IgG4-associated ampullitis and cholangiopathy in Crohn's disease.

Inflammatory bowel disease (IBD) is reported to be associated with autoimmune pancreatitis and IgG4-related sclerosing disease. We report a case of a 28 year old African American male with a long history of upper gastrointestinal tract Crohn's disease (CD) with multiple surgeries who developed medically refractory disease with small bowel obstruction. He had abnormal liver function tests with imaging evidence of chronic pancreatitis and ampullary inflammatory process. He underwent Whipple's procedure. Histopathological evaluation of surgical specimens of the ampulla and distal common bile duct showed accumulation of IgG4-positive plasma cells in the lamina propria. Preoperative endoscopic biopsies also showed chronic active enteritis involving the duodenum and jejunum with increased IgG4-expressing plasma cell infiltration. His serum IgG4 was 164 mg/dL. The association of IgG4-expressing plasma cell accumulation in the gastrointestinal tract with IBD in patients with hepatobiliary manifestation may have pathogenetic, diagnostic and therapeutic implications.

Effects in the control of edema of the papilla of Vater by epinephrine saline irrigation after endoscopic retrograde cholangiopancreatography in an endoscopy center in Japan, 2003 to 2007: exploratory retrospective analysis to evaluate the characteristics of eligible patients with a focus on serum amylase levels.

OBJECTIVE: To evaluate the effects of localized irrigation with epinephrine saline after endoscopic retrograde cholangiopancreatography (ERCP). PATIENTS AND METHODS: One hundred and fourteen patients who underwent ERCP in our institute were treated with or without irrigation using epinephrine diluted in saline after ERCP to prevent post-ERCP pancreatitis. The serum amylase levels, white blood cell counts, and urine amylase levels were measured at 24 and 48 hours after ERCP. RESULTS: The treatment resulted in improvements in all items. A univariate analysis of the explanatory variables between the treatment and untreated groups revealed the treatment to be effective, but not statistically significant. Gender and cannulation of the pancreatic duct were the only variables with significant partial regression coefficients in the multiple regression model with all explanatory variables (p=0.045). When a stratified analysis was conducted using gender as a moderator variable, the treatment became a significant preventive factor (p=0.038), and cannulation of the pancreatic duct was a significant risk factor (p=0.027) in female patients. CONCLUSION: We suggest that irrigating with epinephrine saline into the papilla may be effective for preventing pancreatitis in female patients who received ERCP with cannulation of their pancreatic duct.

Markedly elevated serum CA 19-9 levels in association with a benign biliary stricture due to primary sclerosing cholangitis.

We report on the case of a 55-year-old man with long-standing ulcerative colitis who developed jaundice. This led to a diagnosis of primary sclerosing cholangitis being made, with a dominant stricture in the common bile duct. Serum CA 19-9 was initially markedly raised at 26,321 U/mL but fell promptly into the normal range after stenting of the stricture. Long-term follow up of this patient has failed to show evidence of cholangiocarcinoma. We conclude that serum CA 19-9 levels need to be assessed in the clinical context of biliary obstruction and should ideally be measured after relief of that obstruction, as it may be falsely elevated due to benign biliary strictures.

Spontaneous perforation of the common bile duct with eosinophilia in an 18-month-old girl: a case report and review of literature.

BACKGROUND: Spontaneous perforation of the common bile duct is a rare but important cause of jaundice in infancy, requiring prompt diagnosis and surgical intervention. First described in 1932, fewer than 150 cases have been reported to date. CASE REPORT: The report concerns a 18-month-old girl who developed mild jaundice, abdominal distension, and constipation three days prior to admission. On admission, ascites and hepatomegaly were detected and the complete blood count was normal, but on the 3rd, 4th, and 5th days of admission, the blood cell count revealed elevation of the eosinophil count to 16% of total WBC. Abdominal sonography showed a moderate amount of ascitic fluid and paracentesis demonstrated biliary ascites. Hepatobiliary scintigraphy suggested leakage of bile to the abdominal cavity. Exploratory laparotomy revealed clear bilious ascitic fluid and a ruptured common bile duct at the anterior wall of the junction of the cystic duct and the common bile duct. Three days after T-tube insertion and closure of the perforation, the eosinophil count returned normal. CONCLUSIONS: Perforation of the bile duct may be considered as one of the differential diagnoses of eosinophilia. This point makes our case a unique study, worthy of being reported.

Long-term outcome of endoscopic metallic stenting for benign biliary stenosis associated with chronic pancreatitis.

AIM: Endoscopic metal stenting (EMS) offers good results in short to medium term follow-up for bile duct stenosis associated with chronic pancreatitis (CP); however, longer follow-up is needed to determine if EMS has the potential to become the treatment of first choice. METHODS: EMS was performed in eight patients with severe common bile duct stenosis due to CP. After the resolution of cholestasis by endoscopic naso-biliary drainage three patients were subjected to EMS while, the other five underwent EMS following plastic tube stenting. The patients were followed up for more than 5 years through periodical laboratory tests and imaging techniques. RESULTS: EMS was successfully performed in all the patients. Two patients died due to causes unrelated to the procedure: one with an acute myocardial infarction and the other with maxillary carcinoma at 2.8 and 5.5 years after EMS, respectively. One patient died with cholangitis because of EMS clogging 3.6 years after EMS. None of these three patients had showed symptoms of cholestasis during the follow-up period. Two patients developed choledocholithiasis and two suffered from duodenal ulcers due to dislodgement of the stent between 4.8 and 7.3 years after stenting; however, they were successfully treated endoscopically. Thus, five of eight patients are alive at present after a mean follow-up period of 7.4 years. CONCLUSION: EMS is evidently one of the very promising treatment options for bile duct stenosis associated with CP, provided the patients are closely followed up; thus setting a system for their prompt management on emergency is desirable.

[Fascioliasis of the common bile duct: endoscopic ultrasonographic diagnosis and endoscopic sphincterotomy]. / Distomatose de la voie biliaire principale: diagnostic écho- endoscopique et traitement par sphinctérotomie endoscopique.

Two cases of biliary fasciolasis are reported. The patients presented with biliary pain and/or acute pancreatitis. Pre-operative ultrasound endoscopy showed main bile duct dilation and linear elongated echogenic structures in the common bile duct lumen. Endoscopic retrograde cholangiography and endoscopic sphincterotomy were performed. Parasites were endoscopically removed resulting in disappearance of symptoms and biological abnormalities. Serological tests and pathological examination confirmed the presence of Fasciola hepatica. During follow-up, stool examination failed to show any Fasciola hepatica eggs, and in one case, serology became negative. This report emphasizes the value of ultrasound endoscopy in the diagnosis of unsuspected biliary fasciolasis. This report also confirms the therapeutic role of endoscopic sphincterotomy in patients with obstructive biliary fasciolasis.

Adenomyomatosis of the papilla of Vater: a case illustrating diagnostic difficulties.

AIMS: To describe the extremely rare case of an adenomyoma of the papilla of Vater. CASE REPORT: A 42-year-old woman was hospitalized for epigastralgia and high fever. The clinical presentation and endoscopic, biochemical, and radiologic findings led to the diagnostic impression of a dysfunction of the papilla of Vater. The patient was treated successfully by laparotomy and duodenotomy, incorporating cholangiomanometry and cholangiography. Intraoperative frozen-section examination of a transduodenal papillectomy specimen led to the diagnosis of adenomyomatosis of the papilla. The patient is doing well 38 months postoperatively. CONCLUSION: Such a combined approach to intraoperative diagnosis was important to avoid excessive surgery for a benign periampullary disease.

Hybrid classification of sphincter of Oddi dysfunction based on simplified Milwaukee criteria: effect of marginal serum liver and pancreas test elevations.

To date, when using the Milwaukee classification for sphincter of Oddi dysfunction (SOD), one cannot accurately classify patients with marginal elevations in laboratory tests; ie, < 1.5 x the upper limit of normal (ULN). Since subsequent treatment may depend on how they are classified, we sought to determine whether these patients should be considered as type II or type III. Between January 1993 and October 1996, 113 consecutive patients (82 females and 31 males; ages 12-87 years) without prior sphincterotomy were referred to consider a diagnosis of SOD type II or III. SOD II patients had pancreaticobiliary-type pain and laboratory elevations >1.5 x ULN or dilated ducts, while SOD III patients had pain only. Hybrid patients had pain and marginal laboratory elevations <1.5 x ULN, with normal duct diameters. Drainage times, frequency, duration, and propagation were not assessed. Sphincter of Oddi manometry (SOM) was performed in each case, and the frequency of abnormal biliary and/or pancreatic basal sphincter pressure was compared, with respect to type II, III, and hybrid SOD. Successful SOM was obtained in 113/114 patients: Abnormal basal sphincter pressure was found in 65, 89, and 43% of type II, hybrid, and type III SOD, respectively. We found no statistical difference between type II and hybrid patients. In contrast, there was statistical difference between types II and III patients and between type III and hybrid patients. In conclusion, there was no significant difference in the frequency of elevated basal sphincter pressure in SOD type II versus hybrid, and thus they should be considered as one group.

The effect of lethal endotoxaemia on the liver glycogen metabolism in guineapigs with common bile duct obstruction.

The effect of hepatocellular trauma due to bile reflux and endotoxaemia on liver glycogen metabolism was studied in guineapigs with common bile duct obstruction. Considerable drops in basal glycogen levels of hepatocytes from bile duct ligated (BDL) animals were recorded in comparison with the sham operated (SHAM) ones. However, the regurgitation of bile did not affect the basal blood glucose concentrations of BDL guineapigs. The circulating glucose was consumed, mainly reflecting the level of energy requirement of the peripheral tissues in the endotoxaemic SHAM pair-fed animals and the BDL group. The hepatic glycogen stores failed to prevent the SHAM group from becoming hypoglycaemic at the end of the eighth hour after endotoxin administration. Enhancement in glucose consumption and diminished liver glycogen indicated the necessity of glucose intake in the early phase of extrahepatic bile duct obstruction. It was concluded that both endogenous and exogenous glucose have limited value in improving energy metabolism in lethal endotoxaemia following bile duct obstruction.

Omitting side-holes in biliary stents does not improve drainage of the obstructed biliary system: a prospective randomized trial.

Sludge, which occludes biliary stents, forms mainly around the side-holes of such stents. It has been reported that omitting the side-holes results in less sludge formation and theoretically improves drainage. To compare the clinical efficacy of biliary stents with and without side-holes, we randomized patients with malignant or benign strictures to receive 10FG polyethylene stents either with side-holes (SH) or without side-holes (NSH). The patients were seen at 4, 12, 20, and 28 weeks after stenting for symptom evaluation and serum liver enzyme and bilirubin assays. The stents were replaced only when clinical symptoms of cholangitis developed. Each group included 35 patients. The mean age of patients in the SH group was 68 years, and the ratio of men to women was 1:1.3. In the NSH group, the mean age of patients was 67 years, and the ratio of men to women was 1:1.4. Eight patients with SH stents and eight with NSH stents died before the stents were removed; two NSH stents migrated into the duodenum. During a mean follow-up period of 8.1 weeks (range, 1.1 to 28 weeks for the SH group and 0.6 to 28 weeks for the NSH group), 18 stents were found to be occluded in the SH group and 17 in the NSH group. The median time before total occlusion was 7.8 weeks (range, 2.6 to 28) for SH stents and 7.9 weeks (range, 0.6 to 28) for NSH stents (p > 0.1, NS). The occluded stents removed from these patients were freeze-dried and weighed to quantitate the sludge blocking the stent lumen.(ABSTRACT TRUNCATED AT 250 WORDS)

Increased neutrophil function induced by bile duct ligation in a rat model.

Neutrophil function was studied in rats with common bile duct ligation. Superoxide production stimulated by phorbol myristate acetate, opsonized zymosan or formyl-methionyl-leucyl-phenylalanine; phagocytosis; and chemotaxis were significantly greater in neutrophils from rats with common bile duct ligation than in sham-operated control rats. Enhanced neutrophil activity was observed within 12 hr of bile duct ligation; it remained increased during the 15-day study. Preincubation of neutrophils from control rats with sera of rats with common bile duct ligation did not increase superoxide generation. This suggests that the high superoxide production observed in neutrophils of rats with common bile duct ligation was not an immediate effect of the serum. Neutrophils of rats with portal vein ligation exhibited normal activity, indicating that portal systemic shunting per se is not the underlying mechanism for increased activity. The elevated levels of AST and alkaline phosphatase, indicating liver damage, that appeared within 12 hr of bile duct ligation correlated with the increased superoxide generation.

[Elevation of serum apo E-rich HDL concentration in a patient with cholestatic liver disease].

A remarkable elevation of serum HDL cholesterol concentration (165mg/dl) was found in a 42-year-old Japanese male with chronic pancreatitis who had been cholestatic for several years. An abnormal slow alpha-migrating lipoprotein, larger in particle size and more enriched with cholesteryl ester and apo E than normal HDL, was found in the patient's plasma. Quantitative determination of apo E-rich HDL revealed a striking increase of this lipoprotein in plasma. After choledocho-jejunostomy, a prompt and remarkable decrease of plasma apo E-rich HDL was observed, indicating a direct contribution of cholestasis to the accumulation of apo E-rich HDL in plasma in this patient.

Bleeding after endoscopic sphincterotomy as an underestimated entity.

Hemorrhage is the most frequent complication of endoscopic sphincterotomy, with a reported incidence of 2 to 9 per cent. Previous reports have generally defined this complication clinically, leaving the issue of occult bleeding after sphincterotomy essentially unaddressed. Seventy-five serial sphincterotomies were reviewed to further assess this complication. Nine patients had clinically evident hemorrhage and 27 patients had occult bleeding manifested only by a decrement in hematologic parameters, for a total postsphincterotomy bleeding rate of 48 per cent. No statistically significant risk factors for bleeding were delineated. Endoscopically recognized bleeding at the time of the sphincterotomy was 47 per cent sensitive and 85 per cent specific in predicting postprocedural bleeding. Significant delayed hemorrhage was manifest in three patients, one of whom had clinically occult bleeding. We conclude that bleeding complicates endoscopic sphincterotomy much more frequently than previously reported, although often in a clinically occult manner. Significant delayed bleeding can occur, and may not be clinically apparent. Bleeding recognized endoscopically at the time of sphincterotomy is an insensitive but relatively specific predictor of postprocedural bleeding. As use of endoscopic sphincterotomy increases, careful surveillance for hemorrhagic complications, as well as efforts to identify factors predisposing to the same, will be of increasing importance.

[Is hyperbilirubinemia a risk factor in biliary surgery?]. / L'iperbilirubinemia è un fattore di rischio in chirurgia biliare?

The Authors, by means of a retrospective analysis of the patients operated on for biliary tract diseases (obstructive or not), evaluated the role of the serum bilirubin level as risk factor in this type of surgery. They show data about 474 patients divided into two groups on the basis of serum bilirubin level, examine mortality and morbidity rates also as a function of the age and analyze the results with the chi-square test. The Authors conclude that the serum bilirubin level have no statistically significant influence on the mortality and the morbidity rates, while elderly age results to be a risk factor.

[Use of charcoal enterosorbents in the complex treatment of patients with obstructive jaundice. Report II. Experimental study]. / Primenenie uglerodnykh énterosorbentov v kompleksnom lechenii bo'lnykh s obturatsionnoi zheltukhoi. Soobshchenie II. Eksperimenta'lnoe issledovanie.

In the experiments on rats with modelled bile duct obstruction and hepatic failure, the pronounced detoxicative value of the SKN P-1 and SKN P-2 carbonic sorbents is proved.

Dysfibrinogenemia in obstructive liver disease.

Acquired dysfibrinogenemia was documented in a 4-year-old child with obstructive jaundice of 1-month duration, secondary to a choledochal cyst involving the distal common bile duct. It was characterized by decreased thrombin coagulable protein with elevated immunoassayable fibrinogen resulting in abnormal thrombin and reptilase times. The liver morphology was compatible with extrahepatic obstruction, without evidence of cirrhosis or hepatocyte abnormality. All the coagulation abnormalities promptly resolved after surgical correction of the obstruction. Dysfibrinogenemia has been associated with serious liver disease in adults, including tumors, chronic active hepatitis, and cirrhosis, but never with isolated obstructive jaundice. This report documents a case of acquired dysfibrinogenemia due to extra-hepatic biliary obstruction and also emphasizes the importance of the consideration of this disorder in coagulation abnormalities associated with hepatobiliary disease.