We studied the effects of
spondylodesis on
spinal curvature, functional outcome, level of ambulation and perceived competence in 11 children with
osteogenesis imperfecta (OI). Mean age at surgical intervention was 13.1 years (SD 2.5 years) and follow-up amounted to 3.4 years (SD 2.3 years).
Spinal curvature was measured according to Cobb. The level of ambulation was scored according to the modified criteria of Bleck. Functional abilities and the amount of parental assistance were scored using the Dutch version of the Pediatric Evaluation of Disability Inventory (PEDI). Perceived competence was measured using the Harter Self-Perception Profile for Children. The amount of
fatigue, spinal
pain and presence of subjective
dyspnea were scored with a visual analog scale. The median progression per year before
spondylodesis was 6.1 degrees (interquartile range 2.9 degrees -12.9 degrees ) and after the
spondylodesis it was 5.0 degrees (interquartile range 1.6 degrees -11.0 degrees ). No significant progression or regression in the level of ambulation was found. Perceived competence improved slightly. In the total score of the perceived competence, a borderline significant increase was found ( P-value 0.068). We concluded that
spinal fusion in children with OI does not materially influence functional ability and level of ambulation. Self-perceived competence seemed to improve after surgery. The amount of
pain,
fatigue and subjective
dyspnea seemed to diminish after spinal surgery. Progression of
scoliosis proceeded, as did development of
spinal curvature at the junction of the
spondylodesis. Therefore, oral or intravenous
bisphosphonates before and after spinal surgery should be considered.