Abstract
Ataxia telangiectasia (AT) is a rare neurodegenerative condition which has not previously been reported in the dental literature. It is a life threatening and debilitating disorder of progressive cerebellar ataxia and choreoathetosis, progressive oculocutaneous telangiectasias, immunodeficiency with susceptibility to sinopulmonary infections, impaired organ maturation, X-ray hypersensitivity, and a high incidence of malignancies. Of importance in dentistry is the contra-indication for taking diagnostic radiographs. A 5-year-old girl with AT was referred by her paediatrician for dental care as she had never been seen by a dentist. She presented with caries affecting a number of primary teeth. Diagnostic radiographs could not be taken due to her X-ray hypersensitivity. She was given antibiotic prophylaxis for dental treatment under general anaesthetic. She had composite restorations, stainless steel crowns and extractions completed. She has been reviewed for over 2 years with ongoing enhanced prevention. This case reports a rare condition in which diagnostic dental radiographs are contra-indicated.
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