Abstract
Odontogenic keratocysts (OKCs) may occur in two different forms, either as solitary (non syndromic OKCs) or as multiple OKCs (syndromic OKCs). Multiple OKCs are usually associated with Gorlin–Goltz syndrome with features like skin carcinomas and bifid ribs, eye, and neurologic abnormalities. We report a rare case of Gorlin–Goltz syndrome in a 35-year-old male patient who presented with a swelling in lower left back teeth region since 1 week. Apart from these, other findings observed in the patient were frontal bossing, depressed nasal bridge, ocular hypertelorism, prominent supra orbital ridge, and mild mandibular prognathism. On the basis of clinical and radiological evaluation, Enucleation was planned in all the three quadrants and histopathological evaluation revealed multiple OKC’s. Patient was followed-up multiple times for the duration of six months. This case report highlights the important findings and presentation of a rare case of Gorlin Goltz syndrome along with its review of literature. Keywords: Odontogenic keratocyst, Basal cell carcinoma, Bifid ribs, Gorlin Goltz Syndrome.
Highlights
Odontogenic keratocysts (OKCs) are cysts of developmental origin arising from remnants of dental lamina.[1]
A periodic follow-up is recommended for these lesions due to their high recurrence rates. 4,5 This paper is presenting a rare case of Gorlin–Goltz syndrome with a brief discussion about its pathogenesis, diagnostic criteria, and differences between syndromic and asyndromic OKCs
Gorlin–Goltz syndrome is usually associated with multiple OKCs as its first manifestation; dentists have an important role in its early detection thereby achieving proper management of this syndrome
Summary
Odontogenic keratocysts (OKCs) are cysts of developmental origin arising from remnants of dental lamina.[1] They may appear either as single entity or as multiple cysts associated with syndromes like Gorlin–Goltz syndrome. 2 Jarish and White (1984) first reported this syndrome and later, Gorlin and Goltz documented other features such as multiple jaw cysts, nevoid basal cell carcinomas (BCCs), bifid ribs, and other features; this lesion is called Gorlin–Goltz syndrome or basal cell nevus syndrome or jaw cyst bifid rib syndrome, or multiple nevoid BCC syndrome. Various treatment modalities have been suggested in literature for multiple OKCs. For small lesions enucleation is preferred and for larger lesions marsupialization is preferred. The application of Carnoy’s solution has been suggested to prevent recurrences. 4,5 This paper is presenting a rare case of Gorlin–Goltz syndrome with a brief discussion about its pathogenesis, diagnostic criteria, and differences between syndromic and asyndromic OKCs A periodic follow-up is recommended for these lesions due to their high recurrence rates. 4,5 This paper is presenting a rare case of Gorlin–Goltz syndrome with a brief discussion about its pathogenesis, diagnostic criteria, and differences between syndromic and asyndromic OKCs
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