脑干多发性海绵状血管畸形11例临床分析及文献复习

Multiple brainstem cavernous malformation:clinical analysis of 11 cases and literature review

目的探讨脑干多发性海绵状血管畸形的临床特征和治疗策略。方法回顾性分析11例脑干多发性海绵状血管畸形病人的临床资料。病灶共32个,其中位于脑桥29个,中脑2个,延髓1个。手术组包括手术治疗3例,非手术组包括保守观察7例和γ-刀治疗1例。结果随访11例,时间6～77个月。首次发病时平均KPS评分为(64.5±15.7),随访平均KPS评分为(71.8±15.4),两者无显著差异(P=0.07)。非手术组再次出血4例,手术组无再次出血,2组出血情况无显著性差异(P=0.236)。结论脑干多发性海绵状血管畸形病灶较大和症状较重病人,可采用手术治疗,首选切除主要病灶。无显著症状和病灶较小且深在者可保守观察。

Objective To investigate the clinical characteristics of multiple brainstem cavernous malformation（MBCM） and its treatment strategies.Methods Clinical data of 11 patients with MBCM were analyzed retrospectively.There were 32 lesions,consisting of 29 lesions in the pons,2 in the midbrain and 1 in the medulla.Three patients underwent surgery as surgical group,while 7 patients received conservative management and 1 patient was treated by gamma-knife therapy as non-surgical group.Results Eleven patients were followed up for 6 to 77 months.The mean scores of karnofsky performance scale（KPS） at the first episode of hemorrhage and during follow-up period were 64.5±15.7 and 71.8±15.4 respectively,and there was no significant difference between above scores（P = 0.07）.Rebleeding occurred in 4 patients in non-surgical group and no rebleeding in surgical group,and there was no obvious difference in hemorrhage between the 2 groups（P = 0.236）.Conclusions MBCM patients with severe symptoms or large lesions who can be treated by surgery to remove the main lesions.However,conservative management is practicable for patients without significant symptoms with the lesions being small and deep-seated.