Successful pericardiectomy in Hennekam syndrome

Zehra Karataş; Sevim Karaarslan; Hayrullah Alp; Ali Sarıgül; Tamer Baysal

doi:10.5606/tgkdc.dergisi.2013.6137

Zehra Karataş¹, Sevim Karaarslan¹, Hayrullah Alp¹, Ali Sarıgül², Tamer Baysal¹

¹Necmettin Erbakan Üniversitesi Meram Tıp Fakültesi, Çocuk Kardiyoloji Bilim Dalı, Konya, Türkiye
²Necmettin Erbakan Üniversitesi Meram Tıp Fakültesi, Kalp ve Damar Cerrahi Anabilim Dalı, Konya, Türkiye

DOI : 10.5606/tgkdc.dergisi.2013.6137

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Hennekam syndrome is an autosomal recessive disorder, characterized by lymphangiectasia in internal organs with lymphedema. Pleural or pericardial effusions may present with lymphedema on extremities in the course of the disease. Although a poor long-chain fatty diet is given for treatment, its success rate is low. In this article, we report the results of a successful pericardiectomy performed on a 21-month-old boy who was diagnosed with Hennekam syndrome.

Keywords : Hennekam syndrome; pericardial effusion; pericardiectomy

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