Congenital and Neuromuscular Scoliosis - An In Depth Review of Scoliosis

Congenital Scoliosis
Congenital scoliosis is caused by abnormal vertebral formation. Vertebral absence, partial formation or lack of separation can cause asymmetrical growth and resultant deformity (Fig.9). Patients with congenital scoliosis require a renal ultrasound to rule out renal anomalies such as a single kidney which is the most common associated finding. Magnetic resonance imaging may be necessary to rule out suspected associated abnormalities of the spinal cord or spinal nerves if clinical neurologic examination findings are present. The treatment of congenital scoliosis is individualized and dependent upon the type of vertebral malformation. Early surgical intervention may be required to prevent deformity progression.

Figure 9a. Congenital Scoliosis (AP radiograph).

Figure 9b. Congenital Scoliosis (lateral radiograph).

Figure 9c. Congenital Scoliosis (CT scan of hemivertebra).

Neuromuscular Scoliosis
Spinal deformity is common and often severe in patients with neuromuscular disease especially in those patients who do not walk because of their underlying neurological disease. Seating modification and bracing in the vast majority of cases have no long-term effect on the natural, i.e., untreated course, of spinal deformity in neuromuscular disease.

Such techniques may improve sitting ability but do not alter curve progression. Surgical correction and stabilization are done to prevent curve progression. With modern spinal instrumentation and surgical techniques, most patients do not require post-operative immobilization with braces or casts. In patients with muscular dystrophy, the curve often increases when walking ability diminishes. Surgical intervention is indicated for progressive spine deformity in patients with muscular dystrophy while pulmonary function is still adequate, i.e., preferably when the curves are less than 30° (Fig.10).

Figure 10ab. Spinal deformity in Muscular dystrophy (Pre-op).

Figure 10cd. Spinal deformity in Muscular dystrophy (Post-op).

Myelodysplasia (spina bifida) often produces major progressive deformities from both paralytic and congenital factors, particularly in patients with high levels of paralysis. Progressive spinal deformity in patients with cerebral palsy often leads to difficulty with seating and care, especially in patients who do not walk (Fig. 11).

Figure 11a. Spinal deformity in cerebral palsy (Pre-op).	Figure 11b. Spinal deformity in cerebral palsy (Pre-op).
Figure 11c. Spinal deformity in cerebral palsy (Post-op).	Figure 11d. Spinal deformity in cerebral palsy (Post-op).