Is congenital pulmonary airway malformation really a rare disease? Result of a prospective registry with universal antenatal screening program

Abstract

BACKGROUND/PURPOSE: Congenital pulmonary airway malformation (CPAM) is an increasingly recognized disease with potential mortality. Owing to limited published studies the true incidence is yet to be determined. Therefore we carried out this study with the aim to estimate its true incidence on a population basis in a prospective manner. METHODS: An antenatal ultrasonography program was implemented since 2009. Fetuses with suspected intra-thoracic lesions were monitored by regular follow-ups. Antenatal course, post-natal outcomes and other demographics were compared to those of patients with CPAM in the previous decades (1989-2008). The incidence of CPAM was calculated in different periods. RESULTS: 66 CPAM patients were identified between 2009 and 2014 with 60 patients being detected by antenatal scan. In contrast 45 patients were identified between 1989 and 2008 with 27 patients being detected antenatally. The incidence rate during the past and recent period was estimated as ~1 in 26000 and ~1 in 7200 live births respectively (p=0.029). CONCLUSIONS: With the increasing awareness of clinicians and the universal use of latest ultrasound technology, it is likely that more CPAM cases will be detected in the future. Here we presented our best estimated incidence rate of CPAM, yet only a larger scale study can reveal its true incidence.