Resected case of eosinophilic cholangiopathy presenting with secondary sclerosing cholangitis

Eosinophilic cholangiopathy is a rare condition characterized by eosinophilic infiltration of the biliary tract and causes sclerosing cholangitis. We report a patient with secondary sclerosing cholangitis with eosinophilic cholecystitis. A 46-year-old Japanese man was admitted to our hospital with jaundice. Computed tomography revealed dilatation of both the intrahepatic and extrahepatic bile ducts, diffuse thickening of the wall of the extrahepatic bile duct, and thickening of the gallbladder wall. Under the diagnosis of lower bile duct carcinoma, he underwent pyloruspreserving pancreatoduodenectomy and liver biopsy. On histopathological examination, conspicuous fibrosis was seen in the lower bile duct wall. In the gallbladder wall, marked eosinophilic infiltration was seen. Liver biopsy revealed mild portal fibrosis. He was diagnosed as definite eosinophilic cholecystitis with sclerosing cholangitis with unknown etiology. The possible etiology of sderosing cholangitis was consequent fibrosis from previous eosinophilic infiltration in the bile duct. The clinicopathological findings of our case and a literature review indicated that eosinophilic cholangiopathy could cause a condition mimicking primary sclerosing cholangitis （PSC）. Bile duct wall thickening in patients with eosinophilic cholangitis might be due to fibrosis of the bile duct wall. Eosinophilic cholangiopathy might be confused as PSC with eosinophilia.     

Endoscopic management of postoperative bile leaks

BACKGROUND: Significant bile leak as an uncommon complication after biliary tract surgery may constitute a serious and difficult management problem. Surgical management of biliary fistulae is associated with high morbidity and mortality. Biliary endoscopic procedures have become the treatment of choice for management of biliary Gstulae. METHODS: Ninety patients presented with bile leaks after cholecystectomy ( open cholecystectomy in 45 patients, cholecystectomy with common bile duct exploration in 20 and laparoscopic cholecystectomy in 25). The presence of bile leaks was confirmed by ERCP and the appearance of bile in percutaneous drainage of abdominal collections. Of the 90 patients with postoperative bile leaks, 18 patients had complete transaction of the common bile duct by ERCP and were subjected to bilioenteric anastomosis. In the remaining patients after cholangiography and localization of the site of bile leaks. therapeutic procedures like sphinctero-tomy, biliary stenting and nasobiliary drainage ( NBD ) were performed. If residual stones were seen in the common bile duct, sphincterotomy was followed by stone extraction using dormia basket. Nasobiliary drain or stents of 7F size were placed according to the standard techniques. The NBD was removed when bile leak stopped and closure of the fistula confirmed cholangiographically. The stents were removed after an interval of 6-8 weeks. RESULTS: Bile leaks in 72 patients occurred in the cystic duct (38 patients), the common bile duct (30 ), and the right hepatic duct (4). Of the 72 patients with post-operative bile leak, 24 had associated retained common bile duct stones and 1 had ascaris in common bile duct. All the 72 patients were subjected to therapeutic procedures including sphincterotomy with stone extraction followed by biliary stenting (24 patients), removal of ascaris and biliary stenting (1), sphincterotomy with biliary stenting (18), sphincterotomy with NBD (12), biliary stenting alone (12), and NBD alone (5). Bile leaks stopped in all patients at a median interval of 3 days (range 3-16 days) after endoscopic in- terventions. No difference was observed in efficacy and in time for the treatment of bile leak by sphincterotomy with endoprosthesis or endoprosthesis alone in patients with bile leak after surgery. CONCLUSIONS: Post-cholecystectomy bile leaks occur most commonly in the cystic duct and associated common bile duct stones are found in one-third of cases. Endoscopic therapy is safe and effective in the management of bile leaks and fistulae after surgery. Sphincterotomy with endoprosthesis or endoprosthesis alone is equally effective in the management of postoperative bile leak.     

Endoscopic retrograde cholangiopancreatography during pregnancy without radiation

AIM： To present our experience with pregnant patients who underwent endoscopic retrograde cholangiopancreatography （ERCP） without using radiation, and to evaluate the acceptability of this alternative therapeutic pathway for ERCP during pregnancy. METHODS： Between 2000 and 2008, six pregnant women underwent seven ERCP procedures. ERCP was performed under mild sedoanalgesia induced with pethidine HCI and midazolam. The bile duct was cannulated with a guidewire through the papilla. A catheter was slid over the guidewire and bile aspiration and/or visualization of the bile oozing around the guidewire was used to confirm correct cannulation. Following sphincterotomy, the bile duct was cleared by balloon sweeping. When indicated, stents were placed. Confirmation of successful biliary cannulation and stone extraction was made by laboratory, radiological and clinical improvement. Neither fluoroscopy nor spot radiography was used during the procedure. RESULTS： The mean age of the patients was 28 years （range, 21-33 years）. The mean gestational age for the fetus was 23 wk （range, 14-34 wk）. Five patients underwent ERCP because of choledocholithiasis and/or choledocholithiasis-induced acute cholangitis. In one case, a stone was extracted after precut papillotomy with a needle-knife, since the stone was impacted. One patient had ERCP because of persistent biliary fistula after hepatic hydatid disease surgery. Following sphincterotomy, scoleces were removed from the common bile duct. Two weeks later, because of the absence of fistula closure, repeat ERCP was performed and a stent was placed. The fistula was closed after stent placement. Neither post-ERCP complications nor premature birth or abortion was seen. CONCLUSION： Non-radiation ERCP in experienced hands can be performed during pregnancy. Stent placement should be considered in cases for which complete common bile duct clearance is dubious because of a lack of visualization of the biliary tree.     

A case of methicillin-resistant Staphylococcus aureus infection following bile duct stenting

AIM: To present a case of methicillin-resistant Staphylococcus aureus (MRSA) infection following bile duct stenting in a patient with malignant biliary obstruction. METHODS: A 78-year-old male patient was admitted to a community hospital with progredient painless jaundice lasting over two weeks, weight loss and sweating at night. Whether a stent should be implanted pre-operatively in jaundiced patients or whether these patients should directly undergo surgical resection, was discussed. RESULTS: ERC and a biopsy from the papilla of Vater revealed an adenocarcinoma. In addition, a 7-Ch plastic stent was placed into the common bile duct. Persistent abdominal pain, increasing jaundice, weakness and indigestion led to the transfer of the patient to our hospital. A pylorus-preserving pancreatoduodenectomy was performed. Intraoperatively, bile leaked out of the transected choledochus and the stent was found to be dislocated in the duodenum. A smear of the bile revealed an infection with MRSA, leading to post-operative isolation of the patient. CONCLUSION: As biliary stents can cause severe infection of the bile, the need for pre-operative placement of biliary stents should be carefully evaluated in each individual case.     

Chronic pancreatitis-induced compressed relative stenosis of the distal Common bile duct

BACKGROUND: Fibrosis and enlargement of chronic pancreatitis (CP) can cause biliary stenosis, in which fixed circular stenosis is the common type, by compressing the intrapancreatic portion of the common bile duct. Another type of distal bile duct stenosis is compressed relative stenosis, in which the common bile duct walks along the back of the pancreas partially. METHODS: Thirty patients with dilation of the proximal common bile duct (diameter≥15mm) caused by CP underwent overall and systemic exploratory operation at our hospital. All of the patients were followed up for 3.5 to 15 years, averaging 8.5 years. RESULTS: The intrapancreatic portion of the common bile duct was found to walk along the back of the pancreas, and its anterior wall was compressed flat by enlarged pancreas, but the posterior wall showed a good flexibility because there was no pancreas covering. Bake's dilators bigger than No. 6 (diameter≥4mm) and No. 14 urinary catheter could pass through the distal common bile duct after the posterior wall was separated. Roux-en-Y choledochojejunostomy was performed for 4 patients, and T-tube drainage was carried out for the remaining 26 patients. All of the patients were followed up but 2 were lost. Only 2 patients underwent choledochojejunostomy 3 years after T-tube drainage because of repeated acute pancreatitis attack, and others were normal. CONCLUSIONS: Compressed relative stenosis of the distal common bile caused by CP is a clinical sign, and its diagnosis mainly depends on surgical findings. Most patients can be treated by separating the posterior wall of the pancreas and T-tube drainage as well, but to patients with recurrent CP, choledochojejunostomy may be a feasible alternative.     

Biliary tuberculosis causing cicatricial stenosis after oral anti-tuberculosis therapy

A 36-year-old Philippine woman presented with dark urine and yellow sclera. Endoscopic retrograde cholangiopancreatography (ERCP) confirmed dilatation of the intrahepatic bile ducts and also showed an irregular stricture of the common hepatic duct at the liver hilum. Histological examination of biopsies from the bile duct revealed epithelioid cell granulomas and caseous necrosis. Tubercle bacilli were then detected on polymerase chain reaction (PCR) testing of the bile, giving the diagnosis of biliary tuberculosis. Although microbiological cure was confirmed, the patient developed cicatricial stenosis of the hepatic duct. She underwent repeated treatments with endoscopic biliary drainage (EBD) tubes and percutaneous transhepatic biliary drainage (PTBD) tubes, and the stenosis was corrected after 6 years. We present a case of tuberculous biliary stricture, a condition that requires careful differentiation from the more common malignancies and needs long-term follow-up due to the risk of posttreatment cicatricial stenosis, although it is rare.     

Pseudoaneurysm following laparoscopic cholecystectomy

BACKGROUND:Laparoscopic cholecystectomy(LC)is the operation of choice for removal of the gallbladder. Unrecognized bile duct injuries present with biliary peritonitis and systemic sepsis.Bile has been shown to cause damage to the vascular wall and therefore delay the healing of injured arteries leading to pseudoaneurysm formation.Failure to deal with bile leak and secondary infection may result in pseudoaneurysm formation. This study was to report the incidence and outcomes of pseudoaneurysm in patients with bile leak following LC referred to our hospital. METHODS:A retrospective analysis of our prospectively maintained liver database using pseudoaneurysm, bile leak and bile duct injury following laparoscopic cholecystectomy from January 2000 to December 2005 was performed. RESULTS:A total of 86 cases were referred with bile duct injury and bile leak following LC and of these,4 patients (4.5%)developed hepatic artery pseudoaneurysm(HAP) presenting with haemobilia in 3 and massive intra- abdominal bleed in 1.Selective visceral angiography confirmed pseudoaneurysm of the right hepatic artery in 2 cases,cystic artery stump in one and an intact but ectatic hepatic artery with surgical clips closely applied to the right hepatic artery at the origin of the cystic artery in the fourth case.Effective hemostasis was achieved in 3 patients with coil embolization and the fourth patient required emergency laparotomy for severe bleeding and hemodynamic instability due to a ruptured right hepatic artery.Of the 3 patients treated with coil embolization, 2 developed late strictures of the common hepatic duct. . (CHD)requiring hepatico-jejunostomy and one developed a stricture of left hepatic duct.All the 4 patients are alive at a median follow up of 17 months(range 1 to 65)with normal liver function tests. CONCLUSIONS:HAP is a rare and potentially life- threatening complication of LC.Biloma and subsequent infection are reported to be associated with pseudoaneurysm formation.Late duct stricture is common either due to unrecognized injury at LC or secondary to ischemia after embolization.     

Common bile duct injury by fibrin glue: Report of a rare complication

Fibrin glue is widely used in clinical practice and plays an important role in reducing postoperative complications.We report a case of a 65-year-old man, whose common bile duct was injured by fibrin glue, with a history of failed laparoscopic cholecystectomy and open operation for uncontrolled laparoscopic bleeding.In view of thepersistent liver dysfunction, xanthochromia and skin itching, the patient was admitted to us for further management.Ultrasound, computed tomography, and magnetic resonance cholangiopancreatography(MRCP) revealed multiple stones in the common bile duct, and liver function tests confirmed the presence of obstructive jaundice and liver damage.Endoscopic retrograde cholangiopancreatography was unsuccessfully performed to remove choledocholithiasis, but a small amount of tissue was removed and pathologically confirmed as calcified biliary mucosa.This was followed by open surgery for suspicious cholangiocarcinoma.There was no evidence of cholangiocarcinoma, but the common bile duct wall had a defect of 8 mm × 10 mm at Calot’s triangle.A hard, grid-like foreign body was removed, which proved to be solid fibrin glue.Subsequently, the residual choledocholithiasis was removed by a choledochoscopic procedure, and the common bile duct deletion was repaired by liver round ligament with T-tube drainage.Six months later, endoscopy was performed through the T-tube fistula and showed a well-repaired bile duct wall.Eight months later, MRCP confirmed no bile duct stenosis.A review of reported cases showed that fibrin glue is widely used in surgery, but it can also cause organ damage.Its mechanism may be related to discharge reactions.     

Duct-to-duct biliary reconstruction after radical resection of Bismuth Ⅲ a hilar cholangiocarcinoma

At present, radical resection remains the only effective treatment for patients with hilar cholangiocarcinoma. The surgical approach for R0 resection of hilar cholangiocarcinoma is complex and diverse, but for the biliary reconstruction after resection, almost all surgeons use Roux-en-Y hepaticojejunostomy. A viable alternative to Roux-en-Y reconstruction after radical resection of hilar cholangiocarcinoma has not yet been proposed. We report a case of performing duct-to-duct biliary reconstruction after radical resection of Bismuth Ⅲa hilar cholangiocarcinoma. End-to-end anastomosis between the left hepatic duct and the distal common bile duct was used for the biliary reconstruction, and a singlelayer continuous suture was performed along the bile duct using 5-0 prolene. The patient was discharged favorably without biliary fistula 2 wk later. Evidence for tumor recurrence was not found after an 18 mo follow- up. Performing bile duct end-to-end anastomosis in hilar cholangiocarcinoma can simplify the complex digestive tract reconstruction process.     

Eosinophilic gastroenteritis presenting as small bowel obstruction： A case report and review of the literature

Eosinophilic gastroenteritis is a rare disease of unknown etiology. It is characterized by eosinophilic infiltration of the bowel wall to a variable depth and symptoms associated with gastrointestinal tract. Recently, the authors experienced a case of eosinophilic gastroenteritis presenting as small bowel obstruction. A 51-year old woman was admitted to our hospital complaining of abdominal pain and vomiting. Physical examination revealed a distended abdomen with diffuse tenderness. Complete blood count showed mild leukocytosis without eosinophilia. Computed tomography confirmed a dilatation of the small intestine with ascites. An emergency laparotomy was performed for a diagnosis of peritonitis due to intestinal obstruction. Segmental resection of the ileum and end to end anastomosis were performed. Histologically, there was a dense infiltration of eosinophils throughout the entire thickness of ileal wall and eosinophilic enteritis was diagnosed. The patient recovered well, and was free from gastrointestinal symptoms at the time when we reported her disease.     

Eosinophilic cholangitis: A case report of diagnostically challenging eosinophilic infiltrative biliary obstruction

BACKGROUND Evaluation of biliary strictures primarily focuses on ruling out malignancy in older age groups. With endoscopic tools such as endoscopic ultrasound(EUS)and cholangioscopy, improved biliary visualization has enhanced the investigation of intraluminal biliary lesions and provided modalities for targeted biopsies. Benign biliary strictures, however, may pose a diagnostic dilemma.CASE SUMMARY A 71-year-old female with past medial history of hypothyroidism presenting for abnormal biliary imaging. Patient's previous evaluation was concerning for common bile duct dilation with cholelithiasis, for which she underwent a cholecystectomy. Due to persistent symptoms and worsening liver function tests,she presented to our institution for further workup. Subsequently, the patient underwent an EUS and multiple ERCP's with cholangioscopy; biliary biopsies revealed no evidence of malignancy but concerning for prominent eosinophilic infiltration. After further review of multiple pathology specimens and the benign clinical course, we diagnosed the patient with eosinophilic cholangitis.CONCLUSION Eosinophilic cholangitis is a rare disease and can present as a challenging case diagnostically. This case raises the potential utility of quantitative eosinophilic infiltration reporting in creating an objective diagnostic metric for eosinophilic cholangitis.     

Diagnostic usefulness of precise examinations with intraductal ultrasonography,peroral cholangioscopy and laparoscopy of immunoglobulin G4‐related sclerosing cholangitis

Herein, a case of immunoglobulin G4 (IgG4)‐related sclerosing cholangitis is reported. IgG4 was diagnosed based on observations from peroral cholangioscopy and laparoscopy, and these methods are proposed for definitive and precise diagnosis of this disease. A 76‐year‐old male patient with inguinal Paget's disease had intrahepatic bile duct dilatations detected with computed tomography at his periodic check‐up. Magnetic resonance cholangiography showed stenosis of the upper common bile duct and poststenotic dilatation of left intrahepatic bile ducts. The portal tract and bilateral intrahepatic bile ducts were surrounded by a low‐density area, facing a tumor‐like lesion at segment 2. Cytological examinations of the stenotic and dilated lesions revealed no cellular atypia. Histological examination of the tumor showed normal liver tissue with infiltration of lymphocytes, indicating an inflammatory pseudotumor. Peroral cholangioscopy excluded the possibility of biliary cancer and indicated that the stenotic legion was of submucosal, not mucosal, origin. Laparoscopic observations showed discoloration with wide yellowish‐white lobular markings and wide depressed lesions at segments 2 and 7. Liver histology showed mild cholangitis with infiltration of IgG4‐positive plasma cells around the bile ducts. Serum IgG4 levels were elevated. From these findings, the patient was diagnosed with IgG4‐related sclerosing cholangitis. After treatment with prednisolone, blood liver enzymes and IgG4 rapidly normalized, bile duct dilatations improved, and the hepatic pseudotumor disappeared. The cholangitis did not recur. In this case, biliary cancer was ruled out by observation with peroral cholangioscopy, and the spread of cholangitis in the liver periphery was verified with laparoscopy; this information could not be obtained with other modalities.     

Eosinophilic cholangitis with obstructive jaundice mimicking bile duct carcinoma

A 55-year-old male who presented with obstructive jaundice and radiographically documented extrahepatic biliary tract obstruction is reported. Eosinophilic infiltration of the gallbladder, common bile duct, intrahepatic bile ducts, and bone marrow was observed. Eosinophilic cholangitis, a rare inflammatory condition that clinically resembles a biliary malignancy, should be taken into consideration in the differential diagnosis in the evaluation of presumed neoplasm of the bile ducts.     

Eosinophilic cholangitis: a case of 'malignant masquerade'

BackgroundA variety of causes of inflammatory bile duct stricture can masquerade as hilar cholangiocarcinoma. Eosinophilic cholangitis is a further example.Case outlineA 50-year-old woman with epigastric pain and deranged liver function was found to have a stricture of the common hepatic duct on ERCP with an associated mass on MRl.The lesion was excised with reconstruction of the right and left hepatic ducts, and the patient recovered well. Histopathological examination of the resected gallbladder and bile duct showed diffuse inflammation with a predominant eosinophil infiltrate.The presence of Candida albicans in the bile duct lumen can probably be attributed to the preoperative biliary stent.There was a modest postoperative rise in peripheral eosinophil count.DiscussionA literature search reveals only six previous cases of eosinophilic cholangitis, but similar infiltrates have also been seen in occasional cholecystectomy specimens. As the present patient did not have gallstones, the aetiology remains unclear. Peripheral eosinophilia is an unreliable clue to the diagnosis, which is usually likely to escape detection until the biliary stricture has been resected.     

Eosinophilic cholangitis coexisted with idiopathic thrombocytopenic purpura: Report of a case

Eosinophilic cholangitis is a rare disease of which only 31 cases have been reported. Eosinophilic infiltration causes stricture of the bile duct diffusely or locally, and the imaging of eosinophilic cholangitis resembles primary sclerosing cholangitis or cancer of the bile tract. For eosinophilic cholangitis, treatment with steroid is effective and the prognosis is good. Therefore, its accurate diagnosis is very important. Here, we describe a patient with eosinophilic cholangitis who was also diagnosed with idiopathic thrombocytopenic purpura (ITP). He was treated for ITP using prednisolone, the unexpected sudden interruption of which caused severe deterioration of eosinophilic cholangitis and acute cholecystitis. Cholecystectomy and choledochojejunostomy were performed, and the addition of treatment by prednisolone resulted in a good clinical course. This is the first report on eosinophilic cholangitis coexisting with ITP.     

Death receptor 5 mediated-apoptosis contributes to cholestatic liver disease

Chronic cholestasis often results in premature death from liver failure with fibrosis; however, the molecular mechanisms contributing to biliary cirrhosis are not demonstrated. In this article, we show that the death signal mediated by TNF-related apoptosis-inducing ligand (TRAIL) receptor 2/death receptor 5 (DR5) may be a key regulator of cholestatic liver injury. Agonistic anti-DR5 monoclonal antibody treatment triggered cholangiocyte apoptosis, and subsequently induced cholangitis and cholestatic liver injury in a mouse strain-specific manner. TRAIL- or DR5-deficient mice were relatively resistant to common bile duct ligation-induced cholestasis, and common bile duct ligation augmented DR5 expression on cholangiocytes, sensitizing mice to DR5-mediated cholangitis. Notably, anti-DR5 monoclonal antibody-induced cholangitis exhibited the typical histological appearance, reminiscent of human primary sclerosing cholangitis. Human cholangiocytes constitutively expressed DR5, and TRAIL expression and apoptosis were significantly elevated in cholangiocytes of human primary sclerosing cholangitis and primary biliary cirrhosis patients. Thus, TRAIL/DR5-mediated apoptosis may substantially contribute to chronic cholestatic disease, particularly primary sclerosing cholangitis.     

Primary sclerosing cholangitis in which differential diagnosis from gallbladder carcinoma was difficult

We report a case of localized primary sclerosing cholangitis (PSC) which was difficult to distinguish from gallbladder carcinoma. A 75-year-old woman with elevated serum bilirubin was hospitalized and underwent endoscopic nasobiliary drainage (ENBD). There was no history of diseases such as gallbladder stone, pancreatitis, or ulcerative colitis. Cholangiography through the ENBD tube showed localized stenosis of the common bile duct; the gallbladder could not be seen. Angiography showed no encasement of the hepatic artery. Ultrasonography showed a tumor in the cystic duct, and the tumor had invaded the gallbladder and common bile duct. We diagnosed gallbladder carcinoma on radioimaging, and performed an S4aS5 subsegmentectomy of the liver and resection of the extrahepatic biliary tree. Pathologically, no malignant cells were detected, and fibrosis around bile ducts and infiltration of inflammatory cells into hepatic tissue were found. It is well known that PSC is sometimes difficult to differentially diagnose from cholangiocarcinoma. Our case is of high interest because ultrasonography showed findings suggestive of gallbladder carcinoma. It is therefore necessary to keep the possibility of PSC in mind for the diagnosis and treatment of such localized biliary stenosis.     

Follicular cholangitis mimicking cholangiocarcinoma treated with right hepatectomy: A case report and review of published works

Follicular cholangitis is a new, rare disease that causes severe biliary stricture. We herein describe the findings from a resected case of follicular cholangitis, suggesting a distinct disease entity that causes benign biliary stricture. A 60‐year‐old man who was referred to our hospital due to elevated γ‐glutamyl transpeptidase levels and dilatation of the B8 bile duct. Although bile juice cytology and bile duct brushing cytology showed no malignancy, the dilatation was progressive. Therefore, right hepatectomy combined with caudate lobectomy was carried out on suspicion of intrahepatic cholangiocarcinoma. The wall of the resected bile duct was markedly thickened due to severe fibrosis under the mucosal layer. Histology of the mucosal epithelium indicated no malignancy. Infiltration of plasma cells characterized by remarkable formation of lymphoid follicles with germinal centers was observed around the bile ducts. The patient was diagnosed with follicular cholangitis based on histological findings. We thus observed a rare case of follicular cholangitis. This case and review of published reports suggest that, despite its rarity, follicular cholangitis should be considered at the differential diagnosis of biliary stricture. This case report could contribute to a better understanding of how to address this disease.     

Cytomegalovirus cholangitis and pancreatitis in an immunocompetent patient

Cholangitis and pancreatitis associated with cytomegalovirus (CMV) infection in an immunocompetent patient is reported. Endoscopic retrograde cholangiography performed on a 55-year-old man for evaluation of the cause of jaundice and liver dysfunction revealed a distal focal irregular narrowing of the common bile duct. Microscopic findings of the resected specimen showed chronic cholangitis and CMV pancreatitis. Immunohistochemistry disclosed that epithelial cells in the inflamed bile duct were positive for CMV antigen, which was compatible with CMV cholangitis. Inflammation of the biliary tract or pancreas by CMV has been commonly reported as a complication in immunocompromised patients. Our report appears to be a rare case, but suggests that CMV cholangitis or pancreatitis should be considered in the differential diagnoses of common bile duct stenosis or pancreatitis even in immunocompetent individuals.