INTRODUCTION — Experience with endoscopic retrograde cholangiopancreatography (ERCP) in children has been limited due to multiple factors, including the relatively low incidence of diseases requiring ERCP in this age group, the impression that the procedure is technically difficult in children, and because the indications and safety of ERCP in children have not been well defined. As a result, patients are generally referred to a tertiary care facility or to adult endoscopists who perform a high volume of procedures. Few facilities have experts specifically trained in pediatric therapeutic endoscopy or a high enough volume of these procedures to ensure optimal outcomes. However, in the centers where this special expertise is available, pediatric therapeutic endoscopy can sometimes be used as an alternative to more invasive procedures, such as percutaneous transhepatic cholangiography with catheter placement or surgeries.
This topic review will focus on the technical aspects of performing ERCP in children. Indications for ERCP in children are discussed separately. (See "Endoscopic retrograde cholangiopancreatography (ERCP) for biliary disease in children" and "Endoscopic retrograde cholangiopancreatography (ERCP) for pancreatic disease in children".)
PATIENT PREPARATION AND SEDATION — Because most ERCP procedures in children are performed by adult gastroenterologists, a close working collaboration between an adult and a pediatric gastroenterologist is important during patient preparation and the procedure itself. The preparation and sedation of a child undergoing ERCP is similar to that used for upper gastrointestinal endoscopy. The procedure should be explained to the child in a manner appropriate for the age and level of intellectual and emotional development, and the parent or legal guardian must provide procedure consent.
The endoscopist, together with the anesthesiologist, must choose between monitored anesthesia care and general anesthesia, after considering the pertinent risks and taking into account personal skill and experience and the expected complexity of the procedure. In rare instances, a procedure can be performed under conscious sedation with a combination of meperidine or fentanyl and diazepam or midazolam.
Postprocedure monitoring is the same as for other endoscopic procedures requiring sedation. Personnel with appropriate training in pediatric sedation and monitoring are required for either conscious sedation or general anesthesia.
ANTIBIOTIC PROPHYLAXIS — There are no data to guide recommendations for antibiotic prophylaxis for ERCP in children. As a result, the principles of antibiotic prophylaxis in adults are frequently applied in children. (See "Antibiotic prophylaxis for gastrointestinal endoscopic procedures".)
ERCP is generally associated with a higher risk for bacteremia than upper endoscopy alone, particularly in the presence of biliary obstruction. We suggest the following approach:
●Patients with cystic or obstructing lesions of the biliary or pancreatic ducts should be given antibiotic prophylaxis to prevent cholangitis or infective pancreatitis.
●Antibiotic prophylaxis should be administered to any patient at increased risk for infection, including organ transplant recipients, patients who are immunocompromised, certain cardiac conditions, and those with indwelling catheters.
ENDOSCOPIC EQUIPMENT — In neonates and infants younger than 12 months, ERCP is performed with a pediatric duodenoscope, which has an insertion tube diameter of 7.5 mm, a channel size of 2 mm, and an elevator [1]. If the appropriate duodenoscope is not available, the vendor may be able to loan the device for the procedure. Use of a pediatric duodenoscope is mandatory in neonates and in infants younger than 12 months and is sometimes preferred for small children (eg, younger than three years). Pediatric duodenoscopes have a smaller working channel, and the maximum catheter diameter may be 7 French. This is important when choosing the appropriate sphincterotome, dilators, retrieval baskets, and stents for use in the procedure.
When the child is large enough, an adult duodenoscope (outer diameter 10.8 to 12.1 mm) with all the accessories should be used for therapeutic interventions. This is usually possible for children older than 12 months of age or weighing more than 10 to 15 kg [2]. This was shown in a study that evaluated the safely and efficacy of ERCP in the pediatric population using standard adult duodenoscopes [3]. Among 48 children with a mean age of 13 years (range 2 to 17) who underwent a total of 65 ERCPs with a standard adult duodenoscope, the cannulation success rate was 93.8 percent, with post-ERCP pancreatitis 9.2 percent and no procedure-related mortality. The rate of post-ERCP pancreatitis was somewhat higher than that reported in some larger case series (closer to 3 percent [4]), but due to the small size of this series, it is unclear if this represents a true difference.
Disposable duodenoscopes are available for adults. (See "Preventing infection transmitted by gastrointestinal endoscopy", section on 'Duodenoscopes with disposable designs'.)
TECHNIQUE — Medications that may be useful during ERCP include glucagon and scopolamine (hyoscine) butylbromide (Buscopan, not available in the United States) to reduce duodenal motility and secretin to facilitate identification and cannulation of the minor papilla. Carbon dioxide should be used instead of room air for insufflation for all age groups. Because carbon dioxide is absorbed faster than air, its use for insufflation reduces postprocedure abdominal distension and probably also reduces the risk for venous air embolism, which is a rare complication of cholangioscopy [5]. (See "Uncommon complications of endoscopic retrograde cholangiopancreatography (ERCP)", section on 'Portal vein gas and air embolism'.)
Endoscope insertion and position — The duodenoscope is inserted into the second portion of the duodenum with the patient in the prone position. The optimal route to the papilla is the "short route," which involves straightening the instrument along the lesser curvature of the stomach to bring the papilla directly en face. The instrument is withdrawn slowly, keeping the controls locked and the papilla in view. This involves rotating the scope to the right while angling the tip up. In some infants it is not possible to perform this maneuver. In such cases, cannulation of the papilla is performed with the "long route," in which the instrument lies along the greater curvature of the stomach. Control of the endoscope tip and elevator are more difficult in this configuration, and there may be more patient discomfort.
Cannulation of the papilla — The principles of cannulation are similar to those used in adult patients, with the additional limitations of space within the duodenum in small patients. We strongly favor the clinician wire-guided cannulation technique, which reduces the risk of post-ERCP pancreatitis and increases the success rate of primary cannulation compared with the standard contrast-assisted method, based on experience in adults. A tapered catheter (3.5 French at the distal end) or a small sphincterotome (4 French at the distal end) is introduced into the papilla. The contrast agent is injected manually using a 5 or 10 mL syringe. While some endoscopists prefer nonionic low osmolar contrast agents, there is no proof that these more expensive forms of contrast reduce the incidence of post-ERCP pancreatitis [6]. Dilution of the contrast material to 30 percent is preferred for optimal visualization if ducts are known to be dilated or if filling defects are anticipated. Meticulous fluoroscopy is as important as the concentration of contrast for optimal imaging; filling of common bile duct or pancreatic duct should be monitored continuously during contrast injection.
It is important to minimize the procedure time to avoid abdominal overdistension and respiratory compromise, especially in young infants. To reduce the time of the procedure, the endoscopist must anticipate the equipment that will be used (based on the indication) and have it ready. The pancreatic duct orifice is localized in the right lower quadrant of the major papilla, with the biliary opening located in the left upper quadrant. Having an endoscopic "frontal view" of the major papilla will help gain access to the pancreatic duct, while a "top view," in which the major papilla is localized in the upper field of view, will help gain access to the bile duct. If the guide-wire or contrast does not enter the common bile duct but only refluxes to the duodenum, or if only the pancreatic duct is visualized, the cannula is left in the curved cephalad position and the endoscope is slowly withdrawn. This may change the cannula tip orientation and straighten out the route from the papillary orifice to the common duct. Lateral and vertical angulation of the cannula should be tried if these attempts are unsuccessful. It is helpful to engage the orifice with the cannula tip and then rotate the scope tip and cannula to the left, followed by orientation of the cannula tip in the cephalad direction. Wire-guided cannulation technique followed by deep catheter cannulation is preferred for the prevention of postprocedure pancreatitis.
Monitoring — Because ERCP requires fluoroscopy, it is usually performed in a radiology suite designed for adult patients. Pediatric endoscopy assistants, pediatric anesthesiologists, and specially trained nurses can help reduce preprocedure anxiety, monitor the clinical status of the patient, administer medications, handle catheters, and inject contrast material. The heart rate and oxygen saturation must be continuously monitored. Resuscitation medications and appropriate equipment should be available. A recovery area equipped with monitors and specialized pediatric nurses familiar with the needs of children is necessary.
Because therapeutic ERCP has a greater potential risk for complications compared with diagnostic ERCP, we recommend overnight observation after a therapeutic procedure.
SUCCESS — In children older than one year and adolescents, the rate of successful cannulation is more than 95 percent, comparable with reports in adults [7-25]. In neonates and young infants, the rate of successful cannulation of the common bile duct is often lower than in adults, ranging from 27 to 95 percent in various reports [1,3,7,26-35]. Experience of the endoscopists may account for a large part of the variability.
In our unpublished experience, the procedure was successful in 98 percent of children older than one year (216 of 220 cases). Our ERCP success in neonates and young infants with neonatal cholestasis was 93 percent (171 of 184 cases). The procedure was unsuccessful in 13 patients because of duodenal malrotation in 2 and inability to cannulate in 11.
A retrospective study compared outcomes of more than 200 therapeutic ERCPs performed in pediatric patients (average age 13.3) with those for adult patients with similar procedural indications and matched for complexity [36]. There were no differences in technical success, clinical success, complication rates, or fluoroscopy time between the two cohorts. The pediatric patients were more likely to require general anesthesia and had a longer average length of stay.
COMPLICATIONS — The incidence of complications in children has not been well established in prospective studies. In children older than one year, complication rates are between 3 and 10 percent [11,14,22-26,28,30,32,34,35,37]. Complications include pancreatitis, infection, hemorrhage, and perforation, and are more common among patients undergoing therapeutic ERCP as compared with diagnostic ERCP. Major complications are rare. In general, complication rates of ERCP in infants and children seem to be similar to those in adults.
Since May 2014, data collection began for the Pediatric ERCP Database Initiative (PEDI), an ongoing multicenter (14 institutions) longitudinal database of ERCPs in children. The goal is to collect data on 1100 ERCPs in children, including the indication for the procedure and rates of successful cannulation and complications.
The frequency of complications was reported in a systematic review that included 32 studies involving more than 3000 ERCPs in children [4]. The pooled complication rate was 6 percent overall and 3 percent among ERCPs for neonatal cholestasis. The most common complication was post-ERCP pancreatitis (pooled estimate 3 percent), followed by bleeding, and infection (both less than 1 percent). In one of the largest included studies, post-ERCP pancreatitis occurred in 7.7 percent, and the majority of cases were mild by consensus definition [38]. Procedural characteristics significantly associated with post-ERCP pancreatitis included: pancreatogram, pancreatic sphincterotomy, pancreatic duct stenting, and pancreatic duct stricture dilation. Immediate postsphincterotomy bleeding occurred twice (1.1 percent), and both cases were successfully treated during the procedure. No mortality was observed. The complication rate found in this series closely parallels that observed in adults.
It is unclear whether complication rates are slightly higher in infants younger than one year [1,26-33]. One of the large series cited above reported no complications in children younger than one year [37]. In our unpublished experience with 184 neonates and young infants, minor complications without clinical significance occurred in 24 patients (13 percent), which is higher than our complication rate in children older than one year (1.6 percent). Two neonates had transient opiate-induced respiratory depression, and four young infants had nonopioid respiratory depression, which resolved with oxygen administration. In 17 patients, minor acute duodenal erosions were observed without clinical consequences. One neonate had abdominal distention for 10 hours after completion of ERCP, which resolved without treatment. There were no major complications in either series.
Pancreatitis may be a more common complication of ERCP among children with underlying pancreatitis as compared with those undergoing ERCP for other indications. In a series of 42 ERCP examinations in 37 children and 1 young adult, all of whom had a history of pancreatitis, mild pancreatitis was observed in three patients (8 percent) [11]. No patients with biliary tract pathology developed pancreatitis. Another series reported ten cases of post-ERCP pancreatitis in 67 patients undergoing ERCP for chronic pancreatitis (15 percent). The rate of post-ERCP pancreatitis was highest in the patients undergoing pancreatic duct stent placement (7 of 28 procedures; 25 percent) [39]. In a larger series of 432 ERCPs in 313 patients younger than 19 years, pancreatitis occurred after 47 procedures (10.9 percent) [40]. Post-ERCP pancreatitis was positively associated with pancreatic duct injection and pancreatic sphincterotomy but was less likely in patients with a history of chronic pancreatitis.
To minimize the risk of pancreatitis, a sphincterotome with a guidewire can be used for cannulation. Under fluoroscopic control, the guidewire is advanced into the common bile duct, then the sphincterotome is advanced and contrast injected. This approach avoids injection of contrast into the pancreatic duct and reduces the risk of pancreatitis. There is some evidence that administration of indomethacin via rectal suppository at the time of the ERCP may help to prevent post-ERCP pancreatitis, based on a preponderance of evidence in adults. There are no studies in children. Practice varies in adults and children, with some endoscopists routinely using indomethacin during ERCP for prophylaxis and others using this for selected high-risk patients. (See "Post-endoscopic retrograde cholangiopancreatography (ERCP) pancreatitis".)
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) in infants and children is generally performed at a tertiary care facility or by adult endoscopists who perform a high volume of procedures. In either case, the team must include providers with pediatric experience for patient preparation and for the procedure itself. Few facilities have experts specifically trained in pediatric therapeutic endoscopy or a high enough volume of these procedures to ensure optimal outcomes. (See 'Patient preparation and sedation' above.)
●ERCP in infants and children is performed using monitored anesthesia care or general anesthesia. The choice depends on the pertinent risks, skill and experience of the endoscopist, and the expected complexity of the procedure. Collaboration with a pediatric anesthesiologist and therapeutic endoscopy nurses is strongly recommended. (See 'Patient preparation and sedation' above.)
●ERCP is generally associated with a higher risk for bacteremia than upper endoscopy alone, particularly in the presence of biliary obstruction. In general, patients with cystic or obstructing lesions of the biliary or pancreatic ducts should be given antibiotic prophylaxis to prevent cholangitis or infective pancreatitis. Prophylaxis is sometimes appropriate in patients at increased risk for infection, including organ transplant recipients, those who are otherwise immunocompromised, and patients with indwelling catheters. (See 'Antibiotic prophylaxis' above and "Antibiotic prophylaxis for gastrointestinal endoscopic procedures".)
●Use of a pediatric duodenoscope (insertion tube diameter of 7.5 mm, a channel of 2 mm, and an elevator) is mandatory in neonates and in infants younger than 12 months and is preferred for children younger than three years. A standard adult duodenoscope can be used in older children (older than 12 months of age or weighing more than 10 to 15 kg) and adolescents. (See 'Endoscopic equipment' above.)
●The principles of cannulation are similar to those used in adult patients, but special techniques may be required in small patients because of the limited space within the duodenum and small size of the common bile duct. It is important to minimize the procedure time to avoid abdominal overdistension and respiratory compromise, especially in young infants. The use of carbon dioxide and wire-guided cannulation technique is also favored. (See 'Technique' above.)
●In children older than one year and adolescents, the rate of successful cannulation of the common bile duct at ERCP is comparable with reports in adults. In neonates and young infants, the rate of successful cannulation of the common bile duct is often lower than in adults, ranging from 27 to 95 percent in various reports. Experience of the endoscopists may account for a large part of the variability. (See 'Success' above.)
●Complications of ERCP include pancreatitis, infection, hemorrhage, and perforation. When the procedure is performed by experts with experience in this age group and with appropriate precautions, the incidence of complications is probably similar to that in adults. In children older than one year, complication rates of ERCP are between 3 and 10 percent. Major complications are rare. Pancreatitis may be a more common complication of ERCP among children with underlying pancreatitis compared with those undergoing ERCP for other indications. (See 'Complications' above.)
ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Moises Guelrud, MD, who contributed to an earlier version of this topic review.
