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Wednesday, November 12, 2008

A case of Hennekam syndrome presenting with massive pericardial effusion.]

A case of Hennekam syndrome (lymphangiectasia) presenting with massive pericardial effusion.
Turk Kardiyol Dern Ars. 2008 Jul
Nişli K, Oner N, Kayserili H, Ertuğrul T.
Department of Pediatrics, Istanbul Medicine Faculty of Istanbul University, 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.

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