INTRODUCTION — Endoscopic retrograde cholangiopancreatography (ERCP) has changed the approach to the diagnosis and management of pancreatic disorders in adults. It remains a less common procedure in children, despite accumulating experience since the mid-1980s in the use of ERCP for a variety of indications. In the last two decades, an increased incidence of acute and chronic pancreatitis has been recognized in the pediatric population [1]. ERCP in children and infants requires a high level of expertise and specialized training. It is now routinely used for therapeutic purposes where the special expertise for performing the procedure in children is available.

The pancreatic disorders that can be evaluated by ERCP are described here. The use of ERCP for biliary disorders in children and the technique, success, and complications of ERCP in children are discussed separately. (See "Endoscopic retrograde cholangiopancreatography (ERCP) for biliary disease in children" and "Endoscopic retrograde cholangiopancreatography (ERCP) in children: Technique, success, and complications".)

UNEXPLAINED ACUTE AND RECURRENT PANCREATITIS — ERCP should be considered in selected patients with clinically significant pancreatitis for which an anatomical (including obstructive) etiology is suspected. Potential indications for ERCP in children with pancreatitis are listed in the table (table 1) [2].

Initial evaluation

●Exclude nonanatomical causes – Prior to considering ERCP, specific nonanatomical causes of pancreatitis should be explored. These include infections, systemic diseases (including systemic inflammatory conditions such as systemic lupus erythematosus, hypertriglyceridemia, hypercalcemia, inflammatory bowel disease affecting the area of the major papilla, and, occasionally, celiac disease and autoimmune pancreatitis), drug-induced pancreatitis, and blunt trauma to the pancreas. In acute recurrent or chronic pancreatitis, genetic causes should be explored through genetic testing for PRSS1 (cationic trypsinogen), SPINK1 (serine protease inhibitor Kazal type 1), CFTR (cystic fibrosis transmembrane conductance regulator), and CTRC (chymotrypsinogen C) gene mutations [3]. (See "Clinical manifestations and diagnosis of chronic and acute recurrent pancreatitis in children" and "Hereditary pancreatitis", section on 'Genetic testing' and "Causes and contributing risk factors for chronic pancreatitis in children and adolescents", section on 'Genetic'.)

●Imaging – Imaging with magnetic resonance cholangiopancreatography (MRCP), abdominal computed tomography (CT), and, sometimes, endoscopic ultrasound (EUS) is important to establish the extent of the pancreatitis and to rule out anatomical conditions that may lead to pancreatitis. The goal is to evaluate the pancreatic duct and pancreatic parenchyma, rule out anatomical conditions that may lead to pancreatitis, and evaluate for pancreatic/peripancreatic fluid collections. MRCP should be performed prior to consideration of ERCP for most patients with chronic or acute recurrent pancreatitis. ERCP is more invasive, and the procedure may trigger pancreatitis. When available, EUS often helps to make a diagnosis prior to considering ERCP. As examples, EUS may identify gallbladder sludge, pancreas divisum, congenital biliary anomalies, duodenal duplication cyst, small biliary or pancreatic stones that may have been missed with other imaging modalities, and chronic pancreatitis [4]. On the other hand, ERCP has the advantage of offering therapeutic interventions, such as sphincterotomy, stone removal, and stent placement, so it is a valuable therapeutic procedure once a treatable condition has been diagnosed by CT, MRCP, or EUS [2]. (See "Clinical manifestations and diagnosis of chronic and acute recurrent pancreatitis in children", section on 'Initial imaging'.)

Indications for ERCP — Pediatric guidelines are lacking, and most of the current indications are extrapolated from the adult literature. ERCP is now considered to be mainly a therapeutic procedure and is rarely used for diagnostic purposes alone.

Special expertise in performing ERCP in infants and children is not widely available. In centers in which this expertise is available, ERCP is often appropriate for treating patients with anatomic pancreatic disorders (table 2), as outlined below. However, the risks and benefits of ERCP depend on individual patient characteristics, including age and associated medical comorbidities, and whether an anatomic abnormality identified on noninvasive testing is likely to be treatable with therapeutic ERCP. Thus, the indications described below apply to centers in which expertise in pediatric ERCP is available and may vary with individual patient characteristics.

First attack of pancreatitis — After a first attack of pancreatitis, the first step is noninvasive testing, typically starting with abdominal ultrasound, followed by pancreatic protocol CT scan or MRCP, using secretin stimulation if available. On occasion, EUS may also be indicated.

●In children in whom noninvasive testing reveals a cause that is potentially treatable via ERCP, it is appropriate to proceed to therapeutic ERCP.

●In children in whom the noninvasive test is unrevealing, expert opinion varies about whether to perform an ERCP after an initial attack of pancreatitis. In our practice, we do not perform an ERCP in such patients, because we believe that the likelihood of finding an endoscopically treatable abnormality is low when noninvasive imaging is normal. Reliable data for estimating the risks and benefits of each of these approaches are not available.

The relative merits of ERCP versus MRCP for evaluating common bile duct stones in adults are discussed separately. (See "Choledocholithiasis: Clinical manifestations, diagnosis, and management", section on 'Transabdominal ultrasound' and "Choledocholithiasis: Clinical manifestations, diagnosis, and management", section on 'Additional imaging (MRCP or EUS)'.)

Recurrent pancreatitis — Approximately 10 percent of children with acute pancreatitis will have recurrences [5]. Depending on the age of first episode and underlying etiology, a significant number of patients with acute recurrent pancreatitis will go on to develop chronic pancreatitis [6].

If noninvasive testing (CT or MRCP) and genetic testing is unrevealing, we occasionally recommend proceeding to ERCP. ERCP can identify an underlying anatomic abnormality in up to 75 percent of children with recurrent pancreatitis in whom nonanatomical causes have been excluded [7-17] and in up to 25 percent of those with normal MRCP findings [18]. Various anatomic conditions have been associated with recurrent pancreatitis, which can be categorized as congenital or acquired (table 2). The major diagnoses within these categories will be reviewed below.

ERCP findings and interventions by cause

Congenital anomalies — Several congenital biliary and pancreatic anomalies have been associated with pancreatitis in children, some of which are potentially amenable to therapeutic intervention during ERCP.

Biliary cysts and abnormal pancreaticobiliary junction — Biliary cysts are cystic dilatations that may occur singly or in multiples throughout the intra- and extrahepatic bile ducts. They originally were termed choledochal cysts (involving the extrahepatic bile duct), but the clinical classification was revised in 1977 to include intrahepatic cysts. (See "Biliary cysts" and "Endoscopic retrograde cholangiopancreatography (ERCP) for biliary disease in children".)

A biliary cyst is identified in 6 to 18 percent of cases of acute pancreatitis in children [7-10,12,13,16,17,19]. Most children with biliary cysts and pancreatitis have an anomalous pancreaticobiliary junction (APBJ; also known as an anomalous pancreaticobiliary union) in which the pancreaticobiliary union is located outside the duodenal wall (figure 1A-B). This anomaly has been associated with sphincter of Oddi dysfunction (SOD). The predisposition of patients with APBJ to pancreatitis might be explained by the SOD or might be caused by retrograde flow of bile into the pancreatic duct [20-24]. (See "Clinical manifestations and diagnosis of sphincter of Oddi dysfunction".)

Therapeutic ERCP is helpful for most patients with biliary cysts and pancreatitis. As an example, in a study of nine children with recurrent pancreatitis and APBJ with a long common channel, SOD was suggested by the observation of high basal sphincter pressures. Endoscopic sphincterotomy was successful in preventing further recurrences in 89 percent [21]. Children with a choledochocele (in which the cyst is located within the duodenal wall, also known as type III biliary cyst) may also benefit from sphincterotomy (picture 1 and image 1A-B) [17,25-28]. In other patients with biliary cysts, pancreatitis may be due to pancreatic stones or plugs, which can be removed endoscopically [25]. Type III biliary cysts are treated endoscopically. In patients with biliary cysts with pancreatitis in which a pancreatic stone is found, sphincterotomy with stone removal should be performed before biliary cyst surgical excision in order to prevent further bouts of pancreatitis. Most biliary cysts should also be surgically removed due to long-term risk of malignancy. (See "Biliary cysts".)

A type "long Y" APBJ is not associated with a biliary cyst, but these patients need a cholecystectomy due to an association with gallbladder cancer. (See "Biliary cysts", section on 'Abnormal pancreatobiliary junction and cancer'.)

Pancreas divisum — Pancreas divisum is the most common congenital variant of the pancreas and is caused by failure of fusion of the dorsal and ventral endodermal buds. As a result, each duct drains via its own orifice. The major papilla of Vater drains the ventral duct of Wirsung, while the minor accessory papilla drains the dorsal duct of Santorini (figure 2). Pancreas divisum occurs in approximately 7 percent of individuals in autopsy series [29]. (See "Pancreas divisum: Clinical manifestations and diagnosis".)

The association between pancreas divisum and pancreatitis is controversial since many patients with pancreas divisum (identified during ERCP for indications other than pancreatitis or in autopsy studies) do not have a history of pancreatitis. Indeed, only approximately 5 percent of individuals with pancreas divisum develop pancreatic symptoms (see "Pancreas divisum: Clinical manifestations and diagnosis", section on 'Clinical manifestations'). However, the rate of pancreas divisum is somewhat higher among patients with recurrent pancreatitis than in the general population. As an example, pancreas divisum has been found in approximately 11 percent of 296 children with recurrent pancreatitis reported in the published literature [7,8,10-13,16,17,30,31]. In our own experience with 272 consecutive successful ERCPs performed for a variety of indications, pancreas divisum was found in nine (3.3 percent) [32]. The prevalence was considerably higher (12 percent) among the 50 children older than one year who had recurrent pancreatitis. These observations suggest but do not prove that pancreas divisum is associated with recurrent pancreatitis in children, but they also confirm that most patients with pancreas divisum do not develop pancreatic disease.

Endoscopic treatment of pancreas divisum is generally reserved for patients whose symptoms are recurrent and disabling. The treatment typically consists of sphincterotomy of the minor papilla, with placement of a stent. Clinical improvement with such treatment has been observed in up to 75 percent of patients [12,17,33]. The results are less favorable in children with evidence of chronic pancreatitis in the dorsal pancreas. Individuals with pancreas divisum but no dilation of the dorsal duct (image 2) are more likely to respond to endoscopic therapy than those with pancreas divisum and a dilated dorsal duct (image 3 and picture 2). (See "Treatment of pancreas divisum".)

A study from the INSPPIRE consortium (International Study group of Pediatric Pancreatitis: in search for a cuRE) reported that pancreas divisum was present in 52 of 359 (14.5 percent) subjects with acute recurrent or chronic pancreatitis, acting independently from risk factors for hereditary pancreatitis [34]. ERCP was most helpful if obstructing pancreatic duct stones were present.

Other pancreatic congenital anomalies — Case reports have described an association between annular pancreas (image 4) and recurrent pancreatitis in children [17,35,36]. However, whether there is a causal relationship remains uncertain since many of the reported cases of annular pancreas (in adults and children) had coexistent pancreas divisum [35]. Several other anomalies have been described in association with pancreatitis including "short pancreas" (image 5), agenesis of the dorsal pancreas, and cystic dilation of the pancreatic duct (pancreatocele) (image 6) [17,37].

Duodenal duplication cysts — Case reports have described pancreatitis in association with a duodenal duplication cyst, presumably due to intermittent obstruction of the pancreatic duct [38,39]. ERCP can be helpful for establishing the diagnosis and for definitive treatment [38]. If the cyst is bulging into the intestinal lumen, a wide cystoduodenostomy can be endoscopically performed with excellent results [40]. The appearance of a duplication on EUS is described separately. (See "Endoscopic ultrasound for the characterization of subepithelial lesions of the upper gastrointestinal tract".)

Acquired diseases — The main acquired diseases associated with acute pancreatitis in children are viral or parasitic infections, biliary pancreatitis, certain medications, systemic inflammatory conditions, and, possibly, SOD.

Parasitic infestation — Pancreatitis is occasionally seen as a complication of infestation with Ascaris lumbricoides (image 7) or other parasites among children living in endemic areas. Cryptosporidiosis can invade the biliary and pancreatic ducts from the intestine in immunodeficient patients, leading to pancreatitis (and biliary duct disease) [41,42]. In most cases, the pancreatitis is caused by migration of the worm into the bile duct, causing transient obstruction of the papilla of Vater. Less commonly, the worm migrates into the main pancreatic duct, causing recurrent pancreatitis [43] or, rarely, necrotizing pancreatitis [44]. Worms can sometimes be removed endoscopically [45].

Sphincter of Oddi dysfunction — The hypothesis that SOD may cause acute recurrent pancreatitis in adults is being increasingly questioned. Therefore, interventions designed to evaluate and address this mechanism, including endoscopic manipulations, manometry, and sphincterotomy (biliary and/or pancreatic), should be approached with great caution.

SOD was reported in 12 percent of children (17 of 139) with recurrent pancreatitis described in a series of published reports [12,17,19,33]. In children with recurrent pancreatitis in which CT, MRCP, EUS, and genetic testing have been unrevealing, we perform ERCP, and if this is normal, we typically perform sphincter of Oddi manometry (SOM). No pediatric-specific standards have been developed for SOM, so results are evaluated using adult standards, in which basal pressures >40 mmHg are considered abnormal [46]. Because of the lack of pediatric-specific standards for SOM and the risk of triggering acute pancreatitis, some experts do not perform this test in children. (See "Clinical manifestations and diagnosis of sphincter of Oddi dysfunction", section on 'Sphincter of Oddi manometry'.)

An association between SOD and recurrent pancreatitis in children has not been established, and limited information is available about outcomes of sphincterotomy in these patients. If SOM reveals an elevated sphincter pressure in a child with recurrent pancreatitis, sphincterotomy leads to clinical improvement in a majority of patients. Biliary sphincterotomy alone may not be sufficient to treat such patients, presumably because the pancreatic sphincter is not transected [33,47]. Thus, dual sphincterotomy may be required [17,48]. However, the safety and efficacy of this approach has not been well studied. Either manometry or sphincterotomy can trigger an episode of acute pancreatitis; the risk is probably higher after pancreatic than biliary sphincterotomy [33,49]. In one series, 20 to 30 percent of children developed pancreatitis after endoscopic sphincterotomy for suspected SOD [50]. (See "Treatment of sphincter of Oddi dysfunction" and "Post-endoscopic retrograde cholangiopancreatography (ERCP) pancreatitis".)

HIV infection — Few publications are available on pancreatic involvement in children with acquired immunodeficiency syndrome (AIDS). Pancreatitis is a recognized complication of HIV infection in children and adults. Most cases are associated with use of didanosine or other medications [51-53]. Opportunistic infections may involve the pancreas [51]. The most common are Cytomegalovirus and Cryptosporidium, followed by Pneumocystis jirovecii (carinii), Toxoplasma gondii, and Mycobacterium avium. ERCP may permit ductal decompression in children who have developed strictures due to infections [54].

Autoimmune pancreatitis — Autoimmune pancreatitis is an infrequently recognized disorder of presumed autoimmune etiology that is associated with characteristic clinical, histologic, and morphologic findings [55]. It can occur as a primary pancreatic disorder or in association with other disorders of presumed autoimmune etiology, including inflammatory bowel disease. The disorder is mostly recognized in adults but occasionally reported in children [56,57]. (See "Autoimmune pancreatitis: Clinical manifestations and diagnosis" and "Causes and contributing risk factors for chronic pancreatitis in children and adolescents", section on 'Autoimmune'.)

Autoimmune pancreatitis is highly suspected based on imaging findings (MRCP, CT, and/or EUS) and confirmed by pancreatic biopsy (although this is rarely needed). ERCP is not indicated for diagnosis, but it is occasionally used for temporary biliary stent placement during treatment with corticosteroids (image 8) [57].

OTHER INDICATIONS FOR ERCP

Chronic pancreatitis — Risk factors for chronic pancreatitis can be grouped according to their pathophysiology (table 3); these contributors are termed "risk factors" rather than "causes" because chronic pancreatitis may be the result of more than one pathophysiologic event. (See "Causes and contributing risk factors for chronic pancreatitis in children and adolescents", section on 'Overview of risk factors'.)

Chronic calcific pancreatitis is identified by an imaging study such as a computed tomography (CT) scan. Stones can be localized in the pancreatic parenchyma with normal duct anatomy, can be intraductal, or can be present in both locations. Parenchymal stones are difficult to treat, and extracorporeal shock wave lithotripsy or surgery may be needed if the patient is symptomatic. Ductal stones can be treated with ERCP and retrieval devices like baskets or balloons.

Genetic etiologies of chronic pancreatitis (PRSS1, SPINK, CTRC, and others) affect the acinar cell, leading to premature activation of trypsinogen, which cannot be inactivated, either due to changes in the trypsinogen configuration or a malfunctioning of the inhibitory mechanism. Other mutations such as CFTR affect the ductal system, leading to obstruction and pancreatitis. Early in the clinical presentation, genetic etiologies do not appear to cause a calcific phenotype. As the disease progresses, calcifications can develop and are most frequently seen in patients with PRSS1 mutations. Early-onset pancreatitis is strongly associated with PRSS1 or CTRC mutations, and there is frequently a family history of pancreatitis [58].

Some of the specific causes of chronic pancreatitis can be identified with laboratory evaluation, genetic tests, and noninvasive imaging including magnetic resonance cholangiopancreatography (MRCP). An approach to the evaluation and diagnosis of chronic pancreatis is discussed separately. (See "Clinical manifestations and diagnosis of chronic and acute recurrent pancreatitis in children".)

Diagnostic ERCP — Since the advent of MRCP and endoscopic ultrasound (EUS), ERCP is no longer performed for diagnostic purposes.

When ERCP is performed for chronic pancreatitis, the earliest changes involve the side branches of the main pancreatic duct and include dilatation, contour irregularity, clubbing, and stenosis [7,11,17,33,59]. These changes may be accompanied by mild dilatation of the main pancreatic duct. As the disease progresses, the involvement of the main pancreatic duct increases, producing more marked dilatation, irregularity of the walls, and areas of stenosis or occlusion with intraluminal calcifications (image 9).

Therapeutic ERCP — Patients with chronic pancreatitis, abdominal pain, and a dilated main pancreatic duct are candidates for a decompression procedure. Decompression can be accomplished by therapeutic ERCP or by a variety of surgical procedures. The role of endoscopic versus surgical intervention has not been well established in either adults or children. Management in children is based on expert opinion, guided by indirect evidence from adults with chronic pancreatitis and a few small case series in children.

A role of endoscopic therapy in the treatment of chronic pancreatitis is evolving. The only established indication for therapeutic ERCP in children with chronic pancreatitis is for treating abdominal pain [60]. ERCP may be used to remove intraductal stones, dilate strictures, place stents, and perform pancreatic sphincterotomy, maneuvers that can improve drainage of the pancreatic duct and lessen the pain (image 10 and image 9). Multiple reports in children have suggested that these approaches can be performed safely in expert hands and may relieve symptoms, at least in the short term [2,14,17,25,33,50,61]. For example, 301 children with acute recurrent pancreatitis or chronic pancreatitis were enrolled in the INSPPIRE study (INternational Study group of Pediatric Pancreatitis: In search for a cuRE) [62]. One hundred and seventeen children (38.9 percent) underwent at least one therapeutic ERCP. The procedure was more commonly performed in children with chronic pancreatitis compared with those with acute recurrent pancreatitis (65.8 versus 13.5 percent, p<0.0001). Utility of therapeutic ERCP was reported to be similar in both groups (53 versus 56 percent, p = 0.81). At present, endoscopic therapy should only be performed in specialized centers, preferably in the context of a clinical study.

A small case series suggests a role for surgical intervention in selected children. In a series of 37 children with chronic pancreatitis, those managed by surgical therapy had a lower rate of recurrent pancreatitis and hospitalization as compared with those managed by endoscopic therapy [63]. Some of the patients in this series responded to surgical therapy after failing therapeutic ERCP.

A retrospective single-center case series described ERCPs in 38 children and adolescents, most of whom had chronic pancreatitis [64]. A total of 158 ERCPs were performed with a seven-year follow-up. The post-ERCP complication rate was 3 percent. The severity, frequency of subsequent pancreatitis episodes, and use of analgesia all significantly decreased (p<0.001) after the procedure, with only one child requiring subsequent surgery. These findings suggest that ERCP can be safe and effective for the treatment of chronic pancreatitis in appropriately selected children, where expertise in this technique is available.

Some experts have proposed that ERCP and stenting can be used as a therapeutic trial to assess possible benefit of a surgical duct drainage procedure. As an example, a two-stage management protocol was used in a small series of six children [65]. The patients were first treated with ERCP with pancreatic stenting, and all had relief of pain. Therefore, all of the subjects proceeded to surgical decompression, using longitudinal pancreatojejunostomy (Puestow procedure). All six subjects reported markedly reduced episodes of pain after surgery.

Thus, the very limited evidence available suggests that both endoscopic and surgical therapy have a role in the management of children with chronic pancreatitis and abdominal pain. Larger and randomized studies are needed to determine optimal selection of patients for either of these interventions. Most experience in surgical procedures for chronic pancreatitis is in adult patients, as described separately. (See "Chronic pancreatitis: Management", section on 'Endoscopic therapy'.)

Pancreatic and peripancreatic fluid collections — Pancreatic pseudocysts and walled-off necrosis are commonly seen in the setting of acute, acute recurrent, and chronic pancreatitis and in areas of pancreatic trauma. Associated symptoms include abdominal pain and obstructive symptoms from extrinsic gastric or duodenal compression, leading to postprandial vomiting. While most collections resolve spontaneously, symptomatic collections need drainage procedures.

Symptomatic pancreatic and peripancreatic fluid collections have traditionally been drained surgically or percutaneously. Endoscopic methods have developed as a less invasive alternative to surgical and percutaneous drainage, so that surgical drainage is rarely performed. These methods include cystogastrostomy, cystoduodenostomy, and transpapillary drainage (image 11) [25,60]. In adults, successful pseudocyst resolution has been reported in approximately 80 percent of cases. Results have been similar in children, although experience is limited to a few case reports and case series [66-69]. A combined EUS-ERCP procedure has been successfully performed in children with pseudocysts [70]. (See "Endoscopic interventions for walled-off pancreatic fluid collections".)

Pancreatic trauma — There is considerable variation in management of patients with traumatic pancreatitis and associated ductal injury. Options range from a conservative approach with observation and supportive management, to ERCP with placement of a pancreatic stent, to an aggressive surgical approach.

In patients with traumatic pancreatitis, ERCP can identify the presence and location of duct leakage and may allow endoscopic therapy or identify the need for surgery [71-73]. Some experts perform ERCP as early as the first day after the injury, while others reserve it for traumatic pancreatitis that does not show signs of resolving within one week of the injury [73,74]. Patients with a normal pancreatogram can be treated conservatively, while those with duct disruption can be treated with an intrapancreatic stent bypassing the disrupted site, thereby reserving surgery for those in whom stenting is unsuccessful [72,75].

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Endoscopic retrograde cholangiopancreatography (ERCP)".)

SUMMARY

●Endoscopic retrograde cholangiopancreatography (ERCP) should be considered for patients with pancreatitis for which an anatomical cause is identified radiologically (using magnetic resonance imaging/magnetic resonance cholangiopancreatography [MRI/MRCP], computed tomography [CT], or endoscopic ultrasound [EUS]) and might be treated endoscopically. Potential indications for ERCP in children with pancreatic disease are listed in the table (table 1). (See 'Unexplained acute and recurrent pancreatitis' above.)

●Prior to considering ERCP, specific nonanatomical causes of pancreatitis should be excluded. These include infections, metabolic diseases, systemic conditions, drug-induced pancreatitis, and blunt trauma. In recurrent or chronic pancreatitis, genetic etiologies should be explored. Imaging with abdominal CT, MRCP, or EUS is important to evaluate the pancreatic duct and pancreatic parenchyma, rule out anatomical conditions that may lead to pancreatitis, and evaluate for pancreatic/peripancreatic fluid collections. (See 'Initial evaluation' above and "Clinical manifestations and diagnosis of chronic and acute recurrent pancreatitis in children".)

●Expert opinion varies about whether to perform an ERCP after an initial attack of pancreatitis in children in whom the noninvasive test is unrevealing. In some centers, ERCP is performed in such patients. In other centers, ERCP is performed only if the pancreatitis is recurrent. These indications apply only to centers in which expertise in pediatric ERCP is available and may vary with individual patient characteristics. (See 'Indications for ERCP' above.)

●In 6 to 18 percent of cases of acute pancreatitis in children, a biliary cyst is responsible. Most such children have an anomalous pancreaticobiliary junction (APBJ) in which the pancreaticobiliary union is located outside the duodenal wall (figure 1A-B). This anomaly may be associated with sphincter of Oddi dysfunction (SOD). Therapeutic ERCP is helpful for most of these patients, but most will also require surgical excision of the cyst. However, a type "long Y" APBJ is not associated with a biliary cyst. These patients need a cholecystectomy due to an association with gallbladder cancer. (See 'Biliary cysts and abnormal pancreaticobiliary junction' above.)

●Pancreas divisum occurs in approximately 7 percent of individuals. It is more common among children with recurrent pancreatitis. However, most patients with pancreas divisum do not develop pancreatitis. Endoscopic treatment of pancreas divisum is reserved for patients whose symptoms are recurrent and disabling. (See 'Pancreas divisum' above.)

●Other congenital anomalies that may be associated with pancreatitis include annular pancreas, "short pancreas," cystic dilation of the pancreatic duct (pancreatocele), and duodenal duplication cysts. (See 'Other pancreatic congenital anomalies' above and 'Duodenal duplication cysts' above.)

●SOD is reported in approximately 10 percent of children with recurrent pancreatitis, but a causal association has not been established. The role of SOD as a cause of acute recurrent pancreatitis in adults is being increasingly questioned, and endoscopic interventions for diagnosis or treatment of this purported disorder should be approached with great caution. The diagnosis is traditionally established by performing manometry during ERCP. Sphincterotomy of both the biliary and pancreatic sphincters improves symptoms in some but not all patients with abnormal manometry results. However, sphincterotomy performed for this purpose frequently triggers an episode of acute pancreatitis. (See 'Sphincter of Oddi dysfunction' above.)

●In children with chronic pancreatitis, the most common indication for therapeutic ERCP is abdominal pain. ERCP may be used to remove intraductal stones, dilate strictures, place pancreatic stents, and perform pancreatic sphincterotomy, maneuvers which can help decompress the pancreatic duct and lessen the abdominal pain. (See 'Chronic pancreatitis' above.)

●Endoscopic treatment of symptomatic pancreatic/peripancreatic fluid collections or pancreatic trauma is based on experience in adults. These disorders also are amenable to endoscopic treatment in children. Appropriate patient selection and a high level of expertise in therapeutic ERCP are essential. (See 'Pancreatic and peripancreatic fluid collections' above and 'Pancreatic trauma' above.)

ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Moises Guelrud, MD, who contributed to an earlier version of this topic review.