A 60-year-old man presents to the emergency department with a 2-day history of abdominal pain, nausea, and vomiting. There is no family history of hypercoagulable states. The patient has no significant past medical history and takes no medication. He does not drink alcohol. Temperature is 37℃ (98.6℉), pulse is 102/min, respirations are 18/min, blood pressure is 142/94 mmHg, and oxygen saturation is 99% on room air. On physical examination, there is mild diffuse abdominal tenderness without hepatosplenomegaly or evidence of an abdominal mass. Laboratory studies are shown below. Bedside ultrasound reveals hyperechoic material in the portal vein with impaired blood flow. No hepatic masses are observed. Which of the following tests should be performed to identify the likely underlying condition? Laboratory Test Result Reference Range Hemoglobin 18.1 g/dL 13.5-17.5 g/dL Hematocrit 54% 41-53% AST 19 U/L 8-40 U/L ALT 30 U/L 8-40 U/L

Portal vein thrombosis: Clinical sciences

Chapters:

Introduction0:00–0:35

Portal vein thrombosis, or PVT for short, refers to the formation of blood clots within the portal vein that can result in partial or complete obstruction of the blood vessel.

This condition is commonly associated with cirrhosis and hepatocellular carcinoma, and can result in various complications, such as esophageal and gastric varices, as well as cavernous transformation, pylephlebitis, and mesenteric ischemia.

Now, if your patient presents with a chief concern suggesting portal vein thrombosis, your first step is to perform an ABCDE assessment to determine if the patient is unstable or stable.

ABCDE Assessment 0:35–1:36

If the patient is unstable, first stabilize their airway, breathing, and circulation. Next, obtain IV access and put your patient on continuous vital sign monitoring, including blood pressure, heart rate, and pulse oximetry.

Finally, if needed, provide supplemental oxygen and ensure the patient is nil per os or NPO, meaning nothing by mouth. Now, here’s a clinical pearl!

If your patient is unstable, be sure to assess for severe complications of portal vein thrombosis, like variceal bleeding or mesenteric infarction.

For variceal bleeding, consider variceal banding or sclerotherapy. In case of mesenteric infarction, immediately obtain a surgical consultation!Now, let’s go back to the ABCDE assessment and look at stable patients.

Stable Patient 1:36–2:15

In this case, obtain a focused history and physical examination. Your patient may complain of abdominal pain, which comes and goes abruptly, and could be generalized or localized in the right upper quadrant.

Other symptoms may include bloody vomit or dark, tarry stools. The physical exam may reveal signs of portal hypertension, such as splenomegaly; or signs of cirrhosis, such as jaundice or ascites.

Now, with these findings, you should suspect portal vein thrombosis.Your next step is to order a Doppler ultrasound to assess the blood flow in the portal vein.

Suspect PVT2:15–3:24

If the Doppler ultrasound reveals normal portal vein blood flow, you should consider an alternative diagnosis. Here’s a clinical pearl to keep in mind!

A portal vein thrombus can sometimes be difficult to visualize on ultrasound. If you have a high clinical suspicion for PVT but the ultrasound does not reveal a thrombus, consider following up with a more advanced imaging modality, such as a CT, MRI, magnetic resonance portography, or contrast-enhanced ultrasound.

On the other hand, if the Doppler ultrasound shows hyperechoic material in the portal vein with impaired blood flow, you can diagnose PVT.

Once you diagnose PVT, your next step is to obtain advanced imaging like an abdominal CT or MRI. This will help you to assess the size of the thrombus and liver architecture.

Additionally, be sure to assess for any associated pathologies, such as cirrhosis. First, let’s discuss what to look for when assessing our patient for cirrhosis.

Cirrhosis3:24–5:14

Individuals with cirrhosis might have a history of progressive abdominal fullness, poor appetite, weight loss, weakness, or fatigue.

The physical exam may reveal features of cirrhosis, such as jaundice, palmar erythema, spider angiomata, or a distended abdomen with a fluid wave.

Finally, imaging might reveal changes in the hepatic architecture or cirrhotic nodules. If this is the case, you can diagnose your patient with PVT with cirrhosis.

Here’s a clinical pearl! Some labs that may point to a patient having cirrhosis are low albumin, thrombocytopenia, and elevated coagulation studies.

Next, assess the imaging to check if there’s an associated hepatocellular carcinoma, since it’s common in patients with cirrhosis.

If imaging reveals a hepatic nodule larger than or equal to two centimeters, diagnose PVT with HCC. Management will include chemotherapy medications, like sorafenib.Here’s a clinical pearl!

To confirm HCC, you can also get tumor markers like serum alfa-fetoprotein or AFP, as well as liver biopsy. On the flip side, if the imaging reveals cirrhosis without any evidence of a hepatic nodule, diagnose PVT without HCC.

In this case, treatment may include anticoagulation or a transjugular intrahepatic portosystemic shunt, or TIPS for short.

Okay, now let’s go back and take a look at individuals without cirrhosis. These patients will not have cirrhosis-related features, such as progressive abdominal distension, and there would be no signs of jaundice or a distended abdomen.

PVT without cirrhosis 5:14–6:11

Additionally, CT or MRI scans will reveal normal hepatic architecture without any cirrhotic nodules. In this case, diagnose PVT without cirrhosis.

Now, here’s a high-yield fact! Remember to always assess your patients for thrombophilia in case of PVT without cirrhosis.

This includes assessing for inherited causes of thrombophilia, such as factor V Leiden mutation; JAK2 mutation testing, to evaluate for a myeloproliferative neoplasm; and external causes of a hypercoagulable state, like the use of combined oral contraceptives.Alright, now once you diagnose PVT without cirrhosis, your next step is to order upper GI endoscopy to assess for esophageal or gastric varices.

Varices6:11–6:55

If endoscopy reveals esophageal or gastric varices, diagnose PVT with varices. For management, initiate a nonselective beta blocker, such as carvedilol, nadolol, or propranolol, to prevent future bleeding.

In cases of active bleeding, perform variceal banding or administer sclerotherapy. If there’s no active bleeding, begin treatment of portal vein thrombosis with anticoagulation.

PVT Without Complications6:55–7:18

Now, let's look at cases where the upper gi endoscopy reveals varices are absent. In this case you should assess your patient for PVT complications or sequelae.

If there’s no evidence of complications or sequelae, you can diagnose PVT without complications and start treatment with anticoagulation!

But, this is not always the case! Sometimes, individuals with PVT might have complications associated with PVT, such as cavernous transformation.

PVT with Cavernous Transformation 7:18–8:15

This condition arises when the portal vein becomes completely blocked, leading to the development of multiple smaller collateral vessels as a compensatory mechanism.

Patients with cavernous transformation may report intermittent pain in their right upper abdomen, known as biliary pain.

Additionally, the physical exam might reveal splenomegaly. Imaging will show numerous tortuous collateral vessels.

With these findings you can confirm the diagnosis of PVT with cavernous transformation. Unfortunately, treatment with anticoagulation to recanalize the portal vein is ineffective.

These patients will eventually develop portal hypertension, so you should offer them supportive care. Another important complication to consider is pylephlebitis, which refers to an inflamed and suppurative portal vein thrombus.

PVT With Pylephlebitis 8:15–9:01

In such cases, patients will often say they have fever and abdominal pain. In this case, your next step is to obtain CBC to assess the white blood count.

If the CBC reveals leukocytosis, diagnose PVT with pylephlebitis. Infection is often polymicrobial, with both gram-negative aerobes and anaerobes, especially Bacteroides fragilis.

For management, you should immediately start antibiotics, and consider anticoagulation therapy. Finally, your patient could have PVT with mesenteric ischemia!

PVT With Mesenteric Ischemia9:01–9:49

These individuals will typically report abdominal pain. Imaging will likely reveal bowel wall thickening, mesenteric fat stranding, and pneumatosis intestinalis, which refers to the presence of gas within the bowel wall.

If these findings are present, diagnose PVT with mesenteric ischemia. As far as treatment goes, begin anticoagulation and consult your surgical team immediately.

In addition, the patient may need thrombolysis, a thrombectomy, or TIPS. Finally, if the patient develops bowel infarction, the patient may also need a bowel resection.

Review 9:49–10:49

Alright, as a quick recap... Portal vein thrombosis refers to a blood clot within the portal vein.

Assessment involves a Doppler ultrasound to confirm the presence of a thrombus, and an abdominal CT or MRI scan to determine its size and evaluate for liver cirrhosis.

If cirrhosis is present, look for HCC and if confirmed start chemotherapy. If no HCC is present, consider anticoagulation or TIPS.

For patients without cirrhosis, perform an upper GI endoscopy to assess whether or not your patient has varices. If varices are detected, initiate a nonselective beta blocker.

On the flip side, if there are no varices, consider PVT complications and sequelae, including cavernous transformation, pylephlebitis, and mesenteric ischemia.