Epidemiology

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Etiology

• Arterial Infusion of Floxuridine (5-Fluorodeoxyuridine) (see Floxuridine)
• Biliary Tract Surgery
  • Physiology: vascular injury
• Chemoembolization
• Critical Illness (Curr Opin Crit Care, 2013) [MEDLINE]
  • Physiology: secondary sclerosing cholangitis (see Secondary Sclerosing Cholangitis)
  • Clinical: cirrhosis can develop rapidly
  • Prognosis: poor
• Hereditary Hemorrhagic Telangiectasia (see Hereditary Hemorrhagic Telangiectasia)
  • Physiology: biliary ischemia
• Hypercoagulable State (see Hypercoagulable States)
• Liver Transplant (see Liver Transplant)
  • Epidemiology
    • Liver Transplant is the Most Common Etiology of Ischemic Cholangiopathy
    • Risk of Ischemic Cholangiopathy Post-Liver Transplant is Highest with the Use of Non-Beating Heart Donor Grafts
• Radiation Therapy (see Radiation Therapy) (J Hepatol, 1994) [MEDLINE]

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Physiology

Background

• Hepatic Artery (Via the Peribiliary Plexus) Exclusively Supplies the Major Bile Ducts

Features of Ischemic Cholangiopathy

• Ischemia of the Perihilar Intrahepatic/Extrahepatic Bile Ducts

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Diagnosis

Right Upper Quadrant (RUQ) Ultrasound (see Abdominal-Pelvic Ultrasound)

• Doppler Ultrasound is Useful in These Cases Occurring Post-Liver Transplant to Evaluate for Hepatic Artery Thrombosis

Endoscopic Retrograde Cholangiopancreatography (ERCP) (see Endoscopic Retrograde Cholangiopancreatography)

• Features
  • Multiple Intrahepatic/Extrahepatic Strictures with Diffuse Irregularity and/or Beading of the Ducts
  • Discrete Biliary Strictures Involving the Large Perihilar Bile Ducts: less common
  • Extravasation of Bile/Contrast

MRI Pancreatography (MRCP) (see Magnetic Resonance Cholangiopancreatography)

• xxxx

Liver Biopsy (see Liver Biopsy, [[Liver Biopsy]])

• Usually Not Diagnostic

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Clinical Manifestations

Gastrointestinal/Hepatic Manifestations

• Biliary Stricture with Obstruction
  • Cholestatic Pattern of Liver Function Tests (LFT’s) (Elevated Liver Function Tests, [[Elevated Liver Function Tests]])
  • Clay-Colored Stools (see Stool Discoloration)
  • Dark Urine (see Urine Discoloration)
  • Hyperbilirubinemia/Jaundice (see Hyperbilirubinemia)
  • Pruritus (see Pruritus)

Complications

• Ascending Cholangitis (see Acute Cholangitis)
• Pyogenic Liver Abscess (see Pyogenic Liver Abscess)

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Treatment

Management of Ischemic Cholangiopathy Post-Liver Transplant (see Liver Transplant)

• Endoscopic Dilation/Stenting of Bile Duct
• Re-Liver Transplantation: usually required for cases which occur within the first few months following liver transplant

Management of Ischemic Cholangiopathy in Hereditary Hemorrhagic Telangiectasia (see Hereditary Hemorrhagic Telangiectasia)

• Bevacizumab (Avastin) (see Bevacizumab, [[Bevacizumab]]): has been reported to reverse ischemic cholangiopathy in hereditary hemorrhagic telangiectasia (J Hepatol, 2013) [MEDLINE]

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References

• Common bile duct stricture as a late complication of upper abdominal radiotherapy. J Hepatol. 1994;20(6):693 [MEDLINE]
• Ischemic-like cholangiopathy with secondary sclerosing cholangitis in critically ill patients. Am J Gastroenterol. 2007;102(6):1221 [MEDLINE]
• Hypoxic liver injury and cholestasis in critically ill patients. Curr Opin Crit Care. 2013 Apr;19(2):128-32 [MEDLINE]
• Improvement of ischemic cholangiopathy in three patients with hereditary hemorrhagic telangiectasia following treatment with bevacizumab. J Hepatol. 2013 Jul;59(1):186-9. Epub 2013 Feb 21 [MEDLINE]