back to contentsBasal cell nevoid syndrome
- Report of a case with 20 year's follow-up -
Kanae Niimi, Yukiko Hoshina, Ichiro Suzuki, Tamio Nakajima
First Department of Oral Maxillofacial Surgery, Schoolof Dentistry, Niigata
University
(Chief: Prof.Tamio Nakajima)
Abstract:
@Basal cell nevoid syndrome is an autosomal dominant herediatry disease with multiple pits on palmar and plantar skin, bifid rib, ocular hyperterolism, multiple jaw cysts and many malformations. Most of the jaw cysts found in this syndrome are odontogenic keratocytes. In this paper, 11-year-old boy with basal cell nevoid syndrome who was followed up for 20 years was reported. Clinical examination showed that he had ocular hypertelorism, broad nasal root, temporal and frontal bossings, mandibular prognathism, and diffuse swelling in the right cheek. His chest radiograph showed clavate lib and remaining of 6th cervical lib. Panoramic radiograph showed many cystic lesions in the maxilla and mandible. Analogous findings were also observed in his father and brother. The cysts were treated by marsupialization, enuclation and primary closure, and/or enucleation and packing open. Recurrences occurred three times in two cysts with bony defects during the whereas three new cysts developed period of 20 years. One of the new cysts was discoverd 19 years after his first visit. Regrowth after marsupialization was encountered in two cysts with epithelial islands. Histologically, all cysts were odontogenic keratocysts and there were epithelial islands in the cysts walls in three cysts. A long-term observation is needed because of the high rate of recurrence and developement of new cysts in patients with basal call nevoid syndrome.