Scoliosis

Scoliosis is the abnormal sideways curvature of the spine. The condition affects many patients with muscular dystrophy, a group of genetic disorders characterized by progressive muscle weakness.

In people with muscular dystrophy, scoliosis is caused by weakness in the muscles that normally help to support the spine. In most cases, scoliosis develops after patients have lost the ability to stand and walk.

Scoliosis can cause progressive tilting of the pelvis (pelvic obliquity), which may make sitting difficult or uncomfortable because the weight is not evenly distributed between the buttocks.

As it progresses, scoliosis can alter the shape of the chest cavity, which may affect the mechanics and movement of the chest when a person inhales and exhales. Ultimately, this may cause or contribute to breathing problems that develop in muscular dystrophy patients.

Scoliosis in different types of muscular dystrophy

Scoliosis is more common in some types of muscular dystrophy than in others.

It’s estimated that up to 90% of boys with Duchenne muscular dystrophy will develop scoliosis in the absence of treatment. About one in three people with myotonic dystrophy will develop scoliosis.

Scoliosis also may occur in people with Becker muscular dystrophy, Emery-Dreifuss muscular dystrophy, limb-girdle muscular dystrophycongenital muscular dystrophy, and oculopharyngeal muscular dystrophy.

People with facioscapulohumeral muscular dystrophy can develop scoliosis, though it tends not to be severe in this form of the disease.

Management of scoliosis

Physiotherapy and spinal braces

Scoliosis is generally seen when patients lose their ability to stand and walk. Physiotherapy can be used to ensure that patients’ mobility is maintained for as long as possible.

Some patients who walk well despite moderate scoliosis may benefit from wearing a spinal brace, which is intended to help keep the spine in the correct shape and prevent scoliosis from getting worse. A spinal brace may also be recommended in young children to delay scoliosis until most of the spine’s growth is completed.

Medications and surgery

In some muscular dystrophies, especially DMD, treatment with steroid medications may delay the development of scoliosis because it may prolong walking ability and increase muscle strength. Steroid medications have been shown to improve breathing, heart function, and overall quality of life; however, prolonged use of steroids can cause brittle bones and increase the risk of fractures.

In severe cases, spinal surgery remains the only option to correct scoliosis. Surgery involves connecting the vertebrae, or individual bones in the spinal column, so they do not move. Rods are placed in the spine to hold the vertebrae together until they fuse.

The goals of spinal surgery are to ensure that patients can sit properly, feel less pain, and breathe more easily.

 

Last updated: Jan. 7, 2022, by Marisa Wexler MS

 


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