Natural history of congenital diaphragmatic hernia: implications for management

Abstract

Advances in ultrasonography have made it possible to accurately diagnose congenital diaphragmatic hernia (CDH) prenatally, and recently interest has been expressed in in-utero correction of CDH as a solution to the problem of high mortality. In an attempt to elucidate the natural history of CDH, we analysed the incidence and mortality of this disorder in a large maternity hospital with an autopsy rate of 100% for all stillbirths and neonatal deaths. Between 1973 and 1985, there were 47 cases of CDH among 99,062 births, an incidence of 1 in 2,107 birhts. There were 15 (32%) stillbirths and 32 (68%) live births. All 15 stillborns had lethal associated non-pulmonary anomalies. Of the 32 liveborn patients, 17 died prior to transfer to the referral centre and 11 of these had major associated anomalies. Prematurity was a feature in 65% of patients who died prior to transfer to the referral centre. Nine of the 15 patients who arrived at the referral centre survived. Our date suggest that the only patient who may benefit from prenatal correction of CDH is a fetus who has serious persistent pulmonary hypertension. However, in the absence of reliable criteria for the in-utero prediction of persistent pulmonary hypertension in association with CDH, surgical correction of CDH prenatally is not practicable at present even for this group of patients. Newer therapeutic approaches in the management of persistent pulmonary hypertension should be addressed as the most promising approach in the management of a CDH patient who develops respiratory failure in the first few hours of life.