摘要
目的探讨肝移植治疗小儿先天性门静脉海绵样变的临床效果。方法2例患儿经CT和CTA检查诊断为肝内门静脉系统海绵样变,并伴有严重的门静脉高压症,分别于2004年12月和2005年6月施行肝移植术治疗。从临床症状、血液学指标和影像学检查方面对手术疗效进行评价。结果2例患儿术后消化道出血症状均消失,且无复发;血红蛋白、血球压积、血小板水平等均接近或恢复正常;脾脏亦有一定程度缩小;1例患儿随访至今,情况良好;另1例患儿术后失访2月,3月后超声复查发现大量腹水,门静脉吻合口明显狭窄,再次手术重新植入另一供体门静脉,手术过程顺利,因术后出现严重肺部感染,同时合并肝、肾功能损害,于术后2周死亡。结论肝移植术可成为先天性门静脉海绵样变的有效治疗方法之一,其近期疗效良好。对肝移植的远期效果和并发症,尚需进一步积累病例和随访研究加以评价。
Objective To evaluate the outcome of the cavernous transformation of the portal vein treated in infants by liver transplantation. Methods The diagnosis of cavernous transformation of portal vein with severe hypertension was comfirmed by CT and CTA in two infants. The liver transplantations were performed respectively in these two patients from December 2004 to June 2005. The evaluation of the outcome was made by clinical features, laboratory and imaging examinations. Results The symptoms of alimentary tract hemorrage disappeared and did not recur. Hb, Hct and BPC reached the normal level and the size of spleen became smaller. One patient was well followed with ultrasound outcome. The other patient was lost in follow-up for about two months and found in the third month with large amount of ascites and stoma stenosis of the portal vein. In spite of the successful transplantation of another portal vein, the patient died of severe pulmonary infection with liver and renal dysfunction two weeks later. Conclusion The liver transplantation could be one of the efficient methods to treat the cavernous transformation of portal vein. The short-term efficiency is impressive, while the long-term outcome and the comorbidity need further investigations.
出处
《上海交通大学学报（医学版）》
CAS
CSCD
北大核心
2006年第6期643-646,共4页
Journal of Shanghai Jiao tong University:Medical Science
关键词
肝移植
门静脉海绵样变
门脉高压
小儿
liver transplantation
cavernous transformation of portal vein
portal hypertension
infant
