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


METHODS
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.


METHODS
Resection of CC.


METHODS
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
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.
Department of Surgery, School of Medicine, The Johns Hopkins University, Baltimore, Maryland.
Kevin C
KC
Soares
Department of Surgery, School of Medicine, The Johns Hopkins University, Baltimore, Maryland.
Yuhree
Y
Kim
Department of Surgery, School of Medicine, The Johns Hopkins University, Baltimore, Maryland.
Gaya
G
Spolverato
Department of Surgery, School of Medicine, Emory University, Atlanta, Georgia.
Shishir
S
Maithel
Department of Surgery, University of Virginia Healthcare System, Charlottesville.
Todd W
TW
Bauer
Department of Surgery, Curry Cabral Hospital, Lisbon, Portugal.
Hugo
H
Marques
Department of Surgery, Curry Cabral Hospital, Lisbon, Portugal.
Mafalda
M
Sobral
Department of Surgery, Central Lisbon Hospital Centre, Lisbon, Portugal.
Maria
M
Knoblich
Department of Surgery, School of Medicine, Stanford University, Palo Alto, California.
Thuy
T
Tran
Department of Surgery, Ospedale San Raffaele, Milan, Italy.
Luca
L
Aldrighetti
Department of Surgery, Université Catholique de Louvain, Brussels, Belgium.
Nicolas
N
Jabbour
Department of Surgery, School of Medicine, Stanford University, Palo Alto, California.
George A
GA
Poultsides
Department of Surgery, Medical College of Wisconsin, Milwaukee.
T Clark
TC
Gamblin
Department of Surgery, School of Medicine, The Johns Hopkins University, Baltimore, Maryland.
Timothy M
TM
Pawlik
D015746:Abdominal Pain / Q000209:etiology; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015529:Choledochal Cyst / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D011300:Preoperative Care; D011379:Prognosis