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Duchenne Muscular Dystrophy:
Considerations for Surgery
Introduction
• Planning and proactive assessment and
management of risk is key to safe surgery
– full assessments of heart and lungs important
• Various events may require general
anaesthesia
– Those related to DMD (e.g. muscle biopsy, joint
contracture surgery, spinal surgery, gastrotomy)
– Unrelated to DMD (e.g. acute surgical events)
Introduction
• Condition-specific issues must be considered
for planning of safe surgery
• Surgery should be done in a full-service
hospital with experience of patients with
DMD: doctors should be informed of all
medications
• Consideration must given to “stress steroid”
coverage during surgery, for people treated
with steroids at home.
Anaesthetic Considerations
• Always risks with anaesthetics: special
considerations for DMD include
– Exclusive use of a total intravenous anaesthetic (risk
of malignant hyperthermia-like reactions and
rhabdomyolysis with inhalation anaesthetic agents
such as halothane and isoflurane)
– Absolute contraindication of depolarising muscle
relaxants e.g. suxamethonium chloride
(succinylcholine) due to risk of fatal reactions
Blood-loss Minimisation
• Minimising blood loss is important
especially in major surgery such as spinal
fusion
– Necessary to use mildly hypotensive anaesthetics,
crystalloid bone allograft, and cell-saver technology
– Other interventions can be considered e.g. use of
aminocaproic acid or tranexamic acide to diminish
intraoperative bleeding
– Postoperative anticoagulation with heparin and/or
aspirin is inappropriate
– Use of compression stockings or sequential compression
for prevention of deep-vein thrombosis might be
indicated
Cardiac Considerations
• An echocardiogram and electrocardiogram should be
performed prior to general anaesthesia.
• They should also be performed before undergoing
conscious sedation or regional anaesthesia if the last
investigation was more than one year previously, or if
there has been an abnormal echocardiogram in the
preceding 7-12 months.
• For local anaesthesia, an echocardiogram should be
performed if there had been an abnormal result
obtained previously
Respiratory Considerations (1)
• Even if someone with DMD already has problems with the
breathing muscles, certain measures can make surgery
safer, though there will still be an increased risk.
• A pre-operative assessment of breathing function in a
centre familiar with DMD is very important
– Need for adequate respiratory support during induction of
maintenance of, and recovery from procedural sedation or
general anaesthesia.
– In particular, minimise risk of post-procedure endotracheal
extubation failure, postoperative atelectasis and pneumonia
– Goals can be achieved by providing non-invasively assisted
ventilation and assisted cough after surgery for patients with
significant respiratory-muscle weakness, as indicated by subthreshold preoperative pulmonary function tests
Respiratory Considerations (2)
• Pre-operative training in and postoperative use of noninvasive ventilation and assisted cough is necessary for
patients with baseline peak cough flow below 270L/min or
with baseline maximum expiratory pressure is below 50cm
water (measurements for older teenage/adult patients).
• Pre-operative training in and postoperative use of noninvasive ventilation strongly recommended for patients
with baseline FVC below 50% predicted, and necessary if
FVC below 30% predicted.
• Incentive spirometry not indicated
– Potential lack of efficacy in patients with respiratory muscle
weakness
– Availability of preferred alternatives e.g. mechanical
insufflations-exsufflation
Respiratory Considerations (3)
• After careful consideration of risks and
benefits, patients with significant respiratorymuscle weakness might be eligible for surgery,
albeit with increased risk, if they are highly
skilled pre-operatively in the use of noninvasive ventilation and assisted cough
• Physiotherapists should always be involved if
someone with DMD is having surgery
References & Resources
• The Diagnosis and Management of Duchenne
Muscular Dystrophy, Bushby K et al, Lancet
Neurology 2010 9 (1) 77-93 & Lancet
Neurology 2010 9 (2) 177-189
– Particularly references, p186-188
• The Diagnosis and Management of Duchenne
Muscular Dystrophy: A Guide for Families
• TREAT-NMD website: www.treat-nmd.eu
• CARE-NMD website: www.care-nmd.eu