cover
Principles of Neonatology
Principles of Neonatology
Principles of Neonatology
List of Contributors
List of Contributors
Preface
Preface
Acknowledgments
Acknowledgments
Contents
Contents
1 - Design of Neonatal Intensive Care Units
1 - Design of Neonatal Intensive Care Units
KEY POINTS
Introduction
Patient Safety
Decrease Stressful Stimuli and Increase Positive Stimuli
Infection
Family-Centered Care and Improved Parent-Infant Interactions
Staffing Patterns, Nursing Workload, and Communication
Growth and Weight Gain
Neurodevelopment Outcome
Hybrid Model
Impact of the COVID-19 Pandemic
Summary
REFERENCES
2 - NICU Environment for Parents
2 - NICU Environment for Parents and Staff
KEY POINTS
NICU Parents
Opportunities in the NICU for Reducing Distress
Parents
NICU Nursing Staff
Recommendations to Medical Institutions and Organizations
REFERENCES
3 - Safe Use of Health Information
3 - Safe Use of Health Information Technology
KEY POINTS
Introduction
The Need to ACT: A Focus on Access, Communication, and Tracking
Access to Information
Communication
Tracking
Shared Responsibility in EHR Safety
Usability of EHRs
EHR Security
Actions Suggested by The Joint Commission
Conclusions
REFERENCES
Chapter 4 - Pharmacologic Management of Neonatal Pain and Agitation
4 - Pharmacologic Management of Neonatal Pain and Agitation
KEY POINTS
Introduction
Pathophysiology
Clinical Features
Evaluation
Management
Nonpharmacologic Therapy for Pain and Agitation
Sucrose
Premedication for Endotracheal Intubation
Analgesia
Sedation
Vagolytics
Muscle Relaxation
Conclusions Regarding Premedication for Endotracheal Intubation
Analgesia and Sedation for Other Major Procedures
Continuous Analgesia and Sedation During Mechanical Ventilation
Benzodiazepines
Opioids
Alpha-2 Receptor Agonists
Conclusions Regarding Continuous Analgesia and Sedation During Mechanical Ventilation
Long-Term Outcomes
REFERENCES
Chapter 5 - Neonatal Transport
5 - Neonatal Transport
KEY POINTS
Introduction
Evaluation
Clinical Evaluation
Initial Management Assessment
Laboratory
Imaging
Management
Temperature Regulation
Respiratory Considerations
Infectious Disease
CVS
Hematology
Metabolism
Neurology
Hypoxic-Ischemic Encephalopathy
Cranial Hemorrhages
Social/Emotional Considerations
Communication
Documentation
Consent
Consultation/Medical Director’s Role
Safety
Types of Transport and Other Considerations
Mode of Transport
Decompensation
Unique Challenges
Education and Training
Quality
REFERENCES
Chapter 6 - Radiation Safety in Premature and Critically-Ill Neonates
6 - Radiation Safety in Premature and Critically-Ill Neonates
KEY POINTS
Introduction
Quantifying Radiation Exposure
Image Acquisition to Optimize Dose and Adhere to the ALARA Principle
Appendix 6.1
Why Is My Child Not Shielded Now?
Why Is My Child Not Shielded If I Am Required to Wear a Lead Apron While I Am in the Room With Them?
My Child Previously Had an Imaging Exam Where Shielding Was Used. Why the Change in Practice?
Can I Ask for a Shield for My Child?
REFERENCES
Chapter 7 - Placental Transfusion in the Newborn
CHAPTER
7 - Placental Transfusion in the Newborn
KEY POINTS
Introduction
Pathophysiology of Placental Transfusion
Factors That Determine Placental Transfusion
Time of Cord Clamping
Uterine Contractions
Source of Umbilical Blood Flow
Time to Establishment of Spontaneous Breathing
The Effect of Gravity
Methods of Cord Management After Birth
Immediate Cord Clamping
Delayed Umbilical Cord Clamping
Intact Umbilical Cord Milking
Cut Umbilical Cord Milking
Placental Transfusion
What Are the Benefits of Placental Transfusion?
What Are the Perceived Risks Associated With Placental Transfusion?
When Is Placental Transfusion Indicated?
What Are the Contraindications to Placental Transfusion?
How to Set Up a Quality Improvement Project on Implementation of Placental Transfusion Protocol
Recruit and Motivate the Potential Stakeholders
Address Concerns Raised Regarding DCC
Evaluate Readiness for DCC
Long-Term Outcomes
Reduction of Infant Anemia
Reduction in Infant Death Before Discharge
Effect on Neurodevelopmental Impairment
Conclusions
REFERENCES
Chapter 8 - Neonatal Resuscitation
CHAPTER
8 - Neonatal Resuscitation
KEY POINTS
Introduction
The Transition From Fetal to Neonatal Life
Pathophysiology of Perinatal Asphyxia
Preparation
Personnel
Equipment
Initial Assessment and Management
Drying and Stimulation
Thermal Management (Fig. 8.4)
Respiratory Effort and Heart Rate
Ventilation
Positive Pressure Ventilation (PPV)
Pressures Delivery Devices
Self-Inflating Bag
Flow-Inflating Bag
T-Piece Resuscitation Device
Assessment of Efficacy
Corrective Steps (Figs. 8.3 and 8.6)
Alternative Airways
Endotracheal Tubes (ETTs)
Laryngeal Mask Airway
Continuous Positive Airway Pressure (CPAP)
Sustained Inflation
Support of Circulation
Chest Compressions
Epinephrine
Volume Resuscitation
Telemedicine
Postresuscitation Care (Fig. 8.11)
Electrolyte Disturbances
Cardiovascular Monitoring
Temperature
Respiratory Monitoring
Neurologic Monitoring
Withholding Aggressive Resuscitation
REFERENCES
Chapter 9 - Golden Hour and Thermoregulation
9 - Golden Hour and Thermoregulation
KEY POINTS
The Golden Hour of the Preterm or High-Risk Neonate
Delayed Cord Clamping
Hypothermia
Respiratory Support in the Golden Hour
Fluid Management and Prevention of Hypoglycemia During the Golden Hour
Prevention of Sepsis in the Golden Hour
Optimizing Outcomes by Minimizing Interventions
Future Directions for the Golden Hour
FURTHER READING
REFERENCES
Chapter 10 - Oxygen During Postnatal Stabilization
10 - Oxygen During Postnatal Stabilization
KEY POINTS
Introduction
Oxygen in Utero and During Fetal-to-Neonatal Transition
Evolving Arterial Oxygen Saturation in the First Minutes After Birth
Initial Fio2 for Resuscitation in the Delivery Room
Higher Initial Fio2: Critical Appraisal of Recent Evidence
Final Considerations
REFERENCES
Chapter 11 - Respiratory Distress Syndrome
11 - Respiratory Distress Syndrome
Introduction
Pathophysiology
Diagnosis
Management
Early Interventions to Prevent RDS
Antenatal Steroids
Delayed Cord Clamping
Oxygen Titration
Respiratory Practices in the Delivery Room
Postdelivery Care
Postnatal Strategies to Treat Established RDS
Surfactant
Types of Surfactants
First-Generation Synthetic Surfactants
Animal-Derived Surfactants
Second-Generation Synthetic Surfactants
Third-Generation Synthetic Surfactants
Indications for Surfactant Administration
Timing of Surfactant Administration
Prophylaxis Versus Rescue Surfactant
Early Versus Delayed Rescue Surfactant
Techniques of Surfactant Administration: INSURE, INSURE, and INRecSURE
Other Respiratory Approaches
Caffeine Therapy
Permissive Hypercapnia
Postnatal Steroids
Inhaled Nitric Oxide
Noninvasive Ventilation
Mechanical Ventilation Strategies
Nonrespiratory Strategies
Temperature Control
Fluid Management
Nutritional Management
Judicious Use of Antibiotics
Cardiovascular Management
Complications of Treatment
Complications Due to Endotracheal Tubes
Pulmonary Air Leaks
Ventilator Management of Complications of RDS
Summary
REFERENCES
Chapter 12 - Invasive and Noninvasive Ventilation Strategies
12 - Invasive and Noninvasive Ventilation Strategies
Introduction
Invasive Ventilation
Conventional Mechanical Ventilation
Modes of Ventilation
Intermittent Mandatory Ventilation
Synchronized Intermittent Mandatory Ventilation
Assist Control
Pressure Support Ventilation
Modalities of Ventilation
Pressure Targeted Ventilation
Volume-Targeted Ventilation
Evidence for Use of Different Modes and Modalities of Ventilation
Recommendations
High-Frequency Ventilation
High-Frequency Jet Ventilators
High-Frequency Oscillation
Evidence for Use of HFV in Neonatal Respiratory Disease
Recommendations
Noninvasive Ventilation
Nasal Continuous Positive Airway Pressure
Nasal Prongs Versus Nasal Mask
Evidence for Use of NCPAP as Primary Respiratory Support
Recommendation
Evidence for Use of NCPAP as Secondary Support After Extubation
Nasal Intermittent Positive Pressure Ventilation
SNIPPV Versus NIPPV
NIPPV Versus BiPAP/SiPAP
Evidence for Use of NIPPV as Primary Respiratory Support
Evidence for Use of NIPPV as Secondary Support After Extubation
Recommendations
High-Flow Nasal Canula
Evidence for Use of HFNC as Primary Respiratory Support
Evidence for Use of HFNC as Secondary Support After Extubation
Recommendation
Noninvasive High Frequency Ventilation
Evidence for Use of NIHFV
Noninvasive Neurally Adjusted Ventilatory Assist
Conclusions
REFERENCES
Chapter 13 - Pulmonary Hypertension of the Newborn
13 - Pulmonary Hypertension of the Newborn
Introduction
Pathophysiology
Pulmonary Vascular Remodeling
Abnormal Pulmonary Vasoconstriction
Pulmonary Vascular Hypoplasia
Pulmonary Venous Hypertension
Clinical Conditions Presenting With PPHN
Meconium Aspiration Syndrome
Pneumonia
Respiratory Distress Syndrome
Congenital Diaphragmatic Hernia
Pulmonary Hypoplasia
Prematurity and PPHN
Miscellaneous Causes
Diagnostic Features
Clinical Signs and Symptoms
Peripheral Saturations and the Utility of Blood Gas
Hyperoxia Test
Severity of Hypoxemia in PPHN
Oxygenation Index
P/F Ratio
Alveolar-Arterial Gradient
Oxygen Saturation Index
X-Ray Imaging of the Chest
Echocardiography
Brain Natriuretic Peptide
Evidence-Based Management of PPHN
Supportive Care
Positive Pressure Ventilation and Types of Ventilation
Noninvasive Ventilation
Invasive Mode of Ventilation
Intratracheal Surfactant Therapy (COR IIa and LOE B)
Oxygen Use and Saturation Targets
Maintaining Systemic Pressures
Vasopressors
Pulmonary Vasodilator Therapy
iNO-Resistant PPHN
Prostaglandins
Sildenafil
Milrinone
Bosentan
ECMO in Management of PPHN
Special Scenarios
Preterm PPHN
Congenital Diaphragmatic Hernia
Outcomes in Neonates With PPHN
Conclusion
REFERENCES
Chapter 14 - Bronchopulmonary Dysplasia
14 - Bronchopulmonary Dysplasia
Introduction
Management
Respiratory Support Strategies
Sustained Inflation
Surfactant
Intubate, Surfactant, Extubate
Minimally Invasive Surfactant Administration
Newer Ventilation Strategies
Nasal Intermittent Positive Pressure Ventilation
Noninvasive High-Frequency Ventilation
Volume-Targeted Ventilation
Oxygen Saturation Targets
Pharmacologic Interventions
Corticosteroids
Systemic Corticosteroids in the First 7 Days of Life
Systemic Corticosteroids After 7 Days of Life
Inhaled Corticosteroids
Vitamin A
Caffeine
Inhaled Nitric Oxide
Diuretics
Bronchodilators
New Therapies
Stem Cell Therapies
Insulinlike Growth Factor 1
Long-Term Outcomes
Conclusion
REFERENCES
Chapter 15 - Treatment of Apnea of Prematurity
15 - Treatment of Apnea of Prematurity
Introduction
Pathophysiology
Clinical Features and Evaluations
More Evidence-Based (Direct) Management Approaches
Xanthines
Respiratory Support
Less Evidence-Based (Indirect) Management Approaches
Blood Transfusions
Management of Gastroesophageal Reflux
Infant Positioning
Sensory Stimulation
Long-Term Outcomes
REFERENCES
Chapter 16 - Human Milk
16 - Human Milk
KEY POINTS
Introduction
Preparation of the Mother for Breastfeeding
Information That We Have Found Useful to Encourage Mothers
Appropriate Postures for the Mother and Good Latch-on for the Baby
Milk Expression
Milk Storage at Home
Nutritional Components in Human Milk
Nutritional Implications for the Premature Infant
Immunologic Benefits
Neonatal Morbidity
Chronic Disorders
Obesity and Related Cardiovascular Changes
Cognitive Ability
Benefits of Breastfeeding for Both the Mother and the Infant
Global Impact
Breastfeeding Goals
Prelacteal Feeding
Contraindications to Breastfeeding
REFERENCES
Chapter 17 - Storage and Use of Human Milk in Neonatal Intensive Care Units
17 - Storage and Use of Human Milk in Neonatal Intensive Care Units
KEY POINTS
Introduction
Definition
Infrastructure of Human Milk Storage Units in NICUs8
Space
Equipment
Pasteurizer
Deep Freezer
Refrigerator
Hot Air Oven/Autoclave
Breast Milk Pump
Storage and Expression Containers
Administrative Staff
Uninterrupted Power Supply
Components and Process of a Human Milk Bank9,10
Encouraging Mothers and Recruitment of Donors
Donor Screening
Milk Expression
Milk Handling
Processing of Donated Milk
Allocation of Donated Milk
Documentation
Staff Training
Myths and Doubts About Pasteurized Donor Human Milk11
Is Donor Human Milk at a Par With Mother’s Own Milk?
Is Donor Human Milk Safe for Preterm Neonates?
Is Donor Milk Cost-Effective?
Summary
REFERENCES
Chapter 18 - Possible Benefits and Risks of Using Probiotics in Neonates
18 - Possible Benefits and Risks of Using Probiotics in Neonates
KEY POINTS
Types of Probiotics
Need for Standardization
Clinical Effects of Probiotics in Newborn Infants
Effect of Probiotics on Composite Outcomes Including NEC, LOS, and All-Cause Mortality
Effect of Probiotics on the Risk of NEC
Effect of Probiotics on the Risk of Late-Onset Sepsis and Other Conditions (Table 18.5)
Effect of Probiotics on Fungal Infections
Clinical Decision to Start Routine Probiotic Supplementation
Dosing and Duration
Mechanisms of Probiotic Effects
Development of Microflora During the Neonatal Period
Effects on Intestinal Inflammation
Toll-Like Receptor 4
Toll-Like Receptor 2
NLRP3 Inflammasomes
Antiinflammatory Mediators
Adaptive Immunity
Intestinal Permeability
Paneth Cells
Autophagy
Energy Regulation
Human Milk Oligosaccharides
Short-Chain Fatty Acids
Effects in Probiotics on Normal Turnover of Nutrients
Amino Acids
Vitamins
Lipid Metabolism
Effects in Probiotics on Intestinal Defense and Motility
Bacteriocins and Other Antimicrobial Molecules
Intestinal Motility
Biologic Plausibility for Effects of Probiotics in Preterm Infants
Efficacy of Individual Probiotic Strains
Follow-Up Studies
Parental Perspectives
Research Needs
Alternative Options With Prebiotics and Synbiotics: Quality and Safety
Probiotics During Other Diseases Seen in Neonates and Young Infants
Unknown Effects of Probiotics in Premature Birth
Probiotics and Atopy
Probiotics and Neurobiological Functioning
Conclusions
REFERENCES
Chapter 19 - Enteral Nutrition
19 - Enteral Nutrition
KEY POINTS
Introduction
Preterm Infant Gastrointestinal Tract
Human Milk Production and Delivery
Early Enteral Nutrition
Oral Immune Therapy With Mother’s Milk
Initiation of Feeds
Enteral Feeding Selection
Human Milk
Donor Human Milk
Preterm Infant Formula
Advancement of Feeds
Nutrition Requirements
Protein
Calories
Preterm Infant Enteral Nutrition Management
Human Milk Fortification
Cultural Considerations
Individualized Fortification
Probiotics
Vitamins, Minerals, and Osmolality
Preparation of Feeding
Measures of Feeding Intolerance
When to Modify Feeds
Methods of Feeding
Enteral Feeding Postdischarge
Long-Term Outcomes
Conclusion
REFERENCES
Chapter 20 - Parenteral Nutrition in Neonates
20 - Parenteral Nutrition in Neonates
KEY POINTS
Indications for Parenteral Nutrition
Nutrition Requirements in Parenteral Nutrition
Fluid
Energy
Macronutrients
Protein
Carbohydrates
Lipids
Electrolytes and Macrominerals
Sodium, Potassium, Magnesium, and Chloride
Calcium and Phosphorus
Iron
Vitamins
Trace Elements
Zinc
Copper
Chromium
Selenium
Carnitine
Iodine
Manganese
Aluminum
Compatibilities in Parenteral Nutrition
Practical Calculations for Parenteral Nutrition Administration
Glucose Infusion Rate
Total Fluid, Energy, and Protein Administration
Total Fluid
Total Protein
Total Energy
Lipid Infusion Rate
Calories From Essential Fatty Acids
Meeting the American Academy of Pediatrics’ Recommended 3% of Calories From Linoleic Acid
Intralipid
SMOFlipid
Complications of Parenteral Nutrition
Metabolic Bone Disease
Cholestasis
Contamination of Parenteral Nutrition Components
Parenteral Nutrition Shortages and Clinical Deficiencies
Transition to Enteral Nutrition
REFERENCES
Chapter 21 - Nutrition in Short Bowel Syndrome
21 - Nutrition in Short Bowel Syndrome
KEY POINTS
Introduction
Development of the Gastrointestinal System and Relevance to Short Bowel Syndrome
Nutritional Challenges in Short Bowel Syndrome
Intestinal Adaptation/Rehabilitation
Goals of Intestinal Rehabilitation
Nutritional Strategies in Short Bowel Syndrome
Replacement of Fluid and Electrolyte Losses
Parenteral Nutrition
Intravenous Lipid Emulsions
Enteral Nutrition
Nonnutritional Strategies in the Management of Short Bowel Syndrome
Mucous Fistula Refeeding
Cycling of Parenteral Nutrition
Multidisciplinary Care
Medications
Dietary Fibers
Enteral Fish Oil and Lipid Supplements
Probiotics
Predictors of Successful Outcomes
Future Directions
REFERENCES
Chapter 22 - Neonatal Nutrition Assessment
22 - Neonatal Nutrition Assessment
KEY POINTS
Introduction
Classification of the Newborn
Birth Weight Classification
Features of Fetal Growth Restriction
Anthropometric and Growth Assessment
Growth Charts
Growth Velocity
Using Z-Scores to Describe Growth Rates
Body Mass Index
Body Composition Modalities
Evidence of Healthy Body Composition
Biochemical Assessment
Clinical Assessment
Short Bowel Syndrome
Bronchopulmonary Dysplasia
Metabolic Bone Disease
Nutrition-Focused Physical Exam
Diagnosing Malnutrition in the Newborn
Conclusion
REFERENCES
Chapter 23 - Neonatal Hypoglycemia
23 - Neonatal Hypoglycemia
Introduction
Transitional Neonatal Hypoglycemia
Screening Neonates at Risk for Hypoglycemia
Neonates at Risk for Persistent Hypoglycemia and Indications for Evaluation
Initial Treatment and Stabilization
Evaluation of Persistent Hypoglycemia: The Diagnostic Fast
Hyperinsulinism
Treatment
Surgical Management
Conclusion
REFERENCES
Chapter 24 - Infants of Diabetic Mothers
24 - Infants of Diabetic Mothers
Epidemiology
Pathophysiology
Clinical Features
Fetal Effects
Peripartum Effects
Neonatal Effects
Metabolic
Cardiorespiratory
Hematologic
Congenital Malformations
Management
Prognosis
Conclusion
REFERENCES
Chapter 25 - Evidence-Based Neonatology- Neonatal Pituitary Hormone Deficiencies
25 - Evidence-Based Neonatology: Neonatal Pituitary Hormone Deficiencies
Introduction
Genetics of Pituitary Disease
Syndromes Associated With Pituitary Deficiency
Growth Hormone Deficiency
Diagnosis of GHD
Treatment
Central Hypothyroidism
Clinical Presentation
Diagnosis
Treatment
Gonadotropin Deficiency
Diagnosis
Treatment
Adrenal Insufficiency
Clinical Presentation
Diagnosis
Treatment
Posterior Pituitary Hormone Deficiencies
Diabetes Insipidus
Presentation and Diagnosis
Treatment
Conclusion
REFERENCES
Chapter 26 - Neonatal Thyroid Disease
26 - Neonatal Thyroid Disease
KEY POINTS
Introduction
Pathophysiology
Hypothyroidism
Hyperthyroidism
Clinical Features
Evaluation
Management
Hypothyroidism
Hyperthyroidism
Conclusion
REFERENCES
Chapter 27 - Hypothalamic-Pituitary-Adrenal Axis in Neonates
27 - Hypothalamic-Pituitary-Adrenal Axis in Neonates
KEY POINTS
Introduction
Embryology and Development of the HPA Axis
Clinical Features of Adrenal Insufficiency
Causes of Adrenal Insufficiency
Disorders of Adrenal Gland Development
Impaired Adrenal Gland Function
Congenital Adrenal Hyperplasia
Disorders of Cholesterol Metabolism
Adrenal Hemorrhage
Isolated Disorders of Mineralocorticoid Signaling
Hypopituitarism
Suppression From Exogenous Glucocorticoid
Relative Adrenal Insufficiency
Evaluation
Management
REFERENCES
Chapter 28 - Disorders of Neonatal Mineral Metabolism and Metabolic Bone Disease
28 - Disorders of Neonatal Mineral Metabolism and Metabolic Bone Disease
KEY POINTS
Background
Fetal Bone Development
Postnatal Mineral and Hormone Physiology
Hypercalcemia
PTH-Dependent Hypercalcemia
Neonatal Severe Hyperparathyroidism and Familial Hypocalciuric Hypercalcemia
PTH-Independent Hypercalcemia
Hypocalcemia
Early Neonatal Hypocalcemia
Late Neonatal Hypocalcemia
Hypocalcemia Management
Biochemical Changes in Metabolic Bone Disease of Prematurity
Risk Factors for Metabolic Bone Disease
Biochemical Screening for MBD
Role of Radiographic Screening for MBD
Prevention of MBD
Treatment of MBD
Postdischarge Management
Long-Term Outlook for Growth and Bone Mineralization
Conclusion
REFERENCES
Chapter 29 - Disorders of Sex Development for Neonatologists
29 - Disorders of Sex Development for Neonatologists
KEY POINTS
Definition
Epidemiology
Embryology
Embryology of the Gonadal Ridge
Embryology of the Testis
Embryology of the Ovary
Endocrinology of the Fetal Testis
Embryology of the Internal Ducts and External Genitalia
Genetic Regulation of Sex Determination and Sex Development
Nomenclature and Etiologies of Disorders of Sex Development
46,XX Disorders of Sex Development
Congenital Adrenal Hyperplasia
46,XX Testicular DSD
Ovotesticular DSD
Exposure to Maternal Androgens
Aromatase Deficiency
Gonadal Dysgenesis
46,XY Disorders of Sex Development
Gonadal Dysgenesis
46,XY Ovotesticular DSD
Leydig Cell Hypoplasia
Abnormal Androgen Biosynthesis
StAR Protein Deficiency
Cytochrome P450c17 (17α-Hydroxylase/17,20 Lyase) Deficiency
3β-Hydroxysteroid Dehydrogenase Deficiency
17β-Hydroxysteroid Dehydrogenase-3 Deficiency
5α-Reductase Deficiency
Androgen Insensitivity Syndrome
Persistent Müllerian Duct Syndrome
Sex Chromosome Disorder of Sex Development
45,X/46,XY Karyotypes
Syndromes and Multiple Congenital Anomalies
Evaluation of the Infant With a Disorder of Sex Development
History
Physical Examination
Diagnostic Studies
Management of Disorders of Sex Development in the Neonatal Period
Conclusion
REFERENCES
Chapter 30 - Early-Onset Sepsis
30 - Early-Onset Sepsis
KEY POINTS
Introduction
Epidemiology
Pathophysiology
Risk Factors
Microbiology
Clinical Features
Risk Assessment
EOS Risk Assessment Among Infants Born at ≥35 0/7 Weeks’ Gestation
EOS Risk Assessment Among Infants Born at ≤34 6/7 Weeks’ Gestation
Diagnostic Evaluation
Blood and Cerebrospinal Fluid Cultures
Complete Blood Count
Empiric Therapy
REFERENCES
Chapter 31 - Late-Onset Sepsis
31 - Late-Onset Sepsis
KEY POINTS
Introduction
Epidemiology
Pathophysiology
Risk Factors
Microbiology
Clinical Features
Evaluation
Cultures
Inflammatory Markers
Other Tests
Prevention
Empiric Therapy
Targeted Therapy
Adjunctive Therapy
Long-Term Outcomes
REFERENCES
Chapter 32 - Neonatal Herpes Simplex Virus Infections
32 - Neonatal Herpes Simplex Virus Infections
KEY POINTS
Background
Virus
Epidemiology
Clinical Manifestation
Skin, Eye, and Mouth Disease
CNS Disease
Disseminated Disease
Diagnosis
Management and Prevention
Outcomes
Vaccine
Management of the Asymptomatic Exposed Infant
Conclusions
REFERENCES
Chapter 33 - Postnatal Cytomegalovirus Infection Among Preterm Infants
33 - Postnatal Cytomegalovirus Infection Among Preterm Infants
KEY POINTS
Introduction
Viral Pathogenesis
Disease Pathogenesis
Perinatal Epidemiology
Preterm pCMV
Clinical Features of CMV Infection
Diagnostic Testing
Disease Management
Infection Prevention
REFERENCES
Chapter 34 - Congenital Syphilis
34 - Congenital Syphilis
KEY POINTS
Introduction
Epidemiology
Pathogenesis
Clinical Features
Diagnosis
Treatment
Follow-Up
Prevention
REFERENCES
Chapter 35 - Invasive Fungal Infections in the NICU- Candida, Aspergillosis, and Mucormycosis
35 - Invasive Fungal Infections in the NICU: Candida, Aspergillosis, and Mucormycosis
KEY POINTS
Introduction
Candida
Epidemiology and Case Definition
Risk Factors (See Fig. 35.1)
Prematurity
Medications
Catheters, Tubes, and Feedings
Gastrointestinal Pathology and Abdominal Surgery
Pathophysiology (See Fig. 35.1)
Exposure
Colonization
Infection and Dissemination
Clinical Features, Management, and Treatment
Congenital Cutaneous Candidiasis
Candidemia
Urinary Tract Infection
Central Nervous System Infection
Peritonitis
Pneumonia
Osteoarticular Infection
Endocarditis or Infected Vascular Thrombi
Endophthalmitis
Evaluation (See Figs. 35.5 and 35.6)
Cultures
Non–Culture Based Methods
Management
Antifungal Treatment
Central Venous Catheter Removal
Empiric Treatment (See Fig. 35.5)
Preemptive Treatment
Neutropenia
Prevention (See Fig. 35.7)
Antifungal Prophylaxis
Infection Control Measures
Long-Term Outcomes
Mortality and Morbidity
Aspergillosis and Mucormycosis
Malassezia and Trichosporon Infections
REFERENCES
Chapter 36 - Congenital Heart Defects
CHAPTER
36 - Congenital Heart Defects
KEY POINTS
Overview
Clinical Features
Central Cyanosis
Decreased Perfusion to the Body
Tachypnea
Timing of Presentation
Immediate and Life-Threatening
Symptoms in the Nursery
First 1 to 2 Weeks of Life
At 4 to 6 Weeks of Life
History and Physical Examination
History
Physical Examination
Anthropometric Measurements
Vital Signs
General Examination
Cardiovascular Examination
Pulmonary Examination
Abdominal Examination
Evaluation
Chest Radiograph
Electrocardiogram
Hyperoxia Test
Echocardiography
Blood Tests
Screening for CHD
Management
Long-Term Considerations
REFERENCES
Chapter 37 - Cardiac Defects—Anatomy and Physiology
CHAPTER
37 - Cardiac Defects—Anatomy and Physiology
KEY POINTS
Septal Defects
Atrial Septal Defects
Prevalence
Embryology and Anatomy
Patent Foramen Ovale
Secundum ASD
Primum ASD
Sinus Venosus Defect
Coronary Sinus Defect
Pathophysiology
Ventricular Septal Defects
Prevalence
Embryology and Anatomy
Perimembranous VSDs
Outlet VSDs
Inlet VSDs
Muscular VSDs
Pathophysiology
Large VSDs
Small VSDs
Atrioventricular Canal Defects
Prevalence
Embryology and Anatomy
Classifications of the AV Canal
Complete AV Canal
Intermediate AV Canal
Transitional AV Canal
Partial AV Canal
Outflow Septation and Conal Defects
Tetralogy of Fallot
Transposition of the Great Arteries
Prevalence
A Note on the Conus
Double Outlet Right Ventricle
Prevalence
Truncus Arteriosus
Prevalence
Aortopulmonary Window
Prevalence
A Note on the Classification of the Aortic Arch
Interrupted Aortic Arch
Outflow Tract Pathology
Isolated Pulmonary Valve Stenosis
Prevalence
Pulmonary Atresia With Intact Ventricular Septum
Aortic Stenosis
Coarctation of the Aorta
Prevalence
Arch Anomalies and Ductus Arteriosus
Aortic Arch Anomalies
Embryonic Pharyngeal Arch Arteries
Types of Abnormal Arch and Vessel Constellations
Left Arch
Right Arch
Double Aortic Arch
Pulmonary Artery Sling
Persistent Ductus Arteriosus
Prevalence
Fetal Ductus
Ductus in Preterm Neonates
Inflow Tract Anomalies
Tricuspid Atresia
Prevalence
Ebstein Anomaly
Prevalence
Single Ventricles
Double Inlet Left Ventricle
Hypoplastic Left Heart Syndrome
Prevalence
A Note on Hemodynamics and Single Ventricle Pathophysiology
Oxygen Consumption (VO2)
Oxygen Capacity
Oxygen Content
Oxygen Delivery (DO2)
Arterial-Venous Oxygen Saturation Difference
Cardiac Output
Abnormal Venous Connections
Anomalies of Systemic Venous Connections
Anomalies of the Superior Vena Cava
Retroaortic Innominate Vein
Anomalies of the Inferior Vena Cava
Interruption of the Inferior Vena Cava
Bilateral Inferior Vena Cava
Total Anomalous Pulmonary Venous Return
REFERENCES
Chapter 38 - Medical and Surgical Management of Critical Congenital Heart Disease
CHAPTER
38 - Medical and Surgical Management of Critical Congenital Heart Disease
KEY POINTS
Inadequate Pulmonary Blood Flow
Inadequate Systemic Blood Flow
Inadequate Intracardiac Shunts
Ventricular Dysfunction
Conclusion
REFERENCES
Chapter 39 - Neonatal Arrhythmia and Conduction Abnormalities
CHAPTER
39 - Neonatal Arrhythmia and Conduction Abnormalities
KEY POINTS
Normal Atrioventricular Conduction
Atrioventricular Block
First-Degree Atrioventricular Block
Second-Degree Atrioventricular Block
Mobitz Type I (Wenckebach)
Mobitz Type II (Hay Phenomenon)
Complete Atrioventricular Block
Intraventricular Conduction Delays
Long QT Syndrome
Tachyarrhythmias
Premature Contractions/Extrasystolic Beats
Supraventricular Tachycardia
Atrioventricular Reentrant Tachycardia
Orthodromic Reentrant Tachycardia
Antidromic Reentrant Tachycardia
Permanent Junctional Reciprocating Tachycardia
AVRT Management
Atrial Flutter
AV Nodal Reentrant Tachycardia
Ectopic Atrial Tachycardia
Multifocal Atrial Tachycardia
Management of Supraventricular Tachycardia
Junctional Ectopic Tachycardia
Ventricular Tachycardia
Summary
REFERENCES
Chapter 40 - Pulmonary Hypertension in Chronic Lung Disease
CHAPTER
40 - Pulmonary Hypertension in Chronic Lung Disease
KEY POINTS
Introduction
Pathophysiology
Evaluation
Management
General Care
Oxygen Supplementation
Inhaled Nitric Oxide
Pharmacotherapy
Phosphodiesterase Inhibitors
Endothelin 1 Receptor Antagonists
Prostacyclins
Epoprostenol
Iloprost
Treprostinil
Glucocorticoids
Conclusion
REFERENCES
Chapter 41 - Hemodynamic Assessment and Management of a Critically Ill Infant
CHAPTER
41 - Hemodynamic Assessment and Management of a Critically Ill Infant
KEY POINTS
Introduction
Fluid Dynamics: Understanding the Relationship Between Pressure and Flow
Determinants of Cardiac Output
Preload
Contractility
Afterload
Cardiac Output
Assessment of Cardiac Output by Impedance Cardiometry
Determinants of Systemic Vascular Resistance
Pathophysiology of Shock
Role of Near-Infrared Spectroscopy in Neonatal Shock
REFERENCES
Chapter 42 - A Practical Guide to Evaluating and Treating Severe Neonatal Indirect Hyperbilirubinemia
CHAPTER
42 - A Practical Guide to Evaluating and Treating Severe Neonatal Indirect Hyperbilirubinemia
KEY POINTS
Overview
Determining Causes of Severe Hyperbilirubinemia
Assessing for Hemolysis
End-Tidal Carbon Monoxide (ETCO)
Blood Carboxyhemoglobin
Serum Haptoglobin Concentration
Reticulocyte Percentage and Number
Hemoglobinuria
Hemolytic Causes of Hyperbilirubinemia
Alloimmune Hemolytic Diseases of the Newborn
Nonimmune (DAT-Negative) Hemolytic Disease of the Newborn
RBC Enzymopathies—HMP Shunt and Glutathione Metabolism
Glucose-6-Phosphate Dehydrogenase Deficiency
Glutathione Reductase Deficiency and Glutathione Synthetase Deficiency
RBC Enzymopathies—Glycolytic Pathway
Pyruvate Kinase Deficiency
RBC Enzymopathies—Disorders of Nucleotide Metabolism
RBC Membrane Disorders
Hereditary Spherocytosis
Nonhemolytic Causes of Indirect Hyperbilirubinemia
SLCO1B1 and SLCO1B3
Gilbert Syndrome
Crigler-Najjar Syndrome
Treatment of Severe Hyperbilirubinemia
Intravenous Immunoglobulin
Metalloporphyrins
Phenobarbital
REFERENCES
Chapter 43 - Neonatal Anemia
CHAPTER
43 - Neonatal Anemia
KEY POINTS
Introduction
Fetal and Neonatal Erythropoiesis
Identifying Anemia Using Reference Intervals
When the Cause of Neonatal Anemia Is Not Obvious
Hyporegenerative Anemia
Anemia of Prematurity
Other Hypoproliferative Anemias
Hemorrhagic Anemia
Prenatal Hemorrhage
Perinatal Hemorrhage
Postnatal Hemorrhage
Hemolytic Anemia
Microspherocytes (Fig. 43.12)
Elliptocytes
Bite and Blister Cells
Echinocytes
Schistocytes
Immune-Mediated Hemolytic Disease of the Fetus/Neonate
Rh Hemolytic Disease
ABO Hemolytic Disease
Hemolytic Disease Due to Non-Rh, Non-ABO Antigens
Late Anemia After Hemolytic Disease of the Fetus and Neonate
Hemolytic Disease of the Fetus/Neonate Associated With Infectious Diseases
REFERENCES
Chapter 44 - Evidence-Based Neonatal Transfusion Guidelines
CHAPTER
44 - Evidence-Based Neonatal Transfusion Guidelines
KEY POINTS
Introduction and Pathophysiology
Fetal and Neonatal Oxygen Delivery
Acute and Chronic Anemia
Clinical Studies
Transfusion Studies in Critical Care
Neonatal Transfusion Studies
TOP/ETTNO Studies
Hematocrit Triggers for Transfusion
Eligibility Criteria and Study Design
Outcomes, Morbidities, and Transfusions
Summary of TOP and ETTNO
Evaluation and Management
Transfusions and Outcomes
REFERENCES
Chapter 45 - Neonatal Thrombocytopenia
CHAPTER
45 - Neonatal Thrombocytopenia
KEY POINTS
Platelet Counts in Newborn Infants
Fetal and Neonatal Platelet Production
Platelet Function and Primary Hemostasis
Evaluation of Neonatal Thrombocytopenia
Early Onset of Thrombocytopenia in Well-Appearing Infants
Delayed Onset of Thrombocytopenia
Management
Are Prophylactic Platelet Transfusions Justified?
Can Transfused Platelets Cause Inflammation?
Management of NAIT
Maternal Treatment
Management of Autoimmune Thrombocytopenia
Maternal Management
Syndromic Causes of Neonatal Thrombocytopenia
Nontransfusional Therapies
Altered Platelet Function
Etiology/Pathophysiology
Clinical Presentation and Diagnosis
Evaluation
Treatment
REFERENCES
Chapter 46 - Management of Hypoxic-Ischemic Encephalopathy Using Therapeutic Hypothermia
CHAPTER
46 - Management of Hypoxic-Ischemic Encephalopathy Using Therapeutic Hypothermia
KEY POINTS
Introduction
The Pathogenesis of Brain Cell Death
What Initiates Neuronal Injury?
Cerebral Injury Evolves Over Time
Intracellular Mediators of Delayed Cell Death
Inflammatory Second Messengers
Excitotoxicity After Hypoxia-Ischemia
The Determinants of Neuroprotection With Therapeutic Hypothermia
Timing of Starting Hypothermia: The Earlier the Better
How Cold Is Too Cold?
If Some Is Good, Is More Better?
Clinical Evidence for Therapeutic Hypothermia
Evidence From Randomized Controlled Trials
Is It Possible to Further Optimize Therapeutic Hypothermia?
Is More Better?
Is There Benefit From Cooling Started More Than 6 Hours After Birth?
Should We Cool Infants With “Mild” HIE?
Conclusion
Acknowledgments
REFERENCES
Chapter 47 - Management of Hypoxic-Ischemic Encephalopathy Using Measures Other Than Therapeutic Hypothermia
CHAPTER
47 - Management of Hypoxic-Ischemic Encephalopathy Using Measures Other Than Therapeutic Hypothermia
KEY POINTS
Definition, Diagnosis, and Differential
Definition
Diagnosis
Perinatal History
Neurologic Examination
EEG
Pathophysiology
Acute Phase
Latent/Chronic Phase
Stratification of Patients by Risk
Gestational Age/Prematurity
Encephalopathy Score
Amplitude Integrated EEG/Continuous Video EEG
Near-Infrared Spectroscopy
Imaging
Head Ultrasound
Magnetic Resonance Imaging
Biomarkers
Transport
Supportive Management
Neuroprotective Strategies
Seizure Control
Levetiracetam (Roweepra, Spritam, and Keppra)
Topiramate (Trokendi XR, Qudexy XR, and Topamax)
Shivering Control
Future Adjuvant Therapies
Epoetin-α/Darbepoetin-α
Caffeine
Magnesium Sulfate
Sildenafil
Autologous Cord Blood
Melatonin
Allopurinol
Others
Cannabinoids
Nanoparticles
Postacute Care Follow-Up
Etiology
Clinical Progress
Persistent Deficits
REFERENCES
Chapter 48 - Management of Encephalopathy of Prematurity
CHAPTER
48 - Management of Encephalopathy of Prematurity
KEY POINTS
Introduction
Pathophysiology
White Matter Injury
Neuronal-Axonal Injury
Gray Matter Injury
Clinical Features and Evaluation
Clinical and Imaging Evaluation
Management
Prenatal Treatments: Antenatal Corticosteroids and Magnesium Sulfate
Intrapartum Management—Delayed Cord Clamping
Postnatal Management
Glucose Control
Hypercarbia and Hypocarbia
Caffeine
Seizures and Role of Electroencephalography
Early Developmental Therapy
Outcome
REFERENCES
Chapter 49 - Neonatal Seizures
CHAPTER
49 - Neonatal Seizures
KEY POINTS
Introduction
Pathophysiology
Ion Channels
Ion Gradients
Synaptic Receptors
Other Factors Favoring Hyperexcitability in the Developing Brain
Differential Diagnosis
Evaluation
How Long Should a Newborn Be Monitored for Seizures Using Video-EEG?
When to Consider an Inborn Error of Metabolism as a Potential Seizure Cause?
Genetic Work-Up
Management
When to Discontinue Antiseizure Medications?
Long-Term Outcomes
REFERENCES
Chapter 50 - Stroke in Neonates
CHAPTER
50 - Stroke in Neonates
KEY POINTS
Introduction
Epidemiology
Pathogenesis
Presentation
Evaluation and Management
Outcomes
Recurrence
Neurologic Outcomes
Cognitive and Behavioral Impairment
Epilepsy
REFERENCES
Chapter 51 - Using Biomarkers for Management of Perinatal Brain Injury
CHAPTER
51 - Using Biomarkers for Management of Perinatal Brain Injury
REFERENCES
Chapter 52 - Intraventricular Hemorrhage and Posthemorrhage Hydrocephalus
CHAPTER
52 - Intraventricular Hemorrhage and Posthemorrhage Hydrocephalus
KEY POINTS
Introduction
Incidence and Timing of GM-IVH
Neuropathology
Normal Cerebrospinal Fluid Pathways
Germinal Matrix-Intraventricular Hemorrhage
Pathogenesis of Periventricular Hemorrhagic Infarction
Posthemorrhagic Ventricular Dilatation and Posthemorrhagic Hydrocephalus
Clinical Features
Diagnosis
Clinical Management
Prevention of GM-IVH
Posthemorrhagic Hydrocephalus (PHH)
Long-Term Neurosurgical Management
Conclusions
REFERENCES
Chapter 53 - Management of Neurotrauma
CHAPTER
53 - Management of Neurotrauma
Introduction
Epidemiology
Neonatal Neurotrauma Anatomical Classification
Head Injuries
Scalp Injuries
Scalp Hematomas
Caput Succedaneum
Cephalohematoma
Management
Subgaleal Hematoma/Hemorrhage
Management
Skull Fractures
Management
Diagnosis
Clinical Manifestations
Diagnostic Testing
Treatment
Complications
Intracranial Hematomas
Epidural Hematoma
Subdural Hematoma
Subarachnoid Hemorrhage
Intraventricular and Intracerebral Hemorrhage
Grading
Management
Diagnosis
Clinical Manifestations
Diagnostic Testing
Treatment
Spine and Spinal Cord Injuries
Management
Diagnosis
Clinical Manifestations
Diagnostic Testing
Radiologic Assessment
Treatment
REFERENCES
Chapter 54 - Management of Myelomeningocele and Related Disorders of the Newborn
CHAPTER
54 - Management of Myelomeningocele and Related Disorders of the Newborn
Introduction
Pathophysiology
Clinical Features
Diagnosis
Prenatal Diagnosis and Counseling
Management
Acute Perinatal Management for Open NTD
Operative Technique for Open NTD
Postoperative Management for Open NTD
Long-Term Outcomes
Neurosurgical
Hydrocephalus
Chiari II Malformation
Tethered Cord Syndrome
Urologic
Orthopedic
REFERENCES
Chapter 55 - Treating Neonatal Abstinence Syndrome in the Newborn
CHAPTER
55 - Treating Neonatal Abstinence Syndrome in the Newborn
Introduction
Opioids
Mechanism of In Utero Exposure
Neonatal Opioid Withdrawal Syndrome Symptoms
Treatment of Neonatal Opioid Withdrawal Syndrome
Nonpharmacologic Treatments
Mechanism of Action for Pharmacologic Treatments
Morphine and Methadone
Buprenorphine
Adjunctive Therapies
Long-Term Impact of Neonatal Opioid Withdrawal Syndrome
Stimulants
Kratom Tea
Depressants
Alcohol
Cannabinoids
Selective Serotonin Reuptake Inhibitors
Conclusions
REFERENCES
Chapter 56 - Neonatal Immunity
CHAPTER
56 - Neonatal Immunity
Leukocyte Populations in the Fetus and the Neonate
Innate Immune System
Neutrophils
Function
Transendothelial Migration
Chemotaxis
Phagocytosis
Intracellular Killing
Degranulation
Monocytes and Macrophages
Development
Natural Killer Cells
Development
Function
Noncytotoxic Innate Immune Cells
Development
Function
Adaptive Immune System
Dendritic Cells
Function
T Lymphocytes
Development
T Cell Receptor (TCR) Repertoire
Circulating T Cells
Function
Proliferation
Cytokine Production
Antigen-Specific Responses
Other Subgroups (See Fig. 56.3)
Cytotoxic T Lymphocytes
γδT Cells
T-Regulatory Cells
B Lymphocytes
Development
Immunoglobulin Repertoire
Circulating B Cells
Function
Immunoglobulin Production
Serum Immunoglobulin Levels
Other Subgroups
CD5 Expressing B Cells
Function
T- and B-Cell Interaction
Recruitment of Various Leukocyte Subpopulations
REFERENCES
Chapter 57 - Immunodeficiency Syndromes Seen During the Neonatal Period
CHAPTER
57 - Immunodeficiency Syndromes Seen During the Neonatal Period
Introduction
Innate Immune System
Neutropenia
Diagnosis and Laboratory Evaluation
Management
Defects in Adhesion and Transepithelial Migration
Leucocyte Adhesion Deficiency
Defects in Chemotaxis and Cellular Life Span
Hyper-Immunoglobulin E Syndromes (HIES)
Defects in Chemotaxis and Bacterial Killing
Chédiak-Higashi Syndrome
Warts, Hypogammaglobulinemia, Infections, and Myelokathexis Syndrome
Chronic Granulomatous Disease
Myeloperoxidase Deficiency
Macrophages Derived From Tissue Progenitors and From Monocytes
Adaptive Immune System
Antibody Production Defects
Selective IgA Deficiency
X-Linked Agammaglobulinemia
Transient Hypogammaglobulinemia of Infancy
Hyper-Immunoglobulin M Syndrome
T-Cell Defects
Chromosome 22q11.2 Deletion Syndrome
Immune Deficiency Affecting Multiple Cell Lineages
Severe Combined Immunodeficiency
REFERENCES
Chapter 58 - Neonatal Acute Kidney Injury
CHAPTER
58 - Neonatal Acute Kidney Injury
Introduction
Developmental Considerations
Nephrogenesis
Dynamic Changes in Renal Blood Flow and Physiology After Birth
Definitions of Neonatal AKI
Epidemiology and Risk Factors for Neonatal AKI
Incidence and Outcomes: AWAKEN Study
Perinatal Risk Factors
Evaluation: Prevention and Mitigation of Neonatal AKI
Nephrotoxin Avoidance
Kidney Function Monitoring
Novel Approaches to Determining AKI Risk and Kidney Function
Risk Factor Assessment: Renal Angina Index
Novel Biomarkers
Kidney Function Assessment
Management of Neonatal AKI
Supportive Care
Medication Dosing
Electrolyte Management
Nutrition Management
Fluid Management
Evolving Research on Specific Therapeutics
Indications for Renal Replacement Therapy
Long-Term Outcomes After Neonatal AKI
Summary
REFERENCES
Chapter 59 - Renal Replacement Therapy
CHAPTER
59 - Renal Replacement Therapy
KEY POINTS
Renal Replacement Therapy in Infants With Acute and Chronic Renal Failure
Peritoneal Dialysis
Osmotic Agents
Electrolytes
Buffers
Hemodialysis
CRRT
RRT is Changing the Prognosis of Acute AKI in Neonates
The Impact of RRT on CAKUTs and Inherited Cystic Kidney Diseases
REFERENCES
Chapter 60 - Neonatal Hypertension
CHAPTER
60 - Neonatal Hypertension
KEY POINTS
Introduction
Proper Blood Pressure Measurement
Physiology of Blood Pressure in Neonates
Incidence of Hypertension
Etiology of Hypertension
Clinical Presentation
Evaluation of Hypertension
Management of Hypertension
Long-Term Outcomes
REFERENCES
Chapter 61 - Altered Development of the Kidneys and the Urinary Tract
CHAPTER
61 - Altered Development of the Kidneys and the Urinary Tract
KEY POINTS
Altered Development of the Kidneys and the Urinary Tract
Congenital Abnormalities of the Kidney and the Urinary Tract
Renal Agenesis
Renal Ectopia
Multicystic Kidney Dysplasia
Horseshoe Kidney or Renal Fusion
Duplex Kidneys
Prenatally Diagnosed Hydronephrosis
Ureteropelvic Junction Obstruction
Primary Megaureter
Vesicoureteral Reflux
Posterior Urethral Valves and Prune Belly Syndrome
Obstructive and Nonobstructive Renal Dysplasia
Nonmotile Ciliopathies and Congenital Renal Diseases
Meckel–Gruber Syndrome
Joubert Syndrome and Related Disorders
Short Rib Syndrome
Bardet–Biedl Syndrome
Renal-Hepatic-Pancreatic Dysplasia (RHPD) and Asplenia/Polysplenia
Hereditary Renal Adysplasia or Renal Hypodysplasia/Aplasia (RHDA)
Zellweger Syndrome and Peroxisomal Disorders
Trisomies and Congenital Anomalies of the Kidneys
VACTER-L Association
Bilateral Renal Agenesis (Potter Syndrome)
Caudal Dysplasia Syndrome
Sirenomelia
Evaluation
Functional Disorders
Congenital Nephrotic Syndrome
Neonatal Bartter Syndrome
Renal Tubular Acidosis
Low Nephron Counts and Glomerular Volume
Inherited Renal Cystic Disorders
ARPKD
ADPKD
Medical Management of Renal Anomalies
REFERENCES
Chapter 62 - Retinopathy of Prematurity
CHAPTER
62 - Retinopathy of Prematurity
Introduction
Pathophysiology
Role of Growth Hormone and IGF-1 in ROP
Other Factors With Possible Roles in Pathogenesis of ROP
Hypoxia-Induced Factor 1
Erythropoeitin
Genetics
Oxidative Stress
Adrenergic Receptors
Adenosine and Apelin
Omega 3 Lipids
Clinical Features of ROP
Stages
Stage 0
Stage 1: Demarcation Line
Stage 2: Ridge
Stage 3: Extraretinal Neovascularization
Stage 4: Partial Retinal Detachment
Stage 5: Total Retinal Detachment
Zone
Extent of Disease: Clock Hours
Plus Disease
Preplus Disease
Aggressive-Posterior ROP
Regression of ROP
Management
Screening for ROP
Timing of Screening
One Week or Shorter Follow-Up
One- to Two-Week Follow-Up
Two-Week Follow-Up
Two- to Three-Week Follow-Up
Discontinuation of ROP Examinations
Treatment of ROP
Surgery
Sequelae of Treated ROP
Future Direction
REFERENCES
Chapter 63 - Developmental Anomalies of the Globe and Ocular Adnexa in Neonates
CHAPTER
63 - Developmental Anomalies of the Globe and Ocular Adnexa in Neonates
Introduction to the Eye and Adnexal Structures
Abnormal Eye Structure
Absent or Small Disorganized Eye: Anophthalmos and Microphthalmos (Fig. 63.1)
Clinical Features
Pathophysiology
Evaluation
Management
Small but “Normally Structured” Eye: Nanophthalmos (Fig. 63.2)
Clinical Features
Pathophysiology
Evaluation
Management
Enlarged Eye: Buphthalmos
Abnormal Eyelid Structure/Function
Absence of Eyelids, Adnexal Structures, and “Hidden Eye”: Cryptophthalmos (Fig. 63.3)
Clinical Features
Pathophysiology
Evaluation
Management
Focal Eyelid Defect: Coloboma (Fig. 63.4)
Clinical Features
Pathophysiology
Evaluation
Management
Abnormal Eyelid Fusion (Ankyloblepharon) or Shape (Euryblepharon) (Figs. 63.6 and 63.7)
Clinical Features/Pathophysiology
Evaluation
Management
Abnormally Narrow Eyelid Opening (Palpebral Fissure): Blepharophimosis (Fig. 63.8)
Clinical Features/Pathophysiology
Evaluation
Management
Eyelid Drooping: Congenital Ptosis (Fig. 63.9)
Clinical Features/Pathophysiology
Evaluation
Management
Eyelid Drooping: Congenital Horner Syndrome (Fig. 63.10)
Clinical Features/Pathophysiology
Evaluation
Management
REFERENCES
Chapter 64 - Developmental Anomalies of the Cornea and Iris in Neonates
CHAPTER
64 - Developmental Anomalies of the Cornea and Iris in Neonates
Cornea: Abnormal Appearance or Size
Congenital Corneal Opacification (CCO)
Clinical Features
Pathophysiology
Evaluation
Management
Corneal Crystals
Clinical Features
Pathophysiology
Evaluation
Management
Large Cornea: Megalocornea
Clinical Features
Pathophysiology
Evaluation
Management
Small Cornea: Microcornea
Clinical Features
Pathophysiology
Evaluation
Management
Iris and Pupil: Congenital Abnormalities
Diffuse Iris Underdevelopment: Aniridia
Clinical Features
Pathophysiology
Evaluation
Management
Focal Iris Underdevelopment: Iris Coloboma
Clinical Features
Pathophysiology
Evaluation
Management
Diffuse Bilateral Iris Hypopigmentation: Albinism
Clinical Features
Pathophysiology
Evaluation
Management
Asymmetric Iris Pigmentation: Heterochromia (Fig. 64.10)
Clinical Features
Pathophysiology
Evaluation
Management
Atrophic or Distorted Iris: Anterior Segment Dysgenesis
Clinical Features
Pathophysiology
Evaluation
Management
Excess Iris Tissue: Primary Iris Cysts, Iris Flocculi, and Persistent Pupillary Membrane
Clinical Features
Pathophysiology
Evaluation
Management
Abnormal Iris Lesions: Juvenile Xanthogranuloma and Brushfield Spots
Clinical Features
Pathophysiology
Evaluation
Management
Displaced Pupil and/or Lens: Corectopia and/or Ectopia Lentis
Clinical Features
Pathophysiology
Evaluation
Management
REFERENCES
Chapter 65 - Cataract and Glaucoma
CHAPTER
65 - Cataract and Glaucoma
Congenital Cataract
Presentation
History
Examination
Workup and Management
Laboratory Investigations and Tests
Examination Under Anesthesia
Preoperative Planning
Operative Approach and Issues
Postoperative Management
Complications and Long-Term Prognosis
Glaucoma
Presentation
History
Examination
Workup and Management
Laboratory Investigations and Tests
Examination Under Anesthesia
Imaging Techniques and Functional Testing
Treatment Options
Medications and Special Considerations
Surgical Approaches
Long-Term Management
REFERENCES
Chapter 66 - Neonatal Tracheostomy
CHAPTER
66 - Neonatal Tracheostomy
Introduction
Pathophysiology
Clinical Features and Indications
Management
Preoperative
Procedure49
Tracheostomy Tube Sizes
Postoperative Complications
Postoperative Care
Long-Term Outcomes
Summary
REFERENCES
Chapter 67 - Stridor and Laryngotracheal Airway Obstruction in Newborns
CHAPTER
67 - Stridor and Laryngotracheal Airway Obstruction in Newborns
Introduction
Pathophysiology and Clinical Features
Supraglottic Larynx
Glottic Larynx
Subglottic Larynx
Trachea
Evaluation (Clinical, Laboratory, and/or Imaging)
History
Physical Examination
Special Diagnostic Procedures
Imaging and Laboratory Tests
Management and Long-Term Outcomes
Supraglottic Larynx
Laryngomalacia
Laryngeal Cysts
Glottic Larynx
Vocal Cord Paralysis
Anterior Glottic Web
Subglottic Larynx
Subglottic Stenosis
Subglottic Hemangioma
Trachea
Tracheomalacia
Vascular Rings
Tracheal Stenosis
REFERENCES
Chapter 68 - Pierre-Robin Sequence-Cleft Palate-Related Airway Obstruction Seen in Neonates
CHAPTER
68 - Pierre-Robin Sequence/Cleft Palate-Related Airway Obstruction Seen in Neonates
Introduction
Pathophysiology
Genetic Contributions to PRS
Clinical Features
Evaluation
Management
Feeding Management
Long-Term Outcomes
Summary
REFERENCES
Chapter 69 - Congenital Hearing Loss Seen in Neonates
CHAPTER
69 - Congenital Hearing Loss Seen in Neonates
Introduction
Etiology and Pathophysiology of Sensorineural Hearing Loss
Newborn Hearing Screen (NHS)
Special NHS Considerations
Prematurity
Interpretation of the Effects of Middle Ear Fluid
Auditory Neuropathy Spectrum Disorder
Definitive Diagnosis
Diagnostic Auditory Brainstem Response
Behavioral Testing
Management of Newborns With SNHL
Components of the History in an Infant With SNHL
Hearing Aids and Early Intervention
Diagnostic Testing
Congenital Cytomegalovirus Screening, Diagnosis, and Management in Pediatric SNHL
Genetics
Imaging
Conclusion
REFERENCES
Chapter 70 - Nasal Obstruction in Newborn Infants
CHAPTER
70 - Nasal Obstruction in Newborn Infants
Introduction
General Clinical Features and Recommended Evaluations for Neonatal Nasal Obstruction
Initial Examination
Nasal Endoscopy
Imaging
Evaluation for Other Airway and Pulmonary Lesions
Pulmonary/Sleep Medicine Consultations
Swallowing/Feeding Evaluation
Gastroenterology
Genetics
Considerations for Initial Management of Neonatal Nasal Obstruction
Epidemiology, Pathophysiology, Clinical Features, Evaluation, and Management of More Common Causes of Neonatal Nasal Obstru ...
Neonatal Rhinitis
Epidemiology
Pathophysiology
Clinical Features
Evaluation/Management
Long-Term Outcomes
Nasal and Septal Deviation
Epidemiology
Pathophysiology
Clinical Features
Evaluation/Management
Long-Term Outcomes
Nasolacrimal Duct Cyst/Dacryocystocele
Epidemiology
Pathophysiology
Clinical Features
Evaluation/Management
Long-Term Outcomes
Congenital Nasal Pyriform Aperture Stenosis
Epidemiology
Pathophysiology
Clinical Features
Evaluation/Management
Long-Term Outcomes
Midnasal Stenosis
Epidemiology
Pathophysiology
Clinical Features
Evaluation/Management
Long-Term Outcomes
Choanal Atresia/Stenosis
Epidemiology
Pathophysiology
Clinical Features
Evaluation/Management
Long-Term Outcomes
Midline Congenital Nasal Masses
Epidemiology
Pathophysiology
Clinical Features
Evaluation/Management
Long-Term Outcomes
Conclusions
REFERENCES
Chapter 71 - Upper Extremity Conditions in the Neonate
CHAPTER
71 - Upper Extremity Conditions in the Neonate
Introduction
Embryology
Pathophysiology
Clinical Features, Evaluation, Management, Long-Term Outcomes, and Conditions
Syndactyly
Clinical Features
Evaluation
Management
Long-Term Outcomes
Polydactyly
Clinical Features, Evaluation, and Management
Ulnar/Postaxial Polydactyly/Small Finger Duplication
Radial/Preaxial Polydactyly/Thumb Duplication
Central Polydactyly
Long-Term Outcomes
Radial, Ulnar, and Central Longitudinal Deficiencies
Clinical Features
Evaluation
Management
Long-Term Outcomes
REFERENCES
Chapter 72 - Newborn Spine Deformities
CHAPTER
72 - Newborn Spine Deformities
Introduction
Identification and Treatment in Early Life
Appraisal Literature and Evidence-Based Guidelines
Pathophysiology
Embryologic Development of the Spine16,17
Environmental Etiology of Spinal Malformations17
Growth and Development18,19
Clinical Features
Spina Bifida20–22
Congenitally Abnormal Vertebrae1,2,8,9,11,13,23
Effects on Pulmonary Function10,25–28
Associated Anomalies4,29,30
Caudal Regression Syndrome31
Skeletal Dysplasias32
Evaluation
Physical Exam30
Ultrasound33,34
Radiographs35–38
Computed Tomography37,39,40
Magnetic Resonance Imaging30,41–44
Management
Myelomeningocele Repair20,45–48
Deformity Management Principles1,29,49–52
Growth Preservation Strategies78–85
REFERENCES
Chapter 73 - Hip and Lower Extremity Deformities
CHAPTER
73 - Hip and Lower Extremity Deformities
Developmental Dysplasia of the Hip
Pathophysiology
Clinical Presentation
Treatment
Long-Term Outcomes
Talipes Equinovarus
Pathophysiology
Evaluation
Management
Long-Term Outcomes
Calcaneovalgus
Congenital Hyperextension of the Knee and Congenital Knee Dislocation
Pathophysiology
Evaluation
Management
Long-Term Outcomes
Fibular Hemimelia
Pathophysiology
Evaluation
Treatment
Long-Term Outcomes
Congenital Femoral Deficiency
Pathophysiology
Evaluation
Management
Long-Term Outcomes
Tibial Hemimelia
Pathophysiology
Evaluation
Management
Long-Term Outcomes
Tibial Bowing
Pathophysiology
Clinical Presentation
Management
Long-Term Outcomes
Congenital Vertical Talus
Pathophysiology
Clinical Presentation
Management
Long-Term Outcomes
REFERENCES
Chapter 74 - Fractures and Musculoskeletal Infections in the Neonate
CHAPTER
74 - Fractures and Musculoskeletal Infections in the Neonate
KEY POINTS
Clavicular Fractures
Epidemiology and Risk Factors
Clinical Features and Evaluation
Long-Term Outcomes
Management
Congenital Pseudarthrosis of the Clavicle
Epidemiology and Risk Factors
Clinical Features and Evaluation
Management
Humerus Fractures
Epidemiology and Risk Factors
Clinical Features and Evaluation
Management
Femoral Fractures
Epidemiology and Risk Factors
Clinical Features and Evaluation
Management
Congenital Pseudarthrosis of the Tibia
Epidemiology and Risk Factors
Clinical Features and Evaluation
Management
Bone and Joint Infections
Pathophysiology
Epidemiology and Risk Factors
Clinical Features and Evaluation
Imaging
Management
Long-Term Outcomes
Conclusion
REFERENCES
Chapter 75 - Most Frequently Encountered Inborn Errors of Metabolism
CHAPTER
75 - Most Frequently Encountered Inborn Errors of Metabolism
KEY POINTS
Diagnosis of Conditions With Early Neonatal Onset or Those Not Included in Screening Programs
Urea Cycle Defect
Clinical Features
Diagnosis
Treatment
Clinical Approach to IEMs Detected in NBS Programs
Congenital Hypothyroidism
Introduction
Clinical Features
Diagnosis
Treatment
Congenital Adrenal Hyperplasia
Introduction
Clinical Features
Diagnosis
Treatment
Organic Acidemias
Introduction
Clinical Features
Diagnosis
Treatment
Galactosemia
Introduction
Clinical Features
Diagnosis
Treatment
Phenylketonuria
Introduction
Clinical Features
Diagnosis
Treatment
Maple Syrup Urine Disease
Introduction
Clinical Features
Diagnosis
Treatment
Medium-chain Acyl-CoA Dehydrogenase Deficiency
Introduction
Clinical Features
Diagnosis
Treatment
Citrullinemia
Introduction
Clinical Features
Diagnosis
Treatment
REFERENCES
Chapter 76 - Screening Programs for Early Detection of Inborn Errors of Metabolism in Neonates
CHAPTER
76 - Screening Programs for Early Detection of Inborn Errors of Metabolism in Neonates
Introduction
Background
Selection of Disorders
Description of the Process
Abnormal Newborn Screen Results
False-Positive/False-Negative Results
REFERENCES
Chapter 77 - Genetic Conditions (Including Microarrays, Exome Sequencing)
CHAPTER
77 - An Overview of Genetic Testing
Diagnostic Techniques in Clinical Genetics
Chromosomal Disorders and Karyotyping
Mosaicism
Aneuploidy
Fluorescence In Situ Hybridization
Chromosomal Microarray
Sanger Sequencing
Next-generation Sequencing Technology
Gene Panel Testing
Exome Sequencing
Genome Sequencing
REFERENCES
Chapter 78 - Genetics of Common Birth Defects in Newborns
CHAPTER
78 - Genetics of Common Birth Defects in Newborns
KEY POINTS
Summary
Introduction
Congenital Heart Disease
Evidence for the Genetic Basis of Congenital Heart Disease
Genetic Testing in Congenital Heart Disease
Chromosomal Aneuploidies
Down Syndrome
Trisomy 18 and 13
Turner Syndrome
Copy Number Variations
22q11.2 Deletion Syndrome
Williams-Beuren syndrome
Single-Gene Defects
Monogenic Conditions Causing Syndromic CHD
Alagille Syndrome
Holt-Oram Syndrome
Noonan Syndrome and RASopathies
Heterotaxy and Ciliopathies
GDF1 and Founder Ashkenazi Mutation
Monogenic Causes of Isolated CHD
Transcription Factors
Cell Signaling and Adhesion Models
Structural Proteins
Histone Modifiers
Common Variants and CHD
Recommendations for Clinical Genetic Testing
Conclusion
REFERENCES
Chapter 79 - Common Monogenetic Conditions in Newborns
CHAPTER
79 - Common Monogenetic Conditions in Newborns
Autosomal Recessive Polycystic Kidney Disease
Pathophysiology
Clinical Features
Evaluation
Management
Long-Term Outcomes
Achondroplasia
Pathophysiology
Clinical Manifestations
Evaluation
Management
Long-Term Outcomes
Rasopathies
Pathophysiology
Clinical Features
Evaluation
Management
Long-Term Outcomes
Tuberous Sclerosis Complex
Pathophysiology
Clinical Features
Evaluation
Management
Long-Term Outcomes
Spinal Muscular Atrophy
Pathophysiology
Clinical Features
Evaluation
Management
Long-Term Outcomes
Congenital Myotonic Dystrophy Type 1
Pathophysiology
Clinical Features
Evaluation
Management
Long-Term Outcomes
REFERENCES
Chapter 80 - Common Chromosomal Conditions in Newborns
CHAPTER
80 - Common Chromosomal Conditions in Newborns
Introduction
Turner Syndrome (Monosomy X) and Triple X Syndrome (Trisomy X)
Klinefelter Syndrome
Jacobs Syndrome
Trisomy 13 and Trisomy 18
Trisomy 21
Microdeletion/Microduplication Syndromes
REFERENCES
Chapter 81 - Necrotizing Enterocolitis
CHAPTER
81 - Necrotizing Enterocolitis
Introduction
Risk Factors
The Five Clinical Presentations of NEC in Infants
Classic Textbook NEC
NEC That Fails to Improve After a Period of Medical Therapy
NEC in the Presence of Portal Venous Gas
Staccato NEC
NEC Totalis
Differential Diagnoses
NEC Versus Spontaneous Intestinal Perforation
Diagnosis
Imaging
Biomarkers and Laboratory Parameters for the Diagnosis of NEC
Management
Medical Management
Feeding and Parenteral Nutrition
Antibiotic Therapy
Operative Management
Exploratory Laparotomy
Peritoneal Drainage
Complications From NEC
Postoperative Complications
Liver Bleeding
Recurrent NEC
Intestinal Strictures
Wound Complications
Enterostomy Complications
Short Bowel Syndrome
Neurodevelopmental Complications of NEC
Outcomes and Prevention in Patients Undergoing Surgery for NEC
Summary
REFERENCES
Chapter 82 - Extracorporeal Membrane Oxygenation in Neonates
CHAPTER
82 - Extracorporeal Membrane Oxygenation in Neonates
Introduction
Epidemiology
ECMO—Respiratory
Congenital Diaphragmatic Hernia
Persistent Pulmonary Hypertension of the Newborn
Meconium Aspiration Syndrome
ECMO—Cardiac
ECMO—ECPR
Neurologic Outcomes
Controversies and Ethical Considerations
REFERENCES
Chapter 83 - Intestinal Surgery in the Newborn—Atresias, Volvulus, and Everything Else
CHAPTER
83 - Intestinal Surgery in the Newborn—Atresias, Volvulus, and Everything Else
Introduction
Intestinal Atresia
Pathophysiology
Clinical Features
Evaluation
Management
Long-Term Outcomes
Malrotation and Volvulus
Pathophysiology
Clinical Features
Evaluation
Management
Long-Term Outcomes
Meconium Ileus
Duplication Cysts
Abdominal Wall Defects
Pathophysiology
Clinical Features
Omphalocele
Gastroschisis
Evaluation
Management
Omphalocele
Gastroschisis
Long-Term Outcomes
REFERENCES
Chapter 84 - Pulmonary Surgery in the Newborn
CHAPTER
84 - Pulmonary Surgery in the Newborn
Upper Respiratory Airway Disease
Clinical Characteristics
Diagnosis
Endoscopy
Imaging
Laryngomalacia
Laryngeal Stenosis
Laryngeal Webs and Laryngeal Atresia
Tracheomalacia
Tracheal Stenosis
Vascular Compression of the Airway
Congenital Lung Lesions
Clinical Characteristics
Diagnosis
Prenatal
Postnatal
Management
Prenatal
Postnatal
Congenital Pulmonary Airway Malformation
Bronchopulmonary Sequestration
Congenital Lobar Emphysema
Clinical Presentation
Diagnosis
Management
Bronchogenic Cysts
Diagnosis
Management
Congenital Diaphragmatic Hernia
Anatomy and Embryology
Pathophysiology
Diagnosis
Prenatal Diagnosis
Fetal Ultrasound
Fetal Magnetic Resonance Imaging
Postnatal Diagnosis
Management
Prenatal Care
Prenatal Interventions
Postnatal Care
Cardiopulmonary Resuscitation
Management of Pulmonary Hypertension
Surgical Management
Diaphragmatic Eventration
Clinical Presentation
Diagnosis
Management
REFERENCES
Chapter 85 - Congenital Anorectal Malformations and Hirschsprung Disease in the Neonate
CHAPTER
85 - Congenital Anorectal Malformations and Hirschsprung Disease in the Neonate
Introduction
Anorectal Malformations
Pathophysiology of Anorectal Malformations
Normal Continence
Clinical Features of Anorectal Malformations
Types of Anorectal Malformations
Fecal Control
Evaluation of Anorectal Malformations
Defects Associated With Anorectal Malformations
Management of Anorectal Malformations
Long-Term Outcomes of Anorectal Malformations
Hirschprung Disease
Pathophysiology of Hirschsprung Disease
Clinical Features of Hirschsprung Disease
Evaluation of Hirschsprung Disease
Management of Hirschsprung Disease
Long-Term Outcomes in Hirschprung Disease
Conclusions
REFERENCES
Chapter 86 - Esophageal Surgery in Neonates- Esophageal Atresia, Gastroesophageal Reflux, and Other Congenital Anomalies
CHAPTER
86 - Esophageal Surgery in Neonates: Esophageal Atresia, Gastroesophageal Reflux, and Other Congenital Anomalies
Introduction
EA and TEF
Pathophysiology
Embryology
Anatomy and Classification
Natural History
Clinical Features
Prenatal Diagnosis
Clinical Presentation
Evaluation
Evaluation of Associated Anomalies
Management
Preoperative Management
Operative Repair of EA/TEF
Management of Long-Gap EA
Postoperative Care
Complications
Special Situations in the NICU
Emergency Management of Respiratory Failure in the TEF Neonate
Management of H-Type TEF
Long-Term Outcomes
Surgery for Other Esophageal Pathology in Neonates
Surgery for Gastroesophageal Reflux Disease
Summary
REFERENCES
Chapter 87 - Caring for Families Who Have Previously Endured Multiple Perinatal Losses
CHAPTER
87 - Caring for Families Who Have Previously Endured Multiple Perinatal Losses
Reproductive Story
Ontological Death
Complexity of the Loss of Young Infants
Disenfranchised Grief
Impact of Previous Losses During Pregnancy or Early Infancy
Grief
Role of the Healthcare Professional
REFERENCES
Chapter 88 - Current State of Perinatal Palliative Care- Clinical Practice, Training, and Research
CHAPTER
88 - Current State of Perinatal Palliative Care: Clinical Practice, Training, and Research
Introduction
The Clinical Practice of Perinatal Palliative Care
Availability of Perinatal Palliative Care Across the United States
Triggers to Identify Eligible Patients
Timing of PPC Integration
Typical PPC Services
Location of Care/Systems Issues for PPC
Primary and Subspecialty Perinatal Palliative Care Training
The Interdisciplinary Team
Palliative Care Training
Progress in Perinatal Palliative Care Research
Defining Outcomes
Methodological Challenges
Future Research
REFERENCES
Chapter 89 - Serious Communication in the Neonatal Intensive Care Unit- Evidence for Strategies and Training
CHAPTER
89 - Serious Communication in the Neonatal Intensive Care Unit: Evidence for Strategies and Training
Communication in the Neonatal Intensive Care Unit
What Clinicians Should Do
Guidelines
Shared Decision-Making
What Clinicians Do
Practice Variation
Engaging Parents
Provision of Information
Values
What Parents Want
Antenatal Counseling
Empathetic Communication
Consultation Setting
Engaging Expectant Parents in Decision-Making
Providing Information
Values
End-of-Life Conversations
Empathetic Language and Trust
Decisional Authority
Information
Values
Recommendations for Practice
Communication Education
Conclusion
REFERENCES
Chapter 90 - Pain and Symptom Management in Newborns Receiving Palliative and End-of-Life Care
CHAPTER
90 - Pain and Symptom Management in Newborns Receiving Palliative and End-of-Life Care
Introduction
Pain
Opioids
Additional Medications for Pain Control
Nonpain Symptoms
Anxiety and Agitation
Delirium
Dyspnea
Sialorrhea
Myoclonus
Seizures
Nonpharmacologic Interventions
Symptom Management in the Actively Dying Neonate
Conclusion
REFERENCES
Chapter 91 - Palliative Care Family Support in Neonatology
CHAPTER
91 - Palliative Care Family Support in Neonatology
Prenatal Phase
Clinical Case Report
Epidemiology of Perinatal Death and Parent Outcomes
Role of Perinatal Palliative Care
Spiritual and Cultural Considerations
Communication
Prenatal Decisions: Termination, Imaging, Monitoring
Delivery Decisions: Location, Mode, and Infant Care
Ongoing Decisions: The Unexpected and the Uncertain
Acute Neonatal Intensive Care Phase
Continuation of Clinical Case Report
NICU Hospitalizations and Infant Death in the United States
Parent Experiences and Needs in an Uncertain NICU Environment
Parent Support Needs
Cultural Differences in Parent End-of-Life Experiences
Decision-Making: Central Role for Parents in the NICU
Conflicts Between the Healthcare Team and Parents
Decision-Making at the End-of-Life
Sources of Support for Parents During End-of-Life Decision Making
Use of a Question Prompt List to Empower Parents
Perinatal Palliative Care as an Added Layer of Support in the NICU
Chronic Critical Illness Phase
Continuation of Clinical Case Report
Epidemiology and Definition of Chronic Critical Illness
Bereavement Phase
Continuation of Clinical Case Report
Bereaved Parent Health Epidemiology
Parent Grief Experiences
Grief as a Shared Family Experience
Staff Remembering
REFERENCES
Chapter 92 - Early Diagnosis and Intervention in Neonatal Intensive Care Units
CHAPTER
92 - Early Diagnosis and Intervention in Neonatal Intensive Care Units
Days 1 to 3 of Life
Step 1: Assign Risk of NDD Based on Perinatal Factor Severity
Step 2: Early Parent Participation (Enriched Environment)
Step 3: Mother’s Own Milk
Days 3 to 7 of Life in the NICU
Step 4: Track Head Circumference
Step 5: Compliance With Core Elements of Developmentally Supportive Care14
Step 6: Role of Neonatal Therapy in the NICU
Step 7: Screening
Days 7 to 14
Third to Fourth Week
Prior to Discharge From the NICU
Step 8: Neurologic Examination
Step 9: Discharge Planning24
Components of Discharge Readiness (With Relevance to Neurodevelopment)
Checklist—Predischarge (With Relevance to Neurodevelopment)
Hand Over to Parents and Primary Care Physician
Step 10: Registration for Quality Assurance
REFERENCES
Chapter 93 - Early Diagnosis and Intervention—On Neonatal Follow-Up
CHAPTER
93 - Early Diagnosis and Intervention—On Neonatal Follow-Up
Ensuring Continuity and Compliance After Discharge From the NICU
Role of Developmental Follow-Up Services
Developmental Surveillance—After Discharge From the NICU
Beyond Early Infancy
Vision
Hearing and Language
Referral: Timely Specific Interventions and Interprofessional Integration
Role of Pediatricians
Role of Developmental Specialists
Role of Pediatric Neurologists
Role of Orthopedics
Neurosurgery
Role of Psychologists
Role of Therapists
Health Needs of Parents
Audits and Process of Improvement
REFERENCES
Chapter 94 - Early Detection of Cerebral Palsy
CHAPTER
94 - Early Detection of Cerebral Palsy
Introduction
Pathophysiology
Congentital Abnormalities
Prematurity
Perinatal Stroke
Infection
Hypoxic-Ischemic Encephalopathy
Genetic Associations
Postnatal Injury
Clinical Features and Evaluation of Cerebral Palsy
Guidelines for Diagnosis
Long-Term Outcomes
Motor Outcomes
Nonmotor Outcomes
Survival
Prognosis
Management
Motor
Sensory
Cognition
Sleep, Pain, Spasticity
Behavioral
Parents and Parenting
Conclusion
REFERENCES
Chapter 95 - Use of Neuroimaging to Predict Adverse Developmental Outcomes in High-Risk Infants
CHAPTER
95 - Use of Neuroimaging to Predict Adverse Developmental Outcomes in High-Risk Infants
Introduction
Imaging of the Neonatal Central Nervous System
Cranial Ultrasound
MRI
Types of Brain Injury in the Neonatal Period
Preterm WMI
IVH and PHVD
Cerebellar Injury
Hypoxic Ischemic Encephalopathy
Respiratory Failure Needing Extracorporeal Membrane Oxygenation
Neonatal Arterial Ischemic Stroke
Congenital Heart Disease
Small for Gestational Age Due to Intrauterine Growth Restriction
Congenital Brain Anomalies
Metabolic Disorders
Comparison of TE-cUS and TE-MRI for Predicting Neurodevelopmental Outcomes
Advanced MRI Techniques
Conclusions
REFERENCES
Chapter 96 - Neurodevelopmental Impairment in Specific Neonatal Disorders
CHAPTER
96 - Neurodevelopmental Impairment in Specific Neonatal Disorders
Introduction
Pathophysiology
Hypoxic-Ischemic Encephalopathy
Structural Abnormalities on Neuroimaging
Clinical Outcomes
Intraventricular Hemorrhage
Structural Abnormalities on Neuroimaging
Clinical Outcomes
Bronchopulmonary Dysplasia
Structural Abnormalities on Neuroimaging
Clinical Outcomes
Necrotizing Enterocolitis
Structural Abnormalities on Neuroimaging
Clinical Outcomes
Neonatal Sepsis
Structural Abnormalities on Neuroimaging
Clinical Outcomes
Follow-Up of NICU Survivors
REFERENCES
Chapter 97 - Quality Improvement in Neonatal Care
CHAPTER
97 - Quality Improvement in Neonatal Care
General Considerations
Methodology
Defining and Selecting the Problem to be Addressed
Establishing a Project Team
Establishing a Mission and Target
Measures
Prioritizing Changes
Communication
Maintaining the Gain
Quality Networks
Professional Expectations for QI
Research Versus Quality Improvement
REFERENCES
Chapter 98 - Neonatal Randomized Controlled Trials
CHAPTER
98 - Neonatal Randomized Controlled Trials
Introduction
Clinical Trial Designs
Human Subjects Protections and Neonatal Clinical Trials
Selection of Study Population
Therapeutic Intervention
Choice of Endpoint or Response Variable
Collection of Adverse Event Information
Selection of the Control Group
Randomization
Adaptive Strategies
Blinding or Masking of Assignment
Informed Consent
Recruitment and Retention of Trial Participants
Long-Term Follow-Up in Neonatal RCTs
Stakeholder Engagement in Neonatal RCT Design
Summary Conclusions
Acknowledgment
REFERENCES
Chapter 99 - Designing Clinical Trials in Neonatology—International Trials
CHAPTER
99 - Designing Clinical Trials in Neonatology—International Trials
Introduction
Building a Shared Vision of the Purpose of the Study
How to Encourage Local Buy-in and Participation
Regulatory and Ethical Considerations
Ethics Board Review Process
Regulations Affecting the Intervention Itself
Data Sharing and Data Transfer Agreements
Data and Safety Monitoring Board
Differences in Healthcare Systems and Insurance Structures
Financial Contracts
Informed Consent
Patient Reimbursement and Compensation
Language and Translation of Documents
Strategies to Navigate Regulatory Issues
Guidelines for the Conduct of International Clinical Trials
Potential Sources of Funding
Conclusion
REFERENCES
Chapter 100 - Organization of Neonatal Intensive Care
CHAPTER
100 - Organization of Neonatal Intensive Care
Neonatal Intensive Care Units
Leadership Structure
Medical Staff
Nursing and Ancillary Staff
Data, Quality, and Safety Staff
NICU Provider Workforce Shortages and Strategies
Transition to Home
System Resources
NICU Team
Caregiver
Effective Communication
REFERENCES
Chapter 101 - Leadership and Organizational Culture in Healthcare
CHAPTER
101 - Leadership and Organizational Culture in Healthcare
Leadership
Leadership Types
Authoritative Leadership
Transformational (Collaborative) Leadership
Transactional Leadership
Contingent Leadership
Participative Leadership
Significance of Specific Leadership Styles
Challenges
Organizational Culture
REFERENCES
Chapter 102 - Skin Disorders in Newborn Infants
CHAPTER
102 - Skin Disorders in Newborn Infants
Immaturity of the Skin in Newborn Infants
Development
Transient Cutaneous Changes Seen in Newborn Infants
Minor Congenital Anomalies That Predominantly Involve the Skin
Digital Abnormalities
Complications Associated With Neonatal Intensive Care
Congenital Disorders of Skin Structure Seen in Newborn Infants
Vascular Malformations
Inflammatory Disorders of the Neonatal Skin
Scaling Disorders
Cutaneous Infections
Cutaneous Neoplasms
REFERENCES
