Faculty Collaboration Database

Presentation and Clinical Outcomes of Choledochal Cysts in Children and Adults: A Multi-institutional Analysis. JAMA Surg 2015 Jun;150(6):577-84

Date

04/30/2015

Pubmed ID

25923827

DOI

10.1001/jamasurg.2015.0226

Scopus ID

2-s2.0-84934311658 (requires institutional sign-in at Scopus site)   88 Citations

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.

Author List

Soares KC, Kim Y, Spolverato G, Maithel S, Bauer TW, Marques H, Sobral M, Knoblich M, Tran T, Aldrighetti L, Jabbour N, Poultsides GA, Gamblin TC, Pawlik TM

Author

Thomas Clark Gamblin MD Professor in the Surgery department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Abdominal Pain
Adult
Aged
Child
Child, Preschool
Cholangiopancreatography, Endoscopic Retrograde
Choledochal Cyst
Female
Humans
Infant
Male
Middle Aged
Preoperative Care
Prognosis