Endoscopic retrograde cholangiopancreatography is a technically challenging endoscopic technique that provides a minimally invasive way of evaluating and treating pathologic abnormality in the bile ducts and pancreas. Its utilization in children is increasing rapidly, broadening the understanding of its pediatric indications, clinical utility, and technical limitations. This article updates providers about specific considerations of endoscopic retrograde cholangiopancreatography in children as they relate to appropriate indications, patient preparation, available equipment, as well as expected technical and clinical outcomes following the procedure in pediatric populations.
Key points
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The utilization of endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric population is rapidly expanding.
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Technical and clinical outcomes following pediatric ERCP seem to mirror those seen in adult patients when performed for similar indications.
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Well-designed pediatric studies are needed to clarify appropriate indications and patient selection algorithms, identify pediatric-specific risk factors for adverse events, and develop pediatric specific risk mitigation strategies.
Overview
Endoscopic retrograde cholangiopancreatography (ERCP) is an advanced endoscopic technique used in the diagnosis and treatment of biliary and pancreatic diseases in patients of all ages. To date, the relative infrequency with which this procedure has been performed in children has resulted in a paucity of pediatric-specific data about ERCP. In turn, it has historically been necessary to adapt patient selection algorithms and risk mitigation strategies for children undergoing ERCP from studies involving adult patients. More recently, with increasing use of ERCP in children, has come a better understanding of appropriate indications as well as expected technical and clinical outcomes specific to this population. Recent reports, although limited by retrospective single-center design, suggest that additional well-designed pediatric studies are feasible and warranted. In particular, it is becoming clearer that there are indications for ERCP that may be unique to the pediatric population, whereas other studies have shown the historic practice of adapting adult-based practices to children may be inappropriate in certain instances. With these recent developments in mind, this article serves to update the reader on the current role of ERCP in the management of biliary and pancreatic disease with a focus on pediatric indications, patient preparation, equipment, as well as technical and clinic outcomes following the procedure when it is performed in children.
Overview
Endoscopic retrograde cholangiopancreatography (ERCP) is an advanced endoscopic technique used in the diagnosis and treatment of biliary and pancreatic diseases in patients of all ages. To date, the relative infrequency with which this procedure has been performed in children has resulted in a paucity of pediatric-specific data about ERCP. In turn, it has historically been necessary to adapt patient selection algorithms and risk mitigation strategies for children undergoing ERCP from studies involving adult patients. More recently, with increasing use of ERCP in children, has come a better understanding of appropriate indications as well as expected technical and clinical outcomes specific to this population. Recent reports, although limited by retrospective single-center design, suggest that additional well-designed pediatric studies are feasible and warranted. In particular, it is becoming clearer that there are indications for ERCP that may be unique to the pediatric population, whereas other studies have shown the historic practice of adapting adult-based practices to children may be inappropriate in certain instances. With these recent developments in mind, this article serves to update the reader on the current role of ERCP in the management of biliary and pancreatic disease with a focus on pediatric indications, patient preparation, equipment, as well as technical and clinic outcomes following the procedure when it is performed in children.
Indications
ERCP is a technically demanding procedure in patients of all ages that is associated with a relatively high rate of adverse events (AE) as compared with standard upper and lower endoscopy. Thus, significant efforts have been made to emphasize careful patient selection to limit procedural exposure to those who are most likely to benefit. Recording an indication from a published list of appropriate indications, such as that presented by the American Society for Gastrointestinal Endoscopy (ASGE) in Box 1 , has been recommended as a critical preprocedural quality indicator that should be monitored and reported. Such lists were generated primarily with adult patients in mind, with a goal of emphasizing the utilization of less invasive diagnostic modalities in lieu of diagnostic ERCP when possible, as well as limiting exposure to ERCP in settings where it has been shown to be ineffective by well-designed studies. As pediatric-specific studies are produced and additional experience is gained with alternative diagnostic modalities in children, pediatric-specific indications will likely reflect a similar evidence-basis and rationale.
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The jaundiced patient suspected of having biliary obstruction (appropriate therapeutic maneuvers should be performed during the procedure)
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The patient without jaundice whose clinical and biochemical or imaging data suggest pancreatic duct or biliary tract disease
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Evaluation of signs or symptoms suggesting pancreatic malignancy when results of direct imaging (eg, endoscopic ultrasound, ultrasound, computed tomography, MRI) are equivocal or normal
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Evaluation of pancreatitis of unknown cause
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Preoperative evaluation of the patient with chronic pancreatitis or pseudocyst
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Evaluation of the sphincter of Oddi by manometry
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Empirical biliary sphincterotomy without sphincter of Oddi manometry is not recommended in patients with suspected type III sphincter of Oddi dysfunction
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Endoscopic sphincterotomy
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Choledocholithiasis
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Papillary stenosis or sphincter of Oddi dysfunction
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To facilitate placement of biliary stents or dilation of biliary strictures
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Sump syndrome
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Choledochocele involving the major papilla
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Ampullary carcinoma in patients who are not candidates for surgery
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Facilitate access to the pancreatic duct
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Stent placement across benign or malignant strictures, fistulae, postoperative bile leak, or in high-risk patients with large unremovable common duct stones
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Dilation of ductal strictures
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Balloon dilation of the papilla
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Nasobiliary drain placement
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Pancreatic pseudocyst drainage in appropriate cases
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Tissue sampling from pancreatic or bile ducts
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Ampullectomy of adenomatous neoplasms of the major papilla
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Therapy for disorders of the biliary and pancreatic ducts
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Facilitation of cholangioscopy or pancreatoscopy
The evolution of ERCP in the pediatric population has mirrored the adult experience closely. Early studies (those before 2004) have been summarized previously and were primarily focused on demonstrating the feasibility and diagnostic utility of pediatric ERCP. More recent publications emphasize its utilization as a primarily therapeutic modality and highlight applications of ERCP unique to the pediatric population ( Table 1 ). Based on these primarily single-center experiences, the most common clinical scenarios in which ERCP is undertaken in the pediatric population are pancreaticobiliary and include the following:
| Author (Year) | Procedures (N) | Mean Age (Range) | Success (%) | Therapeutic (%) | Unique Aspects |
|---|---|---|---|---|---|
| Varadarajulu et al, 2004 | 163 | 9.3 y (1 mo–17 y) | 98 | 67 | Similar technical outcomes between children and adults when performed for similar indications |
| Rocca et al, 2005 | 48 | 10 y (1 mo–17 y) | 97 | 84 | ERCP largely a therapeutic modality |
| Cheng et al, 2005 | 329 | 12.3 y (2 mo–17 y) | 98 | 71 | 17% of procedures performed for chronic pain of suspected pancreatic or biliary origin; rates of PEP much higher in this cohort |
| Petersen et al, 2009 | 140 | 60 d (15–174 d) | 87 | 0 | ERCP prevented laparotomy in 25% of patients with suspected BA |
| Iqbal et al, 2009 | 343 | NR | NR | 43 |
|
| Vegting et al, 2009 | 99 | 7 y (3 d–16.9 y) | 71 | 61 |
|
| Jang et al, 2010 | 245 | 8 y (1 mo–16 y) | 98 | 78 | 25% of procedures performed for choledochal cyst |
| Keil et al, 2010 | 104 | 7 wk (3–25 wk) | 91 | 0 | Highlights utility of diagnostic ERCP in the workup of neonatal cholestasis |
| Otto et al, 2011 | 231 | 11.4 y (62 d–21 y) | NR | 69 | 64% of ERCPs performed for AP, ARP, and CP |
| Shteyer et al, 2012 | 27 | 55 d (33 d–89 d) | 81 | 0 | Highlights utility of diagnostic ERCP in the workup of neonatal cholestasis |
| Troendle et al, 2013 | 65 | 15.2 y (1 mo–18.4 y) | 100 | 100 | Evaluated ERCP for choledocholithiasis when performed by a pediatric gastroenterologist |
| Halvorson et al, 2013 | 70 | 12 y (6 y–17 y) | 99 | 94 | Pediatric ERCP performed by adult gastroenterologist |
| Enestvedt et al, 2013 | 429 | 14.9 y (3 mo–21 y) | 95 | 64 |
|
| Agarwal et al, 2014 | 221 | 13.8 y (5 y–18 y) | NR | 71 | Highlights therapeutic utility in pediatric patients with ARP and CP, particularly when coupled with ESWL |
| Saito et al, 2014 | 235 | 4 y (8 d–20 y) | 96 | 3.4 | Diagnostic utility in setting of BA and CC |
| Giefer et al, 2015 | 425 | 13.6 (72 d–18 y) | 95 | 81 |
|
| Troendle et al, 2015 | 432 | 12.7 y (1 mo–19 y) | NR | 86 |
|
| Hiramatsu et al, 2015 | 63 | 3.9 y (4 mo–13 y) | 100 | 0 | Superior role of ERCP compared with MRCP in preoperative examination of children with pancreaticobiliary malunion |
Biliary scenarios ( Fig. 1 )
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Management of choledocholithiasis
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Management of biliary strictures, typically benign and seen after liver transplantation or in the setting of primary sclerosing cholangitis
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Management of bile leak, typically encountered after cholecystectomy
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Preoperative evaluation of choledochol cyst and management of acute obstruction when present
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Preoperative evaluation of pancreaticobiliary maljunction
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Investigation of neonatal cholestasis
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Investigation of biliary pathologic abnormality of unclear cause when results of less invasive imaging modalities are equivocal or unavailable
Pancreatic scenarios ( Fig. 2 )
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Management of chronic pancreatitis, with the goal of improving pancreatic drainage (removal of stones, dilation/stenting of strictures)
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Management of pancreatic divisum with minor papillotomy
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Investigation of recurrent acute pancreatitis of unclear cause
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Management of pancreatic duct leak, typically encountered in setting of abdominal trauma
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Management of pancreatic pseudocyst via transpapillary approach
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Investigation of pancreatic ductal pathologic abnormality of unclear cause when results of less invasive imaging modalities are equivocal or unavailable
A recent review of a large US pediatric inpatient database demonstrated an overall increase in ERCP utilization by 26% from 2000 to 2009. During this time period, there was a decrease in utilization of diagnostic ERCP by 43% and an increase in the utilization of therapeutic ERCP by 69%. Although it is true that ERCP is being used more and more as a therapeutic modality in most patient populations, diagnostic ERCP may be of unique benefit in carefully chosen pediatric populations. Although not routinely recommended as part of the workup of neonatal cholestasis, small case series initially suggested that under select circumstances ERCP may be beneficial. It may be particularly useful in the setting of cholestasis of unclear cause where laparotomy is being contemplated to rule out biliary atresia (BA). In one relatively large study of neonates referred for laparotomy to rule out BA, prelaparotomy ERCP was able to obviate surgery in 25% of 140 patients. The sensitivity and specificity for ERCP for diagnosing BA in this series were 92% and 73%, respectively. Smaller studies have demonstrated similar utility of ERCP in this setting, where ERCP has been shown to obviate laparotomy in 19% to 42% of cases. Although procedural failure rates ranged from 6% to 18%, the negative predictive value approached 100%, suggesting it may be useful in avoiding diagnostic laparotomy in cholestatic neonates. Further work is needed to clarify the role of diagnostic ERCP in this clinical scenario, wherein timely diagnosis of biliary atresia is critically important to optimize clinical outcomes.
Technical considerations
There are several specific technical considerations to planning and performing ERCP in children. In particular, it is important to identify and develop an ideal, pediatric-safe operating environment, and to prepare the patient for the procedure in line with age-appropriate guidelines. Patient monitoring during ERCP must take into account pediatric-specific data and use appropriately sized equipment, while ensuring safety during sedation for the procedure is paramount.
Operating Environment
Once an appropriate indication for ERCP has been established, efforts must be made to identify the most appropriate clinical environment for performing the procedure. The ideal environment will be one in which an appropriately trained, high-volume endoscopist, with significant pediatric experience, performs the procedure while surrounded by support staff familiar with the unique aspects of pediatric care. When ERCP was initially introduced as a feasible technique in children, it was almost exclusively performed by high-volume adult-trained endoscopists in the setting of an adult facility where the equipment was located.
More recently, pediatric-trained endoscopists have reported performing the procedure in the confines of tertiary pediatric facilities with comparable technical success and AE rates. Although ERCP is performed less commonly in children when compared with adults, its utilization is increasing rapidly, leading many larger pediatric facilities to provide access to the procedure at their center. It is critical that endoscopists performing ERCP in children be appropriately trained in the procedure and have an ongoing case volume that is adequate to maintaining procedural competency. Although the ASGE has recommended that 180 ERCPs be performed before competency be assessed, a recent meta-analysis evaluating initial ERCP training and competency suggests that this threshold may be inadequate, and that further research is needed in this arena. Although the optimal ongoing procedural volume remains unknown, it has been suggested that greater than 50 cases per year are necessary to remain competent, because studies have suggested that ongoing case volume less than this threshold may be an independent risk factor for AE in adult patients.
Patient Preparation
As emphasized in a recent ASGE consensus document on endoscopic practice in pediatric patients, there are at least several general considerations that should be taken into account as children and their families are prepared for the procedure:
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Psychological preparation and age-appropriate preprocedural education for both patients and their families
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Obtaining written informed consent from the legal guardian as well as assent from older pediatric patients
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Observation of standard age-appropriate pre-endoscopy dietary restrictions: typically 2 hours for clear liquids, 4 hours for breast milk, and 6 hours for non-human milk and solids
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Consideration of early procedure times for younger patients, who are at risk for dehydration with prolonged fasting
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Attention to ambient temperature throughout all phases of the procedure. Small infants are at risk for developing hypothermia with prolonged procedure times as can occur with ERCP; thus, ready access to warmers as well as the ability to control the temperature of the procedure room is important for these patients.
Furthermore, if the procedure is to be performed by an offsite endoscopist, as occurs at many institutions, mechanisms need to be in place to allow for seamless communication between endoscopist and the physicians caring for the patient before and after the procedure.
Sedation
Best sedation practices for pediatric endoscopic procedures remain controversial. Although pediatric ERCP has been described as safely being performed with either endoscopist-administered moderate sedation or anesthesiologist-administered general anesthesia (GA), there are insufficient outcome data to firmly recommend one form of sedation over another. Although some have described exclusive use of moderate sedation, even for prolonged procedures of high-difficulty grade without apparent ill effect, at least one large pediatric case series has described a need to prematurely discontinue ERCPs or to convert to GA in 7.1% of cases in which such endoscopist-administered procedural sedation was attempted. Ultimately, the decision on the type of sedation to be used will depend on the comfort level of the endoscopist and the ERCP team as well as the resources that are available to them.
Even experienced proceduralists attempting to provide moderate sedation for pediatric patients undergoing ERCP should be aware that the soft-walled trachea of smaller children may be compressed by the relatively large duodenoscopes currently available. This knowledge is particularly pertinent for children near 10 to 15 kg (22–33 lbs), in which it may be appropriate to trial the use of an adult-sized duodenoscope with the intention of maximizing function, but in whom patient/scope mismatch is most likely to occur. In addition, the prone position typically used during ERCP can compromise chest excursion, resulting in hypoventilation, and can make airway rescue technically challenging. Pulse oximetry and hemodynamic monitoring should be routinely used and individuals comfortable with pediatric monitoring and rescue maneuvers should be present throughout the procedure.
Pediatric-Specific Equipment
Pediatric-specific equipment is rarely needed, even in the busiest pediatric centers. Standard adult diagnostic or therapeutic duodenoscopes can be used in most children weighing more than 10 to 15 kg (22–33 lbs), and whatever devices fit through their accessory channels may be used as well. However, although using standard duodenoscopes in children is feasible, endoscopists must take care when passing the upper esophageal sphincter and pylorus. In addition, tracheal compression manifested by hypoxia or inability to ventilate can occur when larger scopes are passed in smaller patients.
Appropriate duodenoscope selection is critical to allow for a safe and effective procedure, but unfortunately, there is little literature available to help guide the endoscopist. Descriptions of diagnostic ERCP in the setting of neonatal cholestasis exclusively use pediatric duodenoscopes with outer diameters ranging from 7.5 to 8.6 mm. Outside of these settings, most case series cite utilization of a similarly sized duodenoscope for children less than 1 year of age.
Between October 2006 and July 2015, the authors’ group performed 385 pediatric ERCPs at their pediatric referral center in Dallas, with immediate access to both pediatric and adult duodenoscopes. In 11 children less than 1 year of age (all also <10 kg) who underwent primarily therapeutic ERCP, a pediatric duodenoscope with an outer diameter of 7.5 mm (Olympus PJF 160; Olympus America, Center Valley, PA) was the scope of choice and successfully used in 9 patients. In 2 patients, a larger duodenoscope with an outer diameter of 10.6 mm was first attempted, but failed to allow for appropriate positioning within the duodenum, resulting in duodenal perforation in one patient. In 27 ERCPs performed in children between 10 and 15 kg, a duodenoscope with an outer diameter of 10.6 mm or larger was used as the scope of choice with success in 93% of cases. In all children greater than 15 kg, a duodenoscope with an outer diameter of at least 10.6 mm was used. Combining the available literature with the authors’ experience, the authors think that pediatric duodenoscopes are the instrument of choice for patients less than 10 kg, whereas adult duodenoscopes should be considered for patients greater than 10 kg, because the larger working channel allows for a greater selection of accessories.
Pediatric duodenoscopes are available for loan on a case-by-case basis, or for purchase if warranted. These scopes currently measure approximately 7.5 mm outer diameter and use a 2.0-mm accessory channel. The smaller accessory channel makes pediatric duodenoscopes more challenging to use because triple lumen catheters are not accepted. Double-lumen sphincterotomes, extraction baskets, and retrieval balloons, which can be used with a 2.0-mm channel, are commercially available from many manufacturers of endoscopic devices, and small stents, up to 5-Fr diameter, are accepted.
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