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.
Abstract

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