The presentation and management of choledochocele (type III choledochal cyst): A 40-year systematic review of the literature.
BACKGROUND
Choledochoceles may cause biliary obstruction and harbor malignancy. We conducted a 40-year systematic review of the literature for this rare anomaly.


METHODS
PubMed and Cochrane databases were accessed 1975-2015 using terms "choledochocele" or "choledochal cyst". Studies reviewed that met the following criteria: English language, published 1975-2015 with human subjects.


RESULTS
325 patients with a choledochocele were identified, including 71 case reports and 254 cases within institutional reviews. 13 pediatric case reports of choledochocele exist, with abdominal pain being the most common symptom (n=11). The most frequent diagnostic and treatment modalities were ultrasound (n=10), and endoscopic sphincterotomy (n=5). No malignancies were reported. 58 adult case reports exist, with the most common presenting symptom being abdominal pain (n=54). Ultrasound was the frequently employed diagnostic modality (n=32). Open procedures were performed more often (n=30). Malignant lesions were identified in 5. In 42 institutional reviews, the frequency of choledochocele was 0.7%. Of those for whom treatment was reported, 69% underwent endoscopic sphincterotomy.


CONCLUSIONS
Choledochocele is a rare malformation. Similarities exist between pediatric and adult patients, but malignancy has only been reported in adults. An algorithm based on patient age, cyst size, lining and amenability to endoscopic resection may be considered as a treatment strategy for this uncommon condition.
Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.
Inna N
IN
Lobeck
Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.
Phylicia
P
Dupree
Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.
Richard A
RA
Falcone
Division of Gastroenterology, Hepatology & Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.
Tom K
TK
Lin
Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.
Andrew T
AT
Trout
Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States.
Jaimie D
JD
Nathan
Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, United States. Electronic address: Greg.Tiao@cchmc.org.
Greg M
GM
Tiao
D000328:Adult; D002648:Child; D015529:Choledochal Cyst / Q000000981:diagnostic imaging / Q000601:surgery; D006801:Humans; D016717:Sphincterotomy, Endoscopic; D014463:Ultrasonography
Biliary cyst; Biliary cystic malformation; Choledochal cyst; Choledochocele; Type III choledochal cyst