Hip morphology in mucopolysaccharidosis type IVA through radiograph, magnetic resonance imaging and arthrogram assessmen
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ORIGINAL PAPER
Hip morphology in mucopolysaccharidosis type IVA through radiograph, magnetic resonance imaging and arthrogram assessment Mingyuan Miao 1 & Zhigang Wang 1 & Haiqing Cai 1 & Liwei Hu 2 & Jingxia Bian 1 & Haoqi Cai 1 Received: 26 February 2020 / Accepted: 27 April 2020 # SICOT aisbl 2020
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. Keywords Mucopolysaccharidosis (MPS) . Hip dysplasia . Magnetic resonance imaging (MRI) . Arthrogram . Cartilage . Labrum
Introduction Mucopolysaccharidoses (MPSs) are rare disorders with abnormal cellular glycosaminoglycan (GAG) accumulation caused by deficiency of lysosomal enzymes. MPSs are classified into several types according to the dysfunctional enzyme responsible for
Mingyuan Miao and Zhigang Wang contributed equally to this study. Zhigang Wang is the co-first author. * Haoqi Cai [email protected] 1
Department of Orthopedic Surgery, Shanghai Children’s Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
2
Department of Radiology, Shanghai Children’s Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
the disorder. MPS IVA, also called Morquio A syndrome, is an autosomal-recessive inherited deficiency of lysosomal a-Liduronidase. This deficiency leads to an accumulation of GAGs, mainly chondroitin-6-sulphate and keratan sulphate, which results in a progressive multi-organ disease, including abnormal musculoskeletal manifestation
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