Does congenital diaphragmatic hernia associated with bronchopulmonary sequestration portend a better prognosis?

Congenital diaphragmatic hernia (CDH) continues to be a devastating disease in the newborn population, with well-documented morbidity and mortality. Bronchopulmonary sequestration is a separate congenital defect that has been associated with CDH. While the association of sequestration with CDH has been reported to be as high as 30-40%, the prognosis associated with the two simultaneous defects is unknown. We reviewed our experience to evaluate if prognosis was better in the CDH infants with associated bronchopulmonary sequestration.

Institutional approval was obtained. Our institutional database was examined from August 1995 to August 2005, identifying all mothers carrying fetuses with pulmonary masses and/or CDH and all neonates treated with bronchopulmonary sequestration and/or CDH. Patients who had both CDH and sequestration were identified by prenatal ultrasound reports, postnatal radiographs, and operative and pathology reports.

16 patients were identified in the fetal or neonatal period with concomitant diagnoses of CDH and bronchopulmonary sequestration. Of those proceeding to delivery, 6 expired and 6 survived. The presence of liver herniation and low lung-to-head ratio on antenatal ultrasound correlated with mortality. However, 2 patients survived with very low lung-to-head ratio that would usually be associated with 100% mortality at our institution. Two diagnoses of bronchopulmonary sequestration were reversed after final pathology revealed liver tissue.

Given the limited series, we cannot conclude that bronchopulmonary sequestration confers an anatomic advantage to patients that have CDH. We did observe survivors in this group that, given their antenatal predictors of CDH severity, would ordinarily have dismal prognosis. The presence of a sequestration may be protective in a subset of patients with severe CDH, or may confound our antenatal predictors of disease severity in these patients.