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目的 探讨联体畸形的诊断和治疗方法 ,增进对该少见疾病的认识 ,提高疗效 ,以及生存率。方法 采用B超、CT及消化道钡餐等检查 ,判定内脏连接情况 ,手术分离 2对联体畸形婴儿。2对病例分别为剑脐联体畸形及胸腹联体畸形 ,均有肝脏连接 ,但胆囊及胆管各自独立 ,于生后第 2 8d、第 96d时分别实施分离手术 ,对于肝脏连接的处理 ,采用“局部血流阻断法” ,行共用肝离断术。结果 2对联体畸形经充分术前准备 ,均手术分离成功 ,术后患儿生长及发育正常。经采用局部血流阻断共用肝离断术方法 ,术中出血少 ,术后肝功能恢复顺利。结论 联体畸形较为罕见 ,充分的术前准备及合理的分离术式可提高患儿的双双存活率。局部血流阻断共用肝离断术 ,仅阻断了离断线局部的肝脏血流 ,对非操作区的肝脏血液供应毫无影响。有利于对创伤及失血耐受性很差的婴幼儿术后康复
Objective To explore the diagnosis and treatment of conjoined deformity, to improve the understanding of this rare disease, to improve the curative effect and survival rate. Methods B ultrasound, CT and gastrointestinal barium meal and other tests to determine the visceral connection, surgical separation of the two conjoined malformed baby. 2 pairs of cases were sirloin conjunctival deformity and thoracoabdominal conjunct deformity, all connected to the liver, but the gallbladder and bile duct were independent, in the postnatal 2d 8d, 96d, respectively, the implementation of separation surgery, the treatment of liver connections, Using the “local blood flow blocking method”, the line of shared liver surgery. Results 2 pairs of conjunctival deformity after full preoperative preparation, were successfully separated surgery, postoperative growth and development of children with normal. After the use of local blood flow blocking the common liver resection method, less bleeding, postoperative recovery of liver function smoothly. Conclusions conjoined deformity is relatively rare, adequate preoperative preparation and reasonable separation of surgery can improve the survival rate of both children. Local blood flow blocking shared liver ablation, blocking only the local blood flow from the disconnected line, had no effect on the hepatic blood supply to the non-operating area. Conducive to post-operative recovery of infants and toddlers with poor trauma and blood loss tolerance