Scoliosis is the abnormal sideways curvature of the spine. The condition affects many patients with muscular dystrophy.

Cause

Scoliosis is caused by weakness in the muscles supporting the spinal column. In most cases, scoliosis develops after patients lose the ability to stand and walk.

The progressive tilting of the pelvis (pelvic obliquity) can cause difficulty in sitting because the weight is not evenly distributed between the buttocks.

Scoliosis in different types of muscular dystrophy

The occurrence of scoliosis varies widely in different types of muscular dystrophy.

It may be present at birth in some cases of congenital muscular dystrophy (CMD). In others, such as Ullrich congenital muscular dystrophy, it generally develops in the first decade of life.

Scoliosis is seen in early teenage years and adolescence in 70-90 percent of Duchenne muscular dystrophy (DMD) patients.

Scoliosis is also observed in other forms of muscular dystrophy such as limb-girdle muscular dystrophy (LGMD), facioscapulohumeral muscular dystrophy (FSHD), and Becker muscular dystrophy.

Scoliosis and breathing difficulties

As it progresses, scoliosis can have a significant impact on the respiratory system because it changes the shape of the chest cavity, which can alter the mechanics and movement of the ribs during respiration. The organs in the thoracic cavity may be displaced and compressed, thereby reducing lung capacity and making breathing less efficient.

Management of scoliosis

There are many ways that scoliosis can be managed in patients with muscular dystrophy. Here are some of the approaches.

Physiotherapy

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.

Spinal braces

Some patients who walk well despite mild scoliosis may benefit from wearing a spinal brace.

A spinal brace may also be recommended in young children to delay scoliosis until most of the growth of the spine is completed.

In cases when scoliosis develops late (generally after age 14) and remains mild, a spinal brace may be the best management strategy. A brace, however, is unlikely to prevent scoliosis from progressing.

Steroid medications

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.

Furthermore, steroid medications have been shown to improve breathing, heart function, and overall quality of life. However, it is known that prolonged use of steroids can cause brittle bones and increase the risk of fractures.

Spinal surgery

In cases where scoliosis severely affects a patient’s quality of life, spinal surgery remains the only option.

Spinal surgery connects 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 or grow together.

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

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