title: [Duchenne muscular dystrophy--contracture preventive operations of the lower extremities with special reference to anesthesiologic aspects].
abstract: So far there is no causal treatment for Duchenne muscular dystrophy up to now, it has been proven, however, that its course can be considerably improved by an early contracture-prophylactic operation of both lower limbs--mostly between age 4 and 6 years--as well as by a surgical stabilization of the spine before any progressive scoliosis appears, that is at the very beginning of the wheel-chair stage: Walking and standing ability can be prolonged for several years and a significant scoliosis can be avoided. A decisive prolongation of life can be achieved by treating the fatal respiratory insufficiency with timely started mechanical ventilation. Our first experiences have shown, that there is no proven justification for a reserved attitude against early lower limb surgery in view of modern anaesthesia. Malignant hyperthermia-reaction and hyperkalaemia are the known anaesthetic rise factors of operations in Duchenne muscular dystrophy. However, we could exclude virtually these rises by choosing the right anaesthesia and by a comprehensive monitoring routine. If need be, malignant hyperthermia can be treated effectively by using the obligatory antidote (Dantrolene). In view of an expected causal treatment in future, early detection of Duchenne muscular dystrophy by newborn screening ("CK-Test") as well as a comprehensive stage-depending treatment programme (early surgery/mechanical ventilation) are nowadays of outstanding importance.
authors.affiliation: Orthopädische Klinik, RTWH Aachen.
authors.forename: R
authors.initials: R
authors.lastname: Forst
authors.forename: A
authors.initials: A
authors.lastname: Krönchen-Kaufmann
authors.forename: J
authors.initials: J
authors.lastname: Forst
mesh_terms: D000768:Anesthesia, General; D000843:Ankle Joint / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D003286:Contracture / Q000601:surgery*; D006801:Humans; D007719:Knee Joint / Q000601:surgery*; D008297:Male; D009136:Muscular Dystrophies / Q000601:surgery*; D011183:Postoperative Complications / Q000517:prevention & control; D012131:Respiratory Insufficiency / Q000517:prevention & control