儿童肝豆状核变性合并脾功能亢进外科治疗

Surgical treatment of wilson's diease with hypersplenism in children

目的探讨脾切除在治疗儿童肝豆状核变性合并脾亢中的临床价值。方法回顾性分析87例儿童肝豆状核变性合并脾亢行脾切除的临床资料;87例均行脾切除术,7例同时行贲门周围血管离断术,术中脾动脉预结扎65例。分别比较术前、术后血象及常规肝功能测定值变化。结果 2例病人术后死亡。85例术后血象及常规肝功能与术前比较有明显改善(P<0.05)。51例随访0.3～5年,33例随访5～10年无OPSI发生,无死亡。1例术后3年死亡。结论脾切除在治疗儿童肝豆状核变性并脾亢中具有重要地位。术前肝功能改善、手术中操作确切止血为手术成功关键。

Objective To explore the status of splenectomy in Wilson＇ s disease with hypersplenism in children, and analyze the related clinical problems. Methods 87 cases of children with hypersplenism caused by Wilson＇ s disease were retrospectively analyzed. 87 cases were performed resection of the spleen,7 cases were performed both portaazygous devascularization and splenectomy. splenic artery ligation were made with 65 cases in surgery, to Compare the determination value changes of preoperative, postoperative blood and routine liver function, to discuss the indication of splenectomy, complication of post-operation and the result after Surgical treatment. Result Two cases died after operation. 85 cases of postoperative blood and routine liver function compared with the preoperative has improved significantly（P 〈 0. 05 ）. No death and OPSI were noted in 0. 3 -5 years in 51 cases,5 - 10 years in 33 cases, one case died in 3 years later after operation. Conclusion Splenectomy has important role in Wilson＇ s disease with hypersplenism in children. Splenectomy in the treatment of children with Wilson＇ s disease and hypersplenism plays an important role in. Improve liver function of pre-operation and stanch bleeding in operating is important for surgery.