ISSN : 1301-5680
e-ISSN : 2149-8156
TURKISH JOURNAL OF
THORACIC AND
CARDIOVASCULAR SURGERY
Turkish Journal of Thoracic and Cardiovascular Surgery     
A CASE OF ELLIS-VAN SYNDROME WITH THIMIC HYPOPLASIA ANT IT’S SURGICAL REPAIRE (CASE REPORT)
Hacı AKAR, M. Kemal DEMİRAĞ, H. Tahsin KEÇELİGİL, *Kemal BAYSAL
Ondokuz Mayıs Üniversitesi Tıp Fakültesi, Kalp ve Damar Cerrahisi Anabilim Dalı
*Ondokuz Mayıs Üniversitesi Tıp Fakültesi, Pediatrik Kardiyoloji Anabilim Dalı
Ellis-van Creveld syndrome (EvC), also called chondroectodermal dysplasia, is a rare occurrence inherited as an autosomal recessive disease and characterized by chondrodysplasia, ectodermal dysplasia, polydactyly and congenital cardiac defects. As congenital cardiac defects, single atrium and endocardial cushion defects are frequent.

In our clinic, a 16 months old girl presenting the classical findings of EvC syndrome, was operated succesfully. Mitral clefts were repaired by direct suturing, single atrium was repaired by creating a new septum with a pericardial patch and coronary sinüs was left in the left atrium. During the peroperatuar exploration, thimic hypoplasia was recognized. We couldn’t find any other cases of EvC syndrome with thimic hypoplasia in the literature.

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