Intrahepatic Lithiasis in a Westerner

Abstract

Intrahepatic lithiasis, defined as calculi proximal to the confluence of the right and left hepatic ducts is a common entity in Asian populations, but rare in Americans, that can be challenging to diagnose in western populations. A 40 year old Caucasian male presented to medical attention multiple times over a period of greater than ten years for recurrent abdominal pain. He initially underwent cholecystectomy at the age of 20 for symptomatic cholelithiasis. A few years later he presented with recurrent abdominal pain mimicking his prior symptoms before cholecystectomy and underwent ERCP which showed common bile duct dilatation and intrahepatic duct dilatation. Endoscopic stone removal and biliary duct stenting were preformed leading to symptom improvement. He subsequently represented with abdominal pain, nausea and vomiting. Repeat ERCP showed common bile duct dilatation and a large obstructing stone of the left hepatic duct. After electrohydraulic lithotripsy dozens of non-faceted, pearly white stones were released from the left lobe of the liver. After representing with similar symptoms, he was treated by ERCP with a fully covered metal stent placed in the common bile duct to allow unimpeded passage of the stones. The patient continues to have occasional episodes of cholangitis from stent occlusion where only a few stones have been observed. But a large number of stones are still visible intrahepatically and a future treatment to be considered is a hepatic resection. Often intrahepatic lithiasis is an asymptomatic process, with some patients becoming symptomatic over a period of years. The most common symptoms of presentation are cholangitis, abdominal pain, or jaundice. The etiology of these stones is most often pigmented stones, and it is theorized that most stones in Western persons are of gall bladder etiology, while parasitical or bacterial infections are most commonly found in Asian patients. A genetic link to an acidic glycoprotein, osteopontin, seems to be involved with hepatolithiasis, but more studies are needed to classify the exact link. Biliary sepsis, intrahepatic abscess and cholangiocarcinoma are all potential complications. Conservative treatment options in mild cases are reasonable, more severe cases may require endoscopic or surgical intervention. This case highlights intrahepatic lithiasis as a rare cause of biliary stones and cholangitis like symptoms in a western person.