2014 The Journal of craniofacial s…

General and stomatologic aspects of bardet-biedl syndrome.

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The Journal of craniofacial surgery Vol. 25 (6) : e575-8 • Nov 2014

OBJECTIVE: The purpose of this study is to present a clinical report of a patient with Bardet-Biedl syndrome, aiming to help the dentist to identify the general aspects, systemic changes, alterations. CLINICAL REPORT: Bardet-Biedl syndrome is defined as a genetic disorder of autosomal recessive condition; this case is a male patient, 20 years old. The general features presented by the patient were strabismus, polydactyly, hypogonadism, obesity, cognitive impairment, and autistic behavior. Dentally, this patient presents with hypotonic face, high-arched palate, dental crowding, unilateral crossbite, and difficulty in cleaning but with good-quality oral health. CONCLUSIONS: Many patients with this syndrome have compromised oral hygiene, difficulty in addressing dental cases, developmental delay, and autistic behavior. Thus, it is important to pay attention to these early children from an early age and encourage preventive approach.

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