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Gorlin-Goltz Syndrome with Jaw and Minimal Skin Manifestations - A Case Report
Anand Mohan Nikunj1, Biswajit Mishra1, Bathri Elangovan1
1Department of Oral and Maxillofacial Surgery, Government Dental College, Raipur, Chhattisgarh, India.
Rationale:
Multiple odontogenic keratocysts (OKC) occurs as a component of nevoid basal cell carcinoma syndrome (NBCCS) with concomitant cutaneous, skeletal, ophthalmic and neurological abnormalities.
Patient Concerns:
A 15-year-old male patient reported with chief complaint of swelling in his upper and lower jaws.
Diagnosis:
Orthopantomogram was taken and it showed multiple radiolucent areas present in the maxilla and mandible. Aspiration of the cystic lesion was done under local anaesthesia and it yielded a cheesy exudate which is characteristic of OKC.
Treatment:
Complete enucleation of the cyst with chemical cauterisation with Carnoy's solution and peripheral ostectomy was done under general anaesthesia.
Outcome:
He was under follow-up for the past 5 years, and there was no recurrence.
Take-Away Lesson:
Multiple OKCs have been known to occur most commonly in syndromic cases. These multiple lesions may be the first manifestation of the NBCCS, or otherwise, they may be because of the multifocal nature of OKCs.
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