儿童门脉海绵样变的诊断、治疗和预后追踪(附9例临床分析)

The diagnosis,treatment and prognosis of portal cavernoma in children: an analysis of 9 cases

目的 总结儿童门脉海绵样变的诊治经验，了解不同治疗方法的效果及预后。方法 对在我院确诊的９ 例１ ～１１ 岁门脉海绵样变患儿的诊断和治疗效果进行分析，并追踪预后。结果 儿童门脉海绵样变以上消化道出血和脾大为首诊原因，超声波检查阳性诊断率为８８．９ ％ ，假阳性率为２．３ ％ ，脾切除＋ 门体分流术后上消化道出血次数减少，每年≤１ 次，比单纯脾切除或脾切除＋ 断流术效果好。结论 有门脉高压表现的儿童应注意门脉海绵样变的可能，超声波可作为常规的临床检查手段，条件许可时脾切除＋ 分流术应作为首选的治疗措施。

Objective To summarize the experience in diagnosis and treatment of portal cavernoma in children and inquire its prognosis of different therapies.Methods Analysing the diagnosis and treatment effect on 9 cases with portal caver cavernoma aged 1～11, 7 cases were followed up. Results The first noticed symptoms showed upper digestive tract hemorrahge and splenomegaly. The postive rate of diagnosis of portal cavernoma by ultrasound was 88.9% and false postive rate was 2.3%. The patients under the operation of portacaval shunt and splenectomy had ≤1 episode of upper digestive tract hemorrage per year, which had better outcome than those under only splenectomy or portacaval devescularization and splenectomy. Conclusions The possibility of portal cavernoma should be considered in children with portal hypertension.Ultrasound can be used as an ordinary method in the diagnosis of the cases. Portacaval shunt and splenectomy is the first choice management in the patients when condition is permited.