CONGENITAL DIAPHRAGMATIC-HERNIA IN MINNESOTA - IMPACT OF ANTENATAL DIAGNOSIS ON SURVIVAL

Objective: We characterized the natural history and true mortality of congenital diaphragmatic hernia (CDH) in newborn patients by identifyi ng all infants born with this condition in a fixed geographic region o ver a 2-year period. We examined this population to determine the freq uency of intrauterine diagnosis, the outcome of prenatally diagnosed i nfants, and the impact of deaths in infants with an unsuspected diagno sis (the ''hidden mortality'') on the overall outcome of this conditio n. Design: This was a retrospective population survey of all infants b orn with CDH in Minnesota between June 1988 and June 1990. Setting: Al l Minnesota birth and death records were reviewed to identify patients with the diagnosis of CDH. A separate survey of all level 3 intensive care nurseries was conducted and the record of each identified patien t was reviewed. Extracorporeal membrane oxygenation was available thro ughout the study period. Main Outcome Measure: Survival to hospital di scharge and short-term morbidity were examined for each patient. Resul ts: Survival was 60% (29/48). Eleven of 19 deaths occurred in patients born prematurely and/or with coexisting major anomalies. Eight percen t (4/48) of patients died within the first hour of life prior to diagn osis (hidden mortality). Intrauterine diagnosis of CDH was made in 15 patients. Survival was 60% (9/15) in infants whose conditions were dia gnosed in utero, a rate identical to that for infants whose conditions were diagnosed in the postnatal period (61% [20/33]). There was no re lationship between age at fetal diagnosis and mortality. Conclusions: The hidden mortality of CDH was low. Almost half of the total mortalit y for CDH was associated with coexisting, additional anomalies. Patien ts who were not offered extracorporeal membrane oxygenation owing to p rematurity, other major anomalies, or birth at a center that did not o ffer extracorporeal membrane oxygenation accounted for 84% (16/19) Of deaths. These data will be useful for determining the impact of new th erapeutic strategies on the mortality of CDH.