Arthrogryposis multiplex congenita. Long-term follow-up from birth until skeletal maturity.
retrospective_cohort · Level III
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- Record sourced from PubMed, PMID 19669823.
- Also identified by DOI 10.1007/s11832-009-0187-4 and PMC identifier 2758174.
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Abstract
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.