Clinico-pathological characteristics of congenital pulmonary lymphangiectasis: Report of two cases

Minseob Eom, Duk Choi Yoo, Shin Kim Youn, Mee Yon Cho, Soon Hee Jung, Young Lee Han

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)740-745
Number of pages6
JournalJournal of Korean medical science
Volume22
Issue number4
DOIs
Publication statusPublished - 2007 Aug

All Science Journal Classification (ASJC) codes

  • Medicine(all)

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