• 1.中國(guó)航空工業(yè)中心醫(yī)院血管外科(北京 100012);;
  • 2.中國(guó)醫(yī)學(xué)科學(xué)院 中國(guó)協(xié)和醫(yī)學(xué)院 北京協(xié)和醫(yī)院血管外科中心(北京 100730);;
  • 3.首都醫(yī)科大學(xué)血管外科研究所(北京 100053);;
  • 4.第二炮兵總醫(yī)院血管外科(北京 100088);

目的  探討肝前型門靜脈高壓癥的臨床特點(diǎn)、診斷、治療方法的選擇及療效。方法  回顧性分析第二炮兵總醫(yī)院及北京協(xié)和醫(yī)院2000年1月至2009年5月期間收治的46例肝前型門靜脈高壓癥(包括2例Abernethy畸形)患者的臨床資料。 全部患者均根據(jù)間接門靜脈造影、CT血管造影和(或)彩超檢查結(jié)果確診為肝前型門靜脈高壓癥。行腸系膜上靜脈-下腔靜脈分流術(shù)23例; 脾切除、脾靜脈-腎靜脈分流術(shù)8例; 門靜脈-下腔靜脈分流術(shù)1例; 附臍靜脈-頸內(nèi)靜脈分流術(shù)2例; 門奇靜脈斷流術(shù)3例; 脾切除、門奇靜脈斷流術(shù)1例; 乙狀結(jié)腸暫時(shí)性造瘺,6個(gè)月后閉瘺1例; 大部分小腸切除術(shù)1例; 經(jīng)股動(dòng)脈插管溶栓4例; 未行手術(shù)2例,僅給予護(hù)肝及對(duì)癥治療。結(jié)果  44例患者隨訪2個(gè)月~5年,平均23.4個(gè)月,1例未手術(shù)者失訪。 34例行分流手術(shù)治療的患者術(shù)后脾功能亢進(jìn)癥狀消失,未再發(fā)生上消化道出血; 行斷流術(shù)者術(shù)后13個(gè)月及2 年因再次出血行腸系膜上靜脈-下腔靜脈分流術(shù)2例; 斷流術(shù)后8個(gè)月因再次出血死亡1例; 溶栓治療后40 d因腸壞死死亡1例,1例未手術(shù)的患兒出院4個(gè)月后再次出現(xiàn)黑便經(jīng)保守治療好轉(zhuǎn)。結(jié)論  肝前型門靜脈高壓癥的治療以降低門靜脈壓力為主,各種分流手術(shù)及腸系膜上動(dòng)脈和(或)脾動(dòng)脈置管溶栓安全有效,但需根據(jù)個(gè)體情況施行。

引用本文: 李震,吳繼東,汪忠鎬,卞策,侯高峰,霍小森,王雷永. 肝前型門靜脈高壓癥的診治. 中國(guó)普外基礎(chǔ)與臨床雜志, 2009, 16(12): 1005-1009. doi: 復(fù)制

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1. 汪忠鎬, 劉勇. 肝外型門靜脈高壓癥(摘譯) [J]. 腹部外科, 1994; 7(2): 95-96.
2. Sarin SK, Kapoor D. Non-cirrhotic portal fibrosis: current concepts and management [J]. J Gastroenterol Hepatol, 2002; 17(5): 526-534.
3. Wang ZG, Jones RS. Budd-chiari Syndrome [J]. Curr Probl Surg, 1996; 33(2): 81-211.
4. Gauthier F. Recent concepts regarding extra-hepatic portal hypertension [J]. Semin Pediatr Surg, 2005; 14(4): 216-225.
5. 姜洪池. 肝硬變門靜脈高壓癥外科治療的今日思考 [J]. 中國(guó)普外基礎(chǔ)與臨床雜志, 2008; 15(12): 871-872.
6. 李曉東, 陳學(xué)明, 汪忠鎬, 等. 肝外型門靜脈高壓癥外科治療42例經(jīng)驗(yàn) [J]. 中國(guó)普外基礎(chǔ)與臨床雜志, 2002; 2(10): 10-12.
7. 陳學(xué)明, 汪忠鎬, 王仕華. 肝總動(dòng)脈-脾靜脈瘺致門靜脈高壓癥1例報(bào)告 [J]. 中華普通外科雜志, 1998; 13(2): 107.
8. Yin DL, Liu LX, Zhang SG, et al. Portal hypertension resulted from paroxysmal nocturnal hemoglobinuria: a case report and review of literature [J]. Int J Hematol, 2009; 89(3): 302-304.
9. Abramowsky C, Romero R, Heffron T. Pathology of noncirrhotic portal hypertension: clinicopathologic study in pediatric patients [J]. Pediatr Dev Pathol, 2003; 6(5): 421-426.
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. 吳繼東, 汪忠鎬, 王仕華, 等. 間接門靜脈造影對(duì)診治肝前型門靜脈高壓癥的意義 [J]. 中國(guó)普通外科雜志, 2004; 13(1): 37-39.
12. Tilaanus HW. Budd-Chiari Syndrome [J]. Br J Surg, 1995; 82(8): 1023-1030.
13. 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(jìn)展 [J]. 中國(guó)普外基礎(chǔ)與臨床雜志, 2008; 15(12): 877-879.
14. 鄧旦, 廖明松, 吳曉波, 等. 門靜脈癌栓與血栓的超聲造影研究 [J]. 中國(guó)普外基礎(chǔ)與臨床雜志, 2008; 15(11): 868-869.
15. Orloff MJ, Orloff MS, Girard B, et al. Bleeding esophago-gastric varices from extrahepatic portal hypertension: 40 years’ experience with portal-systemic shunt [J]. J Am Coll Surg, 2002; 194(6): 717-728.
16. Fuchs J, Warmann S, Kardorff R, et al. Mesenterico-left portal vein bypass in children with congenital extrahepatic portal vein thrombosis: a unique curative approach [J]. J Pediatr Gastroenterol Nutr, 2003; 36(2): 213-216.
17. Wolff M, Hirner A. Current state of portosystemic shunt su-rgery [J]. Langenbecks Arch Surg, 2003; 388(3): 141-149.
18. Sigalet DL, Mayer S, Blanchard H. Portal venous decompr-ession with H-type mesocaval shunt using autologous vein graft: A North American experience [J]. J Pediatr Surg, 2001; 36(1): 91-96.
19. 吳繼東, 汪忠鎬, 王仕華, 等. 兒童肝外型門靜脈高壓癥的診治 [J]. 中國(guó)現(xiàn)代手術(shù)學(xué)雜志, 2003; 7(5): 375-377.
20. Moon SB, Jung SE, Ha JW, et al. The usefulness of distal splenorenal shunt in children with portal hypertension for the treatment of severe thrombocytopenia and leucopenia [J]. World J Surg, 2008; 32(3): 483-487.
21. 任輝, 李志偉, 肖朝輝, 等. 門靜脈高壓癥斷流術(shù)后上消化道再出血診治體會(huì) [J]. 中國(guó)普外基礎(chǔ)與臨床雜志, 2008; 15 (6): 448-449.
22. Feng LS, Li K, Peng QP, et al. Triplex operation for portal hypertension with esophageal variceal bleeding: report of 140 cases [J]. Hepatobiliary Pancreat Dis Int, 2004; 3(4): 534-537.
23. 侯鈺, 孫廣新, 楊俊山. 門靜脈高壓時(shí)臍靜脈或附臍靜脈的血流動(dòng)力學(xué)改變及臨床意義 [J]. 肝膽外科雜志, 2004; 12(5): 332-334.
24. Safieddine N, Mamazza J, Common A, et al. Splenic and superior mesenteric artery thrombolytic infusion therapy for acute portal and mesenteric vein thrombosis [J]. Can J Surg, 2007; 50(1): 68-69.
25. 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.
26. 陳誠(chéng)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.
  1. 1. 汪忠鎬, 劉勇. 肝外型門靜脈高壓癥(摘譯) [J]. 腹部外科, 1994; 7(2): 95-96.
  2. 2. Sarin SK, Kapoor D. Non-cirrhotic portal fibrosis: current concepts and management [J]. J Gastroenterol Hepatol, 2002; 17(5): 526-534.
  3. 3. Wang ZG, Jones RS. Budd-chiari Syndrome [J]. Curr Probl Surg, 1996; 33(2): 81-211.
  4. 4. Gauthier F. Recent concepts regarding extra-hepatic portal hypertension [J]. Semin Pediatr Surg, 2005; 14(4): 216-225.
  5. 5. 姜洪池. 肝硬變門靜脈高壓癥外科治療的今日思考 [J]. 中國(guó)普外基礎(chǔ)與臨床雜志, 2008; 15(12): 871-872.
  6. 6. 李曉東, 陳學(xué)明, 汪忠鎬, 等. 肝外型門靜脈高壓癥外科治療42例經(jīng)驗(yàn) [J]. 中國(guó)普外基礎(chǔ)與臨床雜志, 2002; 2(10): 10-12.
  7. 7. 陳學(xué)明, 汪忠鎬, 王仕華. 肝總動(dòng)脈-脾靜脈瘺致門靜脈高壓癥1例報(bào)告 [J]. 中華普通外科雜志, 1998; 13(2): 107.
  8. 8. Yin DL, Liu LX, Zhang SG, et al. Portal hypertension resulted from paroxysmal nocturnal hemoglobinuria: a case report and review of literature [J]. Int J Hematol, 2009; 89(3): 302-304.
  9. 9. Abramowsky C, Romero R, Heffron T. Pathology of noncirrhotic portal hypertension: clinicopathologic study in pediatric patients [J]. Pediatr Dev Pathol, 2003; 6(5): 421-426.
  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. 吳繼東, 汪忠鎬, 王仕華, 等. 間接門靜脈造影對(duì)診治肝前型門靜脈高壓癥的意義 [J]. 中國(guó)普通外科雜志, 2004; 13(1): 37-39.
  12. 12. Tilaanus HW. Budd-Chiari Syndrome [J]. Br J Surg, 1995; 82(8): 1023-1030.
  13. 13. 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(jìn)展 [J]. 中國(guó)普外基礎(chǔ)與臨床雜志, 2008; 15(12): 877-879.
  14. 14. 鄧旦, 廖明松, 吳曉波, 等. 門靜脈癌栓與血栓的超聲造影研究 [J]. 中國(guó)普外基礎(chǔ)與臨床雜志, 2008; 15(11): 868-869.
  15. 15. Orloff MJ, Orloff MS, Girard B, et al. Bleeding esophago-gastric varices from extrahepatic portal hypertension: 40 years’ experience with portal-systemic shunt [J]. J Am Coll Surg, 2002; 194(6): 717-728.
  16. 16. Fuchs J, Warmann S, Kardorff R, et al. Mesenterico-left portal vein bypass in children with congenital extrahepatic portal vein thrombosis: a unique curative approach [J]. J Pediatr Gastroenterol Nutr, 2003; 36(2): 213-216.
  17. 17. Wolff M, Hirner A. Current state of portosystemic shunt su-rgery [J]. Langenbecks Arch Surg, 2003; 388(3): 141-149.
  18. 18. Sigalet DL, Mayer S, Blanchard H. Portal venous decompr-ession with H-type mesocaval shunt using autologous vein graft: A North American experience [J]. J Pediatr Surg, 2001; 36(1): 91-96.
  19. 19. 吳繼東, 汪忠鎬, 王仕華, 等. 兒童肝外型門靜脈高壓癥的診治 [J]. 中國(guó)現(xiàn)代手術(shù)學(xué)雜志, 2003; 7(5): 375-377.
  20. 20. Moon SB, Jung SE, Ha JW, et al. The usefulness of distal splenorenal shunt in children with portal hypertension for the treatment of severe thrombocytopenia and leucopenia [J]. World J Surg, 2008; 32(3): 483-487.
  21. 21. 任輝, 李志偉, 肖朝輝, 等. 門靜脈高壓癥斷流術(shù)后上消化道再出血診治體會(huì) [J]. 中國(guó)普外基礎(chǔ)與臨床雜志, 2008; 15 (6): 448-449.
  22. 22. Feng LS, Li K, Peng QP, et al. Triplex operation for portal hypertension with esophageal variceal bleeding: report of 140 cases [J]. Hepatobiliary Pancreat Dis Int, 2004; 3(4): 534-537.
  23. 23. 侯鈺, 孫廣新, 楊俊山. 門靜脈高壓時(shí)臍靜脈或附臍靜脈的血流動(dòng)力學(xué)改變及臨床意義 [J]. 肝膽外科雜志, 2004; 12(5): 332-334.
  24. 24. Safieddine N, Mamazza J, Common A, et al. Splenic and superior mesenteric artery thrombolytic infusion therapy for acute portal and mesenteric vein thrombosis [J]. Can J Surg, 2007; 50(1): 68-69.
  25. 25. 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.
  26. 26. 陳誠(chéng)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.