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

Alice Fassier,Raphaël Seringe,Philippe Wicart,Jean Dubousset

doi:10.1007/s11832-009-0187-4

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.

Highlights

Arthrogryposis multiplex congenita (AMC) is a non-progressive disorder, present at birth and characterised by multiple joint contractures and defective muscles, with normal sensations [1,2,3,4]
Because there are 150 different disorders or syndromes that have joint contractures as part of their manifestations, AMC is a diagnosis of exclusion, and making a precise diagnosis is very important for the evaluation of results [7]
From 1975 to 1986, 36 children with AMC amyoplasiatype were treated at our institution

Summary

Introduction

Arthrogryposis multiplex congenita (AMC) is a non-progressive disorder, present at birth and characterised by multiple joint contractures and defective muscles, with normal sensations [1,2,3,4]. This condition was first described by Otto in 1841. The most common feature is the lack of foetal movements This can result from a large number of disorders, including neuropathies, abnormalities of the muscles, connective tissue disorders, conditions that limit the internal space of the uterus or defects of the uterine environment [6]. Because there are 150 different disorders or syndromes that have joint contractures as part of their manifestations, AMC is a diagnosis of exclusion, and making a precise diagnosis is very important for the evaluation of results [7]

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Discussion

Conclusion