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The lower extremity in Morquio syndrome
Arjun A Dhawale1, Mihir M Thacker, Mohan V Belthur
1Department of Orthopaedics, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE 19803, USA.
Surgical intervention can correct lower extremity deformities in children with Morquio syndrome type A (MPS-IVA), including hip subluxation and genu valgum. While shelf acetabuloplasty effectively prevents hip subluxation recurrence, genu valgum correction may require further intervention.
Area of Science:
- Orthopedic surgery
- Pediatric orthopedics
- Rare genetic disorders
Background:
- Morquio syndrome type A (MPS-IVA) causes significant lower extremity deformities in children.
- Surgical outcomes for these deformities are not well-documented.
Purpose of the Study:
- To define lower extremity deformities in MPS-IVA patients.
- To describe the results of surgical interventions for these deformities.
Main Methods:
- Retrospective review of 23 MPS-IVA patients with >2 years follow-up.
- Analysis of surgical vs. non-surgical groups, including hip, knee, and ankle procedures.
- Radiographic measurements to assess deformity correction and recurrence.
Main Results:
- All untreated patients showed progressive hip subluxation, genu valgum, and ankle valgus.
- Surgery improved hip coverage, valgus angles, and lower extremity mechanical axis.
- Recurrence of hip subluxation occurred after some osteotomies, but not after shelf acetabuloplasty. Genu valgum recurrence was common.
Conclusions:
- Lower extremity deformities in MPS-IVA often necessitate surgical correction.
- Shelf acetabuloplasty is effective in preventing hip subluxation recurrence.
- Genu valgum correction recurrence is frequent, requiring further management.
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