Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Portal Vein Obstruction Clinical Presentation

  • Author: Adnan Said, MD, MSPH; Chief Editor: BS Anand, MD  more...
 
Updated: Dec 28, 2015
 

History

In the acute phase, the presentation of portal vein obstruction is relatively uncommon and easily missed because the patient may be asymptomatic. Symptoms most often begin in the chronic or subacute stage. Schistosomiasis can cause presinusoidal portal obstruction by blocking the intrahepatic portal venules with parasite eggs. It does not cause extrahepatic portal vein obstruction, though the clinical manifestations are often similar.

Acute

Patients can present emergently with sudden onset of right upper quadrant pain, nausea, and/or fever. Alternatively, the symptoms of the primary infectious and inflammatory condition that led to portal vein obstruction predominate (eg, right lower quadrant pain in appendicitis).

Progressive ascites, intestinal ischemia resulting from the propagation of the thrombus, or intestinal suffusion secondary to acute portal hypertension can also be the presenting manifestations. Occasionally, variceal bleeding can occur acutely with the development of portal vein thrombosis, particularly in the setting of preexisting varices with cirrhosis.

Spontaneous resolution of acute/recent thrombosis undoubtedly occurs and symptoms abate. In other patients, the acute symptoms often subside as collaterals develop, and the diagnosis may be missed. These patients then present at a later stage with manifestations of portal hypertension.

Chronic

These groups of patients most often present with complications related to portal hypertension. In 90% of cases, variceal bleeding is the presenting complaint. On average, this occurs 4 years after the thrombotic event and has been described as long as 12 years later. Ascites is less frequent, and hepatic encephalopathy is rare in the absence of preexisting cirrhosis.[3]

The specific etiology of the portal vein obstruction not only influences the initial clinical presentation but also the time course and prognosis.

In the presence of cirrhosis with underlying hepatic insufficiency, sudden worsening of hepatic function, development of hepatic encephalopathy, and the development of ascites are all more frequent, leading to worse outcomes.

With intra-abdominal malignancies, bleeding is less commonly the first manifestation because many of these patients do not survive long enough to develop the sequelae of portal hypertension. These patients most often present with sudden ascites, anorexia, right upper quadrant or epigastric pain, and weight loss. Portal vein obstruction may also be discovered incidentally on imaging studies obtained for pain or ascites.

Rarely, patients with portal vein obstruction present with a fever of unknown origin.

Next

Physical Examination

Splenomegaly is found in 75-100% of patients, most presenting in the chronic stage. Mild hepatomegaly is often present, as is right upper quadrant epigastric tenderness, especially in the acute setting.

Ascites is found infrequently. Stigmata of chronic liver disease, such as spider angiomata or palmar erythema, are usually found in the presence of an underlying liver disease.

The presence of caput medusae indicates posthepatic or intrahepatic portal hypertension because it forms by recanalization of the umbilical vein, which connects with the left hepatic branch of the portal vein.[4]  It should not be observed in isolated extrahepatic portal vein obstruction because the obstruction is below the origin of the umbilical vein.

In children, growth retardation may be present.[5]

Abnormalities of the extrahepatic biliary tree may occur in 80% of cases due to compression by choledochal or periportal varices or from ischemic stricturing. These findings manifest by jaundice, cholangitis, hemobilia, cholecystitis, or a hilar mass that can be mistaken for a cholangiocarcinoma.

Previous
 
 
Contributor Information and Disclosures
Author

Adnan Said, MD, MSPH Assistant Professor, Department of Medicine, Section of Gastroenterology and Hepatology, University of Wisconsin School of Medicine and Public Health at Madison; Consulting Staff, Department of Medicine, William S Middleton Memorial Veterans Hospital

Adnan Said, MD, MSPH is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, American Society of Transplantation

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Noel Williams, MD, FRCPC FACP, MACG, Professor Emeritus, Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada; Professor, Department of Internal Medicine, Division of Gastroenterology, University of Alberta, Edmonton, Alberta, Canada

Noel Williams, MD, FRCPC is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

BS Anand, MD Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine

BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Additional Contributors

Ann Ouyang, MBBS Professor, Department of Internal Medicine, Pennsylvania State University College of Medicine; Attending Physician, Division of Gastroenterology and Hepatology, Milton S Hershey Medical Center

Disclosure: Nothing to disclose.

Acknowledgements

Mark Reichelderfer, MD

Disclosure: Nothing to disclose.

Andrew Taylor, MD Professor, Department of Radiology, University of Wisconsin Hospitals and Clinics

Disclosure: Nothing to disclose.

Jennifer T Wells, MD Fellow, Department of Gastroenterology and Hepatology, University of Wisconsin Hospitals and Clinics, Madison

Jennifer T Wells, MD is a member of the following medical societies: American Medical Association and Phi Beta Kappa

Disclosure: Nothing to disclose.

References
  1. Chawla Y, Dhiman RK. Intrahepatic portal venopathy and related disorders of the liver. Semin Liver Dis. 2008 Aug. 28(3):270-81. [Medline].

  2. Abd El-Hamid N, Taylor RM, Marinello D, Mufti GJ, Patel R, Mieli-Vergani G, et al. Aetiology and management of extrahepatic portal vein obstruction in children: King's College Hospital experience. J Pediatr Gastroenterol Nutr. 2008 Nov. 47(5):630-4. [Medline].

  3. Sharma P, Sharma BC, Puri V, Sarin SK. Natural history of minimal hepatic encephalopathy in patients with extrahepatic portal vein obstruction. Am J Gastroenterol. 2009 Apr. 104(4):885-90. [Medline].

  4. Facciuto ME, Rodriguez-Davalos MI, Singh MK, Rocca JP, Rochon C, Chen W, et al. Recanalized umbilical vein conduit for meso-Rex bypass in extrahepatic portal vein obstruction. Surgery. 2009 Apr. 145(4):406-10. [Medline].

  5. Nihal L, Bapat MR, Rathi P, Shah NS, Karvat A, Abraham P, et al. Relation of insulin-like growth factor-1 and insulin-like growth factor binding protein-3 levels to growth retardation in extrahepatic portal vein obstruction. Hepatol Int. 2009 Mar. 3(1):305-9. [Medline]. [Full Text].

  6. Nakao A, Kanzaki A, Fujii T, Kodera Y, Yamada S, Sugimoto H, et al. Correlation Between Radiographic Classification and Pathological Grade of Portal Vein Wall Invasion in Pancreatic Head Cancer. Ann Surg. 2011 Dec 8. [Medline].

  7. Liu Q, Chen J, Li H, Liang B, Zhang L, Hu T. Hepatocellular carcinoma with bile duct tumor thrombi: Correlation of magnetic resonance imaging features to histopathologic manifestations. Eur J Radiol. 2009 Jun 5. [Medline].

  8. Tritou I, Megremis S, Stefanaki E, Goumenakis M, Sfakianaki E. Sonographic detection of transient gas in the portal vein in an infant following abdominal surgery: A possible sign of adhesive small bowel obstruction. J Clin Ultrasound. 2011 Sep 26. [Medline].

  9. Alberti D, Colusso M, Cheli M, Ravelli P, Indriolo A, Signorelli S, et al. Results of a stepwise approach to extrahepatic portal vein obstruction in children. J Pediatr Gastroenterol Nutr. 2013 Nov. 57(5):619-26. [Medline].

  10. Guérin F, Porras J, Fabre M, Guettier C, Pariente D, Bernard O, et al. Liver nodules after portal systemic shunt surgery for extrahepatic portal vein obstruction in children. J Pediatr Surg. 2009 Jul. 44(7):1337-43. [Medline].

  11. Arora A, Sarin SK. Multimodality imaging of primary extrahepatic portal vein obstruction (EHPVO): what every radiologist should know. Br J Radiol. 2015 Aug. 88 (1052):20150008. [Medline].

  12. Kumar A, Sharma P, Arora A. Review article: portal vein obstruction--epidemiology, pathogenesis, natural history, prognosis and treatment. Aliment Pharmacol Ther. 2015 Feb. 41 (3):276-92. [Medline].

  13. Wu XP, Ni JM, Zhang ZY, et al. Preoperative evaluation of malignant perihilar biliary obstruction: negative-contrast CT cholangiopancreatography and CT angiography versus MRCP and MR angiography. AJR Am J Roentgenol. 2015 Oct. 205 (4):780-8. [Medline].

  14. Ha TY, Kim KM, Ko GY, et al. Variant meso-Rex bypass with transposition of abdominal autogenous vein for the management of idiopathic extrahepatic portal vein obstruction: a retrospective observational study. BMC Surg. 2015 Oct 17. 15:116. [Medline].

  15. Arrive L, Hodoul M, Arbache A, Slavikova-Boucher L, Menu Y, El Mouhadi S. Magnetic resonance cholangiography: Current and future perspectives. Clin Res Hepatol Gastroenterol. 2015 Dec. 39 (6):659-64. [Medline].

  16. Yoshimatsu R, Yamagami T, Ishikawa M, et al. Embolization therapy for bleeding from jejunal loop varices due to extrahepatic portal vein obstruction. Minim Invasive Ther Allied Technol. 2016 Feb. 25 (1):57-61. [Medline].

  17. Sekimoto T, Maruyama H, Kobayashi K, et al. Well-tolerated portal hypertension and favorable prognosis in adult patients with extrahepatic portal vein obstruction in Japan. Hepatol Res. 2015 Aug 29. [Medline].

 
Previous
Next
 
The etiology of portal vein obstruction.
Coagulation disorders in portal vein thrombosis.
Portal vein thrombosis with cavernous transformation. The long arrow indicates the splenic vein at the junction with the superior mesenteric vein just below the site of thrombosis. The short arrow points to a serpiginous mass consistent with periportal collaterals, the so-called cavernous transformation of the portal vein.
Hepatocellular carcinoma with portal vein thrombosis. The short arrow indicates the tumor thrombus with an abrupt cut off of the portal vein. The long arrow points to a compensatory, prominent left hepatic arterial branch.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.