Pediatric Surgery: Diagnosis and Management

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