Bile plug syndrome

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. 

Epidemiology

Risk factors

Bile plug syndrome is usually seen in full term neonates. Risk factors for the formation of sludge include total parenteral nutrition, prematurity, diuretics, cephalosporins, congenital heart disease, increased bilirubin load due to haemolytic conditions, and disruption of the enterohepatic circulationinfection, which may be due to intestinal resection ³.

Although starvation, dehydration, cystic fibrosis has been associated, altered biliary dynamics associated with total parenteral nutrition is a more related aetiology, and malabsorptive syndromes ³.

Clinical presentation

The clinical and laboratory findings may be identical tomimic 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 butto 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, thus causingwhich can result in nonvisualisation of the duct due to 'silhouetting'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, caused either by trauma or due to instrumentation (e.g. hepatic biopsy), can appear similar to inspissated bile on sonography. ⁵.