The immediate and long-term outcomes of newborns with congenital diaphragmatic hernia

Abstract

In spite of the innovations in the management of newborns with congenital diaphragmatic hernia (CDH) presenting with respiratory distress at birth, mortality and ongoing morbidity still remain high. This is a retrospective analysis of newborns with CDH to determine the immediate and long-term outcomes among survivors. Medical records of newborns with CDH and respiratory distress at birth between January 1993 and March 2002 were reviewed retrospectively. There were 45 newborns, 29 males and 16 females. Eleven newborns (24%) died during the period of preoperative stabilization, 9 from pulmonary hypoplasia and 2 with complex anomalies who were not resuscitated. Surgery was performed in 34 newborns (76%). Three died postoperatively from severe pulmonary hypoplasia and pulmonary hypertension. Eleven newborns (24%) had sepsis from coagulative-negative staphylococci. Thirty-one of 43 newborns (72%) with isolated CDH were discharged home. Twenty-seven of 31 survivors (87%) had adverse long-term outcome and 2 late deaths were from pulmonary complications. Twenty-nine of 43 newborns (67%) with isolated CDH survived. The principal determinant of survival was pulmonary hypoplasia. Eighty-seven percent of survivors have associated morbidity including ongoing pulmonary, nutritional and neuro-developmental problems. Nevertheless preoperative stabilization and delayed surgery have been a satisfactory mode of management.