Muscular dystrophy is a group of diseases that causes progressively worsening weakness and loss of muscle mass in the body. The third most common type of muscular dystrophy is facioscapulohumeral muscular dystrophy (FSHD). It is named for its effects on the face (“facio”), shoulder girdle (“scapulo”), and upper arm (“humeral”), but it can affect the entire body.
The symptoms of FSHD include shoulder weakness, instability, and pain. The torso, abdomen, lower arms, legs, face, and spine can also be affected. These symptoms usually start in the teenage or early adult years and the muscles slowly deteriorate over time. This condition also affects the facial nerve—another symptom of FSHD is the loss of the ability to smile. This disease is genetic and can be passed down if there is a family history of muscular dystrophy, and it can also occur spontaneously, even if there is no family history of it.
“Symptoms of FSHD include shoulder weakness, instability, and pain, and pain and these symptoms usually start in the teenage or early adult years.”
Why treatment is required
People with FSHD have weakness in the muscles that help raise their arms and they may have winging of the scapula (shoulder blades). This is because the muscles that normally stabilize the shoulder blades against the chest wall are weak or atrophied. These muscles include the serratus anterior and the rhomboids. If these symptoms become severe, treatment with a scapulothoracic fusion can help improve movement, strength, and function. This surgery involves fusing (attaching) the scapula to the ribs. This provides a stable base for the healthy deltoid muscles to help lift the arm. This surgery does not cure FSHD, but it can help improve people’s function and quality of life.
How treatment is performed
Scapulothoracic fusion surgery requires a general anaesthetic, meaning patients are completely unconscious for the procedure. During surgery, a bone graft is taken from the iliac crest—the bony ridge on the back of the hip. Then, a cut is made along the edge of the shoulder blade. The muscles that lie on top of and underneath the shoulder blade are pulled back, the scapula is lifted, and the ribcage is exposed. The scapula and ribs are then prepared to receive the bone graft.
A metal plate is placed over the scapula. Holes are drilled into the scapula, and wires are passed through the plate to secure the scapula to the ribs. The bone graft is then sandwiched between the scapula and the ribs to help the two bones fuse together. Recently an alternative to wires has been developed called FiberTape cerclage fixation. A special 2-mm-thick tape is used, and when wrapped twice around the scapula and ribs, it is stronger than metal wire.
The opening is then stitched closed layer by layer and is secured with Steri-Strips so that no staples are needed. A special dressing is applied so that patients don’t have to undergo multiple dressing changes. People with FSHD have the surgery performed on one shoulder at a time, at least six months apart.
Want to watch the surgery? Check out these videos of Dr. Romeo performing a scapulothoracic fusion:
Risks and benefits
Stabilizing the scapula to the chest wall will cause the patient to lose some range of motion of the shoulder, but this compromise allows the arm to have better overall function, including improved strength, reduced pain, and a better cosmetic appearance. People aren’t able to reach quite as high as before, but they are able to lift things overhead. Reported shoulder motion results include:
- Improved arm elevation strength and endurance
- Minimal change in internal rotation
- Decreased external rotation
Patients typically experience improvements in:
- Motion
- Strength
- Function for daily activities
- Independence for personal care and mobility
Risks of the surgery include the following:
- Pneumothorax (collapsed lung)
- Injury to nearby blood vessels
- Wound infection
- Rib stress fracture
- Failure of the ribs and scapula to fuse
Overall, the procedure is very safe and carries a major complication rate of less than 5%.
“Dr. Romeo has a remarkable 100% fusion success rate, which patients normally reach six to eight weeks after surgery.”
Physical therapy protocols
For the first six weeks, the shoulder is immobilized in a sling and pillow brace; only the wrist, elbow, and hand may be moved. After six weeks, patients should meet with their doctor for an assessment to decide whether they can begin shoulder movement. With approval from their doctor, patients can begin physical therapy. Everything must be done very carefully at this point, as the bone graft is not completely fused yet. Rehab normally takes six to twelve weeks and starts with a supervised physical therapy program. Afterward, the patient will follow a home exercise program for six weeks and then move on to a strengthening program.
Scapular thoracic/glenohumeral fusion physical therapy protocol
Pain control
Before surgery, a regional nerve block with local anesthetic (such as bupivacaine) is used to freeze the area being operated on. The nerve block is long-lasting and lasts for approximately 12–18 hours after surgery. Dr. Romeo uses ultrasound guidance for the safe and effective placement of the nerve block.
As the nerve block gradually wears off, oral pain medications (pills or tablets) may be used to manage discomfort. Dr. Romeo often relies on familiar medications such as Tylenol Extra Strength and nonsteroidal anti-inflammatory drugs such as Naprosyn or Mobic (meloxicam). The use of cold therapy or ice at the surgical site helps reduce swelling, pain, and the need for medication.
Dr. Romeo provides each patient with specific instructions to manage any post-op pain. He has managed thousands of surgeries and has detailed pain management plans for all of his patients. He is also committed to managing pain responsibly to minimize the risk of opioid addiction.
Recovery time
Following surgery, patients normally spend three days in hospital, although some patients may leave after two. They are put in an immobilizing sling and pillow brace to prevent gravity from pulling down on the arm and scapula as it recovers.
The arm will stay in the brace for six weeks. During this time patients must not remove the brace, even while sleeping. This is to prevent the body from moving in any way that could be a risk to the fusion. With approval from their doctor, patients can begin physical therapy six weeks after surgery.
Between three to four months after surgery, patients are usually able to perform their everyday activities without difficulty. By six months, most patients can participate in sports, although it may take nine to twelve months to get back to their previous level of activity. It’s important to be cautious and patient during these stages to avoid injury. This surgery essentially creates a new, fused bone with the scapula and ribs, which will need to be gradually strengthened so that it can handle increased use. People should avoid activities that could cause a collision with another person or surface.
At nine to twelve months, patients may resume activities such as yard work, golf, housework, light carpentry, and childcare.
Results
Dr. Romeo has a remarkable 100% fusion success rate, which patients normally reach six to eight weeks after surgery. At this point in their recovery, patients generally don’t require any pain medication.
FAQs
How is FSHD diagnosed?
A diagnosis of FSHD is confirmed by muscle biopsy, electromyography (EMG), and genetic testing. An orthopaedic surgeon with experience in muscular dystrophy and shoulder disorders can help determine whether a scapulothoracic fusion is appropriate for you.