Anesthesia in 'stone man': myositis ossificans progressiva.
Myositis ossificans progressiva is a rare disease leading to complete ossification of the muscular system. Very little information about this rare disease and its anesthetic implications has appeared in anesthetic literature. This disease is felt to have an autosomal dominant pattern of inheritance and is usually associated with anomalies of the hands and feet. Afflicted patients are frequently misdiagnosed in childhood as having a rheumatologic disorder. Later in life (as true bone is formed in striated muscle, ligaments, and fascia), the correct diagnosis becomes obvious. Although muscles of the heart, diaphragm, larynx, and sphincters are spared, those of the chest wall are not, and pulmonary function progressively deteriorates. Death frequently occurs as a result of a pulmonary infection. Specific anesthetic considerations include positioning to avoid injury, potential need for fiberoptic intubation or tracheostomy, decreased thoracic compliance with the need for increased ventilating pressures, and the ultimate in disuse atrophy contraindicating the use of succinylcholine. Myositis ossificans progressiva can present the anesthesiologist with interesting challenges. Anesthetic management will need to be individualized according to the severity of the disease.
Stark
W H
WH
Department of Anesthesiology, University of Missouri-Columbia School of Medicine.
Krechel
S W
SW
Eggers
G W
GW
D000230:Adenocarcinoma / Q000601:surgery; D000328:Adult; D000769:Anesthesia, Inhalation; D000771:Anesthesia, Intravenous; D005260:Female; D006801:Humans; D009221:Myositis Ossificans / Q000503:physiopathology; D014594:Uterine Neoplasms / Q000601:surgery