Bile plug syndrome, also known as inspissated bile syndrome, is an uncommon cause of jaundice in neonates. Refers to a rare extrahepatic mechanical obstruction of the major bile duct in the perinatal period caused by viscous bile (sludge) within its lumen 3.
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Epidemiology
Risk factors
Risk factors for the formation of sludge include total parenteral nutrition, prematurity, diuretics, cephalosporins, congenital heart disease, increased bilirubin load due to hemolytic conditions, infection, starvation, dehydration, cystic fibrosis, and malabsorptive syndromes 3.
Clinical presentation
The clinical and laboratory findings may mimic those of biliary atresia or a choledochal cyst.
Radiographic features
Ultrasound
Sonographic features of biliary inspissation include:
plug of viscous bile (i.e. sludge) in the intrahepatic common bile duct, with proximal biliary dilatation
the sludge tends to be slightly hyperechogenic to the liver parenchyma, without posterior acoustic shadowing; sludge in the gallbladder may be less echogenic than biliary duct sludge
can cause partial or complete biliary ductal obstruction
sludge in an affected duct may be isoechoic to the liver parenchyma, which can result in nonvisualisation of the duct or the misleading appearance of a narrow duct 2
the amount of obstructing material ranges from an isolated plug of sludge in the common bile duct to extensive filling of the biliary system
Treatment and prognosis
Inspissated bile may resolve spontaneously or with use of ursodeoxycholic acid; however, in some cases, endoscopic retrograde cholangiopancreatography (ERCP) or percutaneous drainage (optionally with irrigation/lavage) may be required 3.
Differential diagnosis
Blood in the gallbladder lumen and/or bile ducts, can appear similar to inspissated bile on sonography 5.
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