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General and Stomatologic Aspects of Bardet-Biedl Syndrome

Ferreira do Amaral, Cristhiane Olivia DDS, MSc*; Logar, Gustavo de Almeida DDS, MSc*; Parisi, Arlete Gomes DDS, MSc*; Takahashi, Karine DDS, MSc; Straioto, Fabiana Gouveia DDS, MSc

The Journal of Craniofacial Surgery: November 2014 - Volume 25 - Issue 6 - p e575–e578
doi: 10.1097/SCS.0000000000001169
Brief Clinical Studies
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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.

From the Departments of *Special Care Dentistry, †Pediatric Dentistry, and ‡Prosthodontics and Geriatic Dentistry, Dental School, University of Western Sao Paulo, Presidente Prudente, Sao Paulo, Brazil.

Received April 29, 2014.

Accepted for publication June 19, 2014.

Address correspondence and reprint requests to Cristhiane Olivia Ferreira do Amaral, DDS, MSc, Department of Special Care Dentistry, University of Western Sao Paulo, Rua Jose Bongiovani, 700, Presidente Prudente, SP, Brazil 19050-920; E-mail: crisamaral@unoeste.br

The authors report no conflicts of interest.

© 2014 by Mutaz B. Habal, MD.