Endoscopic Retrograde Cholangiopancreatography (ERCP) For Pancreatic Disease in Children - UpToDate

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Endoscopic retrograde cholangiopancreatography (ERCP)

for pancreatic disease in children
AUTHOR: Andres Gelrud, MD, MMSc
SECTION EDITORS: Melvin B Heyman, MD, MPH, Douglas G Adler, MD, FACG, AGAF, FASGE
DEPUTY EDITOR: Alison G Hoppin, MD
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Jan 2024.

This topic last updated: Jul 22, 2022.
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 adverse events".)
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
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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.
● Genetic testing – In acute recurrent or chronic pancreatitis, genetic causes should be

explored through genetic testing, typically offered as a "pancreatitis panel", including tests
for PRSS1 (cationic trypsinogen), SPINK1 (serine protease inhibitor Kazal type 1), CFTR (cystic
fibrosis transmembrane conductance regulator), and CTRC (chymotrypsinogen C) gene
mutations [3,4]. (See "Clinical manifestations and diagnosis of chronic and acute recurrent
pancreatitis in children" and "Causes and contributing risk factors for chronic pancreatitis
in children and adolescents", section on 'Genetic' and "Pancreatitis associated with genetic
risk factors", section on 'Genetic testing'.)
● Imaging – Pancreatic imaging 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 "Clinical manifestations and diagnosis of
chronic and acute recurrent pancreatitis in children", section on 'Initial imaging'.)
• Magnetic resonance cholangiopancreatography (MRCP) should be performed prior to

consideration of ERCP for most patients with chronic or acute recurrent pancreatitis.
MRCP has high diagnostic yield for treatable pancreatic conditions and is noninvasive.
Abdominal computed tomography (CT) is also reasonably sensitive but has the
disadvantage of radiation exposure.
• EUS is increasingly used if MRCP is not revealing. 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 [5-8]. The findings on an EUS
examination may also obviate the need for ERCP, potentially sparing the patient the risk
of post-ERCP pancreatitis. (See "Endoscopic ultrasound in chronic pancreatitis".)
If these noninvasive tests identify a relevant condition such as biliary obstruction, we

proceed to therapeutic ERCP [2].
Indications for ERCP — Indications for ERCP in children were initially extrapolated from the
adult literature but are supported by increasing clinical experience with ERCP in the pediatric
age groups, as summarized in consensus guidelines [9,10]. 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 anatomical 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 consisting of laboratory tests and imaging, as outlined above. (See
'Initial evaluation' above.)
• If the noninvasive tests and imaging reveal a cause that is potentially treatable via
ERCP, it is appropriate to proceed to therapeutic ERCP. Biliary pancreatitis (due to
choledocholithiasis or sludge) is a common indication. (See 'Biliary pancreatitis' below.)
• If the noninvasive imaging is unrevealing, we generally do not proceed to ERCP after a

first attack of pancreatitis.
● Recurrent pancreatitis – Approximately 17 percent of children with acute pancreatitis will

have recurrences [11]. 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 [12].
If noninvasive testing (CT or MRCP) and genetic testing is unrevealing, we occasionally

proceed to ERCP in selected patients, but the diagnostic yield is low and must be balanced
against the risk of complications. Post-ERCP pancreatitis occurs in 3 to 12 percent of
pediatric ERCPs and is more common in those who undergo pancreatic duct injection or
pancreatic sphincterotomy [13]. (See "Endoscopic retrograde cholangiopancreatography
(ERCP) in children: Technique, success, and adverse events", section on 'Adverse events'.)
ERCP findings and interventions by cause — Various anatomical 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.
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 20 percent of cases of acute pancreatitis in children [14-

16]. 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). The
incidence of ABPJ is approximately 1:100,000 in the Western world, compared with 1:1000
in Japan [17]. The predisposition of patients with APBJ to pancreatitis is probably related to
retrograde flow of bile into the pancreatic duct but has also been attributed to sphincter of
Oddi dysfunction (SOD) [18-22].
Therapeutic ERCP is helpful for most patients with biliary cysts and pancreatitis. 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)
[23-26]. In other patients with biliary cysts, pancreatitis may be due to pancreatic stones or
plugs, which can be removed endoscopically. The approach to management depends on
the cyst type. 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 [27]. (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 [14,23,28-35]. In our own
experience with 272 consecutive successful ERCPs performed for a variety of indications,
pancreas divisum was found in nine (3.3 percent) [36]. 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 temporary placement of a stent. Clinical improvement with such
treatment has been observed in up to 75 percent of patients [23,32,37]. 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 less
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 subjects
(14.5 percent) with acute recurrent or chronic pancreatitis, acting independently from
genetic risk factors [38]. ERCP was most helpful if obstructing stones were present in the
dorsal pancreatic duct.
● Annular pancreas – Case reports have described an association between annular

pancreas ( image 4) and recurrent pancreatitis in children [23,39,40]. 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 [39].
● Other pancreatic congenital anomalies – Several other anomalies have been described
in association with pancreatitis including:
• Agenesis of the dorsal pancreas (also known as "short pancreas") ( image 5).
• Cystic dilation of the pancreatic duct (pancreatocele) ( image 6) [23,41].
• Duodenal duplication cyst – These are rare; the association with pancreatitis is
presumably due to intermittent obstruction of the pancreatic duct [42-44]. ERCP can be
helpful for establishing the diagnosis and for definitive treatment [45,46]. The
appearance of a duplication cyst on EUS is described separately. (See "Endoscopic
ultrasound for the characterization of subepithelial lesions of the upper
gastrointestinal tract".)
Biliary pancreatitis — Biliary pancreatitis is a common indication for ERCP in children. ERCP is
indicated for children with biliary pancreatitis with cholangitis or with obstruction of the
common bile duct, as reflected in expert consensus guidelines [9,10]. If the pancreatitis is
severe, the procedure should be performed within 24 hours. For biliary pancreatitis without
cholangitis, ERCP is generally indicated for stone extraction but is not urgent.
Other acquired diseases — In addition to biliary pancreatitis, other acquired disorders

associated with pancreatitis are viral or parasitic infections, 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) [47,48]. 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 [49] or, rarely, necrotizing pancreatitis
[50]. Worms can sometimes be removed endoscopically [51]. (See "Ascariasis".)
● HIV infection – Few publications are available on pancreatic involvement in children with
acquired immunodeficiency syndrome (AIDS). Pancreatitis is a recognized complication of
human immunodeficiency virus (HIV) infection in children and adults. Most cases are
associated with use of didanosine (a nucleoside reverse-transcriptase inhibitor in use in
the United States for over 30 years) or other medications [52-54]. Opportunistic infections
may involve the pancreas [52]. 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 [55].
● Autoimmune pancreatitis – Autoimmune pancreatitis is an infrequently recognized

disorder of presumed autoimmune etiology that is associated with characteristic clinical,
histologic, and morphologic findings [56]. 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 [57,58]. (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) [58].
● Sphincter of Oddi dysfunction – The hypothesis that SOD may cause acute recurrent
pancreatitis in adults is being increasingly questioned, and its role in children with
recurrent pancreatitis is even more uncertain. 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.
Diagnosis and management of SOD dysfunction in adults is discussed separately.
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 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 including 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 [59].
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
pancreatitis 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 only
rarely 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
[23,28,31,37,60]. 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, and the
very limited evidence available suggests that both types of interventions have a role. Larger and
randomized studies are needed to determine optimal selection of patients for these
interventions. 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.
● Endoscopic therapy – 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 due to ductal obstruction [61].
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). At present, endoscopic
therapy should only be performed in specialized centers, preferably in the context of a
clinical study.
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 [2,10,37,62-
64], as illustrated by the following studies:
• Among 301 children with acute recurrent pancreatitis or chronic pancreatitis enrolled in
the INSPPIRE study (INternational Study group of Pediatric Pancreatitis: In search for a
cuRE), 117 (38.9 percent) underwent at least one therapeutic ERCP [65]. The procedure
was more commonly performed in children with chronic pancreatitis (66 percent)
compared with acute recurrent pancreatitis (13.5 percent). Utility of therapeutic ERCP
was similar in both groups (53 versus 56 percent).
• A retrospective single-center case series described ERCPs in 38 children and

adolescents, most of whom had chronic pancreatitis [66]. A total of 158 ERCPs were
performed with a seven-year follow-up. The post-ERCP complication rate was 3 percent,
which is somewhat lower than that reported in most other case series. After the
procedure, there were significant decreases in the severity and frequency of abdominal
pain, frequency of pancreatitis episodes, and use of analgesia (p<0.001); only one child
required 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. Complications of ERCP in children are discussed in more detail separately. (See
"Endoscopic retrograde cholangiopancreatography (ERCP) in children: Technique, success,
and adverse events", section on 'Adverse events'.)
● Surgical therapy – The very limited evidence available suggests that surgical therapy also
has a role in the management of children with chronic pancreatitis and abdominal pain:
• 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. A small series of six
children described a two-stage management protocol: The patients were first treated
with ERCP with pancreatic stenting, and all had relief of pain [67]. 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.
• A small case series suggests a role for surgical intervention in selected children. Among
37 children with chronic pancreatitis, those managed by surgical therapy had a lower
rate of recurrent pancreatitis and hospitalization compared with those managed by
endoscopic therapy [68]. Some of the patients in this series responded to surgical
therapy after failing therapeutic ERCP.
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) [61,69]. 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 case reports and case series [70-73]. In a meta-analysis of 187 children, the pooled
clinical success rate was 92 percent [74]. (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, the first step is noninvasive imaging (eg, magnetic
resonance cholangiopancreatography [MRCP] if available with secretin protocol), which has
high sensitivity for identifying duct disruptions [2,75]. Subsequently, ERCP can identify the
presence and location of duct leakage and may allow endoscopic therapy or identify the need
for surgery [72,76,77]. 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
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one week of the injury [77,78]. Patients with a normal pancreatogram can be treated
conservatively. Those with duct disruption can be treated with an intrapancreatic stent bridging
the disrupted site, thereby reserving surgery for those in whom stenting is unsuccessful [76,79].
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
● Overview of indications for endoscopic retrograde cholangiopancreatography (ERCP)

– ERCP should be considered for patients with pancreatitis for which an anatomical cause
is identified radiologically and might be treated endoscopically. Potential indications for
ERCP in children with pancreatic disease are listed in the table ( table 1).
● Role in acute and recurrent pancreatitis – For patients with unexplained acute or
recurrent pancreatitis, the first step in the evaluation is a focused history and laboratory
testing to rule out medications, systemic conditions, or genetic mutations that may trigger
pancreatitis. We also obtain noninvasive imaging, typically magnetic resonance
cholangiopancreatography (MRCP). (See 'Initial evaluation' above.)
• If the imaging reveals a potentially treatable cause (such as biliary or pancreatic

obstruction due to a stone or stricture), it is appropriate to proceed to therapeutic
ERCP.
• If imaging does not reveal an anatomical cause of the pancreatitis, ERCP is unlikely to
be revealing.
(See 'Indications for ERCP' above.)
● Pre-procedure evaluation – 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 magnetic resonance
imaging/magnetic resonance cholangiopancreatography (MRCP), CT, or endoscopic
ultrasound (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".)
● ERCP findings and interventions by cause
• Biliary pancreatitis – ERCP is indicated for children with biliary pancreatitis with
cholangitis or with obstruction of the common bile duct but generally not for mild
biliary pancreatitis without cholangitis. (See 'Biliary pancreatitis' above.)
• Biliary cysts – A biliary cyst is responsible for 6 to 18 percent of acute pancreatitis in

children. Most such children have an anomalous pancreaticobiliary junction (APBJ) in
which the pancreaticobiliary union is located outside the duodenal wall ( figure 1A-B).
Therapeutic ERCP is helpful for most of these patients, but most will also require
surgical excision of the cyst. (See 'Congenital anomalies' above.)
• Pancreas divisum – This is more common among children with recurrent pancreatitis
than in the general population, but 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 'Congenital anomalies' above.)
• Other congenital anomalies – Other congenital anomalies that may be associated

with pancreatitis include annular pancreas, agenesis of the dorsal pancreas (also
known as "short pancreas"), cystic dilation of the pancreatic duct (pancreatocele), and
duodenal duplication cysts. (See 'Congenital anomalies' above.)
● Other indications for ERCP
• Chronic pancreatitis – 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.)
• Pancreatic trauma or pseudocysts – 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.)
● Complications – The most common complication is post-ERCP pancreatitis, which occurs

in approximately 3 to 12 percent of ERCPs in children, and is more common in those who
undergo pancreatic duct injection or pancreatic sphincterotomy. Risk factors and types of
complications are discussed separately. (See "Endoscopic retrograde
cholangiopancreatography (ERCP) in children: Technique, success, and adverse events",
section on 'Adverse events'.)
ACKNOWLEDGMENT
The UpToDate editorial staff acknowledges Moises Guelrud, MD, who contributed to earlier
versions of this topic review.
Use of UpToDate is subject to the Terms of Use.
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In search for a cuRE) Study. Pancreas 2017; 46:764.
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pediatric population: Long-term efficacy and safety. United European Gastroenterol J 2019;
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in identifying pancreatic duct disruption. J Clin Gastroenterol 2012; 46:696.
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abdominal trauma. JOP 2012; 13:187.
Topic 5868 Version 25.0
GRAPHICS
Potential indications for endoscopic retrograde cholangiopancreatography

(ERCP) for pancreatic disease in children
Evaluation of abnormalities on ultrasound, CT, or MRCP
Chronic pancreatitis with stone, strictures, or both
Pancreatic/peripancreatic pseudocyst (symptomatic)
Pancreatic/peripancreatic walled-off necrosis (symptomatic)
Pancreatic trauma
Pancreatic ascites
Pancreatic duct leaks
ERCP: endoscopic retrograde cholangiopancreatography; CT: computed tomography; MRCP: magnetic

resonance cholangiopancreatography.
Graphic 72692 Version 10.0
Anatomic abnormalities associated with pancreatitis in children
Congenital
Biliary anomalies
Biliary cyst
Anomalous pancreaticobiliary junction
Pancreatic anomalies
Annular pancreas
Pancreatic agenesis (dorsal or ventral)
Cystic dilatation of the pancreatic duct (pancreatocele)
Pancreatic anatomical variant
Pancreas divisum
Duodenal anomalies
Duodenal or gastric duplication cysts
Duodenal diverticulum
Acquired
Parasitic infestation
Sphincter of Oddi dysfunction
Pancreatic trauma
Acquired immunodeficiency syndrome
Normal versus anomalous pancreaticobiliary junction
Courtesy of Moises Guelrud, MD.
Types of anomalous pancreaticobiliary junction
B-P: bileopancreatic type; P-B: pancreaticobiliary type.
Endoscopic retrograde cholangiopancreatography (ERCP) in a patient with

choledochocele
In this 14-year-old boy with documented recurrent acute pancreatitis, MRCP revealed cystic dilation of
the major papilla, due to a choledochocele (type III biliary cyst), in which the cyst is located within the
duodenal wall. ERCP revealed a bulging and long intraduodenal segment of the major papilla (panel A).
Biliary sphincterotomy was performed (panel B), and no stones and no sludge were found in the bile
duct.
ERCP: endoscopic retrograde cholangiopancreatography; MRCP: magnetic resonance

cholangiopancreatography.
Courtesy of Andres Gelrud, MD, MMSc, and Moises Guelrud, MD.
Endoscopic retrograde cholangiopancreatography (ERCP) in a patient with an

anomalous pancreaticobiliary union and biliary cyst
A 16-year-old girl with documented recurrent acute pancreatitis underwent endoscopic retrograde
cholangiopancreatography (ERCP). Cholangiogram revealed a long common channel of approximately 2
cm in length (dashed arrow) and the bile duct taking off from the pancreatic duct (arrow). A cystic biliary
dilation is also present. Sphincterotomy provided excellent biliary and pancreatic contrast drainage.
Courtesy of Andres Gelrud, MD, MMSc.
Endoscopic retrograde cholangiopancreatography (ERCP) in a patient with an

anomalous pancreaticobiliary union
A 13-year-old boy with documented recurrent acute pancreatitis underwent endoscopic retrograde
cholangiopancreatography (ERCP). Cholangiogram revealed a long common channel of approximately
2.5 cm in length (arrow) and the pancreatic duct taking off from the mid-common bile duct (dashed
arrow). Sphincterotomy provided symptom relief. In the right upper quadrant, staples from prior
cholecystectomy are present.
Anatomy and embryology of pancreas divisum
The pancreas develops from 2 parts whose ducts are in continuity with the common bile duct. One part is
ventral and the other dorsal to the intestinal tract before rotation. The rotation brings the 2 parts
together with separate ducts. The duct of the dorsal (larger) part later becomes continuous and enters
that of the ventral part, which enters the duodenum with the common bile duct. However, in a congenital
malformation (pancreas divisum), the other 2 parts of the pancreas remain distinct, each with its own
duct.
Endoscopic retrograde cholangiopancreatography (ERCP) of pancreas divisum

with a normal dorsal duct
A child with recurrent acute pancreatitis and MRCP that revealed pancreas divisum. In this image, we can
see the classic finding of the dorsal pancreatic duct crossing the distal common bile duct (arrow) and
ending into the minor papilla (thick arrow). This is diagnostic of pancreas divisum.
ERCP: endoscopic retrograde cholangiopancreatography; MRCP: magnetic resonance

cholangiopancreatogram.
Pancreas divisum with a dilated dorsal duct
A child with recurrent acute pancreatitis and MRCP that revealed pancreas divisum.
(A) MRCP shows the dilated dorsal pancreatic duct crossing the distal common bile duct (thin arrow) and
ending into the minor papilla (thick arrow).
(B) ERCP demonstrates the dilated dorsal duct.
MRCP: magnetic resonance cholangiopancreatogram; ERCP: endoscopic retrograde

pancreatocholangiogram.
Pancreas divisum, minor papilla sphincterotomy
(Panel A) A cannula is introduced into the minor papilla (arrow), which is located above the major papilla
(dashed arrow).
(Panel B) A wire (arrow) is introduced into the dorsal pancreatic duct.
(Panel C) After sphincterotomy of the minor pancreatic papilla, a pancreatic stent (arrow) is placed into
the dorsal pancreatic duct to prevent pancreatitis.
Annular pancreas
Endoscopic retrograde cholangiopancreatography (ERCP) shows a branch of the pancreatic duct

surrounding the descending duodenum. A short ventral pancreas is found.
Reproduced with permission from: ERCP in Paediatric Practice: Diagnosis and Treatment, Isis Medical Media LTD, Oxford University
Press, UK 1997. Copyright ©1997 Oxford University Press.
Endoscopic retrograde cholangiopancreatography (ERCP) showing dorsal

pancreatic agenesis
Partial pancreatic agenesis is also known as hypoplasia of the pancreas or short pancreas. It is an
extremely rare condition in which the dorsal or ventral pancreas is absent. On occasion, patients may
present with abdominal pain or pancreatitis. The diagnosis is made by radiologic examination, which
reveals the agenesis of the pancreatic segment. Complete agenesis of the pancreas is not compatible
with life.
ERCP: endoscopic retrograde cholangiopancreatography.
Press, UK 1997. Copyright © 1997 Oxford University Press.
Endoscopic retrograde cholangiopancreatography (ERCP) showing

pancreatocele
Press, UK 1997. Copyright ©1997 Oxford University Press.
Ascaris infestation of the bile duct
A) Contrast injected into the papilla of vater shows ascaris worms obstructing the common bile duct and
impinging on the main pancreatic duct. B) The worms are removed using a tripod basket.
Press, UK, 1997. Copyright ©1997 Oxford University Press.
ERCP in a patient with autoimmune pancreatitis
ERCP of a 19-year-old male with recurrent pancreatitis, elevated IgG4 suggesting the autoimmune form,
and diffuse "sausage-shape" pancreas on imaging.
(A) Cholangiogram revealing a distal common bile duct stricture (arrow) with mild proximal dilation.
(B) Cholangiogram revealing diffuse intrahepatic ductal strictures (dashed arrows) with mild proximal
dilation.
(C) Pancreatogram showing diffuse narrowing with areas of ductal dilation.
After initiation of prednisone, repeat imaging and IgG4 both normalized.
ERCP: endoscopic retrograde cholangiopancreatography; IgG4: immunoglobulin G4.
Risk factors for chronic or acute recurrent pancreatitis in children
Percent of patients with risk factor

Risk category
(among those examined for the risk)
Genetic
Any mutation 61 (United States/Canada/Israel)*

39 (Japan) ¶
PRSS1 33 (United States/Canada/Israel)*

15 (Japan) ¶
SPINK1 20 (United States/Canada/Israel)*

18 (Japan) ¶
CFTR 28 (United States/Canada/Israel)*
CTRC 7 (United States/Canada/Israel)*

2 (Japan) ¶
CPA1 2 to 4 (Japan) ¶ Δ
1 to 3 (Europe) Δ
Obstructive/biliary
Any obstructive/biliary risk factor 33*
Pancreas divisum 12*
Other obstructive/biliary risk factors ◊ Approximately 21*
Toxic-metabolic
Any toxic-metabolic factor 21*
Hyperlipidemia (most commonly 6*

hypertriglyceridemia)
Drugs (eg, metronidazole, mercaptopurine, 12*

valproate, isoniazid)
Other toxic-metabolic risk factor § Approximately 9*
Autoimmune
Autoimmune pancreatitis 14*
This table shows the proportion of patients with certain risk factors for chronic or acute recurrent
pancreatitis from several different populations. Note that some patients may have more than one risk
factor.
PRSS1: protease serine 1 gene; SPINK1: serine protease inhibitor Kazal-type 1 gene; CFTR: cystic fibrosis
transmembrane conductance regulator gene; CTRC: chymotrypsin C gene; CPA1: carboxypeptidase A1.
* Data from the INSPPIRE cohort (United States, Canada, and Israel) of 301 children <19 years (mean age
at onset of acute pancreatitis 8.1 years) [1] .
¶ Data from a Japanese cohort of 128 children <16 years old (mean age at onset of acute pancreatitis 7.6
years) [2] .
Δ Data from adults with non-alcoholic chronic pancreatitis from Germany (n = 944), France (n = 456),
Czech Republic (n = 21), Poland (n = 123), India (n = 230), and Japan (n = 247) [3] .
◊ Other obstructive/biliary risk factors include pancreatobiliary malunion, gallstones, trauma, sphincter
of Oddi dysfunction, annular pancreas, and other duct obstructions.
§ Other toxic-metabolic includes active and passive tobacco smoking, alcohol use, chronic kidney disease,
and organic acidemias.
References:
1. Kumar S, Ooi CY, Werlin S, et al. Risk factors associated with pediatric acute recurrent and chronic pancreatitis: Lessons
from INSPPIRE. JAMA Pediatr 2016; 170:562.
2. Saito N, Suzuki M, Sakurai Y, et al. Genetic analysis of Japanese children with acute recurrent and chronic pancreatitis. JPGN
2016; 63:431.
3. Witt H, Beer S, Rosendahl J, et al. Variants in CPA1 are strongly associated with early onset chronic pancreatitis. Nat Genet
2013; 45:1216.
Endoscopic retrograde cholangiopancreatogram (ERCP) showing chronic calcifi

pancreatitis with a stone
(A) ERCP in a child showing chronic calcific pancreatitis with dilation of the main pancreatic duct and a
large stone (arrow).
(B) Pancreatic sphincterotomy was performed with successful stone extraction.
(C) ERCP showing 2 pancreatic stents (dashed arrows) that were placed to ensure drainage of the
pancreatic duct.
ERCP: endoscopic retrograde cholangiopancreatography.
Chronic calcific pancreatitis in a patient with PRSS1 gene mutation
Twelve-year-old boy with documented recurrent acute pancreatitis for 5 years, now with chronic calcific
pancreatitis. The evaluation revealed a mutation in the PRSS1 gene (at p.R122H). The patient presented
with worsening constant pain.
(A) CT of the abdomen revealed a ductal stone, with proximal pancreatic duct dilation.
(B) ERCP was performed, revealing a dilated main pancreatic duct, with a filling defect (arrow)
representing a stone.
(C) The stone was successfully extracted using a basket.
CT: computed tomography; ERCP: endoscopic retrograde cholangiopancreatography.
Treatment of a pancreatic pseudocyst by endoscopic placement of stents
(A) MRI in an 8-year-old boy with L-asparaginase-induced acute pancreatitis that was complicated by a
symptomatic pancreatic pseudocyst (arrow).
(B) ERCP showing successful placement of a lumen-apposing metal stent (thick arrow) and a plastic
double-pigtail stent inside. The stent placement was guided by endoscopic ultrasound.
MRI: magnetic resonance imaging; ERCP: endoscopic retrograde cholangiopancreatogram.
Contributor Disclosures
Andres Gelrud, MD, MMSc Consultant/Advisory Boards: AbbVie [Pancreatic exocrine insufficiency]; Ariel
[Genetic testing]; National Pancreas Foundation [Ad honorem]. Speaker's Bureau: AbbVie [Pancreatic
insufficiency]. All of the relevant financial relationships listed have been mitigated. Melvin B Heyman, MD,
MPH Equity Ownership/Stock Options: Amgen [IBD]. Grant/Research/Clinical Trial Support: AbbVie [IBD];
Celgene International [IBD]; Genentech [IBD]; Janssen [IBD]; Lilly [IBD]; Pfizer [Ulcerative colitis]; Takeda
[IBD]. Consultant/Advisory Boards: Gilead [IBD]; Mahana [GI conditions]. All of the relevant financial
relationships listed have been mitigated. Douglas G Adler, MD, FACG, AGAF, FASGE Consultant/Advisory
Boards: Abbvie [Endoscopy]; Boston Scientific [Endoscopy]; Endorotor [Endoscopy]; Merit [Endoscopy];
Olympus [Endoscopy]. Speaker's Bureau: Abbvie [Pancreatology, general GI]. All of the relevant financial
relationships listed have been mitigated. Alison G Hoppin, MD No relevant financial relationship(s) with
ineligible companies to disclose.
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
provided to support the content. Appropriately referenced content is required of all authors and must
conform to UpToDate standards of evidence.
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Official reprint from UpToDate®

Endoscopic retrograde cholangiopancreatography (ERCP)

Literature review current through: Jan 2024.

Endoscopic retrograde cholangiopancreatography (ERCP) has changed the approach to the

UNEXPLAINED ACUTE AND RECURRENT PANCREATITIS

children with pancreatitis are listed in the table ( table 1) [2].

● Exclude nonanatomical causes – Prior to considering ERCP, specific nonanatomical

● Genetic testing – In acute recurrent or chronic pancreatitis, genetic causes should be

• Magnetic resonance cholangiopancreatography (MRCP) should be performed prior to

If these noninvasive tests identify a relevant condition such as biliary obstruction, we

• If the noninvasive imaging is unrevealing, we generally do not proceed to ERCP after a

● Recurrent pancreatitis – Approximately 17 percent of children with acute pancreatitis will

If noninvasive testing (CT or MRCP) and genetic testing is unrevealing, we occasionally

A biliary cyst is identified in 6 to 20 percent of cases of acute pancreatitis in children [14-

Endoscopic treatment of pancreas divisum is generally reserved for patients whose

● Annular pancreas – Case reports have described an association between annular

• Cystic dilation of the pancreatic duct (pancreatocele) ( image 6) [23,41].

Other acquired diseases — In addition to biliary pancreatitis, other acquired disorders

● Parasitic infestation – Pancreatitis is occasionally seen as a complication of infestation

● Autoimmune pancreatitis – Autoimmune pancreatitis is an infrequently recognized

● Endoscopic therapy – A role of endoscopic therapy in the treatment of chronic

• A retrospective single-center case series described ERCPs in 38 children and

Most experience in surgical procedures for chronic pancreatitis is in adult patients, as

PANCREATIC AND PERIPANCREATIC FLUID COLLECTIONS

There is considerable variation in management of patients with traumatic pancreatitis and

SOCIETY GUIDELINE LINKS

● Overview of indications for endoscopic retrograde cholangiopancreatography (ERCP)

• If the imaging reveals a potentially treatable cause (such as biliary or pancreatic

(See 'Indications for ERCP' above.)

● Pre-procedure evaluation – Prior to considering ERCP, specific nonanatomical causes of

● ERCP findings and interventions by cause

• Biliary cysts – A biliary cyst is responsible for 6 to 18 percent of acute pancreatitis in

• Other congenital anomalies – Other congenital anomalies that may be associated

● Other indications for ERCP

• Chronic pancreatitis – In children with chronic pancreatitis, the most common

• Pancreatic trauma or pseudocysts – Endoscopic treatment of symptomatic

● Complications – The most common complication is post-ERCP pancreatitis, which occurs

Use of UpToDate is subject to the Terms of Use.

8. Demirbaş F, Kaymazlı M, Çaltepe G, et al. Endoscopic Ultrasonography in Pediatric Patients

9. Párniczky A, Abu-El-Haija M, Husain S, et al. EPC/HPSG evidence-based guidelines for the

17. Morine Y, Shimada M, Takamatsu H, et al. Clinical features of pancreaticobiliary

22. Sugiyama M, Atomi Y, Kuroda A. Pancreatic disorders associated with anomalous

25. Weisser M, Bennek J, Hörmann D. Choledochocele--a rare cause of necrotising pancreatitis

40. Yogi Y, Shibue T, Hashimoto S. Annular pancreas detected in adults, diagnosed by

41. Rosenstock E, Achkar E. A "short pancreas". Gastrointest Endosc 1986; 32:296.

children receiving dideoxyinosine. Pediatrics 1993; 91:747.

55. Yabut B, Werlin SL, Havens P, et al. Endoscopic retrograde cholangiopancreatography in

66. Kohoutova D, Tringali A, Papparella G, et al. Endoscopic treatment of chronic pancreatitis in

75. Drake LM, Anis M, Lawrence C. Accuracy of magnetic resonance cholangiopancreatography

Potential indications for endoscopic retrograde cholangiopancreatography

Evaluation of abnormalities on ultrasound, CT, or MRCP

Chronic pancreatitis with stone, strictures, or both

Pancreatic/peripancreatic pseudocyst (symptomatic)

Pancreatic/peripancreatic walled-off necrosis (symptomatic)

Pancreatic duct leaks

ERCP: endoscopic retrograde cholangiopancreatography; CT: computed tomography; MRCP: magnetic

Graphic 72692 Version 10.0

Anatomic abnormalities associated with pancreatitis in children

Anomalous pancreaticobiliary junction

Pancreatic agenesis (dorsal or ventral)

Cystic dilatation of the pancreatic duct (pancreatocele)