多发性先天性关节挛缩。从出生到骨骼成熟的长期随访。

Journal of Children's Orthopaedics Pub Date : 2009-10-01 Epub Date: 2009-08-11 DOI:10.1007/s11832-009-0187-4
Alice Fassier, Philippe Wicart, Jean Dubousset, Raphaël Seringe
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引用次数: 74

摘要

目的:本回顾性长期研究的目的是回顾和介绍11例先天性多发性关节挛缩或肌增生症儿童的治疗效果,从出生到骨骼成熟。方法:评估患者的行走能力、开始行走的年龄、所需的移动设备、独立行走的年龄和肌肉力量。结果:我们的研究显示婴儿有严重的肢体受累,但没有脊柱异常。尽管最初病情严重,9例患者最终成为行走者,髋部屈曲挛缩小于20度,膝盖小于15度,6例患者在2.5岁前独立行走。两名非行走者在骨骼成熟时表现出严重的脊柱侧凸,需要脊柱融合。结论:我们得出结论,骨骼成熟时的长期活动状态与出生时病情的严重程度无关。骨骼成熟时行走的预后将在2.5岁前完成。我们认为对严重关节挛缩的儿童进行早期积极治疗是合理的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Arthrogryposis multiplex congenita. Long-term follow-up from birth until skeletal maturity.

Arthrogryposis multiplex congenita. Long-term follow-up from birth until skeletal maturity.

Arthrogryposis multiplex congenita. Long-term follow-up from birth until skeletal maturity.

Arthrogryposis multiplex congenita. Long-term follow-up from birth until skeletal maturity.

Purpose: The aim of this retrospective long-term study was to review and present the effects of treatment for 11 children with arthrogryposis multiplex congenital, or amyoplasia, followed from birth until skeletal maturity.

Methods: We evaluated walking ability, age of beginning to walk, required ambulatory devices, age of independent walking and muscle strength.

Results: Our series showed babies with severe limb involvements without spine abnormalities. Despite the initial severity of involvement, nine patients finally became ambulators with flexion contracture of less than 20 degrees on hips and 15 degrees on knees, and six were independent walkers before the age of 2.5 years. The two non-ambulators presented severe scoliosis at skeletal maturity, which needed spinal fusion.

Conclusion: We conclude that long-term ambulatory status at skeletal maturity is not correlated with the severity of condition at birth. A prognosis for ambulation at skeletal maturity will be done before 2.5 years of age. We believe that early aggressive management of children with severe arthrogryposis is warranted and justified.

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