Tuberous sclerosis complex: A case report

Hospital Dentistry Program, Department of Dental Practice, University of the Pacific Arthur, A. Dugoni School of Dentistry, San Francisco, California, USA.
Special Care in Dentistry 09/2008; 28(5):201-4. DOI: 10.1111/j.1754-4505.2008.00040.x
Source: PubMed


Tuberous Sclerosis Complex (TSC) was first described in the late 1800s as a relative of neurofibromatosis, but it has since been identified as a discrete disorder. Patients with TSC typically present with facial adenomas, seizure disorder, and a developmental disability. The syndrome is caused by mutations in either chromosomes 9 or 16, both of which code for cell development and maturation. This case report describes the unique findings of TSC, and suggests areas for future study. By understanding the dental and medical implications of TSC, early intervention may vastly improve a patient's overall quality of life.

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    ABSTRACT: Tuberous sclerosis is an inherited neurocutaneous disorder that occurs in approximately 1 in 7,500 live births. It is characterized by benign neoplasms of the skin, heart, kidneys, lungs, central nervous system, and mucosa. Oral lesions are usually fibrous in nature and most commonly occur on the anterior gingivae. Patients may suffer with epilepsy and learning difficulties, which may complicate dental management. When a 10-year-old girl with a history of tuberous sclerosis was brought in for a routine dental check-up, a leafy growth on the anterior gingiva was detected. An excisional biopsy was carried out. Histologically, the appearance was described as nonspecific, but was consistent with a diagnosis of tuberous sclerosis. Tuberous sclerosis is a rare condition that may exhibit oral manifestations. The patients may also exhibit epilepsy and learning difficulties. Dental management of affected patients may therefore be more complicated and complex.
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