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Characterization of Hip Morphology in Children With Mucopolysaccharidosis Types I and II
Elizabeth Ashby1, Markus Baker, Deborah M Eastwood
1Department of Orthopaedic Surgery, Great Ormond Street Hospital for Children, London, UK.
Insights
Hip morphology in mucopolysaccharidoses (MPS) I and II shows significant hip migration and femoral head deformity. While acetabular dysplasia varies, some hips remain stable, potentially avoiding surgery.
Area of Science:
- Orthopedics
- Genetics
- Pediatrics
Background:
- Mucopolysaccharidoses (MPS) I and II are rare genetic disorders affecting skeletal development.
- Hip morphology abnormalities are common in patients with MPS.
Purpose of the Study:
- To describe the natural history of hip morphology in children with MPS I and MPS II.
- To analyze changes in hip migration, femoral head sphericity, and acetabular dysplasia over time.
Main Methods:
- Retrospective radiographic analysis of 88 hips in 44 children with MPS I and II.
- Assessment of hip migration, femoral head sphericity, and acetabular dysplasia across different childhood ages.
- Analysis of individual hip morphology changes and rates of progression.
Main Results:
- High prevalence of hip migration and femoral head dysplasia in both MPS I and II.
- Progressive hip migration observed in 75% of hips; progressive femoral head deformity in over 50%.
- Variable acetabular dysplasia, not changing with time; hips were more dysplastic in MPS I than MPS II.
Conclusions:
- Hip morphology in MPS I and II ranges from normal to severely dysplastic.
- Some hips exhibit stability, suggesting surgical intervention may not always be necessary.
- Slow disease progression allows for comprehensive treatment planning.
Background:
The purpose of this study is to describe the natural history of hip morphology in patients with mucopolysaccharidoses (MPS) I and MPS II.
Methodology:
This is a retrospective radiographic analysis of 88 hips in 44 children with MPS I and II. Radiographs were examined to determine hip migration, femoral head sphericity, and acetabular dysplasia at different ages throughout childhood. In individual hips, change in morphology and rate of change were analyzed.
Results:
There was a high rate of hip migration and femoral head dysplasia in both MPS I and MPS II. Progressive migration was seen in three quarters of hips and progressive femoral head deformity in over half of hips. Acetabular dysplasia was variable, ranging from normal to severely dysplastic, but did not change with time. Overall, hips were more dysplastic in MPS I than MPS II.
Conclusions:
Hip morphology is variable in MPS I and MPS II ranging from almost normal to severely dysplastic. Some hips do not deteriorate with time and thus surgical intervention may not be necessary in all cases. Deterioration is slow allowing time to plan a holistic approach to treatment.
Level Of Evidence:
Level IV-case series.
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