Adolescent Neuromuscular Disease
Neuromuscular scoliosis is lateral (side) curvature of the spine that is caused by various neuromuscular conditions. These include cerebral palsy, muscular dystrophy, polio, spinal muscular atrophy, and post spinal cord injury conditions, in addition to other diseases. Patients afflicted with these conditions often develop scoliosis and/or kyphosis or round back. This is frequently associated with back pain, difficulty sitting in a wheelchair, and loss of walking ability over time. Those indivuals who are severely involved may also develop lung dysfunction due to recurrent pneumonia or bronchitis or simply from restriction of the thorax (chest). Some patients have special needs as a result of intellectual impairment, often seen in conditions such as cerebral palsy.
Treatment of patients with neuromuscular scoliosis is tailored to the particular problem (e.g., pain, sitting difficulty) as well as the severity and the location of the curvature. Nonoperative treatment includes bracing for patients who have flexible curves that are under 50 degrees. For those who are nonambulatory, modification of the wheelchair can be done in order to assist in sitting comfort and balance.
Surgical treatment is reserved for those patients who have progressive curvature that interferes with function or is likely to cause difficulties in the years ahead. Patients with muscular dystrophy are treated once curvature reaches 20 degrees or more. This early surgery is done to preserve lung function and ambulatory potential. Surgical treatment is otherwise generally performed for curvature over 50 degrees for most patients. Often, when pelvic asymmetry or obliquity occurs in conjunction with severe curvature, the surgery incorporates the spine and the pelvis in order to correct the pelvic obliquity and spinal imbalance. This can be done through a posterior (back) approach or a combined anterior (front) and posterior approach, depending on the severity of the curvature and the need for additional support in the front of the spine. Success in these patients is measured by the ability to rebalance the spine and to preserve function and comfort and allow meaningful interaction of the patient with his or her environment and family. Although many of these patients are significantly disabled, they can often live fulfilling and productive lives.
Case Example #1
Patient is an 11 year old with an unknown neuromuscular condition related to a genetic condition. Her scoliosis of 72 degrees was corrected with a posterior spinal fusion to 15 degrees with good balance.
Case Example #2
This is a 14 year old male with pervasive developmental delay and a progressive scoliosis of 78 degrees. The patient underwent a thoracoscopic release followed by posterior spinal fusion and his 78 degree curvature was corrected to 20 degrees.
Case Example #3
This is a 19 year old boy with cerebral palsy who developed a 70° scoliosis. This was treated by thoracoscopic spinal fusion and posterior instrumentation with screws and wires - the gold standard at the time. The curvature was corrected to under 15°. His ability to walk improved after surgery.