Outcome and Future Options: 5-Year-old with Increasing Spinal Deformity

To place these values in perspective, Dimeglio reports normal T1-S1 length to be 35 cm at age 10 (45 cm at maturity) in males. Emans' data suggests this T1-T12 length is about the fiftieth percentile based on pelvic width (Figure 6).

Figure 6

On the other hand, the other thoracic parameters, T6 coronal width and T6-sternum sagittal width, were fifth percentile or less (Figures 7, 8).

Figure 7

Figure 8

Pulmonary function tests performed at this time (the patient was again noted to have difficulty with the performance of the test) showed minimal change in absolute forced vital capacity = 1.07 L. (39% pred.), perhaps representing lack of pulmonary development due to failure of the thorax to grow in the coronal width and sagittal depth components, even though the thoracic length seemed to have been sufficiently lengthened. Obviously, the congenital myopathy also played an important role in the forced vital capacity impairment.

Currently and Future Options
The patient has just recently had rod revision to continue lengthening. Discussion now centers on how much further to attempt to lengthen based on length parameters, which appear to show adequate spinal length. Options in the future include a period of observation until puberty, and if progression occurs, conversion to a final correction and fusion. Due to the underlying myopathy and pulmonary status, avoiding an anterior procedure to control crankshaft would be attractive, and thus management by posterior techniques only is the goal.