Spinal Muscular Atrophy - correction of a 127° curve to a 52° in a 7-year-old

Description

This patient has spinal muscular atrophy (SMA), and she first came to Dr. Bridwell when she was 7 years old. Because of SMA, her spinal muscles had weakened and were no longer supporting her spine very well. She had a very severe curve: 127º. Her rib cage was inside her pelvis; that’s how much her spine was curving. She also couldn’t breathe very well, and sitting was difficult for her, unless she was supported.

Treatment

Dr. Bridwell approached this patient’s treatment in two stages. First, he used halo wheelchair traction for 1-2 weeks. Dr. Bridwell used this treatment option because the patient’s curve was so severe, he wanted to correct some of the deformity and improve the patient's pulmonary functions before performing the spinal fusion.

The second, or next, stage was the formal surgery, which was a posterior spinal fusion. The patient’s spine was fused from T1 (the top of the mid-back region, just below the neck) to the sacrum and pelvis (the end of the spine). In this case, sublaminar wires were used at all levels and iliac screws were used into the pelvis on both sides. No screws were used. During the operation, halo-femoral traction was utilized. In addition to applying traction through the halo on the patient's skull, traction was also applied through the femur/thigh bone on one side to facilitate additional correction. Intraoperative halo-femoral traction is a very safe and effective method of obtaining additional correction in patients with severe spinal deformities, particularly patients with neuromuscular scoliosis and marked pelvic obliquity.

Because of the severity of the patient's deformity, her frail nature and very osteopenic bones, her spinal correction was protected by part-time brace wear for several months. The patient is currently 4 years postop and has maintained her correction. Her 127° curve was corrected to 52°.

Result

The patient’s spinal curve went from 127º to 52º—a dramatic improvement. She can breathe much more easily as well.

Currently, Dr. Bridwell and his team are in the process of publishing a paper on using a long fusion for SMA patients. They have noticed that, by and large, SMA patients do well with long fusions before the age of 10, if their curve is greater than 100º.