Congenital Cystic Adenomatoid Malformation of the Lung

Abstract

A 3-day-old infant underwent successful lobectomy for congenital cystic adenomatoid malformation. The literature contains 61 reported cases, 21 (34.4%) of these infants were stillborn. Of the 40 living infants, 29 eventually underwent resection; three deaths occurred which were potentially avoidable. The 11 symptomatic infants who were not operated on died of progressive respiratory distress. Three patterns of clinical presentation are identifiable: (1) stillborn or neonatal death frequently associated with fetal anasarca, prematurity, and maternal polyhydramnios, (2) acute progressive respiratory distress in the newborn, and (3) a more indolent course characterized by pulmonary infections. The frequent association of anomalous pulmonary vessels is emphasized. Operation should be performed as soon as the diagnosis is established. The malformation may affect one or all lobes of one lung. We recommend removal of any involved lobe as well as any additional unexpandable pulmonary tissue.