A 24-year-old woman with no history of interest presented with pruritus, choluria and acholia 5 days before.
There is also pain in the right hypochondrium, loss of appetite and asthenia.
She had no change in bowel habits or skin lesions, and had not reported joint pain at any time.
The only physical examination showed the presence of a cutaneous mucosa not very marked.
The blood count was severely normal.
Biochemistry was the following alkaline phosphatase: 852 IU/dl, AST 148 IU/l, ALT 472 IU/l, total bilirubin 4.8 mg/dl, direct bilirubin 3.5 mg/dl, normal TG 24
Serology was negative for hepatitis A, B and C, as well as for cytomegalovirus.
Analyses of antinuclear, antimitochondral and smooth muscle antibodies were performed, which were also negative.
Abdominal ultrasound showed dilatation of the intrahepatic bile duct.
During the following two weeks bilirubin levels increased to 7 mg/dl and there were no significant changes in clinical course.
Abdominal CT showed dilation of the intrahepatic bile duct especially to the left hepatic lobe.
There was a mass effect on the right branch of the portal without appreciating dilation of the extrahepatic bile duct.
Magnetic resonance imaging was performed in which a sudden stenosis of the bile duct of 1.5 cm long at the confluence level of the two right and left livers was detected.
These images suggest the presence of a cholangiocarcinoma.
The next step was to perform ERCP.
Cytology did not demonstrate the presence of tumor cells and a temporary prosthesis was placed.
Bilirubin levels dropped to 1.8 mg/dl. With the uncertain diagnosis of biliary tract stenosis, the patient underwent surgery.
In the intervention a dissection and exploration of the entire biliary tree was performed.
An inflammatory mass was found that included the anterior branch of the right hepatic artery.
The bile ducts were hard from the union of the cystic duct to the secondary divisions.
The hepatic parenchyma was not infiltrated.
Multiple biopsies were performed including a lymph node of the hilium with benign results.
A wide resection was performed from the common liver to the anteroposterior division of the right hepatic duct.
The postoperative course was satisfactory.
Anatomopathological analysis revealed a chronic inflammatory process with intense fibrotic component suggestive of primary sclerosing cholangitis.
One year later the patient is asymptomatic.
