2例Abernethy畸形的诊断分析
Diagnosis of Abernethy Malformation in 2 Cases
DOI: 10.12677/ACRP.2013.13009, PDF, HTML, 下载: 2,958  浏览: 10,257 
作者: 潘峰, 梁惠民, 孔祥泉：协和医院放射科；周东风, Sandya Adevi：协和医院儿科
关键词: 门静脉；先天发育异常；Portal Vein; Congenital Abnormalities
摘要: 目的：探讨Abernethy畸形的诊断方法。方法：回顾2005年至2012年共计2例Abernethy畸形患者的诊治情况，并对其进行分析。结果：Abernethy畸形临床表现及体征缺乏特异性，超声检查可以有效的发现门静脉闭锁的情况；CTA、MRA及DSA造影可以对Abernethy畸形进行准确诊断及分型，并可全面评估患者腹腔静脉血管畸形情况。结论：腹部超声检查有助于Abernethy综合征的早期筛查；CTA、MRA及DSA造影均可以作为Abernethy畸形的最佳确诊及分型方法.
Abstract: Objective: To explore methods for diagnosis ofAbernethy malformation. Methods: 2 cases of Abernethy malformation were collected from 2005 to 2012 and analyzed. Results: Symptoms and signs in patients of Abernethy malformation were lack of specificity. Ultra-sound examination could be used to detect the atresia of portal vein. CTA, MRA and DSA could be accurately used to diagnose and classify Abernethy malformation, which could also be used to estimate the whole malformation of abdominal arteries and veins. Conclusion:Abdominal ultra-sound examination could be a very good screening method for Abernethy malformation’s diagnosis; CTA, MRA and DSA could be used to accurately diagnosis and classify Abernethy malformation.

文章引用：潘峰, 周东风, Sandya Adevi, 梁惠民, 孔祥泉. 2例Abernethy畸形的诊断分析[J]. 亚洲儿科病例研究, 2013, 1(3): 37-40. http://dx.doi.org/10.12677/ACRP.2013.13009

参考文献

[1] E. R. Howard, M. Davenport. Congenital extrahepatic portocaval shunts—The Abernethy malformation. Journal of Pediatric Surgery, 1997, 32(3): 494-497.
[2] 黄劲柏, 徐海波, 孔祥泉等. Abernethy畸形一例[J]. 中华放射学杂志, 2006, 40(10): 1112-1113.
[3] 王小川, 王蕾, 贾苍松. Abernethy畸形1例[J]. 中华妇幼临床医学杂志(电子版), 2011, 2: 168-169.
[4] 李震, 汪忠镐, 赵克等. Abernethy畸形致下消化道出血的诊治[J]. 中国普外基础与临床杂志, 2010, 1: 83-84.
[5] C. Ratnasamy, A. Kurbegov, S. Swaminathan. Cardiac anomalies in the setting of the Abernethy malformation of the portal vein. Cardiology in the Young, 2007, 17(2): 212-214.
[6] A. Singhal, A. Srivastava, N. Goyal, et al. Successful living donor liver transplant in a child with Abernethy malformation with biliary atresia, ventricular septal defect and intrapulmonary shunting. Pediatric Transplantation, 2009, 13(8): 1041-1047.
[7] K. Ringe, E. Schirg, M. Melter, et al. Congenital absence of the portal vein (CAPV). Two cases of Abernethy malformation type 1 and review of the literature. Radiologe, 2008, 48(5): 493-502.
[8] M. J. Osorio, A. Bonow, G. J. Bond, et al. Abernethy malforma- tion complicated by hepatopulmonary syndrome and a liver mass successfully treated by liver transplantation. Pediatric Transplantation, 2011, 15(7): E149-E151.
[9] T. Hori, Y. Yonekawa, S. Okamoto, et al. Pediatric orthotopic living-donor liver transplantation cures pulmonary hypertension caused by Abernethy malformation type Ib. Pediatric Transplan- tation, 2011, 15(3): e47-e52.
[10] A. Kumar, J. Kumar, R. Aggarwal, et al. Abernethy malforma- tion with portal vein aneurysm. Diagnostic and Interventional Radiology, 2008, 14(3): 143-146.
[11] S. H. Chandrashekhara, A. S. Bhalla, A. K. Gupta, et al. Aber- nethy malformation with portal vein aneurysm in a child. Jour- nal of Indian Association of Pediatric Surgeons, 2011, 16(1): 21-23.