A case of Hennekam syndrome presenting with massive pericardial effusion
TURK KARDIYOLOJI DERNEGI ARSIVI-ARCHIVES OF THE TURKISH SOCIETY OF CARDIOLOGY, vol.36, no.5, pp.325-328, 2008 (ESCI, Scopus, TRDizin)
- Publication Type: Article / Article
- Volume: 36 Issue: 5
- Publication Date: 2008
- Journal Name: TURK KARDIYOLOJI DERNEGI ARSIVI-ARCHIVES OF THE TURKISH SOCIETY OF CARDIOLOGY
- Journal Indexes: Emerging Sources Citation Index (ESCI), Scopus, TR DİZİN (ULAKBİM)
- Page Numbers: pp.325-328
- Keywords: Abnormalities, multiple, child, lymphangiectasis, lymphedema, pericardial effusion/etiology, syndrome
- Istanbul University Affiliated: Yes
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.