Gorlin-Goltz Syndrome — ORE Part 1 MCQ

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ModerateOral PathologyGorlin-Goltz SyndromeORE Part 1

Which syndrome is most strongly associated with the development of odontogenic keratocysts?

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Correct answer: E — Gorlin-Goltz syndrome

Gorlin-Goltz syndrome (also called nevoid basal cell carcinoma syndrome) is the recognised syndromic association with odontogenic keratocysts (OKCs), which may be multiple and can present early. Therefore, multiple OKCs should prompt consideration of this diagnosis. Gardner syndrome is associated with colorectal polyposis and may produce dental abnormalities, osteomas and impacted or supernumerary teeth, but OKCs are not a characteristic feature. Turner syndrome and Down syndrome have recognised craniofacial and dental findings but not a specific association with OKCs. Paget disease affects bone remodelling and may cause jaw enlargement and altered radiographic appearances, rather than odontogenic keratocysts.

Reference: Chandran S, Marudhamuthu K, Riaz R, Balasubramaniam S. Odontogenic Keratocysts in Gorlin-Goltz Syndrome: A Case Report. Journal of International Oral Health. 2015. https://pubmed.ncbi.nlm.nih.gov/26225111/