The outcome of fetuses diagnosed with congenital cystic adenomatous malformation of the lungs: Experience of a regional neonatal center (2004–2016)

Abstract

Objective: Although much is known about the antenatal course of congenital cystic adenomatous malformation (CCAM), the postnatal course is less well documented. The vast majority of infants remain asymptomatic at birth, with the controversy surrounding the postnatal management of such infants. We reviewed the outcome of fetuses diagnosed with CCAM in our center and evaluated their symptom burden during the 1st year of life. Methods: A retrospective review of maternal and infant medical records of all cases with antenatal diagnosis of CCAM managed in a regional perinatal center over 12 years, and infant outcome at 1 year of age is presented. Results: Forty-two eligible singleton pregnancies/infants were identified. Thirteen babies (30.9%) were symptomatic and 29/42 (69.1%) asymptomatic at birth. 7/13 (53.9%) symptomatic infants had associated antenatal complications. The fetal lesions were more likely to remain static or reduce in size during pregnancy in asymptomatic (26/29; 89.6%) than symptomatic babies (8/13; 61.6%). All babies had chest radiographs after birth, but computed tomography (CT) scan was done in only a few symptomatic babies; 7/42 (16.7%) during the neonatal period and a further 3/42 (7.1%) during infancy. The majority (8/13; 61%) of the symptomatic babies had surgical intervention compared to only 1/29 (3.4%) asymptomatic babies who had surgery. However, most babies remained symptom-free during infancy. Death within the cohort was limited to babies who were symptomatic (4/13; 31%). Conclusion: Most pregnancies diagnosed with CCAM remain uncomplicated. The behavior of the prenatal lesion could help predict the postnatal outcome. Our experience highlights that CCAM persistence cannot be excluded from chest radiographs; hence, the need for chest CT scans. Symptom surveillance should help guide the need for surgery in asymptomatic infants. However, only skilled clinicians who can offer long-term follow-up, until the transition into adulthood should oversee surveillance to ensure safety.