What Duchenne Muscular Dystrophy (DMD) Patients Who Need to Have Surgery Under Anesthesia Should Know

While medical advancements have significantly improved the quality of life for DMD patients, surgical interventions remain an important aspect of treatment for managing complications such as scoliosis, fractures, and orthopedic deformities. However, due to the complex nature of DMD, surgery and anesthesia management in these patients require specialized care and a deep understanding of the disease’s impact on the body. In this article, we will explore the nuances of surgery and anesthesia in DMD patients, discussing key considerations, risks, and best practices.

DMD Surgery

Orthopedic and Spinal Surgeries

As DMD progresses, many patients experience orthopedic complications such as scoliosis, contractures, fractures, and joint deformities. These issues often necessitate surgical interventions to maintain mobility, improve quality of life, and prevent further complications. The most common surgeries for DMD patients include:

• Spinal Fusion for Scoliosis: Scoliosis, an abnormal curvature of the spine, is common in DMD patients, especially as they lose the ability to walk and their muscles weaken. A spinal fusion surgery is often required to stabilize the spine and prevent severe deformities that can lead to respiratory complications. The procedure involves the implantation of rods and screws to straighten and fuse the spine.
• Tendon and Joint Releases: Tendon contractures, where tendons become shortened and less flexible, are common in DMD. Tendon releases or lengthening procedures can help improve range of motion and reduce discomfort. Joint surgery may also be performed to address deformities and improve posture and mobility.
• Fracture Repair: As bone strength diminishes due to the progressive muscle weakness and the impact of corticosteroids often used to treat DMD, patients are more prone to fractures. Surgical intervention may be needed to stabilize fractures and ensure proper healing.
• Gastrostomy (G-tube) Insertion: As DMD patients age, they may experience difficulty swallowing due to weakening muscles. Gastrostomy tube placement provides a way to deliver nutrition directly to the stomach, reducing the risk of aspiration and ensuring proper nutrition.

Also Read This: Nutrition Guideline: Foods to Eat and Avoid

Challenges in DMD Surgery

While these surgeries are often necessary, they present unique challenges. DMD patients are at higher risk of complications during and after surgery due to their compromised muscle function, respiratory system, and cardiac health. Preoperative evaluations need to account for:

1. Muscle Weakness and Anesthesia Risks: Muscle weakness and the potential for airway complications require careful planning when considering anesthesia. Respiratory function must be closely monitored.
2. Cardiovascular Issues: Many DMD patients develop heart problems, such as dilated cardiomyopathy, which can increase the risk of anesthesia and surgery-related complications.
3. Risk of Infections: DMD patients are more vulnerable to infections due to muscle weakness and the use of immunosuppressive medications like corticosteroids.
4. Bone Fragility: Bone fragility makes fracture repair more difficult, and the risk of injury is higher during surgical procedures.

DMD Anesthesia

Challenges of Anesthesia in DMD

Anesthesia in patients with DMD poses significant challenges. As DMD affects the muscles, including the diaphragm and other respiratory muscles, these patients are at a higher risk of respiratory complications during surgery. The primary concerns regarding anesthesia in DMD patients include:

1. Respiratory Management:
   • Hypoventilation: Due to weakened respiratory muscles, patients may experience inadequate ventilation during anesthesia. This requires careful monitoring of oxygen levels and respiratory function during the perioperative period.
   • Use of Mechanical Ventilation: Most DMD patients with advanced stages of the disease require mechanical ventilation during and after surgery. Non-invasive ventilation (NIV) via a CPAP or BiPAP machine may also be used pre- and postoperatively for those with milder respiratory involvement.
2. Anesthesia Drug Sensitivity:
   • Muscle Relaxants: Patients with DMD can have an increased sensitivity to certain muscle relaxants, especially depolarizing agents like succinylcholine. Succinylcholine can trigger life-threatening hyperkalemia (elevated potassium levels in the blood) and muscle rigidity in DMD patients, which can lead to severe cardiac arrhythmias.
   • Alternative Anesthesia Agents: Non-depolarizing muscle relaxants and other anesthetic agents may be preferred to avoid triggering such complications. The anesthesiologist should be aware of the patient’s muscle and respiratory function and select appropriate drugs accordingly.
3. Cardiovascular Considerations:
   • Heart Conditions: Anesthesia can exacerbate heart problems in DMD patients, particularly those with dilated cardiomyopathy. Anesthetic agents can depress myocardial function, so careful selection of agents that maintain cardiovascular stability is essential.
   • Preoperative Cardiology Consultation: A preoperative cardiology assessment is crucial for understanding the heart function of DMD patients, especially those with known cardiovascular involvement, to guide anesthesia management.
4. Temperature Regulation: Patients with DMD may have difficulty regulating body temperature during surgery, which can be exacerbated by anesthesia. Maintaining normothermia during the procedure is essential to avoid complications such as shivering or hyperthermia.

Multidisciplinary Approach

Due to the complexity of DMD and the potential for surgical and anesthetic complications, a multidisciplinary approach is essential for the successful management of these patients. The care team may include:

• Neurologists familiar with DMD to assess disease progression.
• Anesthesiologists experienced in managing patients with muscular dystrophies, who can ensure safe anesthesia management.
• Cardiologists to monitor heart function and identify any cardiovascular risks.
• Pulmonologists to assess respiratory function and provide necessary respiratory support during surgery and recovery.
• Orthopedic Surgeons for corrective surgeries, including scoliosis correction and tendon release procedures.
• Physical and Occupational Therapists for postoperative rehabilitation to optimize mobility and recovery.

Preoperative Considerations and Optimization

Before surgery, it is important to optimize the patient’s health to minimize risks. Several preoperative measures should be taken into account:

• Respiratory Function Testing: Pulmonary function tests, including spirometry and forced vital capacity (FVC) measurements, help assess the patient’s ability to breathe independently. Non-invasive ventilation support should be used as needed.
• Cardiac Evaluation: An ECG, echocardiogram, and other tests to assess heart function should be performed. In some cases, cardiologists may need to adjust medications prior to surgery.
• Muscle Strength and Mobility Assessment: This helps determine the potential for recovery after surgery and whether any special measures (like post-operative physical therapy) are needed.
• Infection Control: Patients with DMD may be more susceptible to infections, so preventive measures, including the use of prophylactic antibiotics, may be recommended.

Postoperative Care

After surgery, DMD patients require careful monitoring due to their compromised muscle and respiratory function. Key elements of postoperative care include:

• Respiratory Support: Non-invasive or invasive mechanical ventilation may be required during the recovery period. Monitoring of oxygen saturation and blood gas levels is critical.
• Pain Management: Since patients with DMD may have reduced muscle tone and sensitivity to pain, pain management strategies need to be individualized. Non-opioid analgesics are preferred to avoid the potential for respiratory depression.
• Early Mobilization: Physical therapy should be initiated early to prevent joint contractures, muscle atrophy, and improve postoperative recovery.

Succinylcholine Should Not Be Used

Succinylcholine should not be taken by people who have Duchenne disease. One medication that depolarizes muscles is succinylcholine, also known as suxamethonium. It is occasionally used in emergency situations to help sedated individuals who are having trouble breathing. On the other hand, succinylcholine can result in substantial, potentially deadly, elevations in blood potassium when given to patients who have persistent muscular atrophy, regardless of the underlying etiology.

Avoid Inhaled Anesthetic Agents if You Can

Steer clear of inhalation anesthetics. Desflurane, Enflurane, Halothane, Isoflurane, and Sevoflurane are among the frequently used inhaled anesthetics.

People with Duchenne are susceptible to hyperkalemia, which is the release of excessive potassium into the bloodstream and can lead to potentially fatal cardiac arrhythmias, and rhabdomyolysis, which is the breakdown of skeletal muscle tissue that may induce the production of myoglobin that can harm the kidneys.

Even when succinylcholine was avoided, Duchenne patients have been known to experience severe (and occasionally fatal) muscular breakdown (rhabdomyolysis) when exposed to inhalation anesthetic gases. As a result, we advise that individuals with Duchenne should either avoid or utilize inhalational anesthetic gases sparingly. However, there are some situations in which using these inhaled medications, if at all possible, is recommended by the benefit/risk ratio.

Safe Anesthesia Practices

All intravenous (IV) anesthetic agents are considered to be safe to give to people with Duchenne with close monitoring.

• Barbiturates/Intravenous Anesthetics
  Diazepam (valium), Etomidate (Amidate), Ketamine (Ketalar), Methohexital (Brevital), Midazolam (Versed), Propofol (Diprivan), Thiopental (Pentothal)

• Inhaled Non-Volatile General Anesthetic
  Nitrous Oxide (“laughing gas”)

• Local Anesthetics
  Amethocaine, Articaine, Bupivicaine, Etidocaine, Lidocaine (Xylocaine), Levobupivacaine, Mepivicaine (Carbocaine), Procaine (Novocain), Prilocaine (Citanest), Ropivacaine, Benzocaine (caution re: methemoglobinemia risk), Ropivacaine

• Narcotics (opiods)
  Alfentanil (Alfenta), Codeine (Methyl Morphine), Fentanyl (Sublimaze), Hydromorphone (Dilaudid), Meperidine (Demerol), Methadone, Morphine, Naloxone, Oxycodone, Remifentanil, Sufentanil (Sufenta)

• Muscle Relaxants
  Arduan (Pipecuronium), Curare (The active ingredient is d-Tubocurarine), Metocurine, Mivacron (Mivacurium), Neuromax (Doxacurium), Nimbex (Cisatracurium), Norcuron (Vecuronium), Pavulon (Pancuronium), Tracrium (Atracurium), Zemuron (Rocuronium)

• Anticonvulsants
  Gabapentin (Neurontin), Topiramate (Topamax)

• Anxiety Relieving Medications
  Ativan (Lorazepam), Centrax, Dalmane (Flurazepam), Halcion (Triazolam), Klonopin, Librax, Librium (Chlordiazepoxide), Midazolam (Versed), Paxipam (Halazepam), Restoril (Temazepam), Serax (Oxazepam), Tranxene (Clorazepate), Valium (Diazepam)

Read More: PPMD

Ask Your Doctor Anything You Wonder

Regardless of how trivial they may appear, it is crucial to ask the doctors working on your kid’s care any questions you or your child may have when you meet with them. Consider posing the following queries:

• Is surgery the only option?
• What is the short-term and long-term impact of surgery?
• How long will my child be in hospital?
• What are the potential benefits of having the operation?
• What are the potential risks of having the operation?
• What could happen if my child does not have the operation?

We advise you to take a pen and notebook to your appointments because you and your child will want to ask a lot of additional questions. Writing out the questions beforehand can help you remember to cover all you want to and to take a quick note of the responses you receive.

Conclusion

Surgery and anesthesia in Duchenne Muscular Dystrophy patients are complex and require a highly specialized, multidisciplinary approach. The primary concerns in DMD surgery include muscle weakness, cardiovascular issues, and respiratory function. Anesthesia management must account for these factors, with particular attention to the risks associated with muscle relaxants and ventilation. With careful preoperative optimization, close monitoring during the procedure, and comprehensive postoperative care, the risks associated with surgery and anesthesia in DMD patients can be minimized, leading to improved outcomes and quality of life. As medical advancements continue, the surgical and anesthetic management of these patients will likely continue to improve, ensuring safer and more effective treatments.

Disclaimer: If you have specific questions about how this relates to your child, please ask your doctor. Please note this information may not necessarily reflect treatment at other hospitals.

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