Diagnosis and Treatment of Prehepatic Portal Hypertension_CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Authors：

LI Zhen ¹ , WU Jidong ² , WANG Zhonggao ^1,3 , BIAN Ce ⁴ , HOU Gaofeng ⁴ , HUO Xiaosen ¹ , WANG Leiyong ¹

1.Vascular Surgery, General Hospital of Chinese Aviation, Beijing 100012, China;;
2.Center of Vascular Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences &;
Peking Union Medical College, Beijing 100730， China;;
3.Vascular Institute, Capital University of Medical Science, Beijing 100053, China;;
4.Vascular Surgery, The 2nd Artillery General Hospital, Beijing 100088, China;

Corresponding author：

Keywords：

Prehepatic portal hypertension; Abernethy abnormality; Paraumbilical vein; Shunt; Thrombolytic therapy

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Objective To explore the clinical presentation and diagnosis and treatment of prehepatic portal hypertension （PPH） and discuss its surgical strategies. Methods Forty-six cases of PPH treated in the 2nd Artillery General Hospital and Peking Union Medical College Hospital from January 2000 to May 2009 were analyzed retrospectively, including 2 cases of Abernethy abnormality. All patients were evaluated by indirect portal vein angiography, CT angiography and (or) portal duplex system Doppler ultrasonography before treament. Surgical strategies included: 23 cases with meso-caval shunt, 8 cases with splenectomy and spleno-renal vein shunt, 1 case with porta-caval shunt, 2 cases with paraumbilical vein-jugular vein shunt, 3 cases with portal azygous disconnection, 1 cases with splenectomy and portal azygous disconnection, 1 case with sigmoidostomy and closed the fistula of sigmoid six months later, 1 case with resection of part of small intestine due to acute extensive thrombosis of portal vein system, 4 cases with selective superior mesenteric artery and (or) splenic artery thrombolytic infusion therapy, 2 cases remained no-surgical option and underwent conservative treatment. Results Forty-four patients were followed-up from 2 months to 5 years， average of 23.4 months, one patient without surgical treatment was lost. Satisfactory outcomes were obtained in 34 patients with various shunts, which expressed as a release of hypersplenism and gastrointestinal hemorrhage. Two cases were treated with meso-caval shunt because of rehemorrhage in month 13 and 24 and one died in month 8 after disconnection, one died on day 40 after thrombolytic therapy due to putrescence of intestines, one who remained no-surgical option underwent hemorrhage 4 months later, and then went well by conservative treatment. Conclusion The key of treatment of PPH is to reduce the pressure of hepatic portal vein. Surgical managements of shunt and selective superior mesenteric artery and (or) splenic artery thrombolytic infusion therapy are safe and effective, but individual treatment strategy should be performed.

Citation： LI Zhen,WU Jidong,WANG Zhonggao,BIAN Ce,HOU Gaofeng,HUO Xiaosen,WANG Leiyong. Diagnosis and Treatment of Prehepatic Portal Hypertension. CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY, 2009, 16(12): 1005-1009. doi: Copy

1.	汪忠镐，刘勇. 肝外型门静脉高压症（摘译） [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. 肝硬变门静脉高压症外科治疗的今日思考 [J]. 中国普外基础与临床杂志, 2008； 15(12)： 871-872.
6.	李晓东，陈学明，汪忠镐，等. 肝外型门静脉高压症外科治疗42例经验 [J]. 中国普外基础与临床杂志， 2002； 2（10）： 10-12.
7.	陈学明，汪忠镐，王仕华. 肝总动脉-脾静脉瘘致门静脉高压症1例报告 [J]. 中华普通外科杂志， 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.	吴继东，汪忠镐，王仕华，等. 间接门静脉造影对诊治肝前型门静脉高压症的意义 [J]. 中国普通外科杂志， 2004； 13(1): 37-39.
12.	Tilaanus HW. Budd-Chiari Syndrome [J]. Br J Surg， 1995； 82（8）： 1023-1030.
13.	王欣博，童中勋，刘威，等. 门静脉海绵样变性外科治疗的研究进展 [J]. 中国普外基础与临床杂志, 2008； 15(12)： 877-879.
14.	邓旦, 廖明松, 吴晓波, 等. 门静脉癌栓与血栓的超声造影研究 [J]. 中国普外基础与临床杂志, 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.	吴继东，汪忠镐，王仕华，等. 儿童肝外型门静脉高压症的诊治 [J]. 中国现代手术学杂志， 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.	任辉, 李志伟, 肖朝辉, 等. 门静脉高压症断流术后上消化道再出血诊治体会 [J]. 中国普外基础与临床杂志, 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.	侯钰，孙广新，杨俊山. 门静脉高压时脐静脉或附脐静脉的血流动力学改变及临床意义 [J]. 肝胆外科杂志， 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.	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.

1. 汪忠镐，刘勇. 肝外型门静脉高压症（摘译） [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. 肝硬变门静脉高压症外科治疗的今日思考 [J]. 中国普外基础与临床杂志, 2008； 15(12)： 871-872.
6. 李晓东，陈学明，汪忠镐，等. 肝外型门静脉高压症外科治疗42例经验 [J]. 中国普外基础与临床杂志， 2002； 2（10）： 10-12.
7. 陈学明，汪忠镐，王仕华. 肝总动脉-脾静脉瘘致门静脉高压症1例报告 [J]. 中华普通外科杂志， 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. 吴继东，汪忠镐，王仕华，等. 间接门静脉造影对诊治肝前型门静脉高压症的意义 [J]. 中国普通外科杂志， 2004； 13(1): 37-39.
12. Tilaanus HW. Budd-Chiari Syndrome [J]. Br J Surg， 1995； 82（8）： 1023-1030.
13. 王欣博，童中勋，刘威，等. 门静脉海绵样变性外科治疗的研究进展 [J]. 中国普外基础与临床杂志, 2008； 15(12)： 877-879.
14. 邓旦, 廖明松, 吴晓波, 等. 门静脉癌栓与血栓的超声造影研究 [J]. 中国普外基础与临床杂志, 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. 吴继东，汪忠镐，王仕华，等. 儿童肝外型门静脉高压症的诊治 [J]. 中国现代手术学杂志， 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. 任辉, 李志伟, 肖朝辉, 等. 门静脉高压症断流术后上消化道再出血诊治体会 [J]. 中国普外基础与临床杂志, 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. 侯钰，孙广新，杨俊山. 门静脉高压时脐静脉或附脐静脉的血流动力学改变及临床意义 [J]. 肝胆外科杂志， 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. 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.

CHINESE JOURNAL OF BASES AND CLINICS IN GENERAL SURGERY

Diagnosis and Treatment of Prehepatic Portal Hypertension

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