Kevin C Soares1, Yuhree Kim1, Gaya Spolverato1, Shishir Maithel2, Todd W Bauer3, Hugo Marques4, Mafalda Sobral4, Maria Knoblich5, Thuy Tran6, Luca Aldrighetti7, Nicolas Jabbour8, George A Poultsides6, T Clark Gamblin9, Timothy M Pawlik1. 1. Department of Surgery, School of Medicine, The Johns Hopkins University, Baltimore, Maryland. 2. Department of Surgery, School of Medicine, Emory University, Atlanta, Georgia. 3. Department of Surgery, University of Virginia Healthcare System, Charlottesville. 4. Department of Surgery, Curry Cabral Hospital, Lisbon, Portugal. 5. Department of Surgery, Central Lisbon Hospital Centre, Lisbon, Portugal. 6. Department of Surgery, School of Medicine, Stanford University, Palo Alto, California. 7. Department of Surgery, Ospedale San Raffaele, Milan, Italy. 8. Department of Surgery, Université Catholique de Louvain, Brussels, Belgium. 9. Department of Surgery, Medical College of Wisconsin, Milwaukee.
Abstract
IMPORTANCE: Choledochal cysts (CCs) are rare, with risk of infection and cancer. OBJECTIVE: To characterize the natural history, management, and long-term implications of CC disease. DESIGN, SETTING, AND PARTICIPANTS: A total of 394 patients who underwent resection of a CC between January 1, 1972, and April 11, 2014, were identified from an international multi-institutional database. Patients were followed up through September 27, 2014. Clinicopathologic characteristics, operative details, and outcome data were analyzed from May 1, 2014, to October 14, 2014. INTERVENTION: Resection of CC. MAIN OUTCOMES AND MEASURES: Management, morbidity, and overall survival. RESULTS: Among 394 patients, there were 135 children (34.3%) and 318 women (80.7%). Adults were more likely to present with abdominal pain (71.8% vs 40.7%; P < .001) and children were more likely to have jaundice (31.9% vs 11.6%; P < .001). Preoperative interventions were more commonly performed in adults (64.5% vs 31.1%; P < .001), including endoscopic retrograde pancreatography (55.6% vs 27.4%; P < .001), percutaneous transhepatic cholangiography (17.4% vs 5.9%; P < .001), and endobiliary stenting (18.1% vs 4.4%; P < .001)). Type I CCs were more often seen in children vs adults (79.7% vs 64.9%; P = .003); type IV CCs predominated in the adult population (23.9% vs 12.0%; P = .006). Extrahepatic bile duct resection with hepaticoenterostomy was the most frequently performed procedure in both age groups (80.3%). Perioperative morbidity was higher in adults (35.1% vs 16.3%; P < .001). On pathologic examination, 10 patients (2.5%) had cholangiocarcinoma. After a median follow-up of 28 months, 5-year overall survival was 95.5%. On follow-up, 13 patients (3.3%), presented with biliary cancer. CONCLUSIONS AND RELEVANCE: Presentation of CC varied between children and adults, and resection was associated with a degree of morbidity. Although concomitant cancer was uncommon, it occurred in 3.0% of the patients. Long-term surveillance is indicated given the possibility of future development of biliary cancer after CC resection.
IMPORTANCE: Choledochal cysts (CCs) are rare, with risk of infection and cancer. OBJECTIVE: To characterize the natural history, management, and long-term implications of CC disease. DESIGN, SETTING, AND PARTICIPANTS: A total of 394 patients who underwent resection of a CC between January 1, 1972, and April 11, 2014, were identified from an international multi-institutional database. Patients were followed up through September 27, 2014. Clinicopathologic characteristics, operative details, and outcome data were analyzed from May 1, 2014, to October 14, 2014. INTERVENTION: Resection of CC. MAIN OUTCOMES AND MEASURES: Management, morbidity, and overall survival. RESULTS: Among 394 patients, there were 135 children (34.3%) and 318 women (80.7%). Adults were more likely to present with abdominal pain (71.8% vs 40.7%; P < .001) and children were more likely to have jaundice (31.9% vs 11.6%; P < .001). Preoperative interventions were more commonly performed in adults (64.5% vs 31.1%; P < .001), including endoscopic retrograde pancreatography (55.6% vs 27.4%; P < .001), percutaneous transhepatic cholangiography (17.4% vs 5.9%; P < .001), and endobiliary stenting (18.1% vs 4.4%; P < .001)). Type I CCs were more often seen in children vs adults (79.7% vs 64.9%; P = .003); type IV CCs predominated in the adult population (23.9% vs 12.0%; P = .006). Extrahepatic bile duct resection with hepaticoenterostomy was the most frequently performed procedure in both age groups (80.3%). Perioperative morbidity was higher in adults (35.1% vs 16.3%; P < .001). On pathologic examination, 10 patients (2.5%) had cholangiocarcinoma. After a median follow-up of 28 months, 5-year overall survival was 95.5%. On follow-up, 13 patients (3.3%), presented with biliary cancer. CONCLUSIONS AND RELEVANCE: Presentation of CC varied between children and adults, and resection was associated with a degree of morbidity. Although concomitant cancer was uncommon, it occurred in 3.0% of the patients. Long-term surveillance is indicated given the possibility of future development of biliary cancer after CC resection.
Authors: Kevin C Soares; Seth D Goldstein; Mounes A Ghaseb; Ihab Kamel; David J Hackam; Timothy M Pawlik Journal: Pediatr Surg Int Date: 2017-03-31 Impact factor: 1.827
Authors: T Peter Kingham; Victoria G Aveson; Alice C Wei; Jason A Castellanos; Peter J Allen; Daniel P Nussbaum; Yinin Hu; Michael I D'Angelica Journal: Curr Probl Surg Date: 2020-06-30 Impact factor: 1.909