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