Total Hip Arthroplasty in Mucopolysaccharidosis Type IH
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Published on: August 4, 2022
S O'heireamhoin1, T Bayer, K J Mulhall
1Department of Orthopaedic Surgery, Mater Misericordiae University Hospital, Eccles Street, Dublin 7, Ireland ; Sports Surgery Clinic, Santry Demesne, Suite 4, Dublin 9, Ireland.
Insights
Mucopolysaccharidosis type IH (Hurler Syndrome) causes musculoskeletal issues. Uncemented total hip arthroplasty provided a satisfactory outcome for a patient with Hurler Syndrome, addressing hip dysplasia and avascular necrosis.
Area of Science:
- Orthopedics
- Genetics
- Metabolic Disorders
Background:
- Mucopolysaccharidosis type IH (Hurler Syndrome) is a rare autosomal recessive metabolic disorder.
- Patients experience significant musculoskeletal complications, including hip dysplasia and avascular necrosis.
- While enzyme therapy improves life expectancy, it does not prevent skeletal manifestations.
Observation:
- A 23-year-old female patient diagnosed with Hurler Syndrome presented with hip issues.
- The patient had a history of musculoskeletal manifestations typical of Hurler Syndrome.
Findings:
- The patient underwent uncemented total hip arthroplasty.
- The surgical intervention resulted in a satisfactory clinical outcome.
Implications:
- Total hip arthroplasty is a viable surgical option for managing hip complications in adults with Hurler Syndrome.
- This case highlights the potential for surgical management to improve quality of life in patients with MPS IH.
- Further research into orthopedic interventions for MPS patients is warranted.
Abstract:
Children affected by mucopolysaccharidosis (MPS) type IH (Hurler Syndrome), an autosomal recessive metabolic disorder, are known to experience a range of musculoskeletal manifestations including spinal abnormalities, hand abnormalities, generalised joint stiffness, genu valgum, and hip dysplasia and avascular necrosis. Enzyme therapy, in the form of bone marrow transplantation, significantly increases life expectancy but does not prevent the development of the associated musculoskeletal disorders. We present the case of a 23-year-old woman with a diagnosis of Hurler syndrome with a satisfactory result following uncemented total hip arthroplasty.