2011 - ISBTS 2011 Symposium

Plenary Session I: Disappearing PNLD? + Oral Communications 1

3.107 - Long-term outcomes for infants with intestinal atresia followed at Children’s National Medical Center, Washington, DC

Presenter: Yevgeniya, Nusinovich, Washington, United States
Authors: Yevgeniya Nusinovich1, Mary Revenis1, Clarivet Torres1

Long-term outcomes for infants with intestinal atresia followed at Children’s National Medical Center, Washington, DC

Yevgeniya Nusinovich, Mary Revenis, Clarivet Torres

Children's National Medical Center, Washington, DC, United States

Intestinal atresia is a common cause of intestinal obstruction in neonates. While short-term mortality for children with intestinal atresias has steadily improved, the rate of long-term morbidities has not. The predominant cause of late complications is intestinal dysfunction and prolonged dependence on total parenteral nutrition (TPN), resulting in cholestasis and liver dysfunction. Most studies of long-term outcomes are from prior decades, when treatment protocols were different from today.

We analyzed the outcomes of 118 children treated at our hospital for intestinal atresias during the past 10 years. Survival to discharge was 95%, and 100% for those who had isolated intestinal atresias. All children who died in the NICU had severe cardiopulmonary anomalies.

Twenty of 118 (17%) were TPN-dependent beyond NICU discharge. Nineteen of them (95%) were born preterm, and their mean intestinal length was 33 cm (unknown for 5/20).  At NICU discharge, they required TPN for 90% of their caloric needs. Of these 20 patients, 2 have since died, 2 received transplants, and 2 transferred to Pittsburgh for transplantation and did not return to our care.

The remaining 14 joined the Intestinal Rehabilitation Program (IRP), founded 4 years ago. Their mean caloric requirement from TPN is currently 14%, down from 91% at the time of enrollment in the IRP, and nine patients fully weaned off TPN over an average of 7.7 months. Eleven of the 14 children had hyperbilirubinemia, with mean direct bilirubin level of 7.5 mg/dl. All resolved their cholestasis over an average of 14 weeks.

These results compare favorably to those reported for atresia patients in earlier periods. With programs such as the IRP, patients with short bowel secondary to intestinal atresia can show improvement in liver function and nutritional parameters, and discontinue PN, avoiding the need for transplantation.

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