Clinico-pathological Characteristics of Congenital Pulmonary Lymphangiectasis: Report of Two Cases.

Clinico-pathological Characteristics of Congenital Pulmonary Lymphangiectasis: Report of Two Cases.

J Korean Med Sci. 2007 Aug

Eom M, Choi YD, Kim YS, Cho MY, Jung SH, Lee HY.

Department of Forensic Medicine, Western District Office, National Institute of Scientific Investigation, Jangseong, Korea.

Department of Pathology, Wonju College of Medicine, Yonsei University, Wonju, Korea. soonheej@yonsei.ac.kr

Congenital pulmonary lymphangiectasis (CPL) is a rare, poorly documented disease, characterized by abnormal dilatation of pulmonary lymphatics without lymphatic proliferation. This disease is seen almost exclusively in infancy and early childhood. It can usually be divided into primary (congenital) and secondary forms. The primary form presents in neonates, and the patients mostly die due to the respiratory distress, shortly after birth. The authors experienced two cases of primary CPL in a 13-day-old male neonate and a one-day-old male neonate, showing prominent lymphatic dilatation in the septal, subpleural, and peri-bronchial tissue throughout both lungs. The latter case was associated with congenital cardiac anomaly including single ventricle. These are unique cases of CPL in Korea of which the diagnosis was established through post-mortem examination. Therefore, the authors report these two cases with primary CPL with a review of the literature.

Journal of Korean Medical Science