Aim To evaluate the outcome of fetuses with diagnosis of congenital cystic adenomatoid malformation (CCAM) of the lung.

Method We undertook a retrospective review of all cases with antenatal diagnosis of CCAM, managed in a lead perinatal centre and their outcome at 1year of age over a 7year period.

Results During this period, 26 fetuses with CCAM were identified. Relevant data was unavailable for two fetuses, another fetus had bronchogenic cyst and two were terminated. In the continuing 21 singleton pregnancies, there were associated complications in 13(62%) fetuses and all resulted in birth of live babies at varying gestations. Eight (38%) infants were symptomatic and needed NICU admission while 13(62%) remained asymptomatic and were managed in the post-natal ward. All 21 infants had chest radiograph (CXR). 11(52%) infants had normal CXR and remained asymptomatic in the neonatal period. Of the 10(48%) infants with abnormal CXR, four infants had chest CT scan which confirmed the diagnosis of CCAM in three who underwent emergency lobectomy. From our entire cohort four babies (19%) died during the neonatal period; one post-surgery and the rest from extreme prematurity. Fifteen of the 17 survivors (88%) were followed up and none of them required any surgical intervention.

Conclusions Prematurity in association with CCAM has a poor prognosis. Careful follow-up of infants should help identify those that may need further investigations and surgical intervention. Our experience raises pertinent questions regarding the appropriate modality of radiological investigations for CCAM and the recommendation of surgery in infancy for asymptomatic babies.