脾次全切除术治疗成人型戈谢病的临床应用

Clinical analysis of subtotal splenectomy for the treatment of Gaucher disease

目的探讨脾次全切除术治疗成人型戈谢病的可行性及适用性。方法回顾性分析2017年10月至2019年2月间6例成人型戈谢病病人行脾次全切除术的临床资料,手术均采用保留脾脏下极血管分支支配的同龄人正常容积的脾脏,统计手术时间、术中出血量、术后住院时间、并发症发生情况,观察病人血细胞恢复情况,计算保留的脾脏容积及占原脾的容积比率,观察保留的脾脏血流状况,评价保留脾脏的网状内皮细胞功能。结果 6例行脾次全切除的手术均顺利完成,术中无被迫中转全脾切除病例,术后保留的脾脏无缺血坏死,无围手术期死亡病例。平均手术时间为98 min(80～120 min),术中平均出血量为320 ml(200～500 ml),术后平均住院时间为13 d(12～15 d),术后无腹腔出血、胰漏、感染等并发症发生。术后白细胞计数恢复正常或稍高于正常值,血小板计数迅速上升,超过正常值或翻倍,红细胞计数及血红蛋白值缓慢上升,逐渐接近正常值,彩超和增强CT显示保留的脾动脉分支血流通畅,脾脏血供分布均匀,无缺血及梗死,脾静脉主干虽然会有部分血栓形成,但血流顺畅,平均残脾容积为312 ml(265～360 ml),平均占总容积的6.3%(5%～10%),^(99m)Tc同位素显像显示保留的脾脏组织核素分布基本均匀,网状内皮细胞吞噬功能良好。结论脾次全切除术是一种安全可行、经济有效的治疗成人型戈谢病的备选方法。

Objective To explore the feasibility and applicability of subtotal splenetomy for the treatment of adults Gaucher disease. Methods The clinical data of 6 patients with adult Gaucher disease who underwent subtotal splenectomy from October 2017 to February 2019 were retrospectively analyzed. All patients underwent splenectomy with preservation of the normal volume of the spleen innervated by the lower pole vessels of the spleen.The operational time, the intraoperative blood loss, the hospital stay, the postoperative complication of hemorrhage, pancreatic leakage and infection were noted. Haemocytes changes were observed. The volume of the spleen and the volume ratio of the original spleen were calculated. The blood flow status of the retained spleen was observed. The reticuloendothelial cell function of the retained spleen was evaluated. Results 6 cases of subtotal splenectomy were successfully completed with no conversion to total splenectomy during operation, no ischemic necrosis of retained spleen after subtotal splenectomy, no death during perioperative period. The average operation time was 98 min (80-120 min), the average intraoperative blood loss was 320 ml (200-500 ml), and the average postoperative hospital stay was 13 d (12-15 d). No postoperative complications such as abdominal hemorrhage, pancreatic leakage and infection occurred. Postoperative white blood cell count returned to normal or slightly higher than the normal value, and platelet count rapidly increased, which was higher than the normal value or doubled, and red blood cell count and hemoglobin value slowly increased, which gradually appoached the normal value. Color Doppler ultrasound and enhanced CT showed that the preserved splenic artery branch blood flow was smooth, the distribution of blood supply to the spleen was even without ischemia and infarction. Although there was partial thrombosis in the splenic vein trunk, the blood flow was smooth. The average volume of retained spleen was 312 ml (265-360 ml), accounting for 6.3%(5%-10%)of total volume, 99m Tc isotope imaging showed that the retained spleen tissue nuclide distribution was basically uniform, and reticuloendothelial cell phagocytosis was good. Conclusion Subtotal splenectomy is a safe, feasible, cost-effective alternative treatment for adult Gaucher disease.