Case narrative

Annie is born at full term with good Apgar scores, but weighs only 2350 grams. On physical exam, she is alert but has somewhat low tone. She has abnormal facial features, a short sternum, and over-riding digits. After failure to pass a nasogastric tube, a tracheal–esophageal fistula (TEF) is diagnosed. Chromosome studies confirm trisomy 18, and an echocardiogram shows a moderate ventricular septal defect (VSD) and moderate pulmonary valvular dysplasia.

Ellen, the neonatologist, and Mary, the surgeon, meet Annie’s parents, Bill and Liz, to discuss treatment options. They explain that trisomy 18 is a lethal condition and that most children die within the first few weeks to months. The team does not recommend surgery, explaining that surgery is not in the child’s best interest and will result in unnecessary suffering for Annie. They offer the services of the neonatal palliative care team and reassure Bill and Liz that the palliative care team can assist the family in the grieving process. They can provide comfort care for Annie by keeping an IV in place for hydration and pain relief.