Ch. 54 – Biliary System

1576 Section X Abdomen
Figure 54-27 MRC documenting diffuse biliary strictures characteristic
of primary sclerosing cholangitis.
Clinical Presentation
There are no pathognomonic signs of PSC, and the
natural history is variable. Although patients can be
asymptomatic for up to 15 years, prolonged disease ultimately
leads to progressive hepatic failure. Mean age at
presentation is 40 to 45 years, and most patients are
male. 38 About 75% of patients are symptomatic at presentation
with evidence of cholestatic liver disease such as
jaundice, pruritus, and fatigue. Symptoms of bacterial
cholangitis are uncommon. The condition is characterized
by relapses and remissions, with quiescent periods.
The median survival for patients with PSC from the time
of diagnosis ranges from 10 to 12 years.
Diagnosis
Cholangiography confi rms the diagnosis of PSC with
evidence of diffuse multifocal strictures commonly found
in both intrahepatic and extrahepatic bile ducts. Involvement
of the extrahepatic ducts alone without intrahepatic
duct involvement occurs in 5% to 10% of patients with
PSC. Despite the presence of diffuse disease in most
patients with PSC, the hepatic duct bifurcation is often
the most severely strictured segment of the biliary tree.
A liver biopsy to determine the degree of hepatic fi brosis
or the presence of cirrhosis is also critical in selecting
therapy.
Management
Primary sclerosing cholangitis is a progressive disease
that eventually results in biliary cirrhosis. Patients with
persistent biliary sepsis, biliary cirrhosis, and manifestations
of end-stage liver disease (ascites, variceal bleeding)
are best treated with liver transplantation. Five-year graft
and patient survival after transplantation are excellent
Figure 54-28 MRC demonstrating anastomotic stricture (arrow)
following right lobe living donor liver transplantation. Note the
slight dilation of the biliary tree proximal to the stricture and
signifi cant dilation distal.
at 72% and 85%, respectively. Recurrent PSC has been
reported in up to 10% of patients and may require
retransplantation.
An important consideration in the management of PSC
is the risk for superimposed cholangiocarcinoma, which
occurs in 10% to 20% of cases. 39 Rapid onset of jaundice,
pruritus, and weight loss may be clues to the diagnosis
of cholangiocarcinoma but are not specifi c. Cancer
antigen (CA) 19-9 may be helpful if levels are greater
than 100 U/mL but is not predictive. Repeated brushings
for cytology by endoscopic or percutaneous approaches
should be employed to distinguish benign from malignant
dominant strictures. Long-term survival in patients
with a small incidental cholangiocarcinoma (