基底細胞母斑症候群の5例

概要

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1. We experienced a basal cell nevus syndrome with huge multiple jaw cyst as major symptom and reported the case with other 4 experienced cases in our department. 2. Ocular hyperterorism, broadened nasal root, frontal and biparietal bossing, jaw cyst, bridging of sella turcica and family appearance were observed at all cases. 3. Basal cell nevi and pits were observed in cases 1, 2 and 5, but were unknown in cases 3 and 4. 4. Keratocyst was the histological diagnosis of all jaw cysts. 5. Treatment methods with jaw cysts were: Encleation after murspalization in case 1, presently murspalization and in the future enucleation in case 2, encleation alone in cases 3 and 4, and murspalization alone in case 5. Yet, cases 2 and 5 were recurrence by murspalization alone, therefor it was thought necessary to do complete enucleation including the surrounding healthy bone tissues. 6. As jaw cysts were keratocyst, they were apt to recur, and the importance of long term progress observation is important.