介入治疗小儿先天性肝脏动静脉瘘

Application of transarterial ernbolization for congenital intrahepatic arteriovenous shunt in children

目的探讨经肝动脉介入栓塞治疗小儿先天性肝脏动静脉瘘的方法及其疗效。方法回顾性分析2007年4月至2011年2月复旦大学附属儿科医院收治的3例小儿先天性肝脏动静脉瘘的临床资料。其中，例1为男孩，生后3个月因腹部增大2个月入院，B型超声提示大量腹水、肝脏占位；增强CT提示肝脏左叶内异常高密度影；腹水检查为漏出液；例2为男孩，生后5d因黄疸、心影大3d入院，检查发现同时伴房间隔缺损、动脉导管未闭、心功能衰竭及肺动脉高压，增强CT提示肝脏动静脉瘘；例3为女孩，生后6．5个月因上消化道出血入院，伴脾脏肿大和腹壁静脉曲张，上消化道钡餐和胃镜检查提示食管胃底静脉曲张，B型超声检查提示肝脏内靠近肝门部占位，增强CT发现为异常高密度影；3例患儿均采用股动脉穿刺插管肝动脉DSA造影，确诊为先天性肝脏动静脉瘘后给予肝明胶海绵及Fibercoil介入栓塞治疗。结果3例先天性肝脏动静脉瘘均介入治疗顺利，无术后出血及肝脏功能异常。3例均存活并随访至今。例1术后3周腹水完全消退。例2术后1周黄疸消退，3个月后房间隔缺损现已明显缩小，动脉导管已经闭合；心力衰竭及肺动脉高压消失。例3介入治疗后无消化道出血表现，3个月后检查食管胃底静脉曲张基本消失，脾脏肿大明显减少。3例患儿生长发育均正常。结论小儿先天性肝脏动静脉瘘是罕见疾病，采用经肝动脉DSA造影和栓塞的介入栓塞治疗方法是治疗小儿先天性肝脏动静脉瘘的有效方法。

Objective To explore the efficaeies and outcomes of embolization in the treatment of infant and neonatal congenital intrahepatic arteriovenous shunt. Methods Three cases of infant and neonatal congenital intrahepatic arteriovenous shunt from April 2007 to February 2011 were retrospectively reviewed. One boy aged 3 months had abdominal distension caused by massive aseite. And another 5-day-old boy had jaundice, atrial septal defect/patent ductus arteriosus and heart failure/ pulmonary hypertension. A 6. 5-month-old girl was referred for severe upper gastrointestinal bleeding and melena. Splenomegaly and varicose veins were also noted on abdominal wall. Doppler ultrasonography and enhanced computed tomography （CT） suggested the presence of intrahepatic hepatoportal or arteriovenous shunts in all 3 cases. Transarterial embolization was successfully achieved by coiling of hepatic artery in all cases. Results The shunts were blocked by gelfoam and fiber coil. And ascite disappeared spontaneously within 3 weeks after embolization for case 1. For case 2, atrial septal defect/patent ductus arteriosus closed and heart failure/pulmonary hypertension disappeared. And upper gastrointestinal tract bleeding and melena disappeared in case 3. All patients survived and recovered well. Conclusions Congenital intrahepaticl arteriovenous shunt is rare and transarterial embolization may be an excellent curative option.