Polydactyly and ostium primum type atrial septal defect: Ellis-van Creveld syndrome

Orhan Veli Doğan; Murat Günday; Ergün Haliloğlu; Ali Rıza Akyüz

doi:10.5606/tgkdc.dergisi.2012.119

Orhan Veli Doğan¹, Murat Günday¹, Ergün Haliloğlu¹, Ali Rıza Akyüz²

¹Departments of Cardiovascular Surgery, Ahi Evren Thoracic and Cardiovascular Surgery Hospital, Trabzon, Turkey
²Departments of Cardiology, Ahi Evren Thoracic and Cardiovascular Surgery Hospital, Trabzon, Turkey

DOI : 10.5606/tgkdc.dergisi.2012.119

Comorbid polydactyly and atrial septal defect (ASD) is a very rarely seen anomaly. Basic clinical findings of Ellis-van Creveld syndrome include muscular skeletal disorders in the hands in 100% of cases, additional symmetrical postaxial polydactyly in the feet in 10-25% of cases, disproportionate dwarfism, a narrow thorax leading to respiratory problems, ectodermic dysplasia in the skin, nails, and teeth, a high palate, malocclusion, micrognathia, and congenital heart diseases, especially ASDs, in almost half of the patients (50-60%). In this article, we report a case with polydactyly and primum type ASD along with the surgical treatment and outcomes.

Keywords : Ellis-van Creveld syndrome; polydactyly; primum atrial septal defect

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