The syndromic multiple odontogenic keratocyst in siblings: A familial study

Our aim is to demonstrate the importance of postoperative assessment and highlight the need for a lifetime follow-up of the patient and the siblings in cases of Nevoid Basal Cell Carcinoma Syndrome (NBCCS).

Three siblings out of which two were of syndromic multiple odontogenic keratocysts, with multiple basal cell nevae were followed-up for manifestations of NBCCS from year 2001 till date. Two of the patients were treated for multiple bilateral odontogenic keratocysts (OKCs). Familial occurrence of the syndromic multiple odontogenic keratocysts was studied.

Although NBCCS is associated with multiple OKCs, it does not imply that a patient should have more than one cyst at a given point in time, rather it refers to the lifetime history of the patient. Early diagnosis will often make it possible to use conservative therapies rather than complex treatments.

Recognition of the syndrome permits early treatment in other but possibly asymptomatic relatives. Close attention of the family and past medical history and physical examination will alert the clinician to its presence, allowing for appropriate genetic counseling and serial screening for the development of malignancies and other complications besides OKCs.