Pathomechanics and Management of Scoliosis in Friedreich Ataxia Patients: Preliminary Report

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Abstract
Standardized spinal radiographs of 21 patients were taken periodically with the Scoliosis Chariot and the Throne. The short period of observation and the relatively small number of Friedreich ataxia patients followed requires that these results be interpreted with caution. Between the age groups I (5-10 yr) and II (10-15 yr), a substantial increase in the Cobb values occurs. An increase was observed in the thoracic and thoracolumbar projected surface area indices. The relative rotation between the thoracic and lumbar segments was presumed to be the cause of the sudden increase in the Cobb measurements. For the non-ambulatory patients, a decrease in the lumbar lordosis towards a thoraco-lumbar kyphosis and a sudden increase in the sacral angle and a drop in the lumbo-sacral angle were associated with the seated posture assumed by the patient. Prevention of the progression of established curves was studied. Careful examination of the spine, depending on the age of the child stimulated early orthopedic treatment in any curve of 20.degree. or more. There was always concern for curves of 30.degree. or more. In the growing child, bracing was recommended. In the older child, the curve was usually stable after 16 yr of age. Surgery was usually attempted in curves over 40.degree. in the growing child. The same curve was usually stable after the growth period. For the non-ambulatory patients the prescription of a molded seat with the following characteristics was suggested: a posterior lumbar support, low thoracic lateral supports and a slight inclination of the seating system. This was apparently beneficial in maintaining stability of the spine. An evaluation of such a device is under investigation.

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