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The Bone & Joint Journal
Vol. 98-B, Issue 2 | Pages 229 - 237
1 Feb 2016
Roberts SB Dryden R Tsirikos AI

Aims

Clinical and radiological data were reviewed for all patients with mucopolysaccharidoses (MPS) with thoracolumbar kyphosis managed non-operatively or operatively in our institution.

Methods

In all 16 patients were included (eight female: eight male; 50% male), of whom nine had Hurler, five Morquio and two Hunter syndrome. Six patients were treated non-operatively (mean age at presentation of 6.3 years; 0.4 to 12.9); mean kyphotic progression +1.5o/year; mean follow-up of 3.1 years (1 to 5.1) and ten patients operatively (mean age at presentation of 4.7 years; 0.9 to 14.4); mean kyphotic progression 10.8o/year; mean follow-up of 8.2 years; 4.8 to 11.8) by circumferential arthrodesis with posterior instrumentation in patients with flexible deformities (n = 6).


The Bone & Joint Journal
Vol. 96-B, Issue 8 | Pages 1082 - 1089
1 Aug 2014
Roberts SB Tsirikos AI Subramanian AS

Clinical, radiological, and Scoliosis Research Society-22 questionnaire data were reviewed pre-operatively and two years post-operatively for patients with thoracolumbar/lumbar adolescent idiopathic scoliosis treated by posterior spinal fusion using a unilateral convex segmental pedicle screw technique. A total of 72 patients were included (67 female, 5 male; mean age at surgery 16.7 years (13 to 23)) and divided into groups: group 1 included 53 patients who underwent fusion between the vertebrae at the limit of the curve (proximal and distal end vertebrae); group 2 included 19 patients who underwent extension of the fusion distally beyond the caudal end vertebra.

A mean scoliosis correction of 80% (45% to 100%) was achieved. The mean post-operative lowest instrumented vertebra angle, apical vertebra translation and trunk shift were less than in previous studies. A total of five pre-operative radiological parameters differed significantly between the groups and correlated with the extension of the fusion distally: the size of the thoracolumbar/lumbar curve, the lowest instrumented vertebra angle, apical vertebra translation, the Cobb angle on lumbar convex bending and the size of the compensatory thoracic curve. Regression analysis allowed an equation incorporating these parameters to be developed which had a positive predictive value of 81% in determining whether the lowest instrumented vertebra should be at the caudal end vertebra or one or two levels more distal. There were no differences in the Scoliosis Research Society-22 outcome scores between the two groups (p = 0.17).

In conclusion, thoracolumbar/lumbar curves in patients with adolescent idiopathic scoliosis may be effectively treated by posterior spinal fusion using a unilateral segmental pedicle screw technique. Five radiological parameters correlate with the need for distal extension of the fusion, and an equation incorporating these parameters reliably informs selection of the lowest instrumented vertebra.

Cite this article: Bone Joint J 2014;96-B:1082–9.