Portal vein thrombosis - Clinicals, Diagnosis, and Management

Gastroenterology

Clinicals - 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. 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.
Present with acute onset right upper quadrant abdominal pain, fever and nausea/ vomiting Due to acute portal vein obstruction. 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. 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. 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.
Severe abdominal pain, fever, diarrhea, vomiting Bowel ischemia and infarction may result from propagation of the portal vein thrombus into the mesenteric blood vessels. 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. 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. 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. 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. 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.

Clinicals - 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. 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. 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. 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.
Right hypochondrial tenderness In the acute phase of portal vein thrombosis Right hypochondrial tenderness
In the acute phase of portal vein thrombosis
Hepatomegaly Is an uncommon feature. 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. 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. 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. 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.

Investigations - 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. 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.
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. 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. 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. 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.
Angiography Angiography is rarely required for diagnostic purposes as USS and MRI provide adequate information. 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. Endoscopic ultrasound
Endoscopic ultrasound is considered a newer diagnostic test which has been found to be sensitive and specific for portal vein thrombosis.
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. 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.

Investigations - 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. 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. 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. Blood grouping and save
Blood transfusion may be required in severe variceal bleeding.

Management - Supportive

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. 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. 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.

Management - Specific

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. 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.
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. This is followed by anticoagulation therapy with warfarin for a further 3 months. 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. 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. 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. At the time of shunt creation the thrombus may be aspirated and removed. 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. 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. 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. Liver transplantation
In patients who have a cirrhotic liver, orthotopic liver transplantation can be attempted. 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.

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