Rubinstein-Taybi syndrome: Dental manifestations and management

Rubinstein-Taybi syndrome (RSTS) is an uncommon genetic disorder characterised by a typical facies, small stature, broad angulated thumbs and intellectual impairment. Dental changes are a minor, yet significant component of the condition. Craniofacial growth retardation in RSTS is frequently complic...

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Published inSAJCH : the South African journal of child health Vol. 8; no. 1; pp. 28 - 30
Main Authors Roberts, Tina Sharon, Chetty, M, Stephen, Lawrence, Urban, Michael, Fieggen, Karen, Beighton, Peter
Format Journal Article
LanguageEnglish
Portuguese
Published Health & Medical Publishing Group 01.02.2014
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Summary:Rubinstein-Taybi syndrome (RSTS) is an uncommon genetic disorder characterised by a typical facies, small stature, broad angulated thumbs and intellectual impairment. Dental changes are a minor, yet significant component of the condition. Craniofacial growth retardation in RSTS is frequently complicated by unerupted teeth, while dental caries is related to the inherent intellectual deficit. Dental problems necessitate interdisciplinary management in terms of oral surgery, conservative dentistry, periodontics and orthodontics. When affected individuals are unco-operative, certain dental procedures may warrant general anaesthesia. In these instances, dental and medical staff will combine their expertise to enhance the well-being of the patient. In addition, specific dental changes may alert the medical practitioner to the possible diagnosis of RSTS. In this article we document the oro-dental manifestations and review the oro-dental approach in the management of three patients with RSTS. Our experience in South Africa may be relevant to other countries at a similar stage of development.
ISSN:1994-3032
1999-7671
1999-7671
DOI:10.7196/sajch.569