The syndromic multiple odontogenic keratocyst in siblings: A familial study
In July 2001, a 28-year-old male reported with a chief complaint of pain and swelling of the left lower jaw causing facial asymmetry. The patient reported of similar symptoms on the same side 8 years back. He had visited several dentists over this period of time with a complaint of mobility of teeth on both sides of the jaw and swelling on the left side. He was advised removal of 48, 47, and 38 followed 2 years later by removal of 37, 36, 35, and 34. Since the pain and mobility of the teeth did not subside, the patient reported to us. He did not carry any past histopathological records with him. His past medical history was unremarkable and his general physical examination revealed the presence of skin lesions on the forehead [].\nClinical examination of the head and neck region showed left facial swelling along with an expanded buccal and preauricular area leading to facial asymmetry. He denied any neurosensory problem associated with inferior alveolar nerve.\nIntraoral examination revealed a firm, hard swelling on the crest and buccal vestibule of left mandibular alveolar ridge, around 5 cm in the anterior–posterior direction and overlying the crest of 36, 37, 38. There was no discharge intraorally. All other hard and soft tissue components of oral cavity appeared normal.\nThe OPG showed a multilocular radiolucency on the left side in the body of the mandible. One radiolucent lesion was seen extending from the condylar, coronoid processes up to the body of mandible on the same side. There was another smaller radiolucent lesion evident on the distal aspect of 35 [].\nThe computed tomography (CT) scan confirmed that the lesion was multiloculated and expansion of the cortical bones of the inferior and posterior borders on the left side of mandible was evident. Another radiolucency was seen just adjacent to the main lesion. In our opinion, it could have been an extension of the same lesion, which radiographically appeared as “daughter cyst.”\nOn histopathological examination, the diagnosis of OKC was made [].\nIn July 2000, under general anesthesia, hemi-mandibulectomy was performed followed by placement of reconstruction plate. In June 2002, the reconstruction plate was removed and a nonvascularized iliac crest graft was placed. In subsequent visit, impacted 18 was removed along with the cystic lining and its contents under local anesthesia. The lesion was reported to be a dentigerous cyst [].\nIn 2004, the orthopantomograph showed evidence of resorption of graft. Also, it was noticed that the skin lesions on the face and axilla appeared to be enlarging slowly. Dermatological consultation resulted in agreement with diagnosis of Basal Cell Nevous Syndrome. On submission of histopathological specimen, the skin naevi were reported to be basal cell carcinomas []. Chest radiograph did not show any evidence of bifid rib [].\nIn the clinical and radiographic follow-up from 2007 to 2009, the OPG showed increase in the level of resorption of the graft, although the patient did not have any functional or aesthetic problem. In the recent follow-up visits, the BCC lesions have gradually shown an increase in size as well as number [].\nThe patient is scheduled for regular periodic follow-up with dermatology and maxillofacial department.