Ages: anytime up to 7 years of age, but usually from 2-6 months of age
Clinical features:
(1) The child shows normal growth and development after birth.
(2) There is gradual (occasionally acute) onset of a number of clinical findings.
(3) The child shows progressive abdominal distention with ascites.
(4) There is a failure to thrive or emaciation.
(5) The child is often irritable.
(6) The child may show mild to moderate jaundice.
(7) Stools may be normal or pale.
(8) No history of trauma or surgery.
Laboratory findings:
(1) no evidence of liver dysfunction
(2) elevated bilirubin in the peritoneal fluid (greater than the serum concentration)
Diagnosis can usually be made with an imaging study (intravenous cholangiography, technetium 99Tc HIDA scan, others).
Occasionally the patient will form an inflammatory pseudocyst filled with bile-rich fluid between the liver and stomach.
Differential diagnosis:
(1) traumatic perforation of the common duct
(2) choledochal cyst
(3) acquired extrahepatic biliary atresia
Management:
(1) drainage of the accumulated peritoneal fluid
(2) usually heals spontaneously
Anastomosing the pseudocyst to the small bowel (done if confused with a ruptured choledochal cyst) can be fatal.
