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Deformity Correction in Children with Hereditary Hypophosphatemic Rickets

机译:遗传性低磷酸盐血症患儿的畸形矫正

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摘要

X-linked hereditary hypophosphatemic rickets can induce various multiplanar deformities of the lower limb. We evaluated our ability to correct these deformities and assessed complications and recurrence rates in 10 children (eight girls and a pair of twin boys) followed from early childhood to skeletal maturity. We performed 37 corrective operations in 10 children. Depending on the patient’s age, external fixation was used in 53 segments: Kirschner wires in 18, DynaFix® in three, the Taylor Spatial Frame® device in 13, and the Ilizarov device in 19. Internal fixation with intramedullary nailing was performed in 12. After bone consolidation, we radiographically determined the mechanical axis at an average distance of 0.5 cm medial to the center of the knee. The average mechanical lateral distal femoral angle was 85° (range, 83°–92°) and the average mechanical medial proximal tibial angle was 91° (range, 85°–92°). Deviation of the mechanical axis and knee orientation lines was increased at the followups conducted during a period of 5 to 12 months. Additional followups revealed a recurrence rate of 90% after the first corrective procedure and 60% after a second procedure. Level of Evidence: Level IV, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.
机译:X连锁遗传性低磷酸盐血症性ets病可诱发下肢的多种多平面畸形。我们评估了纠正这些畸形的能力,并评估了10例儿童(八个女孩和一对双胞胎男孩)从幼儿期到骨骼成熟的并发症和复发率。我们对10名儿童进行了37次矫正手术。根据患者的年龄,在53个部分中使用了外部固定:18个中的Kirschner线,三个中的DynaFix ®,13个中的Taylor Spatial Frame ®设备以及Ilizarov在19中进行了装置固定。在12中进行了带髓内钉的内固定。骨固结后,我们通过射线照相法确定了机械轴,距膝盖中心的平均距离为0.5 cm。平均机械外侧股骨远端角为85°(范围83°–92°),平均机械内侧胫骨近端角为91°(范围85°–92°)。在5到12个月的随访期间,机械轴和膝盖方向线的偏差增加了。进一步的随访显示,第一次矫正手术后复发率为90%,第二次手术后复发率为60%。证据级别:IV级,治疗研究。有关证据水平的完整说明,请参见《作者指南》。

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