Amyloidosis

 

 

Background 

 

  • Refers to the extracellular tissue deposition of fibrils

  • Primary amyloidosis: is a plasma cell disorder marked by the accumulation of immunoglobulin light chains.

  • Secondary amyloidosis: is observed in association with several other conditions, including multiple myeloma, rheumatoid arthritis, & a prolonged antigenic challenge, such as may be produced by chronic infection

 

 

Considerations

 

  • Difficult airway: macroglossia, laryngo-tracheo-bronchial tree involvement, stridor

  • Aspiration risk 

  • Multisystem infiltrative disease: 

    • Cardiovascular: 

      • Restrictive cardiomyopathy

      • Diastolic dysfunction 

      • Complete heart block

      • Risk of sudden death

      • Coronary heart disease 

    • Respiratory: interstitial lung disease

    • Renal: nephrotic syndrome & renal failure

    • GI: dysphagia, risk of aspiration secondary to autonomic/peripheral neuropathy

    • Nervous system: autonomic neuropathy, mixed sensory/motor neuropathy

    • Heme: bleeding risk → factor X deficiency, ↓ coagulation factors, platelet dysfunction 

  • Potential co-existing secondary causes: e.g. multiple myeloma & rheumatoid arthritis 

  • Medications: steroids/chemotherapy

 

 

Goals & Conflicts

 

  • RSI for possible gastroparesis, aspiration risk vs. potential difficult airway

  • Prior to neuraxial/regional technique:

    • Document pre-existing neurodeficits 

    • Confirm coagulation profile & clinical assessment of coagulopathy 

  • Mindful of restrictive cardiomyopathy, hemodynamic instability due to autonomic neuropathy