小儿门静脉海绵样变性、门静脉高压症的临床研究(附10例报告)

Reserch on cavernous degenerarion and hypertension of portal vein in children

目的探讨门静脉海绵样变性、门静脉高压症的病因、病理、临床表现、诊断方法、治疗及预后等问题。方法总结我院7例经手术治疗病例的临床及影像学检查资料。结果呕血伴排柏油样便为主要临床表现 ,脾脏增大为体检特征。本组均经彩色多普勒检查提示或明确诊断 ,4例术前行血管造影。胃底及下段食管血管断流、脾切除为共有手术方式 ,5例行不同形式的分流手术。结论部分患儿可能为先天性因素所致。本症诊断主要靠影像学检查 ,治疗同一般门静脉高压症 ,预后与肝脏情况密切相关。

ObjectiveTo discuss etiology, pathology, clinical manifestations, diagnosis, management and prognosis of cavernous degenerarion and hypertension of portal vein.MethodsThe clinical and image data of 7 children with cavernous degeneration and hypertension of portal vein were veviewed.ResultsHematemesis, melena and splenomegaly were the major presentations. The diagnosis was confirmed by color Doppler ultrasonography except 4 cases had angiography. All cases underwent splenectomy and transection of the gastric fundus and esophageal veins. Five of them had an additional portalcaval shunt.ConclusionCongenital factors may play some role in the development of cavernous degeneration and hypertension of portal vein in children. For the disase discussed this paper the image examination is the main diagnostic method, and the management is same with portal hyertension and the prognosis is closely related to the liver function.