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华西医学  2013 

儿童Abernethy畸形

DOI: 10.7507/1002-0179.20130233, PP. 756-760

Keywords: Abernethy畸形,门静脉缺如,门腔分流,儿童

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Abstract:

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。

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