Sturge-Weber综合征所致难治性癫痫的围手术期麻醉管理

Perioperative anesthesia management for refractory epilepsy caused by Sturge-Weber syndrome

目的总结接受大脑半球离断/切除术的Sturge-Weber综合征(Sturge-Weber syndrome,SWS)患儿围手术期麻醉管理要点。方法回顾2017年1月至2020年11月在全麻下行大脑半球离断/切除术的SWS患儿的医疗记录。收集并分析人口统计学、围手术期及术后相关信息。结果8例SWS患儿因难治性癫痫在全麻下行大脑半球离断/切除术,其中7例(87.5%)患儿颜面部存在葡萄酒色斑,4例(50.0%)患儿合并青光眼,未发现有患儿合并气道血管瘤造成困难气道。手术出血均在200 ml以上,所有患儿术中均输注异体红细胞,中位输注量260(146.3~357.5)ml。因术中出血较多,有4例患儿出现不同程度的低血压,较严重的1例患儿同时伴有低血氧饱和度现象。患儿围手术期均未应用使眼内压增高的药物。术后8例患儿出现中度以上贫血,颅内感染发生率较高(7/8,87.5%)。术后远期随访患儿癫痫症状控制Engel分级:Ⅰ级7例、Ⅱ级1例,术后均未见永久性功能障碍。结论SWS所致难治性癫痫大脑半球离断/切除术中出血量较大,严密的术中监测和积极的围手术期管理对于维持循环稳定十分重要。患儿有很大比例合并青光眼,围手术期应谨慎应用使眼内压升高的药物。基于以往研究,术前充分评估患者气道是否合并血管瘤,做好困难气道准备也是十分必要的。

Objective To summarize the key points of perioperative anesthesia management for children with Sturge-Weber syndrome(SWS)who underwent hemispherotomy or hemispherectomy Methods Retrospective analysis was performed using medical records from SWS children who underwent hemispherotomy or hemispherectomy under general anesthesia from January 2017 to November 2020.Their demographic,perioperative and postoperative information were collected and analyzed.Results There were 8 SWS children who underwent such kind of surgery for refractory epilepsy,where 7 children(87.5%)manifested facial wine spots and 4 children(50.0%)were accompanied with glaucoma.No difficult airway caused by hemangioma was found.The intraoperative blood loss was at least 200 ml.Red blood cells were transfused in all children during operation,with a median volume of 260(146.3-357.5)ml.Because of severe bleeding,4 children presented hypotension to various degrees,and one of them was accompanied with low blood oxygen saturation.No drugs were used to increase intraocular pressure during the perioperative period.After operation,8 cases presented moderate or severe anemia,with a high intracranial infection rate(7/8,87.5%).During the long-term follow-up,seizure outcome was evaluated and categorized according to Engel classification,and there were 7 cases of Engel grade Ⅰ and 1 case of Engel grade Ⅱ.Moreover,no permanent dysfunction was found after operation.Conclusions Large-volume hemorrhage is commonly seen during hemispherotomy or hemispherectomy in the treatment of refractory epilepsy caused by SWS.It is necessary to perform strict intraoperative monitoring and active perioperative management to maintain stable circulation.A large proportion of children are accompanied with glaucoma and drugs increasing intraocular pressure should be used cautiously during the perioperative period.Based on previous studies,it is necessary to fully evaluate whether the patient's airway is invaded by hemangioma before operation and to prepare well for difficult airway.