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Hip morphology in mucopolysaccharidosis type IVA through radiograph, magnetic resonance imaging and arthrogram
Mingyuan Miao1, Zhigang Wang1, Haiqing Cai1
1Department of Orthopedic Surgery, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Insights
Children with mucopolysaccharidosis (MPS) type IVA hip morphology shows abnormal bony acetabulum ossification with cartilage and labrum compensation. Pre-operative MRI and arthrograms are crucial for hip assessment in MPS IVA patients.
Area of Science:
- Orthopedics
- Pediatric Radiology
- Medical Imaging
Background:
- Mucopolysaccharidosis (MPS) type IVA is a rare genetic disorder.
- Hip morphology abnormalities are common in children with MPS IVA.
- Understanding these changes is crucial for effective management.
Purpose of the Study:
- To examine the hip morphology in pediatric patients diagnosed with MPS IVA.
- To evaluate the bony, cartilaginous, and labral coverage of the acetabulum.
Main Methods:
- Retrospective chart review of 42 hips in 21 children with MPS IVA.
- Analysis of pelvic radiographs, MRI scans, and arthrograms.
- Measurements included acetabular index (AI), center edge angle (CEA), and femoral head coverage (FHC).
Main Results:
- MPS IVA hips showed significant cartilage and labrum compensation for abnormal bony acetabulum ossification.
- Cartilage thickness increased longitudinally, while labrum flattened horizontally.
- Inter-class correlation coefficients indicated good agreement between MRI and arthrogram measurements, especially for labral AI.
Conclusions:
- Hip morphology in MPS IVA involves compensatory changes in cartilage and labrum.
- Acetabular index from arthrograms may reflect intraoperative labral coverage.
- Pre-operative MRI and intraoperative arthrograms are essential for comprehensive hip assessment in MPS IVA.
Purpose:
This study examined the hip morphology of paediatric patients with mucopolysaccharidosis (MPS) type IVA (MPS IVA).
Methods:
This was a retrospective chart review of 42 hips in 21 children with MPS IVA. Pelvic radiographs and magnetic resonance imaging (MRI) scans of 42 hips and arthrograms of 13 hips were analysed. The bony, cartilaginous and labral coverage of the acetabulum was determined by acetabular index (AI), centre edge angle (CEA) and femoral head coverage (FHC).
Results:
The mean age at the time of radiography was 66.3 ± 21.7 months. The bony, cartilaginous and labral AI in the MRI assessment were 36.3 ± 5.3, 18.3 ± 4.7 and 12.1 ± 4.6 degrees, respectively. The inter-class correlation coefficients (ICCs) for the bony AI, CEA and FHC measurements on radiographs and MRI were 0.936, 0.879 and 0.810, respectively. In the MRI assessment, labrum in 12 of 42 hips appeared as a regular triangle, and it was flat on 30/42 hips. The average arthrographic AI (AAI) was 11.1 ± 2.7 degrees. The ICCs value of AAI versus cartilaginous and labral AI on MRI indicates good agreement but higher in labral AI.
Conclusion:
Hips in MPS IVA exhibited obvious cartilage and labrum compensation in response to abnormal ossification of bony acetabulum. Cartilage in MPS IVA hip increases the thickness in the longitudinal direction, while the labrum becomes flatten in the horizontal direction. The AAI may represent intraoperative labrum coverage. The femora-acetabular harmony is difficult to determine using radiography only, and pre-operative MRI and an intraoperative arthrogram are very important in a hip assessment in MPS IVA.
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