Scleroderma

Question 1

A 45-year-old female is listed for an urgent fixation of her ankle on the trauma list. She has a history of scleroderma and has never had an anaesthetic. You are asked to review her prior to her procedure.

What is scleroderma?

Answer 1

• Autoimmune, multi-system disease that is caused by an increase in collagen production.
• Typically occurs in females between the age of 30 and 50 years.
• Can be either limited or diffuse with various effects on physiological systems.
• “CREST” syndrome is limited: calcinosis, Raynaud’s, oesophageal
  dysmotility, sclerodactyly and telangiectasia.
• Diffuse scleroderma:
• Pulmonary: interstitial fibrosis and pulmonary hypertension.
• Cardiac: pericarditis and pericardial effusion.
• Renal: glomerulosclerosis.
• Gastrointestinal: dysmotility and malabsorption.
• Musculoskeletal: joint contractures and arthritis.

Question 2

What are the main concerns when anaesthetising this patient?

A, B, C…?

Answer 2

Airway:
* This patient is high risk for a difficult airway due to facial deformity and contractures in severe disease. A suitable plan should be made given findings of a thorough airway assessment.

• Increased potential for aspiration due to reflux secondary to oesophageal dysmotility that should be incorporated into the airway plan.

Respiratory:
* Interstitial fibrosis and restrictive lung disease could make ventilation challenging.

• Pulmonary hypertension increases the risk of perioperative morbidity and mortality.

Cardiovascular:
* Cardiac involvement can include hypertension, left-sided cardiac failure, arrhythmias and pulmonary hypertension. Careful assessment with a low threshold for investigations and discussion with the
multidisciplinary team are key when pre-assessing this patient.

• Susceptible to vasospasm if hypothermic. Meticulous temperature monitoring and warming are essential.

Question 3

What are the main concerns when anaesthetising this patient?

General….?

Answer 3

General:
* Joint contractures can lead to difficult patient positioning. Consider positioning the patient in the correct surgical position while awake so as not to move them once anaesthetised.

• Venous access may be challenging due to scleroderma skin changes.
• Regional anaesthesia may be favourable but challenging due to
  musculoskeletal degeneration.
• Consider postoperative high dependency or intensive care due to
  challenging management in a high-risk patient.
• Potential for drug interactions if the patient is on regular treatment.

Question 4

The patient takes 10 mg prednisolone daily. How should this be managed during the perioperative period?

Answer 4

• Due to the risk of adrenal insufficiency, steroid replacement is necessary:
• 100mg intravenous hydrocortisone at induction followed by an infusion of 200 mg hydrocortisone over 24 hours.
• Continue the hydrocortisone infusion postoperatively if the
  patient remains nil by mouth.
• When the patient resumes eating and drinking, give a double dose of hydrocortisone (or equivalent) for up to a week after surgery.

Question 5

The patient states that she has been feeling increasingly short of breath during the last few weeks.

How do you proceed?

Answer 5

• Thorough history focusing on the patient’s associated symptoms including chest pain, limitations on daily activity, ankle swelling, syncope, fatigue and triggers.
• Patient examination (cardiovascular and respiratory).
• Ensure a low threshold for investigations due to the risk of bilateral cardiac involvement and pulmonary hypertension, to include:
• ECG (essential).
• Echo.
• Chest X-ray.
• Early discussion with multidisciplinary team regarding the timing of surgery, the implications of the patient’s comorbidities on surgery and an anaesthetic, and perioperative management. Senior clinician involvement is essential due to complexity of this patient.

Question 6

Surgery is delayed and the patient’s echocardiogram shows raised pulmonary arterial pressures.

What are the physiological goals when anaesthetising a patient with pulmonary hypertension?

Answer 6

• Avoid increases in pulmonary vascular resistance through prevention of hypoxia, hypercapnia, hypothermia, acidosis and pain.
• Maintain coronary perfusion pressures by monitoring and preserving cardiac output and systemic vascular resistance. Blood loss should be corrected rapidly.
• Minimise arrhythmias and maintain normal heart rate and regularity.
• Ensure close and invasive monitoring of blood pressure and cardiac
  output.

Question 7

What is your choice for induction of anaesthesia in this patient?

Answer 7

• The options for anaesthesia include both general and regional techniques. Given the patient’s cardiovascular and respiratory comorbidities, a regional technique is favourable, though may be challenging secondary to the musculoskeletal complications of disease.
• Ensure patient consent, AAGBI monitoring (with intra-arterial blood pressure monitoring), resus equipment, difficult airway trolley and emergency drugs readily available, including vasopressor and inotropic support.
• An experienced senior anaesthetist should be present.
• Ensure careful patient positioning and padding of pressure points.
• Carry out a regional anaesthetic technique: a spinal with a popliteal
  nerve block/catheter.