Primary pulmonary lymphangiectasia in an infant with Trisomy 21

Abstract. Primary pulmonary lymphangiectasia (PPL) is a rare developmental disorder affecting the pulmonary lymphatics, often present antenatally with pleural effusions and hydrops. Neonatal presentation is almost always fatal. It has been associated with Turner’s and Noonan’s syndromes, but the association of Trisomy 21 with PPL is rare. We report on a term infant with Trisomy 21 and pulmonary lymphangiectasia presenting as nonimmune hydrops and bilateral persistent chylothoraces.

*Corresponding Author: 

Dr. Amuchou S. Soraisham, Division of Neonatology, Department of Pediatrics, Foothills Medical Centre, Rm C211, 1403-29th Street, NW, Calgary, Alberta, AB, Canada. Tel.: +1 403 944 4638; Fax: +1 403 944 4892; E-mail: