Newborn Surgery-4판

Contents
Preface to the fourth edition
Preface to the third edition
Preface to the second edition
Preface to the first edition
Author
Part 1 General
Chapter 1 Embryology of malformations
Introduction
General remarks on embryology and the embryology of malformations
Definition of the term “malformation”
Etiology of congenital malformations
Environmental factors
Genetic factors
Embryology and animal models
Surgical models
Chemical models
Genetic models
Viral models
Embryology of malformations
Examples of pathological embryology
Embryology of foregut malformations
Development of the diaphragm
Timing of diaphragmatic defect appearance
Location of diaphragmatic defect
Why lungs are hypoplastic
Development of the embryonic cloaca (EC)
General remarks on the development of the EC
Literature on “normal cloacal development”
Own observations
Normal “cloacal” embryology (rat)
Abnormal “cloacal” embryology (SD-mouse)
Critical remarks on the terminology of the “cloaca”
Hypospadias
Conclusion
References
Chapter 2 Transition to extrauterine life
Introduction
Fetal Circulation and Its Transition at Birth
Closure of the ductus venosus
Closure of the foramen ovale
Fall in PVR
Closure of the DA
Perspectives
The Respiratory System
Fetal breathing movements (FBM)
Control of fetal breathing movements
Peripheral chemoreceptor function in utero
Pharmacological control
Lung growth associated to FBM
Changes at birth
Postnatal breathing
Conclusions and Further Directions
References
Chapter 3 Clinical anatomy of the newborn
Introduction
Growth and proportions
Cardiovascular system
Circulatory changes after birth
The heart
Umbilical vessels
Arteries
Central veins
Respiratory system
Upper airway
Trachea and bronchial tree
Thorax and mechanics of breathing
Abdominal Wall and Gastrointestinal Tract
Abdominal and pelvic cavities
Gastrointestinal tract
Liver 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
Acknowledgments
References
Chapter 4 The epidemiology of birth defects
Birth defects helped define Pediatric surgery
Birth defects remain potent causes of infant mortality and long-term disability
Birth defect epidemiology and teratology emerged from outbreak Investigation
Causation of birth defects remains often complex and uncertain
Birth defects appear to arise typically (but not exclusively) in the first trimester
Classification of birth defects for epidemiological purposes
Counting of birth defects is affected by the definition of stillbirth
Prenatal diagnosis: The greatest challenge to birth defect epidemiology?
Pediatric surgeons often focus on their institutional series of birth defects
A “life-course” approach to birth defects
References
Chapter 5 Prenatal diagnosis of surgical conditions
Introduction
Ultrasound
Magnetic resonance imaging
Amniocentesis
Chorionic villus sampling
Biochemical markers
Percutaneous umbilical blood sampling
Fetal Cells in the Maternal Circulation
Prenatal diagnosis of specific surgical lesions
Neck masses
Sacrococcygeal teratoma
Congenital chest lesions
CCAM and bronchopulmonary sequestration
Congenital diaphragmatic hernia
Gastrointestinal lesions
Esophageal and bowel atresias
Abdominal wall defects
Prenatal diagnosis of renal anomalies
Upper urinary tract obstruction
Lower urinary tract obstruction
Myelomeningocele
Conclusions
References
Chapter 6 Fetal counseling for surgical malformations
Introduction
Congenital malformation
Prenatal diagnosis
US examination
Minimally invasive techniques
Cell-free fetal DNA
Preimplantation genetic diagnosis
Invasive diagnostic tests
Amniocentesis
Chorionic villous sampling
Prenatal maternal serum screening
Fetal blood sampling
Fetal surgery
Genetic diagnoses
Future developments
Specific surgical conditions
Congenital diaphragmatic hernia
Cystic lung lesions
Abdominal wall defects
Exomphalos
Gastroschisis
Tracheoesophageal fistula and esophageal atresia
Gastrointestinal lesions
Abdominal cysts
Sacrococcygeal teratoma
Renal anomalies
Upper urinary tract obstruction
Lower urinary tract obstruction
Conclusion
References
Further Reading
Chapter 7 Fetal and birth trauma
Fetal trauma
Birth trauma
Types of birth trauma
Head injuries
Spinal cord injuries
Peripheral nerve injuries
Intra-abdominal injuries
Bony injuries
Trauma to the genitalia
References
Chapter 8 Transport of the surgical neonate
Introduction
Prenatal transfer
Pretransfer management
Airway management
Temperature regulation
Circulation
Documentation
Transport team
Transport vehicles
Transport incubators
Transport procedure
Receiving center
Special considerations
Gastroschisis
Omphalocele
Pierre Robin syndrome
Choanal atresia
Myelomeningocele
Bladder exstrophy
Cloacal exstrophy
Esophageal atresia with tracheoesophageal fistula
Congenital diaphragmatic hernia
Intestinal obstruction
Necrotizing enterocolitis
Conclusion
References
Chapter 9 Specific risks for the preterm infant
Introduction
General
Respiratory
Respiratory distress syndrome
Pneumothorax
Pulmonary hemorrhage
Bronchopulmonary dysplasia and chronic lung disease
Apnea of prematurity
Cardiovascular
Blood pressure
Patent ductus arteriosus
Congenital heart disease
Central Nervous System
Intraventricular hemorrhage
Cystic periventricular leukomalacia
Retinopathy of prematurity
Gastrointestinal Tract
Feeding and nutrition
Necrotizing enterocolitis
Spontaneous bowel perforation
Inguinal hernia
Umbilical hernia
Infection
Other
Hyperbilirubinemia
Electrolytes
Anemia
References
Chapter 10 Preoperative assessment
Introduction
History and Physical Examination
Maintenance of Body Temperature
Respiratory Function
Cardiovascular Status
Metabolic Status
Acid–base balance
Hypoglycemia
Hypocalcemia
Hypomagnesemia
Hyperbilirubinemia
Coagulation Abnormalities
Laboratory Investigations
Vascular Access
Fluid and Electrolytes, and Metabolic Responses
Renal Function, Urine Volume, and Urine Concentration in the Newborn
Serum Electrolytes and Metabolic Responses in Neonatal Surgical Patients
Preoperative Management of Various Surgical Neonatal Conditions
Fluid Management Program
References
Chapter 11 Anesthesia
Preoperative preparation and evaluation
History
Physical examination
Laboratory investigations
Premedication
Transfer to the operating theater
Operating theater and anesthetic equipment
Breathing systems
Laryngoscopes
Ventilators
Monitoring equipment
Choice of anesthetic agent and technique
Inhalation agents
Halothane
Isoflurane
Enflurane
Desflurane
Sevoflurane
Nitrous oxide
Intravenous agents
Thiopentone
Propofol
Ketamine
Neuromuscular blocking agents
Succinylcholine
Nondepolarizing muscle relaxants
Atracurium and vecuronium
Mivacurium
Rocuronium and sugammadex
Analgesia
Regional anesthesia
Induction of anesthesia and endotracheal intubation
Maintenance of anesthesia
Reversal and extubation
Monitoring
Respiration
Cardiovascular function
Fluid balance
Anesthesia for specific surgical conditions
Esophageal atresia
Congenital diaphragmatic hernia
Intestinal obstruction
Exomphalos and gastroschisis
Congenital lobar emphysema
Myelomeningocele
Special considerations for the premature infant
References
Chapter 12 Postoperative management of the surgical neonate
Introduction
Respiratory management
Ventilatory support
Positive-pressure ventilation
Patient-triggered ventilation and synchronized ventilation
High-frequency oscillatory ventilation
Lung-protective ventilation
Setting ventilator parameters
Respiratory monitoring
Care of the intubated patient in the PICU
Management of the newborn with hypoxic respiratory failure
Nitric oxide
Surfactant replacement therapy
Weaning from ventilation
Postoperative sedation and analgesia
Hemodynamic support in the newborn
Vasoactive medication therapy in neonates
Fluid management and renal function
Temperature regulation and metabolism
References
Chapter 13 Fluid and electrolyte balance in the newborn
Introduction
Water distribution in the fetus and the newborn infant
Functional adjustments to postnatal life
Renal blood flow
Glomerular filtration rate
Water homeostasis
Insensible water loss
Sweating
Transepidermal water loss
Respiratory water loss
Fluid and electrolyte management
Sodium regulation
Renal response to antidiuretic hormone
Sodium balance
Potassium balance
Causes of hypokalemia
Acid–base balance
Metabolic acidosis
Causes of metabolic acidosis
Causes of metabolic alkalosis
Glucose homeostasis
Hypoglycemia
Hyperglycemia
Calcium homeostasis
Hypocalcemia
Causes of hypocalcemia
Clinical manifestations
Management
Perioperative management
Preoperative fluid and electrolyte problems in the neonate
Intraoperative management
Postoperative management
Fluid and electrolyte balance in septic shock
Clinical manifestations
Management
Acute renal failure
Conclusion
References
Chapter 14 Nutrition
Introduction
Growth monitoring
Body composition and energy requirements of the neonate
Parenteral nutrition
Indications
Route of administration
Components of PN
Fluid requirements
Energy sources
Amino acids
Minerals, vitamins, and trace elements
Complications of PN
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
Chapter 15 Access for enteral nutrition
Introduction
Indications
Nasogastric feeding tube versus gastrostomy
Nasogastric decompression tube versus gastrostomy
Gastrostomy in select neonatal surgical pathology
Esophageal abnormalities
Duodenal obstruction
Major abdominal wall defects
Short-gut syndrome
Other surgical pathology
“Nonsurgical” pathology
Advantages and disadvantages of gastrostomies
Technique
Stamm gastrostomy
Percutaneous endoscopic gastrostomy
Minimally invasive gastrostomies
Laparoscopically assisted gastrostomies
Laparoscopic-assisted percutaneous endoscopic approach
Select complications of gastrostomies
Complications related to operative technique
Separation of stomach from abdominal wall
Wound separation, dehiscence, and ventral hernia
Hemorrhage
Infection
Injury to the posterior wall of the stomach
Injury to other organs
Gastrocolic fistula
Complications related to care of stoma
Skin irritation and moniliasis
Tube plugging
Administration of improper feedings
Delay in the reintroduction of a dislodged catheter
Trauma during reinsertion of catheter
Complications related to catheters
Granulation tissue
Leakage
Internal migration
External migration
Perforation of esophagus and small bowel
Persistent gastrocutaneous fistula
Prevention
Conclusion
References
Chapter 16 Stomas of small and large intestine
Introduction
Most common errors
Stoma mislocation
Prolapse
Stenosis
Retraction
Stomas in cloacal exstrophy
Ileostomies
Colostomy closure
References
Chapter 17 Vascular access in the newborn
Introduction
Arterial cannulation
Maintenance
Complications
Umbilical artery catheterization
Access technique
Alternative method for umbilical artery cannulation
Radial artery cannulation
Access technique
Pedal arterial cannulation
Axillary and femoral arterial cannulation
Venous cannulation
Maintenance
Complications
Peripherally inserted central catheter
Access technique
Umbilical vein catheter
Access technique
Percutaneous cannulation
Access technique
Peripheral vein cut-down
Access technique
Central vein cut-down
Intraosseous devices
Access technique
Conclusion
References
Chapter 18 Radiology in the newborn
Introduction
Conventional radiography
Mobile examinations
Fluoroscopic examinations
Ultrasonography
Nuclear medicine
Computed tomography
Magnetic resonance imaging
Interventional techniques
Conclusion
References
Chapter 19 Immune system of the newborn
Introduction
Clinical outcomes in neonatal sepsis and inflammation
Neonatal innate immunity
Monocytes
Neutrophils
The inflammatory response syndromes
Toll-like receptors
Mucosal immunity, human milk, and necrotizing enterocolitis
Neonatal adaptive immune response
T-cell responses
Lymphocyte phenotype
B-cell responses
Immunoglobulins
Cytokines
Immunomodulation
Colony stimulating factors and other immunomodulatory agents
Prebiotics and probiotics
Immune deficiency diseases in the newborn
Neonatal autoinflammatory disorders
Conclusion
References
Chapter 20 Neonatal sepsis
Introduction
Definition and classifications
Epidemiology
Risk factors
Pathophysiology, pathogens, and biomarkers
Diagnosis and workup
Cultures
Lumbar puncture
Radiographs
Hematologic evaluation
Acute phase reactants and biomarkers
Management
Prevention strategies
Treatment
New therapies and preventive strategies
Multidrug resistance and opportunistic infections
Viral sepsis
Outcomes
Conclusions
References
Chapter 21 Hematological problems in the neonate
Introduction
Platelets
Increased destruction
Autoimmune thrombocytopenia
Neonatal alloimmune thrombocytopenia
Peripheral consumption
Hypersplenism
Kasabach–merritt phenomenon
Thrombosis
Type 2B von Willebrand disease
Decreased production
Inherited bone marrow failure syndromes
Fanconi anemia
Inherited platelet disorders
Bernard–Soulier syndrome
Pseudo-vWD
Type 2B vWD
WAS variants (X-Linked thrombocytopenia)
Congenital infections
Neonatal infections
Necrotizing enterocolitis
Miscellaneous conditions
Developmental hemostasis
Inherited bleeding disorders
Von Willebrand disease
Other coagulation factor deficiencies
Acquired bleeding disorders
Hemophagocytic Lymphohistiocytosis
Vitamin K
Vitamin K deficiency bleeding
Liver disease
Extracorporeal life support
Congenital heart disease
Thrombotic states
Acquired states
Central venous catheter devices
Renal artery and vein thrombosis
Acquired protein C/S deficiency
Acquired antithrombin deficiency
Miscellaneous conditions
Inherited thrombotic states
Protein C and protein S deficiency
Antithrombin deficiency
Other inherited thrombophilias
Management
Anemia
Acquired anemia
Alloimmune hemolytic anemia
Autoimmune hemolytic anemia
Infantile pyknocytosis
Blood loss
Anemia of prematurity
Approach to management
Inherited anemia
Red cell membrane defects
Hemoglobinopathies
Red cell enzyme deficiencies
Diamond–Blackfan anemia
Miscellaneous
Polycythemia in the neonatal period
Neutrophils
Alloimmune neutropenia
Autoimmune neutropenia
Autoimmune neutropenia of infancy
Miscellaneous
Severe congenital neutropenia
Shwachman–Diamond syndrome
Hematological disorders in the neonate with down syndrome
References
Chapter 22 Genetics in neonatal surgical practice
Nature and structure of a gene
Decoding the information in DNA
Chromosomes and cell division
Chromosome anomalies and analysis
Genomic array analysis
Patterns of inheritance
Autosomal dominant inheritance
Autosomal recessive inheritance
X-linked recessive inheritance
Polygenic inheritance
Other forms of inheritance
Molecular genetic analysis for single-gene disorders
Clinical genetic approach to diagnosis of malformation syndromes
Definitions
Approach to diagnosis
Further reading
Glossary
Chapter 23 Ethical considerations in newborn surgery
Introduction
Guidelines for Ethical Decision Making
The Best-Interests Standard Applied to Neonatal Surgery
Role of Culture in Decision Making
Summary
References
Chapter 24 Patient safety
Introduction
System thinking
Safety concerns specific to surgery
Safety concerns specific to newborn surgery
Size
Anatomy
Immature organ systems
Acuity of problems
Comorbidity
Healthcare system limitations
Measures to Improve Safety
Checklists
Adapting the checklist for newborn surgery
Reporting and learning
Nontechnical skills
Situational Awareness
Decision Making
Communication and Teamwork
Leadership
Summary and conclusions
References
Chapter 25 Minimally invasive neonatal surgery
Introduction
Thoracoscopy
Physiology and anesthetic considerations
Diagnostic thoracoscopy and biopsy or resection of pulmonary lesions
Mediastinum
Closure of patent ductus arteriosus and aortopexy
Esophageal atresia
Congenital diaphragmatic hernia
Laparoscopy
Physiology of pneumoperitoneum
Abdominal access
Pyloromyotomy
Gastrostomy
Fundoplication
Inguinal hernia
Endorectal pull-through
Malrotation
Duodenal atresia
Biliary pathology
Pelvic pathology
Future directions
Conclusion
References
Chapter 26 Fetal surgery
Introduction
General Principles
Fetal Diagnosis
Fetal Access
Anomalies Amenable to Fetal Surgery
Congenital diaphragmatic hernia
Tumors
Congenital pulmonary airway malformation
Sacrococcygeal teratoma
Abnormalities of Twin Gestations
Twin–twin transfusion syndrome
Twin reversed arterial perfusion
Myelomeningocele
The Future
Stem cells and gene therapy
Summary
References
Chapter 27 Tissue engineering and stem cell research
Introduction
Stem cells
ES cells
Somatic cell nuclear transfer
Induced pluripotent SCs
Fetal SCs
Adult SCs
Scaffolds
Naturally derived materials
Synthetic materials
Natural acellular matrix
Cell-derived ECM scaffolds
Tissue engineering
Conclusion
References
Chapter 28 Surgical aspects of HIV infection
Introduction
Infections
GI tract disease
Intra-abdominal pathology
Perineal disease
Malignancy
Medical aspects of pediatric HIV infection and their effect on surgery
Outcomes of HIV-infected children undergoing surgery
Factors influencing postsurgical complications
HIV-exposed but uninfected children
Conclusion
References
Chapter 29 Liver transplantation
Introduction
History
Indications
Assessment
Surgical technique
Split-liver transplantation and living donation
Medical management
Immunosuppression
Postoperative care
Anti-infection agents
Surgical complications
Medical follow-up and late complications
Retransplantation
Long-term survival and quality of life
Adolescence
Conclusion
References
Part 2 Head and neck
Chapter 30 Choanal atresia
Introduction
Epidemiology
Embryology and Theories of Causation
Clinical Presentation
Radiologic Investigations
Management
Endoscopic resection
Transpalatal resection
Adjuncts to surgery
Stents
Mitomycin C
Conclusions
References
Chapter 31 Pierre Robin sequence
Introduction
Etiology, Pathophysiology
Genetics, incidence
Clinical features (new classification 2015)
Airway management
Nasopharyngeal tube
Endotracheal tube
Tongue–lip adhesion/glossopexy
Tracheostomy
Distraction osteogenesis of the mandible
Tongue positioning and stimulation plate
Noninvasive ventilation
Nutritional management
Cleft palate
Micrognathia/Retrognathia
Skeletal anomalies
Ear problems
Cardiovascular anomalies
Ocular anomalies
Nasal obstruction
Conclusion and future directions
References
Chapter 32 Macroglossia
Introduction
Etiology
Pathology
Presentation
Diagnosis
Management
Surgery
References
Chapter 33 Tracheostomy in infants
Introduction
Indications for tracheostomy
Preoperative Evaluation
Technique
Perioperative Management
Home instruction and care
Complications
Special Situations
Decannulation
References
Chapter 34 Congenital cysts and sinuses of the neck
Introduction
Embryology
Incidence
Branchial anomalies
Thyroglossal duct cysts and sinuses
Congenital midline cervical clefts
Dermoid cysts
Preauricular sinuses and cysts
Ranula
Cystic hygroma (cystic lymphangioma)
Torticollis
References
Chapter 35 Stridor in infants
Introduction
Supraglottic Disorders
Laryngomalacia
Laryngeal cysts
Glottic and subglottic disorders
Vocal fold immobility
Glottic stenosis
Subglottic stenosis
Tracheal Disorders
Tracheomalacia
Vascular anomalies
Complete tracheal rings
Tracheal atresia and agenesis
References
Part 3 Chest
Chapter 36 Congenital thoracic deformities
Introduction
Ectopia cordis
Thoracic ectopia cordis
Thoracoabdominal ectopia cordis (Cantrell pentalogy)
Bifid sternum
Pectus excavatum
Pectus carinatum
Poland syndrome
Thoracic deformities in skeletal disorders
Congenital asphyxiating thoracic dystrophy (Jeune disease)
Spondylothoracic dysplasia (Jarcho–Levin syndrome)
References
Chapter 37 Mediastinal masses in the newborn
Differential diagnosis
Diagnosis
Anterior mediastinum
Middle mediastinum
Posterior mediastinum
Anesthetic management of infants with a mediastinal mass
Operative technique for removal of mediastinal neuroblastoma
Further Reading
Chapter 38 Congenital airway malformations
Introduction
Assessment
Medical history
Signs and symptoms
Diagnostic studies
Endoscopy
Flexible bronchoscopy
Microlaryngoscopy and rigid bronchoscopy
Esophagoduodenoscopy
Congenital laryngeal anomalies
Laryngomalacia
Laryngeal webs
Subglottic stenosis
Subglottic hemangioma
Vocal cord paralysis
Posterior laryngeal cleft
Laryngeal atresia
Congenital high airway obstruction syndrome
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
Tracheobronchial–biliary fistula
Bronchogenic cyst
Bronchial atresia and bronchial lobar agenesis
Bronchial atresia
Bronchial lobar agenesis
Bronchial stenosis
References
Chapter 39 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, right descending aorta, and atretic right aortic arch
Left aortic arch and left ligamentum arteriosum with retroesophageal right subclavian artery
Left aortic arch with anomalous origin of the innominate artery
Cervical aorta
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
Aortic angiography and cardiac catheterization
Bronchoscopy
Indication for intervention
Medical therapy
Surgical therapy
Division of a double aortic arch
Right aortic arch
Pulmonary artery sling
Left aortic arch with anomalous origin of the innominate artery
Results
Traditional thoracotomy approach
Conclusion and future directions
References
Chapter 40 Pulmonary air leaks
Introduction
Pulmonary interstitial emphysema
Presentation and diagnosis
Treatment
Prognosis
Pneumomediastinum
Pneumothorax
Presentation and diagnosis
Treatment
Prognosis
Pneumopericardium
References
Chapter 41 Chylothorax and other pleural effusions in neonates
Introduction
Pathophysiology
Anatomy
Embryology
Etiology
Congenital chylothorax
Congenital pleural effusion with associated diseases
Acquired pleural effusions
Iatrogenic acquired pleural effusions
Noniatrogenic acquired pleural effusions
Clinical symptoms
Diagnosis
Radiology
Laboratory testing
Management
General management principles
Nonoperative management
Operative management
Thoracic duct ligation
Pleurodesis and parietal pleurectomy
Pleuroperitoneal shunts
Outcome
Fetal pleural effusion
Other pleural effusions
Hemothorax
Empyema
References
Chapter 42 Congenital malformations of the lung
Introduction
Embryology
Anatomy
Congenital lobar emphysema
Presentation and diagnosis
Treatment
Polyalveolar lobe
Congenital pulmonary airway malformations
Presentation and diagnosis
Treatment
Pulmonary sequestration
Presentation and diagnosis
Treatment
Bronchogenic cysts
Presentation and diagnosis
Treatment
Pulmonary hypoplasia, aplasia, and agenesis
Lung surgery in newborns
Lobectomy
References
Chapter 43 Congenital diaphragmatic hernia
Introduction
Embryogenesis
Pathophysiology
Diagnosis
Treatment
Preoperative treatment
Operative repair
Postoperative treatment
Prenatal treatment
Prognosis
Long-term outcome
Congenital eventration of the diaphragm
Clinical characteristics
Diagnosis
Management
Operative repair
Outcome
References
Chapter 44 Extracorporeal life support for neonatal cardiorespiratory failure
Introduction
Selection criteria for neonatal ECMO
Gestational age
Birth weight
Bleeding and coagulopathy
Preexisting intracranial hemorrhage
Reversible disease process
Coexisting anomalies
Bridge to diagnosis
Failure of medical management
Clinical management of neonates on ECMO
Venovenous versus venoarterial ECMO
Patient preparation and cannulation
The ECMO circuit
Priming the circuit
Pump management
Oxygenator management
Fluid management
Respiratory management on ECMO
Medical management
Coagulation management
Complications on ECMO
Mechanical complications
Neurologic complications
Renal complications
Weaning from ECMO
ECMO in infants with CDH
Outcome and follow-up of neonates treated with ECMO
Mortality
Feeding and growth sequelae
Respiratory sequelae
Neurodevelopmental sequelae
Summary
references
Chapter 45 Bronchoscopy in the newborn
Introduction
Anesthesia for pediatric bronchoscopy
Rigid bronchoscopy
Indications for rigid bronchoscopy
Instrumentation
Technique of rigid laryngobronchoscopy
Advantages of rigid bronchoscopy
Complications of rigid bronchoscopy
Flexible bronchoscopy
Indications for flexible bronchoscopy
Technique of flexible bronchoscopy
Common pitfalls for the unwary flexible bronchoscopist
Concurrent lesions
Difficult nasal passage
Pharyngeal hypotonia
Complications of flexible bronchoscopy
BAL in children
Conclusion
References
Part 4 Esophagus
Chapter 46 Esophageal atresia and tracheo-esophageal fistula
Introduction
History
Classification
Prognosis
Epidemiology
Genetics
Animal models
Embryology
Associated anomalies
Antenatal presentation
Clinical presentation and diagnosis
Surgical management
Long-gap EA with distal TEF
Right-sided aortic arch
Premature infant with RDS
Postoperative care
Surgical management of isolated (“pure”) EA
H-type TEF
EA–TEF—complications and special considerations
Anastomotic leak
Gastroesophageal reflux
Anastomotic stricture
Tracheomalacia
Recurrent TEF
EA–TEF ADVANCES
QoL and long-term outcome
References
Chapter 47 Congenital esophageal stenosis
Introduction
Pathology
Epidemiology/etiology
Symptoms
Diagnosis
Treatment
Nonsurgical treatment
Surgery
Management of complications following treatment
Prognosis
References
Chapter 48 Esophageal duplication cysts
Introduction
Etiology
Pathology
Presentation
Diagnosis
Management
Thoracoscopic resection
Thoracotomy and resection
Cervical and suprasternal cysts
Surgical complications
Long-term outcome
References
Chapter 49 Esophageal perforation in the newborn
Introduction
Classification and Etiology
Diagnosis and Clinical Manifestations
Management
Conclusion
References
Chapter 50 Gastroesophageal reflux in the neonate and small infant
Introduction
Anatomy
Innervation
The Sphincters
Peristalsis
Causes of Reflux
The Development of the Esophagus in the Neonate and Small Infant
Reflux in the newborn
Symptoms of Pathological Reflux in Newborns and Infants
Diagnosis of reflux
Radiological investigation of esophageal passage
Twenty-four-hour pH monitoring
Combined multiple intraluminal impedance/ pH monitoring
Manometry
Endoscopy and histology
Nuclear medicine investigations
Conservative therapy of reflux disease
Surgical Therapy
Special problems of reflux in the newborn and Infant
Laryngopharyngeal reflux
Reflux-associated infections of the respiratory tract
Reflux and apnea syndrome
Hiatal hernia
Esophageal atresia and diaphragmatic hernia
Reflux in Neurologically impaired Children
Conclusion
References
Part 5 Gastrointestinal
Chapter 51 Pyloric atresia and prepyloric antral diaphragm
Pyloric atresia
Introduction
Etiology
Pathology
History and physical examination
Presentation
Diagnosis
Management
Preoperative management
Operative technique
Laparotomy
Identification of the site of obstruction
Pyloroplasty
Closure of abdomen
Laparoscopic approach
Postoperative management
Complications
Long-term results
Prepyloric antral diaphragm
Introduction and etiology
Pathology
History, presentation, and diagnosis
Management
Complications and long-term results
References
Chapter 52 Infantile hypertrophic pyloric stenosis
Introduction
Etiology
Extrinsic factors
Genetics
Abnormalities of hormonal control
Abnormalities of pyloric innervation
Ganglion cells
Cholinergic and adrenergic innervations
Nitrergic innervation
Synapse formation
Nerve-supporting cells
Abnormalities of the intestinal pacemaker system (ICC)
Abnormalities of ECM proteins
Abnormalities of SMCs
Abnormalities of growth factors
Pathology
Clinical features
Diagnosis
Management
Preoperative management
Operation
Incision
Procedure
Laparoscopic pyloromyotomy
Postoperative care
Complications
Conservative Therapy
References
Chapter 53 Gastric volvulus
Introduction
Etiology
Clinical cases
Case 1
Case 2
Case 3
Pathogenesis
Clinical features
Diagnosis
Treatment
Complications
References
Chapter 54 Gastric perforation
Introduction
Etiology
Clinical Presentation
Diagnosis
Differential diagnosis
Perioperative care
Surgical technique
Conclusion
Future directions
References
Chapter 55 Duodenal obstruction
Introduction
Etiology
Pathophysiology
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
References
Chapter 56 Intestinal malrotation
Definition
Epidemiology
Pathogenesis
Clinical Presentation
Diagnosis
Treatment
Surgery
Open Ladd’s procedure
Laparoscopic Ladd’s procedure
Complications
Controversies
Reference
Further Reading
Chapter 57 Congenital hyperinsulinism
Introduction
Etiology
Pathology
Clinical Presentation
Diagnosis
Management
Medical management
Surgical management
Incision
Exposure
95% Pancreatectomy
Resection of Focal Disease
Postoperative Care
Complications
Long-term results
References
Chapter 58 Jejunoileal atresia and stenosis
Introduction
Etiology
Epidemiology
Pathology
Prenatal Diagnosis
Clinical features
Guidelines for Management
Treatment
Sterilization of skin and draping
Incision
Exploration
Resection
Anastomosis
Gastrostomy/enteral decompression and early enteral feeding
Closure of abdominal wound
Other surgical maneuvers
Postoperative Care
Outcomes
References
Chapter 59 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
References
Chapter 60 Meconium ileus
Definition
Historical data
Incidence
Pathogenesis
Histopathology
Clinical picture
Prenatal US and radiologic pattern
Diagnostic criteria and differential diagnosis
Medical and surgical treatment
Prognosis
References
Chapter 61 Meconium peritonitis
Introduction
Etiology
Pathology
Symptomatology and diagnosis
Treatment
Surgical treatment
Operative treatment
Complications
References
Chapter 62 Duplications of the alimentary tract
Introduction
Embryology
Partial twinning
Split notochord
Embryonic diverticula and recanalization defects
Pathology
Incidence
Esophageal duplication
Treatment
Thoracoabdominal duplication
Treatment
Gastric duplication
Treatment
Pyloric duplication
Treatment
Duodenal duplication
Treatment
Duplication of the small bowel
Treatment
Colonic duplication
Treatment
Rectal duplication
Treatment
References
Chapter 63 Mesenteric and omental cysts
Introduction
Spectrum and Morphology
Clinical characteristics
Diagnosis
Differential diagnosis
Treatment
Outcome
References
Chapter 64 Neonatal ascites
Introduction
Urinary ascites
Chylous ascites
Bile ascites
References
Chapter 65 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
References
Chapter 66 Spontaneous intestinal perforation
Introduction
Epidemiology
Prenatal
Postnatal
Pathology and pathogenesis
Clinical features and diagnosis
Treatment
Primary peritoneal drainage
Laparotomy
Outcome
References
Chapter 67 Hirschsprung’s disease
Introduction
Etiology
Neural crest cell migration
Genetic factors
Pathophysiology
Cholinergic hyperinnervation
Adrenergic innervation
Nitrergic innervation
Interstitial cells of Cajal
Platelet-derived growth factor receptor α+-cells
Enteroendocrine cells
Smooth muscle
Extracellular matrix
Pathology
Diagnosis
Clinical features
Radiological diagnosis
Anorectal manometry
Rectal biopsy
Differential diagnosis
Management
Transanal one-stage endorectal pull-through operation
Preoperative management
Operative technique
Laparoscopic-assisted pull-through
Complications
Long-term outcome
Future therapies
References
Chapter 68 Anorectal anomalies
Anorectal anomalies
Frequency
Classification
Clinical features and diagnosis
Male defects
Perineal fistula
Rectourethral bulbar and rectourethral prostatic fistulas
Rectovesical (bladder neck) fistula
Imperforate anus without fistula
Rectal atresia and stenosis
Female defects
Cutaneous (perineal) fistula
Vestibular fistula
Vaginal fistula
Imperforate anus without fistula
Rectal atresia and stenosis
Persistent cloaca
Preoperative care
Colostomy
Surgical technique for colostomy
Distal colostogram
Definitive repair
Minimal posterior sagittal anoplasty
Limited posterior sagittal anorectoplasty
Posterior sagittal anorectoplasty
Posterior sagittal anorectoplasty and laparoscopy or laparotomy (bladder neck fistula)
Two weeks postoperatively
Results
Bowel management for fecal incontinence
References
Chapter 69 Congenital pouch colon
Introduction
Historical insights
Incidence
Etiology
Pathology
Gross pathology
Histological pathology
Classification
History and physical examination
Presentation
Diagnosis
Management
Preoperative management
Operative management
Postoperative management
Complications
Outcomes
Conclusion and future directions
References
Chapter 70 Congenital segmental dilatation of the intestine
Introduction
Etiology
Clinical features and diagnosis
Pathology
Treatment
Prognosis
Acknowledgments
References
Chapter 71 Intussusception
Prenatal intussusception
Neonatal intussusception
The rotavirus vaccine
Clinical assessment
Nonoperative treatment
Indication for enema
Preparation for gas enema
Technique of enema reduction
Cessation of gas enema
Delayed gas enema after partial reduction
Indications for surgery
Preparation for surgery
Surgical technique
Approach
Manual reduction
Check for a pathological lead point
Technique of resection
Closure
Postoperative instructions
References
Chapter 72 Inguinal hernia
Introduction
Etiology
Epidemiology
Associated conditions
Clinical features
Management of inguinal hernia
Anesthesia
Open inguinal herniotomy
Laparoscopic inguinal hernia repair
Contralateral Exploration
Ipsilateral orchidopexy
Postoperative management
Complications of inguinal herniotomy
Incarcerated inguinal hernia
Diagnosis of incarcerated inguinal hernia
Differential diagnosis of incarcerated hernia
Encysted hydrocoele of the cord
Inguinal lymphadenitis
Torsion of the testes
Management of incarcerated hernia
Surgery for obstructed, irreducible inguinal hernia
Spontaneous reduction with no ischemic bowel
Spontaneous reduction with ischemic bowel
No spontaneous reduction with or without ischemic bowel
References
Chapter 73 Short bowel syndrome and surgical techniques for the baby with short intestines
Introduction
Surgical Tactics in Situations Requiring Extensive Intestinal Resection
Surgical techniques in SBS patients
Inefficient peristalsis
Tapering
Tapering and lengthening
Inadequate intestinal transit time
Antiperistaltic small intestinal segment
Colonic interposition
Intestinal valves and pouches
Results
Conclusion
References
Chapter 74 Megacystis microcolon intestinal hypoperistalsis syndrome
Introduction
Pathogenesis
Prenatal diagnosis
Clinical presentation
Radiological findings
Surgical or autopsy findings
Histological findings
Outcome
References
Part 6 Liver and biliary tract
Chapter 75 Biliary atresia
Introduction
History
Etiological heterogeneity
BASM syndrome
Pathology of BA
Cellular kinetics and inflammation in BA
Viruses and BA
Environmental issues
Epidemiology
Clinical features
Laboratory Findings
Ultrasonography
Miscellaneous diagnostic techniques
Surgery—Kasai portoenterostomy
Cholangiogram
Mobilization and eversion of liver
Portal dissection
Roux loop and portoenterostomy
Caveats to Surgery
Postoperative management
Outcome following portoenterostomy
Effect of age at surgery
Complications
Cholangitis
Portal hypertension
Role of laparoscopic Kasai portoenterostomy
Liver transplantation in BA
References
Chapter 76 Congenital biliary dilatation
Introduction
Etiology
Classification
Clinical signs and symptoms
Diagnosis
Surgery
Choice of operative procedures
Timing of surgery
Complete excision
Mucosectomy/biliary reconstruction
Associated anomalies requiring treatment
IBD dilatation
Anomalies of the pancreatic duct and common channel
Intraoperative endoscopy
Laparoscopic surgery
Postoperative complications and management
References
Chapter 77 Hepatic cysts and abscesses
Introduction
Simple hepatic cyst
Differential diagnosis of complex cystic structures in the liver
Presentation
Diagnosis
Treatment
Prognosis
Polycystic liver disease
Hepatic abscesses
Incidence and etiology
Presentation and diagnosis
Radiographic evaluation
Treatment
Amoebic liver abscess
References
Part 7 Anterior abdominal wall defects
Chapter 78 Omphalocele and gastroschisis
Introduction
Embryology and etiology
Incidence
Associated anomalies
Function of exteriorized viscera
Prenatal diagnosis and management
Newborn management
Surgical management
Postoperative management
Long-term outcome
References
Chapter 79 Omphalomesenteric duct remnants
Introduction
Etiology
Pathology
History, Presentation, and Diagnosis
Persistent omphalomesenteric duct
Omphalomesenteric duct cyst
Meckel diverticulum
Umbilical polyp
Operative management
Excision of a patent omphalomesenteric duct
Meckel diverticulectomy
Excision of an umbilical polyp
Complications
References
Chapter 80 Bladder exstrophy: Considerations and management of the newborn patient
Introduction
Incidence and Inheritance
Embryology
Anatomic considerations
Musculoskeletal defects
Abdominal wall defects
Anorectal defects
Male genital defects
Female genital defects
Urinary defects
Prenatal diagnosis and management
Delivery Room and Nursery Care
Primary bladder closure
Pelvic osteotomy
Bladder, posterior urethral, and abdominal wall closure
Combined bladder closure and epispadias repair
Conclusion
References
Chapter 81 Cloacal exstrophy
Introduction
Embryology
Epidemiology, Genetics, and Environmental Risk Factors
Prenatal Diagnosis
Associated Anomalies
Immediate Postnatal Management
Gender Assignment
Postnatal surgical management
Postoperative Management
Urologic Management
GI Outcomes
Sexual Function and Quality of Life
Conclusions
References
Chapter 82 Prune belly syndrome
Introduction
Etiology
Pathology
Abdominal wall
Urinary tract
Kidney
Ureter
Bladder
Urethra
Testes
Associated anomalies
Antenatal diagnosis
Postnatal Assessment and Investigations
Management
Circumcision
Orchidopexies
Urinary tract reconstruction
Abdominoplasty
Long-term outcome
References
Chapter 83 Conjoined twins
Introduction
Etiology
Classification
Clinical Presentation
Diagnosis
Treatment
Preoperative ethical issues
Preoperative meetings
Separation
Complications
Early and long-term results
References
Part 8 Tumors
Chapter 84 Epidemiology and genetic associations of neonatal tumors
Introduction
Incidence of NNTs
Age and sex
Prenatal Diagnosis
Clinical presentation
Pathology
Tumor types
Etiology and Carcinogenesis
Genetic factors in NNTs
Genes resulting in a high risk of malignancy
The “two-hit” or multistep model of tumor development
Genetically determined high-risk syndromes
Mendelian single-gene-related syndromes
Familial associations with cancer
Other syndromes associated with an increased genetic risk of cancer
Association with congenital malformations
Possible increased susceptibility to environmental toxins
Radiation-induced tumorigenesis
Effect of drugs in pregnancy
Environmental exposure
Specific clinical associations of NNTs
Retinoblastoma
Neuroblastoma
Teratoma
Soft tissue tumors
Rhabdomyosarcoma
STS and the Beckwith–Wiedemann syndrome
Ewing’s family tumors
Hepatoblastoma
Wilms’ tumor
Other tumors
Antenatally diagnosed tumors
Clinical approach to antenatally diagnosed tumors
Therapeutic considerations
References
Chapter 85 Hemangiomas and vascular malformations
Introduction
Vascular Tumors
Infantile hemangioma (IH)
Clinical Features
Diagnosis
Clinical Considerations
Non-Operative Management
Operative Management
Congenital Hemangioma (CH)
Clinical Features
Management
Kaposiform hemangioendothelioma
Clinical Features
Diagnosis
Management
Pyogenic granuloma
Vascular Malformations
Capillary malformation
Clinical Features
Management
Lymphatic malformation
Clinical Features
Diagnosis
Management
Venous malformation
Clinical Features
Diagnosis
Management
Arteriovenous malformation
Clinical Features
Diagnosis
Management
PTEN-Associated Vascular Anomaly
Combined vascular malformations
Capillary Malformation–Arteriovenous Malformation
Klippel–Trenaunay syndrome
CLOVEs Syndrome
New medical treatments
References
Chapter 86 Congenital nevi
Introduction
Congenital melanocytic nevi
Epidemiology
Etiology
Pathology
Presentation
Classification
Malignancy risk
Melanoma
Other tumors associated with CMN
Patient evaluation
History and physical examination
NCM and magnetic resonance imaging
Differential diagnosis
Blue nevi
Mongolian spots
Nevus of Ota/nevus of Ito
Café au lait spots
Nevus spilus
Sebaceous nevi
Spitz nevi
Management of CMN
Small CMN
Large and giant congenital melanocytic nevi
Dermabrasion, curettage, and laser therapy
Surgical excision and reconstruction
Complications
Conclusions
References
Chapter 87 Lymphatic malformations
Introduction
Embryology and etiology
Prenatal diagnosis
Clinical presentation and imaging
Head and neck
Trunk and extremities
Syndromes associated with LMs
Radiographic investigations
Differential diagnosis
Complications
Management
Surgical management
Surgical complications
Conclusion
References
Chapter 88 Cervical teratomas
Introduction
Pathology
Incidence and clinical manifestations
Diagnosis
Differential diagnosis
Management
Conclusion
References
Chapter 89 Sacrococcygeal teratoma
Introduction
Pathology
Presentation
Clinical features
Postnatal diagnosis
Preoperative management
Operation
Postoperative management
Prognosis
References
Chapter 90 Nasal tumors
Introduction
Embryology
Congenital tumors of neurogenic origin
Nasal glioma
Histology
Diagnosis—imaging
Treatment
Technique
Complications
Meningocele and encephalocele
Treatment
Congenital dermoid cysts and dermoid sinus
Treatment
Technique
Nasal teratoma
Treatment
References
Chapter 91 Neuroblastoma
Introduction
Etiology
Inherited genetic factors
Somatic genetic factors
Epigenetic factors
Environmental factors
Evaluation
Presentation
Laboratory findings
Diagnostic imaging
Pathology
Staging
Stratification for risk of recurrence
Management
Risk-based therapy
Surgical management
Special considerations in infants
References
Chapter 92 Soft-tissue sarcoma
Introduction
Genetics
Overview of management
Chemotherapy
Specific tumors
Infantile fibrosarcoma (CIFS)
Rhabdomyosarcoma
Non-rhabdomyosarcoma soft-tissue sarcoma
Summary
Further Reading
Chapter 93 Hepatic tumors
Introduction
Benign liver tumors
Hepatic infantile hemangiomas
Tumor biology
Classification
Clinical presentation
Investigations
Management
Corticosteroids
Chemotherapeutic agents
Endovascular interventions
Surgery
Liver transplantation
Future directions
Mesenchymal hamartoma of the liver
Pathology and pathogenesis
Clinical presentation
Investigations
Treatment
Kaposiform hemangioendothelioma
Pathology and pathogenesis
Clinical presentation
Investigations
Treatment
Focal nodular hyperplasia
Hepatic adenoma
Treatment
Malignant liver tumors
Hepatoblastoma
Epidemiology
Histology
Genetic changes
Clinical findings
Imaging
Staging
Risk groups
Treatment
Adjunct therapies
Malignant rhabdoid tumor
Fibrolamellar HCC
Germ cell tumors
Choriocarcinoma
Teratomas
Primary yolk sac tumors
Principles of liver resections
Conclusion
References
Chapter 94 Congenital mesoblastic nephroma and Wilms tumor
Introduction
Pathology and cytogenetics
Clinical Features
Treatment
Preoperative Preparation
Operative technique
Position
Incision
Laparotomy and Exposure of the Renal Pedicle
Postoperative Care
Complications
References
Chapter 95 Neonatal ovarian masses
Introduction
Etiology
Incidence
Torsion
Pathology
History and physical examination
Presentation
Diagnosis
Evolution
Management
Neonatal anesthesia risks
In utero aspiration
Postnatal aspiration
Operative removal
Complications
Long-term results
References
Part 9 Spina bifida and hydrocephalus
Chapter 96 Spina bifida and encephalocele
Introduction
History
Incidence and epidemiology
Embryology
Pathogenesis
Terminology and classification
Spina bifida occulta
Spina bifida cystica: meningocele
Spina bifida cystica: myelomeningocele
Etiology
Genetics
Dietary factors
Teratogens
Prenatal diagnosis
Alpha-fetoprotein in maternal serum
Ultrasonography
Prenatal MRI
Amniocentesis
Clinical management
Prenatal assessment and antenatal counseling
In utero surgery
Postnatal assessment
Local examination
Motor function
Sensory loss
Bladder and bowel involvement
Hydrocephalus
Chiari II malformation
Skeletal abnormalities
Investigations
Parental discussion
Operative treatment and technique
Postoperative management and complications
Clinical outcome and long-term management
Medical problems
Urinary incontinence
Chiari II malformation and hindbrain dysfunction
Hydrocephalus
Lifestyle issues
Intelligence
Ambulation
Psychosocial problems
Encephalocele
Pathology
Clinical features
Differential diagnosis
Investigations
Treatment
Operative treatment and technique
Postoperative care and complication
Results
Prognosis
References
Chapter 97 Hydrocephalus
Introduction
CSF circulation
Causes of hydrocephalus
Obstructive hydrocephalus
Aqueduct stenosis
Spina Bifida and Chiari malformation
Dandy–Walker complex and posterior fossa cysts
Neoplastic lesions
Communicating (nonobstructive) hydrocephalus
Posthemorrhagic hydrocephalus
Postinfective hydrocephalus
External hydrocephalus
Overproduction of CSF
Clinical features of hydrocephalus in the newborn
Current imaging and investigative techniques for hydrocephalus in the newborn
Treatment options for hydrocephalus in the newborn
Nonsurgical/medical management
Surgical management
External ventricular drainage
Subcutaneous VAD
Ventriculoperitoneal shunt
Neuroendoscopy and ETV
Fetal surgical therapy
Shunt operation technique
Ventriculoperitoneal shunts
VA Shunts
VP shunts
Postoperative care
Outcomes
References
Part 10 Genitourinary
Chapter 98 Urinary tract infections
Introduction
Incidence
Etiology
Presentation
Impact of circumcision
Breastfeeding
Diagnosis
Management
Vesicoureteral reflux
Prophylaxis
Surgery
Conclusion
References
Chapter 99 Imaging of the renal tract in the neonate
Introduction
Commonly available imaging modalities
Abdominal US
Micturating cystourethrogram
Functional imaging
Tracers available
Static renal scintigraphy
Dynamic radionuclide renography
Patient’s preparation
Evaluation of the DRF
Evaluation of drainage
Interpretation of drainage and definition of obstruction
MRI and CT: “cross-sectional imaging”
Specific conditions in the neonatal period
Hydronephrosis
“Bright” kidneys
Cystic kidneys
Tumors
Renal anomalies
Infection
Renal calculi and nephrocalcinosis
Conclusion
Further Reading
Chapter 100 Management of antenatal hydronephrosis
Introduction
Development of the kidney and renal function
The fetus with ANH
Guidelines on ANH
Management of the newborn with ANH
Management in the nursery
Antibiotic prophylaxis
Initial radiologic evaluation
Renal ultrasound
Voiding cystourethrogram
What if the Initial sonogram is normal?
Follow-up evaluation and treatment
Diuretic renogram
Magnetic resonance urography
Ancillary studies
Congenital anomalies causing ANH
UPJ obstruction or anomalous UPJ
Multicystic dysplastic kidney
Primary obstructive megaureter (nonrefluxing)
Ureterocele and ectopic ureter
Posterior urethral valves
Vesicoureteral reflux
Summary
References
Chapter 101 Multicystic dysplastic kidney
Introduction
Etiology
Presentation
Investigations
Ultrasound
Isotope renography (nuclear medicine scan)
Voiding cystourethrography
Natural history of prenatally detected MCDKs
Indications for Surgery
Controversies surrounding “prophylactic” nephrectomy for asymptomatic, prenatally detected MCDKs
Malignant transformation (Wilms tumor)
Timing of surgery
Surgical options
Open nephrectomy
Operative details
Laparoscopic nephrectomy
Equipment and Instruments
Preparation and Position of the Patient
Technique for Transperitoneal Nephrectomy
Postoperative Care
Extraperitoneal laparoscopic nephrectomy
Follow-up
References
Chapter 102 Upper urinary tract obstructions
Introduction
PUJ Obstruction
Prenatal diagnosis
Clinical presentation
Diagnosis
Ultrasound
Radionuclide scans
Pressure-flow study
Treatment
Pyeloplasty
Dismembered Pyeloplasty
Nondismembered Pyeloplasty
Laparoscopic Pyeloplasty
Nephrectomy
Bilateral Pelviureteric Obstruction
Megaureter
Classification
Ureterovesical junction obstruction
Prenatal diagnosis
Clinical features
Diagnosis
Ultrasound
Renal scintigraphy
Intravenous urography
Management
Operation
Intravesical Approach
Cohen’s Method
Endoscopic repair of ureter
Laparoscopic reimplantation
Postoperative course
Complications
Follow-up and results
References
Chapter 103 Ureteral duplication anomalies
Introduction
Embryology
Classification
Clinical manifestation
Prenatal diagnosis
Postnatal diagnosis
Management
Vesicoureteral reflux
Ureteropelvic junction obstruction
References
Chapter 104 Vesicoureteral reflux
Introduction
Etiology
Mechanism of renal scarring
Diagnosis
Antenatally diagnosed reflux
Natural history of prenatally diagnosed VUR
Clinical presentation
Radiological investigations
Ultrasound
Micturating cystography
DMSA scan
Timing of investigations
Management
Medical management
Surgical treatment
Open antireflux procedures
Laparoscopic Ureteral Reimplantation
Endoscopic Treatment of VUR
References
Chapter 105 Ureteroceles in the newborn
Introduction
Pathogenesis
Pathological anatomy and associated pathology
Classification
Incidence
Clinical presentation
Diagnosis
Treatment
Conservative management
Endoscopic puncture or incision
Duplex-system ureteroceles
Ureterocele excision or marsupialization
Procedure
Heminephroureterectomy
Procedure
Ureteroureterostomy
Open procedure
Robot-assisted laparoscopic procedure
Percutaneous drainage
Summary
References
Chapter 106 Posterior urethral valves
Introduction
Historical notes
Epidemiological data
Embryology
Classification
Pathophysiology of PUVs
Secondary consequences
Secondary pathology on bladder and upper urinary tract
Bladder secondary changes
Ureteral pathology
Vesicoureteric reflux
“VURD” syndrome
Renal dysplasia and hydronephrosis
Pulmonary hypoplasia
Clinical features
Investigations
Prenatal diagnosis and management
Postnatal management of neonates with posterior urethral valves
Immediate management
Surgical treatment of congenital PUVs
Primary transurethral retrograde ablation
Primary antegrade ablation
Initial urinary diversion followed by secondary transurethral ablation
Vesicostomy
Upper tract diversion
Temporary nephrostomy diversion on insertion of double-J stents
Early postoperative management
Follow-up and treatment of long-term sequelae and complications
Urethral stricture
Dysfunctional voiding and urinary incontinence
VUR and hydronephrosis
Renal dysplasia
Prognostic factors in PUV
Conclusions
References
Chapter 107 Neurogenic bladder in the neonate
Introduction
Prenatal diagnosis and counseling
Anatomy and micturation physiology
Classification
Videourodynamics
Diagnosis and assessment of neurogenic bladder in the neonate
Management of neurogenic bladder
Clean intermittent catheterization
Pharmacologic treatment of neurogenic bladder overactivity
Botulinum toxin injections
Urinary diversion—vesicostomy
Bladder augmentation and procedures to increase outlet resistance
Bowel management
Conclusion
References
Further Reading
Chapter 108 Hydrometrocolpos
Introduction
Drainage procedure
Summary
Further Reading
Chapter 109 Disorders of sexual development
Introduction
Etiology
Gonad development
Androgens
Anti-Mullerian hormone
Steroids
History
Physical examination
Presentation
Diagnosis
Imaging tests
Sex of rearing
Management
Gonadal treatment
Feminizing genitoplasty
Management of clitoral enlargement
Reconstruction of the UGS
Labioplasty
Urethral/penile reconstruction
Complications
Long-term results
Further Reading
Chapter 110 Male genital anomalies
Embryology
Penis
Circumcision
Hypospadias
Micropenis/buried penis
Rare penile anomalies
Undescended testis
Diagnosis
Treatment
Rare anomalies of the testis
References
Chapter 111 Neonatal testicular torsion
Introduction
Clinical features
Operative technique
References
Part 11 Long-term outcomes in newborn surgery
Chapter 112 Long-term outcomes in newborn surgery
Introduction
What is an “outcome”?
Health-related quality of life
Instrument standards
Unique issues in health services research for the newborn population
Multidimensional generic measures of HRQoL
Pediatric Quality of Life
Child Health and Illness Profile
Child Health Questionnaire™
KINDLR
KIDSCREEN
Other Pediatric Generic Instruments
Condition-Specific Measures of HRQoL
Functional outcomes
Functional Status II
Pediatric Evaluation of Disability Inventory
Functional Independence Measure
Other assessments of functional outcome
Cost-effectiveness and utility measures
Quality of Well-Being Scale
Health utilities index
EQ-5D
Long-term outcomes in specific neonatal surgical disease
Long-term outcomes for surgery In the newborn period
General considerations—prematurity and low birth weight
General considerations—psychological effects of neonatal surgery
Thoracic surgery—general considerations
Malformations of the Tracheobronchial Tree
Pulmonary Resection
Malformations of the Esophagus
Congenital diaphragmatic hernia
Abdominal conditions
General considerations
Gastroschisis
Omphalocele
Malformations of the midgut
Malformations of the hind gut
Anorectal Malformations
Hirschsprung’s Disease
Conclusion
References
Index