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Gorlin syndrome with bilateral polydactyly: a rare case report
Sonu Acharya1, Swagatika Panda2, Kanika Singh Dhull3
1Reader, Department of Pedodontics and Preventive Dentistry, Institute of Dental Sciences, SOA University, Bhubaneswar, Odisha, India.
International Journal of Clinical Pediatric Dentistry
|September 11, 2014
Summary
Gorlin syndrome, a rare genetic disorder, was diagnosed in an 11-year-old boy with multiple odontogenic keratocysts. This case highlights the importance of thorough clinical and radiological evaluation for early diagnosis.
Area of Science:
- Dentistry
- Genetics
- Pediatrics
Background:
- Gorlin syndrome, an autosomal dominant disorder, affects multiple body systems.
- Odontogenic keratocysts are a common feature, often presenting in the jaw.
- Early diagnosis is crucial for managing potential complications.
Observation:
- An 11-year-old male presented with multiple odontogenic keratocysts.
- Clinical examination and radiological investigations were performed.
- Bilateral polydactyly was also noted in this case.
Findings:
- The patient was diagnosed with Gorlin-Goltz syndrome.
- The presence of multiple odontogenic keratocysts led to the diagnosis.
- This case report details a rare presentation of Gorlin syndrome.
Implications:
- Highlights the significance of dentalpathology in diagnosing systemic genetic disorders.
- Emphasizes the need for comprehensive evaluation in pediatric patients with keratocysts.
- Contributes to the understanding of Gorlin syndrome's varied clinical manifestations.
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