小儿肝动脉-门静脉畸形的诊断和介入治疗

Hepatic arterio-portal-venous malformation in children: the diagnosis and interventional treatment

目的探讨小儿肝动脉-门静脉畸形（APVM）的诊断和介入治疗方法。方法回顾性分析4例APVM患儿的诊治经过和疗效观察。男2例，女2例，分别为3．5、4、12、13岁。临床表现为反复上消化道出血和顽固性贫血，所有患儿均被误诊。肝动脉造影确诊后分别以明胶海绵和钢圈（2例）、聚乙烯醇（PVA）微粒（2例）栓塞病变肝动脉分支。结果肝功能和甲胎蛋白（AFP）均正常。肝脏CT和B超显示肝脏大小正常或轻度增大及脾大，有明显门静脉高压征象，无肝内占位病变。肝动脉造影表现为肝动脉分支增多、增粗，门静脉异常显影及胃冠状静脉曲张。3例栓塞术后消化道出血停止，1例迟发再出血。随访0．5～24个月，1例24个月仍存活，1例术后13个月死于尿毒症，2例因放弃治疗分别于术后0．5个月、3个月死亡。结论APVM属先天性疾病，临床容易误诊为肝硬化。肝动脉造影为诊断的金标准。治疗以多次血管内栓塞畸形血管为主，栓塞材料以PVA颗粒较为安全有效。

Objective To study the diagnosis and interventional treatment of arterio-portal-venous malformation （APVM） in liver. Methods 2 patients were male and 2 patients were female. The age was 3.5, 4, 12, 13 years old respectively. Clinical manifestations included repeated upper gastrointestinal hemorrhage and refractory anemia were misdiagnosed as hepatocirrhosis. After confirmed by hepatic arteriography, APVM were embolized with gelfoam plus spring coils （ n = 2） or polyvinyl alcohol （PVA） particles （ n = 2） respectively. Results All patients＇ hepatic function and alpha-fetoprotein level were normal. CT scan and Doppler ultrasound showed normal or slightly enlarged hepatic volume and no spaceoccupying lesion with signs of portal hypertension. Arteriography revealed enlarged and numerous branches of hepatic artery with abnormal portal vein visualization and varicose coronary vein. Gastrointestinal hemorrhage was stopped in three children and recurred in another after first embolization. One boy had survived for 24 months up to now, another died of uremia after 13 months and others died 15 days, 3 months after embolization respectively. Conclusion APVM is a congenital hepatic disease and easily misdiagnosed by pediatrist. Arteriography is the golden standard of diagnosis. Trans-arterial embolization of APVM is the main therapeutic method. Materials of embolization included gelfoam, spring coil and PVA particles, of which the latter is better.