Diagnosis and treatment of neuromuscular scoliosis

  Neuromuscular scoliosis Scoliosis is one of the common deformities in most neuromuscular diseases, and patients with severe deformities are unable to walk or even have difficulty sitting or standing. Neuromuscular scoliosis is defined as an abnormality of the body’s musculo-neural junction channels, resulting in a deformity of the patient’s spine in the coronal and sagittal positions.  Depending on the etiology, it is divided into neurogenic scoliosis and myelopathic scoliosis. The former can be subdivided into upper and lower motor neuron lesions. Upper motor neuron lesions include cerebral palsy, spinal cord cavernosity, and spinal cord trauma; lower motor neuron lesions include post-polio and spinal muscular atrophy. Myopathic scoliosis includes joint flexion, muscular dystrophy, and other types of myopathies.  Compared to idiopathic scoliosis, scoliosis progresses more rapidly and continues to progress in adulthood. Eventually patients lose the ability to sit and stand, as well as other functional losses, such as lung function. Unlike idiopathic scoliosis, brace therapy does not slow the progression of neuromuscular scoliosis and is not an effective treatment. Progressive scoliosis deformities generally require surgical orthosis and fixation.  Proper diagnosis of the primary disease sometimes requires a muscle biopsy. Evaluation of the patient’s nutritional status and pulmonary function is particularly important.  Indications for surgery: progressive development of scoliosis; decreased ability to sit and stand.  Relative contraindications: preoperative assessment of respiratory, cardiac, and nutritional status should be performed to see if the patient can tolerate surgery.  Imaging: In addition to full-length frontal and lateral radiographs of the spine, the most important is traction spinal radiographs to evaluate scoliosis flexibility.  The goal of treatment: to maintain the spine on the horizontal pelvis and balance it in the coronal and sagittal positions. The surgical fusion segment is longer than other scoliosis segments, usually to the sacral spine. The fusion approach is often anterior or posterior, or a combined anterior and posterior approach. Fusion to the sacrum is achieved using the Luque-Galveston technique or sacral screws.