Surgical treatment of pancreas divisum causing pancreatitis in children

Although controversial, pancreas divisum has been implicated as a cause of acute pancreatitis when there is stenosis of the accessory papilla that drains the duct of Santorini. Over the past 5 years, four children with pancreas divisum and recurrent pancreatitis were successfully treated surgically. The diagnosis was made by endoscopic retrograde cholangiopancreatography (ERCP) in each case. Surgical treatment included sphincteroplasty to the accessory papilla to improve drainage of the duct of Santorini, opening the ampulla of Vater to expose the ostium of the duct of Wirsung to enlarge it, and cholecystectomy.     

Pancreas divisum and the dominant dorsal duct syndrome

Pancreas divisum, the most frequent congenital malformation of the pancreas, results from the absence of embryologic fusion of the dorsal and ventral pancreatic ducts which keep an autonomy of drainage. The dorsal pancreatic duct is dominant and drains the major part of the pancreatic fluid through a non adapted accessory papilla. The high prevalence of pancreas divisum in patients presenting recurrent acute pancreatitis, the presence of obstructive pancreatitis electively located on the dorsal pancreatic duct and the results of the treatments targeted on the accessory papilla are the arguments pleading for the pathogenic character of the pancreas divisum. Currently, the diagnosis of pancreas divisum is based on magnetic resonance imaging. For symptomatic patients (after exclusion of patients with intestinal functional disorders), results of endoscopic sphincterotomy or surgical sphincteroplasty are favourable in 75% of patients with recurrent acute pancreatitis. They are worse in patients with chronic pain. Surgical sphincteroplasty must be discussed in the same manner as the endoscopic treatment for sometimes avoiding multiplication of the procedures.     

Pancreas divisum: Its association with pancreatitis

Thirty-three patients with pancreas divisum studied by endoscopic retrograde cholangiopancreatography (ERCP) are described. Documented pancreatitis was present in fifteen patients, and another eleven had recurrent episodes of pain typical of pancreatitis. The major papilla was cannulated in all patients, but the duct of Wirsung was opacified in only twenty-eight and showed changes of pancreatitis in one. Attempts were made to cannulate the minor papilla in fifteen of the thirty-three patients and were successful in four. The duct of Santorini showed typical changes of pancreatitis in one. One patient had pancreatic cancer, and the duct of Wirsung demonstrated only nonspecific abnormalities. In only two cases was pancreatitis due to alcohol abuse. The high incidence of pancreatitis and pancreatic-like pain in patients with pancreas divisum, may be due to the very small ampulla of the duct of Santorini which in these patients drains the majority of the pancreas, creating a marked relative stenosis of the ampulla. Surgery for relief of pain was required in five patients. The operation of choice, when pancreatitis involves the dorsal pancreas, appears to be distal resection with drainage.     

Incomplete pancreas divisum with anomalous choledochopancreatic duct junction with choledochal cyst.

The coexistence of incomplete pancreas divisum, an anomalous choledochopancreatic duct junction, and a choledochal cyst is an extremely rare condition, described in only 3 patients in the available medical literature. The symptoms may be similar to any of these 3 distinct pathological conditions. Magnetic resonance cholangiopancreatography or endoscopic retrograde cholangiopancreatography is usually required for diagnosis. Management of symptomatic pancreas divisum may be accomplished with open accessory duct sphincteroplasty or endoscopic papillotomy with or without stenting. Treatment of choledochal cyst is by complete excision of the cyst whenever possible, with hepaticodochoenterostomy being the treatment of choice. Here, we describe a patient with this complex disorder who was successfully managed with endoscopic minor duct papillotomy with accessory pancreatic duct stenting and resection of the choledochal cyst with hepaticodochojejunostomy.     

Pancreatitis associated with pancreas divisum: results of surgical intervention

The embryologic defect that results when the ventral and dorsal anlages of the pancreas do not fuse has been referred to as pancreas divisum. The patients who present with recurrent pancreatitis or pancreatitis-like pain in association with pancreas divisum should be investigated to determine the presence of minor papilla stenosis. In the present study of 11 patients, 9 became asymptomatic with surgical decompression of the dorsal pancreatic duct. Two surgical failures were related to wrong diagnosis and restenosis of the sphincteroplasty. A delay in the clearance of dye from the dorsal pancreatic duct after endoscopic retrograde cholangiopancreatography was utilized to determine minor papilla stenosis. In the future, secretin ultrasound tests should prove of value in assessing minor papilla stenosis. Once the diagnosis is made, sphincteroplasty of the minor papilla is the most logical procedure because of the good results obtained. In the present study, pancreaticojejunostomy provided good duct decompression as well.     

Dorsal duct sphincterotomy is effective long-term treatment of acute pancreatitis associated with pancreas divisum

Nonbiliary, nonalcoholic pancreatic inflammatory disease was investigated by biochemical investigation, ultrasonography, endoscopic retrograde cholangiopancreatography, and secretin tests. Twenty-five consecutive cases were followed up for 12 months to 10 years after treatment of disease associated with pancreas divisum, diagnosed by endoscopic retrograde cholangiopancreatography. Thirteen patients had no recurrence of acute pancreatitis after dorsal duct sphincterotomy alone, during long-term follow-up (mean, 54 months); one patient had recurrent pancreatitis during 33 months after failed sphincterotomy. Eight patients had variable results 12 months to 8 years (mean, 49 months) after dorsal duct sphincterotomy for pancreatic pain syndrome (without amylase elevation), three were pain free, and one had recurrent pancreatitis. For 10 years after dorsal duct sphincterotomy for chronic pancreatitis, one patient had no pain relief; after subtotal pancreatectomy and pancreaticojejunostomy of the dorsal duct, both for chronic pancreatitis, one patient each was pain free and normoglycemic after 54 and 12 months, respectively. Dorsal duct sphincterotomy alone is successful in achieving long-term freedom from recurrence of acute pancreatitis associated with pancreas divisum. Pancreatic pain syndrome is not consistently improved by dorsal duct sphincterotomy. Chronic pancreatitis associated with pancreas divisum should be treated by resection or drainage procedures, not by dorsal duct sphincterotomy.     

Evaluation and treatment of the dominant dorsal duct syndrome (pancreas divisum redefined)

The controversial association of pancreatitis and pancreas divisum was studied in 100 patients (77 women, 23 men, median age 35) with episodic acute pancreatitis (49%) or "pancreatic pain" (51%). Seventy-one had classic pancreas divisum (type 1); 23 had only a dorsal duct with an absent Wirsung's duct (type 2); and 6 had a filamentous connection between the two duct systems (type 3). Accessory papilla sphincteroplasty was performed in 88 patients, with a mean follow-up of 53 months. The orifice was stenotic at the mucosal level in 66 patients. Seventy percent of patients have shown improvement: 85% if the accessory papilla was stenotic, compared with 27% if it was not (p less than 0.0001); and 82% with discrete attacks, compared with 56% with chronic pain (p = 0.002). Judged against intraoperative calibration of accessory papilla orifice diameter, ultrasonography with secretin stimulation was 78% sensitive for accessory papilla stenosis, with 3% false-positive results. Ultrasonography with secretin stimulation was the best predictor of surgical success: positive = 92% success (attacks or pain) versus negative = 40% success (64% with attacks; 21% with pain). There have been seven restenoses with six reoperations. We conclude that (1) pancreas divisum is but one variety of pancreatic anatomy characterized by a dominant dorsal duct and dependence on secretion through the accessory papilla; (2) accessory papilla stenosis appears to be a necessary cofactor to produce a morbid state, whether episodic pancreatitis or pancreatic pain; (3) presentation with pancreatitis and a positive result on the ultrasound-secretin test are the best predictors of successful accessory papilla sphincteroplasty.     

Intraductal papillary-mucinous tumor of the pancreas head with complete absence of the ventral pancreatic duct of Wirsung

A case of intraductal papillary mucinous tumor of the pancreas with complete absence of the ventral pancreatic duct of Wirsung is presented. A 74-year-old Japanese man was admitted to our hospital because of elevated serum amylase concentration. Abdominal computed tomography (CT) scanning revealed diffuse dilatation of the main pancreatic duct and a diffuse and uncircumscribed area with heterogeneous density in the pancreas head. Endoscopic retrograde cholangiopancreatography revealed that the main pancreatic duct was connected with an accessory papilla and was diffusely dilated, without any irregularity of the duct wall being observed in the entire length of the duct. The common bile duct was detected only by cannulation through Vaters papilla, and no pancreatic duct or its communicating branch was found. Some branches, directed to the dorsal portion of the pancreas head, were found arising from the accessory pancreatic duct. Intraductal ultrasound examination performed through the accessory papilla and the common bile duct revealed a small tumor with a heterogeneous echo level in the pancreas head. From these findings, intraductal papillary-mucinous tumor (IPMT) occurring in the pancreas head was diagnosed, and pylorus-preserving pancreaticoduodenectomy was performed. The resected specimen revealed IPMT in the pancreas head. A roentgenographic study of the resected specimen revealed a defect caused by the tumor located in the pancreatic duct connected with the accessory papilla and showed that there was complete absence of the pancreatic duct connected with Vaters papilla. Surgical resection enabled us to completely analyze the duct system of pancreas divisum. Although it is not known whether there is a relationship between the pathogenesis of IPMT and embryological anomaly of the pancreatic duct system, this case may provide an insight into the pathogenesis of IPMT.     

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 1.4mg/dl. The serum elastase-1 level was elevated to 526ng/dl. Computed tomography showed a cystic lesion, 1cm 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.     

Santoriniplasty in the management of symptomatic pancreas divisum.

OBJECTIVE: To assess the long-term results of sphincteroplasty of the minor papilla in a series of patients with symptomatic pancreas divisum. DESIGN: Retrospective study. SETTING: University hospital, UK. SUBJECTS: 6 consecutive patients with recurrent acute pancreatitis thought to be caused by pancreas divisum. INTERVENTIONS: Surgical enlargement of the minor papilla of Santorini (Santoriniplasty). MAIN OUTCOME MEASURES: The effectiveness of the procedure in preventing further episodes of acute pancreatitis and controlling chronic intermittent pancreatic pain, outside documented attacks of pancreatitis. RESULTS: Median follow up was 4 years (range: 1-6). The procedure was effective in preventing further episodes of recurrent acute pancreatitis (in all patients) but not uniformly good in controlling chronic pancreatic pain (good: 2/6, moderate: 1/6, poor: 3/6). CONCLUSION: The prevention of further attacks of acute pancreatitis by Santoriniplasty indicates that a short term favourable clinical outcome may be achieved by improving drainage of the pancreatic duct through the stenosed minor papilla. Poor pain control may indicate early parenchymal changes or that unsuitable patients had been selected for surgical intervention. At present, there are no objective tests of pancreatic function that can predict which patients are most likely to benefit from Santoriniplasty. Prospective studies with more patients followed up for longer periods of time are needed before the role of the surgical drainage in symptomatic pancreas divisum can be clearly defined.     

Pancreas divisum, pancreatic pseudocyst, and choledochal cyst in an 8-year-old child

An 8-year-old American Indian girl presented with acute pancreatitis. Ultrasonography and computerized tomography (CT) showed two large cystic masses near the head of the pancreas. During 4 weeks of complete bowel rest and total parenteral nutrition, she had multiple exacerbations of pancreatitis without resolution of the cysts. There were no symptoms of biliary ductal obstruction. Endoscopic cholangiopancreatography (ERCP) showed an 8-cm pancreatic pseudocyst, pancreas divisum, and a 10-cm choledochal cyst. The junction of the common bile duct and the pancreatic duct of Wirsung had a normal relationship. She underwent a Roux-en-Y pseudocyst-jejunostomy and an accessory sphincteroplasty. Three months later, a choledochal cyst excision was performed with biliary reconstruction. The patient is well and asymptomatic 1 year later. This case suggests that pancreatic juice reflux into the common bile duct may not be the only etiology of choledochal cyst formation.     

False pancreas divisum. Acquired pancreatic duct obstruction simulating the congenital anomaly.

Eight patients with recurrent acute pancreatitis were found by ERCP to have foreshortening of the duct of Wirsung simulating the radiographic appearance of the congenital anomaly pancreas divisum. In contrast to 44 patients with true pancreas divisum, who were predominantly young (mean 32 years), nonalcoholic (42/44), and female (34/44), patients with false pancreas divisum were older (mean 48 years), male (7/8), and alcoholic (5/8). False pancreas divisum is most often an acquired obstructing lesion resulting from irremediable injury to the pancreatic duct during acute pancreatitis, often associated with pseudocyst formation and subsequent healing by scar at the site of duct injury. Appreciation of the radiographic differences between pancreatograms in true and false pancreas divisum and the different findings at operation allow for proper selection of therapy. Whereas accessory papilla sphincteroplasty appears to be effective for recurrent pancreatitis associated with true pancreas divisum, false pancreas divisum requires distal pancreatectomy or pancreaticojejunostomy to overcome the irreversible obstruction of the main pancreatic duct.     

The cause and treatment of pancreatitis associated with pancreas divisum

Recurrent pancreatitis is more prevalent in the 4% of people with pancreas divisum (nonfused dorsal and ventral ducts), and it has been proposed that the pancreatitis is caused by stenosis at the orifice of the dorsal duct. We have treated 40 patients with pancreas divisum and proven or probable pancreatitis. The diagnoses were made by endoscopic pancreatography showing a foreshortened (less than 6 cm) ventral duct (Wirsung) and confirmed by postoperative pancreatograms showing the separate main duct (Santorini) emptying via the accessory papilla. Of these, 32 patients (25 men, 7 women, median age 30) had recurrent acute pancreatitis (22) or persistent pain (10) without chronic inflammation or fibrosis. Twenty-nine have been treated by transduodenal sphincteroplasty of the accessory papilla; 22 were stenotic (0.75 mm or less) and 7 nonstenotic. Among 25 patients observed for longer than 6 months after surgery, the relief of pain and pancreatitis has been good in 17, fair in 1, and poor in 7. There was no difference between accessory papillotomy alone (10-0-3) v papillotomies of both accessory and major papillae (7-1-4). Patients with stenosis (16-1-1) fared better (p less than 0.001) than those without stenosis (1-0-6). Those presenting with discrete attacks (12-1-2) also fared better (p less than 0.05) than those presenting with chronic pain (5-0-5). The other eight patients (two women, six men, median age 28) had chronic pancreatitis proven by pancreatography and surgical biopsy. In this group, treatment by sphincteroplasty of the accessory papilla failed, and seven patients eventually required a pancreaticojejunostomy (3), distal pancreatectomy (2), or total pancreatectomy (2). In pancreas divisum, pancreatitis is caused by stenosis at the accessory papilla of Santorini. There may be progression from recurrent acute pancreatitis to irreversible fibrosis in some cases. Sphincteroplasty is effective for recurrent acute pancreatitis, but ductal drainage or resection becomes necessary once chronic pancreatitis is established. A preoperative test for stenosis of the accessory papilla is needed to identify patients whose symptoms are genuinely caused by their pancreas divisum.     

胰管开口炎性病变导致的慢性阻塞性胰腺炎的诊断和外科治疗

目的 探讨胰管开口部位炎性病变导致的慢性阻塞性胰腺炎的诊断和外科治疗方式.方法 对我院自2002年1月至2010年11月收治的28例慢性阻塞性胰腺炎患者的临床资料进行回顾性总结.其中13例患者血清淀粉酶和脂肪酶升高伴有反复急性腹痛,经影像学检查显示胰管全程扩张改变,外科探查明确诊断为胰管开口部位炎性病变导致的慢性阻塞性胰腺炎.对此13例患者的临床表现、诊断方法、手术探查发现和治疗方法进行分析,并对术后的状况包括疼痛复发、生活质量、胰腺的影像学变化和血清胰腺酶学进行随访观察.结果 13例患者均具有典型的慢性阻塞性胰腺炎的临床表现,但12例患者的影像学表现有别于十二指肠乳突、壶腹或胰腺导管内肿瘤导致的慢性阻塞性胰腺炎的图像特征,手术探查和影像学揭示多数患者的胆胰共同通道过短或存在胰腺分裂畸形,对12例患者实施十二指肠乳突、壶腹及胰管开口切开成形术或副乳突切开成形术,术后随访结果显示均未再伴有胰腺酶学升高的急性腹痛发作.结论 以胰管扩张为主而不伴有胆管慢性梗阻是胰管开口炎性病变导致的慢性阻塞性胰腺炎的影像学特征,十二指肠乳突炎症或副乳突炎症时容易在过短的胆胰共同通道或胰腺分裂畸形的患者中引发胰管开口的狭窄而发生慢性阻塞性胰腺炎,纠正胰管开口狭窄、改善胰管引流的局部成形术是简单、有效的治疗方法.     

胰管开口炎性病变导致的慢性阻塞性胰腺炎的诊断和外科治疗

目的 探讨胰管开口部位炎性病变导致的慢性阻塞性胰腺炎的诊断和外科治疗方式.方法 对我院自2002年1月至2010年11月收治的28例慢性阻塞性胰腺炎患者的临床资料进行回顾性总结.其中13例患者血清淀粉酶和脂肪酶升高伴有反复急性腹痛,经影像学检查显示胰管全程扩张改变,外科探查明确诊断为胰管开口部位炎性病变导致的慢性阻塞性胰腺炎.对此13例患者的临床表现、诊断方法、手术探查发现和治疗方法进行分析,并对术后的状况包括疼痛复发、生活质量、胰腺的影像学变化和血清胰腺酶学进行随访观察.结果 13例患者均具有典型的慢性阻塞性胰腺炎的临床表现,但12例患者的影像学表现有别于十二指肠乳突、壶腹或胰腺导管内肿瘤导致的慢性阻塞性胰腺炎的图像特征,手术探查和影像学揭示多数患者的胆胰共同通道过短或存在胰腺分裂畸形,对12例患者实施十二指肠乳突、壶腹及胰管开口切开成形术或副乳突切开成形术,术后随访结果显示均未再伴有胰腺酶学升高的急性腹痛发作.结论 以胰管扩张为主而不伴有胆管慢性梗阻是胰管开口炎性病变导致的慢性阻塞性胰腺炎的影像学特征,十二指肠乳突炎症或副乳突炎症时容易在过短的胆胰共同通道或胰腺分裂畸形的患者中引发胰管开口的狭窄而发生慢性阻塞性胰腺炎,纠正胰管开口狭窄、改善胰管引流的局部成形术是简单、有效的治疗方法.     

胰管开口炎性病变导致的慢性阻塞性胰腺炎的诊断和外科治疗

目的 探讨胰管开口部位炎性病变导致的慢性阻塞性胰腺炎的诊断和外科治疗方式.方法 对我院自2002年1月至2010年11月收治的28例慢性阻塞性胰腺炎患者的临床资料进行回顾性总结.其中13例患者血清淀粉酶和脂肪酶升高伴有反复急性腹痛,经影像学检查显示胰管全程扩张改变,外科探查明确诊断为胰管开口部位炎性病变导致的慢性阻塞性胰腺炎.对此13例患者的临床表现、诊断方法、手术探查发现和治疗方法进行分析,并对术后的状况包括疼痛复发、生活质量、胰腺的影像学变化和血清胰腺酶学进行随访观察.结果 13例患者均具有典型的慢性阻塞性胰腺炎的临床表现,但12例患者的影像学表现有别于十二指肠乳突、壶腹或胰腺导管内肿瘤导致的慢性阻塞性胰腺炎的图像特征,手术探查和影像学揭示多数患者的胆胰共同通道过短或存在胰腺分裂畸形,对12例患者实施十二指肠乳突、壶腹及胰管开口切开成形术或副乳突切开成形术,术后随访结果显示均未再伴有胰腺酶学升高的急性腹痛发作.结论 以胰管扩张为主而不伴有胆管慢性梗阻是胰管开口炎性病变导致的慢性阻塞性胰腺炎的影像学特征,十二指肠乳突炎症或副乳突炎症时容易在过短的胆胰共同通道或胰腺分裂畸形的患者中引发胰管开口的狭窄而发生慢性阻塞性胰腺炎,纠正胰管开口狭窄、改善胰管引流的局部成形术是简单、有效的治疗方法.

Abstract：

Objective To explore the diagnostic methods and reasonable surgical interventions for the chronic obstructive pancreatitis due to the inflammatory lesions at the opening of the pancreatic duct Methods From January 2002 to November 2010 the data of 28 patients who were diagnosed as the chronic obstructive pancreatitis (COP) was retrospectively reviewed. Out of the 28 patients, it was analyzed that the clinical manifestations, diagnostic methods, surgical finding and surgical interventions of the 13 patients who were diagnosed as COP due to the inflammatory lesions at the opening of the pancreatic duct in the exploratory operation accompanying recurrent acute abdominal pain with increased serum amylase and lipase,dilation of entire pancreatic duct on imaging before surgery. The conditions included pain recrudescence,quality of life, pancreatic changes on imaging and the serum amylase and lipase after surgery were recorded.Results All the 13 patients had clinical manifestations of COP. However, 12 patients had different manifestations on imaging from those chronic pancreatitis imaging due to tumors at the duodenal papilla,ampulla or inner pancreatic duct. Via exploratory operation and magnetic resonance cholangiopancreatography (MRCP) , there were short pancreaticobiliary common channel or pancreas divisum existing in most patients. There was no acute abdominal pain with the increased serum amylase and lipase in the 12 patients who receiving the transduodenal mastoid, ampulla and pancreatic ductal opening incision and plasty, the paramastoideus incision and plasty in the visit Conclusions The imaging character of COP due to the inflammatory lesions at the opening of the pancreatic duct is the dilation of the pancreatic duct without the chronic obstruction in the bile duct The patients with short pancreaticobiliary common channel or pancreas divisum easily suffer COP due to the stenosis of the pancreatic ductal opening caused by the duodenal mastoiditis or paramastoiditis. The local plasty surgery to correct the stenosis at the pancreatic ductal opening and improve the drainage of the pancreatic duct is an easy and effective management.     

胰管开口炎性病变导致的慢性阻塞性胰腺炎的诊断和外科治疗

目的 探讨胰管开口部位炎性病变导致的慢性阻塞性胰腺炎的诊断和外科治疗方式.方法 对我院自2002年1月至2010年11月收治的28例慢性阻塞性胰腺炎患者的临床资料进行回顾性总结.其中13例患者血清淀粉酶和脂肪酶升高伴有反复急性腹痛,经影像学检查显示胰管全程扩张改变,外科探查明确诊断为胰管开口部位炎性病变导致的慢性阻塞性胰腺炎.对此13例患者的临床表现、诊断方法、手术探查发现和治疗方法进行分析,并对术后的状况包括疼痛复发、生活质量、胰腺的影像学变化和血清胰腺酶学进行随访观察.结果 13例患者均具有典型的慢性阻塞性胰腺炎的临床表现,但12例患者的影像学表现有别于十二指肠乳突、壶腹或胰腺导管内肿瘤导致的慢性阻塞性胰腺炎的图像特征,手术探查和影像学揭示多数患者的胆胰共同通道过短或存在胰腺分裂畸形,对12例患者实施十二指肠乳突、壶腹及胰管开口切开成形术或副乳突切开成形术,术后随访结果显示均未再伴有胰腺酶学升高的急性腹痛发作.结论 以胰管扩张为主而不伴有胆管慢性梗阻是胰管开口炎性病变导致的慢性阻塞性胰腺炎的影像学特征,十二指肠乳突炎症或副乳突炎症时容易在过短的胆胰共同通道或胰腺分裂畸形的患者中引发胰管开口的狭窄而发生慢性阻塞性胰腺炎,纠正胰管开口狭窄、改善胰管引流的局部成形术是简单、有效的治疗方法.     

Surgical management of recurrent pancreatitis in children with pancreas divisum

OBJECTIVE: To analyze an institutional experience with pancreatitis in childhood to clarify the frequency of pancreas divisum in that patient population, the characteristics of pancreatitis in children with pancreas divisum, and the role of surgical management in their treatment. SUMMARY BACKGROUND DATA: The role of pancreas divisum in causing acute and relapsing pancreatitis and chronic, recurring abdominal pain is controversial. Although the anatomical abnormality is present from birth, most investigators have reported cases with onset of symptoms in adulthood. The reported pediatric experience with this disorder is small, and the natural history of pancreatitis in children with pancreas divisum has not been well elucidated. METHODS: A retrospective chart review of all children 18 years of age and younger with a discharge diagnosis of pancreatitis identified 135 patients treated in the authors' institution from 1978 to 1998. Ten patients were found to have anatomical variants of pancreas divisum associated with recurrent or chronic pancreatitis. The medical records of these patients were reviewed for data on the presentation, diagnostic findings, imaging studies, treatment, surgical findings, and pathologic findings in these children. Chart review and telephone calls were used to assess the current state of health in nine patients available for follow-up. RESULTS: Pancreas divisum was identified in 7.4% of all children with pancreatitis and 19.2% of children with relapsing or chronic pancreatitis. Patients had early onset of recurrent episodic epigastric pain and vomiting, at a mean age of 6 years. Three patients had a positive family history of pancreatitis and one was proven by DNA analysis to have hereditary pancreatitis. Pancreatitis was documented by elevated amylase or lipase levels, and endoscopic retrograde cholangiopancreatography was the method of diagnosis of pancreas divisum in all patients. Eight patients had complete pancreas divisum and two had incomplete variants. Eight patients underwent surgery to improve ductal drainage. Seven underwent transduodenal sphincteroplasty of the accessory papilla, along with sphincteroplasty of the major papilla in two (plus septoplasty in one). Three patients underwent longitudinal pancreaticojejunostomy, as a primary procedure in one patient with midductal stenosis and in two because of recurring pancreatitis after sphincteroplasty. The surgical findings and histologic examination of five patients undergoing distal pancreatectomy revealed striking changes of advanced chronic pancreatitis. Patients responding to sphincteroplasty alone showed less severe histologic changes. Overall, three of seven patients had excellent results, three were improved, and one had continued disabling attacks of pancreatitis. The mean duration of follow-up was 7.3 years, and there were no deaths. No patients had endocrine or exocrine pancreatic insufficiency, and none required chronic analgesics. CONCLUSIONS: Pancreas divisum is an important cause of recurrent pancreatitis in childhood and should be sought aggressively in children with more than one episode of pancreatitis or pancreatitis with a history of chronic recurrent abdominal pain. Surgical intervention is directed toward relief of ductal obstruction and may involve accessory duct sphincteroplasty alone or in combination with major sphincteroplasty and septoplasty. Patients with more distal ductal obstruction or ductal ectasia may benefit from pancreaticojejunostomy.     

Surgical management of pancreas divisum

Pancreas divisum is a congenital anatomic variant characterized by nonunion of dorsal and ventral pancreatic ducts in an otherwise fused pancreas. Of 21 patients with divisum documented by endoscopic retrograde cholangiopancreatography, 6 (28 percent) were found to have no reason other than divisum to account for multiple attacks of pancreatitis. Cholelithiasis was present in one patient, who remains free of recurrent pancreatitis after cholecystectomy only. The remaining five patients underwent surgical treatment directed at pancreas divisum in the belief that stenosis of the duct of Santorini at the entrance into the duodenum is responsible for recurrent attacks of pancreatitis. Four of five have done well with follow-up of 12, 13, 18 and 28 months. Successful sphincteroplasty of the duct of Santorini appears to prevent recurrent attacks of pancreatitis due to pancreas divisum. Pancreaticojejunostomy is reserved for those with markedly dilated ducts secondary to chronic pancreatitis.     

Pancreatographic findings in idiopathic acute pancreatitis

Background/purpose Despite extensive evaluation based on clinical history, biochemical tests, and noninvasive imaging studies, the cause of acute pancreatitis cannot be determined in 10 to 30% of patients, and a diagnosis of idiopathic acute pancreatitis is made. The purpose of this study was to clarify the pancreatographic findings in patients with idiopathic acute pancreatitis.Methods Endoscopic retrograde cholangiopancreatography (ERCP) was performed in 34 patients with idiopathic acute pancreatitis, and the pancreatographic findings were examined. Patency of the accessory pancreatic duct was examined by dye-injection endoscopic retrograde pancreatography (ERP) in 16 of the 34 patients.Results In 11 patients (32%), the following anatomic abnormalities of the pancreatic or biliary system were demonstrated: complete pancreas divisum (n = 5), incomplete pancreas divisum (n = 2), high confluence of pancreaticobiliary ducts (n = 2), choledochocele (n = 1), and giant periampullary diverticulum (n = 1). Pancreatographic findings were normal in 17 patients. Eleven of these patients were examined by dye-injection ERP, and all were found to have nonpatent accessory pancreatic duct.Conclusions Anatomic abnormality of the pancreatic or biliary system is one of the major causes of idiopathic acute pancreatitis. Closure of the accessory pancreatic duct may play a role in the development of idiopathic acute pancreatitis in patients with a normal pancreaticobiliary ductal system.