A Case of Congenital Lymphatic Dysplasia Complicated by Hydrops Fetalis.

Yoon,  Young Mi; Kim,  Seon Nyo; Kim,  Hye Rim; Jung,  Young Hwa; Choi,  Chang Won; Kim,  Beyong Il

doi:2017.24.1.40

Neonatal Med > Volume 24(1); 2017 > Article

Case Report

Neonatal Medicine 2017;24(1):40-44.
DOI: https://doi.org/10.5385/nm.2017.24.1.40 Published online February 28, 2017.

A Case of Congenital Lymphatic Dysplasia Complicated by Hydrops Fetalis.

Young Mi Yoon, Seon Nyo Kim, Hye Rim Kim, Young Hwa Jung, Chang Won Choi, Beyong Il Kim

¹Department of Pediatrics, Seoul National University Hospital, Seoul, Korea.
²Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea. choicw1029@gmail.com

Abstract

Congenital lymphatic dysplasia is a rare congenital maldevelopment of the lymphatic system, in which dysfunction of the lymphatic system may cause leakage of lymph fluid into the limbs and the pleural, pericardial, or peritoneal cavity. We experienced a case of hydrops fetalis with subcutaneous lymphedema, chylothorax, chylous ascites and pericardial effusion. Lymphangiography revealed a critical defect of lymphatic system. Here, we report the first case of premature infant with congenital lymphatic dysplasia confirmed by lymphangiography, which is the first reported in Korea.

Key Words: Congenital lymphatic dysplasia; Lymphangiography; Hydrops fetalis