Neonatal Hospital Course and Outcomes of Live-born Infants with Trisomy 18 at Two Tertiary Care Centers in the United States.

Objectives Trisomy 18 is presumed to be a lethal chromosomal abnormality; medical management of infants with this aneuploidy is controversial. Our objective was to describe our approach and experience with trisomy 18 infants. Study Design We reviewed the initial hospital course, management, and factors predicting discharge from the hospital from two large tertiary care neonatal intensive care units in the southern United States over 26 years. Results Of the 29 infants with trisomy 18, 21 (72%) died in the hospital and 8 (28%) were discharged home. 19 (66%) infants received mechanical ventilation and 10 (34%) received inotropic medications. Eight infants had critical congenital heart defects; only one survived to discharge. Three infants underwent major surgeries; one cardiac surgery, one tracheoesophageal fistula repair, and one myelomeningocele repair. Median length of hospital stay was 14 days (range, 0-78) for all the infants and 31 days (range, 18-66) for those that were discharged home. Factors associated with discharge from the hospital were female sex, higher gestational age, and absence of critical congenital heart defects. Median survival time was 13 days and was significantly longer for females compared with males. Our 1-month and 1-year survival rates were 31% and 3.9% respectively. Conclusion A significant proportion of infants with trisomy 18 were discharged home. These data are helpful in counseling parents of infants with trisomy 18.

Defects in long chain fatty acid oxidation presenting as severe cardiomyopathy and cardiogenic shock in infancy.

Inborn errors of fatty acid metabolism are important causes of reversible cardiomyopathy in infancy. Disorders in long chain fatty acid oxidation can lead to cardiomyopathy, as fatty acid beta oxidation is the major source of myocardial energy after birth. We present 2 cases of such disorders with cardiac manifestations during infancy, which responded well to a diet low in long chain fatty acids.

Resection of ventricular rhabdomyomas in infants presenting with cardiac failure.

Rhabdomyomas are the most common cardiac tumours in children. They sometimes cause significant obstruction of the ventricular out flow tracts. We report a series of 3 neonates diagnosed antenatally with multiple rhabdomyomas, who developed significant obstruction of the ventricular outflow tracts following birth. They underwent surgical resection in the neonatal period with good outcome. Antenatal diagnosis of obstructive cardiac tumours allows for planning for appropriate early intervention.

Anatomical Closure of Left-to-Right Shunts in Premature Infants with Bronchopulmonary Dysplasia and Pulmonary Hypertension: A Cautionary Tale.

Closure of a systemic to pulmonary shunt in premature infants with bronchopulmonary dysplasia may be beneficial, but in the presence of pulmonary hypertension is controversial. Here, we discuss two premature infants with pulmonary hypertension who developed acute pulmonary hypertensive crisis after closure of these shunts and hence advise caution.

A multipronged approach is associated with improved breast milk feeding rates in very low birth weight infants of an inner-city hospital.

Breast milk feeding has advantages over formula feeding in premature infants, but its use in them is low. We initiated measures in our inner-city hospital such as starting a dedicated lactation service, counseling the mothers prenatally and postnatally, educating hospital staff, and advocating with our state's Medicaid insurance to provide free home breast pumps. These measures were associated with improvement in our breast milk feeding rates in very low birth weight infants from 22% to 88% over 5 years. This article describes our multipronged approach and can help encourage and guide other units with similar demographics to improve their breast milk feeding rates.