Etiology of recurrent acute pancreatitis,with special emphasis on pancreaticobiliary malformation

PurposeThis study was aimed to investigate etiology and clinical profiles of recurrent acute pancreatitis (RAP), particularly from the morphology of the pancreaticobiliary duct system.Material and methodsPancreaticobiliary morphology was examined in 230 of 381 patients with acute pancreatitis (AP) using endoscopic retrograde cholangiopancreatography. We analyzed factors associated with RAP including the pancreaticobiliary duct system.ResultsRAP was diagnosed in 74 patients (19%). Major etiologies of RAP were alcoholic (38%), idiopathic (26%) and pancreaticobiliary malformation (22%). Patients with alcoholic RAP were significantly younger (47.2±11.6 years) than those with gallstone RAP (67.3±16.8; p<0.05). RAP with pancreaticobiliary malformation (male-to-female ratio: 1:4.3; p<0.01) and gallstone RAP (1:1.7; p<0.05) occurred predominantly in females in comparison with alcoholic RAP (1:0.2). Recurrence rate was 80% for AP with pancreaticobiliary malformation, significantly higher than for the others (p<0.01). Pancreas divisum was suspected as the etiology of mild RAP in 7 patients. Four RAP patients with pancreas divisum underwent endoscopic minor papilla sphincterotomy and improved. Pancreaticobiliary maljunction with biliary dilatation (choledochal cyst) was suspected as the etiology of mild RAP in 3 patients. The 3 RAP patients with choledochal cyst underwent prophylactic flow diversion surgery with complete resection of the dilated common bile duct, and achieved improvement. High confluence of pancreaticobiliary ducts was suspected as the etiology of mild RAP in 6 patients.ConclusionPancreaticobiliary malformation is one of the major causes of RAP. As some of them benefit from endoscopic or surgical treatment, morphology of the pancreaticobiliary duct system should be examined where possible in RAP patients.     

alpha-Smooth muscle actin immunoreactivity may change in nature in interlobular fibrosis of the pancreas in patients with congenital biliary dilatation

Pancreatic fibrosis in patients with congenital biliary dilatation (CBD) or choledochal cyst was studied to determine why biliary pancreatitis seldom progresses to chronic pancreatitis/more progressive state. Pancreatic collagenization in eight patients (three adults with pancreatoduodenectomy and five children with biopsy of the pancreas performed when excising the cyst) with CBD was evaluated histopathologically and immunohistochemically. Interlobular and periductal fibrosis with both collagen Type I and Type III immunoreactivities was found in six out of eight cases and in all four cases in which the pancreatic duct was included, respectively. The interlobular area was seldom immunoreactive for α-smooth muscle actin (α-SMA), a marker for myofibroblasts, but was usually positive for CD34, a human progenitor cell antigen. In contrast, the periductal area was usually immunoreactive for α-SMA, but usually negative for CD34 and immunopositive for bcl-2, indicating a continuously progressive state of fibrosis, in which 'pre-existing'α-SMA immunoreactivity in the interlobular area may change in nature and lead to CD34-positive fibrosis or apoptosis. In conclusion, biliary pancreatitis is not likely to evolve into chronic pancreatitis/more progressive state because 'pre-existing'α-SMA immunoreactivity in the interlobular area may change in nature.     

Fetal choledochal cyst diagnosed at 22 weeks of gestation by three-dimensional ultrasonography: a case report

Choledochal cyst is a cystic or fusiform dilatation of the extra- or intrahepatic bile duct that has rarely been reported in prenatal cases. Here we report a fetus with choledochal cyst diagnosed prenatally by three-dimensional (3-D) ultrasonography at 22 weeks of gestation. We demonstrated an image of choledochal cyst by using a new ultrasound technique, a 3-D multislice view. After close intrauterine followup, surgery was successfully performed and postoperative course was uneventful.     

Experience of congenital choledochal cyst in adults:treatment, surgical procedures and clinical outcome in the Second Affiliated Hospital of Harbin Medical University

This study was undertaken to analyze and evaluate the diagnosis and principal treatment methods for congenital choledochal cyst, focusing on various surgical procedures and clinical outcome. A comprehensive, retrospective study was conducted on 72 adult patients who presented with choledochal cyst from 1985 to 2002. Surgical procedures were cyst excision with hepaticojejunostomy in 25 cases for type I or type IV-B, extrahepatic cyst excision with hepaticojejunostomy in 8 cases for type IV-A, extrahepatic cyst excision with modified hepaticojejunostomy in 2 cases for type IV-B, non-cyst excision with or without hepaticojejunostomy in 27 cases for types I, II, IV-A, IV-B. The early postoperative morbidity and mortality rate were 16.1% (9/62) and 6.5% (4/62) respectively, and the complication rate related to surgical procedure was 30.6% (19/62). The incidence of cholangiocarcinoma with non-cyst excision or non-operated congenital choledochal cyst was 10.8% (4/37). One patient died of primary hepatocellular carcinoma after cyst excision with hepatojejunostomy. In conclusion, our results showed that complete excision of choledochal cyst for types I, II, and IV-B and complete excision of extrahepatic choledochal cyst from the hepatic hilum in type IV-A with hepaticojejunostomy or modified hepaticojejunostomy are the treatment of choice for choledochal cyst in adult patients.     

Management strategy for congenital choledochal cyst with co-existing intrahepatic dilation and aberrant bile duct as well as other complicated biliary anomalies

The purpose of this study was to investigate and discuss imaging methods and management strategies for congenital choledochal cyst with co-existing intrahepatic dilation and aberrant bile duct as well as other complicated biliary anomalies. In this study we reviewed and analyzed 72 patients with congenital choledochal cyst, ranging in age from 15 days to 12 years old and who were seen at our hospital during the past 12 years, from January 1993 to October 2005. The image manifestation and clinical significance of patients with co- existing intrahepatic biliary dilation and aberrant bile duct were carefully examined during operation via MRCP, cholangiography and choledochoscope. Twenty-two cases (30.1%) presented with intrahepatic bile duct dilation and 12 of these were of the cystic type. That is, the orifice of the dilated intrahepatic tract that converged into the common hepatic duct showed membrane or septum-like stenosis. In 10 cases the dilation tapered off from the porta hepatis to the initiating terminals of the intra-hepatic bile ducts and was not accompanied by stenosis. An aberrant bile duct was observed in 2 of the cases. In 3 cases, the right and left hepatic ducts converged at the choledochal cyst. In conclusion, the imaging methods for intrahepatic bile duct dilation possess important clinical significance. Further, for hepatojejunostomy with radical excision of a choledochal cyst, additional operative procedures for intrahepatic stenosis, possible bile duct malformation and pancreaticobiliary common duct calculi can potentially reduce postoperative complications.     

Adenomyoma of common bile duct arising in a type I choledochal cyst

Adenomyoma can be misdiagnosed as an adenocarcinoma, leading to needless and extensive surgical resections. A 45-year-old woman presented with right hypochondrial pain. Magnetic resonance imaging showed a choledochal cyst. Excision of choledochal cyst with Roux-en-Y hepaticojejunostomy was performed. A segment of dilated common bile duct and an attached nodule was received. Sections from the choledochal cyst showed a cyst wall composed of dense fibrous tissue lined by partially ulcerated columnar epithelium. Sections from the nodule showed interlacing whorls of smooth muscle bundles with entrapped glands. The glands were lined by cuboidal to columnar cells without nuclear atypia. This was recognized as an adenomyoma. To the best of our knowledge, this is the first reported case in which an adenomyoma was found associated with a type 1 choledochal cyst. A review of the existing literature and discussion of theories of genesis and the diagnostic pitfalls are presented.     

慢性胰腺炎CT影像解剖学分型及其临床意义

目的 根据慢性胰腺炎CT表现的不同,探讨慢性胰腺炎的CT影像解剖学分型及分型的意义.方法收集1996年1月至2009年1月入住我院经临床或手术证实的213例慢性胰腺炎患者,其中男性156例,女性57例,平均年龄为(48.0±11.5)岁;平均住院天数(18.4±9.2)d;胆系疾病68例(32%),反复发作急性胰腺炎3...     

Choledochal cysts and multilocular cysts of the pancreas

Choledochal cyst is a rare congenital anomaly of the biliary system that may be associated with other abnormalities of the hepatobiliary tract. We report a case of an 11-year-old boy in whom the preoperative evaluation revealed a choledochal cyst and intraoperative cholangiopancreatography showed a cystic mass in the pancreas. Examination of the choledocho-pancreatico-duodenectomy specimen showed a multilocular cyst in the pancreas in addition to a segmental dilation of the common bile duct. The findings in our case adds pancreatic cyst to the spectrum of abnormalities associated with choledochal cyst and may also support the theory that choledochal cyst is the predominant abnormality in a widespread spectrum of the pancreatobiliary duct dysplasia.     

Adenosquamous carcinoma in a congenital choledochal cyst associated with pancreatico-biliary maljunction

Congenital choledochal cyst is occasionally complicated by carcinomatous transformation, mostly adenocarcinoma. Adenosquamous carcinoma arising in a congenital choledochal cyst is very rare. The author herein reports an adenosquamous carcinoma arising in congenital choledochal cyst associated with pancreatico-biliary maljunction. A 34-year-old man with congenital choledochal cyst and recurrent cholangitis had been followed up, and was admitted to hospital to undergo testing for cancer. Imaging modalities including computed tomography, magnetic resonance imaging and endoscopic retrograde cholangiography showed an elevated lesion in the choledochal cyst. Because clinical cytology of bile indicated malignant cells, pancreatico-duodenectomy, cholecystectomy, and resection of the choledochal cyst were performed. Grossly, the choledochal cyst was type I, and its size was 8 × 10 cm. Anomalous pancreatico-biliary ductal union was recognized. An elevated lesion was recognized in the choledochal cyst. Histologically, the lesion was composed of a squamous cell carcinoma element and an adenocarcinoma element; a gradual transition was recognized between the two. The squamous cell carcinoma element contained microcytic cells with mucins. On immunohistochemistry the adenocarcinoma element and microcytic cells were positive for CEA, but the squamous cell carcinoma element was negative for CEA. Both elements were positive for CA19-9. Ki-67 labeling was 53% in the adenocarcinoma element and 48% in the squamous cell carcinoma element. p53 protein was negative in both elements. At the time of writing, the patient was alive after 25 months without recurrence or metastasis. The present case is the second case of adenosquamous carcinoma arising in congenital choledochal cyst in the English-language literature.     

Pancreaticobiliary maljunction and choledochal cysts: from embryogenesis to therapeutics aspects

Pancreaticobiliary maljunction (PBM) and choledochal cysts (CC) are rare and little-known diseases. Several definitions have been proposed for the PBM, but the most widely accepted is an excessive length of the common pancreaticobiliary duct due to the abnormal convergence of the pancreatic and biliary ducts out of the duodenal wall. This anomaly, thought to develop during embryogenesis, is associated with a loss of regulation of the Oddi’s sphincter leading to a pancreaticobiliary or biliopancreatic backflow. This reflux could be responsible, or associated with cystic dilatation of the bile ducts and biliary tract cancers, to various biliary or pancreatic events such as cholangitis or pancreatitis. For the diagnosis of PBM, magnetic resonance cholangiopancreatography has now become the gold standard as a noninvasive imaging tool. However, the main risk of PBM is the development of bile duct cancer, most often on a distended area. PBM without CC increase the occurrence of gallbladder cancer and require a preventive cholecystectomy. Surgical treatment of PBM with concomitant CC is more complex and depends on localization of the dilatation(s) as reported in the Todani’s classification. This review describes the pathogenesis, embryogenesis, clinical features, investigation and management of PBM and CC.     

Duct‐obstructive pancreatitis with granulocytic epithelial lesion in a patient with ulcerative colitis: An atypical manifestation of type 2 autoimmune pancreatitis?

Type 2 autoimmune pancreatitis (AIP) typically presents with diffuse or focal enlargement of the pancreas; however, its diverse clinical presentation has not yet been clarified. We herein described a 46‐year‐old man with a 1‐month history of ulcerative colitis who presented with imaging features of a mass‐like lesion in the pancreatic body with upstream duct dilatation and serum CA19‐9 elevation. He underwent laparoscopic distal pancreatectomy with splenectomy for suspected malignancy. Histologically, the area radiologically suspected to be duct dilatation consisted of necrotic tissue, in which the disrupted main pancreatic duct was involved. The area radiologically suspected to be the mass lesion showed features of pancreatitis without discrete mass. In addition, several ducts showed neutrophilic duct injury similar to granulocytic epithelial lesions observed in type 2 AIP. Immunohistochemistry revealed the aberrant expression of IL‐8 in the pancreatic ductules and infiltrating CD3‐positive T‐lymphocytes, findings recently identified in type 2 AIP. The present case is not typical for either type 2 AIP or other known conditions, but extreme examples of type 2 AIP may present with ductal obstruction because of severe neutrophilic duct injury. IL‐8 immunostaining may also assist in establishing a diagnosis of type 2 AIP with an atypical presentation.     

Choledochal cysts: our ten year experience

We present our experience in the management of choledochal cysts from 1999 to 2009. A retrospective review of all charts with a diagnosis of choledochal cysts in our institution in this ten-year period. Data was collated using Excel. A total of 17 patients were diagnosed with choledochal cyst: 9 females and 8 males. The average age at diagnosis was 28 months (range from 0 to 9 years). The most common presenting symptoms were obstructive jaundice 6 (35%) and abdominal pain and vomiting 4 (23%). Ultrasound (US) was the initial diagnostic test in all cases with 4 patients requiring further investigations. All patients underwent Roux-en-Y Hepaticojejunostomy. The average length of stay was 11 days. Patients were followed up with Liver Function Tests (LFTS) and US 4-6 weeks post-operatively. Three patients developed complications including post-op collection, high drain output requiring blood transfusion and adhesive bowel obstruction. Our overall experience with choledochal cyst patients has been a positive one with effective management and low complication rates.     

Case Review of Impacted Bile Duct Stone at Duodenal Papilla: Detection and Endoscopic Treatment

Purpose

A bile duct stone impacted at the duodenal papilla is an urgent condition that can rapidly lead to either suppurative cholangitis or acute pancreatitis due to almost complete obstruction of the bilio-pancreatic outflow. This study evaluated the clinical characteristics and results of endoscopic treatment for a bile duct stone impacted at the duodenal papilla.

Materials and Methods

Forty-six patients who had been diagnosed with an impacted papillary stone were retrospectively reviewed.

Results

The typical features of acute cholangitis (Charcot''s triad) and pancreatitis were only observed only in 10 patients (21.7%) and 17 patients (37.0%), respectively. After the endoscopic retrograde cholangiopancreatography, 30 patients (65.2%) were found to have a solitary stone impacting the duodenal papilla and 16 patients had one or more stones in the bile duct. On the radiological studies, the former patients were associated more commonly with no visible stone or no bile duct dilatation (p < 0.05). All impacted papillary stones were successfully removed by endoscopic sphincterotomy: 23 by a needle knife and 23 by a pull type papillotome. The procedure-related complications (n = 7, 4 bleeding, 3 pancreatitis) were not serious and did not differ, based on endoscopic findings and the procedure used.

Conclusion

A bile duct stone impacted at the duodenal papilla requires both clinical and radiographic evidence to support the diagnosis. Endoscopic sphincterotomy, either with a needle knife or a pull type papillotome, was safe and effective for removing the impacted papillary stone.     

THE CHOLEDOCHO-PANCREATICO-DUCTAL JUNCTION IN INFANTILE OBSTRUCTIVE JAUNDICE DISEASES

A histopathological study on how the common bile duct and main pancreatic duct open into the duodenum was performed on autopsied cases of infantile obstructive jaundice diseases, and control cases. The so-called "common channel" formation type (Type III) was divided into two types; the junction of the common bile duct and the pancreatic duct was situated in the mucosal or submucosal layer of the duodenum in type IIIa and the junction below the propria muscularis of the duodenum in type IIIb. Type IIIb was identified in 17 out of 28 cases in congenital biliary atresia and in 2 cases of congenital choledochal cyst, while the control cases all belonged to type IIIa. In cases of type IIIb the well-developed sphincter muscle was located in the submucosal layer and it surrounded the common channel after joining of both ducts suggesting the possibility of free communication of both ducts. As the intraductal pressure of pancreatic duct is normally higher than that of the bile duct, reflux of pancreatic juice may occur into the bile duct. Abnormal choledocho-pancreaticoductal junction was suggested to be a pathogenic factor which causes infantile obstructive jaundice diseases.     

阑尾间置重建胆道治疗先天性胆总管囊肿

介绍一种治疗先天性胆总管囊肿的新术式。囊肿切除后用阑尾代胆道,并采用抗返流吻合——胆道阑尾十二指肠吻合术(BAD)。治疗4例先天性胆总管囊肿患儿,随访6月至3年,效果满意。提出该手术具有较高的临床实用价值。     

胰胆管合流部解剖与急性胰腺炎关系的MRCP分析

目的探讨急性胰腺炎的产生与胰胆管合流部解剖的关系。方法回顾分析83例急性胰腺炎患者的MRCP片,对胰胆管合流部进行测量和分析比较;以同期139例非急性胰腺炎患者的MRCP片作为对比分析。结果急性胰腺炎组的共同通道长度明显大于对照组(P0.01);前者胰管与胆管汇合处角度也明显大于后者(P0.01);两组的肝外胆管直径和胰管直径无显著差别(P0.05)。结论急性胰腺炎的发生与胰胆管合流部的解剖密切相关,共同通道过长、胰胆管汇合角度过大是造成急性胰腺炎的重要因素。     

Acute pancreatitis

Acute pancreatitis is characterized by the occurrence of necroinflammatory changes in the pancreas. Three types of necrosis may be distinguished: (1) interstitial tissue necrosis, which subsequently may also involve acinar and ductal cells, (2) ductal necrosis, and (3) acinar necrosis. The first type of necrosis is autodigestive in nature and is typical of the most common forms of acute pancreatitis, which are associated with alcohol, bile duct disease, metabolic conditions, and other rare factors. Clinically, these types of pancreatitis may be either mild or severe (Atlanta classification). The mild form is also known as edematous pancreatitis, because there is edematous swelling of the pancreas combined with tiny foci of interstitial (fat) necrosis. Severe or necrotizing pancreatitis shows large areas of often hemorrhagic necrosis of the pancreatic and particularly the peripancreatic tissue. The ductal type of necrosis is rare and may be seen in pancreatitis associated with prolonged circulatory failure. The acinar type of necrosis is caused by infectious agents. Complications of acute pancreatitis, such as pseudocyst, bleeding, and infection, determine the course of the disease.     

Clinicopathologic and experimental studies on cases of abnormal pancreatico-choledocho-ductal junction

An abnormal pancreatico-choledocho-ductal junction, in which free communication between the pancreatic duct and the common bile duct was present, was observed in 17 out of 120 cases (14.2%) of biliary tract carcinoma and in all of four cases of congenital biliary dilatation, but in noe of about 200 control patients without biliary tract disease. Outstanding findings in the pancreas, such as degeneration of the ductal epithelium and interlobular fibrosis, were found in 6 of 21 cases of abnormal junction. In an experimental study using 63 puppies given choledocho-pancreatic end-to-side ductal anastomosis, dilatation of the bile duct of varying degree occurred and mucosal hyperplasia of the biliary tract was observed in 12 of 25 anastomosed dogs for a duration of more than one month. Cell kinetics in biliary tract mucosa observed by the use of monoclonal antibody against bromodeoxyuridine (BrdU) was obviously elevated in dogs subject to long-term follow-up. As for pancreatic lesions, the incidence and histologic findings were similar to those of human studies, as follows: chronic pancreatitis in only a few animals, mild periductal parenchymatous change in 15 puppies and no significant change in 44 puppies. Therefore, an abnormal pancreatico-choledocho-ductal junction may be one of the significant factors producing biliary dilatation, biliary tract carcinoma and pancreatitis.