Types of Scoliosis
Congenital Scoliosis
Congenital scoliosis accounts for approximately 20% of all scoliosis and is due to prenatal disruption of vertebral formation (hemivertebra, wedge vertebra) or veterbral segmentation (block vertebra, unilateral bar).
A single hemivertebra is the most common anomaly. A positive family history may be present, with 5% to 10% of siblings having a similar disorder (285).While congenital scoliosis may not be clinically evident until later in life, problems related to defective organogenesis may lead to its early detection. Abnormalities of the trachea, esophagus, renal tract, gastrointestinal tract, lungs, heart, radius, ear, lip, and palate often accompany congenital scoliosis. Up to 25% of children may have renal disorders, 10% may have cardiac problems, and 30% may have spinal dysraphism (282,289). Scoliosis is a primary symptom in VATER syndrome (vertebral defects, anal atresia, tracheoesophageal fistula, radial and renal dysplasia) and thoracic insufficiency syndrome. Immediate surgical referral is required if a congenital spine abnormality is identified.
Unilateral, unsegmented bars that restrict growth on one side of the spine while the other grows normally, especially in the thoracic area, produce curves that are the most likely to progress. If in the cervical area, torticollis may be a presenting symptom (288). Unsegmented block vertebra, as seen in Klippel-Feil syndrome, generally do not produce a progressive scoliosis, but restrict range of motion (284). Avoiding activities that place these patients at risk (diving, contact sports) is important.
Imaging MRI of the brainstem and entire spine provide the best evaluative tool to visualize not only bony abnormalities, but spinal cord dysraphism (diastemato- myelia, lipoma, hydromyelia) that may coexist, yet not be evident on routine radiographs (289). Myelography is rarely used.
Close monitoring every three to six months until age 4 and again in the adolescent years is essential (282).Treatment Typically, orthoses are ineffective, except perhaps in small-degree, long, flexible curves. If an orthosis is used, the family, physiatrist, and therapist must work to encourage the child's acquisition of developmental skills through adaptive activities that accommodate their reduced spinal range. Maintaining cardiovascular health and endurance is especially important prior to surgery.
Approximately 50% of children with congenital scoliosis require surgical intervention at an early age, before spinal rigidity or secondary pulmonary deficiencies occur (284). Surgical options are aimed at prevention of deformity. They include hemivertebra excision, convex growth arrest (hemifusion), fusion with instrumentation and allograft, or instrumentation without fusion (sparing growth). Due to concerns over the loss of spinal height, and the impact that fusion of the thoracic spine may have on long-term pulmonary function, numerous nonfusion technologies have been developed.
Congenital Kyphosis Congenital kyphosis is most common at T10-L1 and due to a failure of vertebral segmentation and/or formation. It may accompany myelomeningoceole or spinal dysraphism, and progressive deformity may lead to paralysis. If the curve is less than 50 degrees, it is most often treated surgically by posterior fusion, but anterior-posterior fusion or kyphectomy may be necessary if the curve is larger (282).