Management and outcomes of congenital pulmonary airway malformations and bronchopulmonary sequestrations diagnosed in the fetal period

Abstract Purpose To describe the prenatal and postnatal management and outcomes of fetuses prenatally diagnosed with congenital pulmonary airway malformation (CPAM) or bronchopulmonary sequestration (BPS). Methods This retrospective study included patients with a prenatal ultrasonographic diagnosis of CPAM or BPS who were treated at a quaternary referral hospital between January 2014 and December 2024. Results Twenty-seven fetuses were diagnosed with CPAM or BPS by prenatal ultrasonography. Five (18.5%) showed complete regression of the lesions during gestation. Among the 22 fetuses with persistent malformations at birth, 12 (54.5%) had left-lung malformations and 10 (45.5%) had right-lung malformations. At birth, 12 neonates (54.5%) were asymptomatic, and 10 (45.5%) had respiratory symptoms. Twenty patients underwent surgical resection of the lesion: 15 by thoracoscopy and five by open surgery. There were no deaths, and postoperative outcomes were excellent over a follow-up period of 18 (10–33.5) months. Conclusion Prenatal diagnosis of CPAM or BPS allows appropriate management of these malformations during gestation and after birth. Ultrasonographic follow-up throughout pregnancy is essential because lesions may either regress or increase in size and cause severe fetal complications. Surgical resection was performed in both symptomatic and asymptomatic children, with no deaths and excellent outcomes.