Surgery for muscular dystrophy

Muscular dystrophy can affect the muscles in the arms, legs, face, neck, shoulders, hips, and heart. Depending on the complications an individual experiences, their care team may recommend surgery to address issues such as spinal curvature (scoliosis), joint contractures or stiffness, swallowing difficulties, and heart and breathing issues, to support improved quality of life.

Organizing and navigating surgery for neuromuscular symptoms requires careful coordination between the patient, care team, surgeons, and anesthesiologists. This can help the surgical team identify and manage potential heart and lung health risks, reduce the risk of complications, and support recovery.

This article explains the indications, precautions, and types of surgery associated with muscular dystrophy, as well as how to prepare for surgery and what to expect afterward.

Why might surgery be needed for muscular dystrophy?

Surgery is not routine, but it may be considered to correct progressive complications. A person may need different surgeries depending on the type of muscular dystrophy they have, its severity, and their overall health.

Indications that a person needs surgery for muscular dystrophy include:

• Scoliosis: Some people with muscular dystrophy may require surgery to correct severe or progressive abnormal curvature of the spine.
• Contractures: Muscular dystrophy causes muscles and tendons to shorten and tighten, which affects the positioning and mobility of joints. Contracture surgery for muscular dystrophy can help release affected muscles and tendons to improve movement and joint positioning.
• Cataracts: These are common in people with myotonic dystrophy. Clouding of the eye’s lens can impair vision and require surgical replacement.
• Cardiomyopathy: In Emery-Dreifuss and myotonic muscular dystrophies, weakened heart muscles and electrical system abnormalities can cause dangerous heart rhythms that may eventually require surgical intervention.
• Dysphagia: Duchenne muscular dystrophy can cause chewing and swallowing difficulties that may make it harder for some people to consume enough calories by mouth. In selected cases, a feeding tube may be inserted directly into the stomach via gastrostomy when oral nutrition is no longer adequate or safe. This helps to maintain nutrition and weight.
• Breathing difficulties: During surgery, supplemental breathing support may be necessary due to anesthesia or muscle relaxants used during the procedure; surgeons may place a temporary breathing tube (endotracheal tube) into the main airway. People with severe breathing difficulties that do not respond to noninvasive support, such as BiPAP, may require a tracheostomy. This procedure creates a small surgical opening in the windpipe (trachea) for a breathing tube for long-term respiratory support, which may be temporary or ongoing depending on individual needs. The decision is highly sensitive and involves many factors.
• Diagnosis: In selected cases where the diagnosis remains unclear after clinical and genetic testing, a muscle biopsy can provide further clarity to define which proteins are affected and which type of muscular dystrophy has developed.

How is surgery planned safely?

Some surgeries require general anesthesia, while others may be performed with regional or local anesthesia or sedation, depending on the procedure and the patient’s needs. With general anesthesia, a patient is fully unconscious during the procedure. Anesthesia can carry increased risks in some muscular dystrophies, particularly when heart or breathing complications are present, so planning should be tailored to the specific subtype and overall health of the patient. Choosing a facility with experience treating people with muscular dystrophy can help ensure patients receive appropriate care.

If a patient takes steroids, it is crucial to discuss this with the care team before any surgery. Because surgery stresses the body, the medical team may prescribe a temporary “stress dose” of steroids before and after an operation to help prevent a life-threatening complication called an adrenal crisis.

Heart and lung assessments may be recommended based on the muscular dystrophy subtype, existing complications, procedure, and planned anesthesia. If these show any anomalies, treatment for these issues should take place before other surgeries involving anesthesia move forward.

What types of surgery may be needed?

There are several types of procedures and surgeries that may be needed to manage complications, support mobility, or monitor disease progression in people with muscular dystrophy.

 

Muscle biopsy

A muscle biopsy is a minor surgical procedure in which a surgeon collects a small sample of muscle tissue.

Depending on the location of the muscle biopsy, the age of the individual, their ability to remain still, and their medical needs, general anesthesia is sometimes required. The procedure is brief, and the patient is usually able to leave the hospital on the same day.

Scoliosis corrective surgery

Spinal fusion uses rods affixed to the vertebrae with metal pins, allowing the bones to heal into a single unit. Scoliosis surgery can make future intrathecal procedures more difficult or affect access, depending on surgical anatomy; therefore, future treatment plans should be discussed with the surgical and neuromuscular teams before fusion.

Gastrostomy tube insertion

Gastrostomy delivers nutrition directly into the stomach when oral intake is no longer sufficient or safe. Endoscopic G-tube placement takes about 30 minutes under general anesthesia, guiding the tube through the mouth into the stomach before securing an opening in the abdomen. Most patients can leave the hospital within a day or two.

Some patients may need a slightly more complex procedure, called open gastrostomy. In these cases, a slightly larger incision is made in the wall of the abdomen, and the G-tube is inserted into the stomach. The stomach is then secured to the abdominal wall around the tube to help keep the gastrostomy opening in place.

Tracheostomy

If noninvasive methods (like BiPAP) are no longer enough, a tracheostomy for muscular dystrophy creates a small opening in the neck into the windpipe to attach a ventilator.

Pacemaker surgery

In people with heart issues related to muscular dystrophy, a pacemaker may help to treat certain slow heart rhythms or conduction abnormalities. This can help the heart beat more regularly.

To implant a pacemaker, the surgeon makes a small incision on the left side of the chest, guides the wires through a vein to the heart, places the pacemaker near the collarbone, and closes the cut.

Cataract surgery

The cloudy lens of the eye is surgically replaced with an artificial lens, typically as a brief outpatient procedure.

Tendon surgery and contracture repair

Tendon surgery for muscular dystrophy, known as tenotomy, involves releasing or lengthening shortened tendons (such as the Achilles tendon) to improve foot positioning and joint movement. A cast or splint may be used afterward, and patients will work with a physical therapist before and after surgery to maintain flexibility.

What questions should be asked before surgery?

People with muscular dystrophy who are due to have surgery need to consider both standard and specialized questions ahead of time.

Presurgical questions specific to muscular dystrophy:

• Does the surgical and anesthesia team have specific experience treating individuals with my specific type of muscular dystrophy?
• Which type of anesthesia is planned, and are there any anesthetic agents that should be avoided because of my muscular dystrophy?
• How does the team plan to manage extubation and postoperative breathing support?
• How will postoperative pain be managed to ensure comfort?
• When can physical therapy, weight-bearing, and nutritional intake safely resume after surgery?

General pre-surgical questions:

• Is there another treatment available that treats the same problem?
• What are the benefits of the surgery?
• Will another surgery be required later on?
• What are the possible complications and risks?
• What could happen if the surgery does not take place?
• Is a second opinion recommended?
• Where will the surgery take place?
• What are the costs of this procedure?

Pre-surgical preparation checklist

What to bring when preparing for surgery:

• Medical records: Compile recent neurology notes, respiratory function results when indicated, and recent echocardiogram/ECG reports.
• Emergency anesthesia alert card: Carry a disease-specific anesthesia alert card documenting any anesthetic agents that should be avoided for the specific muscular dystrophy subtype.
• Respiratory equipment: If prescribed, pack personal cough assist machines, BiPAP, masks, and tubing for immediate post-op use in recovery.
• Medication reconciliation: If taking long-term steroids, ask the medical team for individualized instructions about perioperative steroid management.
• Post-discharge equipment: Pre-order adapted seating, transfer equipment, or braces recommended by physical and occupational therapists prior to admission.

What should someone expect after surgery?

The exact expectations after muscular dystrophy surgery will depend on the type of surgery a person has, the type of muscular dystrophy they live with, and their overall state of health. Their neuromuscular care team and the surgeon will set expectations and outline the best steps toward recovery.

Patients with muscular dystrophy often require longer observation in the post-anesthesia care unit or an intensive care unit. This extended monitoring helps the care team ensure that breathing remains stable, oxygenation is adequate, and any postoperative complications are identified promptly. For people who use noninvasive ventilation or have significant respiratory muscle weakness, the respiratory team may plan extubation (endotracheal tube removal) directly to noninvasive ventilation.

Pain management will be necessary after surgery, but opioid medications can significantly affect breathing and bowel movements. Doctors will carefully individualize and monitor opioid treatment and may use local nerve blocks as an alternative when appropriate.

For orthopedic procedures, such as an Achilles tenotomy, bed rest will be limited to reduce the risk of atrophy. Patients should discuss with the surgeon and their care team when they can safely resume normal routines.

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Muscular Dystrophy News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.