Bile plug syndrome

Infant with
jaundice. AP image from a percutaneous cholangiogram shows a dilated intrahepatic biliary tree and a markedly dilated extrahepatic biliary tree containing large filling defects within it. In this case the cholangiogram was therapeutic as well as diagnostic.The diagnosis was bile plug syndrome causing biliary obstruction.
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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 .

Epidemiology

Risk factors

Risk factors for the formation of sludge include total parenteral nutrition, diuretics, cephalosporins, prematurity, congenital heart disease, hemolytic conditions, and disruption of the enterohepatic circulation, which may be due to intestinal resection.

Although cystic fibrosis has been associated, altered biliary dynamics associated with total parenteral nutrition is a more related etiology.

Clinical presentation

The clinical findings may be identical to either biliary atresia or a choledochal cyst.

Radiographic features

Ultrasound

The radiological features may be similar to biliary atresia or choledochal cyst.

  • plug of viscous bile in the common bile duct and proximal biliary dilatation
  • the gallbladder and dilated common bile duct contain sludge, which tends to be slightly hyperechogenic but without posterior acoustic shadowing
  • within the biliary ducts could cause partial or complete biliary ductal obstruction
  • the affected ducts may blend with the surrounding parenchyma, causing silhouetting of the bile ducts
  • the obstructing material ranges from an isolated plug of sludge in the common bile duct to extensive filling of the system

Treatment and prognosis

Inspissated bile may resolve spontaneously or with use of ursodeoxycholic acid; however, sometimes endoscopic retrograde cholangiopancreatography (ERCP) or percutaneous drainage may be required.

Differential diagnosis