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