A 58-year-old Korean female with history significant for splenectomy 20 years ago for “enlarged spleen” and recurrent pyelonephritis presented with chills, nausea, and vomiting. She had severe sepsis with tachycardia, fever, and hypotension to 60s systolic. Labs were notable for a positive urinalysis and leukocytosis with mild transaminitis. The CT scan showed right-sided pyelonephritis with increasing perinephric inflammatory changes, but no abscess. Additionally it identified increased dilatation of the intrahepatic bile ducts in the posterior right hepatic lobe with multiple associated intraluminal calculi. The patient was started on fluids and antibiotics, and a follow-up MRI showed atrophic liver segments 6 and 7 with compensatory left sided hepatic hypertrophy, suspicious for “oriental cholangiohepatitis.” Blood cultures grew Klebsiella Pneumonia and urine cultures grew multi-drug resistant E.Coli indicating two separate sources of infection, likely urinary and cholangitis. The patient was assessed by hepatobiliary surgery for surgical resection of the atrophic liver segments. She completed a 14-day course of IV antibiotics and underwent a partial liver resection, cholecystectomy and gallstone removal from the bile ducts. Post-operative course was significant for multiple biliary drain placements due to biliary leakage, but she was followed up as an outpatient with complete resolution of symptoms.
This is an atypical presentation of the common eastern illness, chronic pyogenic cholangiohepatitis (AKA “oriental cholangiohepatitis”). Patients typically present with poor nutritional status, RUQ pain, sepsis and cholestasis due to fluke calcification over long periods of time. This patient never had this presentation and immigrated to the USA over a decade ago. However, she had classic radiological findings as described above. The standard of treatment when atrophic liver is involved is surgical resection, which facilitated complete resolution of her abnormal symptoms. The parasite that causes this chronic condition is the “Chinese” or “oriental fluke” or Chlonorchis sinenses. Prevalence is high in China, Korea and Vietnam from eating raw or inadequately prepared fresh-water fish. These flukes excyst in the duodenum and release larvae, which travel up the biliary drainage from the ampulla of Vater. Most infected humans hold low worm burden and are asymptomatic, but late sequelae include cholangitis, biliary obstruction, and cholangiohepatitis the latter of which was present in this patient.
Chronic pyogenic cholangiohepatitis is a late sequelae from infection with the Chinese/oriental liver fluke, Chlonorchis sinenses. Although it is endemic only in Asia, it should be high on the differential in patients who have remote history of immigration from Asia regardless of time from travel.