Table of Contents
- Key Points
- Background: The Medical Mystery
- Patient Symptoms and History
- Initial Examination and Test Results
- What the Scans Revealed
- Differential Diagnosis: Considering All Possibilities
- The Correct Diagnosis
- Liver Biopsy Findings
- Treatment Approaches
- What This Means for Patients
- Frequently Asked Questions
- Source Information
Key Points
- A 62-year-old man had abdominal pain after eating for two years.
- He was initially diagnosed with cirrhosis but had preserved liver function.
- CT scan revealed portal vein thrombosis with cavernous transformation.
- The likely cause was abdominal trauma from a car accident five years earlier.
- Liver biopsy confirmed no cirrhosis, only changes from chronic portal vein blockage.
Background: The Medical Mystery
This case involves a 62-year-old man who presented with a two-year history of abdominal pain that occurred after eating. He had previously received a diagnosis of cirrhosis (liver scarring) while living in Costa Rica, but when he came to the United States, his new medical team couldn't access his overseas medical records. The doctors at Massachusetts General Hospital faced a diagnostic challenge because the patient's symptoms and test results didn't perfectly match typical cirrhosis cases.
What made this case particularly interesting was the discrepancy between the presumed cirrhosis diagnosis and the patient's relatively preserved liver function. Typically, cirrhosis causes both portal hypertension (high blood pressure in the liver's blood vessels) and impaired liver function, but this patient maintained normal levels of important proteins that the liver produces.
Patient Symptoms and History
The patient's medical journey began two years before admission when he first developed postprandial abdominal pain (discomfort after eating). One year before admission, while living in Costa Rica, he received a cirrhosis diagnosis. Eight months later, he immigrated to the United States, where his new doctors couldn't access his previous medical records.
Three months before admission, he noticed a reducible umbilical hernia (a bulge near the belly button that could be pushed back in). Six weeks later, his post-meal abdominal pain worsened in both severity and frequency and was accompanied by nausea. One month before admission, the umbilical hernia became unable to be reduced, prompting his first U.S. hospital visit.
Key aspects of his history included:
- Significant weight loss: approximately 20 kg (44 pounds) over two months
- No alcohol use, smoking, or illicit drug use
- History of hepatitis as a child that resolved without treatment
- Motor vehicle accident five years earlier where he was unrestrained and sustained abdominal trauma from the steering wheel
- Worked in cattle farming in Costa Rica with no travel outside the country before immigrating
- No family history of liver disease or blood clotting disorders
Initial Examination and Test Results
On examination at Massachusetts General Hospital, the patient had:
- Body temperature: 36.7°C (98.1°F)
- Heart rate: 49 beats per minute
- Blood pressure: 90/61 mm Hg
- Oxygen saturation: 97% on room air
- Body mass index: 26.7
- Diffuse abdominal tenderness, worse in the upper abdomen
- No leg swelling, abdominal swelling, or confusion
Laboratory tests revealed several important findings shown in this table:
Key Laboratory Results:
- Hematocrit: 40.5% (normal range: 41.0-53.0%)
- Hemoglobin: 14.0 g/dL (normal range: 13.5-17.5 g/dL)
- White blood cell count: 3,900/μL (normal range: 4,500-11,000/μL)
- Platelet count: 70,000/μL (normal range: 130,000-400,000/μL) - significantly low
- Alkaline phosphatase: 118 U/L (normal range: 45-115 U/L) - slightly elevated
- Alanine aminotransferase (ALT): 43 U/L (normal range: 10-55 U/L) - normal
- Aspartate aminotransferase (AST): 53 U/L (normal range: 10-40 U/L) - slightly elevated
- Total bilirubin: 1.8 mg/dL (normal range: 0.0-1.0 mg/dL) - elevated
- Direct bilirubin: 0.5 mg/dL (normal range: 0.0-0.4 mg/dL) - slightly elevated
- International normalized ratio (INR): 1.1 (normal range: 0.9-1.1) - normal
- Albumin: 4.5 g/dL (normal range: 3.3-5.0 g/dL) - normal
Notably, his platelet count was significantly low at 70,000/μL (normal 130,000-400,000), which often occurs with portal hypertension as blood cells get trapped in an enlarged spleen. His liver enzymes were only mildly elevated, and his liver synthetic function (as measured by INR and albumin) remained normal—unusual for advanced cirrhosis.
What the Scans Revealed
Computed tomography (CT scan) of the abdomen and pelvis with intravenous contrast revealed several crucial findings:
The imaging showed an umbilical hernia with a 3.6-centimeter neck containing a non-obstructed loop of small bowel. More importantly, the scans revealed:
- A shrunken liver with lobulated contour and diffuse hypoattenuation (appearing darker than normal on CT)
- Extensive paraesophageal and mesenteric varices (abnormally enlarged veins)
- The main portal vein and superior mesenteric vein were not identifiable, suggesting cavernous transformation (where multiple small blood vessels develop to bypass a blocked main vein)
- Significantly enlarged spleen measuring 18.2 cm (normal is less than 13.0 cm)
Doppler ultrasonography confirmed no blood flow in the main portal vein but normal flow through the hepatic veins, hepatic artery, and inferior vena cava. These findings pointed toward portal vein thrombosis (blood clot in the portal vein) with subsequent cavernous transformation.
Differential Diagnosis: Considering All Possibilities
The medical team considered multiple potential causes for the patient's portal hypertension:
Posthepatic causes: Conditions that block blood flow after it leaves the liver, such as blood clots in the inferior vena cava or heart problems like constrictive pericarditis. These were ruled out based on imaging and the absence of characteristic symptoms like breathing difficulties or leg swelling.
Intrahepatic causes: Conditions within the liver itself, including: - Cirrhosis (initial diagnosis but unlikely due to preserved liver function) - Hepatic amyloidosis or sarcoidosis (ruled out due to lack of typical features) - Hepatic schistosomiasis (a parasitic infection uncommon in Costa Rica)
Prehepatic causes: Conditions before blood reaches the liver, including: - Extrinsic compression of the portal vein (ruled out by imaging) - Portal vein thrombosis (becoming the leading candidate)
The team particularly focused on the portal vein thrombosis possibility, noting that the patient's abdominal trauma from his car accident five years earlier could have caused endothelial injury (damage to blood vessel lining) that initiated blood clot formation.
They also considered why the liver appeared shrunken on imaging. The liver receives approximately 75% of its blood supply from the portal vein, which carries nutrient-rich blood from the intestines. When this supply is blocked by a clot, the liver can atrophy (shrink) over time due to lack of essential hepatotrophic substances, even while maintaining its synthetic function.
The Correct Diagnosis
The final diagnosis was hepatic atrophy due to chronic portal vein thrombosis, with probable mesenteric congestion causing postprandial abdominal pain.
This meant that the blood clot in the portal vein likely started after the abdominal trauma from the car accident five years earlier. Over time, the complete blockage led to:
- Development of collateral blood vessels (cavernous transformation)
- Increased pressure in the portal venous system (portal hypertension)
- Shrinkage of the liver due to reduced blood flow
- Enlargement of the spleen
- Development of varices (enlarged veins) in the esophagus and stomach
The post-meal abdominal pain occurred because eating increases blood flow to the intestines, which then couldn't properly drain through the blocked portal system, causing congestion and pain.
Liver Biopsy Findings
A transjugular liver biopsy (performed through a neck vein) provided definitive evidence:
- No cirrhosis or advanced fibrosis
- Mild distortion of liver architecture with expanded portal tracts
- Multiple irregular blood vessels in portal areas, some showing medial hyperplasia (thickened walls) or intravascular thrombi (clots)
- Vaguely nodular pattern of expansion with compression and atrophy of hepatic plates
- No significant inflammation, fat accumulation, or iron overload
The pathological diagnosis confirmed portal vascular remodeling consistent with portal vein thrombosis effects, nodular regenerative hyperplasia-like changes, hepatic parenchymal distortion and atrophy, and no features of cirrhosis.
Treatment Approaches
Management of portal vein thrombosis depends on whether it's acute (less than 6 months) or chronic (6 months or more) and whether cirrhosis is present. In this chronic, non-cirrhotic case, treatment involved:
First, upper endoscopy revealed grade 3 (large) non-bleeding esophageal varices and portal hypertensive gastropathy. Treatment included:
- Variceal banding (placing rubber bands around enlarged veins to prevent bleeding)
- Nadolol (a non-selective beta-blocker) to reduce pressure in the portal system
Decisions about anticoagulation (blood thinners) were based on assessing the risk of clot extension and underlying thrombotic factors. The hematology service was consulted for input.
Procedures to reduce portal pressure were considered, including:
- Surgical shunt techniques (less common now)
- Transjugular intrahepatic portosystemic shunt (TIPS) - a procedure where a radiologist creates a channel between portal and hepatic veins
- Portal vein recanalization and TIPS placement (PVR-TIPS)
- Angioplasty of the superior mesenteric vein
The interventional radiology team evaluated which procedure would be most appropriate for this patient's specific anatomy.
What This Means for Patients
This case illustrates several important points for patients with abdominal symptoms:
First, abdominal trauma—even from years earlier—can have long-lasting consequences. Patients should always inform their doctors about any significant accidents or injuries, no matter how long ago they occurred.
Second, not all liver problems are cirrhosis. Portal vein thrombosis can cause similar complications (varices, splenomegaly, portal hypertension) while preserving liver function. This distinction is crucial because treatment approaches differ significantly.
Third, the normal INR (1.1) and albumin (4.5 g/dL) in this patient were important clues that his liver was still functioning well despite its shrunken appearance on scans. Patients should understand what these tests measure and why they're important.
Fourth, for patients with portal vein thrombosis, treatment may involve multiple specialists: gastroenterologists, hepatologists, interventional radiologists, and hematologists. A multidisciplinary approach often yields the best outcomes.
Finally, this case highlights the importance of thorough diagnostic evaluation when the clinical picture doesn't perfectly match a common diagnosis. Second opinions and additional testing (like the liver biopsy in this case) can reveal unexpected causes of symptoms.
Frequently Asked Questions
What causes portal vein thrombosis?
Portal vein thrombosis can be caused by conditions that damage the portal vein lining, such as abdominal trauma, or by blood clotting disorders. In this case, a car accident five years earlier likely caused endothelial injury that initiated clot formation. Other causes include cirrhosis, infections, and cancers, but here no underlying liver disease was found.
Why does abdominal pain occur after eating with portal vein thrombosis?
After eating, blood flow to the intestines increases. In portal vein thrombosis, the blocked portal vein cannot drain this blood properly, causing congestion and pain. This patient's postprandial abdominal pain was likely due to mesenteric congestion from the chronic blockage.
How is chronic portal vein thrombosis treated?
Treatment for chronic portal vein thrombosis focuses on managing complications like varices and portal hypertension. This patient received variceal banding and a beta-blocker (nadolol) to reduce portal pressure. Anticoagulation may be considered based on clot extension risk. Procedures like TIPS or portal vein recanalization can also be used.
What is cavernous transformation of the portal vein?
Cavernous transformation is the development of many small blood vessels that bypass a blocked main portal vein. It occurs when chronic portal vein thrombosis prevents normal blood flow, so the body creates collateral channels. Imaging may show the main portal vein is not identifiable, and Doppler ultrasound confirms no blood flow in it.
What is a transjugular liver biopsy and why is it done?
A transjugular liver biopsy is a procedure where a needle is inserted through a neck vein to obtain a liver tissue sample. It is done to diagnose liver conditions without surgery. In this case, it confirmed portal vein thrombosis effects and ruled out cirrhosis, showing no advanced fibrosis.
Can abdominal trauma cause portal vein thrombosis years later?
Yes, abdominal trauma can damage the blood vessel lining, initiating clot formation. In this case, a car accident five years earlier likely caused the portal vein clot. Patients should inform doctors about any past injuries, even if they seem unrelated to current symptoms.
What is the difference between cirrhosis and portal vein thrombosis?
Cirrhosis is liver scarring that impairs liver function, while portal vein thrombosis is a blood clot blocking the portal vein. Both cause portal hypertension, but in portal vein thrombosis, liver function may remain normal. This distinction is important because treatments differ significantly.
Source Information
Original Article Title: Case 6-2025: A 62-Year-Old Man with Abdominal Pain
Authors: Gabrielle K. Bromberg, Katayoon Goodarzi, Robert G. Rasmussen, Kenneth E. Sherman, Sanjeeva P. Kalva, Jonathan N. Glickman, Dennis C. Sgroi, Eric S. Rosenberg
Publication: The New England Journal of Medicine, February 20, 2025; 392:807-16
DOI: 10.1056/NEJMcpc2412516
This patient-friendly article is based on peer-reviewed research from the Massachusetts General Hospital Case Records, originally published in The New England Journal of Medicine.
