Pediatric Surgery: Diagnosis and Management

Description

This comprehensive book provides detailed practical advice on the diagnosis and surgical management of congenital and acquired conditions in infants and children. Following the great success of the first edition, the new edition has been fully updated to reflect the major developments in the field of the past decade. Advances in prenatal diagnosis, imaging, anaesthesia and intensive care as well as the introduction of new surgical techniques, including minimally invasive surgery and robotic technology that have radically altered surgical conditions are now discussed in the book. In addition new chapters have been added on surgical safety in children, surgical problems of children with disabilities and surgical implications of HIV infection in children among others. This is a superbly structured and richly illustrated guide aimed at trainees, young pediatric surgeons and general surgeons with interest in pediatric surgery. The book also serves as a handy, quick and easy reference for those making decisions in daily practice. Pediatric Surgery, Diagnosis and Management features contributions by leading experts in pediatric surgery and pediatric urology who have unique experience in their respective fields. Preface to the Second Edition Contents Contributors Part I: General Principles 1: The Epidemiology of Birth Defects 1.1 Introduction 1.1.1 Birth Defects Are Leading Causes of Infant Mortality and Long-term Morbidity Worldwide 1.1.2 Birth Defects Epidemiology and Teratology Have Emerged from Outbreak Investigations 1.1.3 Causation of Birth Defects Remains Often Complex and Poorly Understood 1.1.4 Birth Defects Appear to Arise Typically (But Not Exclusively) in the First Trimester 1.1.5 Classification of Birth Defects for Epidemiological Purposes 1.1.6 Counting of Birth Defects Is Affected by the Definition of Stillbirth 1.1.7 Prenatal Diagnosis: The Greatest Challenge to Birth Defect Epidemiology? 1.1.8 Pediatric Surgeons Often Focus on Their Institutional Series of Birth Defects 1.1.9 A “Life-Course” Approach to Birth Defects 1.2 Conclusion and Future Directions References 2: Fetal Counselling for Surgical Congenital Malformations 2.1 Introduction 2.2 Historical Overview 2.3 Incidence 2.4 Prenatal Diagnosis 2.4.1 Screening for Fetal Anomalies 2.4.2 Invasive Diagnostic Tests 2.4.2.1 Amniocentesis 2.4.2.2 Chorionic Villous Sampling (CVS) 2.4.2.3 Prenatal Maternal Serum Screening 2.4.2.4 Fetal Blood Sampling (FBS) 2.4.2.5 Fetal Surgery 2.4.2.6 Genetic Diagnoses 2.4.2.7 Future Developments 2.5 Specific Surgical Conditions 2.5.1 Congenital Diaphragmatic Hernia (CDH) 2.5.2 Cystic Lung Lesions 2.5.3 Abdominal Wall Defects 2.5.3.1 Exomphalos 2.5.3.2 Gastroschisis 2.5.4 Tracheo-Oesophageal Fistula (TOF) and Oesophageal Atresia (OA) 2.5.5 Gastrointestinal Lesions 2.5.6 Sacrococcygeal Teratoma 2.5.7 Renal Anomalies 2.5.7.1 Upper Urinary Tract Obstruction 2.5.7.2 Lower Urinary Tract Obstruction 2.6 Conclusion References 3: Transport of the Surgical Neonate 3.1 Introduction 3.2 Prenatal Transfer 3.3 Transfer Management 3.3.1 Pre-transfer Management 3.3.2 Transfer Team/Transfer Vehicle 3.3.3 Receiving Centre 3.4 Special Considerations 3.4.1 Gastroschisis 3.4.2 Omphalocele 3.4.3 Pierre Robin Syndrome 3.4.4 Choanal Atresia 3.4.5 Myelomeningocele 3.4.6 Bladder Exstrophy 3.4.7 Cloacal Exstrophy 3.4.8 Oesophageal Atresia 3.4.9 Congenital Diaphragmatic Hernia 3.4.10 Intestinal Obstruction 3.4.11 Necrotizing Enterocolitis 3.5 Conclusion References 4: Pre-operative Management and Vascular Access 4.1 Introduction 4.2 Prenatal Diagnosis 4.3 History and Physical Examination 4.4 Maintenance of Body Temperature 4.5 Respiratory Function 4.6 Cardiovascular Status 4.7 Metabolic Status 4.7.1 Acid–Base Balance 4.7.2 Hypoglycaemia 4.7.3 Hyperbilirubinaemia 4.7.4 Coagulation Abnormalities 4.7.5 Laboratory Investigations 4.7.6 Fluid and Electrolytes, and Metabolic Responses 4.7.7 Renal Function, Urine Volume and Concentration in the Newborn 4.8 Pain in Neonates 4.9 Pre-operative Management in the Older Child 4.10 Vascular Access References 5: Anaesthesia and Analgesia 5.1 Introduction 5.2 Pre-operative Evaluation and Preparation 5.2.1 History 5.2.2 Physical Examination 5.2.3 Laboratory Investigations 5.2.4 Premedication 5.2.5 Post-operative Planning 5.3 Operating Theatre and Anaesthetic Equipment 5.3.1 Breathing Systems 5.3.2 Laryngoscopes 5.3.3 Ventilators 5.3.4 Monitoring Equipment 5.4 Induction of Anaesthesia 5.4.1 Parental Presence 5.5 Intravenous Agents 5.6 Inhalational Agents 5.6.1 Halothane 5.6.2 Isoflurane 5.6.3 Enflurane 5.6.4 Desflurane 5.6.5 Sevoflurane 5.6.6 Nitrous Oxide 5.7 Neuromuscular Blocking Agents 5.7.1 Succinylcholine 5.7.2 Atracurium and Vecuronium 5.7.3 Mivacurium 5.7.4 Pancuronium 5.7.5 Rocuronium 5.8 Maintenance of Anaesthesia 5.8.1 Inhalational Maintenance of Anaesthesia 5.8.2 Total Intravenous Anaesthesia (TIVA) 5.9 Reversal and Extubation 5.10 Recovery from Anaesthesia 5.11 Post-operative Care 5.11.1 Post-operative Intensive Care Management 5.12 Monitoring 5.13 Cardiovascular Monitoring 5.13.1 Precordial and Oesophageal Stethoscope 5.13.2 ECG 5.13.3 Blood Pressure 5.13.4 Central Venous Pressure 5.14 Respiratory Monitoring 5.14.1 Pulse Oximetry 5.14.2 Capnography 5.14.3 Ventilator Pressure/Volume Monitoring 5.15 Temperature Monitoring 5.16 Neuromuscular Blockade Monitoring 5.17 Other Monitoring 5.18 Fluid Balance 5.19 Special Considerations for the Premature Infant 5.20 Anaesthesia for Specific Surgical Conditions 5.20.1 Oesophageal Atresia 5.20.2 Congenital Diaphragmatic Hernia 5.20.3 Intestinal Obstruction 5.20.4 Exomphalos and Gastroschisis 5.20.5 Myelomeningocele, Shunt (and Revision Shunt) for Spina Bifida 5.20.6 Muscle Biopsy 5.20.7 Herniotomy in the Ex-Premature Infant 5.21 Post-operative Analgesia in Children 5.21.1 Local and Regional Anaesthesia 5.21.2 Opioids 5.21.3 Non-steroidal Anti-inflammatory Drugs (NSAIDs) 5.21.4 Paracetamol (Acetaminophen) 5.22 Day-Case Anaesthesia and Surgery 5.22.1 Preparation of Child and Parents 5.22.2 Premedication 5.22.3 Anaesthetic Technique 5.22.4 Analgesia for Day-Cases 5.22.5 Post-operative Nausea and Vomiting 5.22.6 Discharge Criteria 5.22.7 Reasons for Hospital Admission 5.22.8 Transport Home 5.23 Some Topics of Current Interest to Both Anaesthetists and Surgeons 5.23.1 Fasting Prior to Anaesthesia and Surgery 5.23.2 Upper Respiratory Tract Infection 5.23.3 Anaesthesia and Immunization 5.24 Enhanced Recovery After Surgery (ERAS) in the Paediatric Population 5.25 Conclusion References 6: Respiratory Management of the Surgical Patient 6.1 Introduction 6.2 Respiratory Physiology 6.2.1 Gas Exchange 6.2.1.1 Oxygen Uptake 6.2.1.2 Carbon Dioxide Elimination 6.3 Mechanical Ventilation 6.3.1 Pressure-Control Ventilation 6.3.2 Volume-Control Ventilation 6.3.3 Support Modes 6.3.4 Triggering the Ventilator 6.3.5 Setting Ventilator Parameters 6.4 Alternative Modes of Ventilation 6.4.1 High-Frequency Oscillatory Ventilation (HFOV) 6.4.2 Non-invasive Ventilation (NIV) 6.5 Endotracheal Tube (ETT) Size and Positioning 6.6 Care of the Intubated Patient 6.6.1 Humidification 6.6.2 Endotracheal Tube Suctioning 6.7 Respiratory Monitoring 6.7.1 Invasive Monitoring 6.7.2 Non-invasive Monitoring 6.7.2.1 Pulse Oximetry (SpO2) 6.7.2.2 End-Tidal Carbon Dioxide (PETCO2) 6.7.2.3 Transcutaneous Carbon Dioxide (TcPCO2) 6.8 Conclusions References 7: Fluid Management 7.1 Paediatric Fluid Haemostasis 7.2 Intake 7.3 Output 7.4 Fluid Therapy 7.4.1 Historical Basis: Composition and Rate 7.4.2 Hyponatraemia and ADH Release 7.4.3 Isotonic Crystalloids 7.5 Colloids 7.6 Hypoglycaemia 7.7 The ‘Ideal’ Fluid 7.8 Conclusion References 8: Sepsis 8.1 Introduction Including Definition and Incidence 8.2 Risk Factors 8.2.1 Barriers to Infection 8.3 Pathophysiology of Sepsis 8.3.1 Bacterial Virulence 8.3.2 Microbiome 8.3.3 Neutrophils 8.3.4 Monocytes–Macrophages 8.3.5 Lymphocytes 8.3.6 Immunoglobulins 8.3.7 Cytokines 8.4 Neonates 8.5 Clinical Features and Diagnosis 8.6 Management 8.7 Outcomes 8.8 Conclusion Reference Further Reading 9: Nutrition 9.1 Introduction 9.2 Historical Background 9.3 Body Composition 9.4 Energy Metabolism 9.5 Parenteral Nutrition 9.5.1 Indications 9.5.2 Components of Parenteral Nutrition 9.5.2.1 Fluid Requirements 9.5.2.2 Energy Sources 9.5.2.3 Amino Acids 9.5.2.4 Vitamins and Trace Elements 9.5.3 Complications of Parenteral Nutrition (13) 9.5.3.1 Infectious Complications 9.5.3.2 Mechanical Complications 9.5.3.3 Hepatic Complications 9.6 Enteral Nutrition 9.6.1 Selection of Enteral Feeds 9.6.2 Administration of Enteral Feeds 9.6.3 Complications of Enteral Tube Feeding 9.7 Conclusions References 10: Access for Enteral Nutrition 10.1 Introduction 10.2 Naso-, Orogastric or Naso-, Oroenteric Access 10.2.1 Indication 10.2.2 Insertion and Verification of Placement 10.2.3 Complications 10.2.3.1 Misplacement and Displacement 10.2.3.2 Long-Term Complications 10.2.3.3 Tube Plugging 10.3 Gastrostomy 10.3.1 Indications 10.3.2 Techniques 10.3.3 Bolus and Continuous Feeds 10.3.4 Complications 10.4 Jejunal Access 10.4.1 Indications 10.4.2 Techniques and Feeding 10.4.3 Complications 10.5 Conclusion References 11: Hematological Problems in Pediatric Surgery 11.1 Introduction 11.2 Historical Overview 11.3 Incidence 11.4 Etiopathogenesis 11.4.1 Blood Formation (Hematopoiesis) 11.4.2 Mechanisms of Hemostasis 11.4.3 Natural Anticoagulation Control Mechanisms 11.4.4 Platelets 11.4.5 Blood Groups and Antibodies 11.5 Pathophysiology 11.6 Hematological Disorders Encountered in Pediatric Practice: A Surgical Perspective 11.6.1 Inherited Disorders of Coagulation 11.6.1.1 Hemophilia Diagnosis Differential Diagnosis Management 11.6.1.2 Central Venous Access Devices 11.6.1.3 von Willebrand Disease (VWD) 11.6.2 Platelet Disorders 11.6.2.1 Differential Diagnosis 11.6.2.2 Immune Thrombocytopenia Purpura (ITP) Diagnosis Pathophysiology Management (Neunert et al. 2019) 11.6.3 Disseminated Intravascular Coagulation (DIC) 11.6.3.1 Diagnosis 11.6.3.2 Differential Diagnosis 11.6.3.3 Pathophysiology 11.6.3.4 Management (Rajagopal et al. 2017) 11.6.4 Thrombotic Disorders 11.6.4.1 Diagnosis Differential Diagnosis 11.6.4.2 Management 11.6.5 Asplenia/Hyposplenism/Splenectomy 11.6.5.1 Diagnosis 11.6.5.2 Differential Diagnosis 11.6.5.3 Management 11.6.6 Anemia 11.6.6.1 Pathophysiology 11.6.6.2 Diagnosis 11.6.6.3 Management Hereditary Spherocytosis (HS) 11.6.6.4 Management of HS Sickle Cell Disease (SCD) 11.6.6.5 Diagnosis of SCD 11.6.6.6 Differential Diagnosis 11.6.6.7 Clinical Features and Management of SCD (Murad et al. 2019) 11.6.6.8 Chronic Complications 11.6.6.9 Disease-Modifying Therapy 11.6.6.10 Surgery in Sickle Cell Disease Thalassemia 11.6.7 Neutropenia 11.6.7.1 Diagnosis 11.6.7.2 Differential Diagnosis 11.6.7.3 Management 11.6.8 Leukemia 11.6.8.1 Diagnosis 11.6.8.2 Management of Acute Leukemia 11.6.8.3 Surgical Issues in the Leukemic Patient 11.6.9 Blood Products and Their Use in Children 11.6.9.1 Acute Complications of Blood Transfusion Other Adverse Reactions to Blood Product Transfusion 11.7 Conclusions References 12: Genetics 12.1 Introduction 12.2 A Clinical Genetic Approach to Diagnosis of Malformation Syndromes 12.2.1 Definitions 12.2.2 An Approach to Diagnosis of a Malformation Syndrome 12.3 Genetic Aetiology of Congenital Anomalies 12.3.1 Introduction 12.3.2 Chromosome Disorders 12.3.3 Specific Chromosome Disorders 12.3.3.1 Down’s Syndrome 12.3.3.2 Patau’s Syndrome 12.3.3.3 Edward’s Syndrome 12.3.3.4 Other Chromosome Disorders 12.4 Single Gene Disorders 12.4.1 Autosomal Dominant Inheritance 12.4.2 Autosomal Recessive Inheritance 12.4.3 X-Linked Inheritance 12.4.4 Mitochondrial Inheritance 12.5 Polygenic Disorders 12.6 Next Generation Sequencing 12.7 Conclusions and Future Directions References 13: Ethical Considerations in Pediatric Surgery 13.1 Introduction 13.2 Guidelines for Ethical Decision-Making and Resolution of Ethical Problems 13.3 Informed Consent, Assent, and Dissent 13.3.1 Exceptions to Informed Consent 13.4 Withholding and Withdrawal of Life-Sustaining Treatment 13.5 Multiculturalism 13.6 Surgical Error 13.7 Research and Innovation in Pediatric Surgery 13.8 Ethical Issues in Bariatric Surgery of the Pediatric Patient 13.9 Ethical Issues in the Treatment of Gender Dysphoria in Children and Adolescents 13.10 Physician Wellness 13.11 Conclusion References 14: Minimal Access Surgery in Infants and Children 14.1 Introduction 14.1.1 Laparoscopic and Thoracoscopic Surgery: Technical Considerations 14.2 Thoracoscopic Procedures 14.2.1 Diagnostic Thoracoscopy 14.2.2 Biopsies for Pulmonary Pathologies 14.2.3 Pleural Empyema 14.2.4 Congenital Pulmonary Airway Malformations and Pulmonary Sequestration 14.2.5 Primary Spontaneous Pneumothorax 14.2.6 Mediastinal Masses 14.2.7 Patent Ductus Arteriosus 14.2.8 Esophageal Atresia 14.2.9 Congenital Diaphragmatic Hernias and Diaphragmatic Eventration 14.3 Laparoscopic Procedures 14.3.1 Inguinal Hernia 14.3.2 Pyloric Stenosis 14.3.3 Duodenal Atresia 14.3.4 Malrotation 14.3.5 Gastroesophageal Reflux and Gastric Feeding 14.3.6 Achalasia 14.3.7 Gall Bladder Pathologies 14.3.8 Splenic Pathologies 14.3.9 Pancreatic Pathologies 14.3.10 Meckel’s Diverticulum 14.3.11 Intussusception 14.3.12 Appendicectomy 14.3.13 Anorectal Malformations 14.3.14 Hirschsprung’s Disease 14.3.15 Inflammatory Bowel Disease 14.4 Genitourinary System 14.4.1 Vesicoureteral Reflux 14.4.2 Ureteropelvic Junction Obstruction 14.4.3 Benign Renal Pathologies 14.4.4 Ovarian Pathologies 14.4.5 Impalpable Testis 14.5 Pediatric Oncology 14.6 Conclusion References 15: Surgical Safety in Children 15.1 Introduction 15.2 Measures to Improve Safety 15.2.1 Checklists 15.2.2 Reporting and Learning 15.2.3 Non-technical Skills 15.3 Safety Concerns Specific to Surgery 15.4 Safety Concerns Specific to Paediatric Surgery 15.4.1 Size 15.4.2 Anatomy 15.4.3 Growth 15.4.4 Immature Organ Systems 15.4.5 Acuity of Problems 15.4.6 Co-morbidities 15.4.7 Healthcare System Limitations 15.5 Summary and Conclusions References 16: Surgical Problems of Children with Physical Disabilities 16.1 Introduction 16.2 General Considerations 16.2.1 Multidisciplinary Management, the Medical Home, and Care Coordination 16.2.2 Family-Centered Care 16.2.3 Medical Complexity and Polypharmacy 16.2.4 Contemporary Pain Management and Pre-operative Anesthesia Evaluation 16.2.5 Ethical Complexity 16.2.6 Abuse and Neglect 16.3 Perioperative Organ System Considerations 16.3.1 Neurologic 16.3.1.1 Seizure Disorders (Epilepsy) 16.3.1.2 Spasticity, Body Habitus, and Scoliosis 16.3.1.3 Hydrocephalus Drainage and Shunt Considerations 16.3.1.4 Neurologic Impairment and the Acute Abdomen 16.3.2 Gastrointestinal 16.3.2.1 Nutrition 16.3.2.2 Neurologic Dysphagia, Aspiration, and Feeding Disorders 16.3.2.3 Complications of Enteral Access and Fundoplication 16.3.2.4 Constipation and Defecation Disorders 16.3.3 Cardiovascular 16.3.3.1 Congenital Heart Disease 16.3.4 Respiratory 16.3.5 Integumentary 16.3.5.1 Latex Allergy 16.3.5.2 Decubitus Ulcers 16.3.6 Genetic Syndromes 16.4 Conclusions and Future Directions References 17: Surgical Aspects of HIV Infection in Children 17.1 Introduction and Historical Overview 17.2 Incidence 17.3 Etiopathogenesis 17.4 Pathophysiology 17.5 Pathology 17.6 Diagnosis and Differential Diagnosis 17.6.1 Infections 17.7 Gastrointestinal Tract Disease 17.8 Intra-abdominal Pathology 17.9 Perineal Disease 17.10 Malignancy 17.11 Management and Outcomes 17.12 Factors Influencing Post-surgical Complications 17.13 HIV Exposed But Uninfected Children 17.14 Conclusion References Uncategorized References Part II: Trauma 18: Birth Trauma 18.1 Introduction 18.2 Head 18.2.1 Extracranial Haematomas 18.2.1.1 Caput Succedaneum 18.2.1.2 Subgaleal Haemorrhage 18.2.1.3 Cephalhaematoma 18.2.2 Cranial Injuries 18.2.3 Intracranial Haemorrhage 18.2.3.1 Subdural Haemorrhage 18.2.3.2 Subarachnoid Haemorrhage 18.2.3.3 Epidural Haemorrhage 18.3 Injuries to Peripheral Nerves 18.3.1 Brachial Plexus Injury 18.3.1.1 Erb’s Palsy 18.3.1.2 Klumpke’s Paralysis 18.3.1.3 Injury to Entire Brachial Plexus 18.3.2 Facial Nerve Injury 18.3.3 Phrenic Nerve Injury 18.3.4 Spinal Cord Injury 18.4 Abdominal Organ Injuries 18.5 Fractures 18.5.1 Fracture of Clavicle 18.5.2 Long Bone Fractures 18.6 Conclusions References 19: Pediatric Thoracic Trauma 19.1 Introduction 19.2 Diagnosis 19.3 Differential Diagnosis 19.4 Injury Management 19.5 Immediately Life-Threatening Injuries Found During Primary Survey 19.5.1 Airway Obstruction 19.5.2 Tension Pneumothorax 19.5.3 Open Pneumothorax 19.5.4 Flail Chest 19.5.5 Hemothorax 19.5.6 Cardiac Tamponade and Commotio Cordis 19.6 Potentially Life-Threatening Injuries Found During Secondary Survey 19.6.1 Tracheobronchial Injury 19.6.2 Pulmonary Contusions 19.6.3 Myocardial Contusion 19.6.4 Diaphragmatic Injuries 19.6.5 Esophageal Rupture 19.6.6 Great Vessel Injury 19.7 Non-Life-Threatening Injuries Often Found on Physical Exam or Chest Radiograph 19.7.1 Simple Pneumothorax 19.7.2 Small Hemothorax 19.7.3 Rib Fractures 19.7.4 Chest Wall Laceration 19.7.5 Traumatic Asphyxia (Perthes Syndrome) 19.8 Conclusion Further Reading 20: Abdominal and Genitourinary Trauma 20.1 Introduction 20.2 Diagnosis 20.2.1 Computerized Tomography 20.2.2 Focused Abdominal Sonography for Trauma 20.2.3 Laparoscopy 20.2.4 The “Seat Belt Sign” 20.3 Differential Diagnosis 20.3.1 Liver and Spleen Injury 20.3.2 Bile Duct Injury 20.3.3 Abdominal Compartment Syndrome 20.3.4 Duodenal and Pancreatic Injury 20.3.4.1 Duodenum 20.3.4.2 Pancreas 20.3.5 Renal Injury 20.4 Management 20.4.1 Spleen and Liver Injury 20.4.1.1 Management of the Stable Pediatric Patient with Blunt Spleen or Liver Injury 20.4.1.2 Management of the Pediatric Patient with Blunt Spleen or Liver Injury and Ongoing Bleeding 20.4.2 Interventions for Blunt Spleen or Liver Injury 20.4.2.1 Angioembolization 20.4.2.2 Damage Control Surgery 20.4.3 Bile Duct Injury 20.4.4 Abdominal Compartment Syndrome 20.4.5 Duodenal and Pancreatic Injury 20.4.5.1 Duodenum 20.4.5.2 Pancreas 20.4.6 Renal Injury 20.4.6.1 Renal Interventions 20.5 Conclusion References 21: Surgical Treatment of Severe Head Trauma 21.1 Introduction 21.2 Principles of Treatment 21.3 Management 21.3.1 Mild Head Trauma 21.3.2 Severe Head Injuries 21.3.3 Intensive Care Unit 21.4 Surgical Management of Increased Intracranial Pressure 21.5 Skull Fracture 21.6 Depressed Skull Fractures 21.7 Leptomeningeal Cysts 21.8 Penetrating Cerebral Injuries 21.9 Mass Lesions After Head Injury 21.10 Epidural Hematomas 21.11 Subdural Hematomas 21.12 Intracerebral Hematomas 21.13 Conclusion and Future Direction References 22: Pediatric Orthopedic Trauma 22.1 Introduction 22.2 Brief Historical Overview 22.3 Incidence 22.4 Pathophysiology 22.5 Classification 22.5.1 General Classification 22.5.2 Specific Pediatric Fractures 22.5.2.1 Diaphyseal Fractures Greenstick Fracture Toddler’s Fracture 22.5.2.2 Metaphyseal Fractures Buckle Fracture Incomplete Fracture 22.5.2.3 AO Pediatric Comprehensive Classification of Long-Bone Fractures (PCCF) Guidelines for Correct Classification 22.6 Diagnosis 22.7 Management 22.7.1 Therapy Principles 22.7.1.1 Conservative Methods 22.7.1.2 Surgical Methods 22.7.2 Conservative Treatment 22.7.3 Specific Pediatric Fracture treatments 22.7.3.1 Monteggia Fracture 22.7.3.2 Supracondylar Humeral Fracture 22.7.3.3 Fracture of the Lateral Condyle 22.7.3.4 Metaphyseal Fracture of the Proximal Tibia 22.7.3.5 Fracture of the Medial Malleolus 22.8 Complications 22.9 Conclusions References 23: Injuries to the Tendons of the Hand 23.1 Introduction 23.2 History 23.3 Flexor Tendon Injuries 23.3.1 Anatomy 23.3.2 Epidemiology 23.3.3 Zones 23.3.4 Diagnosis 23.3.5 Therapy 23.3.6 Postoperative Rehabilitation 23.3.7 Outcome and Complications 23.4 Extensor Tendon Injuries 23.4.1 Anatomy 23.4.2 Zones 23.4.3 Diagnosis and Operative Treatment 23.4.4 Injuries of Zone 1 (DIP Joint) 23.4.5 Injuries of Zone 2 (Middle Phalanx) 23.4.6 Injuries of Zone 3 (PIP Joint) 23.4.7 Injuries of Zone 4 (Proximal Phalanx) 23.4.8 Injuries of Zone 5 (MCP joint) 23.4.9 Injuries of Zone 6 (Metacarpals) 23.4.10 Injuries of Zone 7 (Wrist Joint) 23.4.11 Injuries of Zone 8 (Distal Forearm) 23.4.12 Injuries of the Thumb Extensor Tendons 23.5 Conclusion References 24: Burns 24.1 Introduction 24.2 Diagnosis 24.3 Differential Diagnosis 24.4 Aetiology 24.5 Pathogenesis 24.6 Emergency Management 24.7 Burn Wound Assessment 24.8 Airway and Ventilation 24.9 Fluid Resuscitation 24.10 Analgesia and Sedation 24.11 Nutrition and the Hypermetabolic Response 24.12 Infection Prevention and Control 24.13 The Partial-Thickness Burn 24.14 The Deep Burn 24.15 Physical and Psychological Rehabilitation 24.16 Reconstructive Burn Surgery 24.17 Outcomes 24.18 Conclusions References 25: Foreign Bodies 25.1 Introduction 25.2 Airway Foreign Bodies 25.2.1 Ear 25.2.2 Nose 25.2.3 Throat 25.2.4 Larynx 25.2.5 Trachea and Bronchi 25.3 Upper Gastrointestinal Tract Foreign Bodies 25.3.1 Esophageal Foreign Bodies 25.3.1.1 Disk or Button Batteries 25.3.1.2 Coins in the Esophagus 25.4 Subdiaphragmatic Foreign Bodies 25.4.1 Magnetic Foreign Bodies 25.4.2 Genitourinary Foreign Bodies 25.5 Conclusion References 26: Physical and Sexual Child Abuse 26.1 Introduction 26.2 Historical Overview 26.3 Incidence 26.4 Physical Abuse 26.4.1 Soft-Tissue Injuries 26.4.2 Skeletal Injuries 26.4.3 Shaken Baby Syndrome 26.4.4 Abdominal and Thoracic Injuries 26.4.5 Prevention Strategies in Physical Abuse 26.5 Sexual Abuse 26.6 Munchausen By Proxy 26.7 Conclusion References Part III: Head and Neck 27: Pierre Robin Sequence 27.1 Introduction and Historical Overview 27.2 Incidence 27.3 Etiopathogenesis 27.4 Genetics 27.5 Pathology 27.6 Diagnosis 27.7 Differential Diagnosis 27.8 Management 27.8.1 Airway Management 27.8.1.1 Nasopharyngeal Tube 27.8.1.2 Endotracheal Tube 27.8.1.3 Tongue–Lip Adhesion/Glossopexy 27.8.1.4 Tracheostomy 27.8.1.5 Distraction Osteogenesis of the Mandible 27.8.1.6 Tongue Positioning and Stimulation Plate 27.8.1.7 Noninvasive Ventilation 27.8.2 Nutritional Management 27.8.3 Management of Cleft Palate 27.8.4 Management of Micrognathia/Retrognathia 27.8.5 Management of Further Associated Malformations 27.8.5.1 Skeletal Anomalies 27.8.5.2 Ear Problems 27.8.5.3 Cardiovascular Anomalies 27.8.5.4 Ocular Anomalies 27.8.6 Nasal Obstruction 27.9 Conclusion References 28: Choanal Atresia 28.1 Introduction 28.2 Etiopathogenesis 28.3 Pathophysiology 28.4 Pathology 28.5 Diagnosis 28.6 Differential Diagnosis 28.7 Emergency Treatment 28.8 Management 28.9 Endoscopic Technique 28.10 Transpalatal 28.11 Sublabial Transseptal 28.12 Conclusion References 29: Thyroglossal and Branchial Cysts, Sinuses, and Fistulas 29.1 Introduction 29.2 Etiology 29.3 Pathology 29.4 Diagnosis and Differential Diagnosis 29.5 Therapy 29.6 Conclusion References 30: Tracheostomy 30.1 Introduction and Incidence 30.2 Etiopathogenesis 30.3 Pathophysiology 30.4 Pathology 30.5 Diagnosis 30.6 Differential Diagnosis 30.7 Management 30.7.1 Technique 30.7.2 Postoperative Management 30.7.3 Home Instruction and Care 30.7.4 Complications 30.7.5 Decannulation 30.8 Conclusion References Part IV: Chest 31: Chest Wall Deformities 31.1 Introduction 31.2 Pectus Excavatum 31.2.1 Description 31.2.2 History 31.2.3 Incidence and etiology 31.2.4 Pathophysiology 31.2.5 Body Image Effects 31.2.6 Diagnosis and Differential Diagnosis 31.3 Management 31.3.1 Vacuum Bell 31.4 Nuss Procedure: Technique (Fig. 31.4) 31.4.1 Open Operation 31.4.2 Pectus Carinatum 31.4.2.1 Description 31.5 History 31.5.1 Incidence and Etiology 31.5.2 Clinical Features 31.5.2.1 Symptoms 31.5.2.2 Cardiac and Pulmonary Effects 31.5.2.3 Body Image Effects 31.5.3 Evaluation 31.5.4 Treatment 31.5.5 External Brace Treatment 31.5.6 Reverse Nuss Procedure of Abramson 31.5.7 Sandwich Technique 31.5.8 Open Operation 31.6 Uncommon Chest Wall Conditions 31.6.1 Poland’s Syndrome 31.6.2 Sternal Cleft 31.6.3 Jeune’s Syndrome 31.7 Conclusion References 32: Breast Disorders in Children and Adolescents 32.1 Introduction 32.2 Congenital and Developmental Anomalies 32.2.1 Hypoplastic Anomalies 32.2.2 Breast Atrophy 32.2.3 Polythelia and Polymastia 32.2.4 Macromastia and Breast Hypertrophy 32.2.5 Gynecomastia 32.2.6 Mastitis and Abscess 32.2.7 Nipple Discharge 32.3 Breast Masses 32.3.1 Prepubertal Masses 32.3.2 Adolescent Masses 32.3.2.1 Fibroadenomas 32.3.2.2 Phyllodes Tumors 32.3.2.3 Malignant Tumors 32.4 Conclusion Bibliography 33: Congenital Airway Malformations 33.1 Introduction 33.2 Diagnostic Evaluation 33.3 Congenital Laryngeal Anomalies 33.3.1 Laryngomalacia 33.3.1.1 Pathogenesis 33.3.1.2 Diagnosis 33.3.2 Subglottic Stenosis 33.3.2.1 Pathogenesis 33.3.2.2 Diagnosis 33.3.3 Vocal Cord Paralysis 33.3.3.1 Pathogenesis 33.3.3.2 Diagnosis 33.3.4 Posterior Laryngeal Cleft 33.3.4.1 Pathogenesis 33.3.4.2 Classification 33.3.4.3 Diagnosis 33.3.5 Laryngeal Atresia 33.3.5.1 Congenital High Airway Obstruction Syndrome (CHAOS) 33.4 Anomalies of the Trachea and Bronchi 33.4.1 Tracheal Agenesis 33.4.2 Tracheal Webs and Stenosis 33.4.2.1 Tracheal Webs 33.4.2.2 Cartilaginous Ring Aplasia 33.4.2.3 Tracheal Cartilaginous Sleeve 33.4.2.4 Complete Tracheal Rings 33.4.2.5 Diagnosis 33.4.3 Tracheal Diverticulum and Tracheal Bronchus 33.4.4 Tracheomalacia and Bronchomalacia 33.4.4.1 Pathogenesis 33.4.5 Esophageal Lung 33.4.6 Tracheobronchial Biliary Fistula 33.4.7 Subglottic Hemangioma 33.4.7.1 Pathogenesis 33.4.8 Bronchogenic Cyst 33.4.9 Bronchial Atresia 33.4.10 Bronchial Agenesis 33.4.11 Bronchial Stenosis 33.5 Conclusions References 34: Mediastinal Masses in Children 34.1 Introduction 34.2 Diagnosis 34.3 Anterior Mediastinum 34.4 Middle Mediastinum 34.5 Posterior Mediastinum 34.6 Anaesthetic Management of Children with a Mediastinal Mass 34.7 Operative Technique for Removal of Mediastinal Neuroblastoma 34.8 Conclusion Bibliography 35: Pleural Effusion and Empyema 35.1 Introduction 35.2 Etiology and Pathogenesis 35.2.1 Fetal and Congenital Pleural Effusion 35.2.2 Acquired/Pathological Pleural Effusion 35.2.2.1 Hemothorax 35.2.2.2 Chylothorax 35.2.2.3 Hydrothorax Iatrogenic Hydrothorax Pathological/Secondary Hydrothorax 35.2.2.4 Pleural Exudate and Empyema 35.3 Pathology 35.4 Clinical Features 35.5 Imaging 35.5.1 Plain Chest X-Ray in AP Position 35.5.2 Ultrasound 35.5.3 CT and MR Scanning 35.6 Diagnosis and Differential Diagnosis 35.7 Management 35.7.1 Fetal Hydrothorax 35.7.2 Congenital Chylothorax 35.7.3 Pleural Effusion in the Context of Lymphangiomatosis 35.7.4 Chylothorax After Thoracic/Cardiac Surgery 35.7.5 Pleural Effusion Related to Central Venous Catheters 35.7.6 Iatrogenic Hydrothorax 35.7.7 Non-iatrogenic Hydrothorax 35.7.8 Empyema 35.8 Complications 35.9 Conclusion References 36: Congenital Malformations of the Lung 36.1 Introduction and Historical Overview 36.2 Incidence 36.3 Anatomy 36.4 Embryology 36.5 Etiopathogenesis 36.6 Classification, Pathology, and Pathophysiology 36.6.1 Congenital Pulmonary Airway Malformation 36.6.2 Bronchopulmonary Sequestration 36.6.3 Congenital Lung Emphysema 36.6.4 Bronchogenic Cyst 36.7 Diagnosis and Differential Diagnosis 36.8 Presentation 36.8.1 Congenital Pulmonary Airway Malformation 36.8.2 Bronchopulmonary Sequestration 36.8.3 Congenital Lung Emphysema 36.8.4 Bronchogenic Cyst 36.9 Imaging 36.9.1 Ultrasound 36.9.2 Plain Radiography 36.9.3 Computed Tomography 36.9.4 Magnetic Resonance Imaging 36.9.5 Other Imaging 36.10 Management 36.10.1 Congenital Pulmonary Airway Malformation 36.10.1.1 Antenatal Therapy 36.10.1.2 Postnatal Therapy 36.10.2 Bronchopulmonary Sequestration 36.10.3 Congenital Lobar Emphysema 36.10.4 Bronchogenic Cyst 36.11 Short- and Long-Term Postoperative Outcomes 36.12 Conclusion References 37: Congenital Diaphragmatic Hernia 37.1 Introduction 37.2 Incidence 37.3 Etiopathogenesis and Embryology 37.4 Diagnosis 37.5 Differential Diagnosis 37.6 Prognostic Factors 37.7 Prenatal Treatment 37.7.1 Preoperative Management 37.7.2 Timing of Surgery 37.7.3 Surgical Technique 37.7.4 Postoperative Management 37.8 Outcome 37.9 Congenital Diaphragmatic Eventration (CDE) 37.10 Clinical Features 37.11 Diagnosis 37.12 Management 37.13 Operative Repair 37.14 Outcome 37.15 Conclusions References 38: Extracorporeal Membrane Oxygenation 38.1 Introduction 38.2 Patient Management on ECMO 38.3 Complications 38.4 Conclusion Further Reading Part V: Spina Bifida and Hydrocephalus 39: Spina Bifida and Encephalocoele 39.1 Introduction 39.2 Embryology 39.3 Classification 39.3.1 Anencephaly 39.3.2 Encephalocoele 39.3.3 Spina Bifida Occulta 39.3.4 Meningocoele 39.3.5 Myelomeningocoele 39.4 Aetiology 39.4.1 Incidence 39.5 Diagnosis 39.5.1 Antenatal 39.6 Clinical Features 39.6.1 Myelomeningocoele 39.6.2 Meningocoele 39.6.3 Spina Bifida Occulta 39.6.4 Encephalocoele 39.7 Management 39.7.1 Myelomeningocoele 39.7.2 Operative Approach 39.7.3 Meningocoele 39.7.4 Encephalocoele 39.7.5 Hydrocephalus 39.7.6 Clinical Features 39.8 Long-Term Management 39.9 Conclusion Selected References 40: Hydrocephalus 40.1 Introduction 40.2 Historical Overview 40.3 Incidence 40.4 Aetiopathogenesis 40.5 Pathophysiology 40.6 Pathology 40.7 Diagnosis 40.7.1 Clinical Presentation 40.7.2 Plain Radiography 40.7.3 Ultrasonography 40.7.4 Computed Tomography (CT) 40.7.5 Magnetic Resonance Imaging (MRI) 40.8 Differential Diagnosis 40.9 Management 40.9.1 Implantable CSF Shunts 40.9.2 Insertion of VP Shunt: The Technique 40.9.3 Complications of CSF Shunts 40.9.4 Ventriculoperitoneal Shunts and Abdominal Surgery 40.9.5 Endoscopic Third Ventriculostomy (ETV) 40.9.6 ETV: The Technique 40.9.7 ETV with Choroid Plexus Coagulation 40.9.8 Indications for ETV 40.9.9 Complications of ETV 40.9.10 Ventricular Access Devices and Ventriculosubgaleal Shunt 40.9.11 Common Clinical Presentation 40.9.12 Follow-Up of the Patient with Treated Hydrocephalus 40.9.13 Outcome of Treated Paediatric Hydrocephalus 40.10 Conclusion Further Reading References 41: Dermal Sinus Tract and Tethered Cord Syndrome 41.1 Introduction 41.2 Historical Overview 41.3 Incidence 41.4 Aetiopathogenesis 41.5 Pathophysiology and Pathology 41.6 Pathology 41.7 Diagnosis 41.7.1 Clinical Presentation 41.7.2 Investigations 41.8 Differential Diagnosis 41.9 Management 41.10 Surgical Technique 41.11 Conclusion Further Reading References Part VI: Anterior Abdominal Wall Defects 42: Omphalomesenteric Duct Remnants 42.1 Introduction 42.2 Variant Pathology of Omphalomesenteric Duct Remnants 42.3 Meckel’s Diverticulum 42.4 Clinical Presentation 42.5 Investigations and Diagnosis 42.6 Differential Diagnosis 42.7 Management 42.8 Morbidity 42.9 Umbilico-Ileal Fistula (Fig. 42.10) 42.10 Umbilical Sinus 42.11 Umbilical Cyst (Omphalomesenteric Cyst or Vitelline Cyst) 42.12 Persistent Fibrous Cord 42.13 Umbilical Polyp 42.14 Conclusion References 43: Omphalocele and Gastroschisis 43.1 Introduction 43.2 Differential Diagnosis: Types of Anterior Abdominal Wall Defects 43.3 Gastroschisis 43.3.1 Etiology and Incidence 43.3.2 Associated Anomalies 43.3.3 Prenatal Diagnosis 43.3.4 Perinatal Care 43.3.5 Operative Management 43.3.6 Complications 43.4 Omphalocele 43.4.1 Etiology and Incidence 43.4.2 Associated Anomalies 43.4.3 Prenatal Diagnosis 43.4.4 Perinatal Care 43.4.5 Operative Management 43.4.6 Complications 43.5 Conclusion Further Reading 44: Conjoined Twins 44.1 Introduction 44.2 Etiopathogenesis and Incidence 44.3 Classification 44.4 Ethical Issues 44.5 Prenatal Management 44.6 Imaging 44.7 Postnatal Management: Technical Issues of Separation 44.8 Results 44.9 Conclusion References Part VII: Tumors 45: Vascular Anomalies 45.1 Introduction 45.2 Historical Overview 45.3 Hemangiomas and Other Vascular Tumors 45.3.1 Incidence 45.3.2 Etiopathogenesis 45.3.3 Diagnosis 45.3.4 Clinical Course 45.3.5 Associated Malformative Anomalies 45.3.6 Differential Diagnosis 45.3.7 Management 45.3.8 Endangering Complications 45.3.9 Pharmacologic Therapy 45.3.10 Interventional Therapy 45.4 Vascular Malformations 45.4.1 Capillary Malformation 45.4.2 Telangiectasias 45.4.3 Lymphatic Malformation 45.4.4 Venous Malformation 45.4.5 Arteriovenous Malformations 45.5 Combined (Eponymous) Vascular Malformations 45.5.1 Slow-Flow Anomalies 45.5.2 Fast-Flow Anomalies 45.6 Conclusions References 46: Congenital Nevi 46.1 Introduction 46.2 Congenital Nevi 46.2.1 Congenital Melanocytic Nevi (CMN) 46.2.2 Small Congenital Melanocytic Nevi 46.2.3 Large Congenital Melanocytic Nevi 46.2.4 Other Congenital Nevi 46.2.4.1 Café Au Lait Macules 46.2.4.2 Nevus Spilus 46.2.4.3 Blue Nevus 46.2.4.4 Spitz Nevi 46.2.4.5 Mongolian Spots 46.2.4.6 Nevus of Ota/Nevus of Ito 46.2.4.7 Sebaceous Nevi 46.3 Treatment of Congenital Nevi 46.4 Dermabrasion, Curettage, and Laser Treatment 46.5 Methods of Excision of Small and Intermediate Nevi 46.5.1 Elliptical Excision 46.5.2 Wedge Excision 46.5.3 Circular Excision 46.5.4 Serial Excision 46.6 Overview of Current Surgical Treatment of Large and Giant Pigmented Nevi 46.6.1 Scalp 46.6.2 Face 46.6.3 Neck 46.6.4 Trunk 46.6.5 Extremities 46.7 Satellite Nevi 46.8 Conclusions Further Reading 47: Lymphatic Malformations 47.1 Introduction 47.2 Historical Overview and Nomenclature 47.3 Etiopathogenesis and Pathophysiology 47.4 Prenatal Diagnosis 47.5 Diagnosis 47.5.1 Clinical Features 47.5.2 Microcystic Lymphatic Malformation 47.5.3 Macrocystic Lymphatic Malformation 47.5.4 Combined (Microcystic and Macrocystic) Lymphatic Malformation 47.5.5 Primary Lymphedema 47.5.6 Gorham-Stout Disease 47.5.7 Generalized Lymphatic Anomaly 47.5.8 Lymphatic Malformation-Associated Overgrowth Syndromes 47.5.9 CLOVES Syndrome 47.5.10 Klippel-Trenaunay Syndrome and Parkes Weber Syndrome 47.6 Imaging and Histopathology 47.7 Differential Diagnosis 47.8 Management 47.8.1 Sclerotherapy 47.8.2 Principles of Surgical Management 47.8.3 Microcystic Lymphatic Malformation 47.8.4 Macrocystic and Combined (Microcystic and Macrocystic) Lymphatic Malformation 47.8.5 Primary Lymphedema 47.8.6 Gorham-Stout Disease/Generalized Lymphatic Anomaly 47.8.7 Lymphatic Malformation-Associated Overgrowth Syndromes 47.8.7.1 Cloves 47.8.8 Klippel-Trenaunay Syndrome 47.9 Conclusion References 48: Sacrococcygeal Teratoma 48.1 Introduction 48.2 Embryology and Pathology 48.3 Altman’s Classification 48.4 Diagnosis 48.4.1 Antenatal Diagnosis 48.4.2 Neonatal Diagnosis 48.4.3 Investigations 48.5 Differential Diagnosis 48.6 Management 48.6.1 Preoperative Management 48.6.2 Surgery 48.6.3 Postoperative Management 48.7 Long-Term Follow-Up 48.8 Prognosis 48.9 Conclusions References 49: Neuroblastoma 49.1 Introduction 49.2 Historical Overview 49.3 Incidence 49.4 Aetiopathogenesis 49.5 Pathophysiology 49.6 Pathology 49.7 Sites of Disease 49.8 Markers of Disease Behaviour 49.8.1 Biochemical Markers 49.9 Molecular Markers 49.10 Staging 49.11 Presentation 49.11.1 Screening 49.11.2 Clinical Presentation 49.12 Diagnosis 49.13 Differential Diagnosis 49.14 Management 49.15 Chemotherapy 49.16 Biological Therapy 49.17 Surgery 49.18 Radiotherapy 49.19 Outcome 49.20 Conclusion References 50: Soft Tissue Sarcomas 50.1 Introduction 50.2 Incidence 50.3 Pathology 50.3.1 Histology and Immunohistology 50.3.2 Molecular Cytogenetics 50.4 Diagnosis 50.4.1 Imaging 50.4.2 Sampling for Biopsy 50.5 Differential Diagnosis 50.5.1 Clinical Evaluation, Symptoms, and Signs 50.5.2 RMS of Head and Neck Region 50.5.3 RMS of the Trunk and Extremities 50.5.4 RMS of the Genitourinary System 50.6 Management 50.6.1 Staging 50.6.2 Prognostic Factors, Risk Categorization, and Outcome of Management of RMS 50.6.3 Treatment of RMS 50.6.3.1 Principles of Chemotherapy 50.6.3.2 Principles of Radiation Therapy 50.6.3.3 Principles of Surgery for RMS 50.7 Non-rhabdomyosarcoma Soft Tissue Sarcoma (NRSTS) 50.7.1 Histology and Grading of NRSTS 50.7.2 Prognostic Factors for NRSTS 50.7.3 Clinical Evaluation, Diagnosis, and Staging of NRSTS 50.7.4 Management of NRSTS 50.8 Conclusions References 51: Lymphomas 51.1 Introduction 51.2 Historical Overview 51.3 Incidence 51.4 Etiopathogenesis 51.5 Non-Hodgkin Lymphoma (NHL) 51.5.1 Diagnosis 51.5.2 Diagnostic Work-Up and Staging 51.5.3 Pathophysiology 51.5.4 Pathology 51.5.5 Treatment and Results 51.6 Hodgkin Lymphoma (HL) 51.6.1 Diagnosis and Differential Diagnosis 51.6.2 Diagnostic Work-Up and Staging 51.6.3 Pathophysiology 51.6.4 Pathology 51.6.5 Treatment and Results 51.7 Management 51.8 Conclusions and Future Directions References 52: Wilms’ Tumor 52.1 Introduction 52.2 Historical Overview 52.3 Etiopathogenesis 52.4 Pathology 52.5 Diagnosis 52.6 Differential Diagnosis 52.7 Management 52.7.1 Staging 52.7.1.1 SIOP Staging 52.7.1.2 COG Staging 52.7.2 Grading 52.7.3 Radiotherapy 52.7.4 Pharmacotherapy 52.7.5 Surgical Therapy 52.7.5.1 General Aspects 52.7.5.2 Bilateral WT 52.7.5.3 Nephron-Sparing Surgery (NSS) 52.7.6 Nephroblastomatosis 52.7.7 Outcome 52.8 Conclusion References 53: Ovarian Tumors 53.1 Introduction 53.2 Incidence and Epidemiology 53.3 Pathology 53.4 Diagnosis and Evaluation 53.4.1 Clinical Presentation 53.4.2 Laboratory Tests 53.4.3 Radiologic Studies 53.4.3.1 Ultrasonography 53.4.3.2 Computed Tomography 53.4.3.3 Magnetic Resonance Imaging 53.5 Treatment of Ovarian Tumors 53.5.1 Benign Tumors 53.5.1.1 Ovarian Cysts 53.5.1.2 Mature Cystic Teratomas 53.5.2 Malignant Tumors 53.5.2.1 Malignant Germ Cell Tumors 53.5.2.2 Sex Cord-Stromal Tumors 53.5.2.3 Epithelial Tumors 53.5.2.4 Borderline Epithelial Tumors 53.6 Conclusions References 54: Testicular Tumors 54.1 Introduction 54.2 Historical Overview, Incidence and Etiopathogenesis 54.3 Pathophysiology & Pathology 54.4 Diagnosis 54.5 Differential Diagnosis 54.6 Management: Surgical Approach and Technique 54.7 Radical Orchiectomy 54.8 TSS 54.9 Retroperitoneal Lymph Node Dissection (RPLND) 54.10 Complications 54.11 Special Situations 54.11.1 Bilateral Masses 54.11.2 Solitary Testis 54.11.3 Small Testicular Masses 54.11.4 Microlithiasis 54.11.5 GCNIS Remaining after TSS 54.11.6 GCNIS/Gonadoblastoma/GCT in Patient with a Difference of Sex Development 54.11.7 Scrotal Violation 54.11.8 Stromal Tumors 54.12 Outcome and Follow-up 54.13 Conclusion References Part VIII: Gastrointestinal 55: Esophageal Atresia and Tracheoesophageal Fistula 55.1 Introduction 55.2 History 55.3 Incidence 55.4 Etiopathogenesis 55.5 Pathophysiology 55.6 Pathology 55.7 Diagnosis 55.7.1 Clinical Features 55.7.2 Radiological Diagnosis 55.7.3 Differential Diagnosis 55.8 Management 55.8.1 Preoperative Management 55.8.2 Operative Management 55.8.2.1 EA with Distal Tracheoesophageal Fistula (85%) 55.8.2.2 EA with Proximal and Distal Tracheoesophageal Fistula (1.5%) 55.8.2.3 EA with a Proximal Tracheoesophageal Fistula Only (1%) 55.8.2.4 H-Type Fistula (4%) 55.8.2.5 Isolated EA: The Long-Gap Problem (8%) 55.9 Complications 55.9.1 Early Complications 55.9.2 Late Complications 55.10 Long-Term Follow-Up 55.11 Conclusion References 56: Achalasia 56.1 Introduction 56.2 Historical Overview 56.3 Incidence 56.4 Etiopathogenesis 56.4.1 Etiology 56.4.2 Pathophysiology 56.5 Diagnosis 56.5.1 Clinical Features 56.5.2 Radiology 56.5.3 Upper Endoscopy 56.5.4 Esophageal Manometry 56.5.5 High-Resolution Manometry and Esophageal Pressure Topography 56.5.5.1 Classification of Achalasia 56.6 Differential Diagnosis 56.7 Management 56.7.1 Pharmacotherapy 56.7.2 Botulinum Injection 56.7.3 Pneumatic Dilatation 56.7.4 Surgical Myotomy 56.7.5 Peroral Endoscopic Myotomy (POEM) 56.8 Complications 56.8.1 Residual Dysphagia 56.8.1.1 Inadequate Myotomy 56.8.1.2 Surgical Myotomy 56.8.1.3 Poem 56.8.2 Suboptimal Myotomy Site 56.8.2.1 Surgical Myotomy 56.8.2.2 Poem 56.8.3 Postoperative Stricture 56.8.4 Excessive Fundoplication or Excessively Tight Wrap Incorporation with Surgical Myotomy 56.8.5 Postoperative Gastroesophageal Reflux 56.8.6 Intraoperative Esophageal Perforation 56.8.6.1 Surgical Myotomy 56.8.6.2 Poem 56.8.7 Postoperative Leak 56.8.8 Long-Term Outcome 56.8.8.1 Management of End-Stage Achalasia 56.8.8.2 Screening for Carcinoma 56.9 Conclusion References 57: Esophageal Perforations and Caustic Injuries in Children 57.1 Introduction 57.2 Esophageal Physiology and Anatomy 57.3 Etiology of Esophageal Perforation 57.4 Esophageal Perforation in the Newborn 57.5 Boerhaave’s Syndrome 57.6 Button Battery Ingestion 57.7 Diagnosis of Esophageal Perforation 57.8 Differential Diagnosis 57.9 Management of Esophageal Perforation 57.10 Caustic Injuries of the Esophagus 57.11 Epidemiology 57.12 Pathology 57.13 Diagnosis 57.14 Management 57.15 Prevention of Caustic Injuries 57.16 Conclusion References 58: Gastroesophageal Reflux Disease 58.1 Introduction 58.2 History 58.3 Incidence 58.4 Etiopathogenesis 58.5 Pathophysiology 58.6 Pathology 58.7 Diagnosis 58.7.1 Clinical Symptoms 58.7.2 Diagnostic Investigations 58.7.2.1 Upper Gastrointestinal X-Ray Passage 58.7.2.2 24-Hour pH Monitoring 58.7.2.3 pH-Impedance Monitoring (pH/MII) 58.7.2.4 Manometry 58.7.2.5 Endoscopy and Histology 58.7.2.6 Scintigraphy 58.7.3 Differential Diagnosis 58.7.3.1 Reflux and Asthma 58.7.3.2 Reflux and Apnea Syndrome 58.7.3.3 Eosinophilic Esophagitis 58.7.3.4 Barrett’s Esophagus 58.7.3.5 Hiatal Hernia 58.8 Management 58.8.1 Conservative Treatment 58.8.1.1 Babies and Small Infants 58.8.1.2 Conservative Therapy in Older Children and Adolescents 58.8.1.3 Pharmacologic Treatment Alginates Proton Pump Inhibitors (PPI) Histamin-2 Receptor Antagonists (H2RAs) Prokinetics 58.8.2 Surgical Treatment 58.8.2.1 Fundoplication 58.8.2.2 Other Surgical Techniques 58.8.2.3 Complications After Fundoplication 58.9 Conclusion References 59: Esophageal Replacement 59.1 Introduction 59.2 Historical Overview 59.3 Indications for Esophageal Replacement 59.3.1 Age for Esophageal Replacement 59.4 Routes for Esophageal Replacement 59.5 Mobilization of the Cervical Esophagostomy 59.5.1 Operative Procedure 59.6 Colon Interposition 59.6.1 Advantages of Colonic Interposition 59.6.2 Operative Procedure 59.6.3 Postoperative Care 59.6.4 Complications of Colon Interposition 59.6.5 Treatment of Complications 59.6.6 Long-Term Outcome and Follow-Up 59.7 Gastric Transposition 59.7.1 Advantages of Gastric Transposition 59.7.2 Operative Procedure 59.7.3 Complications 59.7.4 Long-Term Outcome and Follow-Up 59.8 Gastric Tube 59.8.1 Operative Procedure 59.8.2 Complications of Gastric Tube 59.8.3 Long-Term Outcome and Follow-Up 59.9 Jejunal Graft 59.9.1 Operative Procedure 59.9.2 Complications 59.9.3 Long-Term Outcome and Follow-Up 59.10 Recent Advances 59.11 Conclusion References 60: Infantile Hypertrophic Pyloric Stenosis 60.1 Introduction Including Definition and Incidence 60.2 Etiology 60.3 Pathology 60.4 Diagnosis 60.4.1 Clinical Features 60.4.2 Physical Examinations 60.4.3 Diagnostic Imaging 60.5 Differential Diagnosis 60.6 Management 60.6.1 Preoperative Management 60.6.2 Operation 60.6.3 Postoperative Feeding 60.6.4 Nonoperative Treatment 60.7 Complications 60.8 Conclusion References 61: Gastrostomy and Jejunostomy 61.1 Introduction 61.2 Gastrostomy 61.2.1 Indications 61.2.1.1 Esophageal Abnormalities 61.2.1.2 Duodenal Obstruction 61.2.1.3 Short Bowel Syndrome 61.2.1.4 Other Pathologies 61.2.2 Choice of Technique 61.2.2.1 Open Technique 61.2.2.2 Percutaneous Endoscopic Gastrostomy (PEG) 61.2.2.3 Minimally Invasive Gastrostomy Techniques Laparoscopically Assisted Gastrostomies Laparoscopic-Assisted Percutaneous Endoscopic Approach 61.2.3 Access Devices 61.2.4 Complications and Management 61.2.4.1 Complications Related to Operative Technique 61.2.4.2 Complications Related to Stoma Care 61.2.4.3 Complications Related to Catheters 61.2.5 Gastrostomy Closure and Persistent Gastrocutaneous Fistula 61.3 Jejunostomies 61.3.1 Indications 61.3.2 Choice of Technique 61.3.3 Devices 61.3.4 Postoperative Care and Complications 61.4 Conclusion References 62: Duodenal Obstruction 62.1 Introduction 62.2 Etiopathogenesis and Pathophysiology 62.3 Associated Malformations 62.4 Prenatal Diagnosis 62.5 Clinical Presentation and Diagnosis 62.6 Differential Diagnosis 62.6.1 Malrotation 62.6.2 Pyloric Atresia and Prepyloric Antral Diaphragm 62.6.3 Gastric Volvulus 62.6.4 Pyloric Stenosis 62.6.5 Jejunoileal Atresia and Stenosis 62.6.6 Preduodenal Portal Vein 62.7 Preoperative Management 62.8 Operation 62.9 Incision 62.10 Exploration and Identification of Pathology 62.11 “Diamond-Shaped” Duodenoduodenostomy 62.12 Side-to-Side Duodenoduodenostomy 62.13 Operative Technique for Duodenal Web 62.14 Laparoscopic Management of DO 62.15 Postoperative Care 62.16 Management of Persistent Megaduodenum by Duodenoplasty 62.17 Outcome and Long-Term Results 62.18 Conclusion References 63: Intestinal Malrotation 63.1 Introduction 63.2 Epidemiology 63.3 Embryology 63.4 Pathogenesis 63.5 Pathology 63.5.1 Incomplete Rotation 63.5.2 Non-rotation 63.5.3 Other Rarer Abnormalities of Rotation/Fixation 63.6 Associated Anomalies 63.7 Clinical Features 63.8 Radiological Diagnosis 63.9 Differential Diagnosis 63.10 Treatment 63.10.1 Ladd Procedure 63.11 Complications 63.12 Asymptomatic Intestinal Malrotation in Special Circumstances: Heterotaxy Syndrome, Congenital Diaphragmatic Hernia, and Anterior Abdominal Wall Defects 63.13 Conclusions and Controversies References 64: Jejuno-Ileal Atresia 64.1 Introduction 64.2 Historical Overview 64.3 Incidence 64.4 Etiopathogenesis 64.5 Pathophysiology 64.6 Pathology 64.7 Management 64.7.1 Clinical Presentation and Diagnosis 64.7.2 Differential Diagnosis 64.7.3 Surgical Management: Making the Infant ‘Safe for Surgery’ 64.7.4 Anaesthesia 64.7.5 Surgical Strategy 64.7.5.1 Standard Surgical Procedure 64.7.5.2 Special Considerations 64.8 The Short Bowel Syndrome 64.9 Postoperative Care 64.10 Conclusion References 65: Meconium Ileus 65.1 Introduction 65.2 Historical Overview 65.3 Incidence 65.4 Etiopathogenesis 65.5 Pathophysiology 65.6 Pathology 65.7 Diagnosis 65.8 Differential Diagnosis 65.9 Management 65.9.1 Operative Management 65.10 Conclusion References 66: Duplications of the Alimentary Tract 66.1 Introduction 66.2 Incidence 66.3 Etiopathogenesis 66.3.1 Partial Twinning 66.3.2 Split Notochord 66.3.3 Embryonic Diverticula and Recanalization Defects 66.4 Pathology 66.5 Diagnosis and Management 66.6 Differential Diagnosis 66.7 Management 66.7.1 Oesophageal Duplication 66.7.2 Thoracoabdominal Duplication 66.7.3 Gastric Duplication 66.7.4 Pyloric Duplications 66.7.5 Duodenal Duplications 66.7.6 Duplications of the Small Intestine 66.7.7 Colonic Duplications 66.7.8 Rectal Duplications 66.8 Conclusion References 67: Necrotizing Enterocolitis 67.1 Introduction 67.2 Historical Overview 67.3 Etiopathogenesis and Pathophysiology 67.3.1 Immature Intestinal Barrier 67.3.2 The Role of Bacterial Pathogens and Other Microbes in NEC 67.3.3 The Role of Enteral Feeding 67.3.4 Inflammatory Mediators and NEC 67.3.5 Red Blood Cell Transfusion and Anaemia 67.3.5.1 Maternal Factors 67.4 Pathology 67.5 Diagnosis 67.5.1 Clinical Features 67.5.2 Laboratory Findings 67.5.3 Radiological Diagnosis 67.5.3.1 Abdominal Series X-Rays 67.5.3.2 Contrast Studies 67.5.3.3 Ultrasound 67.6 Differential Diagnosis 67.7 Management 67.7.1 Medical Management 67.7.2 Probiotics 67.7.3 Surgical Management 67.8 Complications 67.8.1 Strictures 67.8.2 Short Bowel Syndrome 67.8.3 Neurodevelopmental Delay 67.9 Conclusion Further Reading 68: Constipation 68.1 Introduction, Definition and Incidence 68.2 Historical Overview 68.3 Aetiology of Constipation 68.4 Acute Constipation 68.5 Chronic Constipation 68.6 Pathology of Slow Transit Constipation 68.7 Pathophysiology of Slow Transit Constipation 68.8 Differential Diagnosis 68.9 Diagnosis: A Framework 68.9.1 Clinical 68.9.2 Abdominal X-Ray 68.9.3 Transit Studies 68.9.4 Rectal Biopsy 68.9.5 Laparoscopic Colonic Biopsies 68.9.6 Colonic Manometry 68.10 Management 68.10.1 Medical 68.10.2 Surgical 68.11 Complications 68.11.1 Disease Related 68.11.2 Laxatives 68.11.3 Surgical 68.11.4 Quality of Life 68.12 Conclusion References 69: Hirschsprung’s Disease 69.1 Introduction 69.2 Historical Overview 69.3 Incidence 69.3.1 Gender 69.3.2 Birth Characteristics 69.3.3 Race 69.4 Etiopathogenesis 69.5 Pathophysiology 69.6 Pathology 69.7 Diagnosis 69.7.1 Clinical Presentation 69.7.2 Imaging 69.7.3 Anorectal Manometry 69.7.4 Rectal Biopsy 69.8 Differential Diagnosis 69.9 Management 69.9.1 Role of Colostomy 69.9.2 Transanal One-Stage Endorectal Pull-Through Operation 69.9.3 Operative Technique 69.9.4 Laparoscopic-Assisted Pull-Through 69.9.5 Postoperative Care 69.9.6 Complications 69.9.6.1 Anastomotic Leak 69.9.6.2 Retraction of Pull-Through 69.9.6.3 Perianal Excoriation 69.9.6.4 Enterocolitis 69.9.6.5 Constipation 69.9.6.6 Soiling 69.10 Conclusions References 70: Variant Hirschsprung’s Disease 70.1 Introduction 70.2 Intestinal Neuronal Dysplasia 70.2.1 Epidemiology 70.2.2 Pathogenesis 70.2.3 Clinical Presentation 70.2.4 Diagnosis 70.2.5 Management 70.2.6 Outcome 70.3 Intestinal Ganglioneuromatosis 70.3.1 Epidemiology 70.3.2 Pathogenesis 70.3.3 Clinical Presentation 70.3.4 Diagnosis 70.3.5 Management 70.3.6 Outcome 70.4 Isolated Hypoganglionosis 70.4.1 Epidemiology 70.4.2 Pathogenesis 70.4.3 Clinical Presentation 70.4.4 Diagnosis 70.4.5 Management 70.4.6 Outcome 70.5 Immature Ganglia 70.5.1 Epidemiology 70.5.2 Pathogenesis 70.5.3 Clinical Presentation 70.5.4 Diagnosis 70.5.5 Management 70.5.6 Outcome 70.6 Absence of the Argyrophil Plexus 70.6.1 Epidemiology 70.6.2 Pathogenesis 70.6.3 Clinical Presentation 70.6.4 Diagnosis 70.6.5 Management 70.6.6 Outcome 70.7 Internal Anal Sphincter Achalasia 70.7.1 Epidemiology 70.7.2 Pathogenesis 70.7.3 Clinical Presentation 70.7.4 Diagnosis 70.7.5 Management 70.7.6 Outcome 70.8 Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome 70.8.1 Epidemiology 70.8.2 Pathogenesis 70.8.3 Clinical Presentation 70.8.4 Diagnosis 70.8.5 Management 70.8.6 Outcome 70.9 Conclusion References 71: Anorectal Anomalies 71.1 Introduction 71.2 Historical Overview 71.3 Incidence 71.4 Etiopathogenesis 71.5 Pathophysiology and Pathology 71.6 Classification 71.7 Colostomy 71.8 Primary Repair without a Colostomy 71.9 High Pressure Distal Colostogram (Males) 71.10 High Pressure Distal Colostogram (Females) 71.11 Repair of the Malformation 71.12 Cloaca Repair 71.13 Complications 71.14 Prolapse 71.15 Conclusion References 72: Appendicitis 72.1 Introduction 72.2 Historical Overview 72.3 Incidence 72.4 Etiopathogenesis 72.5 Pathology 72.6 Diagnosis 72.7 Laboratory Tests 72.8 Imaging Techniques 72.9 Scoring Systems 72.10 Differential Diagnosis 72.11 Management 72.11.1 Surgical Management 72.11.2 Nonoperative Treatment 72.11.3 Appendix Mass 72.11.4 Impact of Surgical Delay 72.12 Conclusions References 73: Intussusception 73.1 Introduction 73.2 Historical Overview 73.3 Incidence 73.4 Etiopathogenesis 73.5 Pathophysiology 73.6 Pathology 73.7 Diagnosis 73.8 Differential Diagnosis 73.9 Management 73.9.1 Resuscitation 73.9.2 Nonsurgical Treatment 73.9.3 Surgical Treatment 73.9.4 The Role of Laparoscopy 73.9.5 Postoperative Care 73.9.6 Recurrent Intussusception 73.9.7 Chronic Intussusception 73.10 Conclusion References 74: Hernias 74.1 Inguinal Hernia 74.1.1 Introduction 74.1.2 Historical Overview 74.1.3 Incidence 74.1.4 Etiopathogenesis 74.1.5 Diagnosis 74.1.5.1 Clinical Features 74.1.5.2 Incarcerated Inguinal Hernia 74.1.6 Differential Diagnosis 74.1.7 Management 74.1.7.1 Anesthesia 74.1.7.2 Operation 74.1.7.3 Contralateral Exploration 74.1.7.4 Laparoscopic Repair of Inguinal Hernia 74.1.8 Complications 74.2 Congenital Hydrocele 74.2.1 Management 74.3 Femoral Hernia 74.3.1 Management 74.4 Umbilical Hernia 74.4.1 Management 74.5 Epigastric Hernia 74.5.1 Management 74.6 Conclusions References 75: Short Bowel Syndrome 75.1 Introduction 75.2 History 75.3 Incidence and Etiology 75.4 Physiology 75.5 Pathophysiology 75.6 Citrulline 75.7 Intestinal Adaptation 75.8 Nutritional Therapy 75.9 Pharmacologic Supplements 75.9.1 Hormonal Treatments 75.10 Surgical Therapy 75.11 Complications 75.11.1 Central Venous Line Infection 75.11.2 Bacterial Overgrowth 75.11.3 D-Lactic Acidosis 75.11.4 Intestinal Failure-Associated Cholestasis and Liver Failure (IFCL) 75.12 Conclusion and Prognosis References 76: Inflammatory Bowel Disease 76.1 Ulcerative Colitis 76.1.1 Introduction 76.1.2 Aetiology 76.1.3 Pathology 76.1.4 Extraintestinal Manifestations 76.1.5 Diagnosis 76.1.5.1 TÄSTÄ Laboratory Tests Endoscopy Paediatric Ulcerative Colitis Activity Index Differential Diagnostics 76.1.6 Medical Management 76.1.7 Surgical Management 76.1.7.1 Principles and Indications 76.1.7.2 Preoperative Assessment 76.1.8 Operative Approach 76.1.8.1 Selection of Surgical Approach 76.1.8.2 Restorative Proctocolectomy 76.1.9 Postoperative Management 76.1.10 Surgical Complications 76.1.11 Outcomes of Ileoanal Pull-Through 76.1.11.1 Stooling Frequency and Faecal Continence 76.1.11.2 Pouchitis 76.1.11.3 Fertility and Sexual Function 76.1.11.4 Quality of Life 76.1.12 Conclusion and Future Directions 76.2 Crohn’s Disease 76.2.1 Introduction 76.2.2 Aetiology 76.2.3 Pathology 76.2.4 Diagnosis 76.2.4.1 Clinical Features 76.2.4.2 Laboratory Investigations 76.2.4.3 Endoscopy 76.2.4.4 Imaging 76.2.4.5 Differential Diagnosis 76.2.5 Extraintestinal Manifestations 76.2.6 Medical Treatment 76.2.7 Perianal Disease 76.2.8 Surgical Treatment 76.2.8.1 Principles and Indications 76.2.8.2 Timing 76.2.8.3 Preoperative Assessment 76.2.8.4 Operative Approach and Technique Duodenum Small Intestine Large Intestine 76.2.8.5 Fistulising Non-perianal Disease 76.2.8.6 Postoperative Management 76.2.9 Complications 76.2.10 Outcomes of Surgery for Crohn’s Disease 76.2.11 Conclusion and Future Directions References 77: Paediatric Small Bowel Transplantation 77.1 Introduction 77.2 Indications 77.3 Pre-Transplant Assessment 77.4 Types of Transplant Procedures 77.4.1 Living Related Intestinal Transplantation 77.5 Techniques of Transplantation 77.5.1 Immunosuppression in Small Bowel Transplantation 77.6 Medical Complications 77.6.1 Graft Rejection 77.6.2 Infections 77.6.3 Post-Transplant Lymphoproliferative Disorder (PTLD) 77.6.4 Graft Versus Host Disease (GVHD) 77.6.5 Antibody-Mediated Rejection (ABMR) 77.7 Surgical Complications 77.7.1 Intestinal Perforation 77.7.2 Abdominal Compartment Syndrome (ACS) 77.7.3 Pancreatic Complications 77.7.4 Vascular Complications 77.7.5 Other Complications 77.8 Nutritional Outcome and Quality of Life 77.9 Outcome 77.10 Conclusion and Future Directions 77.11 Key Points References 78: Long-Term Outcomes in Pediatric Surgery 78.1 Introduction 78.2 Justification of Long-Term Follow-Up in Pediatric Surgery 78.3 How Do We Study Long-Term Outcomes? 78.4 Long-Term Outcomes of Specific Pediatric Surgical Conditions 78.4.1 Long-Term Outcome in Children with Congenital Diaphragmatic Hernia 78.4.1.1 Chronic Respiratory Tract Disease 78.4.1.2 Gastroesophageal Reflux Disease 78.4.1.3 Failure to Thrive and Grow 78.4.1.4 Skeletal Abnormalities 78.4.1.5 Neurological Deficits 78.4.1.6 Health-Related Quality of Life 78.4.2 Long-Term Outcome in Children with Esophageal Atresia 78.4.2.1 Esophageal Morbidity 78.4.2.2 Respiratory Morbidity 78.4.2.3 Musculoskeletal Morbidity 78.4.2.4 Other Issues of Long-Term Morbidity 78.4.2.5 Quality of Life 78.4.3 Intestinal Failure 78.4.3.1 Definition and Causes of Intestinal Failure 78.4.3.2 Incidence and Mortality 78.4.3.3 Predictors of Intestinal Autonomy 78.4.3.4 Growth and Development 78.4.3.5 Intestinal Failure–Associated Liver Disease (IFALD) 78.4.3.6 Quality of Life 78.4.4 Hirschsprung’s Disease 78.4.4.1 Bowel Function: Classic Rectosigmoid Aganglionosis 78.4.4.2 Evolution of Bowel Function with Advancing Age 78.4.4.3 Urinary and Sexual Function 78.4.4.4 Total Colonic and Panintestinal Aganglionosis 78.4.4.5 Syndromic Hirschsprung’s Disease 78.4.4.6 Hirschsprung’s Disease and Cancer 78.4.4.7 Quality of Life 78.4.5 Anorectal Malformations 78.4.5.1 Mild Anomalies with Perineal Fistula: Long-Term Bowel Function 78.4.5.2 High Anomalies: Long-Term Bowel Function 78.4.5.3 Urinary Tract Problems 78.4.5.4 Genital Anomalies, Fertility and Sexual Problems 78.4.5.5 Vertebral Anomalies and Myelodysplasias 78.4.5.6 Quality of Life in Adult Patients with Anorectal Malformations 78.5 Conclusion and Future Directions References Part IX: Hepatobiliary 79: Biliary Atresia 79.1 Introduction 79.2 Etiology 79.2.1 Congenital Embryopathy 79.2.2 Viral Exposure 79.3 Pathology 79.4 Clinical Features 79.4.1 Diagnosis 79.4.2 Differential Diagnosis 79.5 Management 79.5.1 Kasai Portoenterostomy 79.5.2 Adjuvant Therapy 79.5.3 Prognostic Factors 79.6 Complications 79.7 Cholangitis 79.8 Portal Hypertension 79.8.1 Miscellaneous 79.9 Outcome 79.10 Conclusion Further Reading 80: Choledochal Cyst 80.1 Introduction 80.2 Etiopathogenesis 80.3 Pathophysiology 80.3.1 Cystic/Fusiform-Type Choledochal Cysts 80.3.2 Forme Fruste-Type Choledochal Cysts 80.4 Diagnosis 80.4.1 Prenatal Diagnosis 80.4.2 Clinical Features 80.4.3 Imaging Studies 80.5 Differential Diagnosis 80.6 Open Surgical Management 80.6.1 Cyst Excision 80.6.2 Intraoperative Endoscopy 80.6.3 Excision of the Distal Common Bile Duct 80.6.4 Excision of the Common Hepatic Duct 80.6.5 IHBD Dilatation 80.6.6 Roux-En-Y Hepaticojejunostomy 80.6.7 Hepaticojejunostomy Versus Hepaticoduodenostomy 80.7 Minimally Invasive Surgical Management 80.7.1 Laparoscopic Surgery 80.7.2 Robotic Surgery 80.7.3 Hybrid Surgery 80.8 Postoperative Outcome and Complications 80.9 Conclusion References 81: Hepatic Cysts and Abscesses 81.1 Hepatic Cysts 81.1.1 Simple Non-Neoplastic Cyst 81.1.2 Fibrocystic Disease of the Liver 81.1.2.1 Polycystic Disease 81.1.2.2 Congenital Hepatic Fibrosis 81.1.2.3 Biliary Hamartoma 81.1.2.4 Caroli’s Disease 81.1.3 Cystic Neoplasms of the Liver 81.1.4 Parasitic Cysts 81.2 Hepatic Abscesses 81.2.1 Amoebic Liver Abscesses 81.3 Conclusion Further Reading 82: Portal Hypertension 82.1 Introduction 82.2 Pathophysiology and Definition 82.3 Classification and Etiology 82.4 Clinical Features 82.5 Diagnosis and Investigation 82.6 Treatment and Complications 82.6.1 Emergency Management of Variceal Bleeding 82.6.2 Endoscopic Treatment of Esophageal Varices 82.6.2.1 Injection Sclerotherapy 82.6.2.2 Variceal Ligation (Banding) 82.6.3 Primary Prophylaxis of Variceal Bleeding 82.6.4 Surgery for Portal Hypertension 82.6.5 Other Interventions for Portal Hypertension 82.6.6 Liver Transplantation 82.6.7 Surgery for Budd-Chiari Syndrome 82.6.8 Arterioportal Fistula 82.7 Conclusions and Future Directions References 83: Gallbladder Disease 83.1 Introduction 83.2 Etiology 83.2.1 Gallstone Formation 83.3 Hemolytic Disease 83.4 Non-Hemolytic Disease 83.4.1 Acalculous Gallbladder Disease 83.5 Diagnosis 83.5.1 Clinical Features 83.5.2 Radiologic Diagnosis 83.6 Management 83.6.1 Concomitant Splenectomy 83.6.2 Choledocholithiasis 83.6.3 Gallstone Pancreatitis 83.7 Operative Considerations 83.7.1 Laparoscopic Cholecystectomy 83.7.2 Single-Site Laparoscopic Cholecystectomy 83.8 Complications 83.9 Conclusion References 84: Pancreatic Disorders 84.1 Introduction 84.2 Historical Overview 84.3 Embryology 84.4 Structural Pancreatic Anomalies 84.4.1 Annular Pancreas 84.4.1.1 Diagnosis and Differential Diagnosis 84.4.1.2 Management 84.4.2 Pancreas Divisum 84.4.2.1 Diagnosis and Differential Diagnosis 84.4.2.2 Management 84.4.3 Pancreaticobiliary Malunion 84.5 Congenital Hyperinsulinism 84.5.1 Differential Diagnosis 84.5.2 Diagnosis 84.5.3 Management 84.5.4 Medical Treatment 84.5.5 Surgical Treatment 84.5.6 Postoperative Management 84.5.7 Postoperative Outcome 84.6 Pancreatitis 84.6.1 Acute Pancreatitis 84.6.1.1 Diagnosis and Differential Diagnosis 84.6.1.2 Management 84.6.2 Complications of Acute Pancreatitis 84.6.2.1 Pancreatic Pseudocyst 84.6.2.2 Necrotizing Pancreatitis 84.6.2.3 Pancreatic Fistula 84.6.2.4 Pancreatic Hemorrhage 84.6.3 Acute Recurrent Pancreatitis 84.6.4 Chronic Pancreatitis 84.6.4.1 Diagnosis and Differential Diagnosis 84.6.4.2 Management 84.7 Pancreatic Cysts 84.7.1 Diagnosis and Differential Diagnosis 84.7.2 Management 84.8 Pancreatic Tumors 84.8.1 Diagnosis and Differential Diagnosis 84.8.2 Treatment 84.9 Conclusion References 85: Splenic Disorders 85.1 Introduction 85.2 Anatomy and Physiology 85.3 Pathology 85.4 Splenic Trauma 85.4.1 Causes and Symptoms 85.4.2 Diagnosis 85.4.3 Treatment 85.4.4 Nonoperative Treatment 85.4.5 Prognosis 85.5 Hematologic Diseases 85.6 Hodgkin’s Disease 85.7 Iatrogenic (Intraoperative) Splenic Injury 85.8 Other Indications for Splenectomy 85.9 Prophylaxis Against Postsplenectomy Sepsis 85.9.1 Antibiotic Prophylaxis 85.9.2 Immunization 85.9.3 Management of Suspected Infection 85.10 Wandering Spleen 85.11 Surgical Approaches 85.12 Complications and Adverse Effects 85.13 Conclusion References 86: Hepatic Tumors in Childhood 86.1 Introduction 86.2 History 86.3 Surgical Anatomy 86.4 Evaluation of a Child with Hepatic Mass 86.5 Malignant Liver Tumors 86.5.1 Hepatoblastoma 86.5.1.1 Incidence and Etiology 86.5.1.2 Histopathological Subtypes 86.5.1.3 Clinical Findings 86.5.1.4 Imaging 86.5.1.5 Staging 86.5.1.6 Treatment and Prognosis 86.5.2 Hepatocellular Carcinoma (or Hepatoma) 86.5.2.1 Incidence and Epidemiology 86.5.2.2 Clinical Findings 86.5.2.3 Staging 86.5.2.4 Treatment and Outcome 86.5.3 Rhabdomyosarcoma of Extrahepatic Bile Ducts 86.5.4 Primary Hepatic Non-Hodgkin’s Lymphoma 86.5.5 Metastatic Hepatic Tumors 86.5.6 Benign Hepatic Tumors 86.5.6.1 Vascular Tumors 86.5.6.2 Mesenchymal Hamartoma 86.5.6.3 Focal Nodular Hyperplasia 86.5.6.4 Cysts and Cystic Disease 86.6 Conclusions References 87: Pediatric Liver Transplantation 87.1 Introduction 87.2 Historical Overview 87.3 Indications 87.4 Contraindications 87.5 Assessment 87.6 Surgical Technique 87.7 Living Related Donors 87.8 Split Liver Transplantation 87.9 Medical Management 87.9.1 Post-operative Care 87.10 Immunosuppression 87.11 Anti-Infection Agents 87.12 Surgical Complications 87.13 Common Medical Complications 87.14 Long-Term Survival and Quality of Life 87.15 Conclusion References Part X: Genitourinary Disorders 88: Urinary Tract Infection 88.1 Introduction 88.1.1 Complicated vs. Uncomplicated UTI 88.1.2 Unresolved Infection 88.1.3 Bacterial Persistence 88.1.4 Re-infection 88.2 Historical Overview 88.3 Incidence 88.4 Etiopathogenesis 88.5 Risk Factors 88.5.1 Gender 88.5.2 Circumcision Status 88.5.3 Previous Infection 88.5.4 Bladder and Bowel Dysfunction 88.6 Pathophysiology 88.7 Pathology 88.8 Diagnosis 88.8.1 Urinalysis, Microscopy, and Culture 88.8.2 Urine Collection 88.8.3 Renal-Bladder Ultrasound 88.8.4 Further Work-Up 88.9 Differential Diagnosis 88.10 Management 88.10.1 Uncomplicated UTI 88.10.2 Complicated UTI 88.10.3 Catheter-Associated UTI 88.10.4 Antibiotic Prophylaxis 88.11 Conclusion References 89: Imaging of the Paediatric Urogenital Tract 89.1 Introduction 89.2 Imaging Methods 89.2.1 Typical Imaging Findings in Common Paediatric Urological Conditions 89.3 Imaging Algorithms 89.4 Conclusion Further Reading 90: Management of Antenatal Hydronephrosis 90.1 Introduction 90.2 Development of the Kidney and Renal Function 90.3 The Fetus with Antenatal Hydronephrosis 90.4 Guidelines on Antenatal Hydronephrosis 90.5 Management of the Newborn with Antenatal Hydronephrosis 90.5.1 Management in the Nursery 90.5.2 Antibiotic Prophylaxis 90.5.3 Initial Radiologic Evaluation 90.5.3.1 Renal/Bladder Ultrasound 90.5.3.2 Voiding Cystourethrogram 90.5.3.3 What If the Initial Sonogram Is Normal? 90.5.4 Follow-Up Evaluation and Treatment 90.5.4.1 Diuretic Renogram 90.5.4.2 Magnetic Resonance Urography 90.5.4.3 Ancillary Studies 90.6 Congenital Anomalies Causing ANH 90.6.1 UPJ Obstruction or Anomalous UPJ 90.6.2 Multicystic Dysplastic Kidney 90.6.3 Primary Megaureter (Non-refluxing) 90.6.4 Ureterocele and Ectopic Ureter 90.6.5 Posterior Urethral Valves 90.6.6 Vesicoureteral Reflux 90.7 Conclusions References 91: Upper Urinary Tract Obstructions 91.1 Pelviureteric Junction Obstruction 91.1.1 Historical Overview 91.1.2 Incidence 91.1.3 Etiopathogenesis 91.1.4 Pathophysiology 91.1.5 Diagnosis 91.1.5.1 Prenatal Diagnosis 91.1.5.2 Clinical Presentation 91.1.5.3 Differential Diagnosis 91.1.6 Management 91.2 Megaureter, Ureterovesical Junction Obstruction 91.2.1 Historical Overview 91.2.2 Incidence 91.2.3 Etiopathogenesis 91.2.4 Pathophysiology 91.2.5 Diagnosis 91.2.5.1 Prenatal Diagnosis 91.2.5.2 Differential Diagnosis 91.2.6 Management 91.2.7 Postoperative Course 91.3 Conclusions References 92: Ureteric Duplication Anomalies 92.1 Introduction 92.2 Incomplete Duplication 92.3 Complete Duplication 92.4 Investigations 92.4.1 Renal Ultrasound 92.4.2 Voiding Cystourethrogram (VCUG) 92.4.3 Intravenous Pyelogram (IVP) 92.4.4 DMSA (99mTc Dimercpatosuccinic Acid) Scan 92.4.5 MAG3 (Mercaptoacetyltryglycerine) Scan 92.4.6 Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) Scans with or Without Urogram 92.5 Vesicoureteric Reflux (VUR) 92.6 Ureterocoele 92.7 Ectopic Ureters 92.8 Pelviureteric Junction Obstruction (PUJO) 92.9 Conclusion References 93: Vesicoureteral Reflux 93.1 Introduction 93.2 Etiopathogenesis 93.2.1 Mechanism of Renal Scarring 93.3 Diagnosis 93.3.1 Clinical Presentation 93.3.2 Radiological Investigations 93.3.2.1 Ultrasound 93.3.2.2 Voiding Cystourethrography 93.3.2.3 DMSA Scan 93.3.2.4 Diagnostic Workup 93.4 Management 93.4.1 Medical Management 93.4.2 Surgical Treatment 93.4.2.1 Antireflux Procedures 93.4.2.2 Endoscopic Treatment of VUR Endoscopic Injection Technique Postoperative Care Results of Endoscopic Treatment Complications of Endoscopic Treatment 93.4.3 Treatment Strategy 93.4.4 Follow-Up 93.5 Conclusion References 94: Posterior Urethral Valves 94.1 Introduction 94.2 Embryology, Pathogenesis, and Classification 94.3 Pathophysiology 94.3.1 Antenatal 94.3.2 Lower Urinary Tract 94.3.3 Upper Urinary Tract 94.3.4 The “Valve Bladder Syndrome” 94.4 Diagnosis 94.4.1 Prenatal Diagnosis 94.4.2 Neonatal Diagnosis 94.4.3 Delayed Diagnosis of PUV 94.5 Differential Diagnosis 94.6 Imaging 94.6.1 Ultrasound 94.6.2 Voiding Cystourethrogram (VCUG) 94.6.3 Radioisotope Scan 94.6.4 Urodynamics 94.7 Management 94.7.1 Prenatal 94.7.2 Neonatal 94.7.3 PUV in the Premature Newborn 94.7.4 Complications 94.7.5 Urinary Diversions 94.7.6 Circumcision 94.8 Long-Term Outcomes 94.8.1 Vesicoureteral Reflux 94.8.2 Defunctionalized Bladder 94.8.3 Incontinence and Bladder Dysfunction 94.8.4 Bladder Augmentation 94.8.5 End-Stage Kidney Disease 94.9 Renal Transplantation 94.10 Sexual Function 94.11 Conclusions References 95: Neuropathic Bladder 95.1 Introduction 95.2 Historical Overview 95.2.1 Clean Intermittent Catheterization (CIC) 95.2.2 Mitrofanoff 95.2.3 Additional Surgeries (Szymanski et al. 2020a, b) 95.2.4 Prenatal Diagnosis and Fetal Repair (Metcalfe 2017; Clayton et al. 2020) 95.3 Incidence 95.4 Etiopathogenesis 95.5 Pathophysiology/Pathology 95.6 Diagnosis 95.7 Management 95.8 Conclusions References 96: Bladder Exstrophy 96.1 Introduction 96.2 Historical Overview 96.3 Incidence 96.4 Etiopathogenesis 96.5 Pathophysiology 96.6 Pathology 96.6.1 Musculoskeletal Defects 96.6.2 Abdominal Wall Defects 96.6.3 Genital Defects 96.6.4 Extravesical Genitourinary Defects 96.7 Diagnosis 96.7.1 Prenatal Diagnosis 96.7.2 Postnatal Diagnosis 96.8 Differential Diagnosis 96.9 Management 96.9.1 Immediate Postnatal Management 96.9.2 Surgical Approaches 96.9.3 Modern Staged Repair 96.9.4 Complete Primary Repair 96.9.5 Radical Soft Tissue Mobilization Repair 96.9.6 Postoperative Management 96.9.7 Urinary Continence Surgery 96.10 Complications 96.10.1 Failed Initial Closure 96.10.2 Penile Ischemia 96.10.3 Complications of the Upper Urinary Tract 96.11 Conclusion References 97: Cloacal Exstrophy 97.1 Introduction 97.2 History 97.3 Embryogenesis 97.4 Spectrum of Anatomic Variability 97.5 Preoperative Management 97.6 Operative Management 97.7 Postoperative Care 97.8 Long-Term Management 97.9 Patient Outcomes 97.10 Conclusion References 98: Prune Belly Syndrome 98.1 Introduction 98.2 Etiopathogenesis 98.3 Pathology 98.4 Diagnosis 98.4.1 Antenatal Diagnosis 98.4.2 Newborn Assessment and Investigations 98.4.3 Associated Anomalies 98.5 Differential Diagnosis 98.6 Management 98.7 Conclusion References 99: End-Stage Renal Disease and Renal Transplantation 99.1 Introduction 99.2 Aetiology of End-Stage Renal Disease 99.3 Differential Diagnosis 99.4 Presentation 99.5 Diagnosis 99.6 Complications and Management of End-Stage Renal Disease 99.6.1 Fluid & Electrolyte Balance 99.6.1.1 Fluid Requirements 99.6.1.2 Sodium 99.6.1.3 Potassium 99.6.1.4 Calcium and Phosphate 99.6.1.5 Anaemia 99.6.1.6 Growth & Nutrition 99.6.1.7 Hypertension 99.7 Renal Replacement Therapy (RRT) 99.8 Dialysis 99.8.1 Indications for Initiating Dialysis 99.9 Peritoneal Dialysis (PD) 99.9.1 Physiology of PD 99.9.2 Types of PD 99.9.3 Types of PD Catheters 99.9.4 Types of PD Solution 99.9.5 PD Prescription 99.9.6 Training for PD 99.9.7 Complications of PD 99.9.7.1 Peritonitis 99.9.7.2 Exit Site Infection 99.9.7.3 Hernia/Hydrocele 99.9.7.4 Catheter Malposition/Blockage 99.10 Haemodialysis (HD) 99.10.1 Physiology of HD 99.10.2 Vascular Access for HD 99.10.3 HD Prescription 99.10.3.1 Acute HD 99.10.3.2 Chronic HD 99.10.3.3 Complications of HD 99.11 Renal Transplantation 99.11.1 Transplant Evaluation for Recipient 99.11.2 Donor Characteristics 99.11.3 Indications for Bilateral Nephrectomies Prior to Transplantation 99.11.4 Pre-Emptive Transplantation (PET). 99.11.5 Risk of Recurrence of Primary Disease 99.11.6 Surgical Technique 99.11.7 Surgical Complications 99.12 Transplant Immunobiology 99.12.1 Transplant Immunobiology 99.12.2 Immunosuppressive Treatment 99.12.3 Monoclonal Antibodies 99.12.4 Maintenance Immunosuppressive Therapy 99.12.4.1 Calcineurin Inhibitors 99.12.4.2 Azathioprine and Mycophenolate Mofetil 99.12.4.3 Steroids 99.13 Other Drugs Used at the Time of Transplant 99.14 Medical Complications of a Renal Transplant 99.14.1 Post Transplant Hypertension 99.14.2 Infectious Complications 99.14.2.1 Cytomegalovirus (CMV) 99.14.2.2 Epstein Bar Virus (EBV) 99.14.2.3 BK Virus 99.14.3 Malignancy After Transplant 99.15 Long-Term Graft Survival 99.16 Conclusion Further Reading 100: Different Sexual Development 100.1 Introduction 100.2 Etiology (Sexual Differentiation) 100.2.1 Chromosomal Sex Development 100.2.2 Gonadal Sex Development 100.2.3 Anatomical Sex Development 100.3 Differences/Disorders of Sex Development 100.3.1 Classification 100.4 Differential Diagnosis 100.4.1 Sex Chromosome DSD 100.4.2 46,XY DSD 100.4.3 46,XX DSD 100.5 Diagnosis 100.5.1 Physical Examination 100.5.2 Diagnostic Studies 100.6 Management 100.6.1 Sex Assignment 100.7 Surgical Management 100.7.1 Gonadal Management 100.7.2 Feminizing Surgery (CAH) 100.7.3 Hypospadias Repair 100.7.4 Management of Müllerian Structures 100.7.5 Malformations Associated with DSD Mainly Consist of Cloacal Exstrophy, Permanent Cloaca, Aphallia, and Severe Micropenis 100.7.6 Timing of Surgery 100.8 Conclusion References 101: Cryptorchidism 101.1 Cryptorchidism 101.1.1 Introduction 101.2 Embryology 101.3 Etiology 101.4 Clinical Presentation 101.5 Diagnosis 101.6 Differential Diagnosis 101.7 Investigations 101.8 Rationale for Management 101.9 Treatment 101.10 Complications 101.11 Prognosis 101.12 Conclusion Further Reading 102: Acute Scrotum 102.1 Historical Overview 102.2 Testicular Torsion 102.2.1 Introduction 102.2.2 Incidence 102.2.3 Etiopathogenesis 102.2.3.1 Extravaginal Torsion 102.2.3.2 Intravaginal Torsion 102.2.4 Pathology 102.2.5 Diagnosis 102.2.6 Clinical Features 102.2.7 Differential Diagnosis 102.2.8 Imaging Studies 102.2.8.1 Scrotal Doppler Sonogram 102.2.8.2 Radionucleotide Scans 102.2.8.3 Management 102.2.9 Surgical Approach 102.2.10 Complications 102.3 Epididymitis 102.3.1 Incidence 102.3.2 Overview 102.3.3 Etiopathogenesis 102.4 Torsion of the Testicular Appendage 102.4.1 Incidence 102.4.2 Etiopathogenesis 102.4.3 Pathology 102.5 Conclusion Further Reading 103: Hypospadias 103.1 Introduction 103.2 Historical Overview 103.3 Prevalence 103.4 Aetiopathogenesis 103.5 Pathophysiology 103.6 Pathology 103.7 Diagnosis 103.8 Associated Malformations/Differential Diagnosis 103.9 Management 103.9.1 Preoperative Treatment with Androgens 103.9.2 Preoperative Antibiotic Treatment 103.9.3 Surgical Techniques 103.9.4 Correction of Chordee 103.9.5 Urethroplasty 103.9.6 Glanuloplasty 103.9.7 Foreskin Reconstruction 103.9.8 Postoperative Care 103.9.9 Complications 103.9.10 Postoperative Follow-Up 103.9.11 Postoperative Long-Term Consequences for Life 103.10 Conclusion References 104: Circumcision and Buried Penis 104.1 Circumcision 104.1.1 Introduction 104.1.2 Anatomy 104.1.3 Indications 104.1.3.1 Prevention of Disease 104.1.3.2 Treatment of Disease 104.1.3.3 Presurgical Considerations 104.2 Contraindications 104.3 Procedures 104.3.1 Circumcision Devices 104.3.2 Mogen Shield 104.3.2.1 Gomco Clamp 104.3.2.2 Plastibell Clamp 104.3.2.3 Shang Ring Clamp 104.4 Free Hand Circumcision 104.4.1 Preputial Slit 104.4.2 Complications 104.5 Buried Penis 104.5.1 General 104.5.2 Indications 104.5.3 Preoperative Considerations 104.5.4 Surgical Technique 104.6 Complications 104.7 Conclusions References 105: Hydrometrocolpos 105.1 Introduction 105.2 Historical Overview 105.3 Incidence 105.4 Embryopathology 105.5 Types: Depending Upon the Type of Fluid 105.6 Classification 105.7 Associated Syndromes 105.8 Associated Anomalies 105.9 Antenatal Diagnosis 105.10 Clinical Features 105.11 Differential Diagnosis 105.12 Investigations 105.13 Treatment 105.14 Complications 105.15 Follow-Up 105.16 Conclusion References 106: Gynaecologic Conditions of Childhood 106.1 Introduction 106.2 Gynaecological Conditions Occurring Prior to Puberty 106.2.1 Vulvovaginitis 106.2.2 Prepubertal Vaginal Bleeding 106.2.3 Labial Adhesions/Labial Fusion 106.2.4 Female Genital Mutilation (FGM) 106.3 Ovarian Cysts 106.3.1 Foetal and Neonatal Ovarian Cysts 106.3.2 Ovarian Torsion 106.4 Gynaecological Conditions Occurring After Puberty 106.4.1 Menstrual Dysfunction 106.4.2 Pelvic Inflammatory Disease (PID) 106.4.3 Menstrual Obstruction and Uterovaginal Anomalies 106.4.3.1 Imperforate Hymen 106.4.3.2 Complex Menstrual Obstructive Anomalies Transverse Vaginal Septum Unilateral Menstrual Obstruction 106.5 Mullerian Agenesis: Mayer–Rokitansky–Küster–Hauser Syndrome (MRKH) 106.6 Androgen Insensitivity Syndrome (AIS) 106.7 Congenital Adrenal Hyperplasia 106.8 Conclusion References Index