小儿重症肌无力的外科治疗

Surgical Treatment of Myasthenia Gravis in Children

目的 探讨小儿重症肌无力(MG)的手术适应证,围手术期处理及影响疗效的有关因素。 方法 按Os-serman临床分型分为型(单纯眼肌型)11例,A型(轻度全身型)6例,B型(中度全身型)2例。胸腺病理检查:增生12例,正常组织学表现7例,无合并胸腺瘤者。12例胸腺标本进行了免疫组织化学观察。 结果 全组无手术死亡。术后发生肌无力危象1例,总有效率89.5%。全身型术后缓解率75%,较单纯眼肌型36.3%高。常规病理分型与疗效无关,根据免疫组织化学观察,12例胸腺分为上皮细胞密集型6例和上皮细胞松散型6例,两者术后疗效相差显著。 结论 小儿全身型MG应行手术治疗,而对单纯眼肌型MG的手术适应证应从严掌握,对学龄前儿童尽可能采用药物治疗。使用人工呼吸器是治疗术后肌无力危象的有效措施。胸腺免疫组织化学分型能较好地评估手术后的疗效。

Objective To study the indication for operation, pre and postoperative management and factors affecting the effects of thymectomy on myasthenia gravis (MG) in children. Methods From 1979 to 1996,thymectomies were performed on 19 cases (under 12 years old) of MG. According to Osserman's classification, type Ⅰ (ocular type) 11 cases, type ⅡA (mild generalized type) 6 cases, type ⅡB (moderate generalized type) 2 cases, no type Ⅲ and Ⅳ. Thymus pathology showed lymphoid hyperplasia 12 cases, normal 7 cases, no thymoma found. Twelve cases received immunohistochemical studies. Results No operative death. Myasthenia crisis occurred postoperatively in one case, and was successfully treated by using ventilator for two days. Five cases received chemotherapy 1 5 times. The total effective rate was 89.5%, but the remission rate of generalized MG (ⅡA and ⅡB type) 75% was higher than that of ocular MG 36.3%. According to immunohistochemical study, 12 cases fell into two types:epithelia condensed type and epithelia dispersed type, the difference between operative effects rates of two types were markedly significant ( P <0 05), however, there was no significant difference between routine histopathological types. Conclusion Thymectomy can be recommended for children with generalized MG, but the indication for ocular MG should be restricted. For preschool children medical therapy is proper. Ventilator is useful for postoperative myasthenia crisis. Immunohistochemical patterns are helpful to assess the operative effects.