Prenatally diagnosed congenital lung malformations-A long-term outcome study

To report the natural history of a cohort of children with prenatally diagnosed congenital lung malformations (CLM) which we set out to manage expectantly.

Retrospective review of children born between 1995 and 2013 with a CLM identified on prenatal ultrasound. Prenatal ultrasound data were analyzed along with patient medical records, radiology, and pathology.

One hundred fifty-nine newborns with a fetal lung lesion were identified during the study period. All infants were live born. Twenty-eight (18%) newborns were symptomatic, 17 with transient symptoms, and 11 with persistent symptoms. The latter all underwent lobectomy in the neonatal period with two postoperative deaths. One hundred thirty-one asymptomatic newborns plus the 17 babies with transient symptoms (148 total) were followed during childhood for a median of 6.0 years (0.1-19.2 years). Twenty-one children (13% of the original cohort of 159) became symptomatic at a median age of 2.5 years (9 months-5 years 8 months) with infection and underwent CLM resection. No child became symptomatic after the age of 5 years 8 months. One hundred twenty-seven children remained symptom free during follow-up for a median of 5.75 years (1 month-19 years). We saw no instance of malignancy in the resected specimens.

This study adds further evidence that most children born with CLM identified prenatally are asymptomatic at birth and the majority will remain asymptomatic during childhood. We recommend follow-up to the age of 10 years.