- Preoperative Preparation
- For major procedures:
- an echocardiogram and electrocardiogram should be performed shortly before the scheduled case.
- Cardiac and respiratory decompensation can occur both during and after the operation, so it is crucial to seek the advice of both a cardiologist and a respiratory physician.
- For minor procedures:
- If the patient has normal cardiac function, a cardiac assessment is still suggested if the last evaluation was done more than a year prior.
- For major procedures:
- Anesthetic approach
- TIVA is highly recommended
- Avoid hypotensive anesthesia techniques (to reduce blood loss)
- Avoidance of Certain Anesthesia Agents: It's critical to avoid anesthesia agents that might trigger severe complications in patients with muscular dystrophies. These complications include hyperkalemia and rhabdomyolysis, both of which can have serious, potentially life-threatening consequences.
- Succinylcholine:
- Use of succinylcholine is contraindicated as it can lead to severe hyperkalemia and potentially fatal cardiac arrhythmias. In patients with muscular dystrophy, the risk is exacerbated due to upregulation of acetylcholine receptors outside the neuromuscular junction.
- Inhaled Anesthetics:
- Certain volatile anesthetics may increase the risk of malignant hyperthermia or rhabdomyolysis, especially in susceptible individuals. While not all patients with muscular dystrophy are at increased risk for malignant hyperthermia, caution is advised.
- Non-Depolarizing Muscle Relaxants:
- Muscular dystrophy patients may have prolonged effects of non-depolarizing muscle relaxants due to decreased muscle mass or altered sensitivity. If these agents are used, employ careful monitoring and consider reduced dosages.
- Succinylcholine:
- Extubation
- Whenever feasible, initiate the process of enabling the patient to breathe spontaneously at an early stage. This approach provides them with sufficient time to regain their tidal volumes as closely as possible back to pre-surgery levels.
- Avoid transitioning directly from extubation to solely using supplemental oxygen. Consider the use of non-invasive ventilation supports such as CPAP (Continuous Positive Airway Pressure) or BiPAP (Bilevel Positive Airway Pressure) post-extubation to support the patient's breathing until they can sustain adequate respiratory function on their own.
- Incentive spirometry is not indicated as it is otherwise ineffective in patients with respiratory weakness.
| Agent or step | Concern | Recommendation |
|---|---|---|
| Preoperative workup, major procedures | Cardiac and respiratory decompensation can occur both during and after the operation | Echocardiogram and electrocardiogram shortly before the case; seek advice from both a cardiologist and a respiratory physician. |
| Preoperative workup, minor procedures | — | With normal cardiac function, a cardiac assessment is still suggested if the last evaluation was more than a year prior. |
| Succinylcholine | Severe hyperkalemia and potentially fatal cardiac arrhythmias, with risk exacerbated by upregulation of acetylcholine receptors outside the neuromuscular junction | Contraindicated. |
| Volatile inhaled anesthetics | May increase the risk of malignant hyperthermia or rhabdomyolysis in susceptible individuals | Caution is advised, although not all patients with muscular dystrophy are at increased risk of malignant hyperthermia. |
| Non-depolarizing muscle relaxants | Prolonged effect from decreased muscle mass or altered sensitivity | If used, employ careful monitoring and consider reduced dosages. |
| General technique | — | TIVA is highly recommended; avoid hypotensive anesthesia techniques. |
| Return to spontaneous breathing | Tidal volumes need time to return as closely as possible to pre-surgery levels | Whenever feasible, begin spontaneous breathing at an early stage. |
| After extubation | Weak diaphragm and respiratory muscles can leave ventilation inadequate; traditional extubation-readiness criteria may not be reliable | Do not go straight from extubation to supplemental oxygen alone; consider CPAP or BiPAP support until respiratory function is sustained. |
| Incentive spirometry | Ineffective in patients with respiratory weakness | Not indicated. |
