What is steal syndrome in a patient with liver disease or cirrhosis?

Steal Syndrome in Liver Disease and Cirrhosis

Steal syndrome in patients with liver disease refers to compromised arterial perfusion and ischemia that occurs when blood flow is diverted (or "stolen") away from critical vascular territories, most commonly manifesting as splenic artery steal syndrome (SASS) after liver transplantation or hepatic artery steal syndrome in the context of vascular malformations. 1

Primary Context: Splenic Artery Steal Syndrome After Liver Transplantation

SASS is characterized by arterial hypoperfusion of the liver graft caused by excessive blood flow diversion into the splenic or gastroduodenal arteries, occurring in approximately 4.7% of patients after orthotopic liver transplantation. 2, 3

Clinical Presentation and Timing

• 93.7% of SASS cases are diagnosed within the first 2 months after liver transplantation, making early postoperative surveillance critical 3
• Abnormal liver function tests are the most common presentation (71.9% of cases), manifesting as elevated transaminases, cholestasis, and allograft dysfunction 2, 3
• Less common presentations include thrombocytopenia, acute graft failure, and persistent ascites 3
• If left untreated, SASS carries significant risk for postoperative morbidity and graft loss 2

Diagnostic Approach

Doppler ultrasound is the primary screening tool, demonstrating a high resistance index of the hepatic artery in 84.1% of affected patients. 3

• Conventional celiac trunk angiography confirms the diagnosis by demonstrating nonocclusive hepatic artery hypoperfusion with preferential flow to the splenic artery 2, 3
• Contrast-enhanced ultrasonography provides additional diagnostic utility 4
• Increased spleen volume (≥829 mL) before transplantation is suggestive of potential risk for developing SASS 3

Treatment Strategy

Splenic artery embolization (SAE) is the definitive treatment, performed in 94.7% of cases, and immediately reverses flow abnormalities with improvement in liver function tests in 96.3% of patients. 3, 4

• SAE should target the splenic artery trunk for optimal results 4
• The procedure provides immediate restoration of hepatic arterial perfusion as confirmed by post-procedure Doppler ultrasound 3

Secondary Context: Hepatic Artery Steal Syndrome in Vascular Malformations

In patients with hereditary hemorrhagic telangiectasia (HHT) and liver vascular malformations, steal syndrome presents differently:

Pathophysiology and Presentation

• Arteriovenous shunting through liver vascular malformations diverts blood away from the peribiliary plexus and mesenteric circulation, causing biliary ischemia and mesenteric angina 1
• This occurs through hepatic artery-to-hepatic vein or hepatic artery-to-portal vein shunting 1
• Symptoms include right upper quadrant pain, cholestasis, and in severe cases, biliary necrosis ("hepatic disintegration") 1

Management Principles

Hepatic artery embolization should be avoided in patients with liver involvement by HHT, as it is associated with significant morbidity. 1

• Embolization is absolutely contraindicated in patients with portosystemic shunting or biliary presentation 1
• It can only be considered in nontransplant candidates with intractable heart failure and documented hepatic artery steal syndrome 1
• Liver transplantation is the only curative treatment for acute biliary necrosis syndrome or intractable complications 1

Critical Clinical Pitfalls

• Do not delay diagnosis in the early post-transplant period—routine Doppler screening should be performed to identify high-resistance hepatic artery patterns 3
• Do not perform hepatic artery embolization in cirrhotic patients with portosystemic shunts or biliary symptoms, as this dramatically increases complication risk 1
• In cirrhotic patients with SASS and portal hypertension hypersplenism, splenectomy with pericardial devascularization surgery effectively redirects blood flow to the hepatic artery and improves liver function 5