Pediatr Gastroenterol Hepatol Nutr.  2016 Dec;19(4):286-290. 10.5223/pghn.2016.19.4.286.

Use of Omega-3 Polyunsaturated Fatty Acids to Treat Inspissated Bile Syndrome: A Case Report

• Jun WY
• Cho MJ
• Han HS
• Bae SH

• KMID: 2364771
• DOI: http://doi.org/10.5223/pghn.2016.19.4.286

Abstract

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.

Keyword

Inspissated bile syndrome; Cholestasis; Omega-3 fatty acids

MeSH Terms

Bile Ducts, Extrahepatic
Bile*
Biliary Atresia
Biopsy
Cholangiography
Cholangiopancreatography, Magnetic Resonance
Cholestasis
Common Bile Duct
Diagnosis
Diagnostic Tests, Routine
Fatty Acids, Omega-3
Fatty Acids, Unsaturated*
Female
Humans
Infant
Jaundice
Liver
Sewage
Ultrasonography
Fatty Acids, Omega-3
Fatty Acids, Unsaturated
Sewage

Figure

• 

  Fig. 1 Liver biopsy shows an expanded and inflamed portal tract contains proliferative bile ducts filled with inspissated bile. And there is panlobular cholestasis (H&E stain, ×400).

• 

  Fig. 2 Cholangiography at the time of operation after contrast showed patent gallbladder and common bile duct with good passage through duodenum.

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  Fig. 3 The laboratory findings during the patient's clinical course showed gradual improvement with SMOFlipid® emulsion (Fresenius Kabi Austria GmbH, Graz, Austria), which containing omega-3 PUFAs. After intraoperative cholangiography with normal saline irrigation, laboratory findings improved markedly, confirming inspissated bile syndrome. AST: aminotransferase, ALT: alanine aminotransferase, TB: total bilirubin, DB: direct bilirubin, MRCP: magnetic resonance cholangiopancreatography, DISIDA: diisopropyl iminodiacetic acid scan, PUFA: polyunsaturated fatty acid, UDCA: urodeoxycholic acid.

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