[Year:2019] [Month:January-April] [Volume:9] [Number:1] [Pages:7] [Pages No:46 - 52]
Aim: The aim of this study is to highlight a behavioral pattern of odontogenic keratocysts (OKCs) in syndromic and nonsyndromic patients in terms of clinical course, genetic makeup, local destruction, management modalities, and recurrences such that detection in initial stages and intervention when done prevents extensive morbidity and a better quality of life.
Background: OKC is a common developmental odontogenic cyst affecting the oral and the maxillofacial region, which arises from the dental lamina or its remnants. Multiple OKCs are usually seen in association with the nevoid basal cell carcinoma syndrome (NBCCS) but approximately 5% of the cases have multiple cysts without concomitant syndromic presentation. As it spreads by way of the bone marrow, it results in more destruction before any clinical manifestation appears. Although lesions are benign but are aggressive, locally destructive, and highly recurrent.
Case description: A 15-year-old male patient, a rare case of nonsyndromic multiple OKCs, is presented here along with a review of literature with emphasis on the importance of thorough evaluation, diagnosis, treatment strategy, and strict follow-up.
Conclusion: Although our case does not fulfill the major and minor criteria of syndrome, but considering the reported literature and clinical presentation, early age, multiple OKCs, involvement of both the jaws, cleft lip and cleft palate, and histological correlation, we suggest our case to be a partial expression of NBCCS.
Clinical significance: Any case presenting with single or multiple OKCs should be thoroughly investigated locally and systemically for any other signs of NBCCS or other syndrome. A definite treatment protocol should be formulated depending on patient's age, number and size of lesion, remaining subsequent growth, and proximity to adjacent vital structures, to provide a better quality of life with a minimal amount of morbidity and kept on strict regular follow-up.