Handbook of Pediatric Surgery

Contents
1: Milestones in Pediatric Surgery
Part I: Principles of Surgical Science
	2: Transitional Physiology and Newborn Care
		2.1	 Fetal Circulation
		2.2	 Respiratory Adaptation
		2.3	 Newborn Physiology
		2.4	 Immediate Management After Birth
			2.4.1	 Thermoregulation
			2.4.2	 Cord Care
			2.4.3	 Feeding
		2.5	 Problems of the Premature
			2.5.1	 Respiratory Management
			2.5.2	 Thermoregulation
			2.5.3	 Jaundice
			2.5.4	 Anemia
		2.6	 Neonatal Infections
			2.6.1	 Investigations
			2.6.2	 Management
		2.7	 Fluid Management
		2.8	 Neonatal Nutrition
		Further Reading
	3: Fluids, Electrolytes, and Dehydration
		3.1	 Normal Fluid Physiology
		3.2	 Age-Related Changes
		3.3	 Normal Fluid and Electrolyte Requirements
		3.4	 Insensible Fluid Loss
		3.5	 Postoperative Fluid Regimens
		3.6	 Dehydration
			3.6.1	 Infective Causes
			3.6.2	 Surgical Causes
			3.6.3	 Management
		3.7	 Specific Electrolyte Problems
			3.7.1	 Potassium
				3.7.1.1	 Hyperkalemia (<5.5 mmol/L)
				3.7.1.2	 Hypokalemia
			3.7.2	 Calcium
				3.7.2.1	 Hypocalcemia (Always Check Magnesium Levels Additionally)
				3.7.2.2	 Hypercalcemia
		3.8	 Acid-Base Imbalance
			3.8.1	 Concepts
			3.8.2	 Base Excess (or Deficit)
			3.8.3	 Abnormal Acid-Base States
				3.8.3.1	 Metabolic Acidosis
				3.8.3.2	 Metabolic Alkalosis
		Further Readings
	4: Metabolic Response to Injury and Sepsis
		4.1	 Metabolic Response to Surgery
			4.1.1	 Factors Responsible for the Early Phase Response Include
			4.1.2	 Factors Responsible for the Late Response Phase Include
			4.1.3	 Tissue Response to Injury
		4.2	 Systemic Inflammatory Response Syndrome (SIRS)
		4.3	 Multiple Organ Dysfunction Syndrome (MODS)
		Further Reading
	5: Shock
		5.1	 Classification
		5.2	 Degree of Shock
		5.3	 Clinical Features
			5.3.1	 Differences with Age
		5.4	 Management
			5.4.1	 Principles
			5.4.2	 Colloids Versus Crystalloids
			5.4.3	 Massive Transfusion
		Further Reading
	6: Hematology for Surgeons
		6.1	 Coagulation Tests
			6.1.1	 Normal Process of Coagulation
			6.1.2	 Hemophilia
			6.1.3	 Blood Transfusion (UK Specific)
				6.1.3.1	 ABO System
				6.1.3.2	 Rhesus (D) System
				6.1.3.3	 Minor Groups
			6.1.4	 Need for Blood Transfusion
				6.1.4.1	 Platelets
				6.1.4.2	 Fresh Frozen Plasma
				6.1.4.3	 Cryoprecipitate
			6.1.5	 Transfusion Reactions
			6.1.6	 Alternatives to Blood Transfusion (Jehovah’s Witness)
			6.1.7	 Coombs’ Test
				6.1.7.1	 Transmissible Hazards of Blood Transfusion
			6.1.8	 Sickle Cell Disease
				6.1.8.1	 Sickle Cell Crisis
				6.1.8.2	 Clinical Features
			6.1.9	 Surgery in the Child with SCD
			6.1.10	 Acute Chest Syndrome
				6.1.10.1	 Management
			6.1.11	 Leucocytosis or Neutropenia
		Further Reading
	7: Post-operative Recovery
		7.1	 Analgesia
			7.1.1	 Concepts: Pre-emptive and Multimodal Analgesia
			7.1.2	 Local Anesthesia (LA)
				7.1.2.1	 Percentage Solution of Local Anesthetics
				7.1.2.2	 Adrenaline (Epinephrine)
		7.2	 Pyrexia
			7.2.1	 Immediate (<24 h)
			7.2.2	 Early (2–5 Days)
			7.2.3	 Late (>5 Days)
		7.3	 Wound Infection
			7.3.1	 Organisms
			7.3.2	 Necrotizing Fasciitis
		7.4	 Clavien–Dindo Classification of Complications
		Further Reading
	8: Parenteral Nutrition
		8.1	 Introduction
		8.2	 Indications for Parenteral Nutrition (PN)
		8.3	 Constituents of PN
			8.3.1	 Energy
			8.3.2	 Carbohydrate
			8.3.3	 Amino Acid
			8.3.4	 Lipid
			8.3.5	 Trace Elements and Vitamins
		8.4	 Initiation, Adjustment, and Monitoring of PN
			8.4.1	 Anthropometry
			8.4.2	 Biochemistry
			8.4.3	 Initiating PN
		8.5	 Complications of PN
		8.6	 Concomitant Medications
		8.7	 Long-Term Use of PN
		8.8	 Intestinal Failure-Associated Liver Disease (IFALD)
		8.9	 Multidisciplinary Team (MDT)
		Further Reading
Part II: Trauma and Resuscitation
	9: Trauma Management: General Principles
		9.1	 Basic Approach to Trauma
			9.1.1	 Important Differences Between Children and Adults
		9.2	 Management Plan
			9.2.1	 Primary Survey and Resuscitation
			9.2.2	 ABCDE: Approach
			9.2.3	 Life-Threatening Conditions
			9.2.4	 Other Procedures During Resuscitation
			9.2.5	 Secondary Survey
			9.2.6	 Emergency Treatment
			9.2.7	 Reassessment and System Control
			9.2.8	 Continuing Stabilization and Definitive Care
		9.3	 Important Points About Systemic Injuries
			9.3.1	 Chest Trauma
				9.3.1.1	 Tension Pneumothorax
				9.3.1.2	 Flail Chest
				9.3.1.3	 Cardiac Tamponade
				9.3.1.4	 Abdominal Trauma
				9.3.1.5	 Head and Spine Injury
		9.4	 Supplemental Data
			9.4.1	 Physiological
		Further Reading
	10: Head Injury
		10.1	 Introduction
		10.2	 Classification and Pathophysiology
		10.3	 Clinical Assessment
			10.3.1	 S-100B Protein
			10.3.2	 Imaging
		10.4	 Prevention of Head Injuries in Children
		Further Reading
	11: Thoracic Trauma
		11.1	 Introduction
		11.2	 Mechanisms of Injury
		11.3	 Anatomy
			11.3.1	 Surface Landmarks
		11.4	 Principles of Management
		11.5	 Rib Cage Injury
			11.5.1	 Flail Chest
			11.5.2	 Non-accidental Trauma/Child Abuse
			11.5.3	 Investigation
			11.5.4	 Caveats and Atypical Injury
			11.5.5	 Management
		11.6	 Parenchymal Injury
			11.6.1	 Clinical Features
			11.6.2	 Investigation
			11.6.3	 Treatment
		11.7	 Pneumothorax
			11.7.1	 Investigation
			11.7.2	 Treatment
		11.8	 Hemothorax
			11.8.1	 Investigation
			11.8.2	 Treatment
		11.9	 Great Vessel Injury
			11.9.1	 Aortic Injury
		11.10	 Tracheobronchial Injury
		11.11	 Cardiac Injury
			11.11.1 Commotio Cordis2
				11.11.1.1	 Investigations
			11.11.2 Myocardial contusion
				11.11.2.1	 Investigations
			11.11.3 Treatment
		11.12	 Diaphragm Injury
			11.12.1 Investigation
			11.12.2 Treatment
		11.13	 Esophageal Rupture
			11.13.1 Investigation
			11.13.2 Treatment
		11.14	 Traumatic Asphyxia (a.k.a. Crush Asphyxia) (Perthes3 Syndrome)
		Further Reading
	12: Abdominal Trauma
		12.1	 Epidemiology
		12.2	 Mechanism of Injury
			12.2.1	 Factors Predisposing to Abdominal Injury in the Child
		12.3	 Clinical Features
		12.4	 Management (See Also Chap. 9)
			12.4.1	 Investigations and Imaging
			12.4.2	 Hemodynamically “Unstable” vs. “Stable”
			12.4.3	 Nonoperative Management of Blunt Abdominal Trauma (∼95%)
			12.4.4	 Interventional Radiology
		12.5	 Laparotomy for Abdominal Trauma (∼5%)
			12.5.1	 Principles
		12.6	 Penetrating Injuries
			12.6.1	 Laparotomy
		12.7	 Specific Organ Injury
			12.7.1	 Splenic Injury
				12.7.1.1	 Surgical Options
			12.7.2	 Liver Injury
				12.7.2.1	 Surgical Options
		12.8	 Renal Injury
		12.9	 Pancreatic Injury
		Further Reading
Part III: Neonatal Surgery
	13: Developmental Lung Lesions
		13.1	 Embryology
		13.2	 Prenatal Diagnosis
		13.3	 Pulmonary Agenesis
		13.4	 Bronchopulmonary Sequestration (BPS)
			13.4.1	 Clinical Features
			13.4.2	 Postnatal Investigations
			13.4.3	 Postnatal Management
		13.5	 Congenital Cystic Adenomatoid Malformation
			13.5.1	 Classification
			13.5.2	 Clinical Presentation
			13.5.3	 Management
		13.6	 Congenital Lobar Emphysema
			13.6.1	 Clinical Features
			13.6.2	 Management
		13.7	 Bronchogenic Cyst
			13.7.1	 Clinical Features
			13.7.2	 Management
		Further Reading
	14: Esophageal Atresia
		14.1	 Background
			14.1.1	 VACTERL (Previously VATER) Association
			14.1.2	 CHARGE Syndrome
			14.1.3	 Embryology
		14.2	 Clinical Features
			14.2.1	 Prenatal
			14.2.2	 Postnatal Features and Investigation
				14.2.2.1	 Management
		14.3	 Surgery
			14.3.1	 Esophageal Atresia and Distal TEF
			14.3.2	 “Long-Gap” Esophageal Atresia
			14.3.3	 Surgical Complications
		14.4	 Thoracoscopic Repair
		14.5	 Prognostic Classifications
		14.6	 Long-Term Outcomes
		Further Reading
	15: Congenital Diaphragmatic Hernia
		15.1	 Epidemiology
		15.2	 Associations
		15.3	 Embryology
		15.4	 Pulmonary Hypoplasia
		15.5	 Clinical Features
			15.5.1	 Antenatal
			15.5.2	 Postnatal
		15.6	 Management
			15.6.1	 Delivery Suite
			15.6.2	 In the Intensive Care Unit
			15.6.3	 Second-Line Therapy
		15.7	 Surgery
		15.8	 Complications and Outcome
		15.9	 Contentious Topics
			15.9.1	 Fetal Surgery
			15.9.2	 Minimally Invasive Surgery of CDH
		Further Reading
	16: Congenital Pyloric Stenosis
		16.1	 Historical Aspects
		16.2	 Demographics
			16.2.1	 Risk Factors
			16.2.2	 Genetic Component
			16.2.3	 Associations
		16.3	 Pathophysiology
		16.4	 Clinical Features
			16.4.1	 Differential Diagnosis
			16.4.2	 Diagnosis
			16.4.3	 Management
			16.4.4	 Ramstedt’s Operation
			16.4.5	 Postoperative Care
		Further Reading
	17: Intestinal Atresia
		17.1	 Epidemiology
		17.2	 Embryology
		17.3	 Associations
		17.4	 Classification (After Bland-Sutton, Modified by Grosfeld)
		17.5	 Clinical Features
			17.5.1	 Investigations
			17.5.2	 Differential Diagnosis
		17.6	 Surgery
			17.6.1	 Duodenal Atresia (Duodenoduodenostomy)
			17.6.2	 Jejunal Atresia (Figs. 17.2 and 17.3)
			17.6.3	 Colon Atresia
		17.7	 Outcome
		Further Reading
	18: Intestinal Malrotation
		18.1	 Introduction
		18.2	 Embryology (After Frazer and Robbins)
		18.3	 Etiology
		18.4	 Clinical Features
			18.4.1	 Investigations
				18.4.1.1	 Prenatal: Very Unusual
				18.4.1.2	 Postnatal (Table 18.1)
		18.5	 Management
			18.5.1	 Surgery: Ladd’s Procedure
			18.5.2	 Asymptomatic Patients
		18.6	 Outcome and Complications
		Further Reading
	19: Necrotizing Enterocolitis
		19.1	 Epidemiology
		19.2	 Pathogenesis
		19.3	 Prevention
		19.4	 Clinical Features and Diagnosis
		19.5	 Management
		19.6	 Surgical Options
		19.7	 Outcomes
		Further Reading
	20: Meconium Ileus and Surgical Features of Cystic Fibrosis
		20.1	 Meconium Ileus
			20.1.1	 Definition
			20.1.2	 History
			20.1.3	 Epidemiology
		20.2	 Cystic Fibrosis
			20.2.1	 Pathophysiology
				20.2.1.1	 Lungs
				20.2.1.2	 Gastrointestinal Epithelium
		20.3	 Clinical Features of Meconium Ileus
			20.3.1	 Antenatal Period
			20.3.2	 Postnatal
			20.3.3	 Complicated MI
			20.3.4	 Investigations
				20.3.4.1	 Laboratory Tests
			20.3.5	 Management
				20.3.5.1	 Surgery (re-use original figures)
				20.3.5.2	 Postoperative care
			20.3.6	 Outcome
		20.4	 Meconium Plug Syndrome (MPS)
			20.4.1	 Clinical Features
		20.5	 Distal Intestinal Obstruction Syndrome (DIOS)
			20.5.1	 Clinical Features
			20.5.2	 Management
		20.6	 Fibrosing Colonopathy
		20.7	 Other Surgical Implications of CF
		20.8	 Malignancy
		20.9	 Gene Therapy of Cystic Fibrosis
		Further Reading
	21: Abdominal Wall Defects
		21.1	 Introduction
		21.2	 Embryology
		21.3	 Exomphalos (Fig. 21.1)
			21.3.1	 Clinical Features
			21.3.2	 Investigations
			21.3.3	 Management
		21.4	 Gastroschisis (Fig. 21.2)
			21.4.1	 Clinical Features
			21.4.2	 Management
			21.4.3	 Surgery
			21.4.4	 Complications
			21.4.5	 Outcome
		Further Reading
	22: Anorectal Malformations
		22.1	 Introduction
			22.1.1	 Epidemiology
			22.1.2	 Embryology
			22.1.3	 The Anatomical Classification
			22.1.4	 Associated Anomalies
			22.1.5	 Clinical Features
				22.1.5.1	 Males (Fig. 22.1)
				22.1.5.2	 Females (Fig. 22.2)
		22.2	 Neonatal Management
			22.2.1	 Surgical Management—Colostomy or Primary Repair?
			22.2.2	 Primary Perineal Approach
			22.2.3	 Colostomy
			22.2.4	 High-Pressure Distal Colostogram
			22.2.5	 Main Repair
				22.2.5.1	 PSARP
				22.2.5.2	 Recto-Bladder Neck Fistula
				22.2.5.3	 PSARP For Females with Recto-Vestibular Fistula (Fig. 22.4b and c)
				22.2.5.4	 Perineal Fistula in Females
				22.2.5.5	 Perineal Fistula in Males
				22.2.5.6	 Cloaca
					Common Channel <3 cm
					Common Channel >3 cm
		Further Reading
	23: Hirschsprung’s Disease
		23.1	 Epidemiology
		23.2	 Embryology
		23.3	 Anatomy and Neuroanatomy
		23.4	 Genetics
		23.5	 Etiology
		23.6	 Pathology
			23.6.1	 Variable Affected Segment
		23.7	 Clinical Features
			23.7.1	 Investigations
			23.7.2	 Differential Diagnosis
		23.8	 Management
		23.9	 Surgery
			23.9.1	 Pull-Through Procedure
		23.10	 Outcome
		Further Reading
Part IV: Hepatobiliary
	24: Investigation of Jaundice
		24.1	 Metabolism of Bilirubin (Fig. 24.1)
		24.2	 Scale of Problem
		24.3	 Physiological Jaundice
		24.4	 Surgical Jaundice in Infants
			24.4.1	 Investigations
				24.4.1.1	 Ultrasonography (Table 24.1)
				24.4.1.2	 Liver Biopsy
				24.4.1.3	 Duodenal Aspiration
				24.4.1.4	 Radionuclear Isotope
				24.4.1.5	 Single Photon Emission Computed Tomography (SPECT)
				24.4.1.6	 Percutaneous Transhepatic Cholangiogam
				24.4.1.7	 Endoscopic Retrograde Cholangiopancreatography
				24.4.1.8	 Magnetic Resonance Cholangiopancreatography
				24.4.1.9	 Laparoscopy ± Cholangiography
		24.5	 Investigation of Surgical Jaundice in the Older Child
			24.5.1	 Intramural
			24.5.2	 Extramural
			24.5.3	 Mural
		Further Reading
	25: Choledochal Malformation
		25.1	 History
		25.2	 Epidemiology
		25.3	 Classification
		25.4	 Pathogenesis
		25.5	 Clinical Features
			25.5.1	 Investigations
		25.6	 Surgery—Type 1c (Cystic Malformation)
		25.7	 Outcome
		Further Reading
	26: Biliary Atresia
		26.1	 Epidemiology
		26.2	 Embryology
		26.3	 Classification
		26.4	 Clinical Features
			26.4.1	 Investigations
		26.5	 Surgery
			26.5.1	 Kasai Portoenterostomy (Fig. 26.2)
		26.6	 Complications
		26.7	 Outcome
		Further Reading
	27: Gallbladder Disease
		27.1	 Congenital Anomalies of the Gallbladder
		27.2	 Gallstone Disease
			27.2.1	 Epidemiology
			27.2.2	 Predisposition Groups
			27.2.3	 Chemistry of Gallstones
			27.2.4	 Clinical Features
			27.2.5	 Investigations
			27.2.6	 Management
			27.2.7	 Surgery
		27.3	 Acalculous Cholecystitis
		27.4	 Hydrops of the Gallbladder
		27.5	 Biliary Dyskinesia
			27.5.1	 Investigation
		Further Reading
	28: Pancreatic Disease
		28.1	 Embryology (Fig. 28.1)
			28.1.1	 Annular Pancreas
			28.1.2	 Pancreas Divisum
			28.1.3	 Common Channel
		28.2	 Acute Pancreatitis
			28.2.1	 Surgical Etiology
			28.2.2	 Medical Etiology
				28.2.2.1	 Hereditary (Aka Familial) Pancreatitis
			28.2.3	 Pathology of Pancreatitis
			28.2.4	 Clinical Features
			28.2.5	 Investigations
			28.2.6	 Management
				28.2.6.1	 Surgery
		28.3	 Chronic Pancreatitis
			28.3.1	 Etiology
			28.3.2	 Clinical Features
			28.3.3	 Investigations
			28.3.4	 Surgery
		28.4	 Persistent Hyperinsulinemic Hypoglycemia of infancy
			28.4.1	 Background
			28.4.2	 Associations
			28.4.3	 Pathology
			28.4.4	 Clinical features
			28.4.5	 Investigations
			28.4.6	 Management
		Further Reading
	29: Portal Hypertension
		29.1	 Introduction
		29.2	 Anatomy
		29.3	 Causes of Portal Hypertension
			29.3.1	 Portal Vein Thrombosis
				29.3.1.1	 Aetiology
				29.3.1.2	 Clinical Features
				29.3.1.3	 Investigations
				29.3.1.4	 Management of Acute Bleeding Episode
				29.3.1.5	 Secondary Prophylaxis
				29.3.1.6	 Definitive Surgery
		Further Reading
	30: Liver Transplantation
		30.1	 Indications for Transplantation
			30.1.1	 Acute Liver Failure
			30.1.2	 Chronic Liver Failure
			30.1.3	 Inborn Errors of Metabolism (~20%)
			30.1.4	 Liver Tumors (~10%)
		30.2	 Transplant Assessment
		30.3	 Surgery
			30.3.1	 Donor Allocation
			30.3.2	 Organ Donation
		30.4	 Surgery
			30.4.1	 Graft Preparation
			30.4.2	 Recipient Surgery
				30.4.2.1	 Post-Operative Care
			30.4.3	 Complications
				30.4.3.1	 Poor Graft Function
				30.4.3.2	 Vascular
				30.4.3.3	 Biliary Complications (up to 30%)
				30.4.3.4	 Rejection
				30.4.3.5	 Infection
		30.5	 Outcomes
		Further Reading
Part V: Urology
	31: Exstrophy-Epispadias Anomaly
		31.1	 Bladder Exstrophy-Epispadias Complex (BEEC)
			31.1.1	 Classical Bladder Exstrophy (CBE)
			31.1.2	 Cloacal Exstrophy
			31.1.3	 Epispadias
			31.1.4	 Genetics
		31.2	 Embryology
		31.3	 Clinical Features of BEEC
		31.4	 Objectives of Management
			31.4.1	 Modern Staged Repair of Bladder Exstrophy
			31.4.2	 Kelly Principle (Radical Soft Tissue Reconstruction)
			31.4.3	 Complete Primary Repair of Exstrophy
		31.5	 Complications
		31.6	 Outcome
		31.7	 Primary Epispadias
		31.8	 Cloacal Exstrophy
			31.8.1	 Surgical Principles
		Further Reading
	32: Hypospadias
		32.1	 Embryology
			32.1.1	 Etiology
		32.2	 Clinical Features
			32.2.1	 Classification (Fig. 32.1)
		32.3	 Surgery
			32.3.1	 Principles
			32.3.2	 Preoperative Considerations
			32.3.3	 Surgical Techniques
				32.3.3.1	 Distal Hypospadias
				32.3.3.2	 Proximal Hypospadias
			32.3.4	 Postoperative Phase
		32.4	 Long-Term Outcome
		Further Reading
	33: The Ureter
		33.1	 Anatomy and Embryology
		33.2	 Ureteropelvic Junction (UPJ) Obstruction
			33.2.1	 Etiology
			33.2.2	 Clinical Features
			33.2.3	 Investigations
				33.2.3.1	 Postnatal Ultrasonography: Primary Investigations Tool for Hydronephrosis
				33.2.3.2	 Radioisotope Scan
				33.2.3.3	 Treatment
				33.2.3.4	 Surgery
		33.3	 Duplex Anomalies
			33.3.1	 Clinical Features
			33.3.2	 Investigations
				33.3.2.1	 Ultrasound
				33.3.2.2	 VCUG (Voiding/Micturating Cystourethrogram)
				33.3.2.3	 Radio-Isotope Scan
				33.3.2.4	 MR Urography
				33.3.2.5	 Treatment
		33.4	 Ectopic Ureter
			33.4.1	 Clinical Features
			33.4.2	 Investigations
		33.5	 Surgery
		33.6	 Megaureter
			33.6.1	 Clinical Features
			33.6.2	 Investigations
			33.6.3	 Management
		33.7	 Ureteroceles
			33.7.1	 Types of Ureterocele
			33.7.2	 Clinical Features
				33.7.2.1	 Investigations
				33.7.2.2	 Surgery
		Further Reading
	34: Vesicoureteric Reflux
		34.1	 Introduction
		34.2	 Pathology
		34.3	 Clinical Features
		34.4	 Investigations
			34.4.1	 Grading
		34.5	 Management
			34.5.1	 Reimplantation of Ureters
			34.5.2	 Complications
		34.6	 Outcome
		Further Reading
	35: Posterior Urethral Valves
		35.1	 Anatomy
		35.2	 Classification
		35.3	 Epidemiology
		35.4	 Pathophysiology
			35.4.1	 Genetics
		35.5	 Clinical Features
			35.5.1	 Investigations
		35.6	 Management
		35.7	 Long-Term Management
			35.7.1	 Bladder Dysfunction and the “Valve Bladder”
				35.7.1.1	 VURD Syndrome
				35.7.1.2	 Hydroureteronephrosis (HUN)
		35.8	 Long-Term Outcomes
			35.8.1	 Renal Damage
			35.8.2	 End-Stage Renal disease
			35.8.3	 Renal Transplantation
			35.8.4	 Fertility Issues
			35.8.5	 Urinary Incontinence
			35.8.6	 Urinary Ascites (5–10%)
		35.9	 Antenatal Intervention
		Further Reading
	36: Cryptorchidism
		36.1	 Anatomy
		36.2	 Embryology
		36.3	 Causes
		36.4	 Clinical Features
			36.4.1	 Associated Abnormalities
		36.5	 Management
			36.5.1	 Role of Hormone Therapy
			36.5.2	 Orchidopexy
			36.5.3	 Complications
			36.5.4	 Malignancy
				36.5.4.1	 Risk Factors
		36.6	 Outcome
		Further Reading
	37: Inguinal Hernia Hydrocele and the Acute Scrotum
		37.1	 Abdominal Wall Hernia
		37.2	 Groin Hernia
			37.2.1	 Demography
			37.2.2	 Embryology
			37.2.3	 Contents of Hernia
			37.2.4	 Clinical Findings
				37.2.4.1	 Reducible Hernia
				37.2.4.2	 Incarcerated Hernia
				37.2.4.3	 Investigation
			37.2.5	 Management
				37.2.5.1	 Reducible Hernia
				37.2.5.2	 Incarcerated Hernia
				37.2.5.3	 Laparoscopic Repair
				37.2.5.4	 Contralateral Exploration
				37.2.5.5	 Hernia in Females
				37.2.5.6	 Overnight Admission
				37.2.5.7	 Outcome
		37.3	 Hydrocele
			37.3.1	 Classification
			37.3.2	 Clinical Features
			37.3.3	 Management
		37.4	 Abdominoscrotal Hydrocele
		37.5	 Acute Scrotum
			37.5.1	 Torsion of the Testis
				37.5.1.1	 Demography
				37.5.1.2	 Classification
				37.5.1.3	 Clinical Features
				37.5.1.4	 Investigations
				37.5.1.5	 Surgery
				37.5.1.6	 Manual Detorsion
				37.5.1.7	 Perinatal Testicular Torsion
				37.5.1.8	 Intermittent Testicular Pain
				37.5.1.9	 Outcome
		37.6	 Torsion of Testicular Appendages
		Further Reading
	38: Neurogenic Bladder
		38.1	 Anatomy
		38.2	 Etiology
		38.3	 Classification
		38.4	 Clinical Evaluation
		38.5	 Basics of Urodynamics
		38.6	 Management of Neurogenic Bladder
			38.6.1	 Conservative Management
			38.6.2	 Surgical Management
				38.6.2.1	 Procedures to Augment Storage Capacity of Bladder
				38.6.2.2	 Complications of Cystoplasty
				38.6.2.3	 Mitrofanoff and Monti Continent Catheterizable Channels
				38.6.2.4	 Procedures to Improve Urinary Continence by Increasing Outlet Resistance
				38.6.2.5	 ACE Procedure
		38.7	 Transition to Adult Life
		Further Reading
	39: Urinary Tract Infections
		39.1	 Introduction
		39.2	 Microbiology: Table 39.1
		39.3	 Risk Factors
		39.4	 Clinical Features
		39.5	 Investigations
			39.5.1	 Imaging
		39.6	 Treatment
		39.7	 Outcome
		Further Reading
	40: Disorders of Sex Development
		40.1	 Embryology
		40.2	 Clinical Features
		40.3	 Investigations
		40.4	 Principles of Management
		40.5	 Gender Assignment
		40.6	 Long-Term Outcomes
		Further Reading
	41: Enuresis
		41.1	 Introduction
		41.2	 Physiology
		41.3	 Causes of Enuresis
		41.4	 Clinical Features
			41.4.1	 Examination
			41.4.2	 Investigations
		41.5	 Management
Part VI: Neurosurgery
	42: Spina Bifida
		42.1	 Demography
		42.2	 Etiology
		42.3	 Embryology
		42.4	 Spina Bifida Occulta (SBO)
		42.5	 Spina Bifida Cystica
		42.6	 Managment
			42.6.1	 Prenatal
			42.6.2	 Postnatal
		42.7	 Surgery
			42.7.1	 Spinal Dysraphism (Lipomyeloceles, Dermal Sinus Tract, Tethered Cord)
		42.8	 Long Term Issues
			42.8.1	 Outcome
		Further Reading
	43: Hydrocephalus
		43.1	 Physiology
		43.2	 Anatomy
		43.3	 Classification
			43.3.1	 Functional
			43.3.2	 Congenital or Acquired
		43.4	 Clinical Features
			43.4.1	 Imaging
			43.4.2	 Differential Diagnosis
		43.5	 Management
			43.5.1	 Medical
			43.5.2	 Surgical
				43.5.2.1	 Insertion of Shunt
				43.5.2.2	 Shunt Complications
				43.5.2.3	 Endoscopic Third Ventriculostomy
				43.5.2.4	 Choroid Plexectomy
		43.6	 Outcome
		Further Reading
Part VII: Oncology
	44: Wilms’ Tumor
		44.1	 Introduction
		44.2	 Epidemiology
			44.2.1	 Clinical Patterns
		44.3	 Genetics
		44.4	 Pathology
			44.4.1	 Pattern of Spread-
		44.5	 Clinical Features
			44.5.1	 Investigations
		44.6	 Histological Risk Stratification for Prenephrectomy Chemotherapy-Treated WT
		44.7	 Management
			44.7.1	 Surgery
			44.7.2	 Bilateral Wilms’ Tumor (Stage V)
			44.7.3	 Postoperative Chemotherapy Table 44.2
		44.8	 Prognosis
		Further Reading
	45: Neuroblastoma
		45.1	 Introduction
		45.2	 Sites of Origin
		45.3	 Pathology
			45.3.1	 Shimada System Classification
			45.3.2	 Cytogenetics and Prognostic Factors
		45.4	 Clinical Features
			45.4.1	 Specific Investigations
		45.5	 Staging: Complex and Evolving
			45.5.1	 International Neuroblastoma Risk Group (INRG) Classification System
			45.5.2	 INRGSS: International Neuroblastoma Risk Group Staging System
			45.5.3	 International Neuroblastoma Staging System (INSS)
		45.6	 Management
			45.6.1	 Very Low-Risk Neuroblastoma
			45.6.2	 Low-Risk Neuroblastoma
				45.6.2.1	 Proposed Criteria for Observation
			45.6.3	 Intermediate-Risk Neuroblastoma
			45.6.4	 High-Risk Neuroblastoma
		45.7	 Fetal Tumors
		45.8	 Spinal Cord Compression
		45.9	 Surgery
		45.10	 New Treatments
		Further Reading
	46: Liver Tumors
		46.1	 Introduction
		46.2	 Associations
		46.3	 Pathology
			46.3.1 Hepatoblastoma
			46.3.2 Hepatocellular Carcinoma
		46.4	 Clinical Features
			46.4.1 Investigations
		46.5	 Staging
		46.6	 Management
			46.6.1 Surgery
			46.6.2 Outcome
		46.7	 Hepatic Metastases
		46.8	 Benign Liver Tumors
			46.8.1	 Hemangiomas (AKA Hemangioendotheliomas)
			46.8.2 Pathology
			46.8.3 Clinical Features
			46.8.4 Investigations
			46.8.5 Management
		46.9	 Mesenchymal Hamartomas
		46.10	 Focal Nodular Hyperplasia and Hepatic Adenomas
			46.10.1 Investigations
		Further Reading
	47: Teratomas
		47.1	 Introduction
			47.1.1	 Incidence
			47.1.2	 Embryology
			47.1.3	 Pathology and Classification (Fig. 47.1)
			47.1.4	 Staging
			47.1.5	 Tumor Markers
			47.1.6	 Risk Groups
			47.1.7	 Management
		47.2	 Sacrococcygeal Teratomas
			47.2.1	 Delivery
			47.2.2	 Surgery
			47.2.3	 Outcome
		Further Reading
	48: Other Tumors
		48.1	 Lymphomas
			48.1.1	 Hodgkin’s Lymphoma
				48.1.1.1	 Etiology
				48.1.1.2	 Clinical Features
				48.1.1.3	 Investigations
				48.1.1.4	 Management
			48.1.2	 Non-Hodgkin’s Lymphoma
				48.1.2.1	 Etiology
				48.1.2.2	 Pathology
				48.1.2.3	 Clinical Features
				48.1.2.4	 Investigations
				48.1.2.5	 Management
			48.1.3	 Rhabdomyosarcoma
				48.1.3.1	 Etiology
				48.1.3.2	 Pathology and Genetics
					Cytogenetics
				48.1.3.3	 Investigations
				48.1.3.4	 Clinical Features
				48.1.3.5	 Management
				48.1.3.6	 Outcome
			48.1.4	 Malignant Melanoma
				48.1.4.1	 Clinical Features
				48.1.4.2	 Management
				48.1.4.3	 Prognosis
			48.1.5	 Pancreatic Tumors
				48.1.5.1	 Pancreatoblastoma
				48.1.5.2	 Islet Cell Hyperplasia
				48.1.5.3	 Solid Pseudopapillary Epithelial Neoplasm (Better Known as Frantz’s Tumor)
			48.1.6	 Thyroid Tumors
				48.1.6.1	 Etiological Factors
				48.1.6.2	 Clinical Features
				48.1.6.3	 Investigation
				48.1.6.4	 Surgery
		Further Reading
Part VIII: Gastrointestinal
	49: Gastro-Esophageal Reflux
		49.1	 Normal Physiology
		49.2	 Pathophysiology
		49.3	 Associations
		49.4	 Clinical Features
			49.4.1	 Relationship with Airway Problems
			49.4.2	 Investigations
		49.5	 Management
			49.5.1	 Medical
			49.5.2	 Surgery—Fundoplication (Fig. 49.1)
				49.5.2.1	 Indications
				49.5.2.2	 Relative Contraindications
				49.5.2.3	 Pre-Operative Evaluation
				49.5.2.4	 Surgical Principles
				49.5.2.5	 Procedure (Nissen, Thal, and Toupet) (Fig. 49.1)
				49.5.2.6	 Complications
				49.5.2.7	 Gastro-Jejunal Feeding
				49.5.2.8	 Other Surgical Interventions
		49.6	 Outcome
		Further Reading
	50: Management of Gastrointestinal Bleeding
		50.1	 Background
			50.1.1	 Initial Presentation and Assessment
			50.1.2	 Management
				50.1.2.1	 Upper GI Bleed (Fig. 50.1)
				50.1.2.2	 Lower Gastrointestinal Bleeding (Fig. 50.3)
		Further Reading
	51: Acute Abdomen
		51.1	 Clinical Features
			51.1.1	 Acute Appendicitis (See also Chap. 53)
			51.1.2	 Intussusception (See also Chap. 54)
			51.1.3	 Incarcerated Inguinal Hernia
			51.1.4	 Ovarian Torsion
	52: Miscellaneous Causes of Abdominal Pain
		52.1	 Introduction
		52.2	 Functional Abdominal Pain Disorders
			52.2.1	 Functional Dyspepsia
			52.2.2	 Abdominal Migraine
			52.2.3	 Irritable Bowel Syndrome
		52.3	 Helicobacter Pylori
			52.3.1	 Clinical Features
			52.3.2	 Investigation
			52.3.3	 Eradication
		52.4	 Yersinia Infections
		52.5	 Pediatric Inflammatory Multisystem Syndrome (PIMS or PIMS-TS)
			52.5.1	 Key Clinical Features
		52.6	 Lead Poisoning (Painter’s Colic)
			52.6.1	 Clinical Features
			52.6.2	 Investigation
		52.7	 Porphyrias
			52.7.1	 Acute Intermittent Porphyria (AIP): Example
				52.7.1.1	 Clinical Features
				52.7.1.2	 Investigations
		52.8	 Familial Mediterranean Fever
			52.8.1	 Clinical Features
			52.8.2	 Investigation
		Further Reading
	53: Acute Appendicitis
		53.1	 Epidemiology
		53.2	 Pathology
			53.2.1	 Reasons for Increased Perforation in the Young
		53.3	 Clinical Features
			53.3.1	 Named Signs in Appendicitis
		53.4	 Investigations
			53.4.1	 Investigations
		53.5	 Management
			53.5.1	 Timing
			53.5.2	 Open Appendicectomy
			53.5.3	 Laparoscopic Appendectomy
		53.6	 Outcome
		Further Reading
	54: Intussusception
		54.1	 Epidemiology
		54.2	 Pathogenesis
		54.3	 Clinical Features
			54.3.1	 Investigations
		54.4	 Management
		54.5	 Surgery
			54.5.1	 Outcome
			54.5.2	 Laparoscopic Reduction
		Further Reading
	55: Crohn’s Disease
		55.1	 Epidemiology
		55.2	 Etiology
		55.3	 Pathology
		55.4	 Clinical Features
		55.5	 Differential Diagnosis
		55.6	 Investigations
		55.7	 Management
			55.7.1	 Indications for Surgery
			55.7.2	 Surgical Options
				55.7.2.1	 Fistulas
				55.7.2.2	 Perianal disease
		55.8	 Outcome
		Further Reading
	56: Ulcerative Colitis
		56.1	 Introduction
		56.2	 Epidemiology
		56.3	 Clinical Features
			56.3.1	 Investigations
		56.4	 Management
			56.4.1	 Surgical Treatment of UC
			56.4.2	 Complications
		Further Reading
	57: Constipation
		57.1	 Introduction
		57.2	 Clinical Presentation
		57.3	 Laxatives
		57.4	 Investigations
			57.4.1	 X-rays
			57.4.2	 Rectal Biopsy
			57.4.3	 Transit Studies
			57.4.4	 Contrast Enema
			57.4.5	 Pelvic Ultrasound
			57.4.6	 Anorectal Manometry (ARM)
		57.5	 Role of Manual Evacuation
		57.6	 Role of Rectal Irrigation and Antegrade Continent Enema (ACE)
		57.7	 Role of Botox Injection in Anal Sphincter
		57.8	 Surgical Management with Sigmoid Colectomy with or Without Colostomy
	58: Small Bowel Transplantation
		58.1	 History
		58.2	 Indications
		58.3	 Assessment
			58.3.1	 Abdominal Domain Expansion
			58.3.2	 Nutritional Preassessment
			58.3.3	 Evaluation of Central Veins
			58.3.4	 Viral Screening
		58.4	 Surgery
			58.4.1	 Donor Selection
			58.4.2	 Donor Surgery
				58.4.2.1	 Isolated Intestine Retrieval
				58.4.2.2	 Combined Liver and Intestine Retrieval
				58.4.2.3	 Multivisceral Graft Retrieval
			58.4.3	 Recipient Surgery
				58.4.3.1	 Isolated Intestinal Graft
				58.4.3.2	 Combined Liver and Intestinal Graft
				58.4.3.3	 Multivisceral Transplantation
			58.4.4	 Postoperative Care
			58.4.5	 Complications
				58.4.5.1	 Rejection (Common)
				58.4.5.2	 Sepsis
				58.4.5.3	 Post-transplant Lymphoproliferative Disease
				58.4.5.4	 Graft Versus Host Disease (GVHD)
		58.5	 Outcomes
		Further Reading
Part IX: Miscellaneous
	59: Surgical Neck Pathology
		59.1	 Introduction
		59.2	 Embryology
			59.2.1	 Branchial1 Arch Development
			59.2.2 Thyroid Development
		59.3	 Branchial Fistula/Sinus/Cyst (Rare)
			59.3.1 Clinical Features
			59.3.2 Investigation
			59.3.3 Surgery
		59.4	 Thyroglossal Duct Cyst
			59.4.1 Clinical and Pathological Features
			59.4.2 Investigation
			59.4.3 Surgery: Sistrunk’s Operation
		59.5	 Cervical Node Infections (Common)
			59.5.1 Clinical Features
		59.6	 Nontuberculous Mycobacterium (NTM) (Aka Atypical Mycobacterium)
			59.6.1	 Clinico-Pathology
			59.6.2 Investigation
			59.6.3 Management
		59.7	 Tuberculosis
		59.8	 Cat Scratch Disease or Fever, Felinosis
			59.8.1 Clinical Features
			59.8.2 Investigations
		59.9	 Kawasaki’s Disease
			59.9.1 Clinical Features
			59.9.2 Investigation
			59.9.3 Management
		59.10	 Sternocleidomastoid “Tumor” (Rare)
			59.10.1 Clinical Features
			59.10.2 Investigation
			59.10.3 Management
		59.11	 Lymphatic Malformations
			59.11.1 Associations
			59.11.2 Cystic Hygroma
				59.11.2.1	 Clinical Features
				59.11.2.2	 Investigation
				59.11.2.3	 Management
		59.12	 Dermoid Cysts and Epidermoid Cysts (Common)
		Further Reading
	60: Fetal Surgery: General Principles
		60.1	 Introduction
		60.2	 Ethical Concerns
		60.3	 Accessing the Fetus
		60.4	 Specific Conditions
			60.4.1	 Congenital Diaphragmatic Hernia
			60.4.2	 Twin Anomalies
				60.4.2.1	 Twin-Twin Transfusion Syndrome
				60.4.2.2	 Twin Reversed Arterial Perfusion (TRAP) Sequence
			60.4.3	 Fetal Mass Lesions with Hydrops
			60.4.4	 Early Pregnancy Renal Anhydramnios
			60.4.5	 Bladder Outlet Obstruction (BOO) and Fetal Renal Failure
			60.4.6	 Myelomeningocele (Spina Bifida)
			60.4.7	 Aortic Valve Stenosis
			60.4.8	 Amniotic Band Syndrome
			60.4.9	 Congenital High Airway Obstruction Syndrome
		60.5	 The Future of Fetal Surgery
		Further Reading
	61: Basic Pediatric Laparoscopy and Thoracoscopy
		61.1	 Introduction
		61.2	 General Principles
			61.2.1	 Positioning of the Patient and Port Placement
			61.2.2	 Pneumoperitoneum
			61.2.3	 Pneumothorax
			61.2.4	 Instrumentation
			61.2.5	 Standard Working Pressures
		61.3	 Pyloromyotomy for Pyloric Stenosis (See Chap. 16)
		61.4	 Laparoscopic Fundoplication and Gastrostomy Button (See Chap. 49)
			61.4.1	 Five-Port Nissen Technique
		61.5	 Malrotation and Ladd’s Procedure (See Chap. 19)
		61.6	 Thoracoscopic Procedures
			61.6.1	 Empyema
			61.6.2	 Technique
		61.7	 Esophageal Atresia Repair (See Chap. 14)
			61.7.1	 Technique
		Further Reading
	62: Vascular Anomalies
		62.1	 Pathology
		62.2	 Hemangioma Versus Vascular Malformation
		62.3	 Specific Examples (Table 62.2)
			62.3.1	 Kasabach-Merritt Syndrome
			62.3.2	 Klippel-Trenaunay Syndrome (KTS)
			62.3.3	 Parkes Weber Syndrome (PWS)
			62.3.4	 Sturge-Weber Syndrome (SWS)
		62.4	 Investigations
		62.5	 Treatment
		62.6	 Complications
		Further Reading
	63: Miscellaneous Surgical Issues
		63.1	 Tongue-Tie (Ankyloglossia)
			63.1.1	 Clinical Features
			63.1.2	 Management
		63.2	 Umbilical Issues
			63.2.1	 Embryology and Anatomy
			63.2.2	 Umbilical Hernia
				63.2.2.1	 Associations
				63.2.2.2	 Clinical Features
				63.2.2.3	 Management
				63.2.2.4	 Surgery
			63.2.3	 Umbilical swellings
		63.3	 Rectal Prolapse
			63.3.1	 Clinical Features
			63.3.2	 Differential
			63.3.3	 Investigations
			63.3.4	 Management
			63.3.5	 Surgery
		Further Reading
	64: Bariatric Surgery
		64.1	 Definitions
		64.2	 Prevalence of Childhood/Adolescent Obesity
		64.3	 Clinical Features
		64.4	 Surgery
			64.4.1	 Preoperative Management
			64.4.2	 Bariatric Operations
		64.5	 Outcomes
		Further Reading
	65: Airway Problems in Newborns and Children
		65.1	 Observations
		65.2	 Pathogenesis (Table 65.1)
		65.3	 Clinical Features (Table 65.2)
			65.3.1	 Stridor and Wheezing
		65.4	 Investigations
		65.5	 Management
			65.5.1	 Surgery of the Pediatric Airway Disorders
		65.6	 Outcomes
		Further Reading
	66: Quality and Care Indicators
		66.1	 Introduction
		66.2	 What Is Quality?
			66.2.1	 Institute of Medicine: USA
			66.2.2	 Care Quality Commission (CQC): UK
		66.3	 How Can We Improve Quality?
			66.3.1	 Quality Improvement Approaches
			66.3.2	 Business Process Reengineering
			66.3.3	 Experience-Based Co-design
			66.3.4	 Lean
			66.3.5	 Model for Improvement (Including PDSA)
			66.3.6	 Six Sigma
			66.3.7	 Statistical Process Control
			66.3.8	 Theory of Constraints
		66.4	 Who Provides the Standards for Quality of Care?
		66.5	 Quality of Care in Pediatric Surgery
		Further Reading
	67: Pediatric Orthopedics
		67.1	 Normal Variants in Early Years
		67.2	 Congenital Orthopedic Conditions
			67.2.1	 Developmental Dysplasia of Hip (DDH) (Fig. 67.1)
			67.2.2	 Congenital Talipes Equino-Varus (CTEV)
		67.3	 Acquired Orthopedic Conditions
			67.3.1	 Inflammation and Infection
			67.3.2	 Perthes Disease
			67.3.3	 Slipped Upper Femoral Epiphysis (SUFE)
			67.3.4	 Anterior Knee Pain in Adolescents
			67.3.5	 Osteochondritis at Various Stages of Growth
				67.3.5.1	 Kohler’s Disease of the Navicular
				67.3.5.2	 Sever’s Disease of the Calcaneum
				67.3.5.3	 Osgood-Schlatter’s Disease of the Tibial Tuberosity
				67.3.5.4	 Osteochondritis Dissecans of the Knee
			67.3.6	 Neoplasm, Benign, and Malign
				67.3.6.1	 Bone Cysts
				67.3.6.2	 Fibrous Dysplasia
				67.3.6.3	 Osteochondroma
				67.3.6.4	 Osteosarcoma
					Chondroblastoma
	68: Robotic Pediatric Surgery
		68.1	 Introduction
		68.2	 The Da Vinci™ System and Other Robots
		68.3	 Advantages
		68.4	 Disadvantages
		68.5	 Indications
			68.5.1	 Urological Surgery
			68.5.2	 General Surgery
			68.5.3	 Cardiothoracic Surgery
			68.5.4	 Oncological Surgery
		68.6	 The Future
		Further Reading
	69: Numerical References
		69.1	 Fluid Requirements (Table 69.1)
		69.2	 Normal Heart Rate; Respiratory Rate; Blood Pressure (Table 69.2)
		69.3	 Conversion Rates
		69.4	 Average and Normal (Table 69.4)
		69.5	 Normal Hematological Values (Table 69.5)
		69.6	 Calorie Requirements (Table 69.6)
		69.7	 Common Antibiotic Doses (Tables 69.7 and 69.8)
	70: Anatomical References
		70.1	 Cranial Nerves
		70.2	 Dermatomes and Myotomes
		70.3	 Brachial Plexus
		70.4	 Segmental Liver Anatomy
		70.5	 The Lungs and Diaphragm
		70.6	 Heart
		70.7	 Gastrointestinal Tract
		70.8	 Genitourinary System
		70.9	 Inguinal Anatomy
		70.10	 Pharyngeal Arches
	71: Human Developmental Milestones
		71.1	 Prenatal Development
			71.1.1	 “Egg” Phase: 1–4 Days
			71.1.2	 Embryo Phase
			71.1.3	 Fetal Phase
		71.2	 Ultrasound Scans in Pregnancy
		71.3	 Developmental Milestones
		Further Reading
Index