A Case of Primary Lymphatic Dysplasia Presented with Lymphedema and Chylothorax / 소아알레르기및호흡기학회지

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.