A Case of Congenital Lymphatic Dysplasia Complicated by Hydrops Fetalis.

Author: Young Mi YOON ¹ ; Seon Nyo KIM ; Hye Rim KIM ; Young Hwa JUNG ; Chang Won CHOI ; Beyong Il KIM
Author Information

1. Department of Pediatrics, Seoul National University Hospital, Seoul, Korea.
Publication Type:Case Report
Keywords: Congenital lymphatic dysplasia; Lymphangiography; Hydrops fetalis
MeSH: Chylothorax; Chylous Ascites; Edema*; Extremities; Humans; Hydrops Fetalis*; Infant, Newborn; Infant, Premature; Korea; Lymphatic System; Lymphedema; Lymphography; Pericardial Effusion; Peritoneal Cavity
From:Neonatal Medicine 2017;24(1):40-44
CountryRepublic of Korea
Language:Korean
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.