Controversies in Choledochal Malformations: A Survey among Dutch Pediatric Surgeons

Biliary Atresia and other cholestatic childhood diseases are rare conditions affecting the function and/or anatomy along the canalicular-bile duct continuum, characterised by onset of persistent cholestatic jaundice during the neonatal period. Biliary atresia (BA) is the most common among these, but still has an incidence of only 1 in 10-19,000 in Europe and North America. Other diseases such as the genetic conditions, Alagille syndrome (ALGS) and Progressive Familial Intrahepatic Cholestasis (PFIC), are less common. Choledochal malformations are amenable to surgical correction and require a high index of suspicion. The low incidence of such diseases hinder patient-based studies that include large cohorts, while the limited numbers of animal models of disease that recapitulate the spectrum of disease phenotypes hinders both basic research and the development of new treatments. Despite their individual rarity, collectively BA and other cholestatic childhood diseases are the commonest indications for liver transplantation during childhood. Here, we review the recent advances in basic research and clinical progress in these diseases, as well as the research needs. For the various diseases, we formulate current key questions and controversies and identify top priorities to guide future research.

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“…Preliminary data from a recent Dutch survey highlight that: MRCP was routinely used in 71% and the more invasive ERCP in 29% of departments [66].…”

Section: Choledochal Malformationmentioning

confidence: 99%

Biliary atresia and other cholestatic childhood diseases: Advances and future challenges

Verkade

Bezerra

Davenport

et al. 2016

Journal of Hepatology

141

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“…Early surgery might prevent CM-related symptoms, while later surgery might diminish surgery-related complications [11][12][13]. In the Netherlands, most resections for CM are performed via a laparotomy [14]. However, the laparoscopic approach is gaining popularity [14][15][16][17].…”

Section: Introductionmentioning

confidence: 99%

“…In the Netherlands, most resections for CM are performed via a laparotomy [14]. However, the laparoscopic approach is gaining popularity [14][15][16][17].…”

Section: Introductionmentioning

confidence: 99%

Choledochal Malformation in Children: Lessons Learned from a Dutch National Study

et al. 2017

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Introduction A choledochal malformation (CM) is a rare entity, especially in the Western world. We aimed to determine the incidence of CM in the Netherlands and the outcome of surgery for CM in childhood. Methods All pediatric patients who underwent a surgical procedure for type I-IV CM between 1989 and 2014 were entered into the Netherlands Study group on choledochal cyst/malformation. Patients with type V CM were excluded from the present analysis. Symptoms, surgical details, short-term (\30 days) and long-term ([30 days) complications were studied retrospectively. Results Between January 1989 and December 2014, 91 pediatric patients underwent surgery for CM at a median age of 2.1 years (0.0-17.7 years). All patients underwent resection of the extrahepatic biliary tree with restoration of the continuity via Roux-en-Y hepaticojejunostomy. Twelve patients (12%) were operated laparoscopically. Short-term complications, mainly biliary leakage and cholangitis, occurred in 20 patients (22%), without significant correlations with weight or age at surgery or surgical approach. Long-term postoperative complications were mainly cholangitis (13%) and anastomotic stricture (4%). Eight patients (9%) required radiological intervention or additional surgery. Surgery before 1 year of age (OR 9.3) and laparoscopic surgery (OR 4.4) were associated with more postoperative long-term complications. We did not observe biliary malignancies during treatment or follow-up. Conclusion Surgery for CM carries a significant short-and long-term morbidity. Given the low incidence, we would suggest that (laparoscopic) hepatobiliary surgery for CM should be performed in specialized pediatric surgical centers with a wide experience in laparoscopy and hepatobiliary surgery.

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“…Moreover, there have been concerns that, even after cyst excision, the risk of malignancy remains in the bile duct epithelium remote from the cyst, and increases during long-term follow-up 3,4,18 . In children, a questionnaire-based survey among Dutch paediatric surgeons demonstrated a wide variety of opinions regarding diagnosis, treatment and follow-up, indicating that these challenges are not exclusive to adults 22 .…”

Section: Discussionmentioning

confidence: 99%

Survey of the attitudes of hepatopancreatobiliary surgeons in northern Europe to resection of choledochal cysts in asymptomatic Western adults

et al. 2019

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BackgroundTodani type 1 and 4 choledochal cysts are associated with a risk of developing cholangiocarcinoma. Resection is usually recommended, but data for asymptomatic Western adults are sparse. The aim of this study was to investigate diagnostic interpretation and attitudes towards resection of bile ducts for choledochal cysts in this subgroup of patients across northern European centres.MethodsThirty hepatopancreatobiliary centres were provided with magnetic resonance cholangiopancreatograms and asked to discuss the management of six cases: asymptomatic non‐Asian women, aged 30 or 60 years, with variable common bile duct (CBD) dilatations and different risk factors in the setting of a multidisciplinary team (MDT). The Fleiss κ value was calculated to estimate overall inter‐rater agreement.ResultsFor all case scenarios combined, 83·3 and 86·7 per cent recommended resection for a CBD of 20 and 26 mm respectively, compared with 19·4 per cent for a CBD of 13 mm (P < 0·001). For patients aged 30 and 60 years, resection was recommended in 68·5 and 57·8 per cent respectively (P = 0·010). There was a trend towards recommending resection in the presence of a common channel, most pronounced in the 60‐year‐old patient. High amylase levels in the CBD aspirate led to recommendations to resect, but only for the 13‐mm CBD dilatation. There were no differences related to centre size or region. MDT discussion was associated with recommendations to resect. Inter‐rater agreement was 73·3 per cent (κ = 0·43, 95 per cent c.i. 0·38 to 0·48).ConclusionThe inter‐rater agreement to resect was intermediate, and the recommendation was dependent mainly on the diameter of the CBD dilatation.

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Controversies in Choledochal Malformations: A Survey among Dutch Pediatric Surgeons

Cited by 5 publications

References 42 publications

Biliary atresia and other cholestatic childhood diseases: Advances and future challenges

Biliary atresia and other cholestatic childhood diseases: Advances and future challenges

Choledochal Malformation in Children: Lessons Learned from a Dutch National Study

Survey of the attitudes of hepatopancreatobiliary surgeons in northern Europe to resection of choledochal cysts in asymptomatic Western adults

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