ISSN 1016-5169 | E-ISSN 1308-4488
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A case of Hennekam syndrome presenting with massive pericardial effusion [Turk Kardiyol Dern Ars]
Turk Kardiyol Dern Ars. 2008; 36(5): 325-328

A case of Hennekam syndrome presenting with massive pericardial effusion

Kemal Nişli1, Naci Öner1, Hülya Kayserili2, Türkan Ertuğrul1
1Istanbul University, Istanbul Medical Faculty, Department Of Pediatric Cardiology, Istanbul, Turkey
2Istanbul University, Istanbul Medical Faculty, Department Of Medical Genetics, Istanbul, Turkey

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.

Keywords: Abnormalities, multiple, child, lymphangiectasis; lymphedema; pericardial effusion/etiology; syndrome.

How to cite this article
Kemal Nişli, Naci Öner, Hülya Kayserili, Türkan Ertuğrul. A case of Hennekam syndrome presenting with massive pericardial effusion. Turk Kardiyol Dern Ars. 2008; 36(5): 325-328

Corresponding Author: Kemal Nişli, Türkiye
Manuscript Language: Turkish


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