What is the management plan for a patient with celiac artery occlusion and autosplenectomy?

Management of Celiac Artery Occlusion with Autosplenectomy

The management of celiac artery occlusion with autosplenectomy requires prompt revascularization if the patient is symptomatic, with endovascular therapy (angioplasty and stenting) as the first-line treatment. 1

Diagnostic Evaluation

1. Clinical Assessment:

   • Evaluate for typical symptoms: postprandial abdominal pain, weight loss, food aversion
   • Physical examination may reveal an abdominal bruit
   • Laboratory findings may show anemia, leucopenia, electrolyte abnormalities, and hypoalbuminemia due to malnutrition 1

2. Imaging Workup:

   • CT Angiography (CTA): First-line imaging with 94-95% sensitivity and specificity
     • Should include arterial and venous phases with 1mm slices
     • Will confirm celiac artery occlusion and autosplenectomy 1
   • Duplex Doppler ultrasound: Useful as a screening tool and for follow-up
     • Should be performed in fasting state to avoid bowel gas interference
     • Celiac artery peak systolic velocity >240 cm/s indicates >70% stenosis 1
   • Mesenteric angiography: For confirmation and treatment planning
     • Should be performed in lateral projection during both inspiration and expiration
     • Evaluates collateral circulation development 1

Treatment Algorithm

1. Asymptomatic Patients

• No indication for prophylactic revascularization in asymptomatic patients with celiac artery occlusion 1
• Regular clinical follow-up to monitor for symptom development

2. Symptomatic Patients

• First-line treatment: Endovascular therapy (angioplasty and stenting) 1

  • Advantages: Less invasive, shorter recovery time
  • Caution: Risk of stent-associated thrombosis has been reported 2

• Second-line treatment: Open surgical options if endovascular approach fails

  • Surgical release of median arcuate ligament (if MALS is the cause)
  • Open mesenteric bypass (offers improved patency and lower re-intervention rates) 1
  • Consider vascular reconstruction when needed 1

3. Special Considerations for Autosplenectomy

• Vaccination: Administer vaccines against encapsulated organisms

  • Pneumococcal vaccine (PCV13 followed by PPSV23)
  • Haemophilus influenzae type b vaccine
  • Meningococcal vaccines (MenACWY and MenB)
  • Annual influenza vaccination

• Antibiotic prophylaxis: Consider for high-risk procedures

  • Educate patient about increased infection risk
  • Low threshold for antibiotic treatment with febrile illnesses

Follow-up Protocol

• Clinical evaluation at 1,3,6, and 12 months after intervention 1
• Imaging surveillance:
  • Duplex ultrasound or CTA at 6 months and annually thereafter
  • Monitor for stent patency, restenosis, or development of collateral circulation 1

Potential Complications

• Endovascular treatment complications:

  • Distal mesenteric embolization
  • Branch perforation
  • Dissection
  • Stent dislodgement or thrombosis 1

• Long-term complications:

  • Development of pancreaticoduodenal artery aneurysms (PDAAs) due to increased collateral flow 2
  • PDAAs can be treated with coil embolization if detected 2
  • Restenosis requiring reintervention

Clinical Pearls and Pitfalls

• Pearl: Collateral circulation, particularly through pancreaticoduodenal arcades from the superior mesenteric artery, often prevents significant ischemic symptoms despite celiac occlusion 3

• Pitfall: Stent-associated thrombosis is a significant risk with endovascular treatment of celiac stenosis 2. Close monitoring and appropriate antiplatelet therapy are essential.

• Pearl: In cases where both celiac artery occlusion and pancreaticoduodenal artery aneurysms are present, the aneurysms can be successfully treated with coil embolization while addressing the underlying celiac stenosis 2

• Pitfall: Failure to identify celiac artery occlusion before major abdominal surgery (such as pancreaticoduodenectomy) can lead to severe complications 4