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Oman Med J. 2013 Mar;28(2):128-30. doi: 10.5001/omj.2013.33.

Kocher debre semelaigne syndrome: a rare case report with orofacial manifestations.

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1
Head of department, Department of Oral Medicine and Radiology, Institute of Dental Sciences, Bareilly (UP), India.

Abstract

Kocher Debre Semelaigne Syndrome is a rare syndrome with clinical manifestation of hypothyroidism associated with pseudomuscular hypertrophy. Very few cases have been reported on kocher-debre semelaigne syndrome in the literature with the incidence reported to be less than 10%. As per our knowledge; until now, only one case of oro-facial manifestations has been reported. Here we report one such case of Kocher debre semelaigne syndrome in an 11 year old boy, showing features of macroglossia, multiple impacted teeth, puffed lips and anterior open bite.

KEYWORDS:

Hypothyroidism; Kocher Debre Semelaigne syndrome (KDSS); Myopathy; Orofacial aspects

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