Expect the unexpected – A rare case of Orthokeratinized Odontogenic Cyst and its surgical management

Orthokeratinized Odontogenic Cyst (OOC) is a rare, developmental odontogenic cyst of the dental lamina. It was initially defined as the uncommon orthokeratinized variant of the Odontogenic Keratocyst (OKC), until the World Health Organization’s (WHO’s) classification in 2005 and 2017, where it was separated from the Keratocystic Odontogenic Tumor (KCOT) and has been included as a separate entity from the category of developmental odontogenic cysts respectively. It presents as a unilocular radiolucent lesion involving the posterior mandible and is frequently related to impacted teeth, often similar to other odontogenic cysts. Due to low local aggressiveness and less proliferative activity, it has to be differentiated from the other cysts in terms of surgical management. Here we report a rare case of OOC involving the maxilla along with an impacted canine and discuss the surgical management and why a secondary surgical intervention is unnecessary.

A Rare Odontogenic Cyst in an Uncommon Area: Case Report and Review of the Literature

Objective Orthokeratinized odontogenic cyst is a rare developmental odontogenic cyst of the jaws. It is a less aggressive intraosseous cyst identified by an orthokeratinized epithelium. Case Report A 50-year-old male patient with the chief complaint of swelling in the anterior part of his face, and, intraorally, there was diffuse swelling in the palatal cortex. On panoramic radiography, there was a well-defined unilocular radiolucency on the right side of the maxilla and palatal cortical expansion, and thinning of the buccal and palatal cortexes was observed. The histopathological examination revealed a pathologic cyst that was lined by a thick orthokeratinized epithelium. Therefore, the diagnosis was orthokeratinized odontogenic cyst. Conclusion The orthokeratinized odontogenic cyst displays characteristic clinical, histopathological, and biological features that differ significantly from those of keratocystic odontogenic tumor (KCOT), but it has a better prognosis and lower recurrence rate. Thus, other radiolucent lesions of the jaws, including keratocystic odontogenic tumor (KCOT), must be considered in the differential diagnosis.