Advertisement for orthosearch.org.uk
You currently have no access to view or download this content. Please log in with your institutional or personal account if you should have access to through either of these
The Bone & Joint Journal Logo

Receive monthly Table of Contents alerts from The Bone & Joint Journal

Comprehensive article alerts can be set up and managed through your account settings

View my account settings

Spine

Thoracolumbar kyphosis in patients with mucopolysaccharidoses

clinical outcomes and predictive radiographic factors for progression of deformity



Download PDF

Abstract

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).

Results

In the surgical group (mean age at surgery of 6.6 years; 2.4 to 16.8); mean post-operative follow-up of 6.3 years (3.5 to 10.3), mean pre-operative thoracolumbar kyphosis of 74.3o (42o to 110o) was corrected to mean of 28.6o (0o to 65o) post-operatively, relating to a mean deformity correction of 66.9% (31% to 100%). Surgical complications included a deep wound infection treated by early debridement, apical non-union treated by posterior re-grafting, and stable adjacent segment spondylolisthesis managed non-operatively. Thoracolumbar kyphosis > +38o at initial presentation was identified as predicting progressively severe deformity with 90% sensitivity and 83% specificity.

Discussion

This study demonstrates that severe thoracolumbar kyphosis in patients with MPS can be effectively treated by circumferential arthrodesis. Severity of kyphosis at initial presentation may predict progression of thoracolumbar deformity. Patients with MPS may be particularly susceptible to post-operative complications due to the underlying connective tissue disorder and inherent immunological compromise.

Take home message: Clinical and radiological data were reviewed for all patients with mucopolysaccharidoses with thoracolumbar kyphosis managed non-operatively or operatively in our institution.

Cite this article: Bone Joint J 2016;98-B:229–37.


Correspondence should be sent to Mr A. I. Tsirikos; e-mail:

For access options please click here