CONGENITAL PULMONARY LYMPHANGIECTASIA AND CHYLOTHORAX - A CASE SERIES

• Type: Article in International Scientific Journal
• Date: 2017

Authors

Rocha, G (Author) Other; Soares, P (Author) Other; Inês Azevedo (Author) FMUP; Baptista, MJ (Author) Other; Casanova, J (Author) Other; Moura, CS (Author) Other; Guimaraes, H (Author) Other

Journal

Title: Lymphology

Vol. 50

Pages: 188-196

ISSN: 0024-7766

Publisher: International Society of Lymphology

Indexing

ISI Web of Knowledge - 2 Citations

Scopus - 5 Citations

Other information

Authenticus ID: P-00N-VJ2

Abstract (EN): Congenital pulmonary lymphangiectasia (CPL) and chylothorax (CC) are rare lymphatic developmental disorders. We report six clinical cases of CPL and CC that were admitted to our level III neonatal intensive care unit over the last 20 years. One case of unilateral CC was successfully treated with pleuro-amniotic shunt; three cases of bilateral CC were associated to lung hypoplasia, hydrops fetalis, and generalized lymphangiectasias; one case of CPL was associated with obstructive congenital heart defect; one case of unilateral CC was successfully treated with thoracocentesis and medium-chain triglyceride diet. Mortality was high (66.6%).

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 9