Arthrogryposis multiplex congenita - local experience

Background. Arthrogryposis is a heterogeneous group of problems of the musculoskeletal system, clinically characterized by multiple contractures of the joints of at least two anatomical regions, presenting at birth. The aim of our study was to evaluate the results of a therapeutic approach to patients at the University Pediatric Orthopedic Clinic in Bratislava. Material and methods. Nineteen patients (13 girls, 6 boys) were treated and followed up between 1993 and 2004. Results. Six patients had a clear clinical appearance of classical arthrogryposis multiplex congenita, six had distal arthrogryposis, two girls had Larsen's syndrome, and three had other forms of joint involvement. At a mean age of 18.4 months (range, 9-52 months), 77 surgeries had been performed. Most of the surgical procedures were for clubfoot-like deformities and vertical talus (45 operations) and for hip dislocations (20 operations). All patients who underwent surgery for hip dislocations achieved a full range of hip movement after subsequent physiotherapy. Surgery on the other hip also had good functional outcome. At the last examination, all patients were independently ambulatory, but some (mainly the younger ones) needed help in feeding, hygiene or dressing. Conclusions. In our experience with this group of patients, physiotherapy and occupational therapy supported by orthotic equipment is an inseparable part of their lives. Complementary surgery gives an opportunity to improve the correction of deformities in cases where conservative treatment does not meet the needs of the patient, because their normal intellect gives them the potential for an almost normal adult life.