[Neuroendocrine Tumor of the Ampulla of Vater and Gastrointestinal Stromal Tumor of the Duodenum in a Patient with Von Recklinghausen's Disease].

A 57-year-old woman with von Recklinghausen's disease presented with epigastralgia. Gastroduodenoscopy revealed swelling of the ampulla of Vater in the ventral and caudal direction, forming a hard, elastic mass. She was diagnosed with a tumor of the ampulla of Vater, and a subtotal stomach-preserving pancreaticoduodenectomy and D2 lymph node dissection were performed. The isolated specimen showed an intra-ampullary tumor of the ampulla of Vater and a submucosal tumor in the descending duodenum, which were diagnosed as a somatostatin-producing neuroendocrine tumor and gastrointestinal stromal tumor, respectively, on pathological examination. We believe that the neuroendocrine tumor of the ampulla of Vater and gastrointestinal stromal tumor of the duodenum are common gastrointestinal lesions in von Recklinghausen's disease.

Cytomegalovirus infection of the major duodenal papilla in a renal allograft recipient with severe biliary obstruction and acalculous cholecystitis.

Cytomegalovirus (CMV) can cause severe infections with serious consequences in renal transplant recipients. Disseminated CMV infections can affect almost every organ, but obstructive cholestasis and cholangitis, as a consequence of a CMV-induced papillitis, is extremely rare. We are reporting a rare case of obstructive cholestasis and cholecystitis due to CMV-related inflammation of the major duodenal papilla in a 60-year-old woman 3 months after renal transplantation. In addition, the patient suffered from a disseminated CMV infection with ulcerative esophagitis and gastritis. Because of the severe CMV infection, failure of the renal graft occurred. Obstructive cholestasis was resolved through internal stenting, and the progressive cholecystitis necessitated an emergency cholecystectomy. Following antiviral therapy with ganciclovir, the gastrointestinal ulcerations regressed and renal function was restored. Diagnosis of the CMV-related disease was established only in tissue samples, whereas standard serologic tests had failed.

Frey procedure for the treatment of chronic pancreatitis associated with common bile duct stricture.

BACKGROUND: The Frey procedure (FP) is the treatment of choice for symptomatic chronic pancreatitis (CP). In cases of biliary stricture, biliary derivation can be performed by choledochoduodenostomy, Roux-en-Y choledochojejunostomy or, more recently, reinsertion of the common bile duct (CBD) into the resection cavity. The objective of the present study was to evaluate the outcomes associated with each of these three types of biliary derivation. METHODS: We retrospectively analyzed demographic, CP-related, surgical and follow-up data for patients having undergone FP for CP with biliary derivation between 2004 and 2012 in our university medical center. The primary efficacy endpoint was the rate of CBD stricture recurrence. The secondary endpoints were surgical parameters, postoperative complications, postoperative follow-up and the presence of risk factors for secondary CBD stricture. RESULTS: Eighty patients underwent surgery for CP during the study period. Of these, 15 patients received biliary derivation with the FP. Eight of the FPs (53.3%) were combined with choledochoduodenostomy, 4 (26.7%) with choledochojejunostomy and 3 (20.0%) with reinsertion of the CBD into the resection cavity. The mean operating time was 390 minutes. Eleven complications (73.3%) were recorded, including one major complication (6.7%) that necessitated radiologically-guided drainage of an abdominal collection. The mean (range) length of stay was 17 days (8-28) and the median (range) follow-up time was 35.2 months (7.2-95.4). Two patients presented stricture after CBD reinsertion into the resection cavity; one was treated with radiologically-guided dilatation and the other underwent revisional Roux-en-Y choledochojejunostomy. Three patients presented alkaline reflux gastritis (37.5%), one (12.5%) cholangitis and one CBD stricture after FP with choledochoduodenostomy. No risk factors for secondary CBD stricture were identified. CONCLUSIONS: As part of a biliary derivation, the FP gave good results. We did not observe any complications specifically related to surgical treatment of the biliary tract. However, CBD reinsertion into the resection cavity appeared to be associated with a higher stricture recurrence rate. In our experience, choledochojejunostomy remains the "gold standard" for the surgical treatment for CBD strictures.

Periampullary diverticulum may be an important factor for the occurrence and recurrence of bile duct stones.

BACKGROUND: The aim of this study was to examine the relation between periampullary diverticula (PAD) and biliary tract stones. METHODS: A total of 732 cases of patients with PAD identified from among 6,221 patients who underwent endoscopic retrograde cholangiopancreatography were compared to 750 controls (without PAD) in terms of biliary stone formation, incidence of sphincter of Oddi dysfunction (SOD), and choledocholithiasis. The biliary tract diseases were diagnosed based on medical history, clinical symptoms and signs, and imaging findings using ultrasonography, computed tomography, or magnetic resonance cholangiopancreatography. RESULTS: The PAD group contained a significantly larger number of patients with biliary stones including stones in the primary common bile duct, SOD with bile duct stones (p < 0.05), and choledocholithiasis after cholecystectomy (p < 0.01). CONCLUSIONS: There is a close correlation between PAD and the formation of biliary tract stones. A particularly close correlation was found between PAD and choledocholithiasis after cholecystectomy. PAD may be an important risk factor for the occurrence or recurrence of biliary tract stones.

Long-term results (≥ 10 years) of endoscopic therapy for sphincter of Oddi dysfunction in patients with acute recurrent pancreatitis.

BACKGROUND AND STUDY AIMS: Sphincter of Oddi dysfunction (SOD) is one important cause of idiopathic acute-recurrent pancreatitis (ARP). Several trials have documented complete remission from ARP after endoscopic sphincterotomy during a 2-3-year follow-up. Data with longer follow-up, however, are not available. PATIENTS AND METHODS: Between 1995 and 1998, endoscopic sphincterotomy was performed in 37 patients with manometrically documented SOD and ARP. Afterwards, all patients were prospectively re-evaluated over a period of at least 2 years. In 2008, all patients and their primary physicians were contacted and the patients were interviewed using a structured questionnaire. If a case or situation was unclear, the patients were clinically re-evaluated at our hospital. RESULTS: During the initial prospective 2-year follow-up, relapsing pancreatitis was documented in 5/37 patients (14%). At this point, dual endoscopic sphincterotomy was performed in four patients, and one patient underwent surgical pancreatico-jejunostomy. On retrospective re-evaluation (total follow-up, 11.5±1.6 years) at least one episode of recurrent pancreatitis was found among 19/37 patients (51%). The mean number of relapses that occurred during long-term follow-up (0.7±0.7; range, 0-2) was lower than that recorded at the time of patient enrollment (2.5±0.5; range, 2-4). The recurrence rate did not differ with respect to the patient's first clinical presentation, their demographic data or initial manometric findings. However, relapsing pancreatitis was documented more often in patients who, in the past, had undergone either biliary or pancreatic endoscopic sphincterotomy (12/13 patients) than among those who had undergone dual endoscopic sphincterotomy first (7/24 patients; P<0.05). The median interval for relapsing pancreatitis was 3.5 years (range, 3-84 months). CONCLUSIONS: Follow-up after endoscopic therapy for SOD in patients with ARP should be considered for at least 5 years. For endoscopic treatment, dual endoscopic sphincterotomy may be preferred, although this will not completely prevent recurrence of pancreatitis. Endoscopic therapy nonetheless helped to decrease the frequency of relapse.

Diagnosis and management of choledochoduodenal fistula.

Recent advances in hepatobiliary imaging techniques have led to the increased detection of choledochoduodenal fistula. However, the diagnosis and treatment of choledochoduodenal fistula is still a challenge. In this study, we summarize how patients were diagnosed and treated for choledochoduodenal fistula at our institution. Sixty-six patients with choledochoduodenal fistula were diagnosed and treated in our department from January 2000 to June 2009. Sixty-one patients were treated operatively, whereas five patients were treated with medicine. Patients with choledochoduodenal fistula were confirmed by endoscopic retrograde cholangiography. Of the 61 patients needing surgical intervention, clinical outcomes were excellent in 57 patients, and five patients underwent successful laparoscopic surgery for repairing the choledochoduodenal fistula. Follow-up of these patients for 6 months to 10 years showed they did not suffer from further cholangitis. A patients' past history of biliary disease, upper abdominal pain, fever, and jaundice may lead to choledochoduodenal fistula. Operative therapy, including laparoscopic surgery, was the primary treatment for most patients, regardless of the preoperative diagnosis.

Relationship between pancreaticobiliary maljunction and gallbladder carcinoma: meta-analysis.

BACKGROUND: Reports on the relationship between pancreaticobiliary maljunction (PBM) and gallbladder carcinoma (GBC) are conflicting. The frequency of PBM in GBC patients and the clinical features of GBC patients with PBM vary in different studies. DATA SOURCES: English-language articles describing the association between PBM and GBC were searched in the PubMed and Web of Science databases. Nine case-control studies fulfilled the inclusion criteria and addressed the relevant clinical questions of this analysis. Data were extracted independently by two reviewers using a predefined spreadsheet. RESULTS: The incidence of PBM was higher in GBC patients than in controls (10.60% vs 1.76%, OR: 7.41, 95% CI: 5.03 to 10.87, P<0.00001). The proportion of female patients with PBM was 1.96-fold higher than in GBC patients without PBM (80.5% vs 62.9%, OR: 1.96, 95% CI: 1.09 to 3.52, P=0.12). GBC patients with PBM were 10 years younger than those without PBM (SMD: -9.90, 95% CI: -11.70 to -8.10, P<0.00001). And a difference in the incidence of associated gallstone was found between GBC patients with and without PBM (10.8% vs 54.3%, OR: 0.09, 95% CI: 0.05 to 0.17, P<0.00001). Among the GBC patients with PBM, associated congenital dilatation of the common bile duct was present with a higher incidence ranging from 52.2% to 85.7%, and 70.0%-85.7% of them belonged to the P-C type of PBM (the main pancreatic duct enters the common bile duct). No substantial heterogeneity was found and no evidence of publication bias was observed. CONCLUSIONS: PBM is a high-risk factor for developing GBC, especially the P-C type of PBM without congenital dilatation of the common bile duct. To prevent GBC, laparoscopic cholecystectomy is highly recommended for PBM patients without congenital dilatation of the common bile duct, especially relatively young female patients without gallstones.

Ectopic opening of the common bile duct and duodenal stenosis: an overlooked association.

BACKGROUND: Ectopic opening of the common bile duct into the duodenal bulb (EO-CBD-DB) is a rare disease that may be complicated by duodenal ulcer, deformity, stenosis and biliary stones. The aim of this study is to report clinical presentations, endoscopic diagnosis and treatment of this entity as well as to investigate its association with duodenal stenosis. METHODS: Gastroduodenoscopic findings and radiological imaging were evaluated for ectopic papilla and duodenal stenosis. Diagnostic methods, endoscopic procedures and long-term outcomes of the endoscopic treatment were presented. RESULTS: EO-CBD-DB was found in 74 (77.1%) of the 96 patients with duodenal deformity/stenosis (79 male, 17 female, mean age: 58.5, range: 30-87 years). The papilla with normal appearance was retracted to the bulb in 11 while it was at its usual location in the remaining 11. The history of biliodigestive surgery was more common in patients with EO-CBD-DB who were frequently presented with the common bile duct stone-related symptoms than the other patients. Thirteen (17.6%) of the patients with EO-CBD-DB were referred to surgery. Endoscopic treatment was completed in 60 (81.1%) patients after an average of 1.7 (range: 1-6) procedures. These patients were on follow-up for 24.8 (range: 2-46) months. Endoscopic intervention was required in 12 (20%) of them because of recurrent biliary problems. Treatment of the patient who had stricture due to biliary injury during laparoscopic cholecystectomy is still continued. CONCLUSIONS: The presence of EO-CBD-DB should be considered particularly in middle-aged male patients who have duodenal deformity/stenosis. Endoscopic treatment is feasible in these patients. The long-term outcomes of endoscopic therapy need to be compared with surgical treatment.

Radiation-induced cholangitis with hepatocellular carcinoma.

There are no reports of hepatocellular carcinoma complicating postradiotherapy cholangitis. We report the case of a 45-year-old patient who had undergone upper abdominal radiotherapy for Hodgkin's disease, 21 years before, which was complicated years later by cholangitis with stricture of the common bile duct. Biliodigestive anastomosic surgery was scheduled due to recurrent angiocholitis, and hepatocellular carcinoma was discovered. The patient died from carcinoma some months later.