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Neonatal Gastroesophageal Reflux

Key Points Gastroesophageal reflux (GER) is almost universal in neonates. It is a physiologic process secondary to frequent spontaneous transient lower esophageal sphincter relaxation, relatively large volume liquid diet, and age-specific body positioning. Preterm infants have relative immaturity of neural control of the gastrointestinal tract, leading to delayed gastric emptying and slow gut motility. The diagnosis of GER in the neonatal intensive care unit (NICU) is…

Abdominal Wall Defects

Key Points Gastroschisis is a congenital abdominal wall defect located just to the right of the umbilicus. The bowel that herniates out of the abdomen is not covered by a membrane. Fetuses and newborns with a gastroschisis have a low risk of associated anomalies, except for a 10% to 25% risk of intestinal atresia. The clinical outcomes of patients with gastroschisis are mainly determined by their…

Structural Anomalies of the Gastrointestinal Tract

Key Points Small inclusion cysts in the oral cavity are common in newborns and almost always resolve without treatment. Suspect the diagnosis of esophageal atresia when there is feeding difficulty with an inability to pass a tube from the nose (or mouth) into the stomach. In the most common type of esophageal atresia, there is a fistula from the trachea to the distal esophagus (tracheoesophageal fistula…

Parenteral Nutrition for the High-Risk Neonate

Key Points Early use of parenteral nutrition in the very low birth weight (VLBW) neonate minimizes nutrient store losses and improves growth outcomes. Initial support goals include glucose infusion of 4 to 8 mg/kg/min, amino acids at 2 to 3 g/kg/day, and lipids at 2 g Goal calorie intake for full parenteral nutrition for VLBW infants (1000 to 1500 g) is 90 to 100 kcal/kg/day (3…

Enteral Nutrition

Key Points Early protein intake is associated with improved growth and neurodevelopmental outcomes in premature infants; achieving adequate intake requires a combined parenteral and enteral approach. Mother’s own milk (MOM) is the preferred diet for preterm infants; further research is needed to develop evidence-based clinical guidelines to optimize the use of donor human milk when MOM is unavailable. For very low birth weight infants, unfortified human…

Neonatal Seizures

Key Points Neonatal seizures are common. Clinical assessment alone is insufficient for diagnosis, and EEG evaluation is necessary. Seizures are often symptomatic of an underlying cause requiring investigation. Confirmed seizures should be treated with antiseizure medications. Neonatal Seizures Seizures in the neonate occur in 2 to 4 per 1000 live births and are a cause of neonatal morbidity and mortality. Frequently, this onset is a neurologic…

Neonatal Neuromuscular Disorders

Key Points Evaluation of neonatal hypotonia includes neuromuscular conditions, and the diagnostic work-up should be approached in a stepwise manner. A normal creatine phosphokinase does not completely rule out muscle disease. Electromyography is useful in the diagnostic evaluation of hypotonia and weakness. Spinal muscular atrophy (SMA) can present in the neonatal period and has time-sensitive treatments. Genetic testing for the commonly found gene deletion in SMA…

Neonatal Neurovascular Disorders

Key Points Perinatal stroke is a vascular event causing focal interruption of blood supply and can be categorized based on the vascular distribution of stroke (arterial or venous), age at the time of stroke, and age at presentation. When a stroke is suspected in a neonate, neuroimaging is required for confirmation of diagnosis, followed by risk factor assessment and creation of a specific treatment plan. In…

Neonatal Encephalopathy

Key Points Neonatal encephalopathy is a clinical syndrome that can result from a variety of underlying causes. Hypoxic-ischemic encephalopathy (HIE) is a major cause of neonatal brain injury and mortality worldwide. Magnetic resonance imaging and magnetic resonance spectroscopy are useful imaging tools to identify brain injury and help predict long-term neurodevelopmental outcomes in neonates with HIE. Cerebral arterial or venous thrombosis may be the cause of…

Brain Injury in the Preterm Infant

Key Points Intraventricular hemorrhage (IVH) remains a common cause of chronic neurologic morbidity. Despite a gradual decline in the incidence of most grades of IVH, the increased survival of very low birth weight infants has resulted in an increase in the absolute number of infants with IVH. Preterm infants are currently at much lower risk of severe white matter injury (WMI), which typically results in focal…

Congenital Malformations of the Central Nervous System

Key Points Brain malformations are a significant source of morbidity and death in neonates. Advances in imaging and genetics now allow more specific diagnoses. Early and specific diagnosis allows more precise prognostication, including improved monitoring for complications and treatment decisions. Early diagnosis and management of patients with brain malformations require multidisciplinary team management. Prosencephalic Cleavage and Related Events Normal Prosencephalic Development The prosencephalon refers to the…

Central Nervous System Development

Key Points The brain of the preterm and term newborn infant is actively developing, with several key steps occurring during the third trimester, such as the end of neuronal migration, programmed cell death, the generation of axons and dendrites, and the first wave of synaptogenesis. These processes are controlled by genetic programs but are also very sensitive to environmental factors. Epigenetic mechanisms appear to play a…

Long-Term Neurologic Outcomes in Children With Congenital Heart Disease

Key Points Congenital heart disease (CHD) is a common birth defect with continually improving survival of neonates with complex lesions requiring heart surgery. Neurodevelopmental (ND) abnormalities are common in school-age children and adolescents after neonatal heart surgery. Magnetic resonance imaging (MRI) studies have demonstrated evidence of brain injury and delayed brain development, even before having a corrective operation, in patients with hypoplastic left heart syndrome and…

Congenital Heart Disease

Key Points Congenital heart disease (CHD) is the most common birth heart defect encountered in the clinical setting, affecting 1% of live births. Surgical outcomes for all forms of CHD continue to improve. Early detection through fetal echocardiography, physical examination, and pulse oximetry screening allows for improved neonatal management and decreased short-term and long-term morbidity. The etiology of CHD remains elusive in many cases. Advances in…

Perinatal Arrhythmias

Key Points The term supraventricular tachycardia (SVT) encompasses several different arrhythmias that may have different diagnostic and therapeutic requirements. Tachycardia mechanisms may be caused by conduction reentry, enhanced automaticity, or triggered automaticity. Isolated atrial and ventricular ectopy is common in the fetus and neonate and usually does not require any therapy. Congenital forms of complete heart block may result in slow heart rates but may not…

Patent Ductus Arteriosus in the Preterm Infant

Key Points Clinical signs of a symptomatic patent ductus arteriosus (PDA) usually appear later than echocardiographic signs and are related to the degree of left-to-right ductal shunting. Factors known to play a prominent role in regulation of ductal patency involve those that promote constriction (oxygen, endothelin, calcium channels, catecholamines, and Rho kinase) and those that oppose it (intraluminal pressure, prostaglandins, nitric oxide, carbon monoxide, potassium channels,…

Persistent Pulmonary Hypertension

Key Points Persistent pulmonary hypertension of the newborn (PPHN) can occur with parenchymal lung disease, with pulmonary hypoplasia, or without associated lung disease ( idiopathic ) and has an incidence of 0.2% in term infants and up to 2% in preterm infants. Maternal factors such as diabetes, high body mass index, smoking, use of selective serotonin receptor inhibitors or nonsteroidal anti-inflammatory drugs, and cesarean delivery increase…

Cardiovascular Compromise in the Newborn Infant

Key Points It is difficult to diagnose neonatal shock in its uncompensated phase during the immediate transitional period, while it is even harder to diagnose neonatal shock in its compensated phase using standard clinical monitoring and clinical approach. It is unclear what gestational- and postnatal-age-dependent blood pressure and systemic and organ blood flow values represent hypotension and poor tissue perfusion (respectively), warranting timely intervention in the…

Developmental Biology of the Heart

Key Points The heart forms from cardiac mesoderm as a symmetric linear tube with connections to the primitive arterial and venous systems. The heart tube is formed by 3 weeks’ gestation in the human. The heart tube loops, establishing laterality, and undergoes septation into four chambers with connections to the systemic and pulmonary arteries and veins. The heart is fully formed by 9 weeks’ gestation. External…

Anatomic Disorders of the Chest and Airways

Key Points Prompt diagnosis and early intervention are key in salvaging infants with life-threatening surgical disorders of the airway and chest. Improved prenatal ultrasound has shifted the diagnosis and management of disorders of the chest and airways into the fetal period. Increased utilization of video-assisted thoracoscopic surgery for congenital thoracic lesions has made surgical intervention on the infant chest less invasive and more tolerable. Anomalies of…