Recurrent pyogenic cholangitis (RPC) is a chronic biliary disease endemic to East Asia, characterized by recurrent bacterial infection and pigment stone formation in intrahepatic bile ducts. Also known as 'oriental cholangiohepatitis' or 'hepatolithiasis.' Parasitic infestations (Clonorchis sinensis, Ascaris lumbricoides) and low-protein diet are predisposing factors. The recurrent infection-stone-stricture cycle is pathognomonic. Left hepatic duct involvement is more common than right. Long-term cholangiocarcinoma development risk is 5-10%.
Age Range
25-65
Peak Age
45
Gender
Equal
Prevalence
Uncommon
RPC is fundamentally based on bile duct epithelial damage from parasitic infestation or bacterial infection, altering the biochemical composition of bile. Bacterial beta-glucuronidase enzyme deconjugates bilirubin glucuronide to form free (unconjugated) bilirubin — this combines with calcium to form brown pigment stones. Stones cause duct obstruction, obstruction leads to stasis, stasis to infection, infection to new stone formation — this vicious cycle perpetuates the disease. Recurrent inflammation and obstruction lead to periductal fibrosis and stricture development. On imaging, intraductal stones appear hyperdense on CT and hypointense on T2 MRI because calcium bilirubinate increases X-ray attenuation due to high atomic number and shows paramagnetic effects.
Hyperdense stones (pigment stones) within intrahepatic bile ducts combined with segmental atrophy and capsular retraction. This combination is highly specific for RPC and reflects the chronic consequence of the recurrent infection-obstruction cycle.
Hyperdense stones within intrahepatic bile ducts on non-contrast CT (pigment stones typically 50-100+ HU). Stones follow the duct lumen in linear or tubular configuration. Surrounding ducts may be dilated. Left hepatic system involvement predominates.
Report Sentence
Multiple hyperdense stones within intrahepatic bile ducts, predominantly in the left hepatic system, consistent with hepatolithiasis/recurrent pyogenic cholangitis.
Segmental intrahepatic bile duct dilatation with intraductal filling defects (stones) on MRCP. Stones appear as T2-hypointense filling defects within hyperintense bile fluid. Accompanying strictures typically show segmental and asymmetric distribution. Peripheral duct pruning and CBD dilatation may also be present.
Report Sentence
Segmental intrahepatic bile duct dilatation with multiple hypointense filling defects (intrahepatic stones), predominantly in the left hepatic system on MRCP, consistent with recurrent pyogenic cholangitis.
Segmental hepatic atrophy due to chronic obstruction and recurrent infection — particularly left lateral segment (segments II-III) and/or posterior sector. Compensatory hypertrophy develops in spared segments. Duct dilatation, stones, and capsular retraction accompany the atrophic segment.
Report Sentence
Volume loss, capsular retraction, and dilated intrahepatic bile ducts with accompanying hyperdense intraductal stones in the left lateral segment are consistent with segmental atrophy due to chronic recurrent pyogenic cholangitis.
Periductal hyperintense edema halo around bile ducts during acute attack on T2-weighted images. Heterogeneous signal intensity debris and purulent material may be seen alongside low-signal stones. Reflects active inflammation.
Report Sentence
T2-hyperintense periductal edema around intrahepatic bile ducts with heterogeneous intraductal debris material consistent with acute cholangitis episode.
Segmental intrahepatic bile duct dilatation with intraductal echogenic foci (stones) ± acoustic shadowing on US. 'Arrow-head' sign: echogenic reflection created by stone at distal end of dilated duct. Hepatic parenchymal echogenicity increase may accompany. Left lobe involvement is more prominent than right.
Report Sentence
Segmental intrahepatic bile duct dilatation with intraductal echogenic stones (acoustic shadowing present), predominantly in the left hepatic lobe, consistent with hepatolithiasis.
During acute attacks, contrast-enhanced CT may show periductal enhancement, hepatic abscess formation (hypodense collections with rim enhancement), and periportal edema. Septic portal thrombophlebitis is a rare but serious complication.
Report Sentence
Periductal enhancement and pericanicular hypodense collections with rim enhancement around intrahepatic bile ducts consistent with acute cholangitis and hepatic abscess formation.
Criteria
Intrahepatic stone disease developing without known parasitic infestation or biliary surgery history. Combination of genetic and environmental factors.
Distinct Features
Generally bilateral involvement, slower course. Can also occur in non-Asian populations.
Criteria
Associated with liver flukes such as Clonorchis sinensis or Opisthorchis viverrini, or Ascaris lumbricoides infestation. More common in endemic areas (Southeast Asia).
Distinct Features
Higher cholangiocarcinoma risk (10%+). Duct wall irregularity and parasite shadow may be seen on MRCP. Left duct predominance is prominent.
Criteria
Intrahepatic stones developing after biliary surgery (cholecystectomy, hepaticojejunostomy, Whipple). Biliary stasis and reflux mechanism.
Distinct Features
Stricture at surgical anastomosis site is prominent. Pneumobilia (in biliary-enteric anastomoses) may accompany. Stones typically accumulate proximal to the anastomosis.
Distinguishing Feature
PSC shows diffuse bilateral strictures with beaded pattern, intraductal stones are rare. RPC shows segmental strictures with prominent intraductal stones and debris.
Distinguishing Feature
Cholangiocarcinoma forms a focal mass with delayed enhancement. RPC shows diffuse ductal changes and stones prominently. However, cholangiocarcinoma can develop on RPC background.
Distinguishing Feature
Caroli disease shows saccular biliary dilatation and central dot sign (portal radicle); stones are secondary. In RPC, the stricture-dilatation-stone cycle is the primary pathology.
Distinguishing Feature
Acute cholangitis usually results from single-level obstruction with acute onset. RPC shows chronic course with multiple stones and segmental atrophy.
Urgency
urgentManagement
surgicalBiopsy
Not NeededFollow-up
6-monthAcute attacks require broad-spectrum antibiotic therapy and biliary drainage (percutaneous or endoscopic). Chronic phase treatment includes stone clearance (cholangioscopic lithotripsy, percutaneous transhepatic approach) and stricture dilatation. Hepatic resection (hepatectomy) may be needed for severe segmental disease — curative for localized left lateral segment disease. Cholangiocarcinoma surveillance (MRCP, CA 19-9) is important. Liver transplantation is the last resort for extensive disease.
RPC increases cholangiocarcinoma risk (2-10%). Recurrent infections are treated with antibiotics and biliary drainage. Hepatic resection may be considered for localized disease. Cholangiocarcinoma surveillance is important.