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- Title
Gorlin: Goltz syndrome: A case report.
- Authors
Sahu, Manisha; Nayak, Sushruth; Shrivastava, Sandhya
- Abstract
Introduction: Gorlin-Goltz syndrome is an inherited autosomal dominant disorder uncommonly found and involves multiple organ system. The incidence reported worldwide ranges from 1 in 50,000 to 1 in 150,000. Syndrome comprises the triad of basal cell nevi, jaw keratocysts and skeletal anomalies. A spectrum of other neurological, ophthalmic, endocrine and genital manifestations is known to be variably associated with this triad. The syndrome has numerous diagnostic criteria, but only two major and one minor criteria or one major and three minor criteria are required to arrive at a diagnosis. The keratocystic odontogenic tumor is frequently the presenting manifestation of this syndrome. Case Report: The present paper highlights an incidental detection of Gorlin-Goltz syndrome with multisystem anomalies in a 14-year-old female patient, who had reported for the swelling in the left anterior region of the mandible. In our patient, diagnosis of Gorlin syndrome was established by the presence of two major and four minor criteria. Recurrence in these lesions is the most characteristic feature that was seen in our case. The patient was treated surgically by enucleation and vigorous curettage. Conclusion and Summary: Thus a rare entity, Gorlin-Goltz syndrome, was diagnosed and treated. As the patient is presented to the dental surgeon early than to any other doctors, the responsibility of the dental surgeon is very important for early diagnosis without making further delay. But a long-term follow-up for the entire life is mandatory in the management of the syndrome.
- Subjects
BASAL cell nevus syndrome; BONE abnormalities
- Publication
Journal of Oral & Maxillofacial Pathology (0973029X), 2014, Vol 18, pS109
- ISSN
0973-029X
- Publication type
Article