Semin Liver Dis 2002; 22(1): 043-058
DOI: 10.1055/s-2002-23206
Copyright © 2002 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Extrahepatic Portal Vein Obstruction

Shiv K. Sarin, Shri Ram Agarwal
  • Department of Gastroenterology, G.B. Pant Hospital, New Delhi, India
Further Information

Publication History

Publication Date:
27 March 2002 (online)

ABSTRACT

Extrahepatic portal vein obstruction (EHPVO) is an important cause of noncirrhotic portal hypertension, especially in Third World countries. The etiology and clinical presentation are different in children and adults. The portal vein is transformed into a cavernoma, resulting in portal hypertension and oesophagogastic varices. In addition, extensive collateral circulation develops, involving paracholecystic, paracholedochal and pancreaticoduodenal veins resulting in formation of ectopic varices, and portal biliopathy. Besides variceal bleeding, which is the commonest presentation, patients may have symptomatic portal biliopathy, hypersplenism, and growth retardation. Although the liver may appear normal, functional compromise develops in the long term. Variceal bleeding in EHPVO can be successfully managed by endoscopic obliteration of varices, which has low morbidity but requires repeated visits, or by portosystemic shunt surgery, which provides good control of bleeding, possibly helps growth retardation, hypersplenism, and protects against future development of portal biliopathy but is associated with surgical mortality and is sometimes not feasible due to nonavailability of a satisfactory vessel.

REFERENCES

  • 1 Anand C S, Tandon B N, Nundy S. The causes, management and outcome of upper gastrointestinal hemorrhage in an Indian hospital.  Br J Surg . 1983;  70 209-211
  • 2 Alvarez F, Bernard O, Brunelle F. Portal obstruction in children. I. Clinical investigations and hemorrhage risk.  J Pediatr . 1983;  103 696-702
  • 3 Mitra S K, Kumar V, Dutta D V. Extrahepatic portal hypertension: a review of 70 cases.  J Pediatr Surg . 1978;  13 51-54
  • 4 Webb L J, Sherlock S. The aetiology, presentation and natural history of extra-hepatic portal venous obstruction.  Q J Med . 1979;  192 627-639
  • 5 Rosch J, Dotter C T. Extrahepatic portal obstruction in childhood and its angiographic diagnosis.  Am J Radiol . 1981;  112 143-149
  • 6 Shaldon S, Sherlock S. Obstruction to the extrahepatic portal system in childhood.  Lancet . 1962;  1 63-67
  • 7 Gibson J B, Johnson G W, Rodgers H W. Extrahepatic portal venous obstruction.  Br J Surg . 1965;  152 129-39
  • 8 Maddrey W C, Basu Mallik C H, Iber F L. Extrahepatic portal obstruction of the portal venous system.  Surg Gynaecol Obstet . 1968;  127 989-998
  • 9 Myers N A, Robinson M J. Extrahepatic portal hypertension in children.  J Pediatr Surg . 1973;  8 467-473
  • 10 Odievre M, Pige G, Alagille D. Congenital abnormalities associated with extrahepatic portal hypertension.  Arch Dis Child . 1977;  52 383-385
  • 11 Thompson E N, Sherlock S. The aetiology of portal vein thrombosis with particular reference to the role of infection and exchange transfusion.  Q J Med . 1964;  33 465-480
  • 12 Househam K C, Bowie M D. Extrahepatic portal venous obstruction.  S Afr Med J . 1983;  64 234-236
  • 13 Boles E T, Wise W E, Birken G. Extrahepatic portal hypertension in children. Long-term evaluation.  Am J Surg . 1986;  151 734-739
  • 14 Cardin F, Graffeo M, McCormick P A. Adult ``idiopathic'' extrahepatic venous thrombosis: importance of putative ``latent'' myeloproliferative disorders and comparison with cases with known etiology.  Dig Dis Sci . 1992;  37 335-339
  • 15 Stringer M D, Heaton N D, Karani J. Patterns of portal vein occlusion and their aetiological significance.  Br J Surg . 1994;  81 1328-1331
  • 16 Orozco H, Takahashi T, Mercado M A. Surgical management of extrahepatic portal hypertension and variceal bleeding.  World J Surg . 1994;  18 246-250
  • 17 Orloff M J, Orloff M S, Rambotti M. Treatment of bleeding esophagogastric varices due to extrahepatic portal hypertension: results of portal systemic shunts during 35 years.  J Paediatr Surg . 1994;  29 142-154
  • 18 Vleggaar F P, van Buuren R H, Schalm S W. Endoscopic sclerotherapy for bleeding oesophagogastric varices secondary to extrahepatic portal vein obstruction in an adult caucasian population.  Eur J Gastroenterol . 1988;  10 81-85
  • 19 Tizard J PM. Portal hypertension following exchange transfusion through the umbilical vein.  Proc R Soc Med . 1962;  55 772-779
  • 20 Yadav S, Dutta A K, Sarin S K. Do umbilical vein catheterization and sepsis lead to portal vein thrombosis?.  <~>A prospective, clinical and sonographic evaluation. J Pediatr Gastroenterol Nutr . 1993;  17 392-396
  • 21 Guimaraes H, Castelo L, Guimaraes J. Does umbilical vein catheterization to exchange transfusion lead to portal vein thrombosis?.  Eur J Pediatr . 1998;  157 461-463
  • 22 Larroche J. Umbilical catheterization: its complications.  Biol Neonate . 1970;  16 101-103
  • 23 Valla D, Casadevall N, Huisse M G. Myeloproliferative disorders in portal vein thrombosis in adults.  Gastroenterology . 1988;  94 1063-1069
  • 24 Denninger M H, Helley D, Valla D. Prospective evaluation of the prevalence of factor V Leiden mutation in portal or hepatic vein thrombosis.  Thromb Haemost . 1997;  78 1297-1298
  • 25 Mahmoud A EA, Elias E, Beauchamp N. Prevalence of the factor V Leiden mutation in hepatic and portal vein thrombosis.  Gut . 1997;  40 798-800
  • 26 Seixas C A, Hessel G, Ribeiro C C. Factor V Leiden is not common in children with portal vein thrombosis.  Thromb Haemost . 1997;  77 258-261
  • 27 Chamouard P, Pencreach E, Maloisel F. Frequent factor II G20210A mutation in idiopathic portal vein thrombosis.  Gastroenterology . 1999;  116 144-148
  • 28 Jansen H LA, Meinardi J R, Vleggaar F P. Factor V Leiden mutation, prothrombin gene mutation, and deficiencies in coagulation inhibitors associated with Budd-Chiari syndrome and portal vein thrombosis: results of a case-control study.  Blood . 2000;  96 2364-2368
  • 29 Egesel T, Buyukasik Y, Dundar S V. The role of natural anticoagulant deficiencies and factor V Leiden in the development of idiopathic portal vein thrombosis.  J Clin Gastroenterol . 2000;  30 66-71
  • 30 Denninger M H, Chait Y, Casadevall N. Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors.  Hepatology . 2000;  31 587-591
  • 31 Okuda K, Ohnishi K, Kimura K. Incidence of portal vein thrombosis in liver cirrhosis. An angiographic study in 708 patients.  Gastroenterology . 1985;  89 279-286
  • 32 Groszman R J, Vorbioff J, Riley E. Splanchnic hemodynamics in portal hypertensive rats: measurement with gamma-labeled microspheres.  Am J Physiology . 1982;  242 G156-G160
  • 33 Arora R, Mohanty M, Nundy S. Phlebothrombosis as a common pathogenic denominator in noncirrhotic portal fibrosis and extrahepatic portal venous obstruction.  Ind J Med Res . 1984;  79 392-403
  • 34 Sengupta K P, Basu Mallik C K, Maddrey W C. Liver changes in extrahepatic portal venous obstruction.  Ind J Med Res . 1968;  56 1643-1650
  • 35 Mikkelsen W P, Edmondson H A, Peters R L. Extra and intrahepatic portal hypertension without cirrhosis (hepatopetal sclerosis).  Ann Surg . 1965;  162 602-620
  • 36 De Gaetano M A, Lafortune M, Patriquin H. Cavernous transformation of the portal vein: patterns of intrahepatic and splanchnic collateral circulation detected with Doppler sonography.  Am J Radiol . 1995;  165 1151-1155
  • 37 Warren W D, Millikan W J, Smith R B. Noncirrhotic portal vein thrombosis. Physiology before and after surgery.  Ann Surg . 1980;  192 341-349
  • 38 Dilawari J B, Chawla Y K. Spontaneous (natural) splenoadenorenal shunts in extrahepatic portal venous obstruction: a series of 20 cases.  Gut . 1987;  28 1198-1200
  • 39 Lebrec D, Bataille C, Bercoff E. Hemodynamic changes in patients with portal venous obstruction.  Hepatology . 1983;  3 550-553.
  • 40 Sarin S K. Noncirrhotic portal hypertension. In: Bosch J, Groszman R, eds. Portal Hypertension: Pathophysiology and Treatment Oxford, UK: Blackwell Science 1994: 27-53
  • 41 Braillon A, Moreau R, Hadengue A. Hyperkinetic circulatory syndrome in patients with presinusoidal portal hypertension: effect of propranolol.  J Hepatol . 1989;  9 312-318
  • 42 Voigt M D, Trey G, Levitt N S. Autonomic neuropathy in extra-hepatic portal vein thrombosis: evidence for impaired autonomic reflex arc.  J Hepatol . 1997;  26 634-641
  • 43 Manisha R, Sinha S, Kapoor D. Prevalence of autonomic dysfunction in cirrhotic and non-cirrhotic portal hypertension.  Am J Gastroenterol (in press) . 2002; 
  • 44 MacGilchrist A J, Reid J L. Impairment of autonomic reflex in cirrhosis.  Am J Gastroenterol . 1990;  85 288-292
  • 45 Gaudin C, Braillon A, Poo J L. Plasma catecholamines in patients with presinusoidal portal hypertension: comparison with cirrhotic patients and non portal hypertensive subjects.  Hepatology . 1991;  13 913-916
  • 46 Thompson E N, Williams R, Sherlock S. Liver function in extrahepatic portal hypertension.  Lancet . 1964;  2 1352-1356
  • 47 Misra A, Guptan R C, Sarin S K. Monoethylglycinexylidide (MEGX): a sensitive noninvasive method to differentiate NCPF and child's A chronic liver disease.  Ind J Gastroenterol . 1997;  16(Suppl 2) 101
  • 48 Sarin S K, Agarwal S R. Idiopathic portal hypertension.  Digestion . 1998;  59 420-423
  • 49 Baggenstoss A H, Wallaeger E E. Portal hypertension due to chronic occlusion of the extrahepatic portion of the portal vein: its relation to ascites.  Am J Med . 1956;  21 16-19
  • 50 Reichen J. Liver function and pharmacological considerations in pathogenesis and treatment of portal hypertension.  Hepatology . 1990;  11 1066-1078
  • 51 Rozga J, Jeppsson B, Bengmark S. Hepatotrophic factors in liver growth and atrophy.  Br J Exp Pathol . 1985;  66 669-678
  • 52 Farrel G C, Koltai A, Zaluzny L. Effects of portal vein ligation on sex hormone metabolism in male rats: relationship to lowered hepatic cytochrome P450 levels.  Gastroenterology . 1986;  90 299-305
  • 53 Sarin S K, Bansal A, Sasan S. Portal-vein obstruction in children leads to growth retardation.  Hepatology . 1992;  15 229-233
  • 54 Hahn M, Massen O, Nencki M. Die eckache fistel zwischen der unteren Hahevene und der pfortaden und folgen furden organismus.  Arch Exp Pathol Pharmakol . 1893;  32 162-210
  • 55 Mehrotra R N, Bhatia V, Dabadghao P. Extrahepatic portal vein obstruction in children: anthropometry, growth hormone, and insulin-like growth factor 1.  J Paediatr Gastroenterol Nutr . 1997;  25 520-523
  • 56 Fraser J, Brown A K. A clinical syndrome associated with a rare anomaly of vena portel system.  Surg Gynecol Obstet . 1944;  78 520-524
  • 57 Gibson J B, Johnston G W, Fulton T T. Extrahepatic portal venous obstruction.  Br J Surg . 1965;  52 129-139
  • 58 Nagral A S, Dalvi B V, Khanna M U. Intrapulmonary vascular dilatation with hypoxemia in extrahepatic portal vein obstruction.  Ind J Gastroenterol . 1993;  12 149-151
  • 59 Behera D, Singh M, Chawla Y. Pulmonary function abnormality in patients with portal hypertension with or without chronic liver disease.  Ind J Chest Dis Allied Sci . 1998;  40 33-39
  • 60 Sarin S K, Bhatia V, Makwane U. ``Portal biliopathy'' in extrahepatic portal vein obstruction.  Ind J Gastroenterol . 1992;  11 82
  • 61 Dilawari J B, Chawla Y K. Pseudosclerosing cholangitis in extrahepatic portal venous obstruction.  Gut . 1992;  33 272-276.
  • 62 Khuroo M S, Yattoo G N, Zargar S A. Biliary abnormalities associated with extrahepatic portal venous obstruction.  Hepatology . 1993;  17 807-813
  • 63 Malkan G H, Bhatia S J, Bashir K. Cholangiopathy associated with portal hypertension: diagnostic evaluation and clinical implications.  Gastrointest Endosc . 1999;  49 344-348
  • 64 Nagi B, Kochhar R, Bhasin D. Cholangiopathy in extrahepatic portal venous obstruction.  Acta Radiol . 2000;  41 612-615
  • 65 Chandra R, Kapoor D, Tharakan A. Portal biliopathy.  J Gastroenterol Hepatol . 2001;  16 1086-1092
  • 66 Dhiman R K, Puri P, Chawla Y. Biliary changes in extrahepatic portal venous obstruction: compression by collaterals or ischemic?.  Gastrointest Endosc . 1999;  50 646-652
  • 67 Bhatia V, Jain A K, Sarin S K. Choledocholithiasis associated with portal biliopathy in patients with extrahepatic portal vein obstruction: management with endoscopic sphincterotomy.  Gastrointest Endosc . 1995;  42 178-181
  • 68 Kochlar S, Kochhar R, Nagi B. Portal billopathy in extrahepatic and intrahepatic portal hypertension.  J Gastroenterol Hepatol . 2001;  16(Suppl) A78
  • 69 Chawla A, Dewan R, Sarin S K. The frequency and influence of gallbladder varices on gallbladder functions in patients with portal hypertension.  Am J Gastroenterol . 1995;  90 2010-2014
  • 70 Prasad C VR, Kaur U, Marwaha N. Hemostatic alterations in non-cirrhotic portal fibrosis, extrahepatic portal venous obstruction and Budd-Chiari syndrome.  Ind J Gastroenterol . 1990;  9 57-69
  • 71 Sheth S G, Deo A M, Bichile S K. Coagulation abnormalities in non-cirrhotic portal fibrosis and extra hepatic portal vein obstruction.  J Assoc Physicians India . 1996;  44 790-791
  • 72 Robson S C, Kahn D, Kruskal J. Disordered hemostasis in extrahepatic portal hypertension.  Hepatology . 1993;  18 853-857
  • 73 Bajaj J S, Bhattacharjee J, Sarin S K. Coagulation profile and platelet function in patients with extrahepatic portal vein obstruction and non-cirrhotic portal fibrosis.  J Gastroenterol Hepatol . 2001;  16 641-646
  • 74 Webb L J, Ross M, Markham R L. Immune function in patients with extrahepatic portal venous obstruction and the effect of splenectomy.  Gastroenterology . 1980;  79 99-103
  • 75 Robson S C, Saunders R H, Kruskal J B. Immune dysfunction in extrahepatic portal hypertension-identification of immunosuppressive factors.  Hepatology . 1988;  8 1428
  • 76 Seth A, Yadav S, Sasan S. Systemic and gut mucosal immunity in children with extrahepatic portal vein obstruction.  Ind J Gastroenterol . 1992;  11 81
  • 77 Sarin S K, Lahoti D, Saxena S P. Prevalence, classification and natural history of gastric varices: long-term follow-up study in 568 patients with portal hypertension.  Hepatology . 1992;  16 1343-1349
  • 78 Sarin S K, Dasika V, Sreenivas, et al. Factors influencing development of portal hypertensive gastropathy in patients with portal hypertension.  Gastroenterology . 1992;  102 994-999
  • 79 Sarin S K, Shahi H M, Jain M. The natural history of portal hypertensive gastropathy: influence of variceal eradication.  Am J Gastroenterol . 2000;  95 2888-2893
  • 80 Ganguly S, Sarin S K, Bhatia V. The prevalence and spectrum of colonic lesions in patients with cirrhotic and noncirrhotic portal hypertension.  Hepatology . 1995;  21 1226-1231
  • 81 Chawla Y, Dilawari J B. Anorectal varices: their frequency in cirrhotic and non-cirrhotic portal hypertension.  Gut . 1991;  32 309-311
  • 82 Voorhees A B, Price J B. Extrahepatic portal hypertension. A retrospective analysis of 127 cases and associated clinical implications.  Arch Surg . 1974;  108 338-341
  • 83 Zwiebel W J. Sonographic diagnosis of hepatic vascular disorders.  Semin Ultrasound CT MR . 1995;  16 34-48
  • 84 Fonkalsrud E W, Myers N A, Robinson M J. Management of extrahepatic portal hypertension in children.  Ann Surg . 1974;  180 487-493
  • 85 Clatworthy H W, Boles E T. Extrahepatic portal bed block in children: pathogenesis and treatment.  Ann Surg . 1959;  150 371-383
  • 86 Voorhees A B, Chaitman E, Schneider S. Portal-systemic encephalopathy in the noncirrhotic patient. Effect of portal systemic shunting.  Arch Surg . 1973;  107 659-663
  • 87 Kahn D, Terblanche J, Kitane S. Injection sclerotherapy in adults with extrahepatic portal venous obstruction.  Br J Surg . 1987;  74 600-602
  • 88 Howard E R, Stringer M D, Mowat A P. Assessment of injection sclerotherapy in management of 152 children with esophageal varices.  Br J Surg . 1988;  75 404-408
  • 89 Dilawari J B, Chawla Y K, Ramesh G N. Endoscopic sclerotherapy in children.  J Gastroenterol Hepatol . 1989;  4 155-160
  • 90 Chawla Y K, Dilawari J B, Ramesh G N. Sclerotherapy in extrahepatic portal venous obstruction.  Gut . 1990;  31 213-216
  • 91 Bhargava D K, Dasarathy S, Sundaram K R. Efficacy of endoscopic sclerotherapy on long-term management of oesophageal varices: a comparative study of results in patients with cirrhosis of the liver, non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal venous obstruction (EHO).  J Gastroenterol Hepatol . 1991;  6 471-475
  • 92 Yachha S K, Sharma B C, Kumar M. Endoscopic sclerotherapy for esophageal varices in children with extrahepatic portal venous obstruction: a follow-up study.  J Pediatr Gastroenterol Nutr . 1997;  24 49-52
  • 93 Sarin S K, Agarwal S R. Gastric varices and portal hypertensive gastropathy.  Clin Liver Dis . 2001;  5 727-767
  • 94 Fonkalsrud E W. Surgical management of portal hypertension in childhood: long term results.  Arch Surg . 1980;  115 1042-1045
  • 95 Bismuth H, Franco D. Portal diversion for portal hypertension in early childhood.  Ann Surg . 1976;  183 439-446
  • 96 Bismuth H, Franco D, Alagille D. Portal diversion for portal hypertension in children. The first ninety patients.  Ann Surg . 1980;  192 18-24
  • 97 Alvarez F, Bernard O, Brunelle F. Portal obstruction in children. II. Results of surgical portasystemic shunts.  J Pediatr . 1983;  103 703-707
  • 98 Pande G K, Sahni P, Nundy S. Extrahepatic obstruction causing portal hypertension.  J Gastroenterol Hepatol . 1988;  3 99-107
  • 99 Mitra S K, Rao K LN, Narasimhan K L. Side-to-side lienorenal shunt without splenectomy in non-cirrhotic portal hypertension in children.  J Pediatr Surg . 1993;  28 398-402
  • 100 Prasad S A, Gupta S, Kohli V. Proximal splenorenal shunts for extrahepatic portal venous obstruction in children.  Ann Surg . 1994;  219 193-196
  • 101 Warren W D, Henderson J M, Millikan W J. Management of variceal bleeding in patients with noncirrhotic portal vein thrombosis.  Ann Surg . 1988;  207 623-634
  • 102 Henderson J M, Millikan W J, Galambos J T. Selective variceal decompression in portal vein thrombosis.  Br J Surg . 1984;  71 745-749
  • 103 Alagille D, Carlier J C, Chiva M. Long-term neuropsychological outcome in children undergoing portal systemic shunts for portal vein obstruction without liver disease.  J Pediatr Gastroenterol Nutr . 1986;  5 861-886
  • 104 Guharay B N, Sain P, Ramerjee D. Direct splenocaval shunt for selective decompression of portal hypertension in children.  Surgery . 1980;  87 271-279
  • 105 Maksoud J G, Miles S. Distal splenorenal shunt in children.  Ann Surg . 1982;  195 401-405
  • 106 Cohen J, Edelman R R, Chopra S. Portal vein thrombosis: a review.  Am J Med . 1992;  92 173-181
  • 107 Yoshioka H, Kuroda C, Hori S. Splenic embolization for hypersplenism using steel wires.  Am J Radiol . 1985;  144 1269-1274
  • 108 Sangro B, Billao I, Herraro J. Partial splenic embolization for the treatment of hypersplenism in cirrhosis.  Hepatology . 1993;  18 309-314
  • 109 Kenawi M M, El Ghamrawi K A, Mohammad A A. Splenic irradiation for the treatment of hypersplenism.  Br J Surg . 1997;  84 860-861
  • 110 Chaudhary A, Dhar P, Sarin S K. Bile duct obstruction due to portal biliopathy in extrahepatic portal hypertension: surgical management.  Br J Surg . 1988;  85 326-329
  • 111 Kato T, Romero R, Koutouby R. Portosystemic shunting in children during the era of endoscopic therapy: improved postoperative growth parameters.  J Pediatr Gastroenterol Nutr . 2000;  30 419-425
    >