Lethal nonpulmonary anomalies associated with congenital diaphragmatic hernia: implications for early intrauterine surgery

Abstract

Recently, interest has been expressed in the correction of diaphragmatic hernia in utero as a solution to the problem of high mortality from this condition. Before such a procedure can be adopted in the human, studies aimed at establishing the contribution such advanced techniques would make in reducing mortality are required. Between 1973 and 1982, there were 36 cases of congenital diaphragmatic hernia among 75,512 births--an incidence of 1 in 2097 births. there were 11 (31%) stillbirths and 25 (69%) livebirths. the liveborn patients were divided into two groups: group A included 15 patients who died prior to transfer to the referral center; group B included ten neonates who arrived at the referral center and were operated upon. Twenty (56%) cases of diaphragmatic hernia (11 stillbirths and 9 liveborns who did not live long enough to be transferred to the referral center) were found to have lethal nonpulmonary associated anomalies at autopsy. In view of the high association of lethal anomalies it appears that fetal surgery would have a limited role in reducing mortality in congenital diaphragmatic hernia.