Comprehensive Review of Abernethy Malformation Cases in China_West China Medical Journal

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Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu, Sichuan,610041, P. R. China;

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Keywords：

Abernethy畸形; 门静脉缺如; 门腔分流; 儿童

DOI：

10.7507/1002-0179.20130233

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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。

Citation： WANG Yamei,ZHOU Yanru,TAO Yuhong.. Comprehensive Review of Abernethy Malformation Cases in China. West China Medical Journal, 2013, 28(5): 756-760. doi: 10.7507/1002-0179.20130233 Copy

1.	Alonso-Gamarra E, Parron M, Perez A, et al. Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review[J]. Radiographics, 2011, 31(3): 707-722.
2.	Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies[J]. J Pediatr Surg, 1994, 29(9): 1239-1241.
3.	Kohda E, Saeki M, Nakano M, et al. Congenital absence of the portal vein in a boy[J]. Pediatr Radiol, 1999, 29(4): 235-237.
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, 17(1): 83-84.
5.	罗优优, 马鸣, 王培鑫, 等. Abernethy畸形一例[J]. 中华儿科杂志, 2008, 46(12): 937-938.
6.	华俊, 张余, 牟玮. 先天性门静脉缺如一例[J]. 中华放射学杂志, 2010, 44(11): 1218-1219.
7.	陈国庭. Abernethy畸形一例[J]. 中华普通外科杂志, 2005, 20(5): 328.
8.	李震, 汪忠镐, 卞策. 儿童Abernethy畸形致门静脉高压症一例[J]. 中华普通外科杂志, 2009, 24(6): 516.
9.	张连海, 庄岩, 王克来. 先天性门静脉缺如一例[J]. 中华小儿外科杂志, 2010, 31(2):106.
10.	卢菁菁, 杨晓英, 王润兰, 等. 超声诊断先天性门静脉缺失1例报告[J]. 吉林医学, 2006, 27(3): 322-322.
11.	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, 7(2): 168-169.
12.	杨性安, 王刚, 朱通伟. 超声诊断Abernethy畸形一例分析[J]. 现代实用医学, 2010, 22(8): 952-953.
13.	王磊, 李欣, 王春祥, 等. 先天性门静脉缺如一例[J]. 临床放射学杂志, 2008, 27(12): 1748.
14.	姚永良, 顾松, 褚珺, 等. Abernethy畸形一例[J]. 中华小儿外科杂志, 2012, 33(6): 477-478.
15.	陈勇, 申鹏, 韦传军, 等. Abernethy畸形的影像学表现[J]. 中华放射学杂志, 2005, 39(8): 874-876.
16.	陈诚豪, 王大勇, 李小松, 等. 先天性肝外门腔静脉分流并下消化道出血1例[J]. 临床小儿外科杂志, 2007, 6(2): 78-79.
17.	Hu GH, Shen LG, Yang J, et al. Insight into congenital absence of the portal vein: is it rare?[J]. World J Gastroenterol, 2008, 14(39): 5969-5979.
18.	Chandler TM, Heran MK, Chang SD, et al. Multiple focal nodular hyperplasia lesions of the liver associated with congenital absence of the portal vein[J]. Magn Reson Imaging, 2011, 29(6): 881-886.
19.	Lisovsky M, Konstas AA, Misdraji J. Congenital extrahepatic portosystemic shunts (Abernethy malformation): a histopathologic evaluation[J]. Am J Surg Pathol, 2011, 35(9): 1381-1390.
20.	Iida T, Ogura Y, Doi H, et al. Successful treatment of pulmonary hypertension secondary to congenital extrahepatic portocaval shunts (Abernethy type 2) by living donor liver transplantation after surgical shunt ligation[J]. Transpl Int, 2010, 23(1): 105-109.
21.	Ding XY, Chen F, Zhao XX, et al. A rare cause of cyanosis: hepatopulmonary syndrome caused by congenital extrahepatic portosystemic shunt[J]. Case Report Vasc Med, 2011, 2011(1): 508171-508173.
22.	Grazioli L, Alberti D, Olivetti L, et al. Congenital absence of portal vein with nodular regenerative hyperplasia of the liver[J]. Eur Radiol, 2000, 10(5): 820-825.
23.	Niwa T, Aida N, Tachibana K, et al. Congenital absence of the portal vein: clinical and radiologic findings[J]. J Comput Assist Tomogr, 2002, 26(5): 681-686.
24.	Mistinova J, Valacsai F, Varga I. Congenital absence of the portal vein-Case report and a review of literature[J]. Clin Anat, 2010, 23(1): 750-758.
25.	Noe JA, Pittman HC, Burton EM. Congenital absence of the portal vein in a child with Turner syndrome[J]. Pediatr Radiol, 2006, 36(6): 566-568.
26.	侯志彬, 李欣, 王春祥, 等. MSCT血管造影诊断儿童门静脉畸形[J]. 中国医学影像技术, 2011, 27(10): 2083-2086.
27.	Franchi-Abella S, Branchereau S, Lambert V, et al. Complications of congenital portosystemic shunts in children: therapeutic options and outcomes[J]. J Pediatr Gastroenterol Nutr, 2010, 51(3): 322-330.
28.	Sanada Y, Urahashi T, Ihara Y, et al. The role of operative intervention in management of congenital extrahepatic portosystemic shunt[J]. Surgery, 2012, 151(3): 404-411.
29.	Lee SA, Lee YS, Lee KS, et al. Congenital intrahepatic portosystemic venous shunt and liver mass in a child patient: successful endovascular treatment with an amplatzer vascular plug (AVP)[J]. Korean J Radiol, 2010, 11(5): 583-586.

1. 　Alonso-Gamarra E, Parron M, Perez A, et al. Clinical and radiologic manifestations of congenital extrahepatic portosystemic shunts: a comprehensive review[J]. Radiographics, 2011, 31(3): 707-722.
2. 　Morgan G, Superina R. Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies[J]. J Pediatr Surg, 1994, 29(9): 1239-1241.
3. 　Kohda E, Saeki M, Nakano M, et al. Congenital absence of the portal vein in a boy[J]. Pediatr Radiol, 1999, 29(4): 235-237.
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, 17(1): 83-84.
5. 　罗优优, 马鸣, 王培鑫, 等. Abernethy畸形一例[J]. 中华儿科杂志, 2008, 46(12): 937-938.
6. 　华俊, 张余, 牟玮. 先天性门静脉缺如一例[J]. 中华放射学杂志, 2010, 44(11): 1218-1219.
7. 　陈国庭. Abernethy畸形一例[J]. 中华普通外科杂志, 2005, 20(5): 328.
8. 　李震, 汪忠镐, 卞策. 儿童Abernethy畸形致门静脉高压症一例[J]. 中华普通外科杂志, 2009, 24(6): 516.
9. 　张连海, 庄岩, 王克来. 先天性门静脉缺如一例[J]. 中华小儿外科杂志, 2010, 31(2):106.
10. 　卢菁菁, 杨晓英, 王润兰, 等. 超声诊断先天性门静脉缺失1例报告[J]. 吉林医学, 2006, 27(3): 322-322.
11. 　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, 7(2): 168-169.
12. 　杨性安, 王刚, 朱通伟. 超声诊断Abernethy畸形一例分析[J]. 现代实用医学, 2010, 22(8): 952-953.
13. 　王磊, 李欣, 王春祥, 等. 先天性门静脉缺如一例[J]. 临床放射学杂志, 2008, 27(12): 1748.
14. 　姚永良, 顾松, 褚珺, 等. Abernethy畸形一例[J]. 中华小儿外科杂志, 2012, 33(6): 477-478.
15. 　陈勇, 申鹏, 韦传军, 等. Abernethy畸形的影像学表现[J]. 中华放射学杂志, 2005, 39(8): 874-876.
16. 　陈诚豪, 王大勇, 李小松, 等. 先天性肝外门腔静脉分流并下消化道出血1例[J]. 临床小儿外科杂志, 2007, 6(2): 78-79.
17. 　Hu GH, Shen LG, Yang J, et al. Insight into congenital absence of the portal vein: is it rare?[J]. World J Gastroenterol, 2008, 14(39): 5969-5979.
18. 　Chandler TM, Heran MK, Chang SD, et al. Multiple focal nodular hyperplasia lesions of the liver associated with congenital absence of the portal vein[J]. Magn Reson Imaging, 2011, 29(6): 881-886.
19. 　Lisovsky M, Konstas AA, Misdraji J. Congenital extrahepatic portosystemic shunts (Abernethy malformation): a histopathologic evaluation[J]. Am J Surg Pathol, 2011, 35(9): 1381-1390.
20. 　Iida T, Ogura Y, Doi H, et al. Successful treatment of pulmonary hypertension secondary to congenital extrahepatic portocaval shunts (Abernethy type 2) by living donor liver transplantation after surgical shunt ligation[J]. Transpl Int, 2010, 23(1): 105-109.
21. 　Ding XY, Chen F, Zhao XX, et al. A rare cause of cyanosis: hepatopulmonary syndrome caused by congenital extrahepatic portosystemic shunt[J]. Case Report Vasc Med, 2011, 2011(1): 508171-508173.
22. 　Grazioli L, Alberti D, Olivetti L, et al. Congenital absence of portal vein with nodular regenerative hyperplasia of the liver[J]. Eur Radiol, 2000, 10(5): 820-825.
23. 　Niwa T, Aida N, Tachibana K, et al. Congenital absence of the portal vein: clinical and radiologic findings[J]. J Comput Assist Tomogr, 2002, 26(5): 681-686.
24. 　Mistinova J, Valacsai F, Varga I. Congenital absence of the portal vein-Case report and a review of literature[J]. Clin Anat, 2010, 23(1): 750-758.
25. 　Noe JA, Pittman HC, Burton EM. Congenital absence of the portal vein in a child with Turner syndrome[J]. Pediatr Radiol, 2006, 36(6): 566-568.
26. 　侯志彬, 李欣, 王春祥, 等. MSCT血管造影诊断儿童门静脉畸形[J]. 中国医学影像技术, 2011, 27(10): 2083-2086.
27. 　Franchi-Abella S, Branchereau S, Lambert V, et al. Complications of congenital portosystemic shunts in children: therapeutic options and outcomes[J]. J Pediatr Gastroenterol Nutr, 2010, 51(3): 322-330.
28. 　Sanada Y, Urahashi T, Ihara Y, et al. The role of operative intervention in management of congenital extrahepatic portosystemic shunt[J]. Surgery, 2012, 151(3): 404-411.
29. 　Lee SA, Lee YS, Lee KS, et al. Congenital intrahepatic portosystemic venous shunt and liver mass in a child patient: successful endovascular treatment with an amplatzer vascular plug (AVP)[J]. Korean J Radiol, 2010, 11(5): 583-586.

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Comprehensive Review of Abernethy Malformation Cases in China

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