李震 1 , 汪忠鎬 1,2 , 趙克 3 , 卞策 3 , 韓冰 3 , 郭巍 3 , 崔琦 3 , 霍小森 1 , 王雷永 1
  • 1.中國航空工業(yè)中心醫(yī)院血管外科（北京 100012）;;
  • 2.首都醫(yī)科大學(xué)血管外科研究所（北京 100053）;;
  • 3.中國人民解放軍第二炮兵總醫(yī)院心胸血管外科（北京 100088）;

目的  探討Abernethy畸形致下消化道出血的診斷及治療方法。
方法  回顧1例Abernethy畸形患者的診治情況，并結(jié)合相關(guān)文獻進行分析。
結(jié)果  血管造影確診后經(jīng)積極術(shù)前準(zhǔn)備，行剖腹探查、乙狀結(jié)腸造瘺術(shù)，術(shù)后3周開始給予中藥保留灌腸至術(shù)后7周，6個月關(guān)閉造瘺,3周后繼續(xù)輔以中藥保留灌腸4周?；颊唔樌謴?fù)出院，隨訪9個月未再次出血。
結(jié)論  正確的診斷及合理的治療方案是救治的關(guān)鍵。

引用本文： 李震,汪忠鎬,趙克,卞策,韓冰,郭巍,崔琦,霍小森,王雷永. Abernethy畸形致下消化道出血的診治. 中國普外基礎(chǔ)與臨床雜志, 2010, 17(1): 83-84. doi: 復(fù)制

1. Abernethy J. Account of two instances of uncommon formation in the viscera of the human body [Ｊ]. Phil Trans R Soc, 1793; 83: 5966.
2. 陳國庭, 李恒平. Abernethy畸形一例 [Ｊ]. 中華普通外科雜志, 2005; 20(5): 328.
3. 陳誠豪, 王大勇, 李小松, 等. 先天性肝外門腔靜脈分流并下消化道出血1例 [Ｊ]. 臨床小兒外科雜志, 2007; 6(2): 7879.
4. Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic portosystemic shunts [Ｊ]. Pediatr Radiol, 2003; 33(9): 614620.
5. Kumar A, Kumar J, Aggarwal R, et al. Abernethy malformation with portal vein aneurysm [Ｊ]. Diagn Interv Radiol, 2008; 14(3): 143146.
6. Oei M, Wessling J. Abernethy malformation-congenital aplasia of the portal vein a 29yearold patient [Ｊ]. Rofo, 2007; 179(2): 167169.
7. Abernethy LJ. Classification and imaging of vascular malformations in children [Ｊ]. Eur Radiol, 2003; 13(11): 24832497.
8. Howard ER, Davenport M. Congenital extrahepatic portocaval shuntsthe Abernethy malformation (Review) [Ｊ]. J Pediatr Surg, 1997; 32(3): 494497.
9. Moon SB, Park KW, Jung SE. Abnormal direct entry of the umbilical vein into the portal vein: report of a case [Ｊ]. Pediatr Surg Int, 2008; 24(11): 12431245.
10. Ringe K, Schirg E, Melter M, et al. Congenital absence of the portal vein (CAPV). Two cases of Abernethy malformation type 1 and review of the literature [Ｊ]. Radiologe, 2008; 48(5): 493502.
11. Witters P, Maleux G, George C, et al. Congenital venovenous malformations of the liver: widely variable clinical presentations [Ｊ]. J Gastroenterol Hepatol, 2008; 23(8 Pt 2): e390e394.
12. Stringer MD. The clinical anatomy of congenital portosystemic venous shunts [Ｊ]. Clin Anat, 2008; 21(2): 147157.
13. Morikawa N, Honna T, Kuroda T, et al. Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an Abernethy malformation [Ｊ]. J Pediatr Surg, 2008; 43(2): e35e38.
14. Emre S, Arnon R, Cohen E, et al. Resolution of hepatopulmonary syndrome after auxiliary partial orthotopic liver transplantation in Abernethy malformation. A case report [Ｊ]. Liver Transpl, 2007; 13(12): 16621668.
15. 吳繼東, 汪忠鎬, 王仕華, 等. 兒童肝外型門脈高壓癥的診治 [Ｊ]. 中國現(xiàn)代手術(shù)學(xué)雜志, 2003; 7(5): 375377.
16. 王欣博, 童中勛, 劉威, 等. 門靜脈海綿樣變性外科治療的研究進展 [Ｊ]. 中國普外基礎(chǔ)與臨床雜志， 2008; 15(12): 877879.
17. 蓋保東, 王正峰, 劉東. 門脈高壓性胃病 [Ｊ]. 中國普外基礎(chǔ)與臨床雜志, 2008; 15(12): 880882.
18. Tercier S, Delarue A, Rouault F, et al. Congenital portocaval fistula associated with hepatopulmonary syndrome: ligation vs liver transplantation[Ｊ]. J Pediatr Surg, 2006; 41(2): e1e3.
  1. 1. Abernethy J. Account of two instances of uncommon formation in the viscera of the human body [Ｊ]. Phil Trans R Soc, 1793; 83: 5966.
  2. 2. 陳國庭, 李恒平. Abernethy畸形一例 [Ｊ]. 中華普通外科雜志, 2005; 20(5): 328.
  3. 3. 陳誠豪, 王大勇, 李小松, 等. 先天性肝外門腔靜脈分流并下消化道出血1例 [Ｊ]. 臨床小兒外科雜志, 2007; 6(2): 7879.
  4. 4. Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic portosystemic shunts [Ｊ]. Pediatr Radiol, 2003; 33(9): 614620.
  5. 5. Kumar A, Kumar J, Aggarwal R, et al. Abernethy malformation with portal vein aneurysm [Ｊ]. Diagn Interv Radiol, 2008; 14(3): 143146.
  6. 6. Oei M, Wessling J. Abernethy malformation-congenital aplasia of the portal vein a 29yearold patient [Ｊ]. Rofo, 2007; 179(2): 167169.
  7. 7. Abernethy LJ. Classification and imaging of vascular malformations in children [Ｊ]. Eur Radiol, 2003; 13(11): 24832497.
  8. 8. Howard ER, Davenport M. Congenital extrahepatic portocaval shuntsthe Abernethy malformation (Review) [Ｊ]. J Pediatr Surg, 1997; 32(3): 494497.
  9. 9. Moon SB, Park KW, Jung SE. Abnormal direct entry of the umbilical vein into the portal vein: report of a case [Ｊ]. Pediatr Surg Int, 2008; 24(11): 12431245.
  10. 10. Ringe K, Schirg E, Melter M, et al. Congenital absence of the portal vein (CAPV). Two cases of Abernethy malformation type 1 and review of the literature [Ｊ]. Radiologe, 2008; 48(5): 493502.
  11. 11. Witters P, Maleux G, George C, et al. Congenital venovenous malformations of the liver: widely variable clinical presentations [Ｊ]. J Gastroenterol Hepatol, 2008; 23(8 Pt 2): e390e394.
  12. 12. Stringer MD. The clinical anatomy of congenital portosystemic venous shunts [Ｊ]. Clin Anat, 2008; 21(2): 147157.
  13. 13. Morikawa N, Honna T, Kuroda T, et al. Resolution of hepatopulmonary syndrome after ligation of a portosystemic shunt in a pediatric patient with an Abernethy malformation [Ｊ]. J Pediatr Surg, 2008; 43(2): e35e38.
  14. 14. Emre S, Arnon R, Cohen E, et al. Resolution of hepatopulmonary syndrome after auxiliary partial orthotopic liver transplantation in Abernethy malformation. A case report [Ｊ]. Liver Transpl, 2007; 13(12): 16621668.
  15. 15. 吳繼東, 汪忠鎬, 王仕華, 等. 兒童肝外型門脈高壓癥的診治 [Ｊ]. 中國現(xiàn)代手術(shù)學(xué)雜志, 2003; 7(5): 375377.
  16. 16. 王欣博, 童中勛, 劉威, 等. 門靜脈海綿樣變性外科治療的研究進展 [Ｊ]. 中國普外基礎(chǔ)與臨床雜志， 2008; 15(12): 877879.
  17. 17. 蓋保東, 王正峰, 劉東. 門脈高壓性胃病 [Ｊ]. 中國普外基礎(chǔ)與臨床雜志, 2008; 15(12): 880882.
  18. 18. Tercier S, Delarue A, Rouault F, et al. Congenital portocaval fistula associated with hepatopulmonary syndrome: ligation vs liver transplantation[Ｊ]. J Pediatr Surg, 2006; 41(2): e1e3.