Controversies in Choledochal Malformation in Children: An International Survey among Pediatric Hepatobiliary Surgeons and Gastroenterologists
Background: While congenital choledochal malformation (CCM) is relatively well known within the pediatric surgical and pediatric gastroenterological communities, many controversies and questions remain. Methods: In this paper, we will discuss the results of an international Delphi survey among membe...
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MDPI AG
2022-02-01
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| Series: | Journal of Clinical Medicine |
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| author | Jan B. F. Hulscher Joachim F. Kuebler Janneke M. Bruggink Mark Davenport Stefan Scholz Claus Petersen Omid Madadi-Sanjani Nagoud Schukfeh |
| author_facet | Jan B. F. Hulscher Joachim F. Kuebler Janneke M. Bruggink Mark Davenport Stefan Scholz Claus Petersen Omid Madadi-Sanjani Nagoud Schukfeh |
| author_sort | Jan B. F. Hulscher |
| collection | DOAJ |
| description | Background: While congenital choledochal malformation (CCM) is relatively well known within the pediatric surgical and pediatric gastroenterological communities, many controversies and questions remain. Methods: In this paper, we will discuss the results of an international Delphi survey among members of the European Reference Network RARE-LIVER and of the faculty of the Biliary Atresia and Related Diseases (BARD) network to identify the most common practices as well as controversies regarding diagnosis, treatment and follow-up of this still enigmatic disease. Results: Twenty-two individual respondents completed the survey. While there seems to be agreement on the definitions of CCM, preoperative workup, surgical approach and follow-up still vary considerably. The mainstay of treatment remains the removal of the entire extrahepatic biliary tract, clearance of debris both proximally and distally, followed by reconstruction with (according to 86% of respondents) a Roux-en-Y hepaticojejunostomy. Nonetheless, both laparoscopic and robotic-assisted resections are gaining ground with the suggestion that this might be facilitated by concentration of care and resources in specialized centers. However, long-term outcomes are still lacking. Conclusions: As even post-surgical CCM has to be considered as having premalignant potential, follow-up should be well-organized and continued into adulthood. This seems to be lacking in many centers. International cooperation for both benchmarking and research is paramount to improving care for this rare disease. |
| first_indexed | 2024-03-09T21:39:50Z |
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| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-09T21:39:50Z |
| publishDate | 2022-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-0925d118ddd447149841d7683a0ea68d2023-11-23T20:32:27ZengMDPI AGJournal of Clinical Medicine2077-03832022-02-01114114810.3390/jcm11041148Controversies in Choledochal Malformation in Children: An International Survey among Pediatric Hepatobiliary Surgeons and GastroenterologistsJan B. F. Hulscher0Joachim F. Kuebler1Janneke M. Bruggink2Mark Davenport3Stefan Scholz4Claus Petersen5Omid Madadi-Sanjani6Nagoud Schukfeh7Division of Pediatric Surgery, Department of Surgery, University Medical Center Groningen, 9800RB Groningen, The NetherlandsEuropean Reference Network RARE-LIVER, 20246 Hamburg, GermanyDivision of Pediatric Surgery, Department of Surgery, University Medical Center Groningen, 9800RB Groningen, The NetherlandsDepartment of Paediatric Surgery, Kings College Hospital, London SE5 9RS, UKDivision of Pediatric General and Thoracic Surgery, UPMC Children’s Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, PA 15224, USAEuropean Reference Network RARE-LIVER, 20246 Hamburg, GermanyEuropean Reference Network RARE-LIVER, 20246 Hamburg, GermanyEuropean Reference Network RARE-LIVER, 20246 Hamburg, GermanyBackground: While congenital choledochal malformation (CCM) is relatively well known within the pediatric surgical and pediatric gastroenterological communities, many controversies and questions remain. Methods: In this paper, we will discuss the results of an international Delphi survey among members of the European Reference Network RARE-LIVER and of the faculty of the Biliary Atresia and Related Diseases (BARD) network to identify the most common practices as well as controversies regarding diagnosis, treatment and follow-up of this still enigmatic disease. Results: Twenty-two individual respondents completed the survey. While there seems to be agreement on the definitions of CCM, preoperative workup, surgical approach and follow-up still vary considerably. The mainstay of treatment remains the removal of the entire extrahepatic biliary tract, clearance of debris both proximally and distally, followed by reconstruction with (according to 86% of respondents) a Roux-en-Y hepaticojejunostomy. Nonetheless, both laparoscopic and robotic-assisted resections are gaining ground with the suggestion that this might be facilitated by concentration of care and resources in specialized centers. However, long-term outcomes are still lacking. Conclusions: As even post-surgical CCM has to be considered as having premalignant potential, follow-up should be well-organized and continued into adulthood. This seems to be lacking in many centers. International cooperation for both benchmarking and research is paramount to improving care for this rare disease.https://www.mdpi.com/2077-0383/11/4/1148choledochal malformationhepaticojejunostomyDelphi surveybiliary atresia |
| spellingShingle | Jan B. F. Hulscher Joachim F. Kuebler Janneke M. Bruggink Mark Davenport Stefan Scholz Claus Petersen Omid Madadi-Sanjani Nagoud Schukfeh Controversies in Choledochal Malformation in Children: An International Survey among Pediatric Hepatobiliary Surgeons and Gastroenterologists Journal of Clinical Medicine choledochal malformation hepaticojejunostomy Delphi survey biliary atresia |
| title | Controversies in Choledochal Malformation in Children: An International Survey among Pediatric Hepatobiliary Surgeons and Gastroenterologists |
| title_full | Controversies in Choledochal Malformation in Children: An International Survey among Pediatric Hepatobiliary Surgeons and Gastroenterologists |
| title_fullStr | Controversies in Choledochal Malformation in Children: An International Survey among Pediatric Hepatobiliary Surgeons and Gastroenterologists |
| title_full_unstemmed | Controversies in Choledochal Malformation in Children: An International Survey among Pediatric Hepatobiliary Surgeons and Gastroenterologists |
| title_short | Controversies in Choledochal Malformation in Children: An International Survey among Pediatric Hepatobiliary Surgeons and Gastroenterologists |
| title_sort | controversies in choledochal malformation in children an international survey among pediatric hepatobiliary surgeons and gastroenterologists |
| topic | choledochal malformation hepaticojejunostomy Delphi survey biliary atresia |
| url | https://www.mdpi.com/2077-0383/11/4/1148 |
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