Preparing for Surgery in Duchenne Muscular Dystrophy

Surgery preparation in Duchenne muscular dystrophy is the multidisciplinary planning that happens before any procedure requiring sedation or general anesthesia, from a dental extraction to a scoliosis fusion. It includes pre-operative cardiac and pulmonary assessment, anesthesia review, steroid stress-dose planning, equipment coordination, and family logistics. Done well, it turns a higher-risk procedure into a planned, predictable event. Done poorly, it produces avoidable complications.

This post is a practical overview for families. The actual plan belongs to the surgical, anesthesia, and neuromuscular teams working together.

Why surgery preparation matters more in DMD

Three structural facts shift the calculus:

• Anesthesia risk profile. Succinylcholine is contraindicated, volatile inhalational agents are best avoided, and total intravenous anesthesia is the standard general approach. For background, see anesthesia safety in DMD.
• Reduced respiratory and cardiac reserve. Surgeries that healthy patients tolerate easily can decompensate a patient with low vital capacity or subclinical cardiomyopathy. Pre-operative assessment matters more.
• Adrenal suppression. Patients on long-term corticosteroids need stress dosing around the procedure to prevent adrenal crisis.

Specialist literature emphasises the value of a multidisciplinary perioperative approach in DMD, anticipating complications across cardiology, pulmonology, endocrinology, and anesthesia. (Orthopaedic Management in DMD, perioperative considerations, PMC)

Pre-operative assessment

Most centres with DMD experience run a pre-operative evaluation that includes:

• Cardiology. Echocardiogram and sometimes cardiac MRI to assess left ventricular function and any subclinical cardiomyopathy. For background, see cardiac care in DMD and cardiac MRI in DMD.
• Pulmonology. Pulmonary function tests including forced vital capacity and peak cough flow, plus sleep evaluation when relevant. For background, see respiratory care in DMD and sleep in DMD.
• Endocrinology or neuromuscular team. Confirmation of the steroid stress-dose protocol, including IV access and timing for parenteral dosing if oral medications cannot be taken peri-operatively. For background, see endocrine care in DMD.
• Anesthesiology. Review of the anesthesia plan and any specific concerns, including airway and post-operative ventilation. (Guidelines for Perianesthesia Care of the DMD/BMD Patient, PubMed)
• Surgical team. Procedure-specific planning, including positioning, monitoring, and post-operative care expectations.

A pre-operative meeting that brings these teams together, even briefly, is one of the most useful steps a family can request. Many centres have a perioperative surgical home model that coordinates this formally. (Perioperative surgical home for gastrostomy in DMD, PubMed)

Common surgeries in DMD

Procedures that come up routinely include:

• Dental procedures (extractions, complex restorations).
• Port and PICC placements for infusions.
• Gastrostomy tube placement when nutrition needs change.
• Cardiac catheterisation when indicated.
• Scoliosis spinal fusion. For background, see scoliosis in DMD.
• Orthopedic procedures for contractures.
• Eye procedures (cataracts can develop from long-term steroids).
• General surgery (appendectomy, hernia repair).

Each procedure has its own profile of physiological stress, post-operative recovery time, and respiratory and pain management considerations. The level of planning scales accordingly.

Steroid stress dosing around surgery

The single most missed precaution by general surgical teams is steroid stress dosing. A patient on long-term corticosteroids cannot abruptly stop them, and surgery is a stressor that demands a cortisol response the suppressed adrenals cannot mount. Without supplemental steroids, the result can be adrenal crisis.

A written protocol describing pre-, intra-, and post-operative steroid dosing should be on file in the surgical record and physically with the patient. The PJ Nicholoff Steroid Protocol is a widely used reference for DMD and Becker patients. (PJ Nicholoff Steroid Protocol, PMC)

For background, see emergency preparedness for DMD families and corticosteroids in DMD.

Choosing where to have the procedure

For DMD patients, the experience of the surgical centre with neuromuscular disease matters as much as the technical skill of the surgeon. Practical guidance:

• For elective procedures, choose a centre with experience in neuromuscular anesthesia when possible. Pediatric academic centres usually have this.
• Confirm that anesthesia avoids succinylcholine and volatile agents.
• Verify post-operative respiratory support availability, including non-invasive ventilation and cough assist.
• Confirm that cardiology is reachable peri-operatively.
• Plan transfer of records, especially the steroid protocol and emergency information card.

For background, see the DMD care team explained.

What to bring to the hospital

A practical checklist for any DMD hospital admission:

• Emergency information card and current medication list.
• Steroid stress-dose protocol, in writing.
• Cardiac and respiratory baseline reports.
• The patient’s own cough assist, ventilator, and BiPAP if used at home, with settings documented.
• Spare batteries and charging cables.
• Comfort items, especially for longer stays.
• Contact details for the neuromuscular team and any specialist who knows the patient.

For background, see emergency preparedness for DMD families.

Family logistics

Surgeries are a family event, not just a patient event. A few practical considerations:

• Plan childcare for siblings during the admission.
• Identify which parent will stay overnight; alternate when possible.
• Notify school and employers of expected timing, with margin for unexpected extension.
• Confirm insurance pre-authorization and benefits coverage well in advance.
• Plan transport home, especially if the patient has new equipment or restrictions.

For background, see how DMD reshapes family roles.

Post-operative recovery

Recovery in DMD often takes longer than in healthy patients of the same age. Practical expectations:

• Post-operative respiratory support, including non-invasive ventilation, is often used proactively rather than as a rescue measure.
• Pain management is calibrated to avoid excessive sedation that compromises breathing.
• Early mobilisation within the patient’s capacity is encouraged.
• The cough assist may be used more frequently in the days after surgery.
• Corticosteroid tapering back to baseline follows the stress-dose protocol.

A planned return-to-school or return-to-routine date is usually set at discharge and then adjusted.

For background, see returning to school after a Duchenne diagnosis for the school re-entry framework that applies after hospital admissions as well.

What families can ask

A short list at the pre-operative meeting:

• Has the team planned the anesthetic to avoid succinylcholine and volatile agents?
• Is the steroid stress-dose protocol confirmed and accessible to all teams involved?
• What is the plan for post-operative respiratory support?
• Who is coordinating between cardiology, pulmonology, and the surgical team?
• What is the expected length of stay, and what triggers an extension?
• What signs at home should bring the patient back to the hospital?

Asking turns a complex multi-team event into a structured plan.

What is still uncertain

Optimal pre-operative pulmonary thresholds for some elective procedures, the role of newer cardiac monitoring during surgery, and best models for the perioperative surgical home in DMD continue to evolve.

The reasonable framing is that surgery in DMD is safe when planned by clinicians familiar with the disease, and that the planning is a family-involved process rather than a clinician-only one. The decisions belong to the patient, family, and surgical team.

For related reading, see anesthesia safety in DMD, emergency preparedness for DMD families, the DMD care team explained, and DMD standards of care.

Disclaimer: This post is informational and does not constitute medical advice. Decisions about diagnosis or treatment must be made with a qualified care team.