Pediatric Surgery. General Principles and Newborn Surgery

✍ Scribed by Prem Puri

• Publisher: Springer
• Year: 2020
• Tongue: English
• Leaves: 1276
• Category: Library

✦ Table of Contents

Preface
Contents
About the Editor
Contributors
Part I: General
1 Embryology of Congenital Malformations
Misconceptions and/or Outdated Theories Concerning Normal and Abnormal Embryology
A Shortage of Study Material (Both Normal and Abnormal Embryos)
A Shortage of Explanatory Images of Embryos and Developing Embryonic Organs
Difficulties in the Interpretation of Serial Sections
Animal Models Used for Applied Embryology
Embryos of Different Species for the Study of Normal Embryology
Surgical Models
Chemical Models
Genetic Models
Spontaneous Malformations Without Genetic Background
Scanning Electron Microscopic Atlas of Normal and Abnormal Development in Embryos
Foregut Development
Normal Foregut Development
Development of the Diaphragm
Normal Development
Abnormal Development
Animal Model
Development of the Hindgut
Normal Development
Abnormal Development
Development of the External Genitalia and the Urethra
Normal Development of the External Genitalia
Abnormal Development of the External Genitalia (Hypospadia Formation)
The Development of the Midgut
Traditional Theories
Own Observations
Testicular Descent
The Role of the Gubernaculum
References
2 The Epidemiology of Birth Defects
Introduction
Birth Prevalences Are the Cornerstone of Birth Defects Epidemiology
Why Is It Important to Collect Birth Defects Data?
The Practical Challenges of Birth Defects Epidemiology
Ethical and Scientific Considerations
On the Causation´´ of Birth Defects Birth Defects Epidemiology and the Pediatric Surgeon Conclusion and Future Directions Cross-References References 3 Prenatal Diagnosis of Congenital Malformations Introduction Ultrasound MRI Amniocentesis Chorionic Villus Sampling (CVS) Biochemical Markers Percutaneous Umbilical Blood Sampling (PUBS) Fetal Cells in the Maternal Circulation Prenatal Diagnosis of Specific Surgical Lesions Neck Masses Sacrococcygeal Teratoma (SCT) Congenital Chest Lesions: Congenital Pulmonary Adenomatoid Malformation and Bronchopulmonary Sequestration Congenital Diaphragmatic Hernia (CDH) Gastrointestinal Lesions Esophageal and Bowel Atresias Abdominal Wall Defects Prenatal Diagnosis of Renal Anomalies Upper Urinary Tract Obstruction Lower Urinary Tract Obstruction (LUTO) Myelomeningocele Conclusion and Future Directions Cross-References References 4 Fetal Counseling for Congenital Malformations Introduction Congenital Malformation Prenatal Diagnosis Fetal Imaging Ultrasound Examination Fetal Echocardiography Magnetic Resonance Imaging Minimally Invasive Diagnostic Tests Prenatal Maternal Serum Screening Genetic Diagnoses Invasive Diagnostic Tests Amniocentesis Chorionic Villus Sampling (CVS) Fetal Blood Sampling (FBS) Future Developments Fetal Surgery Specific Surgical Conditions Congenital Diaphragmatic Hernia (CDH) Cystic Lung Lesions Abdominal Wall Defects Exomphalos Gastroschisis Tracheoesophageal Fistula (TOF) and Esophageal Atresia (OA) Gastrointestinal Lesions Abdominal Cysts Sacrococcygeal Teratomas Renal Anomalies Upper Urinary Tract Obstruction Lower Urinary Tract Obstruction Conclusion and Future Directions Cross-References References 5 Anatomy of the Infant and Child Introduction Growth and Proportions Cardiovascular System The Fetal Circulation Circulatory Changes After Birth The Heart Central Veins Umbilical Vessels Arteries Respiratory System Upper Airway Trachea Bronchial Tree Thorax and Mechanics of Breathing Abdominal Wall and Gastrointestinal Tract Abdominal and Pelvic Cavities Gastrointestinal Tract Liver, Gallbladder, and Spleen Genitourinary System Kidneys and Suprarenal Glands Bladder and Ureter Genitalia and Reproductive Tract Musculoskeletal System Skull and Face Vertebral Column, Pelvis, and Limbs Nervous System Skin and Subcutaneous Tissue Conclusion Cross-References References 6 Perinatal Physiology Introduction The Fetal Circulation and Its Transition at Birth Closure of the Ductus Venosus Closure of the Foramen Ovale Fall in Pulmonary Vascular Resistance Closure of the Ductus Arteriosus The Respiratory System Fetal Breathing Movements (FBM) Peripheral Chemoreceptor Function In Utero Pharmacological Control Lung Growth Associated with FBM Changes at Birth Conclusion References 7 Fetal Surgery Introduction Imaging and Diagnostics Principles of Maternal-Fetal Surgery Indications and Contraindications Anesthesia Positioning and Draping Incision and Exposure Closure Postoperative Care Outcomes Fetoscopy Ex Utero Intrapartum Therapy (EXIT) Anatomic Anomalies Currently Managed by Fetal Surgery Congenital Bronchopulmonary Malformations Pathophysiology Diagnosis Treatment Outcomes Congenital Diaphragmatic Hernia Pathophysiology Diagnosis Treatment of CDH Outcomes in CDH Myelomeningocele Pathophysiology and Natural History Diagnosis Treatment Outcomes Sacrococcygeal Teratoma Pathophysiology and Natural History Diagnosis Treatment Outcomes Twin-to-Twin Transfusion Syndrome Pathophysiology of TTTS Diagnosis of TTTS Treatment of TTTS Outcomes of TTTS Conclusion and Future Directions Cross-References References 8 Specific Risks for the Preterm Infant Introduction General Respiratory Respiratory Distress Syndrome Pneumothorax Pulmonary Hemorrhage Bronchopulmonary Dysplasia (BPD) and Chronic Lung Disease (CLD) Apnea of Prematurity Cardiovascular Blood Pressure Patent Ductus Arteriosus Congenital Heart Disease Central Nervous System Intraventricular Hemorrhage Periventricular Leucomalacia Retinopathy of Prematurity (ROP) Gastrointestinal Tract Feeding and Nutrition Necrotizing Enterocolitis Spontaneous Bowel Perforation Inguinal Hernia Umbilical Hernia Infection Other Hyperbilirubinemia Electrolytes Anemia References 9 Pediatric Clinical Genetics Introduction Diagnosis of Malformation Syndromes Introduction Definitions Used with Congenital Anomalies A Clinical Genetic Approach to Diagnosis of Malformation Syndromes Genetic Etiology of Congenital Anomalies Introduction Chromosome Disorders Down´s Syndrome Patau´s Syndrome Edward´s Syndrome Other Chromosome Disorders Single-Gene Disorders Autosomal Dominant Inheritance Single-Gene Disorders and Autosomal Recessive Inheritance Single-Gene Disorders and X-Linked Recessive Inheritance Single-Gene Disorders and Atypical Forms of Inheritance Polygenic/Multifactorial Disorders Genetic Testing Chromosomal G-Banding Analysis (Standard Karyotyping) Fluorescent In Situ Hybridization Array Comparative Genomic Hybridization/Chromosomal Microarray Analysis Specific Testing for Common Known Single-Gene Mutations Next-Generation Sequencing and Gene Panels Conclusions and Future Directions Cross-References Further Reading 10 Transport of Sick Infants and Children Introduction Transport Management Airway Management Circulation and Homeostasis Documentation Transport Team Vehicles and Equipment Transport Vehicles Monitoring and Equipment Transport Procedure Receiving Center Special Considerations for Neonates Prenatal Transfer Neonatal Transfer Temperature Regulation Transport Incubators Conclusion and Future Directions Cross-References References 11 Pediatric Respiratory Physiology Introduction Embryonic Phase (Until Eighth Week of Gestation) Pseudoglandular Phase (5th-17th Week of Gestation) Canalicular Phase (16th-26th Week of Gestation) Saccular Phase (24th Week of Gestation Until Birth) Alveolar Phase (36th Week of Gestation to 18 month-4 years Postnatal) Prenatal Development of the Pulmonary Surfactant System The Role of Lung Fluid and Fetal Breathing Movements in Lung Organogenesis and Growth Physiology of Transition from Intra- to Extrauterine Life Respiratory Physiology of the Neonatal Period Anatomy Pulmonary Circulation Pulmonary Gas Exchange Lung Volumes Airway Resistance Lung Compliance Respiratory Distress Syndrome Pathophysiology Incidence Clinical Symptoms X-Ray Findings in RDS Prevention/Therapy Antenatal Corticosteroids CPAP Surfactant Perspective Persistent Pulmonary Hypertension of the Newborn Etiology of PPHN Diagnosing PPHN General Management of PPHN Specific Medical Treatment Conclusion and Future Directions Cross-References References 12 Pediatric Cardiovascular Physiology Introduction Determinants of Cardiac Output Preload General Principles Therapeutic Implications Afterload General Principles Therapeutic Implications Contractility General Principles Therapeutic Implications Heart Rate General Principles Therapeutic Implications Regulation of Whole-Body Oxygen Delivery The Fetal Circulation How Selected Types of Congenital Heart Disease Affect Cardiovascular Physiology Conclusions and Future Directions Cross-References References 13 Pediatric Hepatic Physiology Introduction Liver Blood Flow Fetal Life and Transitional Circulation Postnatal Life Metabolic Functions of Liver Bile Formation Bilirubin Physiology of Newborn Jaundice Bile Acids Hepatic (or Canalicular) Bile Hormonal Control of Bile Flow Carbohydrate Metabolism Protein Synthesis Albumin Nitrogen Metabolism Lipid Metabolism The Role of the Liver in Hemostasis and Clotting Conclusions and Future Directions Cross-References References General Reading 14 Metabolism of Infants and Children Introduction The Metabolic Response to Stress Nutritional Assessment/Estimation of Energy Expenditure During Illness Nutrient Reserves and Requirements Macronutrient Intake Review of Protein Metabolism and Requirement During Illness Protein Quality Modulating Protein Metabolism Carbohydrate Metabolism Glucose Control Lipid Metabolism Conclusion and Future Directions Cross-References References 15 Fluid and Electrolyte Balance in Infants and Children Introduction Water Distribution in Infants and Children Effective Circulating Volume (ECV) Serum Osmolality Water Homeostasis Insensible Water Loss Sweating Renal Blood Flow Glomerular Filtration Rate Disorders of Body Fluid Volume: Dehydration and ECF Depletion Volume Depletion Dehydration Risk Factors for Dehydration Consequences of Dehydration Symptoms and Signs of Dehydration Degree of Dehydration Laboratory Testing Fluid and Electrolyte Management Electrolytes Sodium Dysnatremia Hyponatremia Hypernatremia Potassium Hyperkalemia Symptoms and Signs of Hyperkalemia Diagnostic Tests Treatment of Hyperkalemia Hypokalemia Causes of Hypokalemia Symptoms and Signs of Hypokalemia Complications of Hypokalemia Investigations of Hypokalemia Treatment of Hypokalemia Calcium Hypercalcemia Hypocalcemia Magnesium Hypomagnesemia Hypermagnesemia Phosphate Hypophosphatemia Treatment of Hypophosphatemia Hyperphosphatemia Acid-Base Balance Acid-Base Balance Disorders Metabolic Acidosis Metabolic Alkalosis Perioperative Management in Infants and Children Intraoperative Management Postoperative Management Fluid and Electrolyte Balance in Septic Shock Clinical Manifestations Management Total Parenteral Nutrition (TPN) Conclusions and Future Directions Cross-References References 16 Vascular Access in Infants and Children Introduction Peripheral Venous Access Central Venous Access Peripherally Inserted Central Catheter (PICC) Umbilical Venous Catheters (UVC) Tunneled and Cuffed Catheter Implantable Vascular Access Devices (Ports) Intraosseous Access Arterial Vascular Access Umbilical Artery Catheterization Radial Artery Cannulation Pedal Arterial Cannulation Axillary and Femoral Arterial Cannulation Complications and Their Management Acute Complications Long-Term Complications Conclusions and Future Directions Cross-References References 17 Nutrition in Infants and Children Introduction Changes in Body Composition Components of Energy Needs Energy Requirements of Surgical and Critically Ill Infants Parenteral Nutrition Indications Route of Administration Components of Parenteral Nutrition Fluid Requirements Glucose Lipids Amino Acids Minerals, Vitamins, and Trace Elements Complications of Parenteral Nutrition Infectious Complications Mechanical Complications Hepatic Complications Enteral Nutrition Feeding Routes Selection of Enteral Feeds Administration of Enteral Feeds Complications of Enteral Tube Feeding Conclusion and Future Directions References 18 Access for Enteral Nutrition Introduction Naso-, Orogastric or Naso-, Oroenteric Access Gastrostomy Historical Perspective Indications Esophageal Abnormalities Duodenal Obstruction Major Abdominal Wall Defects Short-Gut Syndrome Other Surgical PathologiesNonsurgical´´ Pathologies
Choice of Procedure/Technique
Open Technique
Percutaneous Endoscopic Gastrostomy
Minimally Invasive Gastrostomies
Choice of Access Device
Complications and Management
Complications Related to Operative Technique
Complications Related to Care of Stoma
Complications Related to Catheters
Gastrostomy Closure and Persistent Gastrocutaneous Fistula
Jejunostomy
Indications
Choice of Procedure
Needle Catheter Jejunostomy
Catheter Placement Directly into the Jejunum
Catheter Placement in a Partially Excluded Loop
Devices
Postoperative Care and Complications
Conclusion and Future Directions
Cross-References
References
19 Tracheostomy in Infants
Introduction
Indications for Tracheostomy
Preoperative Work-up and Planning
Technique
Position of Patient
Incision and Dissection to Expose the Trachea
Opening the Trachea and Insertion of the Cannula
Early Postoperative Care
Home Instruction and Care
Complications
During Procedure
Immediate Post-Op
Surgical Emphysema
Bleeding
Obstruction, for Example, Clot and Mucus
Dislodgement
Late Post-Op and Homecare
Special Considerations
Ex Utero Intrapartum Therapy (EXIT)
Emergency Needle Cricothyroidotomy
Decannulation
Practical Steps of the Procedure
Weaning
Capping
Conclusion and Future Directions
Cross-References
References
20 Pediatric Airway Assessment
Introduction
Anatomy
Assessment of the Pediatric Airway
Examination
Radiology Investigations
Rigid Microlaryngoscopy Bronchoscopy
Differential Diagnosis of Pediatric Stridor
Conclusion
Cross-References
References
21 Stomas of Small and Large Intestine
Introduction
Most Common Errors in Colostomies
Stoma Mislocation
Prolapse
Stenosis
Retraction
Stomas in Cloacal Exstrophy (Soffer et al. 2000)
Colostomies in Hirschsprung´s Disease
Ileostomies
Colostomy Closure
Surgical Technique
Conclusion and Future Direction
Cross-References
References
22 Preoperative Assessments in Pediatric Surgery
Introduction
Preoperative Assessment for Day Case Surgery
Clinic Design
Patient Assessment
History and Clinical Examination
Perinatal History
Medical History
Medication History
Allergy History
Immunization History
Anesthetic History
Social History
Clinical Examination
Investigations
Further Information
Fasting Guidelines
Follow-Up
General Education Opportunities
Completion of the Assessment
Preoperative Assessment for Inpatient Surgery
Investigations
Blood Grouping and Crossmatching
Preoperative Mechanical Bowel Preparation
Optimization of Nutrition
Specific Considerations
The Child with an Incidental Murmur
Endocarditis Prophylaxis
The Child with an Upper Respiratory Tract Infection (URTI)
The Obese Child
The Child with Diabetes
The Child with Cystic Fibrosis
Preoperative Assessment of the Neonate
History and Clinical Examination
Temperature Regulation
Respiratory Function
Cardiovascular Status
Metabolic Status
Acid-Base Balance
Hypoglycemia
Hypocalcemia
Hypomagnesemia
Hyperbilirubinemia
Coagulation Abnormalities
Laboratory Investigations
Fluid and Electrolytes and Metabolic Responses
Conclusion and Future Directions
Cross-References
References
23 Principles of Pediatric Surgical Imaging
Introduction
Effects of Radiation on Children
Environmental Issues for a Pediatric Radiology Department
Patient Preparation
Order of Examinations
Waiting Area
Examination/Procedure Rooms
Sedation and Anesthesia in the Radiology Department
Techniques
Natural Sleep
Sedation
General Anesthesia
Assessment
Consent
Monitoring
Equipment
Personnel
Contrast Media and Use in the Pediatric Radiology Department
Barium Preparations
Iodinated Contrast Agents
Contrast Use in the Fluoroscopy Suite
Contrast Use in the CT Suite
Contrast Use in the MRI Suite
Contraindications/Side Effects/Adverse Reactions
Pediatric Interventional Radiology
Conclusion and Future Directions
Cross-References
References
24 Hematological Problems in Pediatric Surgery
Introduction
Hematological Basic Science
Blood Formation (Hematopoiesis)
Mechanisms of Hemostasis
Natural Anticoagulation Control Mechanisms
Platelets
Blood Groups and Antibodies
Hematological Disorders Encountered in Pediatric Practice: A Surgical Perspective
Hemophilia
Clinical Features
Treatment
Central Venous Access Devices (CVADs)
von Willebrand Disease
Platelet Disorders
Immune Thrombocytopenia Purpura (ITP)
Clinical Features
Pathophysiology
Clinical Course and Treatment (Provan et al. 2010)
Disseminated Intravascular Coagulation (DIC)
Treatment
Thrombotic Disorders
Treatment
Asplenia/Splenectomy/Hyposplenism
Anemia
Hereditary Spherocytosis
Sickle Cell Disease (SCD)
Clinical Features and Management (Yawn et al. 2014)
Chronic Complications
Disease-Modifying Therapy
Surgery in Sickle Cell Disease
Key Principles of Perioperative Management
Thalassemia
Neutropenia
Leukemia
Management of Acute Leukemia
Surgical Issues in the Leukemic Patient
Blood Products and Their Transfusion
Acute Complications of Blood Transfusion
Other Adverse Reactions to Blood Product Transfusion
Conclusion and Future Directions
Cross-References
References
25 Surgical Safety in Children
Introduction
Background: The History of Surgical Error
Tackling Concerns - Improvement Methodology
Improvement Programs
Policy-Based Care
Human Factors in Surgery
Definitions and Examples in Other Industries
The Evolution of Non-technical Skills in Aviation: Crew Resource Management (CRM)
From Aviation to Surgery
NOTSS (Non-technical Skills for Surgeons)
NOTSS and Implications for Surgical Performance
Situation Awareness
Decision-Making
Leadership
Communication and Teamwork
Personal Awareness
Surgical Checklists
Surgical Briefings
Conclusion and Future Directions
References
26 Anesthesia and Pain Management
Introduction
Preoperative Optimization
Fasting Prior to Anesthesia and Surgery
The Operating Theater and Anesthetic Equipment
Breathing System
Laryngoscopes
Ventilators
Anesthesia
Monitoring
Cardiovascular Monitoring
Respiratory Monitoring
Temperature
Neuromuscular Blockade
Induction and Maintenance of Anesthesia
Recovery
Fluid Balance
Postoperative Pain Management
Pain Assessment
Analgesia
Regional Anesthesia
Special Considerations in the Premature Infant
Anesthesia and the Developing Brain
Anesthesia for Specific Surgical Conditions in the Newborn
Esophageal Atresia
Congenital Diaphragmatic Hernia
Intestinal Obstruction
Exomphalos and Gastroschisis
Herniotomy for the Ex-premature Infant
Postoperative Admission to Surgical NICU/PICU
Conclusion and Future Directions
Cross-References
References
27 Immunology and Immunodeficiencies in Children
Introduction
Monocytes
Neutrophils
Toll-Like Receptors
Mucosal Immunity, Human Milk, and Necrotizing Enterocolitis (NEC)
Neonatal Innate Immunity
Pediatric Adaptive Immune Response
Lymphocyte Phenotype
B-Cell Responses
Immunoglobulins
Cytokines
The Inflammatory Response Syndromes
Clinical Outcomes in Neonatal Sepsis and Inflammation
Pediatric Sepsis
Primary Immunodeficiencies
B-Cell Deficiencies
T-Cell Deficiencies
Combined B- and T-Cell Deficiencies
Complement Deficiencies
Phagocytic Cell Deficiencies
Acquired Immunodeficiency
Immunomodulation
Conclusion and Future Directions
Cross-References
References
28 Sepsis
Introduction
Terminology
Epidemiology
Pathogenesis
Barriers to Infection
Host Response
Cellular Immunity
Macrophages
Lymphocytes
Humoral Factors
Complement
Immunoglobulins
Cytokines
Bacterial Virulence
Neonatal Defenses
Diagnosis
Treatment of Sepsis in Children
Neonatal Septic Shock
Conclusion and Future Directions
Cross-References
References
29 Principles of Minimally Invasive Surgery in Children
Introduction
Basic Principles
Patient Selection and Preoperative Workup
Ergonomics
Advances in Technology and Intraoperative Considerations
Technical Considerations
Risks and Benefits
Evolution of Single Site Laparoscopic Surgery (SILS)
Role of Robotics in Pediatrics
Conclusion
Cross-References
References
30 Innovations in Minimally Invasive Surgery in Children
Introduction
From Open to Laparoscopic in Pediatric Surgery
Laparoscopes and Hand Instruments
Trocars
Insufflators
Neonatal Laparoscopy
Needlescopic Surgery
Laparoscopic Inguinal Hernia Repair
Hiding the Scars
Single-Incision Laparoscopy
Single-Incision Laparoscopic Techniques in Pediatric Patients
Single-Port Laparoscopic Cholecystectomy: Considerations in Children
Key Steps
2x2´´ Laparoscopic Cholecystectomy: Scarless Surgery in Children Key Steps Single-Incision Laparoscopic Gastrostomy: Utilizing an Operative Hysteroscope Key Steps Appendectomy Key Steps Natural Orifice Endoluminal Surgery (NOTES) Other Innovations Robotic Surgery Technical Benefits of Robotic Surgery Technical Limitations of Robotic Surgery Patient Benefits of Robotic Surgery Costs Safety Learning Curve Future Telemedicine Gastric Stimulators Conclusion and Future Directions Cross-References References 31 Fast-Track Pediatric Surgery Introduction Historical Aspects Current Concept The Evolution of Fast-Track Pediatric Surgery The Concepts and Components of Fast-Track Pediatric Surgery Conclusion and Future Directions Cross-References References 32 Ethical Considerations in Pediatric Surgery Introduction Defining the Best Interests Standard Applying the Best Interests Standard Guidelines for Ethical Decision-Making Informed Consent and Assent Multiculturalism Prenatal Surgical Consultation and Fetal Surgery Ethical Issues in Pediatric Bariatric Surgery Surgeons and Industry Surgical Error Best Interests and Innovation and Clinical Research Conclusion and Future Directions Cross-References References 33 Childhood Obesity Introduction Measuring Overweight and Obesity in Children Indices Based on Weight for Height Measures Consequences of Different Growth Standards Skinfolds and Body Circumference Global Burden of Childhood Obesity Childhood Obesity in LMICs Childhood Obesity and Social Economic Class Pathways to Childhood Obesity Developmental Mismatch Maternal Obesity and Gestational Diabetes Mellitus Health Consequences of Childhood Obesity Childhood Obesity and Surgery Related Complications How Early Should Prevention for Childhood Obesity Start? Conclusion and Future Directions Cross-References References 34 Surgical Problems of Children with Physical Disabilities Introduction Multidisciplinary Management Family-Centered Care Non-accidental Trauma: Abuse and Neglect General Perioperative Issues Ethical Complexity Perioperative Considerations in Specific CSHCN Conditions Cerebral Palsy Spasticity, Body Habitus, and Scoliosis General Perioperative Neurologic Considerations Nutrition, Feeding, and Gastroesophageal Reflux Neurologic Dysphagia and Aspiration Evaluation of a Possible Feeding Disorder Gastrostomy Jejunostomy Gastroesophageal Reflux and Delayed Gastric Emptying Complications of Gastrostomy and Fundoplication Surgical Outcomes of Enteral Access or Fundoplication Decubitus Ulcers Down Syndrome: Trisomy 21 Gastrointestinal Malformations and Down Syndrome Other Operative Considerations in Patients with Down Syndrome Spina Bifida Latex Allergy Resulting in Intraoperative Anaphylaxis Hydrocephalus Drainage and Shunt Considerations Spina Bifida and the Acute Abdomen Esophagitis Neurogenic Bowel Antegrade Colonic Irrigation Conclusion and Future Directions Cross-References References 35 Clinical Research and Evidence-Based Pediatric Surgery Introduction Common Types of Study Design Concept of Evidence-Based Medicine (EBM) The 5As of an EBM Cycle Asking an Answerable Clinical Question in thePICO´´ Format
Searching for and Selecting the Best Available Evidence for a PICO Question
Critical Appraisal of the Identified Evidence
Interpreting the Internal Validity of a Study Result by Assessing the Role of Chance, Bias, and Confounding
Look Out for Possibility of Bias in Relation to Different Study Designs
Have Potential Confounding Factors Been Addressed?
Examining the Role of Chance and Statistical Significance of the Result
Examining the Clinical Significance of the Result
Causality Assessment of the Result by Bradford Hill´s Criteria
Is the Conclusion Applicable to the Patient in the Clinical Question?
Clinical Research in Pediatric Surgery
Constructing a Research Question and Designing the Study
Planning the Logistics for a Clinical Research
Data Acquisition and Analysis
Sample Size Estimation
Preparing for a Grant Application
Communicating the Research Findings in an Evidence-Based Format
Conclusion and Future Directions
Cross-References
References
36 Tissue Engineering and Stem Cell Research
Introduction
Stem Cells
Embryonic Stem Cells
Somatic Cell Nuclear Transfer
Induced Pluripotent Stem Cells
Fetal Stem Cells
Adult Stem Cells
Scaffolds
Naturally Derived Materials
Synthetic Materials
Natural Acellular Matrix
Cell-Derived ECM Scaffolds
Regenerative Medicine
Conclusion
References
37 Patient- and Family-Oriented Pediatric Surgical Care
Introduction
Core Principles
History of Patient and Family-Oriented Care
Additional Resources
Delivering News
The Prepared Messenger
Pre-managing One´s Personal Story and Pain
Physical Presence
Communicating News
What to Say
Conclusion and Future Directions
Cross-References
References
38 Surgical Implications of Human Immunodeficiency Virus Infection in Children
Introduction
Infections
Gastrointestinal Tract Disease
Intra-abdominal Pathology
Perineal Disease
Malignancy
Medical Aspects of Pediatric HIV Infection and Their Effects on Surgery
Outcomes of HIV Infected Children Undergoing Surgery
Factors Influencing Post-surgical Complications in HIV Infected Children
HIV Exposed Uninfected (HIVe) Children
Conclusion and Future Directions
Cross-References
References
Part II: Newborn Surgery: Head and Neck
39 Choanal Atresia
Introduction
Epidemiology
Embryology
Suggested Etiology
Clinical Features
Diagnosis
Management
Endoscopic Technique
Transpalatal Technique
Controversies in Choanal Atresia Management
Nasal Stents
Mitomycin C
Outcomes
Conclusion and Future Directions
Cross-References
References
40 Macroglossia
Introduction
Etiology and Pathology
Presentation
Diagnosis
Management
Surgery
Conclusion and Future Directions
Cross-References
References
41 Pierre Robin Sequence
Introduction
Epidemiology
Etiology
Genetics
Clinical Features
Airway Management
Nutritional Management
Cleft Palate
Micrognathia/Retrognathia
Skeletal Anomalies
Ear Problems
Cardiovascular Anomalies
Ocular Anomalies
Nasal Obstruction
Conclusion and Future Directions
Cross-References
References
42 Lymphatic Malformations in Children
Introduction
Classification
Diagnosis
Prenatal Management
Postnatal Management
Operative Approach to Classic Neck Lymphatic Malformation
Conclusions and Future Directions
Cross-References
References
43 Stridor in the Newborn
Introduction
Laryngeal Anomalies
Laryngomalacia
Vocal Cord Paralysis
Subglottic Stenosis
Subglottic Hemangioma
Other Laryngeal Anomalies
Laryngeal Clefts
Laryngeal Web
Congenital Laryngeal Cysts
Laryngeal Lymphangioma
Tracheomalacia
Other Airway Pathology
Conclusion and Future Directions
Cross-References
References
Part III: Newborn Surgery: Esophagus
44 Esophageal Atresia
Introduction
Incidence and Etiology
Embryology
Associated Malformations
Classification
Diagnosis
Prenatally
Postnatal Symptoms
Radiological Diagnosis
Management
Preoperative Management
Operative Management
Esophageal Atresia with Distal Tracheoesophageal Fistula (85%)
Esophageal Atresia with Proximal and Distal Tracheoesophageal Fistula (1.5%)
Esophageal Atresia with a Proximal Tracheoesophageal Fistula Only: A Long Gap Problem (1%)
H-Type Fistula (4%)
The Long Gap Problem (8.5%)
Complications
Early Complications
Late Complications
Long-Term Follow-Up
Conclusions and Future Directions
Cross-References
References
45 Congenital Esophageal Stenosis
Introduction
Pathology
Epidemiology/Etiology
Symptoms
Diagnosis
Treatment
Nonsurgical Treatment
Surgery
Management of Complications Following Treatment
Prognosis
Conclusion and Future Directions
Cross-References
References
46 Esophageal Duplication Cyst
Introduction
Embryology
Relevant Anatomy
Pathology
Clinical Features and Presentation
Antenatal
Incidental
Symptomatic
Malignancy
Investigations
Management
Thoracoscopic Surgery
Thoracotomy
Surgical Management of Various Types of Esophageal Duplication Cysts
Cervical and Suprasternal Cyst
Left-Sided Thoracic Inlet Duplication Cyst
Right-Sided Thoracic Duplication Cyst
Intra-abdominal Esophageal Duplication Cyst
Neuroenteric Cyst
Surgical Complications
Conclusions and Future Directions
Cross-References
References
47 Esophageal Perforation in the Newborn
Introduction
Classification and Etiology
Diagnosis and Clinical Manifestations
Management
Conclusion and Future Directions
Cross-References
References
Part IV: Newborn Surgery: Chest
48 Congenital Airway Malformations
Introduction
Patient Assessment
Medical History
Signs and Symptoms
Diagnostic Studies
Congenital Laryngeal Anomalies
Laryngomalacia
Subglottic Stenosis
Vocal Fold Paralysis
Posterior Laryngeal Cleft
Laryngeal Atresia
Congenital High Airway Obstruction Syndrome
Subglottic Hemangioma
Anomalies of the Trachea and Bronchi
Tracheal Agenesis and Atresia
Tracheal Stenosis and Webs
Tracheal Webs
Cartilaginous Ring Aplasia
Tracheal Cartilaginous Sleeve
Complete Tracheal Rings
Tracheal Diverticulum and Tracheal Bronchus
Airway Malacia
Esophageal Bronchus
Bronchogenic Cyst
Bronchial Atresia and Bronchial Lobar Agenesis
Bronchial Atresia
Bronchial Lobar Agenesis
Bronchial Stenosis
Conclusion and Future Directions
Cross-References
References
49 Vascular Rings
Introduction
Definition and History
Frequency
Embryology
Forms
Double Aortic Arch
Right Aortic Arch
Right Aortic Arch with Aberrant Left Subclavian Artery and Left Ligamentum Arteriosum
Right Aortic Arch with Mirror Image Branching and Retroesophageal Ligamentum Arteriosum
Pulmonary Artery Sling
Vascular Rings Associated with Left Aortic Arch
Left Aortic Arch with Right Descending Aorta and Right Ligamentum Arteriosum
Left Aortic Arch and Left Ligamentum Arteriosum with Retroesophageal Right Subclavian Artery
Clinical Findings
Associated Syndromes and Noncardiac Conditions
Diagnostic Tests
Laboratory Studies
Chest Radiography
Barium Esophagram
Echocardiography and Color Flow Doppler
Computerized Tomography Scan, Magnetic Resonance Imaging, and Digital Subtraction Angiography
Bronchoscopy
Indication for Intervention
Surgical Therapy
Division of a Double Aortic Arch
Traditional Thoracotomy Approach
Video-Assisted Technique
Right Aortic Arch
Pulmonary Artery Sling
Left Aortic Arch with Anomalous Origin of the Innominate Artery
Results
Conclusion and Future Directions
Cross-References
References
50 Pulmonary Air Leaks of the Neonate
Introduction
Pneumothorax
Presentation and Diagnosis
Treatment
Prognosis
Pulmonary Interstitial Emphysema
Presentation and Diagnosis
Treatment
Prognosis
Pneumomediastinum
Pneumopericardium
Pneumoperitoneum
Conclusion and Future Directions
Cross-References
References
51 Chylothorax and Other Pleural Effusions in Neonates
Introduction
Anatomy and Embryology of the Lymphatic System
Pathophysiology of Chyle
Etiology and Presentation of Chylothorax
Congenital Chylothorax
Acquired Chylothorax
Clinical Features of Chylothorax
Presenting Symptoms
Consequence of Continuous Chylous Flow
Confirming the Diagnosis
Management of Chylothorax
Nonoperative Management
Operative Management
Pleuroperitoneal Shunts
Other Surgical Alternatives
General Management Principles
Fetal Chylothorax (Hydrothorax)
Other Pleural Effusions
Hemothorax
Empyema
Cross-References
References
52 Congenital Malformations of the Lung
Introduction
Embryology
Anatomy
Congenital Pulmonary Airway Malformations/Congenital Cystic Adenomatoid Malformations
Pathology
Diagnosis
Radiographic
Clinical Features
Management
Fetal Therapy
Postnatal Therapy
Pulmonary Sequestrations
Pathology
Diagnosis
Management
Congenital Lobar Emphysema
Pathology
Diagnosis
Presentation and Diagnosis
Clinical Features
Management
Bronchogenic Cysts and Congenital Lung Cysts
Pathology
Presentation and Diagnosis
Management
Pulmonary Hypoplasia, Aplasia, and Agenesis
Lung Surgery in Newborns
Complications and Long-Term Outcomes
Conclusions and Future Directions
Cross-References
References
53 Congenital Diaphragmatic Hernia
Introduction
Etiology
Embryogenesis
Pathophysiology
Diagnosis
Treatment Modalities
Prenatal Treatment
Preoperative Treatment
Operative Repair
Postoperative Treatment
Prognosis
Outcome
Congenital Eventration of the Diaphragm (CDE)
Clinical Features
Diagnosis
Management
Operative Repair
Outcome
Conclusions and Future Directions
Cross-References
References
54 Extracorporeal Membrane Oxygenation for Neonatal Respiratory Failure
Introduction
Types of Extracorporeal Membrane Oxygenation
Technical Equipment
Surgical Technique
Patient Management in ECMO
Complications
Conclusion and Future Directions
Cross-References
References
Part V: Newborn Surgery: Gastrointestinal
55 Pyloric Atresia and Prepyloric Antral Diaphragm
Introduction
Pyloric Atresia
Definition and Classification
Epidemiology
Etiopathogenesis
Diagnosis
Prenatal Diagnosis and Counselling
Clinical Presentation
Laboratory
Radiologic Findings
Differential Diagnosis
Treatment
Complications
Outcome
Prepyloric Antral Diaphragm
Definition
Epidemiology
Etiopathogenesis
Diagnosis
Clinical Presentation
Radiologic Findings
Differential Diagnosis
Treatment
Complications
Outcome
Conclusion and Future Directions
Cross-References
References
56 Infantile Hypertrophic Pyloric Stenosis
Introduction
Epidemiology
Etiology
Genetic Factor
Extrinsic Factors/Environmental Factors
Pathophysiology
Clinical Presentation and Evaluation
Clinical Presentation
Evaluation
Physical Examination
Diagnostic Imaging
Blood Chemistry
Differential Diagnosis
Management
Preoperative Management
Operative Care
Nonoperative Treatment
Prophylactic Antibiotics
Postoperative Feeding
Other Postoperative Considerations
Postoperative Apnea
Postoperative Emesis
Conclusion and Future Directions
Cross-References
References
57 Gastric Volvulus
Introduction
Etiology
Pathogenesis
Clinical Features
Diagnosis
Treatment
Complications
Conclusion and Future Directions
Cross-References
References
58 Gastric Perforation
Introduction
Etiology
Clinical Presentation
Diagnosis
Differential Diagnosis
Perioperative Care
Surgical Technique
Conclusion and Future Directions
Cross-References
References
59 Duodenal Obstruction
Introduction
Etiology
Associated Malformations
Prenatal Diagnosis/History
Clinical Presentation and Diagnosis
Preoperative Management
Operation
Incision
Exploration and Identification of Pathology
``Diamond-Shaped´´ Duodenoduodenostomy
Side-to-Side Duodenoduodenostomy
Operative Technique for Duodenal Web
Laparoscopic Management of DO
Postoperative Care
Management of Persistent Megaduodenum by Duodenoplasty
Outcome and Long-Term Results
Conclusion and Future Directions
Cross-References
References
60 Malrotation
Introduction
Epidemiology
Pathophysiology
Clinical Presentation
Imaging Studies
Surgery
Open Ladd´s Procedure
Laparoscopic Ladd´s Procedure
Complication of Surgery
Controversies
Conclusion and Future Directions
Cross-References
References
61 Congenital Hyperinsulinism
Introduction
Epidemiology and Types
Histology
Diagnosis
Treatment
Medical Treatment
Surgical Treatment
Diffuse CHI
Open Near-Total Pancreatectomy
Laparoscopic Near-Total Pancreatectomy
Focal CHI
Surgery for Focal CHI
Focal Lesion in Head or Neck of Pancreas
Focal Lesion in Body or Tail of Pancreas
Postoperative Management
Conclusion and Future Directions
Cross-References
References
62 Jejunoileal Atresia and Stenosis
Introduction
Demographics
Etiology/Pathophysiology
Classification
Pathology
Prenatal Findings
Clinical Presentation
Postnatal Radiology
Differential Diagnosis
Management
Postoperative Care
Postoperative Complications
Prognosis and Outcomes
Conclusion and Future Directions
Cross-References
References
63 Colonic and Rectal Atresias
Colonic Atresia
Introduction
Etiology
Presentation
Diagnosis
Management
Preoperative
Operative
Postoperative
Complications and Long-Term Results
Rectal Atresia
Introduction
Etiology
Presentation
Diagnosis
Management
Complications and Long-Term Results
Conclusion and Future Directions
Cross-References
References
64 Duplications of the Alimentary Tract
Introduction
Pathogenesis
Distribution and Presentation
Clinical Features
Lingual
Esophageal
Thoracoabdominal
Gastric
Duodenal
Pancreatic
Small Bowel
Colonic
Rectal
Prenatal Diagnosis
Associated Anomalies
Imaging
Surgical Treatment
Esophageal
Thoracoabdominal
Gastric
Duodenal
Pancreatic
Small Bowel
Colonic and Appendiceal
Rectal
Conclusion and Future Directions
References
65 Mesenteric and Omental Cysts
Introduction
Clinical Presentation
Diagnosis
Differential Diagnosis
Treatment
Follow-Up
Outcome and Conclusion
Cross-References
References
66 Necrotizing Enterocolitis
Introduction
Epidemiology of Necrotizing Enterocolitis Worldwide
Pathogenesis
Risk Factors
Mechanisms of Intestinal Mucosal Injury
Histopathologic Findings
Risk Factors for NEC in the Full-Term Infant
Clinical Features
Presentation
Laboratory Findings
Associated Comorbidities
Management
Nonoperative
Operative Indication and Technique
Outcomes
Overall
Postoperative Outcomes
Long-Term Outcomes
Gastrointestinal
Neurodevelopmental Outcome
Conclusions and Future Directions
Cross-References
References
67 Meconium Ileus
Introduction
History
Definition and Classification
Simple MI
Complex MI
Epidemiology
Pathogenesis and Pathophysiology
Diagnosis
Prenatal Detection
Postnatal Presentation
Imaging
Laboratory Tests
Differential Diagnosis
Non-CF Related Meconium Obstruction
Intestinal Atresia
Intestinal Malrotation and Midgut Volvulus
Hirschsprung´s Disease
Meconium Plug Syndrome and Neonatal Small Left Colon
Hypothyroidism
Treatment
Nonoperative Management
Operative Management
Simple MI: Operative Management
Complicated MI: Operative Management
Outcomes and Follow-Up
Conclusion and Future Directions
Cross-References
References
68 Meconium Peritonitis
Introduction
Etiopathogenesis
Clinical Findings and Diagnosis
Treatment
Complications
Conclusion and Future Directions
Cross-References
References
69 Neonatal Ascites
Introduction
Etiology and Pathophysiology
Clinical Presentation
Diagnosis
Management
Conclusions and Future Directions
Cross-References
References
70 Hirschsprung´s Disease
Introduction
Historical Overview
Incidence
Etiopathogenesis
Pathophysiology
Cholinergic Hyperinnervation
Adrenergic Innervation
Nitrergic Innervation
Interstitial Cells of Cajal
Platelet-Derived Growth Factor Receptor α+-Cells (PDGFRα+)
Smooth Muscle Cells
Extracellular Matrix
Genetics Factors
Associated Anomalies
Diagnosis
Imaging
Anorectal Manometry
Rectal Biopsy
Differential Diagnosis
Management
Role of Colostomy
Transanal One-Stage Endorectal Pull-Through Operation
Operative Technique
Laparoscopic-Assisted Pull-Through
Postoperative Care
Complications
Anastomotic Leak
Retraction of Pull-Through
Perianal Excoriation
Enterocolitis
Constipation
Soiling
Long-Term Outcome
Conclusion and Future Directions
Cross-References
References
71 Hirschsprung-Associated Enterocolitis
Introduction
Incidence/Diagnosis
Radiologic Imaging
Differential Diagnosis
Pathogenesis
Treatment
Preventive Strategies
Conclusion and Future Directions
Cross-References
References
72 Variants of Hirschsprung Disease
Introduction
Variants of HD
Intestinal Neuronal Dysplasia (IND)
Pathogenesis
Epidemiology
Clinical Presentation
Diagnosis
Management
Outcome
Hypoganglionosis
Pathogenesis
Epidemiology
Clinical Presentation
Diagnosis
Management
Outcome
Internal Anal Sphincter Achalasia
Pathogenesis
Epidemiology
Clinical Presentation
Diagnosis
Management
Outcome
Megacystis Microcolon Intestinal Hypoperistalsis Syndrome
Pathogenesis
Epidemiology
Clinical Presentation
Diagnosis
Histological Findings
Management
Outcome
Summary and Future Directions
Cross-References
References
73 Anorectal Malformations
Introduction
Incidence
Classification
Associated Malformations
Cardiovascular Anomalies
Gastrointestinal Anomalies
Sacral Anomalies
Spinal Cord Anomalies
Vertebral
Genitourinary Anomalies
Gynecologic Anomalies
Anorectal Anatomy and Pathophysiology
Sphincter Mechanism
Sensation and Proprioception
Colonic and Rectosigmoid Motility
Clinical Findings and Initial Management
Limited Posterior Sagittal Anorectoplasty
Colostomy
Management After Colostomy (High-Pressure Distal Colostogram)
Anorectal Reconstruction
Basic Principles
Posterior Sagittal Anorectoplasty for Anomalies in Males
Rectourethral Fistula (Bulbar and Prostatic)
Recto-bladder Neck Fistula
Imperforate Anus Without a Fistula
Rectal Atresia and Stenosis (Hamrick et al. 2012)
Posterior Sagittal Anorectoplasty for Anomalies in Females
Vestibular Fistula
Cloaca
Cloacas with a Common Channel Shorter than 1 cm
Cloacas with a Common Channel Length Between 1 and 3 cm
Cloacas with a Common Channel Longer than 3 cm
General Principles of Postoperative Care
Outcomes
Complications
Continence
Conclusion and Future Directions
Cross-References
References
74 Congenital Pouch Colon
Introduction
Historical Insights
Incidence
Etiology
Pathology
Gross Pathology
Histopathological Findings
Classification
History and Physical Examination
Presentation
Diagnosis
Management
Preoperative Management
Operative Management
Postoperative
Complications
Outcomes
Conclusion and Future Directions
Cross-References
References
75 Congenital Segmental Dilatation of the Intestine
Introduction
Etiology
The Clinical Features and Diagnosis
Criteria
Pathology
Treatment
Prognosis
Conclusion and Future Directions
Cross-References
References
76 Short Bowel Syndrome
Introduction
Definition
Incidence and Etiology
Pathology
Management
Nutritional Therapy
Supplemental Therapy
Surgical Therapy
Complications
Prognosis
Conclusion and Future Directions
Cross-References
References
Part VI: Newborn Surgery: Liver and Biliary Tract
77 Biliary Atresia
Introduction
History
Extrahepatic Pathology
Cystic Biliary Atresia
Etiological Heterogeneity
Biliary Atresia Splenic Malformation (BASM) Syndrome
Intrahepatic Pathology
Epidemiology
Clinical Features
Diagnosis
Blood Tests
Imaging
Liver Biopsy
Cholangiogram
Screening for Biliary Atresia
Management
Surgery: Kasai Portoenterostomy (KPE)
Options and Alternatives
Adjuvant Therapy for Biliary Atresia
Corticosteroids
Ursodeoxycholic Acid (UDCA)
Chinese Herbs
Postoperative Complications
Cholangitis
Portal Hypertension and Esophageal Varices
Ascites
Nutrition and Growth Failure
Developmental Delay and Neurocognitive Function
Outcome and Results
Prognostic Factors
Age at Kasai Portoenterostomy
Surgical Experience
Liver Histology and Biliary Remnant
Conclusion and Future Directions
Cross-References
References
78 Congenital Biliary Dilatation
Introduction
Etiology
Embryology
Anatomy
Classification
Clinical Presentation
Diagnosis
Treatment
Incidental CBD
Open Surgery
Intraoperative Endoscopy
Postoperative Complications and Management
Malignancy
Laparoscopic Treatment
Outcomes
Conclusion and Future Directions
Cross-References
References
Part VII: Newborn Surgery: Anterior Abdominal Wall Defects
79 Omphalocele
Introduction
Embryology and Associated Malformations
Antenatal Diagnosis and Perinatal Management
Neonatal Resuscitation and Investigation
Surgical Management
Primary Closure
Staged Closure
Postoperative Management After Primary or Final Staged Closure
Long-Term Outcomes
Gastrointestinal and Nutritional Problems
Respiratory Problems
Psychological Problems
Conclusion and Future Directions
Cross-References
References
80 Gastroschisis
Introduction
History
Prenatal Considerations
Preoperative Assessment and Preparation
Anesthesia
Operative Procedure
Primary Closure
Staged Closure
Postoperative Care
Complications
Outcome
Conclusion and Future Directions
Cross-References
References
81 Omphalomesenteric Duct Remnants
Introduction
Embryology
Pathology and Clinical Presentation
Meckel´s Diverticulum
Umbilical Mucosal Polyp/Umbilical Cyst
Omphalomesenteric Duct Cyst
Persistent Omphalo-Mesenteric Duct (Patent or Obliterated)
Management
Meckel´s Diverticulectomy
Excision of Omphalomesenteric Duct
Excision of an Umbilical Polyp
Complications
Conclusion and Future Directions
Cross-References
References
82 Conjoined Twins
Introduction
Etiology
Classification
Clinical Presentation
Diagnosis
Treatment
Preoperative Ethical Issues
Preoperative Meetings
Separation
Wall Reconstruction
Complications
Early and Long-Term Results
Conclusion and Future Directions
Cross-References
References
Part VIII: Newborn Surgery: Spina Bifida and Hydrocephalus
83 Spina Bifida and Encephalocele
Introduction
A Historical Perspective
Embryological Development
Classification and Pathogenesis
Brain
Spinal Cord
Other Defects
Spinal Dysraphism
Spina Bifida Occulta
Spina Bifida Cystica: Meningocele
Spina Bifida Cystica: Myelomeningocele
Etiology
Genetics
Dietary Factors
Diabetes and Obesity
Teratogens
Prenatal Diagnosis
Maternal Serum Alphafetoprotein
Imaging
Amniocentesis
Clinical Management
Prenatal Assessment and Counseling
In-Utero Surgery
Postnatal Assessment
Inspection
Motor Assessment
Sensory Assessment
Assessment of Bladder and Bowel Function
Hydrocephalus
Arnold-Chiari Type II Malformation
Skeletal Abnormalities
Investigations
Parental Counseling
General Management, Operative Treatment, and Technique
Postoperative Management and Complications
Long-Term Management and Outcome
Medical Problems
Urinary Incontinence
Hydrocephalus
Chiari II Malformation and Hindbrain Dysfunction
Tethered Cord
Lifestyle Concerns
Intellect
Ambulation
Psychosocial Problems
Encephalocele
Classification
Etiology
Pathology
Clinical Features
Differential Diagnosis
Investigations
Treatment
Operative Treatment and Technique
Postoperative Care and Complication
Prognosis
Conclusion and Future Directions
References
84 Hydrocephalus
Introduction
CSF Physiology
Pathology of Hydrocephalus
Aqueduct Stenosis
Spina Bifida and Chiari Malformation
Dandy-Walker Complex and Posterior Fossa Cysts
Neoplastic Lesions
Posthemorrhagic Hydrocephalus
Postinfective Hydrocephalus (PIH)
External Hydrocephalus
Overproduction of CSF
Symptoms and Signs of Hydrocephalus in the Newborn
Imaging and Investigation of Hydrocephalus in the Newborn
Management of Hydrocephalus in the Newborn
Nonsurgical/Medical Management
Surgical Management
External Ventricular Drainage (EVD)
Subcutaneous Ventricular Access Devices (Reservoir)
Ventriculosubgaleal Shunt
Ventriculoperitoneal Shunt (VPS)
Neuroendoscopy and Endoscopic Third Ventriculostomy (ETV)
Fetal Surgical Therapy
Shunt Operation Technique
Ventriculoperitoneal Shunts
Ventriculoatrial Shunts
Postoperative Care
Conclusions and Future Directions
Cross-References
References
Part IX: Newborn Surgery: Long-Term Outcomes in Newborn Surgery
85 Long-Term Outcomes in Newborn Surgery
Introduction
Rationale of Long-Term Follow-Up Programs
How to Analyze Long-Term Outcomes
Long-Term Outcomes of Specific Newborn Surgical Conditions
Long-Term Outcome in Children with Congenital Diaphragmatic Hernia
Introduction
Chronic Respiratory Tract Disease
Gastroesophageal Reflux Disease
Failure to Thrive and Growth
Skeletal Abnormalities
Neurological Deficits
Health-Related Quality of Life
Long-Term Outcome in Children with Esophageal Atresia
Introduction
Esophageal Morbidity
Respiratory Morbidity
Musculoskeletal Morbidity
Other Issues of Long-Term Morbidity
Quality of Life
Intestinal Failure
Definition and Causes of Intestinal Failure
Incidence and Mortality
Predictors of Intestinal Autonomy
Growth and Development
Intestinal Failure-Associated Liver Disease
Quality of Life
Hirschsprung´s Disease
Rectosigmoid Aganglionosis
Bowel Function
Evolution of Bowel Function with Advancing Age
Anal Sphincter Function
Fecal Incontinence
Constipation
Urinary and Sexual Function
Total Colonic and Panintestinal Aganglionosis
Syndromic Hirschsprung´s Disease
Hirschsprung´s Disease and Cancer
Quality of Life
Anorectal Malformations
Low Anomalies: Long-Term Bowel Function
High Anomalies: Long-Term Bowel Function
Urinary Tract Problems
Genital Anomalies, Fertility, and Sexual Problems
Vertebral Anomalies and Myelodysplasias
Quality of Life in Adult Patients with Anorectal Malformations
Conclusion and Future Directions
Cross-References
References
Index