Patients with Friedreich ataxia who face scoliosis correction or other major surgical procedures require a carefully adapted perioperative approach. Their cardiac and medical status, mobility needs, and decreased reserve of strength create specific risks that standard surgical protocols do not fully address.
This protocol applies to individuals with Friedreich ataxia who are undergoing scoliosis surgery or other major surgery, where standard post-operative and anesthetic management must be modified to account for the condition.
The protocol addresses post-operative mobilization strategy and specific anesthetic management considerations for this population — the complete structured guidance, including sequencing and monitoring parameters, is in the full protocol.
DOI: 10.1186/s13023-022-02568-3
We suggest early mobilization following surgery over standard post-operative management for individuals with Friedreich ataxia undergoing surgery.
Consideration should be given to appropriate management of peri-operative pain in people with Friedreich ataxia; consideration should be given to the use of nondepolarizing muscle relaxants, in particular accurate assessment of neuromuscular block throughout anesthesia; consideration should be given to avoiding risks associated with hyperkalemia; there should be careful monitoring of fluid balance and cardiovascular function in people with Friedreich ataxia undergoing anesthesia.
Propofol has been used in a large number of individuals with Friedreich ataxia and there is no published evidence that documents adverse events related to the use of propofol in this population.
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