دانلود کتاب Pediatric Surgery. General Principles and Newborn Surgery

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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Misconceptions and/or Outdated Theories Concerning Normal and Abnormal Embryology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 Pathologies
``Nonsurgical´´ 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 the ``PICO´´ 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