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IP International Journal of Maxillofacial Imaging


Scheithauer-Marie-Sainton Syndrome


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Author Details: Kundoor Vinay Kumar Reddy, Krishna Priya Pasupuleti, Anurag Ellanki, Kotya Naik Maloth, Kesidi Sunit

Volume : 2

Issue : 2

Online ISSN : 2581-3838

Print ISSN : 2581-382X

Article First Page : 85

Article End Page : 87


Abstract

Cleidocranial dysplasia (CCD) is an autosomal dominant developmental anomaly with craniofacial, skeletal and dental malformations. The gene causing CCD has been mapped to chromosome 6p21 within a region containing CBFA1, a member of the runt family of transcription factors. It is characterised by failure of membranous ossification resulting in absence or pseudoarthrosis of clavicle, open fontenelles, impacted and supernumerary teeth. The short stature, long and narrow neck with marked droopeding shoulders. CCD patients exhibit prognathic mandibular appearance due to maxillary hypoplasia and also exhibit relatively short cranial base. Here with describing a case of Scheithauer-Marie-Sainton syndrome, in a 30 year old male patient.

Key words:
Brachycephalic, Hypertelorism, Hypoplastic, Supernumerary teeth