From an Atlas of General Affections of the Skeleton

This congenital affection is characterised by the formation of columns of bone in the soft tissues with progressive limitation of movement. It is usually associated with microdactyly. According to Vastine et al. (1948), it was von Dusch who named the condition myositis ossificans progressiva in 1868, though cases had been described many years before. This title is unfortunate because the primary change is in the connective tissues, fasciae and tendons, the muscle fibres being affected only secondarily ; and moreover the changes are not of an inflammatory type. The condition was reviewed by Rosenstirn (1918) who collected 120 cases from the literature ; in a few of these he rightly questioned the diagnosis, and he suggested that “ fibro-cellulitis ossificans progressiva “ was a more accurate title. The subject was reviewed by Nutt in 1923 and he added fourteen more cases from the literature together with one of his own. It was also reviewed by Mair in 1932. Other titles that have been suggested are “ fibrositis ossificans progressiva “ (Greig 1931) and “ hyperplasia fascialis ossificans progressiva “ (Goto 1912, reported by Rosenstirn). Hereditary and familial influences-There is little evidence of hereditary or familial influence. Sympson (1886) reported a father and son with digital deformity but only the son developed myositis. Drago (1919) reported a patient whose mother had microdactyly. Burton-Fanning (1901) published the case of a father and son, both of whom had myositis ossificans. Gaster (1905) reported the affection in a grandfather, father and three sons-five cases in three generations. It has also occurred in twins, both of whom developed myositis (Vastine et at. 1948). Sex-Both sexes are affected, males more often than females in the proportion of approximately three to two. Age-It is obvious that microdactyly arises in foetal life, but the other manifestations usually develop in childhood before the age of ten years. In about 16 per cent. of cases the early signs were noted in the first year of life ; and, in some, striking changes in the muscles have been observed so soon after birth that they must have begun in foetal life (Hutchinson 1860, Rosenstirn 1918, Mair 1932). Only occasionally is the onset delayed until adolescence. Mair (1932) found only two records of cases in which the first symptoms appeared after the age of twenty years (Frejka 1929, Hirsch and LOw-Beer 1929). Etiology-Maldevelopment of the great toe must begin in early foetal life, and the fault that is responsible for changes in the muscles, no matter how long their onset may be delayed, is also congenital. The cause of this mesodermal fault is unknown. No error in calcium metabolism has been proved. It has been suggested that the underlying cause may be similar to that which is responsible for local ossification after injury, the difference between the two conditions being only one of degree (Painter 1921); but study of the two types of case provides little support for this suggestion. The disorder has been reported in dogs and, in at least one instance, it was associated with microdactyly (Rosenstirn 1918). Clinical signs-Severe crippling may supervene within a few years or it may be delayed for three or four decades. In a typical case localised swellings appear during infancy and childhood, usually in the region of the head or neck and, sooner or later, in the trunk. The swellings may be painful but quite often they are painless and not tender. As a rule they are small, lasting only a few days or weeks, then subsiding or even disappearing altogether, only to be succeeded by others. Injury may sometimes determine the site of a lesion and start the whole