Neonatal Med.  2017 Feb;24(1):40-44. 10.5385/nm.2017.24.1.40.

A Case of Congenital Lymphatic Dysplasia Complicated by Hydrops Fetalis

Affiliations
  • 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.

Keyword

Congenital lymphatic dysplasia; Lymphangiography; Hydrops fetalis

MeSH Terms

Chylothorax
Chylous Ascites
Edema*
Extremities
Humans
Hydrops Fetalis*
Infant, Newborn
Infant, Premature
Korea
Lymphatic System
Lymphedema
Lymphography
Pericardial Effusion
Peritoneal Cavity
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