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Quantitative analysis of lower limb and pelvic deformities in children with X-linked hypophosphatemic rickets

Article dans une revue avec comité de lecture
Author
ccBONNET-LEBRUN, Aurore
1001017 Institut de Biomécanique Humaine Georges Charpak [IBHGC]
ccLINGLART, Agnès
1099208 Centre de référence des maladies rares du métabolisme du calcium et du phosphate
DE TIENDA, Marine
1001017 Institut de Biomécanique Humaine Georges Charpak [IBHGC]
414766 Hôpital Necker - Enfants Malades [AP-HP]
OUCHRIF, Younes
414766 Hôpital Necker - Enfants Malades [AP-HP]
1001017 Institut de Biomécanique Humaine Georges Charpak [IBHGC]
BERKENOU, Jugurtha
ASSI, Ayman
301405 Université Saint-Joseph de Beyrouth [USJ]
WICART, Philippe
414766 Hôpital Necker - Enfants Malades [AP-HP]
1001017 Institut de Biomécanique Humaine Georges Charpak [IBHGC]
ccSKALLI, Wafa
1001017 Institut de Biomécanique Humaine Georges Charpak [IBHGC]

URI
http://hdl.handle.net/10985/23744
DOI
10.1016/j.otsr.2021.103187
Date
2021-12
Journal
Orthopaedics &Traumatology:Surgery &Research

Abstract

Introduction X-linked hypophosphatemia (XLH) rickets mainly causes leg deformities in children that can get worse as they grow. We hypothesized that quantifying the bone parameters will help to document and monitor these deformities in children with XLH. Methods Thirty-five growing children affected by XLH were included in this cross-sectional study. Biplanar radiographs were taken with an EOS system allowing 3D reconstructions of the pelvis and legs. Sixteen geometric parameters were calculated for the legs and pelvis. A control group of 40 age-matched patients was used to define the reference values for these geometric parameters. Results For the legs, significant differences (p < 0.05) appeared between the XLH patients and the control group in the neck-shaft angle, femur/tibia length ratio and HKS. Among the 70 legs in the XLH group, 23 were in genu varum, 25 were in genu valgum and 22 were straight. There were significant differences between the genu varum and genu valgum subgroups in the femoral mechanical angle and the HKS. A strong correlation was found between the femoral mechanical angle and tibiofemoral angle (r² = 0.73) and between the femoral mechanical angle and HKS (r²=0.69) The sacral slope and acetabular anteversion were significant different from the reference values. Discussion Quantitative radiological parameters derived from 3D reconstructions show that the deformities in XLH patients are 1) mainly in but not limited to the femoral shaft; 2) highly variable from one person to another. Some of these radiological parameters may be useful for the diagnosis and monitoring of XLH patients.

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