Background: Primary sclerosing cholangitis is a rare form of progressive biliary inflammation and fibrosis of unknown etiology that ultimately destroys the liver, leading to cirrhosis, liver failure, and death. Only one prior case has been reported in pregnancy.
Case: Our patient had the diagnosis of primary sclerosing cholangitis made 2 years before conception. Her course was remarkable for retroplacental hemorrhage at 14-16 weeks and preplacental hemorrhage at 28-34 weeks, with fetal growth retardation, spontaneous premature rupture of the membranes, meconium-stained amniotic fluid, fetal bradycardia, and peripartal exacerbations of her disease. Most uniquely, however, in our case the fetal compromise was associated with an extreme elevation of the bile acid level to greater than 2000 mg/dL in the fetal circulation.
Conclusion: The extreme elevation of fetal bile acidemia at levels greater than 40 times normal in our case may well represent the pathophysiologic link between aberrations of maternal bile acid metabolism during pregnancy and fetal compromise.