Bile plug syndrome

Bile plug syndrome, also known as Inspissated bile syndrome, refersis 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 viscus bile (sludge) within its lumen ³. 

EpidemiologyClinical Presentation

The cause is unclear, although associations with total parenteral nutrition (TPN) and cystic fibrosis have been made. 

Risk factors for the formation of sludge include TPN(?)total parenteral nutrition, diuretics, cephalosporins, prematurity, congenital heart disease, hemolytic conditions, and disruption of the en- terohepaticenterohepatic circulation, which may be due to intestinal resection ³.

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

heThe clinical and radiographic findings may be identical to either biliary atresia or choledochal cyst. 

Radiology Features

 On US scans,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 CBDcommon bile duct contain sludge, which tends to be slightly hyperechogenic but without pos- teriorposterior acoustic shadowing (3,49) (Fig 7). Inspissated bile may resolve sponta- neously or with ursodeoxycholic acid; however, at times endoscopic retrograde cholangiopancreatographyorpercutane- ous drainage may be needed 3

Treatment

3 Although cystic fibrosis has been associated with a few reported cases, altered biliary dynamics associated with TPN offers a more plausible aetiology. This results in the production of hyper-concentrated viscous bile which ma)' he prone to inspissation, with resulting obstruction.¹

Bile plug syndrome is uncommon, although surgically correctable cause of extrahepatic biliary duct obstruction in the neonate. At present, ultrasonography mar be unable to differentiate this entity from a choledochal cyst, with an accurate diagnosis made only at the time of surgery.¹

nspissated Bile Syndrome (Bile-Plug Syndrome)

 . (47).³

Inspissated Bile Syndrome

Inspissated bile syndrome is an uncommon cause of jaundice in neonates. Sludge may be seen within the gallbladder as low-level echoes within the lumen (,,,,Fig 14), at times dependent. Inspissated bile is slightly more echogenic but does not cause shadowing. It may also be seen within the biliary ducts associated withcould cause partial or complete biliary ductal obstruction. The affected ducts may blend with the surrounding hepatic parenchyma, causing silhouetting of the bile ducts and thus difficulty. 

The obstructing material ranges from an isolated plug of sludge in determining thatthe common bile duct to extensive filling of the ducts are dilated (,2)system.

Inspissated bile syndromemay resolve spontaneously or with  use of ursodeoxycholic acid; however, sometimes endoscopic retrograde cholangiopancreatography or percutaneous drainage may be associated with massive hemolysis (Rh incompatibility), hemorrhage (intraabdominal, intracranial, or retroperitoneal), and increased enterohepatic circulation in various intestinal diseases (Hirschsprung disease, intestinal atresias, and stenoses) (,49–,51)required.²

Differential Diagnosis: 

• Choledochal cyst
• Biliary atresia