1. Choledochocele
Oliver Bellevue

2. Background Choledochal cysts in United States 1:13,500.
Significantly more common in Asia, with rates as high as 1:1,000 in Japan.
Female dominance (3 to 4:1) in general but does not hold up for Type 3
20% prenatally diagnosed
60% diagnosed within first decade of life
20% diagnosed later in childhood or adulthood

3. 5 types by Todani in 1977

4. 5 types by Todani in 1977

5. Pancreaticobiliary maljunction
long common channel
reflux, damage to cyst wall
thought to be responsible for cystic dilation
also responsible for malignancy
80% of choledochal cysts have PBM, but not type 3
Type 3 is associated with prior cholecystectomy and Pancreas Divisum
increased sphincter pressure, acquired v congenital?
even without cystic dilation, long common channel is associated with increased risk of carcinoma

6. Pancreaticobiliary maljunction

7. Pancreaticobiliary maljunction
Long = insertion of the bile duct greater than 15 mm proximal to the
ampulla of Vater

8. Choledochocele
Can be lined with duodenal mucosa, biliary mucosa, or “unclassifiable mucosa”
Also can be mistaken for duodenal duplication
Large review of 878 cases in Japan
77.7 percent were type I
2.0 percent were type II
1.4 percent were type III (12 patients)
18.9 percent were type IV
PMID:

9. Type 3 - Choledochocele
O'Neill JA: Choledochal cyst. Curr Probl Surg 1992

10. Or 5 types…
Type A. The common bile duct terminates in the choledochocele, which drains into the duodenum.
Type B. The choledochocele drains into the adjacent portion of the common bile duct and thence via the papilla.
Type C. The common bile duct terminates in the choledochocele, which drains into the duodenum via a fistula. Also, the choledochocele communicates with the pancreatic duct and drains via the papilla.
Type D. Arrangement is the same as in type C, except there is no fistula between the choledochocele and duodenum.
Type E. The common bile duct, which ends in the choledochocele, is completely separate from the pancreatic duct.
Kagiyama S, Okazaki K, Yamamoto Y. Anatomic variants of choledochocele and manometric measurements of pressure in the cele and the orifice zone. Am J GastroenteroI 1987

11. Risk of Cancer
Fewer than 200 cases reported in English literature (2011)
Two types of reports in the literature
- cancer found at time choledochocele was identified
- drainage preformed with limited follow-up

12. 11 with complete follow up
Choledochocele, an Overlooked Diagnosis: Report of 15 Cases and Review of 56 Published Reports from 1984 to 1992 S. D. Ladas, I. Katsogridakis, P. Tassios, T. Tastemiroglou, T. Vrachliotis, S. A. Raptis Endoscopy, 1995
15 patients
Twelve patients were treated by endoscopic sphincterotomy, 3 treated surgically
11 with complete follow up
Eleven (76 %) of the 14 patients who had complete follow-up were symptom-free, with normal liver function tests at a median of 18 months (range 12-97).
Two patients reported episodes of acute cholangitis, and one patient died of a misdiagnosed ampullary carcinoma arising in the CDCL.

13. 6 patients with choledochocele
All treated with endoscopic drainage
8-13 year follow up
No malignancy
One patient with progressive, asymptomatic dilation

14. 17 patients with choledochocele
Endoscopy, 2002
17 patients with choledochocele
All treated with endoscopic sphincterotomy
3 year follow up
No malignancy

15. 28 patients with choledochoceles
80% managed with endoscopic drainage
32 mo follow up
No cancer

16. 21 patients, mean age 68 years
0.3% of all patient who had ERCP during study period
No follow up reported
2 patients with synchronous GB cancer, one with cholangiocarcinoma = rate of 14%
(None had undergone prior drainage procedure)

17. 11 patients
0.4% of all patient who had ERCP during study period
No follow up reported
3 patients with malignancy (27%) compared to 0.9% rate of malignancy in patients without choledochocele, p =

18. Age of Carcinoma Risk (All Types of Cysts)
PMID: