長期経過観察中に多発性顎嚢胞の発生をみた基底細胞母斑症候群の1例

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We encountered a case of Basal cell nevus syndrome in which multiple cysts occurred during long-term follow-up.<BR>The patient was a 8-year-old boy. Frontal and temporal bossing, ocular hyperterolism, mandibular prognathism, multiple jaw cysts, pit, calcification of the flax cercbri and bifid-rib were observed.<BR>Primordial cyst in tire kit maxilla and follicular cysts in the bilateral mandible were confi rmed 4 years after the operation for the left mandibular cyst. From the histopthological investigation, the process of cyst formations in this ease seemed to be identical with Brocas theory. The growth of the cysts was rapid in a short period of 1-2 years.<BR>We performed an enucleation with closure for the c ysts in the maxilla and enucleation with packing open for those in the mandible. No recurrence has occurred up to now, but a long-term follow-up is required in consideration of occurrence and recurrence of the cyst.