural Abnormalities
				53.2.1.3	 Radiologic Features
				53.2.1.4	 Treatment
					Ulceration
					Pharmacotherapy
					Embolic Therapy
					Laser Therapy
					Surgical Therapy
			53.2.2	 Congenital Hemangioma
			53.2.3	 Hepatic Hemangioma
			53.2.4	 Pyogenic Granuloma
			53.2.5	 Kaposiform Hemangioendothelioma and Kasabach-Merritt Phenomenon
		53.3	 Vascular Malformations
			53.3.1	 Embryology and Development of the Vascular and Lymphatic Systems
			53.3.2	 Capillary Malformations
				53.3.2.1	 Cutis Marmorata Telangiectasia Congenita
				53.3.2.2	 Telangiectasia
			53.3.3	 Venous Malformation
				53.3.3.1	 Treatment
			53.3.4	 Arteriovenous Malformations
				53.3.4.1	 Treatment
			53.3.5	 Complex-Combined Vascular Malformations
				53.3.5.1	 Capillary-Lymphaticovenous Malformation
					Treatment
				53.3.5.2	 CLOVES Syndrome
				53.3.5.3	 Parkes Weber Syndrome
				53.3.5.4	 PTEN Hamartoma-Tumor Syndrome
		References
	54: Tumors of the Head and Neck
		54.1	 Nasal Glioma (Figs. 54.1, 54.2)
			54.1.1	 Historical Notes and Incidence
			54.1.2	 Pathology and Embryology
			54.1.3	 Clinical Picture
			54.1.4	 Treatment
		54.2	 Nasal Dermal Sinus (Epidermoid or Dermoid Cyst) (Figs. 54.3, 54.4)
			54.2.1	 Pathology and Embryology
			54.2.2	 Clinical Picture and Treatment
		54.3	 Congenital Anterior Cephaloceles (Frontonasal and Frontoethmoidal) (Figs. 54.5, 54.6)
			54.3.1	 Pathology and Embryology
			54.3.2	 Clinical Picture and Treatment
		54.4	 Hemangioma (Fig. 54.7)
			54.4.1	 Incidence and Pathology
			54.4.2	 Clinical Picture
			54.4.3	 Treatment
		54.5	 Rhabdomyosarcoma (Fig. 54.8)
		54.6	 Myofibromatosis (Fig. 54.9)
			54.6.1	 Historical Notes and Incidence
			54.6.2	 Pathology
			54.6.3	 Clinical Picture
			54.6.4	 Treatment
		54.7	 Lipoblastoma
		54.8	 Foregut Duplication Cysts
			54.8.1	 Incidence
			54.8.2	 Pathology and Embryology
			54.8.3	 Clinical Picture
			54.8.4	 Treatment
		54.9	 Congenital Epuris (Fig. 54.10)
			54.9.1	 Historical Notes and Incidence
			54.9.2	 Pathology and Embryology
			54.9.3	 Clinical Picture
			54.9.4	 Treatment
		54.10	 Germ Cell Tumors (Fig. 54.11)
		54.11	 Cervical Teratoma
			54.11.1	 Incidence and Pathology
			54.11.2	 Clinical Picture
			54.11.3	 Treatment and Prognosis
		54.12	 Epignathi and Nasopharingeal Teratoma
		54.13	 Facial Teratoma
		References
	55: Cystic Hygroma and Lymphatic Malformations
		55.1	 Classification of LMS
		55.2	 Embryology
		55.3	 Clinical Features
		55.4	 Treatment Strategies for LMS
		55.5	 OK-432 Injection Therapy
		55.6	 Protocol of OK-432 Therapy for LMS
		55.7	 Outcomes of Nonsurgical Treatment (OK-432 Sclerotherapy)
		55.8	 Surgical Treatment
		55.9	 Choice of New Therapies
		55.10	 Massive Pleural Effusion and Ascites
		References
	56: Liver Tumors
		56.1	 Malignant Liver Tumours
			56.1.1	 Malignant Epithelial Tumors: Hepatoblastoma and Hepatocellular Carcinoma
				56.1.1.1	 Pathology
				56.1.1.2	 Clinical Presentation
				56.1.1.3	 Diagnostics
				56.1.1.4	 Staging and Risk Stratification
				56.1.1.5	 Treatment
				56.1.1.6	 Treatment of Lung Metastases
				56.1.1.7	 Chemoembolization, Radiofrequency Ablation, and Cryoablation
				56.1.1.8	 Prognosis
			56.1.2	 Liver Sarcoma
			56.1.3	 Malignant Rhabdoid Tumor of the Liver
			56.1.4	 Infantile Choriocarcinoma
		56.2	 Benign Liver Tumors
			56.2.1	 Hemangioma
				56.2.1.1	 Pathology
				56.2.1.2	 Clinical Presentation and Diagnostic Workup
				56.2.1.3	 Therapy
			56.2.2	 Hamartoma
				56.2.2.1	 Pathology
				56.2.2.2	 Clinical Presentation and Diagnostic
				56.2.2.3	 Therapy
				56.2.2.4	 Prognosis
			56.2.3	 Adenoma
			56.2.4	 Focal Nodular Hyperplasia (FNH)
		References
	57: Neuroblastoma
		57.1	 Introduction
		57.2	 History
		57.3	 Epidemiology
		57.4	 Molecular and Genetic Pathogenesis
		57.5	 Histopathology
		57.6	 Staging and Risk Grouping
		57.7	 Fetal Neuroblastoma
		57.8	 Clinical Presentation
		57.9	 Diagnosis and Initial Evaluation
		57.10	 Surgical Management
		57.11	 Chemotherapy
		57.12	 Radiation Therapy
		57.13	 Other Therapies
		57.14	 Screening
		57.15	 Survival and Late Effects
		References
	58: Neonatal Soft Tissue Sarcomas
		58.1	 Introduction
		58.2	 Epidemiology
		58.3	 Histopathology
		58.4	 Genetics and Biology
		58.5	 Specific Tumour Types
			58.5.1	 Rhabdomyosarcoma
			58.5.2	 Infantile Fibrosarcoma
			58.5.3	 Malignant Rhabdoid Tumour
			58.5.4	 Ewing’s Sarcoma Family of Tumours
			58.5.5	 Desmoplastic Small Round Cell Tumour (DSRCT)
			58.5.6	 Clear Cell Sarcoma of Soft Tissue
			58.5.7	 Synovial Sarcoma
		58.6	 Genetic Predisposition Syndromes
		58.7	 Clinical Presentation and Differential Diagnosis
		58.8	 Making the Diagnosis
			58.8.1	 Principles
			58.8.2	 Tumour Markers
			58.8.3	 Imaging
			58.8.4	 Biopsy
		58.9	 Specific Tumour Types
			58.9.1	 Rhabdomyosarcoma
			58.9.2	 Congenital Infantile Fibrosarcoma
			58.9.3	 Extracranial Malignant Rhabdoid Tumour (MRT)
			58.9.4	 Desmoplastic Small Round Cell Tumour
			58.9.5	 Malignant Ectomesenchymoma
			58.9.6	 Ewing’s Sarcoma Family of Tumours
			58.9.7	 Synovial Sarcoma
			58.9.8	 Epithelioid Sarcoma
			58.9.9	 Malignant Peripheral Nerve Sheath Tumour (MPNST)
			58.9.10 Clear Cell Sarcoma of Soft Tissue
			58.9.11 Low-Grade Fibromyxoid Sarcoma
			58.9.12 Hemangioendothelioma
			58.9.13 Haemangiopericytoma
			58.9.14 Mesenchymal Chondrosarcoma
			58.9.15 Myofibroblastic Lesions and Aggressive Fibromatosis/Desmoid
				58.9.15.1	 Myofibromatosis
				58.9.15.2	 Inflammatory Myofibroblastic Tumours
				58.9.15.3	 Solitary Intestinal Fibromatosis
				58.9.15.4	 Aggressive Fibromatosis or Desmoid Tumours
			58.9.16 Staging
		58.10	 Risk Stratification
		58.11	 Multimodality Therapy
		58.12	 Primary Site Management
			58.12.1 Surgical Management
			58.12.2 Radiotherapy
			58.12.3 Site-Specific Surgical Treatment
				58.12.3.1	 Head and Neck
				58.12.3.2	 Orbital Rhabdomyosarcoma
				58.12.3.3	 Vagina
				58.12.3.4	 Bladder/Prostate
				58.12.3.5	 Paratesticular Rhabdomyosarcoma
				58.12.3.6	 Extremities
		References
	59: Renal Tumours
		59.1	 History
		59.2	 Incidence and Epidemiology
		59.3	 Pathology
		59.4	 Cytogenetics
		59.5	 Tumour Markers
		59.6	 Clinical Features
		59.7	 Treatment
			59.7.1	 Preoperative Preparation
			59.7.2	 Operative Technique
				59.7.2.1	 Position
				59.7.2.2	 Incision
				59.7.2.3	 Postoperative Care
				59.7.2.4	 Complications
		References
	60: Ovarian and Genital Tract Neoplasms
		60.1	 Neonatal Ovarian Tumors
			60.1.1	 Introduction
			60.1.2	 Pathological Entities Encountered in Neonates
			60.1.3	 Presentation and Differential Diagnosis
				60.1.3.1	 Clinical Signs of Atypical Hormonal Secretion
				60.1.3.2	 Radiological Lesions Diagnosed Fortuitously or During Follow Up of Prenatal Pelvic Cystic Lesion
				60.1.3.3	 Palpable Abdominal Mass or Other Rare Presentations
			60.1.4	 Management and Prognosis
				60.1.4.1	 Preoperative Management
				60.1.4.2	 Radiological Features
				60.1.4.3	 Biological Markers
				60.1.4.4	 Surgical Procedure
				60.1.4.5	 Prognosis
		60.2	 Neonatal Genital Tract Tumors
			60.2.1	 Introduction
			60.2.2	 Pathological Entities Encountered in Neonates and Their Molecular Characteristics
			60.2.3	 Presentation and Differential Diagnosis
				60.2.3.1	 Presentation
				60.2.3.2	 Differential Diagnosis
			60.2.4	 Management and Prognosis
				60.2.4.1	 Imaging and Histological Studies
				60.2.4.2	 Therapeutic Strategies
				60.2.4.3	 Prognosis
		References
	61: Sacrococcygeal Teratoma
		61.1	 Introduction
		61.2	 Pathology
		61.3	 Fetus with SCT
		61.4	 Newborn Presentation
		61.5	 Associated Conditions
		61.6	 Investigations after Birth
		61.7	 Staging
		61.8	 Postnatal Management
		61.9	 Prognosis and Outcomes
		References
Part IX: Urology
	62: Management of Impaired Renal Function in the Newborn
		62.1	 Introduction
		62.2	 Normal Renal Function in the Newborn
			62.2.1	 Glomerular Filtration Rate
			62.2.2	 Water Homeostasis
			62.2.3	 Sodium Homeostasis
			62.2.4	 Potassium Homeostasis
			62.2.5	 Tubular Function
		62.3	 Impaired Renal Function in the Newborn
			62.3.1	 Pre-renal
			62.3.2	 Intrinsic-Renal
			62.3.3	 Post-renal
		62.4	 Evaluation of the Newborn with Renal Impairment
		62.5	 Management
			62.5.1	 Fluid Management
			62.5.2	 Nutrition and Growth
			62.5.3	 Electrolytes
			62.5.4	 Drug Handling
			62.5.5	 Hypertension
			62.5.6	 Renal Replacement Therapy
				62.5.6.1	 Peritoneal Dialysis
				62.5.6.2	 Blood Based Dialysis
		62.6	 Long Term Outcome
		References
	63: Newborn Urinary Tract Infections
		63.1	 Introduction
		63.2	 Epidemiology and Risk Factors
			63.2.1	 Antenatal Abnormalities
			63.2.2	 Circumcision
		63.3	 Diagnosis
			63.3.1	 Clinical
			63.3.2	 Rapid Diagnostic Techniques
			63.3.3	 Urine Culture
			63.3.4	 Microbiology
		63.4	 Treatment
			63.4.1	 Initial Treatment
			63.4.2	 Prophylactic Antibiotics
		63.5	 Investigations
		63.6	 Urinary Tract Infection and Normal Renal Ultrasound
		63.7	 The Diagnosis of Vesicoureteric Reflux
		References
	64: Indications for Investigation of the Urinary Tract in the Newborn
		64.1	 Introduction
		64.2	 Antenatal Diagnosis
		64.3	 Postnatal Presentation
		64.4	 Imaging Modalities
			64.4.1	 Ultrasound Scan
			64.4.2	 Micturating or Voiding Cystourethrogram
			64.4.3	 Nuclear Medicine Imaging/Isotope Scans
			64.4.4	 DMSA (Dimercaptosuccinic Acid) Scan
			64.4.5	 MAG3 (Mercapto Acetyl Tri-Glycerine) Scan
			64.4.6	 DTPA (Diethylenetriamine Pentaacetic Acid)
			64.4.7	 Magnetic Resonance Imaging (MRI)
			64.4.8	 Computed Tomography (CT)
		64.5	 Summary
		References
	65: Urinary Tract Obstruction and Dilatation
		65.1	 Introduction
		65.2	 Antibiotic Prophylaxis
		65.3	 Prenatally Detected Urinary Tract Anomalies and Their Antenatal Management
		65.4	 Incidence
		65.5	 Antenatal Investigations
		65.6	 Antenatal Intervention
		65.7	 General Principles of Postnatal Management
		65.8	 Multicystic Dysplastic Kidney
		65.9	 Isolated Pelvicalyceal Dilatation
		65.10	 Assessment of Antenatal HDN
		65.11	 Pelvi-Ureteric Junction Obstruction
		65.12	 Diagnosis and Indication for Intervention in PUJ Obstruction
			65.12.1	 Management
			65.12.2	 Vesico-Ureteric Reflux
			65.12.3	 Investigations
			65.12.4	 Treatment
			65.12.5	 Primary Non-refluxing Megaureter
			65.12.6	 Duplication Anomalies
			65.12.7	 Megacystis
			65.12.8	 Management
			65.12.9	 Posterior Urethral Valves
			65.12.10	 Pathology
			65.12.11	 Clinical Presentation
			65.12.12	 Diagnosis and Investigations
			65.12.13	 Management
			65.12.14	 Bladder in PUV
			65.12.15	 Urinary Diversion
		65.13	 Long Term Outcomes
			65.13.1	 Renal Failure
			65.13.2	 Sexual Function and Fertility
			65.13.3	 VUR
			65.13.4	 Urinary Incontinence
		References
	66: Renal Cystic Disease and Vascular Lesions of the Adrenal and Kidney
		66.1	 Renal Cystic Disease
			66.1.1	 Multicystic Dysplastic Kidney
				66.1.1.1	 Pathogenesis
				66.1.1.2	 Clinical Presentation and Diagnosis
				66.1.1.3	 Natural History
				66.1.1.4	 Treatment
			66.1.2	 Autosomal Recessive Polycystic Kidney Disease
			66.1.3	 Autosomal Dominant Polycystic Kidney Disease
			66.1.4	 Solitary Renal Cysts
		66.2	 Vascular Lesions of the Adrenal and Kidney
			66.2.1	 Adrenal Haemorrhage
				66.2.1.1	 Aetiology
				66.2.1.2	 Clinical Features
				66.2.1.3	 Diagnosis
				66.2.1.4	 Treatment
			66.2.2	 Renal Vein Thrombosis
				66.2.2.1	 Pathogenesis
				66.2.2.2	 Aetiology
				66.2.2.3	 Clinical Manifestations
				66.2.2.4	 Diagnosis
				66.2.2.5	 Treatment
				66.2.2.6	 Late Sequelae
			66.2.3	 Renovascular Hypertension in Neonate
				66.2.3.1	 Clinical Presentation and Diagnostic Approach
				66.2.3.2	 Treatment
		References
	67: Prune Belly Syndrome
		67.1	 History
		67.2	 Epidemiology
		67.3	 Pathogenesis
		67.4	 Antenatal Presentation
		67.5	 Clinical Presentation
			67.5.1	 Urinary Tract
			67.5.2	 Genital Tract
			67.5.3	 Abdominal Wall
			67.5.4	 Orthopaedic Anomalies
			67.5.5	 Cardiovascular Anomalies
		67.6	 Surgical Management
		References
	68: Disorders of Sex Development
		68.1	 Introduction
		68.2	 History
		68.3	 Classification
		68.4	 Prognosis
		68.5	 Epidemiology
		68.6	 Genetics
		68.7	 Embryology
		68.8	 Associated Anomalies
		68.9	 Antenatal Presentation
		68.10	 Clinical Presentation
		68.11	 Management
		68.12	 Long-Term Follow-Up and Prognosis
		References
	69: Male Genital Tract
		69.1	 Penis
			69.1.1	 Phimosis
			69.1.2	 Circumcision
			69.1.3	 Paraphimosis
			69.1.4	 Hypospadias
			69.1.5	 Inconspicuous or Concealed Penis
		69.2	 Undescended Testis/Cryptorchidism
			69.2.1	 Introduction
			69.2.2	 Embryology
			69.2.3	 Classification
			69.2.4	 Incidence
			69.2.5	 Diagnosis
				69.2.5.1	 History
				69.2.5.2	 Examination
				69.2.5.3	 Investigation
				69.2.5.4	 Pathological Changes in UDTs
			69.2.6	 Management
				69.2.6.1	 Medical
				69.2.6.2	 Surgical
					Aims of Surgery
				69.2.6.3	 Palpable UDTs
			69.2.7	 Impalpable Testis
			69.2.8	 Complications of Surgery
			69.2.9	 Outcome Following Surgery
				69.2.9.1	 Testicular Size and Position
				69.2.9.2	 Fertility
				69.2.9.3	 Semen Analysis
			69.2.10	 Malignancy
		69.3	 Acute Scrotal Pathology
			69.3.1	 Introduction
			69.3.2	 Torsion of Appendix Testis
			69.3.3	 Testicular Torsion
			69.3.4	 Intravaginal Torsion
			69.3.5	 Extravaginal Torsion
			69.3.6	 Idiopathic Scrotal Oedema
			69.3.7	 Epididymitis
			69.3.8	 Orchitis
			69.3.9	 Other
				69.3.9.1	 Hernia/Hydrocoele
				69.3.9.2	 Malignancy
				69.3.9.3	 Henoch-Schönlein Purpura
			69.3.10	 Summary
		69.4	 Varicocele
		69.5	 Epididymal Cyst/Spermatocele
		References
Part X: Outcomes in Newborn Surgery
	70: Long-Term Outcomes in Neonatal Surgery
		70.1	 Introduction
		70.2	 Rationale of Long-Term Follow-Up Programmes
		70.3	 How to Analyse Long-Term Outcomes
		70.4	 General Long-Term Consequences of Neonatal Surgery and Anaesthesia
		70.5	 Factors that Influence the Long-Term Outcome
			70.5.1	 The Type of the Malformation/Condition
				70.5.1.1	 Oesophageal Atresia
				70.5.1.2	 Hirschsprung’s Disease
				70.5.1.3	 Anorectal Malformations
			70.5.2	 Aging
			70.5.3	 Risk for Malignancies
			70.5.4	 Undefined Factors
		References
	71: Long Term Outcomes in Pediatric Urology
		71.1	 Vesicoureteric Reflux
			71.1.1	 Background
			71.1.2	 Long-Term Outcome
		71.2	 Pelvicuretero Junction/Ureteropelvic Junction Stenosis
			71.2.1	 Background
			71.2.2	 Long-Term Outcome
		71.3	 Megaureters
			71.3.1	 Background
			71.3.2	 Long-Term Outcome
		71.4	 Bladder Exstrophy
			71.4.1	 Background
			71.4.2	 Long-Term Outcome
		71.5	 Epispadias
			71.5.1	 Long-Term Outcome
		71.6	 Neuropathic Bladder
			71.6.1	 Background
			71.6.2	 Long-Term Outcome
		71.7	 Mitrofanoff
			71.7.1	 Background
			71.7.2	 Long-Term Outcome
		71.8	 Posterior Urethral Valves
			71.8.1	 Background
			71.8.2	 Long-Term Outcome
		71.9	 Hypospadias
			71.9.1	 Background
			71.9.2	 Long-Term Outcome
		71.10	 Intersex
			71.10.1 Background
			71.10.2 Long-Term Outcome
		71.11	 Cloacal Malformation
			71.11.1 Background
			71.11.2 Long-Term Outcome
		71.12	 Robotics
		71.13	 Stem Cells
		References
	72: Evidence Based Neonatal Surgery
		72.1	 Introduction
		72.2	 Oesophageal Atresia
		72.3	 Congenital Diaphragmatic Hernia
		72.4	 Atresia of the Mid and Hind Gut
		72.5	 Anorectal Malformations
		72.6	 Anterior Abdominal Wall Defects
		72.7	 Congenital Lung Lesions
		72.8	 Hirschsprung Disease
		72.9	 Inguinal Hernia
		72.10	 Necrotising Enterocolitis
		72.11	 Pyloric Stenosis
		72.12	 Commentary
		References
Index