Pancreaticobiliary maljunction: etiologic concepts based on radiologic aspects

BACKGROUND: The purpose of this study was to develop a new concept of the embryonic etiology of pancreaticobiliary maljunction (PBM) based on cholangiopancreatograms. METHODS: The subjects were 202 patients with PBM (60 men and 142 women) in whom the junction of the pancreatic and bile ducts was radiologically diagnosed as being located outside of the duodenal wall; 133 of the 202 patients also had congenital cystic dilatation of the bile duct (CCBD). RESULTS: The length of the duct from the junction to the orifice of the major papilla (the common channel) ranged from 0.5 to 5 cm on the cholangiopancreatograms. Small radicles of the pancreatic duct arose from the common channel in 36 of the 202 patients. This finding suggests that the common channel is itself the main pancreatic duct in patients with PBM. Moreover, cholangiopancreatography revealed that in 99 of the 202 patients, there was a narrowed duct segment distal to the biliary cyst in patients with CCBD or distal to the normal bile duct in those without CCBD; the length of the narrowed segment varied. Histologic examination revealed smaller branches that had arisen from this narrowed segment in 2 anatomic specimens. This also suggests that the narrowed ductal segment belongs to the pancreatic duct system. CONCLUSION: PBM is an anomaly that is probably caused by a disturbance in the embryologic connections (misarrangement) of the pancreatic and biliary duct system that occurs extremely early during gestation when the bile duct joins with the ventral pancreatic duct system. PBM is not due to an arrest of the normal migration of the common channel into the duodenal lumen during embryonic development.     

Distal common bile duct stenosis secondary to benign duodenal ulceration: report of a case

Obstructive jaundice is a very rare complication of peptic ulcer disease. We report a patient who presented with symptoms suggestive of malignant distal common bile duct stenosis. A final diagnosis of benign duodenal ulcer with stricture of the common bile duct was made. A short review of the literature is also presented.     

Embryogenesis of pancreaticobiliary maljunction inferred from development of duodenal atresia

The embryogenesis of pancreaticobiliary maljunction is inferred from the embryogenesis of duodenal atresia. The epithelial cells of the duodenum begin to proliferate and completely plug the lumen, but a process of vacuolation recanalizes the duodenum. Recanalization of the common duct frequently appears with two lumina and openings into the duodenum with two orifices. These two major canals create a narrow segment of the duodenum and this narrow zone is the area in the duodenum that is most prone to faulty recanalization and atresia formation. A bifid biliary system inserts at blind upper and lower pouches of the duodenum, and the common bile duct inserts in a Y fashion. The common bile duct inserts at the stenotic segment, and the end result is a T-shaped formation in patients with duodenal stenosis. During the development of the bile duct, abnormal fusion may occur between the bile duct and branches of the right ventral pancreatic duct. The site in the bile duct where a branch of the pancreatic duct joins is likely to develop atresia due to disturbance of the recanalization process, as seen in duodenal atresia. Severe impairment of vacuolation causes divided atretic bile duct at the site where the pancreatic duct inserts in a Y-fashion into the upper and lower bile duct. Moderate impairment of vacuolation causes a stenosis at the site where the pancreatic duct inserts in a T-shape, with a moderate dilatation of the bile duct.     

A case of gallbladder adenomyomatosis with pancreaticobiliary maljunction and an anomaly of the cystic duct joined the common channel

A 46-yr-old woman was admitted to our hospital with mild epigastric pain. Ultrasonography and computed tomography revealed an extremely thickened gallbladder wall. Endoscopic retrograde cholangiopancreatography demonstrated that the main pancreatic duct joined the nondilated common bile duct at the outer point of the duodenal wall (P-C type of pancreaticobiliary maljunction), and the cystic duct joined the common channel directly. The intraoperative amylase levels of the bile juices both in the common bile duct and the cystic duct were high. A cholecystectomy was performed. The wall of the gallbladder was markedly thick, yellowish, elastic, and soft. Histologically, Rokitansky-Aschoff sinus proliferation, hypertrophy of smooth muscles, and fibrosis were seen. The diagnosis was a generalized type of adenomyomatosis. The pathogenesis of the adenomyomatosis was believed to result from chronic stimulation as a result of pancreatic juice reflux. The etiology of this unusual type of junction was considered to be the result of the combination of pancreaticobiliary maljunction and an anomaly of lower junction of the cystic duct.     

A Case of Groove Pancreatitis with Duodenal Stenosis

A 58‐year‐old man presented with a 2‐month history of nausea and vomiting. Blood levels of hepatic enzymes and pancreatitis markers were slightly elevated. Hypotonic duodenographic and endoscopic examinations revealed stenosis encircling the descending duodenum. A computed tomography (CT) scan showed inflammatory changes in the head of the pancreas and thickening of the duodenal wall. Magnetic resonance cholangiography demonstrated stenosis of the intrapancreatic segment of the common bile duct and diffuse dilatation of the main pancreatic duct, without irregularity. At laparotomy, microscopic examination of a needle biopsy specimen of the head of pancreas revealed no evidence of malignancy. Distal gastrectomy with Billroth II anastomosis was performed. Microscopically, fibrous thickening of the duodenal wall, serositis and hyperplasia of Brunner's glands were found. The presence of duodenal stenosis due to segmental pancreatitis, referred to as groove pancreatitis, was confirmed. The elevated blood levels of pancreatitis markers returned to the normal range 8 months after the operation. Ultrasonic echography and abdominal CT also revealed a marked reduction in dilatation of the extrahepatic bile duct and the main pancreatic duct. In patients suspected of having pancreatic carcinoma or regional pancreatitis, groove pancreatitis should be included in the differential diagnosis.     

Ectopic opening of the common bile duct in the duodenal bulb: clinical implications

BACKGROUND: An ectopic opening of the common bile duct in the duodenal bulb is extremely rare, and the clinical significance of this anomaly has not been clarified. This study analyzed the clinical implications and cholangiographic findings of this anomaly. METHODS: A total of 18 patients (15 men, 3 women; median age, 51 years) with an ectopic opening of the common bile duct in the duodenal bulb were studied. Medical records, endoscopic findings, and cholangiographic and other radiographic findings were reviewed. RESULTS: All 18 patients had biliary pain; 7 had fever and chills. Fifteen (83%) had diffuse dilatation of the extrahepatic bile ducts with or without intrahepatic bile duct dilation. Associated bile duct stones were found in 10 (56%) patients. The papilla in the bulb had an orifice stained with bile at endoscopy, which was associated with duodenal ulcer disease found in 13 (72%) patients. The distal end of the common bile duct was tapered and narrowed and had a hook shape in all patients. CONCLUSION: An ectopic opening of the common bile duct in the duodenal bulb may be associated with clinical entities such as recurrent or intractable duodenal ulcer, choledocholithiasis, or acute cholangitis. Although these openings are rare, knowledge of the endoscopic and radiographic findings of an ectopic opening of the common bile duct in the duodenal bulb may help to clarify the cause of chronic recurrent duodenal ulcer disease in some patients and prevent damage to the bile duct during surgery.     

How many biopsies should be performed during percutaneous transhepatic cholangioscopy to diagnose biliary tract cancer?

BACKGROUND: The sensitivity of biopsy in the diagnosis of cholangiocarcinoma using percutaneous transhepatic cholangioscopy is not well defined. METHODS: Patients with a biliary tract malignancy (n = 52) underwent directed biopsy during percutaneous transhepatic cholangioscopy using a 1.8 mm diameter forceps. Histologic findings were correlated with endoscopic appearance. RESULTS: A diagnosis of carcinoma was made in all four patients with a tumor of the major duodenal papilla and in all 15 patients with a polypoid bile duct tumor with two biopsies from the mass. In 19 patients with stenotic bile duct cancer, a positive diagnosis was made in 95% of cases when three biopsies were taken from the margin of the stenotic area. When cholangioscopy showed a tortuous, dilated vessel (n = 10), the diagnosis of cancer was made with two biopsies taken from the margin of the stenosis. In 14 patients with metastatic bile duct cancer, the diagnosis was made in only 43% of cases when three biopsies were taken from the margin of the stenosis. When combined with results from the three biopsies taken from within the area of stenosis, the sensitivity for diagnosing pancreatic cancer improved from 20% to 60%. CONCLUSIONS: Directed cholangioscopic biopsies are highly sensitive for the diagnosis of cholangiocarcinoma but less sensitive for cancer metastatic to the bile duct. The numbers and locations of the biopsies required to make a diagnosis of carcinoma depend on the origin and cholangioscopic appearance of the tumor.     

Segmental groove pancreatitis accompanied by protein plugs in Santorini's duct

"Groove pancreatitis", a form of segmental pancreatitis affecting the head of the pancreas, is local-ized within the "groove" between pancreas head, duo-denum, and common bile duct. Differentiation between groove pancreatitis and pancreatic head carcinoma is often difficult. We report a case of groove pancreatitis in which a hypoechoic mass between the duodenal wall and pancreas was clearly imaged, and narrowing of the second portion of the duodenum and bile duct stenosis were also found. The diagnosis was confirmed by surgery (pylorus-preserving pancreato duodenectomy). The patient was relieved from abdominal pain post operation. Up to the present, the patient has been good condition. We review the clinicopathologic and radiologic features of groove pancreatitis in the Japanese literature and discuss the possible role of Santorini's duct in its pathogenesis. We consider that impacted protein plugs in Santorini's duct are a pathogenic factor in the development of groove pancreatitis. Therefore, the findings of Santorini's duct on endoscopic retrograde pancreatography are very important in the diagnosis of groove pancreatitis. Groove pancreatitis presents various clinical features, such as biliary stenosis, duodenal stenosis, and pancreatic mass, and often masquerades as pancreatic head carcinoma. This condition should be kept in mind in the differential diagnosis of pancreatic head carcinoma. (Received Apr. 17, 1997; accepted Sept. 26, 1997)     

Endoscopic diagnosis of pancreaticobiliary maljunction

Pancreaticobiliary maljunction (PBM) is a congenital anomaly defined as a junction of the pancreatic and bile ducts located outside the duodenal wall, usually forming a markedly long common channel. As the action of the sphincter of Oddi does not functionally affect the junction in PBM patients, continuous pancreatobiliary reflux occurs, resulting in a high incidence of biliary cancer. PBM can be divided into PBM with biliary dilatation (congenital choledochal cyst) and PBM without biliary dilatation (maximal diameter of the bile duct ≤ 10 mm). The treatment of choice for PBM is prophylactic surgery before malignant changes can take place. Endoscopic retrograde cholangiopancreatography (ERC P) is the most effective examination method for close obs ervation of the pattern of the junction site. When the communication between the pancreatic and bile ducts is maintained, despite contraction of the sphi ncter on ERCP, PBM is diagnosed. In these pat ients, levels of pancreatic enzymes in the bile are gene rally elevated, due to continuous pancreatobiliary reflux via a long common channel. Magnetic resonance cholangiopancreatography and 3D-computed tomography can diagnose PBM, based on findings of an anomalous union between the common bile duct and the pancreatic duct, in addition to a long common channel. Endoscopic ultrasonography and intraductal ultra sonography can demonstrate the junction outside the duodenal wall, and are useful for the diagnosis of asso ciated biliary cancer. Gallbladder wall thickness on ultra so nography can be a screening test for PBM.     

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.     

A case of intrahepatic cholangiocarcinoma associated with Type IV choledochal cyst

Anomalous union of the pancreaticobiliary duct (AUPBD) is a congenital anomaly that is defined as a junction of the bile duct and pancreatic duct outside the duodenal wall. This anomaly results in a loss of normal sphincteric mechanisms at the pancreaticobiliary junction. As a result, regurgitation of pancreatic juice into the biliary system develops and causes choledochal cysts, choledocholithiasis, cholangitis, pancreatitis and malignancy of the biliary tract. Gallbladder cancer or common bile duct cancer associated with AUPBD and choledochal cysts have been frequently reported. But, intrahepatic cholangiocarcinoma associated with this condition has been only rarely reported. Here, we report a case of intrahepatic cholangiocarcinoma associated with AUPBD and choledochal cyst.     

Groove pancreatitis: a rare segmental form of chronic pancreatitis

Groove pancreatitis is a rare form of segmental chronic pancreatitis which is localized within the head of the pancreas, the duodenum and the common bile duct. Symptoms are due to common bile duct stenosis or duodenal stenosis. Radiologically, there is a pancreatic mass, which hinders differential diagnosis with pancreatic carcinoma. We report here a case of groove pancreatitis observed in a 41-year-old man treated by pancreatoduodenectomy. Histological features of the groove scar were noted. Our case and cases reported in the literature lead to hypotheses concerning the pathogenesis and clinical, biological, and radiological features suggestive of the diagnosis.     

Recent advances in pancreaticobiliary maljunction

The purpose of this review is to evaluate our current knowledge of the embryologic etiology of pancreaticobiliary maljunction (PBM), its diagnosis, clinical aspects, and treatment, and to clarify the mechanisms of PBM involvement in carcinogenesis. Although the embryologic etiology of PBM still awaits clarification, an arrest of the migration of the common duct of the biliary and pancreatic ducts inwards in the duodenal wall has hitherto been speculated to result in a long common channel in PBM. However, we propose the hypothesis that the etiology of PBM is caused by a disturbance in the embryonic connections (misarrangement) of the choledochopancreatic duct system in the extremely early embryo. That is, PBM is an anomaly caused by a misarrangement whereby the terminal bile duct joins with a branch of the ventral pancreatic duct system, including the main pancreatic duct. PBM is frequently associated with congenital bile duct cyst (CCBD). However, these two anomalies are thought to have different embryonic etiologies. The diagnostic criteria for PBM are the radiological and anatomical detection of the extramural location of the junction of the pancreatic and biliary ducts in the duodenal wall. However, in PBM patients with a short common duct (less than 1 cm in length), detection of the extramural location is difficult. The clinical features of PBM are intermittent abdominal pain, with or without elevation of pancreatic enzyme levels; and obstructive jaundice, with or without acute pancreatitis, while the clinical features of PBM patients with CCBD are primary bile duct stone and acute cholangitis. The optimum approach for the treatment of PBM is the prevention of the reciprocal reflux of bile and pancreatic juice in the pancreas and the bile duct system. To achieve these aims, the surgical approach is most effective, and complete biliary diversion procedures with bile duct resection (for example, choledochoduodenostomy or choledochojejunostomy of the Roux‐en‐Y type) are most useful. Recently, it has been recognized that the development of biliary ductal carcinoma is associated with PBM. That is, the development of gallbladder cancer occurs frequently in PBM patients without CCBD, and bile duct cancer originating from the cyst wall also occurs in PBM patients with CCBD. It is speculated that the pathogenesis of the bile duct or gallbladder cancer in PBM patients involves the reciprocal reflux of bile and pancreatic juice. Investigations of epithelial cell proliferation in the gallbladder of PBM patients, and of K‐ras mutations andp53 suppressor gene mutations, loss of heterozygosity ofp53, and overexpression of thep53 gene product in gallbladder cancer and noncancerous lesions in PBM patients have been carried out in various laboratories around the world. The results support the conclusion that PBM is a high risk factor for the development of bile duct carcinoma.     

A new embryologic hypothesis of annular pancreas

Annular pancreas is a developmental anomaly of the pancreas. There are two major hypotheses concerning development of the annular pancreas from the ventral pancreatic anlage; adhesion of the right ventral anlage to the duodenal wall (Lecco's theory), and persistence of the left ventral anlage (Baldwin's theory) reported in 1910, but each theory has some problems and can account for only a few types of annular pancreas. We report a new embryologic hypothesis of annular pancreas which can account for the developmental mechanism of three types of arrangement of annular ducts. The tip of the left ventral anlage adheres to the duodenum and stretches to form a ring. Whether the tip is proximal or distal to the bile duct creates several arrangements of the annular duct.     

Choledochoduodenal fistula at the anterior wall of the duodenal bulb: a rare complication of duodenal ulcer

A 38 year-old man was admitted to our hospital with the chief complaint of epigastralgia. His laboratory data revealed leukocytosis and increased serum amylase, and abdominal ultrasonography revealed diffuse swelling of the pancreas. Thus, he was diagnosed as having acute pancreatitis. Moreover, abdominal computed tomography showed pneumobilia in the gallbladder and the common bile duct. Gastroduodenal fiberscopy demonstrated peptic ulcer scars around a foramen with smooth margins at the anterior wall of the duodenal bulb. The bile juice flowed from the bottom of the foramen. Endoscopic retrograde cholangiopancreatography revealed the fistula between the common bile duct and the anterior wall of the duodenal bulb, but not the posterior wall. However, there was no pancreatico-biliary maljunction and no stones in the gallbladder or bile duct. This is a rare case of choledochoduodenal fistula at the anterior wall of the duodenal bulb caused by duodenal peptic ulcer disease.     

A Case Report of Anomalous Pancreaticobiliary Duct Connection with Choledocholithiasis and Acute Pancreatitis

Abstract: A 25-year-old man was admitted to hospital with epigastric pain. He had had a history of episodic abdominal pain since early childhood. An anomalous pancreaticobiliary duct connection was seen by endoscopic retrograde cholangiopancreatography. In many cases, this type of abnormality is caused by an anomaly in the ventral pancreas. In this case, however, the common bile duct, with calculi, was joined to the pancreatic duct which did not arise from the ventral pancreas but from the dorsal pancreas. The pancreatic duct arising from the ventral pancreas was absent in this case. The patient underwent a prophylactic cholecystectomy, a transduodenal sphincteroplasty, a choledocholithotomy, a partial resection of the common bile duct, and a hepaticojejunostomy, performed by a Roux-en-Y anastomosis. His postoperative recovery was satisfactory. An anomalous pancreatobiliary duct connection allows pancreatic juices and bile to mix. This is considered to be an etiological factor in pancreatitis and choledocholithiasis.     

A Case of Anomalous Arrangement of the Pancreaticobiliary Ductal System Demonstrated by Intraductal Ultrasonography

A 55-yr-old female was hospitalized with epigastrie pain. Conventional ultrasonography revealed marked dilation of the common bile duct (CBD). Endoscopic retrograde cholangiopancreatography showed fusiform dilation of the CBD. The common channel of the pancreatic duct and choledochus was 20 mm long. A diagnosis of congenital choledochal dilation accompanied by anomalous arrangement of the pancreaticohiliary ductal system (AAPBDS) was made. Intraductal ultrasonography (IDUS) was performed. IDUS demonstrated the union of the pancreatic duct and choledochus within the pancreatic parenchyma. This meant that the union existed outside the duodenal wall, confirming the diagnosis of AAPBDS. Although endoscopic retrograde cholangiopancreatography alone could show the maljunction in this case, simultaneous IDUS will be useful in making an accurate diagnosis of AAPBDS.     

The papilla of Vater just below the pylorus presenting as recurrent duodenal ulcer bleeding

The papilla of Vater emptying into the duodenal bulb site is extremely rare and considered an aberrant condition. We report here a case with recurrent duodenal ulcer bleeding associated with this anomaly. A 42-year-old man was admitted to St. Mary Hospital because of tarry stool for three days. Despite no documented etiology to explain recurrent ulceration, the patient had about ten episodes of ulcer bleeding since 1995. On duodenoscopy, 1.0 x 0.6 cm sized active stage duodenal ulcer with oozing was observed at the posterior wall side below the pylorus. The papilla of Vater was bulging just below the pylorus. Bile juice was excreted from its opening. Pancreatic duct and common bile duct, which drained into the bulb site, were observed on ERCP. In this report, we show that recurrent duodenal ulcer can be associated with the papilla of Vater just below the pylorus.     

EUS diagnosis of ectopic opening of the common bile duct in the duodenal bulb： A case report

Among the various congenital anomalies of the biliary system, an ectopic opening of the common bile duct (CBD) in the duodenal bulb is extremely rare. ERCP is essential for diagnosing the anomaly. A 55-year-old male was admitted to hospital for severe right upper quadrant abdominal pain, followed by fever, chills, elevated body temperature and mild icterus. The diagnosis of ectopic opening of CBD in the duodenal bulb was established on endoscopic ultraso-nography (EUS), which clearly demonstrated dilated CBD, with multiple stones and air in the lumen, draining into the bulb. A normal pancreatic duct, which did not drain into the bulb, was also observed. This finding was confirmed on ERCP and surgery. As far as we know, this is the first case of this anomaly diagnosed by EUS. Ectopic opening of the CBD in the duodenal bulb is not an incidental finding, but a pathologic condition which can be associated with clinical entities such as recurrent or intractable duodenal ulcer, recurrent biliary pain, choledocholithiasis or acute cholangitis. Endoscopic ultrasonography features allow preoperative diagnosis of this anomaly and can replace ERCP as a first diagnostic tool in such clinical circumstances. Embryology of the anomalies of the extrahepatic biliary tree has been also reviewed.     

Laparoscopic drainage of an intramural duodenal hematoma

A 21-year-old man was admitted with vomiting and abdominal pain 3 days after sustaining blunt abdominal trauma by being tackled in a game of American football. A diagnosis of intramural hematoma of the duodenum was made using computed tomography and upper gastrointestinal tract contrast radiography. The hematoma caused obstructive jaundice by compressing the common bile duct. The contents of the hematoma were laparoscopically drained. A small perforation was then found in the duodenal wall. The patient underwent laparotomy and repair of the injury. Laparoscopic surgery can be used as definitive therapy in this type of abdominal trauma. (Received Nov. 12, 1997; accepted Aug. 21, 1998)