[Ehlers-Danlos syndrome with concomitant joint contractures].
We present 6 children with Ehlers-Danlos syndrome (EDS) with concomitant joint contractures. In 3 of them bilateral clubfeet together with hip, and (or) knee, or wrist flexion contractures were observed. In all of them, after initial soft tissue surgery for clubfeet, reccurences were noted requiring another surgery. Clubfeet were the only manifestation of joint contractures in another 2 children. In one of them soft tissue surgery was succesful, in the second a hypercorrection was noted. One child with EDS had a recurvatum knee deformity which was succesfully treated conservatively. We speculate on two different forms of EDS with joint contractures: a mild one, in which clubfeet are the only joint contractures, and in which a soft tissue surgery may lead to overcorrection, and a severe one, with multiple joint contractures like in arthrogryposis multpiplex congenita, in which soft tissue surgery for clubfeet and joint contractures may lead to reccurences.
Lejman
Tadeusz
T
Oddział Ortopedyczno-Urazowy, Katedra Chirurgii Pediattycznej, Wydział Lekarski Uniwersytetu Jagiellońskiego.
Kowalczyk
Bartłomiej
B
D000293:Adolescent; D001176:Arthrogryposis / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D003286:Contracture / Q000209:etiology* / Q000601:surgery*; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000175:diagnosis; D005260:Female; D006801:Humans; D016916:Joint Deformities, Acquired / Q000209:etiology* / Q000601:surgery; D007593:Joint Instability / Q000150:complications; D008297:Male; D012867:Skin / Q000473:pathology; D016896:Treatment Outcome