Annular pancreas associated with carcinoma in the dorsal part of pancreas divisum

Summary A carcinoma in the dorsal part of the pancreas divisum with an annular pancreas in the anterior part is reported. A 79-yr-old female was admitted in our hospital complaining of epigastralgia. Computed tomography (CT) and ultrasound (US) showed an irregular mass in the pancreatic body. A pancreatogram obtained through the major duodenal papilla demonstrated only the ventral pancreatic duct that encircled the duodenum. Contrast medium injected from the minor duodenal papilla showed Santorini’s duct obstruction at the neck portion of the pancreas without communication with the ventral pancreatic duct. The patient died with liver metastases. Autopsy confirmed annular pancreas and a 6-cm tumor in the pancreatic body extending to the pancreatic head and pancreas divisum. Pancreatic carcinoma; histologically a moderately differentiated adenocarcinoma; originated from the dorsal part of pancreas divisum. To our knowledge this is the first report of pancreatic carcinoma associated with annular pancreas coexistent with pancreas divisum.     

Prevalence and clinical evolution of isolated ventral pancreatitis in alcoholic chronic pancreatitis

BACKGROUND: The role of pancreas divisum in chronic pancreatitis is controversial. Ductal changes limited to the pancreatic ventral duct (isolated ventral pancreatitis) have only been described as isolated case reports. METHODS: In a cohort of 542 patients with chronic pancreatitis we determined the frequency of ventral chronic pancreatitis among patients with pancreas divisum and analyzed the clinical presentation of 10 patients with isolated ventral alcohol-induced pancreatitis compared with 10 patients with isolated dorsal alcohol-induced pancreatitis and 30 patients with chronic pancreatitis and without pancreas divisum. Magnetic resonance pancreatography under secretin stimulation was used to evaluate the status of the dorsal pancreatic duct in some patients during follow-up. RESULTS: We identified 62 (11.4%) patients with pancreas divisum and 16 (2.9%) patients with incomplete pancreas divisum. The ventral duct was affected by chronic pancreatitis in 74% of these patients. Isolated ventral or dorsal duct alterations were identified in 14% and 26%, respectively, of patients with pancreas divisum. Patients with isolated ventral pancreatitis had pain symptoms similar to those of the two other groups but had no endocrine or exocrine insufficiency on initial presentation. After a mean follow-up of 44 months, 83% of patients studied with isolated ventral pancreatitis developed alterations of the dorsal ductal part of the gland. CONCLUSION: We conclude that isolated ventral alcohol-induced pancreatitis is one of the first manifestations of generalized pancreatic disease where the anatomic factor of pancreas divisum plays only a marginal role.     

Ventral pancreatectomy associated with segmental duodenectomy including the major papilla

During embryonic development, the head of the pancreas comprises ventral and dorsal primordia. The embryological fusion plane between the ventral and dorsal primordia reportedly separates the adult pancreas into the ventral and dorsal pancreas. The duct of Wirsung drains the ventral pancreas and terminates in the major papilla, while the duct of Santorini drains the dorsal pancreas and terminates in the minor papilla. However, complete resection of the ventral pancreas is difficult and impractical because the lower bile duct is buried in ventral pancreatic parenchyma and resection may lead to postoperative ischemic necrosis of the duodenum, particularly around the major papilla. We have therefore performed ventral pancreatectomy associated with segmental duodenectomy including the major papilla in 3 cases with intraductal papillary mucinous neoplasm that involved only the duct of Wirsung.     

An annular pancreas derived from paired ventral pancreata, supporting Baldwin's hypothesis

An annular pancreas is a rare malformation. It is generally accepted that the ring formation originates from a single ventral pancreas, as suggested by Lecco. No reports of resected and/or autopsied annular pancreata derived from paired ventral pancreata, thus supporting Baldwin's hypothesis, have been published. We describe an annular pancreas originating from paired ventral pancreata, thus supporting Baldwin's hypothesis, and attempt to clarify the pathogenesis of an annular pancreas. The patient was a 1-day-old Japanese male newborn, born at 32 weeks of pregnancy. He died from respiratory failure owing to esophageal atresia the next day. Autopsy incidentally revealed an annular pancreas that was examined histologically. Multiple 4 microm thick serial sections were obtained from paraffin-embedded pancreatic tissue. Sections for histologic analysis were subjected to hematoxylin-eosin staining and pancreatic polypeptide immunostaining. An unusually large pancreatic duct encircled by pancreatic tissue ran around the duodenum, and the duct was confirmed to flow into the major papilla. The islets of the encircling pancreas were positive for pancreatic polypeptide. A normal main and accessory pancreatic duct were also identified. Histologic and immunohistochemical evaluation revealed that the ring formation originated from the left lobe of paired ventral pancreata. An annular pancreas that was investigated histopathologically and immunohistochemically and found to support Baldwin's hypothesis is described.     

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.     

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.     

Total pancreas divisum caused by ventral pancreas malrotation showing biliary pancreatitis

A 52-year-old man was admitted to our hospital complaining of abdominal pain. A blood test showed high serum levels of biliary enzymes and amylase. Surprisingly, a computed tomography scan revealed complete separation of the ventral and dorsal pancreas and swelling of the ventral pancreas with choledocholithiasis. Surgical cholecystectomy was performed to remove the bile duct stones after endoscopic removal was unsuccessful. The complete separation of the pancreatic parenchyma caused by ventral pancreas malrotation seen in this case is extremely rare, and may provide important information regarding embryologic development, deformity, and malfunction of the pancreas.     

Annular pancreas associated with pancreatolithiasis: a case report

We present a case of annular pancreas associated with pancreatolithiasis. A 41 year-old Japanese man with epigastric pain was admitted to the surgical service at Miyazaki Medical College Hospital. Contrast duodenography revealed severe stenosis of the descending duodenum. Cholangiography showed a stenotic segment of the intrapancreatic common bile duct surrounded by calcifications. Computed tomography of the abdomen revealed calcifications in the posterior region of the pancreatic head. Percutaneous cannulation of the pancreatic ductal system, using ultrasonic guidance, demonstrated a slightly dilated pancreatic duct in the body, stenosis of the duct of Wirsung in the pancreatic head, a normal duct of Santorini, and calcifications in the duct of an annular pancreas which communicated with the duct of Wirsung. At surgery, the second portion of the duodenum was completely encircled by the annular pancreas, and a Whipple procedure was performed. Including this patient, 170 adult cases of annular pancreas have been reported in Japan since 1922. Surgery was performed on 122 patients; 106 of these procedures were well documented. A Whipple procedure was performed on 16 patients, including the present case. Nine of these 16 patients had associated malignant disease, while the others had benign pancreatic disease. This is the fifth reported case of pancreatolithiasis associated with an annular pancreas in Japan. This case emphasizes that an annular pancreas may predispose to localized chronic pancreatitis and pancreatolithiasis.     

Intraductal papillary-mucinous adenoma developed in the ventral pancreas in a patient with pancreas divisum

A 34-year-old man was admitted to our hospital with the chief complaints of back pain and epigastralgia. The physical examinations on admission disclosed no abdominal tumor. The serum concentration of total bilirubin was 1.4?mg/dl. The serum elastase-1 level was elevated to 526?ng/dl. Computed tomography showed a cystic lesion, 1?cm in diameter, in the head of the pancreas, without dilatation of the main pancreatic duct. Endoscopic retrograde cholangiopancreatography via the papilla of Vater and the accessory papilla revealed an enlarged ventral pancreatic duct and pancreas divisum. The preoperative diagnosis was mucin-producing pancreatic tumor in the ventral pancreas of a patient with pancreas divisum. A pylorus-preserving pancreatoduodenectomy was performed. The gross findings of the cut surface of the resected specimen disclosed mural nodules in the dilated duct of the ventral pancreas. A histological examination of the mural nodules in the ventral pancreas revealed mucin and intraductal papillary adenoma. Benign tumors associated with pancreas divisum are rare; to the best of our knowledge, only three cases have been reported. Although in these three patients the tumor developed in the dorsal pancreas, the tumor developed in the ventral pancreas in our patient.     

Clinical Features of Patients with Pancreas Divisum

Abstract: Between 1972 and 1992, 18, 128 ERCPs were performed. Pancreas divisum (PD) was diagnosed in 184 of these patients (1.02%). The clinical features of 108 cases were investigated (M53, F55, average age 54) who were encountered during the last 8 years. In a morphological study, we examined changes in both the ventral and dorsal ducts. An ERP of the dorsal pancreas demonstrated severe changes (SE) in 2 of 73 patients (3%), moderate changes (MO) in 4 (5%) and minimal changes (MI) in 17 (23%). When the ventral pancreas was examined, SE and MO occurred in one each of the 89 patients (1%), and MI in 5 (6%). The rate of ductal abnormalities in the dorsal pancreas (32%) was significantly higher than the rate in the ventral pancreas (8%). However, the dorsal duct changes did not correlate with alcoholism. The exocrine or endocrine function in the majority of the patients with PD was normal or slightly disturbed. An investigation of abdominal pain revealed that patients who complained of pancreatic-type pain were 33%, and this rate was higher than the rate in the control group without PD (12.8%). In conclusion, it was suggested that PD was related to mild dorsal pancreatitis. (Dig Endosc 1994; 6: 80–86)     

Clinical relevance of an unfused pancreatic duct system.

In man, the main pancreatic duct is normally derived from ventral and dorsal embryological buds of the pancreas. In a minority of people, failure of fusion of the two buds results in separate drainage of the dorsal and ventral pancreas, so that the accessory duct provides the main drainage for the gland. Patients with this anomaly demonstrated at endoscopic retrograde pancreatography (ERP) have been investigated to assess whether non-fusion of the main pancreatic duct predisposes to the development of pancreatitis. A failure of fusion of the pancreatic ducts was seen in 21 out of 449 (4.7%) successful pancreatograms; four of these 21 patients had definite clinical evidence of pancreatitis and two patients had possible pancreatic disease, but in the remainder the anomaly was not considered to be clinically relevant. An abnormal pancreatogram suggesting pancreatitis was present in 116 out of the 428 patients (27.1%) with a normally fused duct system. The anomaly was found as frequently in the whole series as it was seen in patients with pancreatitis. These findings suggest that embryological failure of pancreatic duct fusion does not predispose to the development of pancreatitis. However, the presence of this anomaly may lead to misinterpretation of ultrasonographic and CT scan findings.     

Mucin-producing adenoma associated with pancreas divisum and hepatic hilar carcinoma: An autopsy case

We present the autopsy case of an 82-year-old Japanese woman with a mucin-producing adenoma accompanied by pancreas divisum and a hepatic hilar carcinoma. She had suffered from a cholangiocellular carcinoma at the hepatic hilus for 2 months, which was treated with radiation and chemotherapy. She did not complain of any abdominal pain. Obstructive jaundice deteriorated despite percutaneous transhepatic bile duct drainage, and she died of hepatic insufficiency. At autopsy, a hepatic tumor was confirmed to have caused severe obstructive jaundice. Histological examinations showed moderately to poorly differentiated cholangiocellular adenocarcinoma with squamous metaplasia, probably due to radiation. A yellowish mucinous tumor was found in the head of the pancreas near the minor papilla. It consisted of multiple rice-sized cystic lesions with thin septa. Although it had no capsule, its margin was clear. Neither a wide opening of the major or minor papilla nor mucous drainage was observed. Gross examinations revealed unfused pancreatic ducts. The slightly dilated dorsal duct and a branch of the mildly dilatated ventral duct showed tumor involvement. Histological examinations showed mild atypia of the epithelia, and this pancreatic tumor was diagnosed as branch duct-type mucin-producing adenoma with postradiation dysplasia. The combination of a mucin-producing tumor and pancreas divisum is rare, and this is only the third reported case.     

Embryology of the pancreatic duct system

BACKGROUND/AIMS: It has been suggested that the distal portion of the dorsal pancreatic duct and the ventral pancreatic duct usually merge into the main pancreatic duct, and the proximal portion of the dorsal pancreatic duct becomes the accessory pancreatic duct. In this study, we investigated the embryology of the accessory pancreatic duct roentgenographically and immunohistochemically. METHODS/RESULTS: The accessory pancreatic duct shows two different patterns in pancreatograms: the long and the short type. The accessory pancreatic duct of the long type forms a straight line and joins the main pancreatic duct at the neck portion of the pancreas. The accessory pancreatic duct of the short type joins the main pancreatic duct near its first inferior branch. Long inferior branches from the accessory pancreatic duct were found in 74.6% with the long type, significantly more often than in the short type (29. 3%). Patency of the long type was (74.5%) significantly greater than in the short type (36.2%). Immunohistochemically, we found the main pancreatic duct between the junction with the accessory pancreatic duct and the neck portion of the autopsy pancreas in the short type was located within the ventral pancreas, characterized by pancreatic polypeptide-rich islets. CONCLUSION: The long type represents a continuation of the main duct of the dorsal primordium. The short type is very likely formed by the proximal main duct of the dorsal primordium and its long inferior branch, with the main duct of the dorsal primordium at the point of connection with the main duct of the ventral primordium being obliterated and replaced by this additional communication.     

Clinical and radiological findings in dominance of Santorini's duct

BACKGROUND/AIMS: Few studies on Santorini's duct dominance, in which the ventral pancreatic duct is narrower than and anastomoses with Santorini's duct have been performed. We examined clinical and radiological findings in cases characterized by dominance of Santorini's duct. METHODS: We reviewed 3,800 cases of endoscopic retrograde cholangiopancreatography. Clinical and pancreatographic findings including caliber, course, terminal shape, and patency of Santorini's duct were examined in cases of Santorini's duct dominance. RESULTS: Twenty-nine cases were diagnosed as Santorini's duct dominant. Chronic pancreatitis, acute relapsing pancreatitis, pancreatic-type pain, and hyperamylasemia not associated with obvious pancreatitis were observed in 3, 1, 5, and 6 cases, respectively. Cholangiopancreatographic findings indicated congenital choledochal cyst (n = 2), branch fusion between the ventral and dorsal pancreatic ducts (n = 23), and normal pancreatic duct system characterized by a straight course through the body and tail to join the ventral pancreatic duct in the neck portion of the pancreas (n = 4). Regarding terminal shapes of Santorini's duct, cudgel type (n = 9) and spindle type (n = 8), which showed frequent patency, were observed significantly more frequently than in controls. Patency of Santorini's duct was observed in 90% (17/19). CONCLUSIONS: Many Santorini's duct-dominant cases exhibited branch fusion between the ventral and dorsal pancreatic ducts. Although Santorini's duct functions well in most cases in which it is dominant, pancreatitis or pancreatic-type pain occurs in half of such cases due to relative impairment of function of the minor duodenal papilla.     

Congenital anomaly of pancreas divisum as cause of obstructive pain and pancreatitis.

Pancreas divisum occurs when the embryological ventral and dorsal parts of the pancreas fail to fuse, so that pancreatic drainage is mainly through the accessory papilla. In 169 patients with primary biliary tract disease who underwent pancreatography incidental to endoscopic cholangiography, the incidence of pancreas divisum was 3.6%. Among 78 patients with unexplained recurrent pancreatitis, the incidence was 25.6%. The hypothesis that the duct anomaly can cause obstructive pain and pancreatitis is presented, and supported by a detailed analysis of 47 patients with the condition.     

Polysplenia syndrome with congenital agenesis of dorsal pancreas presenting as acute pancreatitis and the role of endoscopic ultrasonography in its diagnosis

A 49-year-old female was admitted to our hospital for acute pancreatitis. The abdomen CT scan incidentally showed midline liver with hepatomegaly, centrally located gallbladder, pancreas truncation, right sided small bowel, left sided large bowel, interruption of the inferior vena cava with azygos continuation, preduodenal portal vein, and multiple spleens in the left upper quadrant. In MRCP, the head of pancreas was enlarged and short main pancreatic duct without accessory duct was showed. EUS revealed enlarged ventral pancreas with a main pancreatic duct of normal caliber, absence of the accessory pancreatic duct and the dorsal pancreas. She was diagnosed as polysplenia syndrome with agenesis of dorsal pancreas. It is a rare congenital anomaly frequently associated with various visceral anomalies including multiple spleens, impaired visceral lateralization, congenital heart diseases, gastrointestinal abnormalities and azygos continuation of the inferior vena cava. We report a case of polysplenia syndrome with agenesis of dorsal pancreas presenting acute pancreatitis.     

Isolated ventral pancreatitis in a patient with pancreas divisum

A previously healthy 22-yr-old woman was found to have chronic pancreatitis restricted to the ventral pancreas in association with pancreas divisum. When conservative treatment failed, sphincteroplasty of both papillae was performed with good results. This well-documented case represents a clear exception to the commonly held concept that obstruction of the dorsal duct is the essential pathogenic factor in the development of pancreatitis associated with pancreas divisum. The importance of visualizing both the dorsal and ventral pancreatic ducts to direct therapy for patients with idiopathic pancreatitis is discussed. Theories of the pathogenesis of concomitant pancreatitis and pancreas divisum are reviewed.     

ENDOSCOPIC APPROACH TO THE MINOR DUODENAL PAPILLA: SPECIAL EMPHASIS ON ENDOSCOPIC MANAGEMENT ON PANCREAS DIVISUM

Diagnostic and therapeutic approach to the minor duodenal papilla including standardized technique was reviewed. In cases in which a pancreatogram is not achieved or those in which only a small portion of the ductal anatomy is visualized via the major duodenal papilla, cannulation of the minor papilla provides a second route of access to the ductal system. Successful minor papilla cannulation requires meticulous attention to technique. As the orifice of the minor papilla is usually of pinpoint size, needle‐tipped catheters are useful. As minor papilla cannulation in pancreas divisum carries the risk of severe pancreatitis, the procedure should be performed with more caution. In some patients with pancreas divisum, an increased resistance to flow across the small orifice results in dorsal pancreatic duct hypertension and clinical symptoms including acute recurrent pancreatitis, chronic pancreatitis, and pancreatic‐type pain. Pancreas divisum patients with acute recurrent pancreatitis are the best candidates for endoscopic management for dorsal‐duct decompression including endoscopic minor papilla sphincterotomy and stenting.     

Annular pancreas detected in adults, diagnosed by endoscopic retrograde cholangiopancreatography: report of four cases

Four cases of annular pancreas diagnosed by endoscopic retrograde cholangiopancreatography (ERCP) are described and 105 cases of this anomaly in adults in Japan were reviewed. Among 105 cases, abdominal pain was the most frequent symptom. Concerning associated diseases, peptic ulcer was present in 24.8% and pancreatitis in 13.3%. In case 1, duodenal ulcer and pancreatic cyst were noted. Pancreatolithiasis was found in two cases (case 1 and 2). Case 4 presented the clinical features of acute pancreatitis. Out of 105 cases, well-described 26 were divided into six types. The following results were obtained. 1) The most frequent type was that in which the annular duct arose from the duct of Wirsung. 2) The next most frequent type was that in which the main pancreatic duct encircled the duodenum. 3) The other types corresponded to those in which the annular duct arose from the duct of Santorini and the common bile duct. We emphasized that ERCP is the most important procedure to find the characteristic features and to establish the therapeutic strategy in cases of annular pancreas.