History

At presentation, patients may have FSHD—a genetic disorder that leads to progressive muscular weakness and muscle loss and primarily affects face, shoulder, and upper arm muscles. This condition affects about 5 per 100,000 people. Genetic studies often demonstrate a deletion in the long arm of chromosome 4. Facial symptoms often accompany this condition and can vary from ptosis to speech problems.⁴ Brachial plexus injuries from birth or recent trauma may be noted. Trauma or other conditions may be elucidated that have lead to muscle injury or peripheral nerve injury.

Physical Examination

Primarily patients have pain combined with loss of forward elevation and abduction. Depending on the cause, various forms of scapular winging may be present. Medial winging of the scapula caused by serratus anterior or long thoracic nerve injury typically occurs with superior migration and medial rotation of the inferior border of the scapula. Lateral winging of the scapula caused by trapezius or spinal accessory nerve injury is associated with inferior migration and lateral rotation of the inferior border of the scapula. More subtle winging resulting from rhomboid or dorsal scapular nerve injury will manifest similarly to lateral winging with inferior migration and lateral rotation of the inferior border. In most patients the deltoid and rotator cuff function is preserved. Range of motion is usually less than 90 degrees of forward elevation and abduction. Atrophy of the shoulder girdle should also be noted. These visual cues will help lead the examiner to determine if any nerve injury is present and which nerves are involved. Another test that is helpful is to stabilize the scapula with one’s hand and determine if the patient’s range of motion improves. This will provide insight to the possible motion to be gained from the procedure by providing a stable platform for function.

Imaging

No imaging modalities will provide significant insight into this particular diagnosis. The pathology is a dynamic phenomenon and is not structural. Plain radiographs may demonstrate abnormal scapular positioning with significant elevation of the superomedial border or lateral translation depending on the underlying pathology. Nerve injury patterns might be identified on magnetic resonance imaging (MRI) but again are not diagnostic. Electromyography can be useful in determining nerve injury and the extent of injury. It is important to identify whether transient neurapraxic changes are present versus a more permanent injury. Neurapraxic injuries resulting from trauma often will spontaneously recover within 1 year. These patients often can be followed and conservative measures used to maintain range of motion and muscle strength in functioning groups.

Indications

Indications for scapulothoracic fusion include pain and persistent loss of function. These basic complaints can be related to nerve injury with persistent electromyographic changes for longer than 1 year with no recovery, FSHD, or other neuromuscular disease. In these patients, extensive physical therapy and other conservative measures have typically failed. Some patients have undergone muscle transfers for scapular winging caused by nerve injury, yet have persistent symptoms. Patients with FSHD are not candidates for muscle transfers owing to the global nature of the disease process, and fusion is indicated. Scapulothoracic arthrodesis is essentially a salvage procedure for these patients.

Contraindications

Axillary nerve injury and poor deltoid function are contraindications for scapulothoracic arthrodesis. The deltoid becomes one of the prime movers for shoulder function and range of motion with fusion and must be working properly. Relative contraindications include poor bone quality, smoking, and significant pulmonary compromise. Fusion does alter rib cage excursion, which can affect respiratory function.

Anesthesia and Positioning

The patient is brought to the operating room and given preoperative antibiotics. After induction of general anesthesia and intubation, the patient is moved to a prone position. Care must be taken to ensure that the patient’s abdomen is free to maximize ventilation and minimize intra-abdominal pressure. Bony prominences should be padded and the arms should be in a position to avoid awkward shoulder positioning and nerve compression. The operative arm is placed in 90 degrees of abduction and external rotation with 30 degrees of horizontal adduction. The upper arm, neck, and spine all the way to the posterior superior iliac spine should be prepped into the surgical field. This allows for some movement of the extremity and harvesting of bone graft during the procedure.

Surgical Landmarks

Surgical landmarks that should be marked before the procedure include the spinous processes of C7 to T4, the associated thoracic ribs, and the superior, medial, and lateral borders of the scapula (Fig. 32-1). Typically with the arm positioned as described earlier, the spine of the scapula overlies the fourth rib and the scapula is rotated approximately 30 degrees in relation to the spinous processes.

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