Caroli Disease: When Your Ducts Aren't in a Row
Caroli Disease: When Your Ducts Aren't in a Row
Medical therapy is geared toward reducing the incidence of intrahepatic stones. Oral ursodeoxycholic acid has been shown to have some efficacy in reducing cholesterol stone formation and bile stagnation by increasing bile flow. In patients presenting with acute cholangitis, appropriate antibiotic coverage in recommended. Patients with esophageal varices and portal hypertension secondary to congenital hepatic fibrosis should be treated with a nonselective beta-blocker. ERCP can be performed to relieve biliary obstruction; however, it will not be effective in obstructions of distal IHBDs.
In patients with monolobar involvement and recurrent life-threatening cholangitis, hepatic lobar resection may be performed. In diffuse hepatic disease, liver transplantation has been a successful treatment modality.
Although this patient presented in the sixth decade of life, it is clear from her history that she had had symptoms of Caroli disease since her second or third decade, with steady worsening in severity. This correlates with the varying age of onset and rate of progression seen with this disease entity.
Because the patient has diffuse, bilobar disease and has experienced multiple episodes of cholangitis, definitive treatment will be liver transplantation despite her normal liver function and nonfibrotic hepatic parenchyma.
Treatment of Caroli Disease
Medical therapy is geared toward reducing the incidence of intrahepatic stones. Oral ursodeoxycholic acid has been shown to have some efficacy in reducing cholesterol stone formation and bile stagnation by increasing bile flow. In patients presenting with acute cholangitis, appropriate antibiotic coverage in recommended. Patients with esophageal varices and portal hypertension secondary to congenital hepatic fibrosis should be treated with a nonselective beta-blocker. ERCP can be performed to relieve biliary obstruction; however, it will not be effective in obstructions of distal IHBDs.
In patients with monolobar involvement and recurrent life-threatening cholangitis, hepatic lobar resection may be performed. In diffuse hepatic disease, liver transplantation has been a successful treatment modality.
Conclusion
Although this patient presented in the sixth decade of life, it is clear from her history that she had had symptoms of Caroli disease since her second or third decade, with steady worsening in severity. This correlates with the varying age of onset and rate of progression seen with this disease entity.
Because the patient has diffuse, bilobar disease and has experienced multiple episodes of cholangitis, definitive treatment will be liver transplantation despite her normal liver function and nonfibrotic hepatic parenchyma.
