[Arthrogryposis Multiplex Congenita - Part II.].
It is a follow-up to Part I of the communication where the authors report on the present possibilities of the diagnostics and therapy of arthrogryposis multiplex congenita. In evaluating the patients with congenital contractures they proceed from the classification of Hall et al. of 1982 and 1983. A 24-member group is made up of patients with classic and distal arthrogryposis. The diagnostics is based on the clinical-genetical examination including dermatoglyphical and electromyographical examination. Electromyographical examination aims at the determination of the prognosis and impairment of mobility. EMG examination has proved neurogenic lesion in 17 children and myogenic lesion which was combined with neurogenic lesion in 3 children. After repeating EMG examination the authors found out in 5 children out of 6 the progression of neurogenic lesion. Dermatoglyphical examination performed in 13 patients (with taking sole prints in four of them) showed considerable changes which are significant for arthrogryposis. In evaluating the clinical findings the verticality of the course of papillary lines correlates with the degree of the clinical impairment as well as the increase of atdangles. The surgical treatment of contractures of lower extremities is indicated on the basis of the authors' own experience as early as in the first year of life. The result of the therapy is to a decisive extent influenced - apart from the severity of congenital contractures - by the timely and longterm comprehensive treatment carried out by an orthopaedic surgeon and rehabilitation specialist closely cooperating together and in case of occurrence of other associated congenital defects also by other specialists. Adequate involvement of parents is a necessary condition for a successful treatment. Key words: arthrogryposis multiplex congenita, amyoplasia, distal arthrogryposis, electromyography, dermatoglyphes, therapy.
Marík
I
I
Ortopedická klinika 2. lékarské fakulty KU, Praha.
Kuklík
M
M
Kraus
J
J
Schejbalová
A
A
Kubát
R
R