Oriental Cholangiohepatitis: Hong Kong Disease in the United States

Abstract

Oriental cholangiohepatitis, also known as Hong Kong disease, is a chronic disease initially triggered by a parasitic infection that results in biliary tree strictures and multiple stones formation in the intrahepatic and extrahepatic ducts. This leads to biliary stasis which ultimately causes recurrent pyogenic cholangitis (RPC). Although the disease is exclusively endemic in Asian countries, several cases has been identified in the United states. A 25 year old Burmese male who immigrated to the USA 3 years ago presented to the hospital with a sudden onset of severe sharp right upper quadrant abdominal pain, fever and jaundice. His only medical history was Opisthorchis viverrini liver fluke infection that was previously treated with praziquantel. On physical exam, he was febrile, tachycardic and hypotensive. He also had altered mental status, scleral icterus and right quadrant abdominal tenderness. Laboratory data showed WBCs 16.1 k/ul, alkaline phosphatase 152 IU/l, AST 58 IU/dl, ALT 71 IU/l, and total bilirubin 1.6 mg/dl (Direct bilirubin 1 mg/dl). Abdominal CT showed dilated intrahepatic bile ducts. MRCP showed numerous intrahepatic filling defects. The patient met all criteria for Reynolds pentad and was diagnosed with septic shock secondary to cholangitis. Intravenous fluids and empiric broad spectrum antibiotics were started. An ERCP was performed which illustrated filling defects in the proximal common bile duct. Pus was drained from the biliary ducts. Blood cultures grew Escherichia coli. Stool for ova and parasite was negative. The patient's symptoms resolved and was discharged in good condition. Over the following two years, the patient was admitted 4 times with recurrent cholangitis that was treated with intravenous antibiotics and biliary drainage with ERCP. He underwent a common bile duct resection with hepaticojejunostomy. The patient did not have any recurrence within 2 years after surgery.1349_A.tif Figure 1: MRCP Showing common bile duct dilation with minimal stone/debris1349_B.tif Figure 2: MRCP Showing intrahepatic bile ducts strictures1349_C.tif Figure 3: MRCP Showing moderate intrahepatic bile duct dilatation CBD stones and dilation with multiple filling defectsOriental cholangiohepatitis can present with RPC which could be life threatening. While treatment with praziquantel is successful in eradicating the offending organism in 90% of the cases, this does not result in resolution of biliary fibrosis. Due to the influx of immigrants, oriental cholangiohepatitis should be suspected in patients from endemic areas. Patients with RPC should be referred for early surgical evaluation to prevent associated morbidity and mortality.