Arthrogryposis: clinical manifestations and management

• Published in: Archives de pédiatrie : organe officiel de la Société française de pédiatrie 2015-08, Vol.22 (8), p.830-839
• Main Authors: Ayadi, K, Trigui, M, Abid, A, Cheniour, A, Zribi, M, Keskes, H
• Format: Article
• Language: fre
• Subjects: Adolescent; Arthrogryposis - diagnosis; Arthrogryposis - therapy; Child; Child, Preschool; Female; Humans; Infant; Male; Retrospective Studies; Treatment Outcome; Young Adult
• ISSN: 1769-664X
• DOI: 10.1016/j.arcped.2015.05.014

Arthrogryposis is a condition existing at birth, seen in different diseases that have in common the existence of multiple joint stiffness. The clinical presentation is diversified and the functional prognosis depends on the etiology, which makes therapeutic options different from one case to another. The objective of this study was to describe clinical manifestations observed in arthrogrypotic patients and show the different therapeutic methods and functional results. This was a retrospective study over a period of 12 years from 2000 to 2012, based on clinical examinations of 23 patients with arthrogryposis. The mean age of the patients was 6.6 years. The four limbs were involved in 19 cases and only the lower limbs in four cases. The etiology was amyoplasia in nine patients, distal arthrogryposis in three, peripheral neuropathy in two, and muscular atrophy in one infantile patient. Foot deformities were observed in 22 cases, with 25 talipes equinovarus deformities (clubfoot), six convex feet, and five valgus feet. The Ponseti method was applied in 12 clubfeet with recurrence in eight cases. The other clubfeet had functional treatment and necessitated surgical release in 11 cases. Deformation of the knee was seen in 15 patients, flessum in 16 knees, restriction of flexion in eight cases, genu recurvatum in three, stiffness in extension in two, and agenesis of the patella in one case. Surgical treatment was carried out in only one case. Other cases of deformity of the knee were treated by rehabilitation with slight improvement. Deformation of the hip was seen in 15 patients, with 16 hips dislocated and four hips in flessum. The dislocation of the hip was treated orthopaedically in two cases and surgically in six cases, with reduction in all cases but persistence of stiffness. Involvement of the shoulders was observed in eight cases, the elbow in seven cases, the wrist in ten cases, and the fingers in 16 cases. The treatment was based on physical therapy and orthotics in all cases, and improvement was better in the wrist and fingers. Arthrogryposis has different clinical presentations. Successful treatment is not constant. Multidisciplinary care is necessary and should be early and continued in order to gain the maximum autonomy and facilitate patients' social integration.