Surgery for scoliosis for SMA

Overview
About 90 percent of children with spinal muscular atrophy (SMA) develop scoliosis, an abnormal curvature of the spine. Scoliosis can interfere with movement, make breathing more difficult, and worsen posture. In general, surgery for scoliosis has been found in studies to preserve breathing function and improve comfort, appearance, and quality of life. There are several different types of surgery available for treating scoliosis.

What does it involve?
One surgical option involves the placement of growing rods such as the MCGR system, which involves magnetic rods that can be adjusted remotely in a doctor’s office. Growing rods typically attach to the spine in two places, above and below the curve, to straighten the abnormality and can be adjusted as the child grows. Surgery is usually performed around age 10 or older, after most development is finished.

Another treatment option is the vertical expandable prosthetic titanium rib (VEPTR). VEPTR is a curved metal rod that is implanted. VEPTR attaches to a child’s ribs, spine, or pelvis in a vertical position using hooks. Similarly to growing rods, VEPTR can be adjusted as the child grows to ensure proper fit and stabilization. VEPTR may be considered for younger children who have severe scoliosis.

Growing rods and VEPTR implantations require surgery and can be inserted through incisions through the back.

These surgeries stabilize the spine and prevent worsening scoliosis. They also help elongate the trunk, improve posture, and allow for more room for the lungs to make breathing easier.

Surgical spinal fusion, another common option, may interfere with trunk growth or worsen breathing difficulties in people with SMA. Thus, surgical implantation of rods or other devices to straighten the curvature of the spine have been shown to be more effective among those with SMA.

Intended outcome
Surgical treatments for scoliosis generally straighten spinal curvature and open the chest cavity to allow proper growth and development of the lungs. These treatments allow for modifications in size as the child grows to allow ongoing support through childhood and adulthood.

Results
For patients with SMA, growing rods and VEPTR increase the space-lung ratio in order to mitigate pulmonary problems. These treatments also increase spinal support, curb the progression of pelvic misalignment, and straighten the spine.

Constraints
Any surgery carries risks including blood clots, blood loss, infection, breathing problems, reactions to medication, and heart attack or stroke during the surgery. Risks associated with surgery of the chest include pain, bleeding, infection, heart or lung problems, and pneumonia.

Spinal surgery presents risks of damage to the lungs, heart, or major blood vessels as well as damage to the spinal cord which could result in temporary or permanent paralysis. There is also risk of skin infection and risk of device rejection.

Other complications can result due to rod breakage or hook dislodgment. The child will need to return regularly (roughly every six months) to lengthen the device to ensure proper fit and allow for growth. It is important to follow up with your doctor after surgery to ensure complications are kept at a minimal level.

For more details, visit:
1. Scoliosis in SMA ーSMA Foundation
2. Scoliosis Surgery Techniques ー Scoliosis Research Society
3. Growing Rod Surgery ー Children’s Hospital of Philadelphia
4. Vertical Expandable Prosthetic Titanium Rib (VEPTR) ーSYNTHES Spine
5. Rods Surgery Can Benefit Scoliosis Patients with SMA ーJohns Hopkins