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Thoracolumbar kyphosis in treated mucopolysaccharidosis 1 (Hurler syndrome)
M Naveed Yasin1, Raphael Sacho, Neil J Oxborrow
1From the Royal Manchester Children's Hospital, Manchester, United Kingdom.
Insights
Thoracolumbar kyphosis in children with Hurler syndrome averages 38° and 45% progress. An initial angle over 45° predicts progression, but surgery offers good correction for this rare condition.
Area of Science:
- Pediatric Orthopedics
- Genetics
- Metabolic Disorders
Background:
- Limited literature exists on Hurler syndrome (mucopolysaccharidosis I) and thoracolumbar kyphosis.
- The natural history and intervention indications for this spinal deformity are not well-established.
Purpose of the Study:
- To investigate the severity and natural history of thoracolumbar kyphosis in children with Hurler syndrome.
- To identify risk factors for deformity progression.
- To evaluate the outcomes of surgical interventions for this condition.
Main Methods:
- Retrospective analysis of spinal radiographs from 33 children with Hurler syndrome.
- Mean follow-up of 3.5 years, with some patients receiving bone marrow or enzyme replacement therapy.
- Surgical intervention data for 7 patients with progressive deformity.
Main Results:
- Initial thoracolumbar kyphosis averaged 38° at 17 months.
- 45% of patients experienced progression exceeding 10° over 2 years.
- An initial kyphosis >45° predicted progression (sensitivity 67%, specificity 88%).
Conclusions:
- Thoracolumbar kyphosis in Hurler syndrome is variable, with significant progression in nearly half of cases.
- Initial deformity magnitude is a key predictor of progression.
- Surgical interventions, including anterior fusion and VEPTR, yield good correction.
Study Design:
A retrospective radiographical follow-up study of thoracolumbar deformity in 33 children with mucopolysaccharidosis 1 (Hurler syndrome).
Objective:
To report the severity, natural history, risk factors for progression, and results of intervention for thoracolumbar kyphosis in children with Hurler syndrome.
Summary Of Background Data:
Literature on the subject of thoracolumbar kyphosis in Hurler syndrome and its treatment is limited to small case series. The natural history and thus indications for intervention are unknown.
Methods:
Patients who had been treated with bone marrow transplantation and/or enzyme replacement therapy were followed up with erect radiographs of the spine. Mean follow-up period was 3.5 years (range, 2-12 yr). Radiographs were retrieved and analyzed retrospectively. Seven patients underwent varied forms of surgical intervention for progressive deformity, the technique and principles of which are described.
Results:
The thoracolumbar kyphosis on initial radiographs obtained at a mean age of 17 months measured 38° (95% confidence interval, 34°-42°). Fifteen of the 33 patients (45%) followed for more than 2 years developed a deformity that made a progression of more than 10°. The magnitude of the initial deformity was predictive of whether the deformity progressed (univariate analysis, P < 0.001). An initial kyphosis angle greater than 45° was predictive of progression of more than 10° with sensitivity of 67% and specificity of 88%. All patients who underwent surgical intervention had sustained improvement in the magnitude of thoracolumbar deformity.
Conclusion:
Thoracolumbar kyphosis in Hurler syndrome is of variable severity with an average deformity, in our series, of 38° at a mean age of 17 months. Forty-five percent of patients developed progression of greater than 10°. Patients with an initial deformity greater than 45° seemed to be more likely to progress. Surgical interventions in the form of anterior fusion, combined anterior and posterior surgery and use of the vertical expandable prosthetic titanium rib provided good correction.
Level Of Evidence:
N/A.
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