Rubinstein-Taybi syndrome: Dental manifestations and management

South African Journal of Child Health

Karen Fieggen²  M Chetty³  Tina Sharon Roberts³  Lawrence Stephen³  Peter Beighton⁴  Michael Urban¹ 

[1] Division of Molecular Medicine and Human Genetics, Faculty of Medicine and Health Sciences, Stellenbosch University, Parow, Cape Town, South Africa;Division of Human Genetics, Faculty of Health Sciences, University of Cape Town, South Africa;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;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

关键词: Rubinstein-Taybi syndrome;    craniofacial;    dysmorphology;    genetic;    dental manifestations;   

DOI  :  

学科分类：农业科学（综合）

来源: Health and Medical Publishing Group

【 摘 要 】

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.

【 授权许可】

Unknown