DIAPHRAGMATIC AGENESIS AS A DISTINCT CLINICAL ENTITY

Anatomically, diaphragmatic agenesis (DA) represents the most extreme form of congenital diaphragmatic defects, but clinically it has not be en defined separately from Bochalek's hernia (BH). Between 1986 and 19 92, the authors treated 55 neonates who had diaphragmatic defects. For ty-eight of them presented within 24 hours of birth and comprised the study group. Seventeen neonates (35.4%) were found to have DA; the oth er 31 (64.6%) had BH. There were no differences in the maternal age, g estation course, gender ratio, birth weight, or incidence of co-existi ng congenital anomalies between the two groups. However, there were si gnificant differences with respect to the incidence of antenatal diagn osis (76.4% for DA patients v 12.5% for BH patients; P=.0004), mean (/-SD) Apgar scores at 1 (4.1 +/- 2.0 v 5.7 +/- 2.3; P=.034) and 5 minu tes (5.5 +/- 2.7 v 7.6 +/- 2.2; P=.016), mean duration of preoperative stabilization (2.8 +/- 2.0 v 2.1 +/- 1.9 days; P=.044) and mean durat ion of postoperative respiratory support (27.7 +/- 13.6 v 9.3 +/- 8.0 days: P=.002). Complications occurred in all seven survivors of DA and in only four (19.0%) of 21 survivors of BH (P=.0008). The neonates wi th DA had a significantly poorer long-term survival rates (29.4% v 64. 5%; P=.04). Diaphragmatic agenesis is a distinct clinical entity; its unique short-term and long-term problems require careful management. C opyright (C) 1994 by W.B. Saunders Company