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Short Communications: Hepatology

Endoscopic Retrograde Cholangiopancreatography in Infants: Availability Under Threat: A Survey on Availability, Need, and Clinical Practice in Europe and Israel

Koot, Bart G.P.; Kelly, Deirdre A.; Hadzic, Nedim; Gonzales, Emmanuel§,||; Hierro, Loreto; Davenport, Mark#; Keil, Radan∗∗; Fockens, Paul††; Baumann, Ulrich†,‡‡

Author Information
Journal of Pediatric Gastroenterology and Nutrition: August 2020 - Volume 71 - Issue 2 - p e54-e58
doi: 10.1097/MPG.0000000000002752
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Abstract

What Is Known/What Is New

What Is Known

  • Endoscopic retrograde cholangiopancreatography in children younger than 1 year is a highly specialized procedure.
  • Guidelines do not specify the indications for endoscopic retrograde cholangiopancreatography in this group.
  • Duodenoscopes for infant endoscopic retrograde cholangiopancreatography can no longer be purchased or maintained since 2014.

What Is New

  • Several infant endoscopic retrograde cholangiopancreatography facilities have disappeared in Europe since 2014.
  • Substantial differences exist between centers in indications and execution of this procedure.
  • Efforts are needed to secure future availability of infant endoscopic retrograde cholangiopancreatography and to come to consensus on the optimal use of this indispensable technique.

Endoscopic retrograde cholangiopancreatography (ERCP) is used in infants (younger than 1 year of age) as a diagnostic and therapeutic tool in biliary disorders. After the introduction of the first small diameter duodenoscope suited for ERCP in infants in the early 1980s, its indications have evolved and its use became more widespread (1). The diagnostic indications for infant ERCP have shifted in the last decade mainly due to improvement in magnetic resonance imaging. It, however, remains an indispensable diagnostic tool in many clinical scenarios. As a therapeutic tool it can prevent more invasive treatment in case of choledocholithiasis, and biliary strictures or perforations. ERCP in infants is highly specialized and complex care as these are rare disorders in infants and the procedure is complex to perform in this age group. Both a highly skilled ERCP operator and specific equipment are needed to successfully perform this procedure.

The PJF 7.5, PJF 160, and PJF 240 duodenoscope (Olympus, Tokyo, Japan) are the only endoscopes that have been developed for infant ERCP. Because of their smaller size (outside diameter 7.5–8 mm and working channel 2 mm) compared to adult therapeutic duodenoscopes, they are suited for children weighing <10 kg. Since 2014 these endoscopes are, however, no longer supported for maintenance by the manufacturer and no new duodenoscope for infant ERCP is manufactured since 2014. ERCP facilities may already have disappeared in centers due to this development. In addition, no comprehensive guidelines on the indications and practice of infant ERCP exist. We aimed to evaluate the availability, need, and clinical practice of infant ERCP in Europe (including Israel).

METHODS

Survey

The survey was a self-administered electronic questionnaire with 17 questions. It queried the clinical practice of infant ERCP covering availability, yearly number of procedures performed, indications, and the execution of ERCP procedures. All questions were closed-ended or multiple choice except for 2 open questions. These open questions were on the specifications of the tools used for therapeutic interventions and on preventive measures taken in infant ERCP procedures. The survey had been designed by 2 of the authors (B.G.P.K. and U.B.) using the survey administration application Google Forms (Google LLC, Mountain View, CA; Supplemental Digital Content, http://goo.gl/forms/WO1NP4MX3U).

Survey Distribution

In March 2016 an invitation e-mail containing a direct link to the survey was sent to all 45 centers connected to the hepatology interest group of European Society for Pediatric Gastroenterology Hepatology and Nutrition. This interest groups covers 24 European countries (Austria, Belgium, Croatia, Czech Republic, Denmark, Finland, France, Germany, Greece, Hungary, Ireland, Italy, The Netherlands, Norway, Poland, Portugal, Serbia, Slovenia, Spain, Sweden, United Kingdom, Russia, Switzerland, Turkey) and Israel. Nonresponders were sent a reminder e-mail 2 months after the initial survey distribution email. No incentives were used to increase the response rate. The survey was updated in July 2019 on the availability of infant ERCP duodenoscopes among the centers that had indicated they had an infant ERCP scope available in 2016.

Analysis

Descriptive statistics (frequencies, medians, and ranges) were used to analyze the responses. No comparative statistics were used. All answers to the 2 open questions were reported.

RESULTS

Response was obtained from 30 of 45 (66%) centers from 18 countries (Belgium, Croatia, Czech Republic, France, Germany, Greece, Hungary, Ireland, Italy, Netherlands, Poland, Portugal, Serbia, Spain, Sweden, United Kingdom, Turkey, and Israel). Almost 75% (22/30) of the centers that responded were pediatric liver transplant centers. In 14/30 ERCP facilities for infants had been available up to 2014. Five out of these 14 centers no longer have an infant ERCP duodenoscope in their center due to breakdown of the duodenoscope between 2014 and 2019. Two of these 5 centers indicated that since the beginning of 2019 they resumed ERCPs in infants using an ERCP duodenoscope provided by the manufacturer for each case on request.

In the 11 centers that currently perform infant ERCP, the estimated median number of infants with an indication for ERCP was 5 (ranges 1–20) per year. ERCP procedures in infants were performed by adult GE only, pediatric GE only and both adult GE and pediatric GE in 7, 1, and 3 out of 11 centers, respectively. The minimal weight to perform an ERCP using the PJS 160 or 240 duodenoscope varied from 2.5 to 5 kg between centers, whereas the weight to switch to an adult therapeutic duodenoscope varied from 6 to 20 kg. The indications for ERCP for both diagnostic and therapeutic purposes among all centers that treat or refer children for infant ERCP are depicted in Figure 1. Remarkably, its use particularly for the therapeutic indications differed substantially between centers (Fig. 1 E–G). The therapeutic interventions performed in treating centers were papillotomy in 7 out of 11, balloon assisted stone extraction in 4 out of 11 and biliary tract dilatation in 5 out 11 centers. The use of antibiotics in case of biliary stones (3/11 always, 4/11 mostly, 2/11 never, 2/11 unknown) or strictures varied widely (5/11 always, 3/11 mostly, 2/11 never, 1/11 unknown). Ten centers filled out the open question on the use of preventive measures other than antibiotics; 8 had no other measures and 2 used NSAIDs for postprocedural pancreatitis prophylaxis. The open question on the tools used for therapeutic interventions was filled out by 3 respondents; 2 reported the use of Minitome (Cook Medical Inc, Bloomington, IN) and 1 reported the use of Rotacut (Medi-Globe, Tempe, AZ) for papillotomy. Finally, availability of ERCP facilities was valued as very important by 16 out of 30 centers that responded in this survey, this included 10 of the 11 centers currently performing this procedure. Nineteen out of 30 centers would purchase a new infant ERCP scope if available; this included all the 11 centers currently performing this procedure.

F1
FIGURE 1:
The diagnostic (A–D) and therapeutic (E–G) indications for ERCP in infants (n = 30 centers).

DISCUSSION

This survey establishes that infant ERCP is a low-volume procedure in all pediatric hepatology centers in Europe and Israel that perform this procedure. Substantial differences exist between centers in indications, type of interventions performed, and the procedure for infant ERCP. Five out of 14 centers no longer have an infant ERCP duodenoscope in their center due to the stop in production and maintenance of these duodenoscopes.

A recent survey on pediatric endoscopy practice patterns in the United States, Canada, and Mexico established that ERCP (including infants and children) also is a low-volume procedure in the vast majority of centers that performed this procedure (2). This survey did not query on the indications and practice of infant ERCP. It, however, showed that 61% of respondents felt that arrangements for performing ERCP in their center were inadequate. One of its main recommendations was that high-volume adult gastroenterologists should perform ERCPs and other more complex therapeutic endoscopic procedures. In view of that, it is remarkable that in our survey 4 centers indicated that a pediatric gastroenterologist performed infant ERCPs.

Few guidelines on the use of infant ERCP exist. A technical report by American Society for Gastrointestinal Endoscopy gives a description of the available tools for infant ERCP (11). The European Society of Gastrointestinal Endoscopy and European Society for Pediatric Gastroenterology Hepatology and Nutrition guideline from 2017 on endoscopy in children provides 2 recommendations on infant ERCP concerning weight (10 kg) above which regular adult ERCP scope can be used and on its place in the diagnosis of biliary atresia (12). No comprehensive guideline on infant ERCP addressing indications, and optimal utilization of infant ERCP exists. Studies on the indications and results of infant ERCP are scarce and consist of retrospective case series mostly including both children and infant cases. In a systematic review by Usatin et al (3) covering 1995 to 2016 seven case series (>10 cases) are reported that exclusively (n = 4) or separately (n = 3) reported on indication, success, and complications of ERCPs in infants. Between January 2016 and January 2019 two additional case series (>10 cases) have been published, both of which updated a previously reported cohort (4,5). In Table 1 the 7 case series published since 1995 that include separate data on >10 infants are listed (4–10). The reported success rate of infant ERCP ranges from 85% to 94%. Few cases of the therapeutic use of ERCP in infants are reported as the scope of 4 of the 7 studies is limited to the diagnostic use of ERCP in case of suspected biliary atresia and choledochal malformation (4,6,7,10). In the only series of those with doubtful diagnosis of biliary atresia after liver biopsy was performed, ERCP could prevent diagnostic laparoscopy in 42% of cases (7). Based on their finding the authors argue that ERCP is best used in selected cases of doubtful biliary atresia after a full work-up rather than routinely in the diagnosis of biliary atresia (13). In the large series by Keil et al (5) 10.2% of ERCPs in infants were therapeutic, predominately for stone extraction and/or common bile duct stenting. This study reports an overall success rate of 92.5% of all procedures in infants. The pooled complication rate calculated from all reported 696 procedures in these case series was 6.1% (95% confidence interval: 4.3%–7.9%). Due to the retrospective design of all studies and inconsistency in reporting among studies the validity of this rate is, however, limited. The most frequently reported complication was increased pancreatic enzymes; no case of necrotic pancreatitis is reported; all reported complications healed conservatively and no mortality is reported. These studies suggest that infant ERCP is in general safe and successful in infants for both diagnostic and therapeutic indications and can, particular in specific cases of doubtful biliary atresia, choledocholithiasis, and biliary strictures, prevent more invasive radiological or surgical interventions. It underscores the importance of infant ERCP as a highly specialized and valuable technique. Guidelines and the available studies, however, do not clearly define the indications and optimal utilization of infant ERCP.

T1
TABLE 1:
Case series on infantile endoscopic retrograde cholangiopancreatography (>10 cases) published 1995 to 2019

The lack of guidelines is a possible explanation for the differences in indications, types of interventions performed and practical execution of ERCP procedures found among centers in this survey. This gap of knowledge could be overcome firstly by consensus among experts on the optimal use of infant ERCP and secondly by collaborative research. These steps could potentially improve the quality of infant ERCP care.

The trend of decreasing availability of infant ERCP duodenoscopes and facilities determined in this survey is an unwanted development as infant ERCP is an indispensable diagnostic and therapeutic tool. To counter this trend a concerted effort by the hepatology community is needed to secure availability of infant ERCP facilities in the whole of Europe. This effort could entail working with manufacturers to secure the development of a new duodenoscope for infant ERCP in the near future, establishing how ERCP care can best be secured in the circumstance of decreasing availability of facilities and determining the need and optimal distribution and organization of infant ERCP facilities in Europe. In the later deliberation access to this care needs to be weighed against sufficient volume of procedures to maintain skills and the costs of maintaining infant ERCP facilities. In addition, since developing a new duodenoscope for an infant ERCP will not be profitable for manufacturers, the possibilities to obtain support from regulators for manufacturers willing to develop a duodenoscope could be explored. The recent initiative by Olympus to provide the service of a “travelling scope,” a PJF 160 duodenoscope which is sent to centers on request within 1 to 2 working days, has temporarily overcome the problem of decreasing availability of ERCP facilities.

Main limitations of this survey are that not all countries were covered and not all hepatology centers in Europe responded. Because most major European hepatology centers have been included, these results are likely to be a fair representation of the availability and use of infant ERCP in Europe and Israel.

CONCLUSION

This survey establishes that availability in Europe (including Israel) is decreasing as several centers have been forced to abandon ERCP procedures due to duodenoscope failure since 2014. The recent initiative of a “travelling scope” provided by the manufacturer has temporarily overcome this problem. Infant ERCP is a low-volume procedure in all centers and substantial differences exist between ERCP centers in indications, types of interventions performed, and practical execution of ERCP in infants. A concerted effort by hepatology community is needed to secure future availability of this highly specialized and indispensable care in the whole of Europe. In addition, consensus on the optimal use of infant ERCP and collaborative research could potentially further improve the quality of care.

REFERENCES

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Keywords:

endoscopic retrograde cholangiopancreatography; infants; survey

Copyright © 2020 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition