University of Miami, JFK Medical Center Palm Beach Atlantis, FL
Laura Suzanne K. Suarez, MD1, Larnelle N. Simms, MD1, Curtis Scott, MD2; 1University of Miami, JFK Medical Center Palm Beach, Atlantis, FL; 2West Palm Beach VA Medical Center, West Palm Beach, FL
Introduction: Recurrent pyogenic cholangitis (RPC) was previously known as "Oriental Cholangiohepatitis" as it was found almost exclusively in people who lived in Southeast Asia. In the United States, cases have been increasingly documented in cities with immigrants from endemic countries. However, there are no known reports of RPC in individuals who only briefly visited endemic areas.
Methods: A 47-year-old Caucasian man presented with two days of jaundice, acholic stools, and dark urine, and five years of self-limited fevers and abdominal pain. Multiple previous work-up were normal aside from mild cholestasis and unexplained eosinophilia of 8.9%. Upon admission, blood cultures grew Klebsiella pneumoniae. Magnetic resonance cholangiopancreatography and endoscopic resonance cholangiopancreatography of the abdomen were consistent with RPC (Figure 1 and 2). He improved with antibiotics and biliary decompression (Figure 3). Further attacks were minimized by elective common bile duct exploration and stent replacement. The cause of his recurrent bouts of cholangitis was still unclear however twenty years prior, he served in Japan, South Korea, and Guam for one year. Discussion: RPC is a recurrent syndrome of bacterial cholangitis due to biliary strictures and stone formation occurring within the intrahepatic ducts rather than the gallbladder. It is strongly associated with malnutrition and biliary infestation by Ascaris lumbricoides or Clonorchis sinensis. C. sinensis has a lifespan of 20 years and is endemic in China, Japan, Taiwan, Vietnam, and Korea. It is postulated to cause a disruption in the bile duct’s epithelial barrier leading to a cascade of transient portal bacteremia, secondary stone formation, and increased biliary pressure. Repeated attacks can lead to progressive damage to the bile ducts and liver parenchyma resulting in liver abscesses, cirrhosis and cholangiocarcinoma.
Stool tests for ova and parasites have low sensitivity but should be done in patients with newly diagnosed RPC. Ultrasound is the preferred initial test to demonstrate segmental biliary dilation, hepatolithiasis, and liver abscess. The left hepatic duct is more severely and frequently affected due to its more horizontal transfiguration which may affect bile drainage. Treatment is by biliary decompression. Dilated segments taper toward the thick and fibrous strictures thus when operative plastic repair is attempted, restenosis is common. Surgery is reserved for severe cases.
Figure 1. Magnetic resonance cholangiopancreatography without contrast of the bile ducts: The liver demonstrates intra-hepatic duct dilation. The left hepatic duct measures 13 mm and the right hepatic duct measures 12 mm. There is a filling defect in the proximal common bile duct suggestive of a calculus
Figure 2. Endoscopic retrograde cholangiopancreatography showing the "Arrowhead sign" (red arrows). The arrowhead appearance of the bile ducts is reflected by the decreased arborization of the peripheral ducts and multiple intrahepatic biliary structures. The rapid tapering of the intrahepatic ducts resulting to less acute branching patterns is due to extensive periductal fibrosis.
Figure 3. Spyglass cholangioscopy showing shockwave lithotripsy of the calculus encasing the lumen of the proximal common bile duct.
Disclosures: Laura Suzanne Suarez indicated no relevant financial relationships. Larnelle Simms indicated no relevant financial relationships. Curtis Scott indicated no relevant financial relationships.