We reviewed the records of 32 adult patients with choledochal cysts (C
DC) to determine the characteristics of the associated pancreatic dise
ase. Eighteen patients (56%) had 30 documented episodes of pancreatiti
s with epigastric pain and elevated serum amylase levels. Three patien
ts developed a prolonged course with a pancreatic phlegmon and one pat
ient died secondary to a pancreatic abscess after endoscopic retrograd
e cholangiopancreatography (ERCP). Pancreatitis occurred in all types
of CDC and was not related to the age, gender, or race of the patient.
There was an association with the size of the CDC: 90% of patients wi
th CDC greater than or equal to 5 cm developed pancreatitis compared w
ith only 9% of patients with CDC < 5 cm (p < 0.0004). In addition, ERC
P was performed in 14 patients and demonstrated an abnormal pancreatic
obiliary duct junction in eight (57%). All eight patients with an abno
rmal pancreaticobiliary junction developed pancreatitis compared with
only 2 out of 6 patients with normal pancreatic duct anatomy (p < 0.00
6). Patients undergoing surgical bypass rather than resection also ten
ded to have higher rates of pancreatitis (80 vs. 50%). One patient wit
h a Type I CDC and chronic pancreatitis was treated with surgical rese
ction of the CDC and pancreatic head; this combined procedure relieved
the pain. Microscopic examination of the CDC and the abnormal ''commo
n channel'' within the pancreas revealed identical fibrous thickening
of the duct walls with focal chronic inflammation and loss of surface
epithelium. In conclusion, these data stress the previously unrecogniz
ed high incidence of symptomatic pancreatic inflammatory disease that
accompanies adult CDC. Diagnostic ERCP and surgical manipulations of t
he pancreas should be done with care to avoid precipitating pancreatit
is. CDC resection is preferred to surgical bypass to avoid anastomotic
stricture with cholangitis and to minimize the chance for ongoing pan
creatitis.