Clinical and radiological findings related to tuberous sclerosis complex: a case report.

Abstract

AIM:

The aim of this report is to present the intraoral and extraoral clinical features of a young female patient with tuberous sclerosis complex (TSC) who exhibited multiple hamartomas of various organ systems including a fibromatous growth on the gingiva.

BACKGROUND:

TSC is a rare neurocutaneous syndrome exhibiting multiple hamartomatous proliferations that may involve multiple organs such as the brain, kidney, heart, eyes, lungs and skin. Oral manifestations such as enamel pitting and fibromatous growth of the gingiva are commonly seen in patients with TSC.

REPORT:

A 16-year-old female patient presented with a painless interdental gingival growth in the maxillary left incisor and canine region that bled occasionally. Associated findings were adenoma sebaceum (angiofibromas) on the face, a Shagreen patch on the lumbosacral region, ash leaf spots on the trunk, and subangual fibromas (Koenen's tumor) on the nails of fingers and toes. The final diagnosis of TSC was made on the basis of the clinical findings of the skin, computerized tomography (CT) findings of the brain and kidney, ultrasonographic findings of kidney, and a histopathologic evaluation of the gingival growth which met the major and minor criteria required for a diagnosis of TSC.

SUMMARY:

This case report is a good example of the complex nature of patients with TSC. The diagnosis and management of these patients varies depending on the specific presentation of the disease. Dentists need to be mindful of the systemic issues as well as oral conditions related to this disease. Dental practitioners are advised to work closely with the patient's physician during treatment.

• Authors: Siddharth Gupta, Rahul Bhowate, Shirish S Degwekar
• Journal: The journal of contemporary dental practice (J Contemp Dent Pract) Vol. 9 Issue 4 Pg. 85-91 (May 01 2008) ISSN: 1526-3711 [Electronic] India
• PMID: 18473031 (Publication Type: Case Reports, Journal Article)

Topics

• Adolescent
• Angiofibroma (diagnosis)
• Cuspid (pathology)
• Diagnosis, Differential
• Facial Neoplasms (diagnosis)
• Female
• Fibroma (diagnosis)
• Gingival Diseases (diagnosis)
• Hamartoma (diagnosis)
• Humans
• Hyperpigmentation (diagnosis)
• Nail Diseases (diagnosis)
• Skin Neoplasms (diagnosis)
• Tuberous Sclerosis (diagnosis)