肝豆状核变性患者消化道出血的治疗

Management of gastrointestinal hemorrhage in patients with Wilsons disease

目的 评价贲门周围血管断流及脾切除术治疗肝豆状核变性患者消化道出血的疗效。方法 回顾性分析贲门周围血管离断术、脾切除术治疗合并严重肝病、消化道大出血的肝豆状核变性患者１５例资料。结果 １５例中急诊手术２例。手术方式包括贲门周围血管离断及脾切除术１２例．单纯脾切除术３例。术前铜离子络合剂驱铜治疗至少３个月。手术指征是食管下段和胃底静脉曲张破裂，消化道大出血反复发作和严重的脾功能亢进。术后消化道出血停止，白细胞、血红蛋白和血小板明显升高。术后继续驱铜治疗，经（４．２±３．０）年随访，未见消化道出血复发，神经系统症状改善，可正常生活。结论 在有效驱铜治疗的同时，对合并食管下段和胃底静脉曲张破裂、消化道出血的肝豆状核变性患者行贲门周围血管断流及脾切除术是安全、有效的，是药物驱铜治疗肝豆状核变性的重要辅助措施。

Objective To evaluate the pericardia devascularization with splenectomy for gastrointestinal hemorrhage in patients with Wilsons disease (WD) . Methods Fifteen WD patients with severe liver disease who underwent surgical intervention for gastrointestinal bleedings were reviewed retrospectively. Results Two of fifteen patients underwent emergency operation. The operative procedures included pericardia devascularization with splenectomy performed in 12 cases and simple splenectomy in other 3 patients. The patients had been treated with the copper-chelating agent for more than 3 months preoperatively. The indications of surgical intervention were serious hypersplenism and recurrent episodes of gastrointestinal hemorrhage from esophagogastric varices. Gastrointestinal bleedings were controlled, leukocyte and hemoglobin as well as blood platelet were significantly increased postoperatively. The medical regimen of eliminating copper continued and neurologic symptoms in the WD patients were improved. Conclusions Combined with effectively eliminating copper, the pericardia devascularization with splenectomy is safe and valuable for gastrointestinal hemorrhage from variceal hemorrhage in WD patient. It is a valuable auxiliary therapy also for copper-reducing pharmacotherapy for Wilsons disease.