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Greig Cephalopolysyndactyly Syndrome with Oral Manifestations: A Rare Case Report
Jiji V Unni1, Deepak Daryani1, K C Sreejan1
1Department of Oral Medicine and Radiology, Malabar Dental College and Research Centre, Edappal, Kerala, India.
Abstract:
Greig cephalopolysyndactyly syndrome (GCPS) is one of the autosomal dominant-inherited syndromes, caused by haploinsufficiency of the GLI3 gene. It is a rare, multiple congenital syndrome with an estimated rate of 0.009%. With the classic clinical triad of preaxial polydactyly with cutaneous syndactyly of at least one limb, hypertelorism, and macrocephaly, presumptive diagnosis of GCPS is made. The purpose of this article is to report a case of GCPS with emphasis on craniofacial and oral features.
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