Pediatr Allergy Respir Dis.  2001 Mar;11(1):61-66.

A Case of Primary Lymphatic Dysplasia Presented with Lymphedema and Chylothorax

• Kim SY
• Kim BJ
• Ma JS

Abstract

Primary lymphatic dysplasia is a congenital maldevelopment of the lymphatic system, interfering with its function and causing an effusion of chyle or lymph into the limbs and pleural or peritoneal cavity. We describe a case of primary lymphatic dysplasia in a 34-months old girl, presented with lymphedema at birth and refractory chylothorax. This case was treated by microlymphaticovenous anastomosis on 16 days after birth. Conservative therapy, such as total parentral nutrition with repeated thoracentesis was ineffective to control chylothorax. Ligation of the thoracic duct was performed.

Keyword

Primary lymphatic dysplasia; Lymphedema; Chylothorax; Child

MeSH Terms

Child
Child, Preschool
Chyle
Chylothorax*
Extremities
Female
Humans
Ligation
Lymphatic System
Lymphedema*
Parturition
Peritoneal Cavity
Thoracic Duct