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Scapulothoracic Fusion for Fascoscapulohumeral Muscular Dystrophy

Muscular Dystrophy

A scapulothoracic fusion is used to treat a disorder called Muscular dystrophy. Muscular dystrophy is a group of diseases that cause an ongoing weakness or loss of muscle mass in the body. The third most common type of muscular dystrophy is known as Fascioscapulohumeral Muscular Dystrophy. The symptoms of this include shoulder weakness or shoulder instability, and these symptoms usually start in one’s teenage years or early adult years. This condition is unique in that it can affect the facial nerve. Another symptom of this can also be one’s inability to smile. This disease can be passed down if there is a family history of muscular dystrophy, however it can also become present even if there is no family history of it.

 

Functional deficits occur because of the scapulohumeral involvement, meaning the patient is unable to stabilize the scapula against their chest wall. When these functional deficits become severe, the treatment for this would be a scapulothoracic fusion, which includes fusing the scapula to the thoracic ribs. A diagnosis of this condition is confirmed by a muscle biopsy, an electromyography (EMG), and genetic testing.

After surgery, patients are immobilized with a sling combined with a pillow. This supports the scapula, and prevents the arm from moving around a lot. Patients can move their elbow and wrist as tolerated as long as the shoulder stays mobilized. The sutures are removed at the one week follow up. After 2-3 weeks, the patients are allowed pendulum exercises in addition to elbow and wrist range of motion and grip strengthening. Active forward elevation is not started until six weeks, and at three months, only if necessary, physical therapy is prescribed for patients who still have difficulty regaining range of motion and strength.  At six weeks, x-rays are also taken to ensure fusion of the scapulothoracic junction.