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Hip morphology in mucopolysaccharidosis type IVA through radiograph, magnetic resonance imaging and arthrogram assessment

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Abstract

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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Funding

Approval of financial support was given by the Medium and Long-Term Clinical Research Foundation of Shanghai Children’s Medical Center (Grant No. ZCQ-SCMC2018–1), the Clinical Research Cultivation Foundation of the Shanghai Shenkang Hospital Development Center (Grant No. SHDC12018X31), the Natural Science Foundation of Shanghai (Grant No. 17ZR1417900) and the National Natural Science Foundation of China (Grant No. 81801919).

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Author notes

  1. Mingyuan Miao and Zhigang Wang contributed equally to this study.

Authors and Affiliations

  1. Department of Orthopedic Surgery, Shanghai Children’s Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China

    Mingyuan Miao, Zhigang Wang, Haiqing Cai, Jingxia Bian & Haoqi Cai

  2. Department of Radiology, Shanghai Children’s Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China

    Liwei Hu

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  1. Mingyuan Miao
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  2. Zhigang Wang
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Corresponding author

Correspondence to Haoqi Cai.

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Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

This study was approved by the Ethical Committee of the Shanghai Children’s Medical Center, Shanghai Jiao Tong University School of Medicine.

Informed consent

Informed consent was obtained from legal guardian of each participant in the study.

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Zhigang Wang is the co-first author.

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Miao, M., Wang, Z., Cai, H. et al. Hip morphology in mucopolysaccharidosis type IVA through radiograph, magnetic resonance imaging and arthrogram assessment. International Orthopaedics (SICOT) 44, 1677–1683 (2020). https://doi.org/10.1007/s00264-020-04600-2

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  • DOI: https://doi.org/10.1007/s00264-020-04600-2

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