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Bilateral hip replacement in three patients with lysosomal storage disease: Mucopolysaccharidosis type IV and
1Department of Orthopaedic Surgery, Nelson R. Mandela School of Medicine, University of Kwa-Zulu Natal, South Africa. jordanandkaren@telkomsa.net
Insights
Joint replacement in children with lysosomal storage diseases is challenging but feasible. Careful screening for skeletal and cardiac issues, along with scaled prostheses, ensures successful outcomes.
Area of Science:
- Orthopedic Surgery
- Pediatric Orthopedics
- Rare Diseases
Background:
- Lysosomal storage diseases (LSDs) can cause progressive hip degeneration.
- Management of joint replacement in pediatric LSD patients is not well-documented.
Observation:
- Three children with LSDs underwent bilateral total hip replacement in early childhood due to degenerative hip changes.
- Patient stature necessitated the use of appropriately scaled prostheses.
- Associated skeletal and cardiac abnormalities required careful pre-operative screening.
Findings:
- One patient experienced a periprosthetic fracture post-surgery, requiring internal fixation.
- All six hips demonstrated satisfactory clinical and radiological outcomes at mid-term follow-up.
- Successful management involved addressing unique challenges in pediatric LSD patients.
Implications:
- This case series highlights the importance of tailored surgical approaches for joint replacement in pediatric LSD patients.
- Early identification and management of associated comorbidities are crucial for surgical safety and success.
- Further research is needed to establish best practices for orthopedic interventions in LSDs.
Abstract:
The management of joint replacement in lysosomal storage diseases has not been well reported. We present three patients with progressive degenerative changes of the hips who required bilateral total hip replacement in early childhood. The stature of the patients make it essential to have access to appropriately scaled prostheses. Consideration has to be given to associated disorders of the skeleton which must be carefully screened to ensure safety in providing appropriate anaesthesia as well as ensuring that there is no cardiac abnormality. In one patient, a periprosthetic fracture was sustained in one hip in the early post-operative course requiring internal fixation. The patient made a full recovery and all six hips were clinically and radiologically satisfactory at mid-term review.
