A fetal lung lesion consisting of bronchogenic cyst, bronchopulmonary sequestration, and congenital cystic adenomatoid malformation: The missing link?

A fetus was found to have a large left thoracic cyst on routine prenatal ul trasound at 23 weeks of gestation. This lesion caused compression of the no rmal left lung tissue and contralateral mediastinal shift. At 23 weeks of g estation the cyst was percutaneously aspirated without subsequent reaccumul ation of fluid. Serial ultrasounds showed decrease in the size of the cyst. The clinical diagnosis of congenital cystic adenomatoid malformation was m ade, At birth, the child had no respiratory distress, and a CT scan confirm ed the finding of a fluid-filled cyst in the left chest. At the time of res ection, a nonaerated extralobar bronchopulmonary sequestration (with a syst emic arterial blood supply and separate pleural investment) was found, The dominant cyst had ciliated respiratory epithelium with cartilage, indicativ e of a bronchogenic cyst, and the remainder of the specimen had the histolo gic hallmarks of a congenital cystic adenomatoid malformation. The coexiste nce of three separate anomalies in one lesion suggests a common embryologic al link for these malformations. Copyright (C) 2001 S.Karger AG,Basel.