Description

This ERN RARE-LIVER joint Webinar was held on 15 July 2025 with UMCG-Groningen, UKE-Hamburg and Amsterdam University Medical Centre.

CM is characterised by the abnormal dilatation of the bile duct, which can occur in the extrahepatic bile duct, intrahepatic bile ducts, or both. The bile duct of these patients is affected before birth. Some malformations are not uniformly dilatated but are more irregular, a condition termed the fusiform variant (7). CM occurs with a higher prevalence in Asian populations than in Western populations, where the incidence is estimated to be 1:100,000 to 1:150,000 live births. Recent advancements in diagnostic imaging during antenatal screening with ultrasound have increased the detection rates of CMs.The Todani classification system categories CMs into five types based on anatomical imaging or operative findings, each with its own treatment algorithm. Historically, the management of CMs involved drainage procedures, such as cystoduodenostomy or cystojejunostomy. These procedures have largely been abandoned due to their inability to resolve the underlying problem, the persistence of recurrent infections, and the risk of malignant transformation of the in situ malformation. The recognition of CM as a premalignant condition has led to a shift towards complete or near-complete cyst excision, making it the standard therapy for patients that are fit for surgery. The resection is followed by a biliodigestive reconstruction, typically via Roux-en-Y hepaticojejunostomy. 

For the genuinely a-symptomatic child with CM, determining the optimal timing of surgery is challenging due to the unknown risk of complications arising. For the adult with advanced age balancing the risk between CCA and perioperative risks is challenging. We address the evidence available for the European setting.

  • Diagnosis and classification of CM
  • Risk of CCA in CM
  • Resection vs follow up
  • Timing of therapy
  • Type of biliary reconstruction
  • Complications of resection and complication of wait

We invite you to share any questions or topics you’d like to see addressed in ERN upcoming webinar. Submit your questions here: https://ec.europa.eu/eusurvey/runner/f92346c1-f28b-f1b1-cfce-e980c347bb0b

Chairs

  
Joost PH Drenth

 

Joost PH Drenth is a gastroenterologist and professor of Hepatology with the Department of Gastroenterology and Hepatology of the Amsterdam University Medical Centre, The Netherlands. He was head of the Department of Gastroenterology and Hepatology of the Radboudumc, Nijmegen (2010-2023). He served as Editor-in-Chief of the UEG journal (2019-2024), and is the current vice-president of the UEG. His research interest include rare liver diseases, and initiated rigorous clinical trials in many rare diseases such as hereditary angiodysplasias, autoimmune disorders, and polycystic liver disease. His research endeavours resulted in  ~600 peer reviewed articles.

Speakers

  
Ruben de Kleine

Ruben de Kleine is a staff surgeon at the University Medical Centre Groningen in the Netherlands, where he works in the field of rare liver diseases for adults and children. His Ph.D. thesis was about choledochal malformations and paediatric acute liver failure. Since October 2018, he has performed robotic-assisted resections of choledochal malformations on adults and children. He is the lead for the biliary malformation group within the ERN Rare Liver.
Omid Madadi-Sanjani

Omid Madadi-Sanjani is a board certified general surgeon and paediatric surgeon, with special interest in paediatric hepatobiliary surgery working in the Department of Visceral Transplantation and the Department of Paediatric Surgery at the University Medical Centre Hamburg-Eppendorf. His research focus is the surgical management of rare paediatric liver diseases, mainly biliary atresia (BA) for which he is part of the evaluation process for German BA centralization of care and the screening for early BA detection. For that, he was part of the expert consultant group of the Federal Joint Committee for the screening of BA in Germany. 

 

Log in to post comments