Congenital bile duct cyst (BDC) is a more indolent disease in children compared to adults, except for Todani type IV-A BDC: results of the European multicenter study of the French Surgical Association.
To compare clinical presentation, operative management and short- and long-term outcomes of congenital bile duct cysts (BDC) in adults with children.



Retrospective multi-institutional Association Francaise de Chirurgie study of Todani types I+IVB and IVA BDC.



During the 37-year period to 2011, 33 centers included 314 patients (98 children; 216 adults). The adult population included more high-risk patients, with more active, more frequent prior treatment (47.7% vs 11.2%; p < 0.0001), more complicated presentation (50.5% vs 35.7%; p = 0.015), more synchronous biliary cancer (11.6% vs 0%; p = 0.0118) and more major surgery (23.6% vs 2%; p < 0.0001), but this latter feature was only true for type I+IVB BDC. Compared to children, the postoperative morbidity (48.1% vs 20.4%; p < 0.0001), the need for repeat procedures and the status at follow-up were worse in adults (27% vs 8.8%; p = 0.0009). However, severe postoperative morbidity and fair or poor status at follow-up were not statistically different for type IVA BDC, irrespective of patients' age. Synchronous cancer, prior HBP surgery and Todani type IVA BDC were independent predictive factors of poor or fair long-term outcome.



BDC is a more indolent disease in children compared to adults, except for Todani type IV-A BDC.
Department of General and Digestive Surgery, Timone Hospital, Marseille, France.
Mehdi
M
Ouaissi
Department of Digestive and Endocrine Surgery, Robert Debré Hospital, Reims, France.
Reza
R
Kianmanesh
Department of HPB Surgery, Beaujon Hospital, Clichy, France.
Emilia
E
Ragot
Department of HPB Surgery, Beaujon Hospital, Clichy, France.
Jacques
J
Belghiti
University Center of Pediatric Surgery of Western Switzerland, University Hospitals of Geneva, Switzerland.
Barbara
B
Wildhaber
Department of HPB Surgery, Gemelli University Hospital, Roma, Italy.
Gennaro
G
Nuzzo
Department of Pediatric Surgery, Mother and Children Hospital, Lyon, France.
Remi
R
Dubois
Department of Pediatric Digestive Surgery, Neker Hospital, Paris, France.
Yann
Y
Revillon
Department of Digestive and HPB Surgery, Henri Mondor Hospital, Creteil, France.
Daniel
D
Cherqui
Department of Digestive and HPB Surgery, Henri Mondor Hospital, Creteil, France.
Daniel
D
Azoulay
Department of Digestive Surgery, Michallon Hospital, Grenoble, France.
Chritian
C
Letoublon
Department of Digestive Surgery and Transplantation, Claude Huriez Hospital, Lille, France.
François-René
FR
Pruvot
Hospices Civils de Lyon, Pôle Information Médicale Evaluation Recherche, Unité de Recherche Clinique, Lyon, France.
Adeline
A
Roux
Department of Digestive Surgery and Hepatic Transplantation, Hôpital de la Croix-Rousse, Lyon, France.
Jean-Yves
JY
Mabrut
Department of Abdominal Surgery and Transplantation, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain (UCL), Brussels, Belgium. Electronic address: jfgigot@hotmail.com.
Jean-François
JF
Gigot
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001662:Biliary Tract Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D015529:Choledochal Cyst / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D015897:Comorbidity; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000209:etiology; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult