Sex: male
Age: 29 years
History
Crohn's disease with affecting the ileum and the entire colon including the rectum. Medically treated for 4 years.
Laboratory data
Serum bilirubin raised x 4, alkaline phosphatase raised x 10.
Physical findings
Seems to have a chronic disease. Enlargement of the liver and spleen.
Case text
The patient presented with yellow colour of the sclerae and fatigue during 3 weeks, no abdominal pain. The frequency of defecation normal, stools normal, 2 kg weight loss.
Image 1
ERC.
Image 2-5
Ultrasonography including Doppler.
Image 2: Oblique view of the porta hepatis.
Image 3: Doppler of the portal vein.
Image 4: Doppler of the main branch of the portal vein.
Image 5: Oblique view of the spleen.
Image 6-8
Post contrast spiral CT of the liver 4 years after image 1 and 2-5.
Spiral CT, 120 ml Iodinated, 2 ml/sec, delay 60 sec.
Image 6: Axial view at a level near the diaphragm.
Image 7: Axial view at a level 45 mm caudally.
Image 8: Axial view at a level 75 mm caudally.
Image 9-12
TIPS and embolization of varices.
Image 9: CO2 portography.
Image 10: Portography after transhepatic puncture of the right portal vein.
Image 11: Portography after placement of the stent.
Image 12: Portography following embolization of varices.
Image 1
1. What is the abnormality present on the cholangiogram?
Diffusely distributed, multifocal, short, annular strictures, involving the bile ducts are seen. The ducts between the strictures have a normal dimension or are only slightly dilated. Grade 1 esophageal varices, but no hypertensive gastrophathia was seen at the endoscopy.
Image 2-5
2. What are the abnormalities present on the ultrasonograms?
Enlarged liver and spleen is noticed. No dilation of intra- or extrahepatic ducts. Prominent periportal echogenicity and diffuse parenchymal changes are observed. No varices was seen and the speed of flow in the portal vein and the main branches were low, but within normal range.
Image 6-8
3. What are the abnormalities present on the CT scan?
Periesophageal contrast enhanced varices is seen just anterior to the esophagus as it passes through the diaphragm. The varices can be followed to the perigastric region. The liver attenuation pattern is patchy because of areas with low perfusion. The left and caudate lobes are relatively enlarged, compared to the right lobe of the liver. The spleen is enlarged and collaterals between the splenic and the renal vein are observed. Enlargement of lymph nodes is noticed and so are small amounts of ascites. Grade 3 esophageal varices and hypertensive gastropathia is seen at endoscopy.
Image 9-12
4. What is seen on the TIPS series?
Wedged CO2 portography shows the left portal vein occluded and a normal main and right portal vein. Transhepatic puncture of the right portal vein was performed, a 10 mm Wall-stent protheses was placed, and the parenchymal tract between the hepatic and portal vein dilated with a 10 - mm diameter angioplasty balloon. The portal venogram shows no filling at the intrahepatic portal veins, but still shows filling of the coronary vein and varices.
Embolization of the coronary vein was performed with coils, and the next portal venogram shows no filling of the varices.
The portosystemic gradient was reduced to 8 mmHg by the procedure.
Final diagnosis
Portal hypertension in a patient with primary sclerosing cholangitis and Crohn’s disease.
Differential diagnosis
None.
Discussion
Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease of the biliary tract, characterized by progressive inflammation and fibrosis of the intra- and extrahepatic bile ducts, and of the periportal tissues, leading to bile duct obliteration, cholestasis, and, eventually, biliary cirrhosis.
PSC is often associated with Crohn's disease. The radiologic gold standard for the diagnosis of PSC is direct cholangiography. The cirrhosis and portal hypertension can be seen on CT as a small nodular liver, splenomegalia, varices, and ascites. PSC predisposes to development of cholangiocarcinoma.
Patients, who develop cirrhosis, will be candidates for a liver transplantation.
The mortality of a first variceal hemorrhage is approximately 50 %.
TIPS is a highly effective and safe alternative for the control of portal hypertensive complications and is a bridge to trans-plantation in this patient.