脾切除、贲门周围血管离断术在肝豆状核变性中的治疗地位

Therapeutic effects of splenectomy and portal-systemic venous devascularization on hepatolenticular degeneration

目的 探讨脾切除、贲门周围血管离断术在治疗肝豆状核变性中的地位及适应证。方法 对在我院行脾切除、贲门周围血管离断术的 5例肝豆状核变性的病人的病情进行回顾性分析 ,并对其进行随访调查 ,评价该手术的效果。结果 脾切除、贲门周围血管离断术对于治疗肝豆状核变性病人的上消化道出血、脾功能亢进效果明显 ,有利于恢复驱铜药物治疗。结论 肝功能尚在代偿期的肝豆状核变性的病人 ,若肝硬化合并有门静脉高压症表现 ,上消化道出血或脾功能亢进致三系细胞低 ,进而影响药物驱铜治疗时 ,可行脾切除、贲门周围血管离断术以改善症状。

Objective To investigate the therapeutic effect and indications of splenectomy and portal-systemic venous devascularization in treatment of hepatolenticular degeneration (HLD). Methods The clinical data of 5 patients with HLD treated with splenectomy and portal-systemic venous devascularization in our hospital were retrospectively. Meanwhile, all the patients were followed up to evaluate the effect of the treatment. Results After the treatment, the hemorrhage of upper digestive tract and hypersplenism were relieved and the patients resumed the pharmaceutical therapy for removing copper. Conclusions Splenectomy and portal-systemic venous devascularization can be employed for treatment of HLD patients with portal hypertension who has normal liver function but can not continue the pharmaceutical therapy for removing copper because of the hemorrhage of upper digestive tract and hypersplenism.