A 45-year-old man presented with a history of right upper quadrant pain. There was neither jaundice nor palpable abdominal mass. Laboratory values, including liver function tests, were within normal limits.
Prepared by Peter Hrehorovich, MD, Ruwini de Silva, MD, and
Suresh Maximin, MD, Department of Radiology, Sisters of Charity
Medical Center, Staten Island, NY.
A 45-year-old man presented with a history of right upper
quadrant pain. There was neither jaundice nor palpable abdominal
mass. Laboratory values, including liver function tests, were
within normal limits.
Adult presentation of a variation of a Todani type 1C
choledochal cyst, specifically a fusiform dilatation with the
additional complication of a degree of biliary obstruction from a
stricture at the distal common bile duct.
Initial plain films of the abdomen showed no abnormality. The
liver and spleen were normal in size.
An abdominal ultrasound disclosed multiple dependent foci within
the gallbladder, suggesting tiny calculi or small foci of sludge
(figure 1). There was mild-to-moderate central intrahepatic ductal
dilatation with marked extrahepatic ductal dilatation out of
proportion to that seen in the intrahepatic system. This suggested
the possibility of a choledochal cyst rather than obstruction from
a stone or a mass.
Contrast-enhanced computed tomography (CT) of the abdomen
confirmed mild-to-moderate central intrahepatic biliary ductal
dilatation (figure 2). There was also marked dilatation of the
extrahepatic ductal system consistent with a choledochal cyst. The
pancreas was unremarkable, and the remainder of the examination was
within normal limits.
Hepatobiliary imaging with 7mCi Tc 99m mebrofenin administered
intravenously revealed prompt excretion into a somewhat prominent
intrahepatic biliary tree (figure 3). There was slight delay in
gallbladder visualization, which occurred at 2 hours, ascribed to
chronic cholecystitis. Ductal dilatation was consistent with
separate intrahepatic and extrahepatic biliary ductal
An endoscopic retrograde cholangiopancreatography (ERCP) (figure
4) confirmed the presence of a 6-cm area of fusiform dilatation
involving the common bile duct with the cystic duct inserting
directly into the cyst. Above this fusiform dilatation, there was a
more uniform, generalized biliary ductal dilatation, suggesting
that there may be a distal obstructive component. There was an
anomalous union of the common bile duct and the pancreatic duct
into a very long common channel with a stricture noted at the
distal portion of the common bile duct, immediately following the
termination of the choledochal cyst, and prior to the union with
the pancreatic duct. Otherwise, the pancreatic duct was of normal
Cyst excision with Roux-en-Y hepaticojejunostomy was performed,
and the patient recovered without complication. Pathology of the
specimen was consistent with chronic inflammation, and revealed no
evidence of malignancy. No gallstones were identifiable at
Choledochal cysts, cystic dilatation of the common bile duct,
are an uncommon but potentially serious condition that has been
reported throughout the world in various age groups. Reported cases
have ranged in age from infancy to 80 years, with predominance in
children and women. The pathogenesis of the cysts is a matter of
considerable discussion. Babbit
proposed the common channel theory. He postulated that an abnormal
pancreaticobiliary duct junction allowed reflux of pancreatic
secretions into the biliary system at a critical time of its
formation, resulting in enzymatic destruction of the duct wall with
consequent cystic dilatation. Alternative theories propose that
these cysts form a part of a spectrum of embryonic malformations of
the biliary and pancreatic duct systems.
Regardless of the etiology, multiple abnormalities have been
reported and classified. Todani's classification is a useful format
for cataloging the reported cases.
Clinical presentation is often very nonspecific. Intermittent
right upper quadrant discomfort is frequently the only symptom. The
classic triad of abdominal pain, jaundice, and a right upper
quadrant mass is reported in less than one third of the cases.
Consequently, a high index of suspicion is required to recognize
choledochal cysts when various imaging studies are ordered for
patients presenting with vague abdominal complaints.
The present case report presents the sonographic, isotope, and CT
findings demonstrating the presence of choledochal cysts.
Endoscopic retrogradecholangiopancreatography provided the definite
diagnosis in this reported case.
Choledochal cysts carry a risk for malignancy, cholelithiasis,
pancreatitis, and liver abscesses. They are associated with portal
hypertension due to cystic compression of the portal vein and may
result in ascites. Bile peritonitis due to cyst rupture has also
been reported. Consequently, once a definitive diagnosis has been
established, surgical intervention is indicated.