Inspissated
bile syndrome (IBS) is a rare condition in which thick intraluminal
bile, including
bile plugs,
sludge, or stones, blocks the
extrahepatic bile ducts in an
infant. A 5-week-old
female infant was admitted for evaluation of
jaundice and acholic stool.
Diagnostic tests, including ultrasound sonography,
magnetic resonance cholangiopancreatography, and a hepatobiliary scan, were not conclusive. Although the
diagnosis was unclear, the clinical and
laboratory findings improved gradually on
administration of urodeoxycholic
acid and
lipid emulsion containing omega-3
polyunsaturated fatty acids (PUFAs) for 3 weeks. However, a
liver biopsy was suggestive of
biliary atresia. This finding forced us to perform intraoperative
cholangiography, which revealed a patent
common bile duct with impacted thick
bile. We performed
normal saline irrigation and the symptom was improved, the final
diagnosis was IBS. Thus, we herein
report that IBS can be treated with omega-3 PUFAs as an alternative to surgical intervention.