April 3, 2002 --
1- Isolated Pulmonary: The developmental defect is isolated to the pulmonary lymphatics. Felt to be related to failure of regression of the pulmonary lymphatics after the 16th week of gestation. It results in severe interstitial lung disease and has a poor prognosis. Rarely children may live beyond the neonatal period.
2- Secondary to Pulmonary Venous Obstruction: This form is associated with congenital heart disease and accounts for 30% of cases. Cardiac abnormalities include: Hypoplastic left heart, total anomalous pulmonary venous return type III, and pulmonic vein atresia.
3- Generalized: (Systemic)- Characterized by lymphangiectasia throughout the body. The pulmonary manifestations are typically mild. Patients may have an associated lymphangioma.
(1) AJR 1999; Chung CJ, et al. Children with congenital pulmonary lymphangiectasia: After infancy. 173: 1583-1588