基底细胞痣综合征(附24例报告)

Clinical characteristics of basal cell nevus syndrome (BCNS) :24 cases report

目的 :总结基底细胞痣综合征 (BCNS)主要临床特点及颌骨囊肿的治疗体会。方法 :对 1981 0 3～1999 0 9收治的 2 4例BCNS进行临床分析。结果 :2 4例均患有颌骨囊肿 ,平均初诊年龄 17岁。其它主要症状发生率 :多发痣 37.5 % ,掌足陷凹 2 9.2 % ,脊柱肋骨畸形 70 .8% ,颅内钙化 79.2 %。颌骨囊肿引起的面部畸形是BCNS早期就诊的主要原因 ,具有多发性、发病年龄早及同一患者囊肿发病时间有先后等特点 ,手术治疗复发率 2 2 .0 %。结论 :颌骨囊肿是造成BCNS患者生理心理损害的主要症状之一 ,青春期前尤其是 10岁左右患颌骨囊肿者应注意其是否患有BCNS。

砄bjective: To summarize the clinical characteristics of basal cell nevus syndrome (BCNS) and the treatment experience of cysts in jaws. Methods: 24 patients of BCNS from Mar. 1981 to Sept. 1999 were retrospectively investigated. Results: Of all the cases, the mean age for the first examination was 17 years old. The respective incidence of the main symptoms were multiple nevi in 9 cases (37.5%), palmar and/or plantar pits in 7 (29.2%), spine and/or ribs deformity in 17 (70.8%), intracranial calcification in 19 ( 79.2% ), specific facial profile in 16(66.7%). The cysts of jaws were the main causes for early examination, which were characterized by their multiple and preadolescent occurrences. The cysts were surgically removed and the postoperative recurrence rate of the cysts was 22.0%. Conclusion: Much attention should be paid to the cysts of jaws, the main symptoms, and cause of the physiological and psychological impairment in the patients of BCNS.