先天性联体畸形的外科处理

目的 总结4例联体儿分离术的经验,对联体儿诊治提供参考。方法 回顾性分析自1982～2001年,我院小儿外科共收治的4例先天性联体儿外科分离手术的临床资料,着重在手术时机、产前诊断、合并畸形、分离技术、术后监护、随访等方面。结果 4例联体儿畸形,其中胸部-脐联合畸形3例,坐骨联体1例,男性1例,余均为女性。均不同程度合并先天性心脏缺损,坐骨联体儿合并严重大血管畸形,泄殖腔畸形及Pieer-Robin综合征。2例产前超声检查获得诊断。2例紧急分离术时间为出生后7d内,存活1名。另2例延迟分离手术时间>1个月,均存活。随访存活的5名患儿,1982年分离的剑突-脐联体儿其中1名于术后4年“肺炎”死亡。另1名已20岁,健在。另一对胸部-脐联体儿术后1年发育良好。1例坐骨联体儿1名死亡,1名失随访。结论 手术时机与分离方式必需依据每一对联体儿局部环境及器官结构而定。紧急分离术存活率低,能延迟行分离术则存活率高。

Surgical management of congenital conjoined twins

Objective To sum up experiences in surgical separation from four conjoined twins.Methods A retrospective review was made in 4 conjoined twins admitted from 1982 to 2001. The data in-cluded prenatal diagnosis, associated malformations, operation timing, separate technique, postoperativemonitor and follow-up outcome. Results There were thorax-omphalopagus in 3 cases and ischiopagus in 1.All cases had associated congenital cardiac malformations. The ischiopagus case associated with vascularanomaly, Pieer-Robin syndrome and cloaca anomaly. Two cases were diagnosed prenatally by ultrasono-graphy. Two twins needed to be emergent separation during 7 days after birth with one survived. All ofthe other 2 conjoined twins that received a delayed operation over 30 days after birth were survival. Thefollow-up results of the survivors showed that one of the thorax-omphalopagus twins died from pneumonitis4 years postoperatively and one was at the age of 20 years with good general condition; the other thorax-omphalopagus twins were 1. 5 years old and living well for one year after operation, and the ischiopagustwins were lost connection. Conclusions Satisfactory results of conjoined twins can be gained by prenataldiagnosis; delayed separation; improved technique of anesthesia; completed surgical strategy and periopera-tive monitor. The associated severe cardiovascular malformations are the main factors influencing on theprognosis of conjoined twins.