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Original Investigation
June 2015

Presentation and Clinical Outcomes of Choledochal Cysts in Children and Adults: A Multi-institutional Analysis

Author Affiliations
  • 1Department of Surgery, School of Medicine, The Johns Hopkins University, Baltimore, Maryland
  • 2Department of Surgery, School of Medicine, Emory University, Atlanta, Georgia
  • 3Department of Surgery, University of Virginia Healthcare System, Charlottesville
  • 4Department of Surgery, Curry Cabral Hospital, Lisbon, Portugal
  • 5Department of Surgery, Central Lisbon Hospital Centre, Lisbon, Portugal
  • 6Department of Surgery, School of Medicine, Stanford University, Palo Alto, California
  • 7Department of Surgery, Ospedale San Raffaele, Milan, Italy
  • 8Department of Surgery, Université Catholique de Louvain, Brussels, Belgium
  • 9Department of Surgery, Medical College of Wisconsin, Milwaukee
JAMA Surg. 2015;150(6):577-584. doi:10.1001/jamasurg.2015.0226

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.