History

Fact Explanation
The patient may be asymptomatic Acute portal vein thrombosis is most often asymptomatic and may go unnoticed. In a majority of these patients the thrombus resolves spontaneously and no further progression occurs. In some the portal vein obstruction persists for a longer duration and enters into a chronic state.[1] [2]
Present with acute onset right upper quadrant abdominal pain, fever and nausea/ vomiting Due to acute portal vein obstruction.
Initial presentation maybe with features of cirrhosis Cirrhosis is often asymptomatic or present with nonspecific symptoms. Most patients are diagnosed in advance disease. The patient may develop right hypochondrial pain, jaundice, bleeding manifestations and abdominal swelling due to ascites.
Present with complications of cirrhosis and portal hypertension Variceal bleeding may be the first presentation of the patient. It may be torrential and carries a high mortality rate. Ascites and hepatic encephalopathy are relatively rare complications of chronic portal vein thrombosis. In the setting of pre-existing chronic liver disease, acute portal vein thrombosis may precipitate the above mentioned complications.[2]
Severe abdominal pain, fever, diarrhea, vomiting Bowel ischemia and infarction may result from propagation of the portal vein thrombus into the mesenteric blood vessels.
Symptoms of the primary aetiology Malignancy of the liver may predispose a thrombotic state. Right hypochondrial pain, weight loss and anorexia may be the presenting symptoms of hepatocellular carcinoma. Chronic pancreatitis presents with chronic epigastric pain which radiates to the back, nausea & vomiting and chronic diarrhea.
Risk factors/ Associations Both inherited and acquired coagulation disorders may predispose to thrombosis of the portal vein. Inherited causes : Factor 5 Leiden disease, antithrombin 3 deficiency and Protein C & S deficiency.[3] Ask for a family history of thrombotic disease. Acquired causes : Liver disease, disseminated intravascular coagulation, pregnancy, oral contraceptive pill, malignancy, myeloproliferative disease, lupus anticoagulant syndrome etc.
References
  1. SOBHONSLIDSUK A, REDDY KR. Portal vein thrombosis: a concise review. Am J Gastroenterol [online] 2002 Mar, 97(3):535-41 [viewed 08 August 2014] Available from: doi:10.1111/j.1572-0241.2002.05527.x
  2. PARIKH S, SHAH R, KAPOOR P. Portal vein thrombosis. Am J Med [online] 2010 Feb, 123(2):111-9 [viewed 08 August 2014] Available from: doi:10.1016/j.amjmed.2009.05.023
  3. AMITRANO L, GUARDASCIONE MA, BRANCACCIO V, MARGAGLIONE M, MANGUSO F, IANNACCONE L, GRANDONE E, BALZANO A. Risk factors and clinical presentation of portal vein thrombosis in patients with liver cirrhosis. J Hepatol [online] 2004 May, 40(5):736-41 [viewed 08 August 2014] Available from: doi:10.1016/j.jhep.2004.01.001
  4. FRANCOZ CLAIRE, VALLA DOMINIQUE, DURAND FRANçOIS. Portal vein thrombosis, cirrhosis, and liver transplantation. Journal of Hepatology [online] 2012 July, 57(1):203-212 [viewed 08 August 2014] Available from: doi:10.1016/j.jhep.2011.12.034

Examination

Fact Explanation
Peripheral stigmata of chronic liver disease Circulatory changes may lead to development palmar erythema and spider telangiectasia. Endocrine changes may cause testicular atrophy, gynaecomastia in men and breast atrophy in females.
Jaundice In chronic liver disease impaired bilirubin conjugation and cholestasis may lead to yellowish discoloration of skin and mucous membranes.
Caput medusae Caput medusae is a portosystemic shunt across the superficial abdominal wall blood vessels. Portal vein thrombosis may result in portal hypertension which may lead to shunting of blood through the regressed umbilical vein.[1]
Right hypochondrial tenderness In the acute phase of portal vein thrombosis
Hepatomegaly Is an uncommon feature.
Splenomegaly Splenomegaly is a consistent feature in chronic disease. It may also be rarely seen in acute portal vein thrombosis. Portal hypertension leads to congestion of blood within the spleen.
Ascites Due to portal hypertension.
Assess airway, breathing and circulation in patients who present in a collapsed state Prompt resuscitation is required in patients who present with torrential upper gastrointestinal hemorrhage due to variceal bleeding. Inspect the airway, clear any secretions, blood and verify its patency. Inspect for chest movements, listen and feel for breathing. Measurement of the pulse rate, blood pressure, capillary refill time and urine output can be used to determine the status of the circulation.
References
  1. DE GAETANO AM, LAFORTUNE M, PATRIQUIN H, DE FRANCO A, AUBIN B, PARADIS K. Cavernous transformation of the portal vein: patterns of intrahepatic and splanchnic collateral circulation detected with Doppler sonography. AJR Am J Roentgenol [online] 1995 Nov, 165(5):1151-5 [viewed 08 August 2014] Available from: doi:10.2214/ajr.165.5.7572494

Differential Diagnoses

Fact Explanation
Budd-Chiari Syndrome Budd-Chiari Syndrome occurs when there is thrombosis of the main hepatic veins. In a majority of patients a specific cause is not found. Inherited thrombophilic disorders, pregnancy, oral contraceptives and malignancies of the kidney,liver may precipitate the disease. Acute thrombus formation may present with acute abdominal pain, marked ascites and acute liver failure. The liver is enlarged due to congestion and is tender on palpation. Some patients may present with gradually progressive ascites. Chronic venous congestion within the liver may lead to hepatocyte injury and fibrosis, eventually progressing to cirrhosis. Diagnosed is based on visualization of the occlusion by USS, CT and MRI, doppler studies demonstrates sluggish or reversed blood flow.[1]
Alcoholic liver disease Heavy alcohol consumption and chronic viral hepatitis are the leading causes of cirrhosis. Alcoholic liver disease is a spectrum of disease ranging from - fatty liver, alcoholic hepatitis and cirrhosis. Fatty liver disease is usually asymptomatic. In alcoholic hepatitis the patient may develop jaundice, malnutrition and features of portal hypertension. The presentation may be acute with fever, abdominal pain and jaundice following a bout of heavy alcohol consumption.[2] Most patients are diagnosed late with cirrhosis and its complications. On physical examination peripheral stigmata of chronic liver disease may be seen. Palmar erythema, spider naevi and parotid enlargement are characteristic features of alcoholic cirrhosis. Diagnosis is by clinical information.
Chronic viral hepatitis Chronic infection with Hepatitis B & C viruses may progress to cirrhosis. The viruses are usually acquired through contact of body fluids - during sexual contact, needlestick injuries and vertical transmission. The patient initially develops a prodromal infection with fever, malaise, anorexia which is followed by abdominal pain, nausea, vomiting and diarrhea. Jaundice, pale stools and dark urine develop gradually. Physical signs are minimal. The liver may be slightly enlarged and is tender. Failure to eradicate the virus completely leads to chronic hepatocellular injury. This may progress to cirrhosis. Diagnosis is usually based on clinical information and serological studies.[3]
References
  1. MENON K.V. NARAYANAN, SHAH VIJAY, KAMATH PATRICK S.. The Budd–Chiari Syndrome. N Engl J Med [online] 2004 February, 350(6):578-585 [viewed 09 August 2014] Available from: doi:10.1056/NEJMra020282
  2. LUCEY MICHAEL R., MATHURIN PHILIPPE, MORGAN TIMOTHY R.. Alcoholic Hepatitis. N Engl J Med [online] 2009 June, 360(26):2758-2769 [viewed 09 August 2014] Available from: doi:10.1056/NEJMra0805786
  3. RYDER S D. ABC of diseases of liver, pancreas, and biliary system: Chronic viral hepatitis. [online] 2001 January, 322(7280):219-221 [viewed 09 August 2014] Available from: doi:10.1136/bmj.322.7280.219

Investigations - for Diagnosis

Fact Explanation
Ultrasound scan Ultrasound scan is the first line diagnostic investigation which has a high sensitivity and specificity. The thrombus can be visualized as an echogenic mass within the portal vein. Ultrasound is easy to perform, non-invasive and easily available. USS may aid in detection of the primary aetiology – hepatocellular carcinoma, chronic pancreatitis.[1]
Doppler study The blood flow within the portal vein can be assesses with Doppler studies. The blood flow may be sluggish or be reversed in severe portal hypertension.
CT scan Contrast enhanced CT can be used to visualize the intra-luminal thrombus.
MRI/ MRA Magnetic resonance imaging is an accurate diagnostic investigation. Acute thrombosis appears as hyperdense lesions on both T1- and T2-weighted images while older clots appear hyperdense only on T2-weighted images. Additional advantages of MRI are visualization of the liver parenchyma, exclusion of esophageal collaterals and determination of portal and hepatic vessel flow.[1]
Angiography Angiography is rarely required for diagnostic purposes as USS and MRI provide adequate information.
Endoscopic ultrasound Endoscopic ultrasound is considered a newer diagnostic test which has been found to be sensitive and specific for portal vein thrombosis.[2]
Coagulation studies Inherited thrombophilic disorders need to be considered when other aetiological agents are excluded. Measurement of protein C & S levels is required to exclude their deficiency. Similarly assessment of anti-thrombin 3 level and factor 5 level is required to exclude antithrombin 3 deficiency and Leiden disease respectively. The fact that the levels of these factors may be low due to hepatic dysfunction should be kept in mind when interpreting test results.
References
  1. SOBHONSLIDSUK A, REDDY KR. Portal vein thrombosis: a concise review. Am J Gastroenterol [online] 2002 Mar, 97(3):535-41 [viewed 08 August 2014] Available from: doi:10.1111/j.1572-0241.2002.05527.x
  2. LAI L, BRUGGE WR. Endoscopic ultrasound is a sensitive and specific test to diagnose portal venous system thrombosis (PVST). Am J Gastroenterol [online] 2004 Jan, 99(1):40-4 [viewed 08 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/14687139

Investigations - Fitness for Management

Fact Explanation
Liver function tests Liver function tests are usually normal in isolated portal vein thrombosis with no pre-existing liver disease. In acute portal vein thrombosis LFT are usually normal or may be mildly elevated. In the presence of cirrhosis the transaminase levels are elevated with a AST : ALT ratio of > 1. The serum bilirubin level is elevated. The serum albumin level is lowered due to impaired production.
Coagulation studies Prothrombin time test and APTT are carried out as baseline investigations prior to treatment. Elevated PT and APTT values indicate reduced clotting factor production due to hepatic dysfunction. Cautions should be taken when initiating long term anti-coagulants.
Blood grouping and save Blood transfusion may be required in severe variceal bleeding.
References

Management - General Measures

Fact Explanation
Patient education The patient should be provided adequate information regarding the course, aetiology, complications and treatment options available for the disease. The patient should be counseled regarding the complications of cirrhosis and portal hypertension.
Resuscitation of patients who present with severe hemorrhage Patients who present with severe hemorrhage require prompt resuscitation. Clear blood and other secretions from the mouth and maintain airway patency by head tilt and jaw lift. An oropharyngeal airway may be required if the above maneuvers fail. Supply oxygen via a face mask. Establish intravenous access by two large-bore cannula. Initiate fluid resuscitation, blood may be required in severe hemorrhage.[1]
References
  1. JALAN R. UK guidelines on the management of variceal haemorrhage in cirrhotic patients. [online] 2000 June, 46(90003):1iii-15 [viewed 08 August 2014] Available from: doi:10.1136/gut.46.suppl_3.iii1

Management - Specific Treatments

Fact Explanation
Management of acute variceal bleeding Both medical and surgical measures can be used. Endoscopic banding or sclerotherapy are the preferred treatment modalities. The risk of rebleeding is minimum with these methods. In severe hemorrhage visualization of the sites of bleeding can be difficult. Vasoconstrictors somatostatin and octreotide and vasodilators such as isosorbide mononitrate can also be used.[1]
Thrombolysis Acute portal vein thrombosis requires thrombolysis. Patients are usually treated with heparin. Systemic thrombolysis can be avoided by administering tissue-type plasminogen activator via the transhepatic route. This method is particularly useful if the thrombus has extended into the mesenteric vessels. Transcatheter thrombolysis requires further evaluation due to a high complication rate.[2] This is followed by anticoagulation therapy with warfarin for a further 3 months.
Management of inherited thrombophilic disorders These patients require life-long anticoagulation. Use of anticoagulative therapy in patients with chronic liver disease requires caution and careful drug monitoring. Patients with chronic liver disease have an increased tendency of bleeding due to reduced clotting factor production.
Surgical therapy The portal vein obstruction can be bypassed by surgical creation of shunts. Distal splenorenal shunt and Transjugular intrahepatic portosystemic shunt (TIPS) can be attempted in patients with portal vein thrombosis. A correctly placed shunt during TIPS is associated with adequate symptom relief and less chance of rebleeding.[3] At the time of shunt creation the thrombus may be aspirated and removed.
Liver transplantation In patients who have a cirrhotic liver, orthotopic liver transplantation can be attempted.[4] The prognosis or these patients can assessed using scoring systems : Child-Pugh classification and Model for end-stage liver disease (MELD) score. The parameters used to calculate the MELD score are serum bilirubin, INR and serum creatinine. This score is also used for prioritization of liver transplant patients.
References
  1. JALAN R. UK guidelines on the management of variceal haemorrhage in cirrhotic patients. [online] 2000 June, 46(90003):1iii-15 [viewed 08 August 2014] Available from: doi:10.1136/gut.46.suppl_3.iii1
  2. HOLLINGSHEAD M, BURKE CT, MAURO MA, WEEKS SM, DIXON RG, JAQUES PF. Transcatheter thrombolytic therapy for acute mesenteric and portal vein thrombosis. J Vasc Interv Radiol [online] 2005 May, 16(5):651-61 [viewed 08 August 2014] Available from: doi:10.1097/01.RVI.0000156265.79960.86
  3. SENZOLO M, TIBBALS J, CHOLONGITAS E, TRIANTOS CK, BURROUGHS AK, PATCH D. Transjugular intrahepatic portosystemic shunt for portal vein thrombosis with and without cavernous transformation. Aliment Pharmacol Ther [online] 2006 Mar 15, 23(6):767-75 [viewed 08 August 2014] Available from: doi:10.1111/j.1365-2036.2006.02820.x
  4. LENDOIRE J, RAFFIN G, CEJAS N, DUEK F, BARROS SCHELOTTO P, TRIGO P, QUARIN C, GARAY V, IMVENTARZA O. Liver transplantation in adult patients with portal vein thrombosis: risk factors, management and outcome HPB (Oxford) [online] 2007, 9(5):352-356 [viewed 08 August 2014] Available from: doi:10.1080/13651820701599033