Scleroderma
Characteristics
Inflammation
Vascular sclerosis
Fibrosis of the skin and viscera
Classification
Limited
Involvement of skin up to elbows
Associated with CREST syndrome (Calcinosis, Raynauds, Oesophageal hypomotility, sclerodactyl, telangiactasia)
Pulmonary fibrosis can occur
Diffuse
Widespread effects
Effects on anaesthesia
Skin
Reduced mandibular motion and reduced mouth opening from tight skin (potential difficult airway, FOI may be req to facilitate intubation through small aperture)
Oral and nasal telangiactasia (may bleed profusely if traumatised)
Raynauds phenomenon (possible difficult IV, concern with Raynauds and ART lines, potentially difficult regional anaes, must attempt to avoid peripheral vasocon)
CVS
Chronic HTN and vasomotor instability - reduced vascular volume (risk hypotension at induction). Pericardial effusions, myocardial fibrosis, dilated cardiomyopathy, CCF
GI
Hypomotility of lower oesophague and reduced LOS tone (aspiration risk - RSI)
Malabsorption (Coagulopathy risk (vit K malabsorption) - regional may be CI, anaemia risk)
Resp
Pulmonary fibrosis: Reduced pulmonary compliance (sensitive to opioids)
Reduced diffusion capacity and vulnerability to hypoxaemia (difficulty oxygenating and ventilating (may require higher fiO2 and pressures
Pulmonary HTN (avoid factors which increase PVR -acidosis, hypoxaemia) - may progress to cor pulmonale
May benefit from post op HDU, benefit from chest physio, consider regional
Renal
Renal artery stenosis: Avoid anaesthetic drugs that are excreted mainly by renal clearance
NSAIDS CI in renal impairment; regional techniques may be useful
Monitor renal function
Fluid balance
Eyes
Protect to prevent corneal abrasions