Abstract

Recurrent pyogenic cholangitis (RPC) is a condition characterized by intrahepatic stone formation of unknown etiology causing biliary obstruction and recurrent cholangitis. Almost exclusively reported in East Asia, there is increasing incidence in the West due to human migration. Rarely, sporadic cases have been reported in people with no links to East Asia. We report our experience with 5 patients with RPC (4 East Asians and one Caucasian) managed at our institution in the past decade. We compared the Caucasian female with no travel history or links to East Asia with 4 East Asian females, all of whom presented with features of cholangitis comprising fever, right upper quadrant pain, and jaundice. All had a prior history of cholecystectomy with recurrent post-cholecystectomy cholangitis. Diagnosis was made from history and characteristic findings on abdominal computerized tomography (CT) and endoscopic retrograde cholangiopancreatography (ERCP). Individual clinical, radiological, and interventional details are described in Tables 1 and 2. All 4 East Asian patients had significant dilation of the left intrahepatic duct (IHD), common hepatic duct (CHD), and common bile duct (CBD); in addition to pigment stones and benign biliary stricture. In contrast, the Caucasian patient had a mixture of pigment and cholesterol stones, less intrahepatic dilation, and no biliary stricture or liver atrophy. An interdisciplinary approach was employed in management. All 4 East Asian patients underwent surgery (Table 2) with the goal of resecting the area of recurrent infection, biliary stasis, and hepatic atrophy. The Caucasian patient refused surgery and had one more episode of cholangitis requiring repeat ERCP and stone extraction, while the East Asians who underwent surgical management had a favorable outcome with no further episodes of cholangitis. Increased awareness and timely diagnosis is needed for early involvement of a multi-disciplinary team in the management of patients with RPC. Regular surveillance is crucial as there is a high chance for stone recurrence. Repeated attacks can lead to progressive damage to the bile ducts and liver parenchyma resulting in liver abscesses, cirrhosis, and atrophy; along with increased risk of cholangiocarcinoma and death due to sepsis and liver failure. Resection of the affected hepato-biliary segments has been shown to lead to better quality of life, lower rates of secondary biliary cirrhosis, cholangiocarcinoma, and mortality.Table: Table. Clinical CharacteristicsTable: Table. Radiological and Pathological Findings

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