Conservative post-natal management of antenatally diagnosed congenital pulmonary airway malformations.

Abstract

Management of congenital pulmonary airway malformations (CPAM) is controversial, especially for asymptomatic patients. We aim to describe the clinical manifestations and management of CPAM at a tertiary paediatric hospital using a retrospective audit. Infants with CPAM were identified on the Fetal Diagnostic Unit database from 2007 to 2014. Information on antenatal and post-natal management was collected from medical record. Thirty-five infants with antenatally diagnosed CPAM were included. Fetal CPAM volume ratio (CVR) was calculated from antenatal ultrasound measurement and used to categorise the infants into three groups of large (CVR ≥ 1.6, n = 8), medium (CVR of 0.5-1.6, n = 12) and small CPAM (CVR of ≤0.5, n = 15), respectively. Ten infants (10/35 = 29%) were symptomatic in the neonatal period. Overall, nine infants (26%) had surgical resection, among whom eight had large or medium-sized CPAM lesions as defined by the antenatal CVR. Three infants had neonatal emergency surgery and the remaining six had late elective surgery. Histology of eight cases showed CPAM, but one case showed congenital lobar emphysema. Criteria for surgery varied and included persistent symptoms after birth, complications during childhood and persistently abnormal chest X-ray. Most asymptomatic infants with CPAM were safely managed using a conservative approach, with no significant increase in late symptoms or complications. Conservative management of CPAM may be considered for infants/children who remain asymptomatic, especially those with a small lesion. For large and medium-sized CPAM, delineation using computed tomography is required, and surgery may be beneficial to prevent late symptoms and the risk of emergency surgery.