儿童Abernethy畸形  

Comprehensive Review of Abernethy Malformation Cases in China

作　　者：王亚妹[1] 周艳茹[1] 陶于洪[1] 

机构地区：[1]四川大学华西第二医院儿科,成都610041

出　　处：《华西医学》2013年第5期756-760,共5页West China Medical Journal

摘　　要：目的提高对儿童Abernethy畸形的认识。方法归纳总结2001年1月-2012年11月我国文献报道的13例儿童Abernethy畸形(即先天性肝外门腔分流,临床分为Ⅰa型、Ⅰb型和Ⅱ型)的临床表现和治疗方法。结果 13例儿童Abernethy畸形中,11例Ⅰ型(Ⅰa型4例、Ⅰb型7例)和2例Ⅱ型患儿,1岁内发病6例,确诊前病程≥5年6例。在11例Ⅰ型患儿中,便血3例,呕血4例,肝功能异常4例,肝硬化1例,肝性脑病2例,肝脏结节3例,脾脏肿大4例,脾功能亢进3例,门脉高压3例,合并畸形3例。在2例Ⅱ型患儿中,便血1例,肝功能异常伴肝硬化、脾脏肿大和脾功能亢进1例,肝脏结节1例;无呕血和合并畸形。门脉系统血液分流到下腔静脉2例、髂内静脉4例、左肾静脉1例、奇静脉1例、右心房2例和盆静脉丛1例,其余2例不详。患儿多采用保守治疗,如有巨脾、便血或呕血,脾脏切除和结扎乙状结肠周围血管手术也有采用。结论 Abernethy畸形以Ⅰ型患儿为主,发病年龄较早,临床表现不具特异性。诊断依赖于影像学检查,螺旋CT血管造影对诊断有较高价值。个体化治疗应根据畸形类型和病情而定。Objective To acquire more knowledge about Abernethy malformation （congenital extrahepatic portosystemic shunt, including type Ia, type Ib and type Ⅱ ） in children. Methods All children with Abernethy malformation published previously in China were reviewed. The clinical, radiological and pathological manifestations and treatment were summarized. Results From January 2001 to November 2012, 13 children diagnosed to have Abernethy malformation were reported. Among them, 11 were diagnosed as type I Abernethy malformation, including 4 cases of type Ia and 7 cases of type lb. However, only 2 children were diagnosed to be with type Ⅱ Abernethy malformation. Of the 11 type I cases, 6 occurred by the age of one year old, and the duration ofpre-diagnosis in 6 children was over 5 years. These 11 cases with type I presented with hematochezia （n=3）, hematemesis （n=4）, liver dysfunction （n=4）, liver cirrhosis （n=l）, hepatic encephalopathy （n=2）, hepatic nodule （n=3）, splenomegaly （n=4）, hypersplenism （n=3）, portal hypertension （n=3） and other associated malformations （n=3）. In the 2 type 11 cases, one child presented with hematochezia, while another one had liver dysfunction accompanied with liver cirrhosis, splenomegaly and hypersplenism. No hematemesis or other associated malformations was found in children with type II. The sites of drainage for portal vein system were the inferior vena cava （2/11）, internal iliac vein （4/11）, left renal vein （1/11）, azygos vein （1/11）, right atrium （2/11） and pelvic venous plexus （1/11）, and there were no records of specific draining sites in two cases. The majority of these cases received conservative treatment. Surgical techniques such as splenectomy and surgical ligation of the veins of sigmoid were used to treat splenomegaly, hematochezia or hematemesis. But liver transplantation and resection of liver nodule were not reported. Conclusion Most Chinese children with Abernethy malformation are type I Abernethy malfor

关 键 词：ABERNETHY畸形 门静脉缺如 门腔分流 儿童 

分 类 号：R726.5[医药卫生—儿科]