脾-左肾上腺静脉分流术治疗儿童门静脉海绵样变性

Spleno-left adrenal vein shunt for portal cavernous transformation

目的初步探讨脾静脉-左肾上腺静脉分流术对儿童门静脉海绵样变所致门静脉高压症的疗效。方法总结8例儿童门静脉海绵样变致门静脉高压症上消化道出血病例的临床及随访资料，分析该手术的疗效。结果本组8例在分流术前后门静脉压力分别为（30±11）mmHg和（22±7）mmHg，两者相比差异有统计学意义，P〈0．01。术后随访6个月～5年，无死亡及再次发生上消化道出血病例，肝功能良好。术前外周血中三系下降的7例患儿术后血常规WBC计数为（7．64±4．46）×10^9／L，RBC计数为（4．54±0．97）×10^12／L，Hb为（133±5）g／L，PLT计数为（355．40±107．36）×10^9/L，与术前相比差异均有统计学意义，P〈0．05。术前l例P岍计数降低的患儿术后P岍计数为333×10^9／L，较术前显著升高。4例术后血氨升高，由术前的（18±7）μmol／L升高至术后的（60±17）μmol／L，两者相比差异有统计学意义，P〈0．05，但无1例发生肝性脑病，且智力发育良好。消化道钡餐X线或胃镜检查提示6例食管胃底静脉曲张程度好转，血管超声显示吻合口无狭窄，血管内无血栓形成。结论脾一左肾上腺静脉分流术治疗儿童门静脉海绵样变所致门静脉高压症可获得良好的效果.

Objective To evaluate the effect of spleno-lefl adrenal vein shunt for the treatment of portal hypertensive upper GI bleeding caused by portal vein cavernous transformation in children. Methods Spleno-lefi adrenal vein shunt was performed in 8 children with portal hypertension due to cavernous transformation. The clinical data was reviewed. Results Portal vein pressure decreased significantly from （ 30 ± 11 ） mm Hg to （ 22 ± 7 ）mm Hg after shunt. There was no mortality pefioperatively and during the follow-up, There were no recurrent hemorrhage nor hepatic encephalopathy occurring in the follow-up and all the children have normal intelligence and normal liver function though blood ammonia level increased significantly from（ 18 ±7） μmol/L to （60 ± 17） μmo]/L in 4 cases. In 7 cases in which preoperative whole blood cell count significantly decreased, the postoperative WBC, RBC, Hh and PLT was（ 7.64 ± 4.46） ×10^9/L, （4. 54 ± 0. 97 ） ×10^12/L, （ 133 ± 5 ） g/L and （ 355.40 ± 107.36 ） × 10^9/L respectively （ all P 〈 0. 05）. In one case suffering from preoperative low PLT count the postop PLT reached 333 × 10^9/L, which was significantly higher than that preoperatively. Esophageal varices ameliorated in 6 eases. No stenosis of anastomotic stoma and thrombosis developed. Conclusion Spleno-lefi adrenal vein shunt is an effective procedure to treat portal vein cavernous transformation induced portal hypertension in children.