How is scoliosis treated in patients with muscular dystrophy?

Updated: Aug 17, 2020
  • Author: Twee T Do, MD; Chief Editor: Jeffrey D Thomson, MD  more...
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Scoliosis is another common problem in patients with muscular dystrophy. It is more common in Duchenne MD than in other forms, with an incidence of 75-90%. [56, 57, 22] The scoliosis develops early and tends to be rapidly progressive, especially when patients become nonambulatory. The curve is usually thoracolumbar or lumbar with associated pelvic obliquity, thoracic kyphosis, and lumbar hyperlordosis. The abnormal sagittal alignment may cause problems with seating systems, even modified systems, and the rapid progression of the scoliosis requires frequent wheelchair adjustments. Braces are not effective in progressive paralytic or neuromuscular curves, and surgery is often indicated. [60]

Advances in pulmonary care and cardiac drugs may negate the absolute need for scoliosis surgery in Duchenne MD, allowing patients to live into adulthood. A 10-year retrospective study showed that 44 of 123 nonambulatory patients aged 17 or older were managed satisfactorily without surgery. [26] Although this is only a single report, it gives much hope for many dystrophic patients with scoliosis.

The technique of choice for scoliosis when the curve measures 20° or more in patients who are nonambulatory is a posterior spinal fusion from T2 to the sacrum. The indication for earlier operative stabilization of the spine in these patients is due to the rapidly deteriorating cardiopulmonary function. [29] The FVC is at its maximum in children younger than 10 years. After this point, the FVC rapidly declines, and anesthetic complications rise dramatically in those patients with an FVC less than 30%.

Spinal fusion is extended to the pelvis, with complete obliteration of the facet joints to ensure arthrodesis. The instrumentation used has often consisted of a Luque rod with segmental sublaminar wires to the L5 level, with bone arthrodesis extending into the sacrum. Currently, however, this method is being employed less frequently; most spine surgeons are now using pedicle screws with extension of the instrumentation to the pelvis. [61, 62]

Occasionally, instrumentation and fusion are extended only to L5 because of the diffuse osteopenia in the sacrum, early surgery and low magnitude curves, or because of the possible complications of instrument failure. The literature has cited a high incidence of failure and revision with fusion short of the sacrum; therefore, spinal fusions in patients with MD are extended to the pelvis, especially if done after the curve magnitude is over 30º. [63, 64, 65]

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