Scleroderma 

 

 

Considerations 

 

  • Potential difficult airway (microstomia, ↓ neck mobility, bleeding nasal/oral telangiectasia)

  • Aspiration risk (esophageal dysmotility, hypotonic lower esophageal sphincter)

  • Multi-system disease:

    • Cardiovascular: hypertension, coronary disease, myocardial fibrosis & LV failure, arrhythmia

    • Resp: restrictive lung disease, pulmonary fibrosis, pulmonary hypertension, cor pulmonale

    • Renal failure & “renal crisis”

    • Skin: raynaud's, vasoconstriction, sensitive to cold:

      • ​Radial arterial line may be contraindicated 

  • Consequences of dermal thickening, contractures:

    • Difficult vascular access/positioning/monitoring

    • Nerve entrapment/pain syndromes

  • Medications: immunosuppressant, vasodilators (ACE inhibitors), pain medications 

 

 

Anesthetic Goals/Issues  

 

  • Secure airway safely & avoid aspiration:

    • Potential difficulty with bag mask ventilation, laryngoscopy & surgical technique

    • Consider regional over GA

  • Recognize several potential precipitants for hemodynamic instability:

    • Volume depletion, hypertension 

    • Myocardial dysfunction, arrythmia, pulmonary hypertension, cor pulmonale

  • Avoid precipitants of:

    • Vasoconstriction episodes: e.g., hypothermia, sympathetic stimulation

    • Pulmonary hypertension 

  • Address pulmonary disease

  • Potential for hypoxemia (fibrosis), acute lung injury/barotrauma (restrictive lung disease) 

  • Post-op ventilation

 

 

Conflicts 

 

  • Aspiration vs. cardiac disease & hemodynamic instability

  • Aspiration vs. difficult airway

  • ? difficult regional vs. risk of post-op ventilation

  • TEE vs compromised esophagus 

 

 

Treatment of Limb Raynaud's Crisis Due to Arterial Line 

 

  • Emergency situation 

  • Warm limb 

  • Consult vascular surgery 

  • Consider sympathetic block (i.e., stellate ganglion) 

 

 

Pregnancy Considerations 

 

  • Essentially as above 

  • Very careful airway management & aspiration prevention 

  • Possible prolonged neural blockade reported in literature so very carefully titrated epidural warranted 

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