期刊
JOURNAL OF CLINICAL MEDICINE
卷 11, 期 4, 页码 -出版社
MDPI
DOI: 10.3390/jcm11041148
关键词
choledochal malformation; hepaticojejunostomy; Delphi survey; biliary atresia
This paper discusses the controversies and common practices regarding the diagnosis, treatment and follow-up of congenital choledochal malformation (CCM) based on an international Delphi survey. The survey results show that there is agreement on the definitions of CCM, preoperative workup, surgical approach and follow-up, but significant variations still exist in actual practice.
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.
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