Abstract
Study Design: Retrospective chart review.
Objective: To assess the clinical and radiological outcome of surgery for both dystrophic and non-dystrophic curves resulting from neurofibromatosis Type I.:
Subjects: 10 patients (7 females, 3 males) underwent surgical correction for neurofibromatous kypho-scoliosis between 1997–2003. The mean age at surgery was 16 years (range 8–37 years). Average follow-up 20 months (range 9 months – 4.5 years). Seven patients had MRI proven dystrophic curves (group I). These underwent 2–3 level apical vertebrectomy, followed by 2–3 weeks in Halo traction, followed by instrumented posterior spinal fusion and anterior rib strut grafting. Three patients had non-dystrophic curves (group II). Two underwent posterior instrumented fusion and one (aged 8 years) underwent convex epiphyseodesis with posterior Luque trolley.
Outcome Measures: Cobb angle, thoracic kyphosis, lumbar lordosis, global apical vertebral translation (AVT), regional AVT, coronal and sagittal balance, complications and Modified SRS Outcomes Instrument completed at final follow.
Results: For the dystrophic curves the Cobb angle improved from a mean of 81.5 degrees (mean bending film to 76 degrees) to 26.6 degrees post-operatively (68% correction) and 35.8 degrees at final follow-up (56% correction) and the global AVT improved from 61.5 mm to 29 mm at final follow-up. The average score for the modified SRS outcome instrument was 91.6 (Good). For the non-dystrophic curves the Cobb angle improved from a mean of 57.5 degrees (mean bending film to 47 degrees) to 23.5 degrees post-operatively ( 60% correction) and 24.6 degrees at final follow-up (57% correction) and the global AVT improved from 56.8 mm to 27.8 mm at final follow-up. The average score for the modified SRS outcome instrument was 98.5 (Good). All complications occurred in the dystrophic group including superficial infection in 2, dural leaks in 3, temporary brachial plexus injury in 1, worsening of lower limb neurological deficit in 1 and one death (upper GI haemorrhage). There was no failure of metalwork or evidence of pseudarthrosis identified. Seven of eight patients stated that they would have the surgery done again.
Conclusions: Non-dystrophic curves maybe treated by posterior fusion alone achieving 60% Cobb correction and 55% AVT correction. Close observation should be maintained for the appearance of dystrophic features and deterioration of correction. Dystrophic curves should be treated early and aggressively by two/three stage apical vertebrectomy, grafting and posterior spinal fusion. In this series 68% coronal Cobb and 63% AVT correction was achieved post-operatively. Complications can be expected with scoliosis associated with more than 50 degrees of kyphosis.
These abstracts were prepared by Mr. Brian J C Freeman FRCS (Tr & Orth). Correspondence should be addressed to him at The Centre for Spinal Studies and Surgery, University Hospital, Queens Medical Centre, Nottingham NG7 2UH.
