title: [A case of Hennekam syndrome presenting with massive pericardial effusion].
abstract: Hennekam syndrome is an autosomal recessive disease characterized by intestinal lymphangiectasia accompanied by severe lymphedema of the limbs, genitalia, and face, and learning difficulties. A 38-month-old boy was admitted with breathing difficulty. He had facial abnormalities and preputial hyperplasia consistent with Hennekam syndrome. Lymphangiography showed lymphedema in the left eye and right foot. Teleradiography showed cardiomegaly and echocardiography showed massive pericardial effusion. He first underwent pericardiocentesis for the removal of pericardial effusion, but pericardial tube drainage was required upon recurrence of effusion. On the fifth day, the drain was removed because of significant decrease in the drainage.
authors.affiliation: Istanbul Universitesi Istanbul Tip Fakültesi, Cocuk Sağliği ve Hastaliklari Anabilim Dali, Istanbul, Turkey. kemalnisli@yahoo.com
authors.forename: Kemal
authors.initials: K
authors.lastname: Nişli
authors.forename: Naci
authors.initials: N
authors.lastname: Oner
authors.forename: Hülya
authors.initials: H
authors.lastname: Kayserili
authors.forename: Türkan
authors.initials: T
authors.lastname: Ertuğrul
mesh_terms: D000015:Abnormalities, Multiple; D002675:Child, Preschool; D006801:Humans; D008201:Lymphangiectasis, Intestinal / Q000150:complications / Q000151:congenital*; D008208:Lymphatic System / Q000002:abnormalities*; D008209:Lymphedema / Q000150:complications / Q000151:congenital*; D008297:Male; D010490:Pericardial Effusion / Q000175:diagnosis* / Q000209:etiology*; D013577:Syndrome