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Arthrogryposis multiplex congenita. Long-term follow-up from birth until skeletal maturity
Alice Fassier1, Philippe Wicart, Jean Dubousset
1Université de Lyon, Faculté Laennec, Hôpital Femme-Mère-Enfant, 59, Boulevard Pinel, 69677, Bron Cedex, France, alice.fassier@chu-lyon.fr.
Insights
Children with arthrogryposis multiplex congenital (amyoplasia) can achieve independent walking, even with severe initial limb involvement. Early intervention is key for improving long-term ambulatory outcomes in these patients.
Area of Science:
- Pediatric Orthopedics
- Developmental Pediatrics
- Rehabilitation Medicine
Background:
- Arthrogryposis multiplex congenital (amyoplasia) presents with severe limb contractures at birth.
- Long-term functional outcomes, particularly ambulation, require further investigation.
- Understanding predictors of ambulation is crucial for early intervention strategies.
Purpose of the Study:
- To review the long-term effects of treatment in children with arthrogryposis multiplex congenital (amyoplasia).
- To assess factors influencing walking ability and independence from birth to skeletal maturity.
- To establish prognostic indicators for ambulation in severe arthrogryposis cases.
Main Methods:
- Retrospective review of 11 children with arthrogryposis multiplex congenital (amyoplasia).
- Evaluation of walking ability, age of first steps, need for assistive devices, and muscle strength.
- Follow-up from birth until skeletal maturity.
Main Results:
- Nine out of eleven patients achieved ambulation with minimal joint contractures (hips <20°, knees <15°).
- Six patients became independent walkers before 2.5 years of age.
- Two non-ambulators developed severe scoliosis requiring spinal fusion.
Conclusions:
- Long-term ambulatory status is not solely predicted by initial disease severity.
- Prognosis for ambulation can be determined before 2.5 years of age.
- Early, aggressive management is recommended for children with severe arthrogryposis multiplex congenital (amyoplasia).
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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