Mehdi Ouaissi1, Reza Kianmanesh2, Emilia Ragot3, Jacques Belghiti3, Barbara Wildhaber4, Gennaro Nuzzo5, Remi Dubois6, Yann Revillon7, Daniel Cherqui8, Daniel Azoulay8, Chritian Letoublon9, François-René Pruvot10, Adeline Roux11, Jean-Yves Mabrut12, Jean-François Gigot13. 1. Department of General and Digestive Surgery, Timone Hospital, Marseille, France. 2. Department of Digestive and Endocrine Surgery, Robert Debré Hospital, Reims, France. 3. Department of HPB Surgery, Beaujon Hospital, Clichy, France. 4. University Center of Pediatric Surgery of Western Switzerland, University Hospitals of Geneva, Switzerland. 5. Department of HPB Surgery, Gemelli University Hospital, Roma, Italy. 6. Department of Pediatric Surgery, Mother and Children Hospital, Lyon, France. 7. Department of Pediatric Digestive Surgery, Neker Hospital, Paris, France. 8. Department of Digestive and HPB Surgery, Henri Mondor Hospital, Creteil, France. 9. Department of Digestive Surgery, Michallon Hospital, Grenoble, France. 10. Department of Digestive Surgery and Transplantation, Claude Huriez Hospital, Lille, France. 11. Hospices Civils de Lyon, Pôle Information Médicale Evaluation Recherche, Unité de Recherche Clinique, Lyon, France. 12. Department of Digestive Surgery and Hepatic Transplantation, Hôpital de la Croix-Rousse, Lyon, France. 13. Department of Abdominal Surgery and Transplantation, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain (UCL), Brussels, Belgium. Electronic address: jfgigot@hotmail.com.
Abstract
AIM: To compare clinical presentation, operative management and short- and long-term outcomes of congenital bile duct cysts (BDC) in adults with children. METHODS: Retrospective multi-institutional Association Francaise de Chirurgie study of Todani types I+IVB and IVA BDC. RESULTS: 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. CONCLUSION: BDC is a more indolent disease in children compared to adults, except for Todani type IV-A BDC.
AIM: To compare clinical presentation, operative management and short- and long-term outcomes of congenital bile duct cysts (BDC) in adults with children. METHODS: Retrospective multi-institutional Association Francaise de Chirurgie study of Todani types I+IVB and IVA BDC. RESULTS: 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. CONCLUSION: BDC is a more indolent disease in children compared to adults, except for Todani type IV-A BDC.
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