Anomalous Pancreaticobiliary Junction Research Articles

Self-expandable metallic stents for malignant biliary obstruction with an anomalous pancreaticobiliary junction

Anomalous pancreaticobiliary junction (APBJ) is associated with pancreaticobiliary cancer. Limited data are available on endoscopic biliary drainage for unresectable malignant biliary obstruction with APBJ. This study evaluated the efficacy and safety of self-expandable metallic stents (EMSs) for the management of malignant biliary obstruction with APBJ. Between 1993 and 2005, 324 patients with unresectable malignant biliary obstruction underwent insertion of an EMS. Six of these patients with concomitant APBJ constituted the subjects of this study. Early (</=30 days after EMS insertion) and late (>30 days after EMS insertion) stent-related complications and stent patency were evaluated in these six patients. The cause of biliary obstruction was gallbladder cancer in four patients and pancreatic cancer in two patients. Uncovered EMSs were inserted across the common channel without performance of a biliary sphincterotomy. The diameter of the uncovered EMS used was based on the diameter of the common channel. For all six patients, endoscopic biliary drainage was successful, and their jaundice subsided steadily. None of the six patients experienced early complications, including acute pancreatitis. The mean stent-related complication-free period was 163 days. Stent occlusion caused by tumor ingrowth occurred in two patients. Acute cholangitis and cholecystitis were observed in one patient each. Uncovered EMSs are effective for palliation of unresectable malignant biliary obstruction in patients who have APBJ without increasing the risk of stent-related early complications.

Latent Gallbladder Carcinoma in a Young Adult Patient with Acute Cholecystitis: Report of a Case

Acute cholecystitis associated with gallbladder carcinoma is very rare in young patients (younger than 30 years of age). Moreover, a definitive preoperative diagnosis is difficult. A 26-year-old man was referred to our hospital with a 5-day history of right upper quadrant pain. Computed tomography and ultrasonography demonstrated an enlarged gallbladder with a diffuse thick wall and a 2-cm gallstone obstructing the cystic duct. Magnetic resonance cholangiopancreatography showed no evidence of an anomalous pancreaticobiliary junction. The patient showed an elevation in the white blood cell count, serum C-reactive protein, and alkaline phosphate; however, total bilirubin, alanine aminotransferase, and tumor markers including carcinoembryonic antigen and carbohydrate antigen 19-9 were all within the normal ranges. The preoperative diagnosis of gallstone-induced acute cholecystitis was made and an open cholecystectomy was thus performed 2 days after admission. The macroscopic findings showed a necrotic enlarged gallbladder with a thick wall and a gallstone, but no intraluminal nodular lesion. Histologic examinations revealed well-differentiated focal adenocarcinoma in the gallbladder mucosa, but no venous, lymphatic, or perineural invasion. The postoperative course has been uneventful with no recurrence 18 months postoperatively.

Self-Expandable Metallic Stents for Malignant Biliary Obstruction with Anomalous Pancreaticobiliary Junction

Background: Anomalous pancreaticobiliary junction (APBJ) is associated with pancreaticobiliary cancer. Limited data are available for biliary drainage for unresectable malignant biliary obstruction with APBJ. The aim of this study is to evaluate the efficacy and the safety of self-expandable metallic stents (EMSs) for the treatment of malignant biliary obstruction with APBJ. Methods: Between 1993 and 2005, a total of 324 patients with unresectable malignant biliary obstruction received EMS insertion. Of those, 6 patients had concomitant APBJ and were subjects to the present study. Early (≤30 days after EMS insertion) and late (>30 days after EMS insertion) stent-related complications and stent patency were evaluated in these 6 patients. Results: The cause of biliary obstruction was gallbladder cancer in 4 patients and pancreatic cancer in 2. Uncovered EMSs were inserted across the common channel without biliary sphincterotomy. The diameter of uncovered EMSs employed was chosen according to the diameter of the common channel. In all 6 patients, endoscopic biliary drainage was successful and jaundice steadily subsided. None of these 6 patients developed early complications including acute pancreatitis. Mean serum amylase level was not statistically different between before and 18-24 hrs after stent insertion (129.5 IU/l vs. 148.3 IU/l, p = 0.28). Mean stent -related complication free period was 163 days (range 83-268 days). Stent occlusion due to tumor ingrowth occurred in 2 patients (86 days and 83 days after EMS insertion, respectively). Acute cholangitis with no evidence of stent occlusion was observed in one patient 116 days after EMS placement. Acute cholecystitis occurred in one patient 106 days after stent insertion. Conclusions: Uncovered EMSs are effective for palliation of unresectable malignant biliary obstruction in patients with APBJ without increasing risk of stent-related early complication.

Diagnosis and Surgical Management of Gallbladder Cancer: A Review

Gallbladder cancer is one of the most lethal carcinomas and continues to pose many challenges for surgeons. Identifiable risk factors for carcinoma of the gallbladder include cholelithiasis, an anomalous pancreaticobiliary junction, and focal mucosal microcalcifications. Adenocarcinoma is the primary histologic type in most patients and the tumor is frequently associated with Kras and p53 mutations. Radiologic and endoscopic advances in endoscopic ultrasonography and magnetic resonance cholangiopancreatogram, plus helical computed tomography, have enhanced preoperative staging. Surgical options include cholecystectomy for disease limited to the mucosa (Tis/T1) or a radical cholecystectomy (subsegmental resection of segments IVB and V plus a hepatoduodenal ligament lymphadenectomy) for advanced disease without signs of distant metastasis (T2-4/N0-N2). Some surgeons have advocated more radical hepatic resection including extended right hepatectomy or central bisegmentectomy plus caudate lobectomy. Japanese surgeons have reported studies that included patients having a pancreaticoduodenectomy to improve distal ductal margins and lymphadenectomy for T3 and T4 cancers. These patients have a lower rate of local recurrence but no survival advantage. Options for adjuvant therapy remain limited. Radiation therapy with fluorouracil radiosensitization is the most commonly used postoperative treatments. Current trials are investigating the role of capecitabine, oxaliplatin, and bevacizumab in the management of gallbladder carcinoma.

Double common bile duct: A case report

Double common bile duct (DCBD) is a rare congenital anomaly in which two common bile ducts exist. One usually has normal drainage into the papilla duodeni major and the other usually named accessory common bile duct (ACBD) opens in different parts of upper gastrointestinal tract (stomach, duodenum, ductus pancreaticus or septum). This anomaly is of great importance since it is often associated with biliary lithiasis, choledochal cyst, anomalous pancreaticobiliary junction (APBJ) and upper gastrointestinal tract malignancies. We recently recognized a rare case of DCBD associated with APBJ with lithiasis in better developed common bile duct. The opening site of ACBD was in the pancreatic duct. The anomaly was suspected by transabdominal ultrasonography and finally confirmed by endoscopic retrograde cholangiopancreatography (ERCP) followed by endoscopic sphincterotomy and stone extraction. According to the literature, the existence of DCBD with the opening of ACBD in the pancreatic duct is most frequently associated with APBJ and gallbladder carcinoma. In case of DCBD, the opening site of ACBD is of greatest clinical importance because of its close implications with concomitant pathology. The adequate diagnosis of this rare anomaly is significant since the operative complications may occur in cases with DCBD which is not recognized prior to surgical treatment.

Extracystic biliary carcinoma associated with anomalous pancreaticobiliary junction and cysts

Anomalous pancreaticobiliary junction with cystic dilatation of the biliary tract is usually associated with carcinoma arising from the cyst wall. We report an extracystic location of biliary carcinoma in the presence of anomalous pancreaticobiliary junction and cysts in a patient with obstruction of the origin of the left hepatic duct who underwent hepatobiliary resection. Cholangiocarcinoma was found to have arisen in a noncystic left hepatic duct, in conjunction with cystic dilatation involving both the cystic and common bile ducts. The present case supports a relationship between anomalous pancreaticobiliary junction and biliary carcinogenesis that may affect the extracystic biliary tree.

Spontaneous Rupture of Extrahepatic Choledochal Cyst: Two Pediatric Cases and Literature Review

Spontaneous rupture of extrahepatic choledochal cysts is very rare. Neonatal cases generally present with biliary ascites, and older children with acute abdomen. Although the cause is unclear, accumulation of protein plugs in an anomalous pancreaticobiliary junction, irritation of the cyst wall due to reflux of pancreatic juice, and weakness due to a developmental error such as common channel syndrome have all been suggested to play contributing roles. The treatment of choice is complete excision of the cyst and hepatico-jejunostomy. In newborns who are in a poor condition, the primary treatment should be simple peritoneal drainage.

Anomalous pancreaticobiliary junction (APBJ) with the drainage of the uncinate process into the minor papilla: demonstration by MRI

We report a case of a 30-year-old patient with anomalous pancreaticobiliary junction (APBJ) that has not been described before. The patient had a clinical history of recurrent attacks of pancreatitis, cholangitis and cholecystitis that were confirmed by abnormal laboratory values. Endoscopic retrograde cholangiopancreatography revealed a 20 mm long junction of choledoc and pancreatic duct, and uncinate process draining into the minor papilla. On MR cholangiopancreatography, strictures at the junction of hepatic duct, increased gallbladder wall thickness and intraductal stone in the pancreatic duct were demonstrated as complementary findings. Other MRI findings included decreased signal intensity of the pancreas consistent with fibrosis from past pancreatitis attacks and atrophy of the left liver lobe.

Pancreatic juice can reflux into the bile duct in patients without anomalous pancreaticobiliary junction

Pancreatic juice can reflux into the bile duct in patients without anomalous pancreaticobiliary junction

Endoscopic papillary balloon dilation causes transient pancreatobiliary and duodenobiliary reflux

Endoscopic papillary balloon dilation reduces sphincter function at least transiently or partially, which may allow pancreatobiliary and duodenobiliary reflux to occur. This study prospectively evaluated pancreatobiliary and duodenobiliary reflux after endoscopic papillary balloon dilation. In 12 patients with choledocholithiasis, ductal bile was sampled for amylase concentration and bacterial culture during ERCP, before and at 7 days to 5 years after endoscopic papillary balloon dilation. To provide comparative and control data, ductal bile was sampled in 12 patients with gallbladder cholesterol polyps and 6 with anomalous pancreaticobiliary junction who did not undergo endoscopic papillary balloon dilation. Amylase concentrations in ductal bile from patients with choledocholithiasis before endoscopic papillary balloon dilation were marginally significantly higher (before Bonferroni correction) compared with concentrations in bile from patients with gallbladder polyps. The concentration of amylase in bile was significantly increased at 7 days after endoscopic papillary balloon dilation compared with that before endoscopic papillary balloon dilation; the level was comparable with that of patients with an anomalous pancreaticobiliary junction. Subsequently, the amylase concentration gradually decreased and was approximately equal to the pre-endoscopic papillary balloon dilation level at 1 year. Bacteriocholia was frequent (67%-92%) for up to 3 months after endoscopic papillary balloon dilation but was rare thereafter. Endoscopic papillary balloon dilation causes transient pancreatobiliary and duodenobiliary reflux. However, reflux is no longer present at 1 year after endoscopic papillary balloon dilation.

P0817 CLINICAL PROFILE OF PANCREATIC DISORDERS IN CHILDREN IN NORTH INDIA

Introduction: To determine the profile of Pancreatic disorders in children (age <16 yrs) presenting to a tertiary health care centre in North India. Methods: 68 children (<16 yrs) presenting to the pediatric medical services of the hospital with pancreatic disorders were recruited in the study from Oct. 1998 to Nov. 2003. The mean age was 10.2 yrs (range 0.5–16 yrs) with male: female ratio of 38: 30. Results: There were 27 patients of acute pancreatitis and 24 cases of chronic pancreatitis. 14 patients had recurrent pancreatitis while 3 cases were of cystic fibrosis with pancreatic insufficiency. Etiology of acute pancreatitis which could be identified in 17(62.9%) included traumatic 6 (22.2%), biliary 4(14.8%), mumps 4 (14.8%), and hepatitis A in 11.1%. Complications were seen in 14 patients (51.8%) in the form of pseudocyst 10 (37%), necrosis 6 (22.2%),haemorrhage 4 (14.8%), infection 2 (7.4%), seizures & acute renal failure in one each. There were no deaths. The etiology in most of the cases of recurrent pancreatitis was idiopathic 8 (57.1%),while there was one case each of gallstones, choledochal cyst, anomalous pancreatico biliary junction and hyperparathyroidism with hypercalcemia. 2 cases had family history of more than 2 family members suffering from pancreatitis. Out of 24 patients of chronic pancreatitis, 11(45.8%) were of calcific variety while the rest 54.1% were non-calcific. Complications were seen in 13 (54.1%) in the form of pseudocyst 7(29.1%), stricture of the pancreatic duct 6(25%), atrophy of the gland 2(8.3%), gastroduodenal artery aneurysm and compartmental portal hypertension in one case, exocrine pancreatic insufficiency and diabetes mellitus in one each. Conclusion: Pancreatic disorders in children require a high degree of clinical suspicion. Traumatic pancreatitis was the commonest cause of acute pancreatitis next to idiopathic. Complications were seen in 51.8% of acute pancreatitis patients with pseu-docyst in 37% & necrosis in 22.2%,with no mortality. 54.1% of chronic pancreatitis cases had complications with pseudocyst in 29.1% and stricture of pancreatic duct in 25%. Chronic non calcific variety (54.1%) was slightly more common than calcific variety and diabetes mellitus / steatorrhoea have been uncommon in North Indian children.

Biliopancreatic reflux via anomalous pancreaticobiliary junction

A PREVIOUSLY HEALTHY 32-YEAR-OLD WOMAN experienced an attack of mild acute pancreatitis manifested by right upper quadrant abdominal pain and hyperamylasemia (1676 IU/L; normal range, 50 to 240 IU/L). The patient had no history of excessive alcohol intake or jaundice. Conservative treatment improved the acute pancreatitis. Endoscopic retrograde cholangiopancreatography showed a long (11 mm) common channel, a dilated (48 mm) extrahepatic bile duct with gallstones, and nondilated normal pancreatic ducts (Fig 1). These findings represented anomalous pancreaticobiliary junction (APBJ) associated with choledochal cyst (Todani’s type I).1 Drip infusion cholangiography (DIC)-multidetector row computed tomography (MDCT) was performed using Aquilion (Toshiba Medical System, Tokyo, Japan) with four high-resolution detectors. After intravenous injection of a 100-mLdose of iotroxate meglumine (Biliscopin DIC 50; ScheringAG, Berlin, Germany),maximum intensity projection (MIP) images obtained by DIC-MDCT showed a long common channel, a dilated extrahepatic bile duct, and a proximalmain pancreatic duct (of the pancreatic head) (Fig 2). At 30minutes after ingestion of 4 g of dried egg yolk (Molyork; Toho Chemical Co, Osaka, Japan), DIC-MDCT demonstrated the main pancreatic duct more distally (Fig 3). Axial images revealed yolk-induced contraction of the nonopacified gallbladder. This patient underwent excision of the extrahepatic bile duct and gallbladder followed by

Role of endoscopic ultrasonography in diseases of the extrahepatic biliary system.

Endoscopic ultrasonography (EUS) is one of the most recent advances in gastrointestinal endoscopy. Available EUS devices include echoendoscopes, such as radial scanning and linear array echoendoscopes, and catheter ultrasound probes. Endoscopic ultrasonography has various applications, such as staging of gastrointestinal malignancy, evaluation of submucosal tumors, and has grown to be an important modality in evaluating the pancreaticobiliary system. With regard to the biliary system, EUS is useful for the detection and staging of ampullary tumors, detection of microlithiasis and choledocholithiasis and evaluation of benign and malignant bile-duct strictures. Endoscopic ultrasonography may be used as an adjunct to transabdominal ultrasound for the detection and characterization of gallbladder polyps. In addition, EUS is helpful in the staging of gallbladder cancer as well as in diagnosing anomalous pancreaticobiliary junction with its associated pancreatobiliary diseases. The present paper reviews the current applications of EUS for imaging and intervention in diseases of the extrahepatic biliary system.

Choledochal Cyst Mimicking a Pancreatic Pseudocyst: Case Report and Review

Choledochal cyst (CC) is a relatively rare congenital anomaly of the biliary ducts. Its origin and formation are a matter of considerable investigation and debate, although CC is frequently associated with anomalous pancreaticobiliary junction (APJ).1 The prevalence of CC is relatively high in Asian countries, especially among women.2 The classic triad of abdominal pain, jaundice, and abdominal mass occurs in less than a third of patients.3 Many complications, including malignant degeneration, have been associated with CC, and, thus, surgical treatment is mandatory where possible.4 Although it reliably depicts CC, ERCP is invasive and has a potential for morbidity. Noninvasive techniques, such as MRCP, are potential alternatives for the diagnosis of CC and APJ.5 A case is reported of CC that was diagnosed as a pancreatic pseudocyst and initially treated by endoscopic drainage.

Congenital dilatation of the bile duct: changes in diagnostic tools over the past 19 years.

Because of recent improvements in diagnostic procedures, many cases of congenital dilatation of the bile duct (CDBD) have been diagnosed in early life. To determine the mode of presentation and changes in diagnostic tools, medical records of 34 children (25 girls, nine boys) with CDBD who were treated between 1982 and 2000 were reviewed. The age at presentation ranged from 0 to 16 years (median 4.9 +/- 0.7 years), with two patients being diagnosed using prenatal ultrasonography. Of the 32 patients diagnosed post-natally, 21 (66%) complained of abdominal pain. There were also 21 (66%) cases of vomiting. Sixteen patients (50%) experienced both these symptoms. Ten (31%) cases presented with jaundice and three (9%) presented with a palpable mass. Ultrasonography was used as a diagnostic tool in 29 cases (85%). The finding of a dilated common bile duct on abdominal ultrasonography was helpful for early diagnosis. Spiral computed tomography was also a useful and accurate imaging method for diagnosis. Anomalous pancreaticobiliary junction (PBJ) was demonstrated in 44% of patients with CDBD. Magnetic resonance cholangiopancreatography, which can be performed non-invasively, is useful for the diagnosis and pre-operative assessment of CDBD in children. The average length of time from presentation to diagnosis could be shortened from 46 days (1982-1988) to 13 days (1995-2000). Recent improvements in diagnostic procedures make immediate, non-invasive diagnosis of CDBD and PBJ possible.

Acute pancreatitis associated with biliary disease in children.

Biliary disease is one of the most common causes of acute pancreatitis in adults; however, this cause and outcome in children have rarely been described in the literature. Therefore, the present study was conducted to evaluate the role of biliary disease as a cause of acute pancreatitis in children. The present study included 56 children with acute pancreatitis, of which 16 (29%) cases were associated with biliary disease. The 16 cases consisted of four boys and 12 girls ranging in age from 2 to 13 years. The underlying causes of the biliary disease were evaluated, as well as its clinical presentations, management modalities, and outcomes. The causes of biliary disease in the 16 children included choledochal cyst in seven, biliary sludge in six, gallstone in two, and anomalous pancreaticobiliary junction in one. Acute pancreatitis with biliary disease showed increased presentation of jaundice and abnormalities in a liver-function test. Therapeutic interventions were performed more frequently in acute pancreatitis associated with biliary disease. All seven children with choledochal cysts needed hepaticojejunostomy. Of eight children with biliary sludge or gallstones, five children remained free of and two suffered from repeated attacks of pancreatitis after endoscopic papillotomy. Mortality did not occur. The present study suggests that biliary disease can be one of the causes of acute pancreatitis in children and has the clinical characteristics of jaundice and/or abnormalities in a liver-function test. Appropriate therapeutic interventions should be considered as the treatment modality.

Anomalous pancreaticobiliary junction without congenital choledochal cyst.

Anomalous pancreaticobiliary junction (APBJ) without congenital choledochal cyst (CCC) carries a high risk of gallbladder carcinoma development. The aim of this study was to obtain information allowing early diagnosis and appropriate management. The clinical features, imaging findings and surgical outcome of 18 patients with APBJ without CCC were analysed retrospectively. Fourteen patients had symptoms, including those of acute pancreatitis (five patients). In 16 patients the gallbladder showed abnormalities, including carcinoma (eight) and mucosal hyperplasia (11). Ultrasonography detected gallbladder carcinoma with 100 per cent sensitivity and mucosal hyperplasia with 91 per cent sensitivity. A long common channel was demonstrated by endoscopic retrograde cholangiopancreatography (ERCP) in all patients, endoscopic ultrasonography in nine of ten, and magnetic resonance cholangiopancreatography (MRCP) in five of five. Five of eight patients with gallbladder carcinoma underwent extended cholecystectomy with bile duct excision. Three patients with cancer and eight with no cancer had cholecystectomy alone. None developed bile duct carcinoma or acute pancreatitis after operation. All patients without malignancy remained asymptomatic for a mean follow-up period of 4.7 years. Prophylactic cholecystectomy is recommended for patients with APBJ without CCC. For early diagnosis of APBJ, gallbladder abnormalities on ultrasonography or acute pancreatitis of unknown aetiology should prompt further investigation with ERCP or less invasive imaging modalities such as endoscopic ultrasonography and MRCP.

A pair of monozygotic twins with anomalous pancreaticobiliary junction and pancreatitis

The authors describe a pair of white 7-year-old monozygotic twin girls with the same anomaly of the pancreaticobiliary junction (APBJ), in whom the clinical presentation and disease evolution are slightly divergent. The pathogenesis and genetic control of the disease are discussed. J Pediatr Surg 37:1485-1487. Copyright 2002, Elsevier Science (USA). All rights reserved.

Laparoscopically assisted resection of choledochal cyst and Roux-en-Y reconstruction.

Laparoscopic surgery for a congenital choledochal cyst was accomplished in five of eight adult patients for whom it was attempted (63%). Creation of a Roux-en-Y jejunal limb by midline minilaparotomy and hepaticojejunostomy using a laparoscopic sewing instrument facilitated the procedure. Congenital choledochal cyst is a good indication for laparoscopic surgery. However, only two case reports are available at this writing. Eight adult patients, ages 19 to 61 years (mean, 32.6 years), underwent laparoscopically assisted resection of the choledochal cyst and Roux-en-Y hepaticojejunostomy. The whole procedure was accomplished in five patients (63%). The duration of the procedure ranged from 525 to 680 min (average, 616 min). Open conversion in three patients was necessitated by severance of a small common hepatic duct because of disorientation caused by previous laparoscopic cholecystectomy, electrocautery injury to the common channel distal to the anomalous pancreaticobiliary junction, or heavy adhesion around the cyst secondary to recent severe cholangitis. Creation of a Roux-en-Y jejunal limb by midline minilaparotomy and hepaticojejunostomy using a laparoscopic sewing instrument facilitated the procedure. Laparoscopically assisted resection of the choledochal cyst and hepaticojejunostomy are technically feasible and deserve further clinical trials.

Pancreatic disorders associated with anomalous pancreaticobiliary junction

Background: The aim of this study was to establish an optimal management strategy for pancreatic disorders associated with anomalous pancreaticobiliary junction (APBJ). Methods: In 64 adult surgical cases of APBJ (common channel 15 mm or longer) (43 with and 21 without choledochal cyst), associated pancreatic disorders, pancreatographic results, and treatment outcomes were analyzed. Results: Of 64 patients, 56 had pancreatobiliary symptoms. Twenty-four patients (38%) had pancreatic disorders: acute pancreatitis (n = 20), chronic calcifying pancreatitis (n = 2), and pancreatic carcinoma (n = 2). Twenty patients (31%) had abnormal pancreatograms. The incidence of acute pancreatitis was significantly higher in patients with an abnormal pancreatogram, particularly dilatation, protein plugs or stones of the common channel or main pancreatic duct, and coexisting pancreatic ductal anomaly. All patients with choledochal cyst underwent cyst excision and hepaticojejunostomy. Eleven patients without choledochal cyst or pancreatobiliary carcinoma underwent cholecystectomy alone. Protein plugs and pancreatic stones were extracted through the bile duct stump or by sphincterotomy. No patients experienced pancreatitis during a mean postoperative follow-up of 6.7 years. Conclusions: In managing APBJ, attention should be paid to the possibility of associated pancreatic disorders and an abnormal pancreatogram. APBJ with choledochal cyst requires cyst excision. Cholecystectomy alone may be adequate for APBJ without cyst. (Surgery 1999;126:492-7.)