Congenital cystic adenomatoid malformation (CCAM)—accuracy of diagnosis, population prevalence at delivery and outcome in a general population

Abstract

The diagnosis of CCAM can often be made antenatally. However, most available data regarding accuracy of antenatal diagnosis, incidence and outcome are derived largely from tertiary centres and may not reflect general population data. This paper describes the antenatal appearances and postnatal outcome for all cases of suspected CCAM notified to the Trent Congenital Anomalies Register from 1997 to 2001. This was a retrospective collection of antenatal and paediatric data for notified cases. Thirty-seven cases of CCAM were suspected antenatally; 21/37 cases were confirmed postnatally as having a CCAM (positive predictive value 57%); 18/21 cases were delivered at term as livebirths, 15 of whom have undergone successful surgery; 13/37 cases had apparently resolved by delivery. Three further cases were found later to be cases of lung sequestration or lobar emphysema. Five cases of CCAM were detected after delivery (sensitivity of antenatal detection 81%). The population prevalence at delivery was nine per 100 000 deliveries. Four antenatally suspected cases of CCAM developed hydrops, three required in-utero intervention and delivered as livebirths and the remaining case was terminated. The overall mortality was 14%, of which the majority were terminations.