妊娠合并重症肌无力七例临床分析

摘要

目的 探讨妊娠合并重症肌无力(MG)患者的临床特点、孕期处理及妊娠结局.方法 1983年10月至2010年10月北京协和医院妇产科共收治妊娠合并MG患者9例,同期入院孕妇为38683例,妊娠合并MG的发生率为0.023％.9例患者中有2例为意外妊娠,在早孕期行人工流产术,其余7例继续妊娠,对此7例患者的临床资料进行分析.结果 (1)发病情况:7例妊娠合并MG患者中,6例在孕前明确诊断为MG,诊断MG至妊娠的时间间隔平均为5.9年,此6例患者均在孕前经神经内科医师评估认为MG病情稳定,可以妊娠.另外1例在孕晚期首次发病.(2)孕期处理:7例妊娠合并MG患者中,有4例患者在孕前行胸腺切除术,其中1例患者术后发生3次MG危象,一直行药物治疗,但妊娠期间MG病情稳定；另外3例患者术后未行药物治疗,其中2例妊娠期间MG病情稳定,1例在妊娠33周病情恶化,加用药物治疗后病情稳定,并妊娠至足月.7例患者中2例孕前未行胸腺切除术,在早孕期MG病情即加重,经调整药物剂量后病情稳定,其中1例妊娠至足月,另1例因同时合并系统性红斑狼疮(SLE)、狼疮性肾炎及抗磷脂综合征于孕31周终止妊娠.7例中1例孕晚期首次发病的患者经药物治疗后病情稳定,妊娠至足月.(3)分娩方式及结局:7例妊娠合并MG患者中,有2例经阴道分娩,其中1例使用胎头负压吸引器助娩；另外5例行择期剖宫产术,其中1例因同时合并SLE、1例因羊水过少、MG病情恶化行剖宫产术,另3例因患者及家属极度顾虑MG病情对母婴的影响而要求行剖宫产术.7例新生儿中有3例为小于胎龄儿( SGA),所有新生儿均转入新生儿监护病房,均未发生新生儿MG.结论 MG患者在孕前需由神经内科医师进行病情评估,病情稳定者可以妊娠.孕期MG的病情变化不可预测,需由神经内科和产科协作进行病情监测,以期获得较好的妊娠结局.其新生儿应转诊至儿科严密监护.%Objective To discuss the interaction of pregnancy and myasthenia gravis(MG) and the management of pregnancy with MG.Methods Seven cases of pregnancy with MG in Peking Union Medical College Hospital were analyzed retrospectively,with respect to the therapy of MG,pregnancy complications and outcomes.Results Totally 38 683 pregnant women were admitted to Peking Union Medical College Hospital between Oct.1983 and Oct.2010.Among them there were 9 patients suffered from MG,with the incidence of 0.023％.Two pregnancies were terminated because of personal reasons,and seven continued.( 1 ) Onset of MG:in the 7 cases,6 were diagnosed before conception,with the mean course of 5.9 years.The other one occurred in the third trimester.(2) Management:all the cases were under close surveillance during pregnancy.Four women took thymectomy before conception,and one of them kept taking medication after surgery. In those who received thymectomy,3 cases remained stable and 1 case worsened during prenancy.The latter one took medication at 33 weeks,and continued to full term.MG exacerbated in the other three women who had not undergone thymectomy before conception.Among them, one woman complicated with systemic lupus erythematosus and lupus nephritis delivered the baby at 31 weeks.(3) Delivery and neonatal outcomes:cesarean deliveries were performed in 5 cases and the other two underwent vaginal deliveries.All the newborns were admitted to neonatal intensive care unit for surveillance.There were three smaller than gestational week (SGA) infants.No MG was observed in newborns.Conclusions Patients with MG should have an overall evaluation before conception.The course of MG during pregnancy is unpredictable.They may get a promising outcome under the control of a multidisciplinary team including obstetricians and neurologists.Newborns should be carefully monitored for sings of transitory MG in the department of pediatrics.