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