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Ellis-van Creveld Syndrome Associated With Thymic Hypoplasia

Haci Akar, MD, Cüneyt Konuralp, MD¹, Kemal Baysal, MD², Ferat Kolbakir, MD

Department of Cardiovascular Surgery 2 Department of Pediatric Cardiology Ondokuz Mayıs University Faculty of Medicine Samsun, Turkey 1 Department of Cardiovascular Surgery Siyami Ersek Thoracic and Cardiovascular Surgery Center Istanbul, Turkey
For reprint information contact: Cüneyt Konuralp, MD Tel: 90 216 349 9120 Fax: 90 216 363 3642 email: ckonuralp{at}usa.net Aye Çavu Sokak, No: 7/6, Huri Apt., Suadiye, Istanbul, 81070, Turkey.

A 16-month-old girl presented with the classic signs of Ellis-van Creveld syndrome. Mitral clefts were repaired by direct suturing. The single atrium was repaired by creating a new septum with a pericardial patch, leaving the coronary sinus in the left atrium. During perioperative exploration, thymic hypoplasia was recognized.