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Related Experiment Videos

Mobility in Hurler syndrome.

Colm Taylor1, Philip Brady, Ann O'Meara

  • 1Department of Orthopaedic Surgery, Our Lady's Hospital for Sick Children, Crumlin, Dublin 12, Ireland.

Journal of Pediatric Orthopedics
|April 5, 2008
PubMed
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Hematopoietic stem cell transplant improves survival for Hurler syndrome patients. Long-term musculoskeletal issues like hip dysplasia and genu valgum persist, requiring ongoing orthopedic management.

Area of Science:

  • Medical Genetics
  • Pediatric Orthopedics
  • Metabolic Disorders

Background:

  • Hurler syndrome is a rare autosomal recessive metabolic disorder.
  • Hematopoietic stem cell transplant (HSCT) improves survival but long-term musculoskeletal outcomes are not well-defined.
  • Musculoskeletal abnormalities are common in Hurler syndrome, impacting quality of life.

Purpose of the Study:

  • To evaluate the long-term musculoskeletal abnormalities in patients with Hurler syndrome after HSCT.
  • To assess the development and management of hip dysplasia and genu valgum post-HSCT.
  • To determine the effectiveness of orthopedic interventions in this cohort.

Main Methods:

  • Retrospective review of 23 patients with Hurler syndrome post-HSCT (mean follow-up 8.5 years).

Related Experiment Videos

  • Clinical examinations at an annual multidisciplinary clinic.
  • Serial radiological studies (hip dysplasia, genu valgum) and surgical intervention data were analyzed.

    • Main Results:

    • All patients exhibited acetabular dysplasia and femoral head ossification failure.
    • Hip surgeries (derotation, osteotomy) were performed in 12 patients.
    • Genu valgum was common, with 6 patients undergoing medial epiphyseal stapling, reducing tibiofemoral angle by 8 degrees.
    • Independent mobility was achieved, but restricted internal hip rotation was a significant clinical finding.

    Conclusions:

    • HSCT improves survival in Hurler syndrome, but significant musculoskeletal abnormalities persist.
    • Orthopedic interventions can manage hip dysplasia and genu valgum, improving function.
    • Long-term monitoring is crucial to understand disease progression and intervention efficacy.