Fassier Alice, Wicart Philippe, Dubousset Jean, Seringe Raphaël
Université de Lyon, Faculté Laennec, Hôpital Femme-Mère-Enfant, 59, Boulevard Pinel, 69677, Bron Cedex, France,
J Child Orthop. 2009 Oct;3(5):383-90. doi: 10.1007/s11832-009-0187-4. Epub 2009 Aug 11.
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.
We evaluated walking ability, age of beginning to walk, required ambulatory devices, age of independent walking and muscle strength.
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.
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.
这项回顾性长期研究的目的是回顾并呈现11例先天性多发性关节挛缩症（又称先天性肌发育不全）患儿从出生至骨骼成熟期间的治疗效果。
我们评估了患儿的行走能力、开始行走的年龄、所需的助行器械、独立行走的年龄以及肌肉力量。
我们的研究系列显示，患儿存在严重的肢体受累但无脊柱异常。尽管最初受累情况严重，但9例患者最终能够行走，髋关节屈曲挛缩小于20度，膝关节屈曲挛缩小于15度，且6例在2.5岁之前能够独立行走。另外2例不能行走的患者在骨骼成熟时出现严重脊柱侧弯，需要进行脊柱融合术。
我们得出结论，骨骼成熟时的长期行走状态与出生时病情的严重程度无关。在2.5岁之前可以对骨骼成熟时的行走预后进行评估。我们认为，对患有严重关节挛缩症的儿童进行早期积极治疗是必要且合理的。
