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Clinical Spectrum of Cleidocranial Dysplasia: A Case Report

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Author(s): P Sakhi, P Yadav, R Susmitha, A Chawla, CJ Yadav, J Gupta

Journal: National Journal of Community Medicine
ISSN 0976-3325

Volume: 1;
Issue: 2;
Start page: 162;
Date: 2010;
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Keywords: Autosomal Dominant | Marie and Sainton disease | Mutational dysostosis | Cleidocranial dysostosis.

ABSTRACT
Background: The developmental anomaly involving skeleton and teeth is Cleidocranial dysplasia. It is an autosomal disorder with equal sex distribution. It is also known as Marie and Sainton disease, Mutational dysostosis and Cleidocranial dysostosis. Skeletal defects of several bones are noted in this disorder in the form of partial or complete absence of clavicles, late closure of the fontanels, presence of open skull sutures and multiple wormian bones. Case presentation: In this case report, we describe an otherwise healthy 28year-old male with a chief complaint of chronic pus discharge from the mandibular left canine and premolar edentulous region. Conclusion: Cleidocranial dysplasia is very rare in occurrence, incidence being 1: 1,000,000. Since early diagnosis of Cleidocranial dysplasia is essential for initiating the appropriate treatment approach, clinicians should be aware of the characteristic features. We report a case of Cleidocranial dysplasia because of its rarity.
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