More than 60% of patients have other associated abnormalities, such as VACTERL association (vertebral defects, imperforate anus, cardiac anomalies, tracheoesophageal fistula, renal anomalies, limb abnormalities such as radial agenesis) or Klippel-Feil syndrome. Renal anomalies occur in 20% of children with congenital scoliosis, with renal agenesis being the most common; 6% of children have a silent, obstructive uropathy suggesting the need for evaluation with ultrasonography. Congenital heart disease occurs in about 12% of patients. Spinal dysraphism (tethered cord, intradural lipoma, syringomyelia, diplomyelia, and diastematomyelia) occurs in approximately 20% of children with congenital scoliosis. These disorders are frequently associated with cutaneous lesions on the back and abnormalities of the legs and feet (e.g., cavus foot, neurologic changes, calf atrophy). MRI is indicated in evaluation of spinal dysraphism.
The risk of spinal deformity progression in congenital scoliosis is variable and depends on the growth potential of the malformed vertebrae. A unilateral unsegmented bar typically progresses, but a block vertebra has little growth potential. About 75% of patients with congenital scoliosis will show some progression that continues until skeletal growth is complete, and about 50% will require some type of treatment. Progression can be expected during periods of rapid growth (before 2 years and after 10 years).
Treatment of congenital scoliosis hinges on early diagnosis and identification of progressive curves. Orthotic treatment is not helpful in congenital scoliosis. Early spinal surgery should be performed once progression has been documented. This can help prevent major deformities. Patients with large curves that cause thoracic insufficiency should undergo surgery immediately.
Progressive spinal deformity is a common and potentially serious problem associated with many neuromuscular disorders, such as cerebral palsy, Duchenne muscular dystrophy, spinal
Figure 202-3 Types of closed vertebral and extravertebral spinal anomalies that result in congenital scoliosis. A, Partial unilateral failure of formation (wedge vertebra). B, Complete unilateral failure of formation (hemivertebra). C, Unilateral failure of segmentation (congenital bar). D, Bilateral failure of segmentation (block vertebra).
muscular atrophy, and spina bifida. Spinal alignment must independent when they can sit in a wheelchair without exterbe part of the routine examination for a patient with neuro-nal support. Progressive curves can impair sitting balance, muscular disease. Once scoliosis begins, progression is usually which affects quality of life. Orthotic treatment is usually inefcontinuous. The magnitude of the deformity depends on the fective in neuromuscular scoliosis. Surgical intervention may severity and pattern of weakness, whether the underlying dis-be necessary with frequent fusion to the pelvis. ease process is progressive, and the amount of remaining musculoskeletal growth. Nonambulatory patients have a higher incidence of spinal deformity than ambulatory patients. Compensatory Scoliosis In nonambulatory patients, the curves tend to be long and Adolescents with a leg-length discrepancy (Chapter 200) may sweeping, produce pelvic obliquity, involve the cervical spine, have a positive screening examination for scoliosis. Before and also produce restrictive lung disease. If the child cannot correction of the pelvic obliquity, the spine curves in the stand, then a supine or seated anteroposterior radiograph of same direction as the obliquity. However, with identification the entire spine, rather than a standing posteroanterior view, and correction of any pelvic obliquity, the curvature should is indicated. resolve, and treatment should be directed at the leg-length dis-