What are the considerations for patients with scleroderma undergoing general anesthesia?

Anesthetic Management of Scleroderma Patients

Patients with scleroderma undergoing general anesthesia require meticulous attention to airway management, cardiopulmonary complications, and temperature control, with the primary concerns being difficult intubation from microstomia and skin tightening, pulmonary hypertension, and renal crisis. 1, 2, 3

Preoperative Assessment

Critical Organ System Evaluation

Airway Assessment:

• Evaluate mouth opening capacity and temporomandibular joint mobility, as progressive fibrosis causes microstomia making laryngoscopy and intubation extremely difficult 1, 2
• Assess for skin tightening around the face and neck that restricts neck extension 1
• Document baseline airway anatomy as awake fiberoptic intubation may be necessary 1

Cardiopulmonary Function:

• Obtain pulmonary function tests (FVC, MIP, MEP, PCF) to assess for restrictive lung disease from pulmonary fibrosis 4, 2
• Perform echocardiography to evaluate for pulmonary hypertension and right ventricular dysfunction, which are major causes of perioperative mortality 2, 3
• Screen for pericardial effusion and myocardial fibrosis 2, 5

Renal and Gastrointestinal:

• Check baseline renal function as scleroderma renal crisis can be precipitated by perioperative stress 2, 3
• Assess for esophageal dysmotility and gastroesophageal reflux, which increases aspiration risk 2

Intraoperative Management

Airway Strategy

For patients with limited mouth opening (<3 cm) or significant facial/neck involvement:

• Use awake fiberoptic intubation as the gold standard approach 1
• Alternative: Shikani optical stylet under rapid sequence induction has been successfully used in localized scleroderma with adequate mouth opening 1
• Have multiple airway devices immediately available including video laryngoscopy 1

Anesthetic Technique

Preferred approach is total intravenous anesthesia (TIVA):

• Use propofol and remifentanil as primary agents 4
• Absolutely avoid succinylcholine due to risk of hyperkalemic cardiac arrest and rhabdomyolysis in patients with connective tissue disease 4
• Use non-depolarizing muscle relaxants (rocuronium, vecuronium) if needed 6

Hemodynamic Management

Maintain strict physiologic parameters:

• Keep mean arterial pressure within 10-20% of baseline to ensure adequate organ perfusion, particularly to kidneys and prevent renal crisis 4, 3
• Maintain SpO2 ≥95% continuously to prevent hypoxemia-related complications 4
• Avoid hypotension which can precipitate renal crisis 3

Temperature Control

Prevent hypothermia aggressively:

• Maintain normothermia throughout the procedure as temperature extremes can trigger vasospasm and Raynaud's phenomenon 6
• Use forced-air warming devices and warmed intravenous fluids 6

Positioning and Skin Protection

• Pad all pressure points meticulously as skin is fragile and prone to breakdown 6, 2
• Avoid excessive trauma during positioning which could cause skin blistering 6
• Be cautious with joint positioning as arthritis and contractures are common 2

Postoperative Management

Monitoring Requirements

All scleroderma patients require intensive monitoring:

• Admit to ICU or high-dependency unit for continuous cardiopulmonary monitoring for at least 24-48 hours 4
• Continue SpO2 monitoring continuously for minimum 24 hours postoperatively 4
• Monitor for delayed respiratory complications from restrictive lung disease 4

Watch for specific complications:

• Pulmonary edema and pulmonary hypertension (major causes of postoperative mortality) 3
• Acute renal crisis (can develop rapidly postoperatively) 3
• Aspiration pneumonia from gastroesophageal reflux 2

Oxygen Therapy

• Use supplemental oxygen cautiously as excessive oxygen can mask hypoventilation in patients with baseline restrictive lung disease 4
• Target SpO2 ≥95% but monitor respiratory rate and effort closely 4

Common Pitfalls to Avoid

• Never assume a normal airway - even localized scleroderma can progress to involve facial structures 1
• Do not use regional anesthesia as sole technique for major procedures, as positioning difficulties and skin changes make placement challenging 2
• Avoid drugs that cause histamine release (morphine, atracurium, mivacurium) as they may trigger vasospasm 6
• Do not discharge to regular ward - these patients require extended monitoring even after seemingly uncomplicated procedures 4, 3