Recurrent Pyogenic Cholangitis Research Articles

Hepatolithiasis: Epidemiology, presentation, classification and management of a complex disease.

The term hepatolithiasis describes the presence of biliary stones within the intrahepatic bile ducts, above the hilar confluence of the hepatic ducts. The disease is more prevalent in Asia, mainly owing to socioeconomic and dietary factors, as well as the prevalence of biliary parasites. In the last century, owing to migration, its global incidence has increased. The main pathophysiological mechanisms involve cholangitis, bile infection and biliary strictures, creating a self-sustaining cycle that perpetuates the disease, frequently characterised by recurrent episodes of bacterial infection referred to as syndrome of "recurrent pyogenic cholangitis". Furthermore, long-standing hepatolithiasis is a known risk factor for development of intrahepatic cholangiocarcinoma. Various classifications have aimed at providing useful insight of clinically relevant aspects and guidance for treatment. The management of symptomatic patients and those with complications can be complex, and relies upon a multidisciplinary team of hepatologists, endoscopists, interventional radiologists and hepatobiliary surgeons, with the main goal being to offer relief from the clinical presentations and prevent the development of more serious complications. This comprehensive review provides insight on various aspects of hepatolithiasis, with a focus on epidemiology, new evidence on pathophysiology, most important clinical aspects, different classification systems and contemporary management.

Enfermedades inflamatorias de páncreas y vía biliar

Inflammatory involvement of the bile duct and pancreas can present in different ways depending on the etiology and place of involvement. These are rare diseases; the most common is autoimmune pancreatitis, which has two subtypes: type 1, which is considered the pancreatic manifestation of systemic IgG4-related disease (IgG4-RD) and type 2, which is considered a primarily pancreatic disease. The most common clinical presentation is in the form of jaundice and pancreatic masses with or without abdominal pain, which necessitates a differential diagnosis with pancreatic adenocarcinoma. Treatment is based on corticosteroids, with good response rates, but there is a non-negligible risk of recurrence which sometimes requires treatment with anti-CD20 (rituximab) or immunosuppressants. IgG4-RD can also affect the bile duct, in which case it is called IgG4-related sclerosing cholangitis, and treatment is similarly based on corticosteroids. Lastly, recurrent pyogenic cholangitis is a rare type of inflammatory disease of the bile duct characterized by the formation of recurrent intrahepatic and extrahepatic bile duct stones and recurrent episodes of cholangitis that often require endoscopic, radiological, or surgical treatment in addition to medical treatment.

S1954 Recurrent Pyogenic Cholangitis: A Case Report

S1954 Recurrent Pyogenic Cholangitis: A Case Report

S1991 Abdominal Cutaneous Drainage: A Rare Presentation of Recurrent Pyogenic Cholangitis

S1991 Abdominal Cutaneous Drainage: A Rare Presentation of Recurrent Pyogenic Cholangitis

S1943 A Rare Case of Recurrent Pyogenic Cholangitis With Atypical Features

S1943 A Rare Case of Recurrent Pyogenic Cholangitis With Atypical Features

Percutaneous transhepatic biliary stone removal: A single-center retrospective study

Objectives: To report, the authors’ experience with percutaneous transhepatic biliary stone removal, focusing on the technical aspects and overall performance, as well as reviewing few challenging cases. Material and Methods: All cases of percutaneous transhepatic biliary stone removal from 2015 to 2022 were retrieved, including intrahepatic locations. Cases with negative initial cholangiogram suggestive of stone passage were excluded from the study. The demographic data, procedural indications and details, stone characteristics, fluoroscopic images, success rate, and complications were retrospectively evaluated by two experienced observers. Results: The final study included 67 patients (mean age = 76.8). Majority of indications were altered gastric anatomy and failed endoscopic retrograde cholangiopancreatography. The mean largest stone size was 9 mm and most cases had multiple stones. Balloon sphincteroplasty was performed in all cases and mechanical lithotripsy in 24%. Fourteen cases required repeated trials, and they were mostly staged, depending on technical factors and patient condition. Recurrent pyogenic cholangitis (RPC) usually required repeated trials, and two separate sites of catheterization were made in the same lobe for one challenging case. Complete stone retrieval was not achieved in 9% (six out of 67) of cases: failure of mechanical lithotripsy (n = 2); failure of balloon occlusion technique (n = 1); refluxed stone (n = 1); and multiplicity of intrahepatic stones (n = 2). Three minor complications were encountered, including sepsis and self-controlled hemobilia. No major complications. Conclusion: Percutaneous extrahepatic biliary stone removal is a safe and technically feasible procedure after a failed endoscopic approach or altered biliary tract anatomy. Cases of RPC or previous Whipple’s operation could be technically challenging. Careful pre-procedural planning and anatomical review are crucial.

Rare primary intrahepatic lithiasis in a young patient

Introduction. Intrahepatic lithiasis (IHL) is a disease that occurs in middle-aged and elderly people. Presentations of IHL in the young are rare, and considerations in the differential diagnosis include primary sclerosing cholangitis, recurrent pyogenic cholangitis, bile acid transporter defect, Caroli?s disease, and other known genetic diseases. Treatment is often complex, all in order to prevent complications. In this report, we describe the diagnosis and treatment, with the application of a flexible ureteroscope of 4 Fr, of a younger patient with intra-hepatic lithiasis. Case report. A 25-year-old man appeared with a known diagnosis of IHL and a recurrent attack of abdominal pain that required medical treatment. Magnetic resonance imaging of the abdomen showed segmental stenosis of the left bile duct and segmental bile duct for the lateral section with intraductal calculi and its proximal dilatation and mild dilatation of the bile ducts for liver segments II and III. During surgery, a cholangiography and ultrasonography of the liver were performed. Through choledochotomy, the bile ducts were flushed, and extirpation of the several calculi was performed. The bile ducts were examined with a chole-dochscope, and the remaining concrements were removed with a flexible ureteroscope. Conclusion. Segmental liver bile ducts may be explored with a flexible ureteroscope without bile duct injury or trauma. In selected cases, with isolated lithiasis in one liver lobe and the absence of concomitant diseases, IHL can be treated surgically without liver resection. This case is unique because we did not perform liver resection but duct stone extraction, which was an appropriate treatment since there was no recurrence during the two-year follow-ups.

Secondary Bacterial Peritonitis and Pelvic Abscess Complicating Recurrent Pyogenic Cholangitis

Secondary Bacterial Peritonitis and Pelvic Abscess Complicating Recurrent Pyogenic Cholangitis

Overview of recurrent pyogenic cholangitis

BackgroundThe clinical illness known as recurrent pyogenic cholangitis (RPC) is characterized by recurrent suppurative cholangitis caused by hepatolithiasis and extrahepatic stones in the biliary channels. The actual pathophysiology of RPC is still unknown, but certain enteric bacterial species colonizing the biliary tract in conjunction with potential dietary variables have been suggested as potential causes. RPC is frequently referred to as having hepatobiliary surgery, especially segmental hepatectomy, as the only effective treatment. Objectivesto determine the prevalence of recurrent pyogenic cholangitis, explain the typical symptoms a patient with the condition would exhibit, and describe the diagnosis and treatment of RPC. Methodsthe PubMed database and EBSCO Information Services were utilized to choose the articles. In our review, all pertinent articles related to both our topic and other articles were used. Other articles that have nothing to do with this subject were not included. The group members looked through a certain format in which the data had been extracted. Conclusion Recurrent pyogenic cholangitis is a complicated illness, and it is becoming more common in Western nations. Radiologists need to understand how to diagnose this condition and how to use imaging as a benchmark for subsequent treatment. Before the surgical intervention, the intrahepatic distribution of the patient's condition must be determined, and the combined efforts of gastroenterologists, radiologists, and surgeons are necessary for the effective treatment of recurrent pyogenic cholangitis.

S2887 Stones, Strictures and Atrophy: Treating Sequelae of Infectious Hepatolithiasis

Introduction: Primary intrahepatic cholelithiasis has been well-associated with brown or pigmented stones and recurrent pyogenic cholangitis. However, other important potential chronic complications exist beyond cholangitis of which gastroenterologists should be aware. Case Description/Methods: We present a case of a 57 year-old man with history of diabetes who returned from a 13-month stay in Japan to be admitted to an outside facility with DKA and cholangitis. He was found to have a liver abscess and left hepatic duct stricture treated with sphincterotomy, biliary stenting via ERCP, abscess drainage, and cholecystectomy. He followed up externally for stent removal with normalization of liver chemistries however CT imaging 1 year later showed persistent left hepatic duct stricture with intrahepatic segmental dilation. He experienced no early satiety, weight loss, or jaundice. He was referred to our service for further evaluation of chronic indeterminate biliary stricture. Endoscopic ultrasound demonstrated extensive small and large left-sided hepatolithiasis (A). Cholangiography during ERCP demonstrated complete filling defect of the left intrahepatic biliary ductal system preventing selective guidewire passage. Cholangioscopy was performed with successful electrohydraulic lithotripsy (EHL) of stone burden in the left main hepatic system for selective wire passage and stenting of the left intrahepatic system with a plastic stent. Biliary epithelium appeared smooth and benign. The patient was given antibiotics with no complications. ERCP was repeated in 6 weeks with further cholangioscopy with EHL fragmentation and removal of stones in left intrahepatic ductal branches, however ductal disease remained throughout smaller distal branches of the left lobe on cholangiogram. The main duct was protected with a fully-covered metal stent and antibiotics were given. Follow-up MRI/MRCP demonstrated atrophic left liver lobe (C) in addition to our findings. Left partial hepatectomy was thus performed by Surgery for definitive therapy of the diseased lobe and the patient did well post-operatively with benign pathology. (Figure) Discussion: Even in the absence of a typical recurrent cholangitis clinical syndrome, treatment of other chronic sequelae of severe primary hepatolithiasis is important in the prevention of progression to cirrhosis. Multiple interventional endoscopic methods are available and should be utilized to establish the diagnosis and optimize therapy; which may include surgery in severe cases.Figure 1.: (A) Linear EUS view of left lobe intrahepatic cholelithiasis; (B) cholangioscopic view of intrahepatic cholelithiasis fragmented by electrohydraulic lithotripsy; (C) MRI with left lobe atrophy and hepatolithiasis.

S2919 Bile Duct Injury Due to Ketamine Use

Introduction: Ketamine is used routinely in the hospital for anesthesia, but is also used recreationally for its hallucinogenic and dissociative side effects. Chronic recreational use has been linked to bile duct damage and ulcerative cystitis. We report a case of ketamine induced sclerosing cholangiopathy. Case Description/Methods: A 42-year-old Cantonese woman with past medical history of bilateral hydronephrosis with stents leading to chronic kidney disease presented with abdominal pain associated with abnormal transaminases. Alkaline phosphatase was high at 1,017 IU/L with gamma glutamyl transferase of 2,310 IU/L with normal bilirubin. Abdominal imaging showed diffuse dilatation of the common bile duct. Viral serologies and anti-mitochondrial antibody were negative. She reported chronically elevated liver tests of unknown etiology. Social history was notable for prior alcohol abuse. She denied family history of liver diseases. Her pain resolved and plan was for outpatient follow-up, but she re-presented with sepsis with a rise in bilirubin to 2.9 mg/dL (Table 1). Given prior abnormal imaging, a magnetic resonance cholangiopancreatography was obtained, which showed increased intra- and extrahepatic bile duct dilation with irregular appearance of the central intrahepatic ducts with possible stricture. Differential diagnoses were recurrent pyogenic cholangitis, sclerosing cholangitis, and IgG4 disease. Her IgG level was high at 2,779 mg/dL with IgG4 elevated at 213 mg/dL. Liver biopsy showed chronic cholestatic liver injury consistent with sclerosing cholangitis (Image 1). IgG4 stain was negative. Upon further investigation, patient admitted to at least a decade of daily ketamine use, which was likely the culprit of both her kidney and liver diseases. Discussion: Due to her age, race, gender and rarity of ketamine induced cholangiopathy, she was not directly asked about ketamine use. Since the early 2000s, ketamine has emerged as the illicit drug of choice in Hong Kong and has seen increased use throughout Asia. The biliary damage from ketamine has been studied by a Chinese group that found 62% of the 257 chronic ketamine users had biliary tract anomalies. The greater the alkaline phosphatase, the higher the likelihood of finding biliary tract anomalies on imaging. Ketamine cessation has resulted in normalization of liver tests and imaging, however worsening cholangiopathy has been seen despite abstinence. In patients who present with unexplained cholangiopathy, chronic ketamine use should be considered.Figure 1.: Liver biopsy: A mild lymphoplasmacytic inflammation is noted in some triads with focal and mild interface activity. The bile ducts are injured (irregular contour, unevenly distributed nuclei, intracytoplasmic vacuoles) with focal periportal cholestasis. Immunohistochemical stain for keratin 7 confirms predominantly periportal cholestatic hepatocytes. Keratin 19 shows widespread loss of canals of Hering and focal bile duct loss. Rhodanine stain confirms periportal hepatocellular copper accumulation compatible with a chronic cholestatic process. Table 1. - Patient's hepatic function panels during initial encounter, subsequent encounter, and at least 6 months after subsequent encounter Liver Tests Reference Range & Units Initial Hospitalization Subsequent Hospitalization Most Recent Liver Tests Total bilirubin 0.2 – 1.2 mg/dL 0.8 2.9 4.5 Direct bilirubin 0 – 0.5 mg/dL 0.7 1.8 3.3 Aspartate aminotransferase 5 – 34 IU/L 75 104 88 Alanine transaminase 0 – 37 IU/L 74 129 91 Alkaline phosphatase 40 – 150 IU/L 1017 1106 845 Gamma glutamyl transferase 12 – 43 IU/L 2310 2516 1897

A CASE RECURRENT PYOGENIC CHOLANGITIS AFTER CHOLECYSTECTOMY

TYPE: Late Breaking Case Report TOPIC: Critical Care INTRODUCTION: Recurrent Pyogenic Cholangitis (RPC) after cholecystectomy and choledocholithiasis treated with endoscopic retrograde cholangiopancreatography (ERCP) has been reported in less than two percent of patients. CASE PRESENTATION: A 78-year-old female status post ERCP and laparoscopic cholecystectomy for treatment of choledocholithiasis two years prior, now presenting with three days of right upper quadrant abdominal pain and fevers. Vital signs demonstrated fever, tachycardia, and hypotension. Physical examination revealed scleral icterus and right upper quadrant abdominal tenderness to palpation. Laboratory findings demonstrated thrombocytopenia, acute kidney injury, transaminitis, and hyperbilirubinemia. Intravenous fluids and antibiotics were initiated. Patient was admitted to the intensive care unit for management of severe sepsis secondary to severe acute cholangitis. Blood cultures grew Klebsiella pneumoniae. Magnetic resonance cholangiopancreatography (MRCP) showed choledocholithiasis and dilated common bile duct. ERCP was performed with cannulation of the common bile duct (CBD), sphincterotomy, and removal of sludge. No stones were seen on ERCP. The patient was discharged home with oral antibiotics. The patient returned to the hospital one week after discharge with worsening right upper quadrant abdominal pain and jaundice. Repeat ERCP showed a 10-millimeter stone in the CBD, which was unable to be removed. Biliary stent was placed. Abdominal pain and jaundice improved; she was discharged with oral antibiotics. DISCUSSION: RPC occurs due to repeated hepatolithiasis, which forms strictures secondary to repeated inflammation. Hepatolithiasis has been predicted to occur secondary to malnutrition, which decreases enzymes that inhibit bilirubin deconjugation. This in turn increases unconjugated bilirubin leading to hepatolithiasis. CONCLUSIONS: RPC requires a multidisciplinary approach to determine appropriate management. DISCLOSURE: Nothing to declare. KEYWORD: Recurrent Pyogenic Cholangitis

Secondary sclerosing cholangitis: mimics of primary sclerosing cholangitis.

Sclerosing cholangitis is a chronic cholestatic disease characterized by stricturing, beading, and obliterative fibrosis of the bile ducts. Sclerosing cholangitis is considered primary (PSC) if no underlying etiology is identified or secondary (SSC) if related to another identifiable cause. In this article, we will review the clinical features, pathogenesis, diagnosis, and imaging findings of PSC and SSC, with an emphasis on features that may aid in the distinction of these entities. We will also discuss various etiologies of SSC including recurrent pyogenic cholangitis, other infectious etiologies, ischemic damage, toxic insults, and immunologic, congenital, and miscellaneous causes, highlighting the unique imaging findings and clinical context of each diagnosis.Graphical abstract

EUS-guided hepaticogastrostomy using a rendezvous technique to treat left intrahepatic duct stones in a patient with recurrent pyogenic cholangitis.

Video 1EUS-guided hepaticogastrostomy using a rendezvous technique to enable cholangioscopy and electrohydraulic lithotripsy of left intrahepatic duct stones.

Hepatolithiasis followed by recurrent cholangitis as a consequence of inadequate hepaticojejunostomy for common bile duct injury

Introduction. Hepatolithiasis (HL) is defined as gallstones present in bile ducts above the common bile duct confluence, regardless of the coexistence of gallstones in other parts of the biliary tract. HL is common among patients with recurrent pyogenic cholangitis. Chronic infection can lead to the development of malignancy. Case outline. A 65-year-old woman presented with intermittent fever, jaundice, abdominal pain, and nausea. Eighteen years previously, the patient had an open cholecystectomy due to acute cholecystitis. During the early post-operative days, the patient developed icterus. Intrahepatic biliary ductal dilatation was confirmed by abdominal ultrasound. Due to suspicion of iatrogenic common bile duct injury, the patient underwent a second operation, during which the said injury was confirmed. ?Non-Roux-en-Y? hepaticojejunostomy (HJ) was performed as a problem-resolving procedure. Despite the performed biliary bypass, the patient continued to have episodes of recurrent cholangitis over the 18 years. Given the patient?s recurrent symptoms and results of MRI consistent with HL, surgical treatment was indicated. A left hepatectomy was performed, with Roux-en-Y HJ biliary reconstruction. The post-operative course was uneventful, after which the patient has been symptom-free. Conclusion. The main purpose of treating HL is to eliminate infection which leads to recurrent cholangitis and subsequent hepatic fibrosis. Adequate solution of HL decreases the need for repeated interventions and prevents progression of the disease to cholangiocarcinoma.

S3483 Recurrent Pyogenic Cholangitis

Introduction: Recurrent pyogenic cholangitis is a chronic infective process characterized by recurrent attacks of acute bacterial cholangitis, in the setting of intrahepatic biliary ductal dilation and strictures, traditionally seen in South East Asia. Case Description/Methods: We present a 39 year old female who immigrated to the United States from Burma referred to gastroenterology clinic for abnormal liver tests and computed topography (CT) scan. Patient also reported intermittent right upper quadrant abdominal pain, fatigue and decreased appetite. Labs were significant for mildly elevated aspartame transferase (AST), alanine transferase (ALT) and alkaline phosphatase (AP). CT and magnetic resonance cholangiopancreatography (MRCP) were notable for intrahepatic biliary ductal dilation with multiple non-enhancing filling defects suggestive of stones or sludge, most pronounced in the left lobe of the liver and left hepatic lobe atrophy. Common bile duct was non-dilated without stones, sludge, wall thickening or abnormal enhancement. Liver elastography was performed which revealed cirrhosis. Patient did not have evidence of acute infection at this time but overall clinical presentation was consistent with recurrent pyogenic cholangitis. Discussion: Treatment options for recurrent pyogenic cholangitis include antibiotic therapy with signs of sepsis, removal of extrahepatic biliary stones via endoscopic retrograde cholangiopancreatography, decompression of intrahepatic biliary system via percutaneous biliary drainage and less likely surgical removal of diseased liver segment and potential liver transplantation. After discussion between an advanced endoscopist, general gastroenterologists, interventional radiologists and surgeons, she was started on ursodeoxycholic acid with plan for antibiotic therapy if evidence of acute infection. She will continue surveillance MRCP every 6 months for evaluation of hepatocellular carcinoma and cholangiocarcinoma.Figure 1.: Patient's CT image demonstrating intrahepatic biliary ductal dilation with hyperdense material concerning for biliary sludge, chronic obstruction of the left intrahepatic biliary tree and left hepatic lobe atrophy.

S2761 Recurrent Oriental Cholangiohepatitis Despite Partial Hepatic Resection

Introduction: Recurrent pyogenic cholangiohepatitis (RPC), also known as “Oriental Cholangiohepatitis”, is commonly found in people of Southeast Asian origin. RPC is characterized by recurrent cholangitis secondary to bile stasis and stone formation proximal to biliary strictures. We present a case of recurrent pyogenic cholangiohepatitis with hepatic abscess in a Vietnamese male with history of partial hepatic resection. Case Description/Methods: A 42 year old Vietnamese male with a past medical history of recurrent pyogenic cholangitis and hepatolithiasis status post hepaticojejunostomy in 2009, followed by abscess resection with culture positive Klebsiella, segment 3 resection, and biliary stenting in 2013 presented with two days of RUQ pain, fevers and jaundice. On arrival, the patient was afebrile, tachycardic and mildly hypotensive. Laboratory evaluation demonstrated alkaline phosphatase 360 IU/L, total bilirubin 14.4 mg/dL, ALT 93 IU/L, AST 113 IU/L, WBC 24,000, INR 1.3. Findings on CT scan of the abdomen included 10 cm multiloculated liver abscess in segment 4A, dilated biliary tract with small pneumobilia and dense material, and a 3.9 x 2.8 cm mass-like hyperdense region in the portal triad region visualized in arterial phase. The patient was started on broad spectrum antibiotics and underwent percutaneous abscess drainage with 350 mL of purulent material removed. MRCP the following day redemonstrated a liver abscess measuring 9.5 cm, RUQ drain in place, diffuse dilatation of the intrahepatic and extrahepatic bile ducts with multiple filling defects consistent with calculi, and a 14mm common bile duct. Cultures from the abscess grew ESBL E.coli and Provotella, and the patient was transitioned to Ertapenem with plan to continue antibiotics for four weeks. The patient was discharged home with abscess drain in place. Discussion: RPC is a recurrent syndrome of cholangitis triggered by a parasitic infection which causes biliary fibrosis leading to strictures and stone formation within intrahepatic ducts, most often on the left. Treatment includes antibiotics, stricture dilation, biliary drainage, stone removal, and liver resection. Although liver resection of the segment decreases the risk of recurrent cholangitis, abscess, and eliminates the risk of carcinoma in that segment, resection does not resolve the biliary fibrosis throughout the remaining segments leaving the patient at risk for recurrence. Liver transplant can be considered for complicated cases that do not respond to conventional therapies.Figure 1.: 10 cm multiloculated liver abscess in segment 4A.

S1574 Strictures and Stones: A Case of Recurrent Pyogenic Cholangitis

S1574 Strictures and Stones: A Case of Recurrent Pyogenic Cholangitis

Surgical management of hepatolithiasis: an institutional experience of a rare disease

Hepatolithiasis remains as a rare condition in the majority of western world countries. Although genetic, dietary and environmental factors have been associated with the disease, the exact etiology of this entity remains elusive. Several approaches; surgical and non-invasive have been well described. However, surgery remains the standard of care for definitive treatment of the disease. We sought to present the recent experience of our clinic regarding the surgical management of hepatolithiasis in the backgound of a narrative literature review. All adult patients with hepatolithiasis admitted to our center during the period 2018 to 2020 were included in the study. Patients’ demographics, comorbidities and preoperative characteristics were collected by chart review. All patients underwent open left lateral hepatectomy. Three cases with hepatolithiasis were identified. All of them were male and had been suffering from recurrent pyogenic cholangitis. All patients remained asymptomatic and disease-free through the entire follow-up, except one case that was complicated with bile leakage during the immediate postoperative period. Hepatolithiasis is currently a rare condition in the western world. Surgery is safe and effective treatment approach for refractory cases and, provided that it is performed by experts in hepatobiliary surgery, it remains imperative for the definitive treatment of the disease.

Duplication of the common bile duct manifesting as recurrent pyogenic cholangitis: A case report

BACKGROUNDDuplication of the extrahepatic bile duct (DCBD) is an extremely rare congenital anomaly of the biliary system. There are five types of DCBD according to the latest classification. Among them, Type V is characterized by single drainage of the extrahepatic bile ducts. Reports on DCBD Type V are scarce.CASE SUMMARYA 77-year-old woman presented with recurrent epigastric pain but without fever or chills. Computed tomography revealed a dilated common bile duct (CBD) that harboured multiple choledocholithiasis. Endoscopic retrograde cholangio-pancreatography (ERCP) was performed, and the stones were extracted using a Dormia basket. She was discharged without any complications; however, she visited the emergency department a day after she was discharged due to epigastric pain and fever. Laboratory findings were suggestive of cholestasis. After urgent ERCP for stone removal, magnetic resonance cholangiopancrea-tography was performed to evaluate remnant choledocholithiasis. Magnetic resonance cholangiopancreatography revealed a DCBD Type Va and remnant choledocholithiasis in the right CBD. Both CBDs were accessed, and the stones were cleared successfully during a subsequent ERCP.CONCLUSIONIn this article, we report an extremely rare case of DCBD manifesting as recurrent pyogenic cholangitis. This case highlights the importance of recognizing DCBD because stones in the unrecognized bile duct could make the patient’s prognosis critical.