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Tuberous sclerosis: a case report.

K L Girish Babu¹, Kavitha Rai, Amitha M Hegde

¹Department of Pedodontics and Preventive Dentistry, A.B. Shetty Memorial Institute of Dental Sciences, Deralakatte, Mangalore-575018, Karnataka, India.

The Journal of Clinical Pediatric Dentistry

|September 16, 2004

Summary

Tuberous sclerosis (TS) is a genetic disorder presenting with seizures, intellectual disability, and skin lesions. This case highlights TS-associated self-harm behaviors and dental trauma, managed with dental treatment and protective measures.

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Area of Science:

Neurology
Genetics
Dermatology

Background:

Tuberous sclerosis (TS) is a rare genetic disorder characterized by the development of hamartomas in multiple organs.
The classic triad of TS includes seizures, intellectual disability, and facial angiofibromas.
Behavioral issues, including self-mutilation, can occur in individuals with TS.

Observation:

A pediatric case of tuberous sclerosis (TS) is presented.
The patient exhibited the classic TS triad: seizures, mental deficiency, and angiofibromas.
Additionally, the child presented with self-mutilating behavior leading to fractured anterior teeth.

Findings:

Comprehensive dental treatment was performed under general anesthesia to address the fractured teeth.
A custom mouth guard was fabricated and provided to the patient.

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The mouth guard aims to prevent further traumatic dental injuries.

Implications:

This case underscores the importance of recognizing and managing behavioral manifestations in TS.
Early intervention and protective dental measures are crucial for patients with TS and self-harm behaviors.
Multidisciplinary care is essential for optimizing outcomes in individuals with tuberous sclerosis.