Case Reports

Rubinstein-Taybi syndrome: Dental manifestations and management

Tina Sharon Roberts, M Chetty, Lawrence Stephen, Michael Urban, Karen Fieggen, Peter Beighton

Abstract


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.


Authors' affiliations

Tina Sharon Roberts, Faculty of Dentistry, University of the Western Cape, Bellville, Cape Town, South Africa; University of the Western Cape/University of Cape Town Dental Genetics Clinic, Red Cross War Memorial Children’s Hospital, Cape Town, South Africa

M Chetty, Faculty of Dentistry, University of the Western Cape, Bellville, Cape Town, South Africa; University of the Western Cape/University of Cape Town Dental Genetics Clinic, Red Cross War Memorial Children’s Hospital, Cape Town, South Africa

Lawrence Stephen, Faculty of Dentistry, University of the Western Cape, Bellville, Cape Town, South Africa; University of the Western Cape/University of Cape Town Dental Genetics Clinic, Red Cross War Memorial Children’s Hospital, Cape Town, South Africa

Michael Urban, Division of Molecular Medicine and Human Genetics, Faculty of Medicine and Health Sciences, Stellenbosch University, Parow, Cape Town, South Africa

Karen Fieggen, Division of Human Genetics, Faculty of Health Sciences, University of Cape Town, South Africa

Peter Beighton, Division of Human Genetics, Faculty of Health Sciences, University of Cape Town, South Africa; University of the Western Cape/University of Cape Town Dental Genetics Clinic, Red Cross War Memorial Children’s Hospital, Cape Town, South Africa

Full Text

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Keywords

Rubinstein-Taybi syndrome; craniofacial; dysmorphology; genetic; dental manifestations

Cite this article

South African Journal of Child Health 2014;8(1):28-30. DOI:10.7196/SAJCH.569

Article History

Date submitted: 2013-01-24
Date published: 2014-02-05

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