Solitary or multifocal strictures of variable length
• Cholangiography: Gold standard for diagnosis
Appearance may be identical to PSC with “beading” of biliary tree (alternating stenosis, normal ducts, and mild dilatation)
Biliary casts appear as filling defects within duct lumen
• CT: Scattered irregular biliary dilatation with bile duct wall thickening and hyperenhancement
Presence of intrahepatic biloma or liver infarct should prompt careful assessment for HAT/HAS
Bile duct casts appear as intraductal hyperdense material
CT not sensitive for early stage ischemic injury
• MR: Linear high T1 signal intensity may be visualized within dilated central ducts, characteristic of biliary cast
PATHOLOGY
• Classically due to hepatic artery thrombosis, but possible without arterial compromise (ischemic-type biliary lesions)
• Also associated with prolonged warm and cold ischemic time, ABO incompatibility, and chronic rejection
CLINICAL ISSUES
• Initial treatment: Endoscopic or percutaneous dilatation/stenting of strictures and clearing of biliary casts
• Roux-en-Y hepaticojejunostomy for extrahepatic strictures unresponsive to dilatation/stenting
• Retransplantation may be necessary in patients with secondary biliary cirrhosis, recurrent cholangitis, or progressive cholestasis
TERMINOLOGY
Synonyms
• Ischemic cholangitis, ischemic cholangiopathy
Definitions
• Nonanastomotic biliary strictures in liver allograft originally described in setting of hepatic artery thrombosis (HAT) or stenosis (HAS), but now known to occur due to a wide variety of other microangiopathic and immunological injuries
IMAGING
General Features
• Best diagnostic clue
Nonanastomotic biliary strictures in liver allograft
• Location
Can involve intrahepatic &/or extrahepatic ducts
Predominant involvement of middle 1/3 of common bile duct and hepatic duct confluence > intrahepatic ducts
2 common patterns
– Strictures beginning at hilum and extending peripherally
– Multiple scattered intrahepatic strictures
• Morphology
Can be solitary or multifocal strictures
Variable length: Short or long segment
Radiographic Findings
• Cholangiography (ERCP or PTC) is gold standard for diagnosis of ischemic cholangitis
Cholangiographic appearance may be nearly identical to primary sclerosing cholangitis
Luminal irregularity of bile ducts with beaded appearance (alternating sites of stenosis, normal ducts, and mild dilatation)
– Strictures evolve over time, beginning as sites of irregularity and developing into fibrotic strictures
– Ductal narrowing with upstream dilatation
– Rare diffuse duct necrosis and biliary sloughing
Biliary casts appear as filling defects within duct lumen
May demonstrate communication of bile ducts with intrahepatic bilomas
CT Findings
• Scattered irregular biliary dilatation with bile duct wall thickening and hyperenhancement
Presence of intrahepatic biloma or liver infarct in post-transplant setting should prompt careful assessment of hepatic artery for HAT/HAS
– Doppler US to screen, then CTA confirmation if Doppler positive
Bile duct casts, highly suggestive of ischemic cholangiopathy, appear as linear hyperdense material within bile duct
– May not be readily distinguishable from stone on CT (both may appear hyperdense)
Biliary necrosis, debris, and bilomas with advanced ischemia (particularly in setting of HAS/HAT)
• CT not sensitive for early stage ischemic-type biliary lesions (ITBL)
Transplanted liver may not develop biliary dilatation despite severe ductal stenosis
If high clinical suspicion for ITBL, proceed to cholangiography (ERCP, PTC, or MRCP)
• CTA can show hepatic artery narrowing or thrombosis
MR Findings
• Strong correlation between MRCP and cholangiography
High sensitivity, specificity, and predictive values for evaluation of ischemic-type biliary injury
• Hepatobiliary contrast agents (i.e., Eovist) can be used for cholangiographic images in hepatobiliary phase
Most often utilized to evaluate for strictures at hepaticojejunostomy
• T2WI, MRCP, and T1WI C+ Eovist cholangiographic images demonstrate luminal irregularity, stenosis, and scattered biliary ductal dilatation
• May be associated with T2-hyperintense intrahepatic bilomas or liver infarcts
• Linear high T1 signal intensity may be visualized within dilated central ducts, characteristic of biliary cast
Extremely uncommon in absence of ischemic cholangiopathy and virtually diagnostic
MR allows distinction between cast (T1 hyperintense) and stones (hypointense on all pulse sequences)
• Advantage of noninvasively evaluating other biliary complications (anastomotic stricture, stones, leak, etc.)
• MRA can show hepatic artery thrombosis or stenosis
Ultrasonographic Findings
• Grayscale ultrasound
Poor sensitivity for early stage ischemic-type biliary injury
May show intrahepatic ductal dilatation and thickening
Biliary casts appear as echogenic material within dilated bile ducts
Advanced biliary ischemia due to HAT or HAS may result in presence of intrahepatic fluid collections (bilomas)
Extrahepatic biliary dilatation is nonspecific finding in post-transplant liver and does not necessarily imply ischemic cholangiopathy
– Nonobstructive dilatation of extrahepatic ducts (without intrahepatic biliary dilatation) may be due to papillary dyskinesia or discrepancy between size of donor and recipient ducts
• Pulsed Doppler
Evaluate for evidence of HAT or HAS
– Hepatic artery stenosis (or chronic HAT with collaterals)
Turbulent flow within hepatic artery with focal aliasing at site of stenosis
Usually occurs at or near anastomosis and affects 11% of patients (mean 3 months after surgery)
Tardus parvus waveform (systolic acceleration time > 100 msec): Rounded spectral Doppler waveforms with delayed systolic upstrokes
Intrahepatic arterial resistive index < 0.5
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