Osteopathies

OSTEOPATHIES, osteopathia (from Greek osteon- bone and pathos - suffering, lesion), a collective term used to denote such lesions of the skeletal system which are difficult to attribute to any definite category of pathological processes (disorders of circulation, inflammation, hypertrophy, etc.) due to the unclear primary pathogenetic and etiological factor underlying them. To denote a specific pathological form, explanatory adjectives are added to the term O., indicating either the most pronounced anatomical change, the degree of spread, or the nature of accompanying diseases, if they exhibit a certain constancy and regularity, etc. Among the individual forms to which this name is most frequently applied, mention should be made of: 1) osteopathia, s. osteodystrophia fibrosa (see Fibrous Osteitis), 2) osteopathie hypertrophiante pneumique (see Marie's Disease), 3) osteopathia hyperostotica (syn. osteosclerosis-fragilis eburnisans generalisata, Marmorknochen, disease of Albers-Schönberg). The main anatomical feature of Albers-Schönberg disease is more or less widespread sclerosis of the bones, resulting from the gradual thickening of old and the formation of new trabeculae of the spongy substance with a corresponding decrease in bone marrow spaces, which ultimately leads to the transformation of the spongy substance of various parts of the skeleton into compact mass. As a result of this, upon X-ray examination, the difference between the cortical and Ill lli spongy layers completely disappears, and the bones on negative films acquire a uniformly white, "marble" appearance. This last feature is particularly pronounced also because the lime content in the bone tissue, as some studies show, turns out to be significantly increased. Apparently it is this increase and the associated decrease in tissue elasticity that leads to the fact that the strength of the bones, despite their compaction, not only does not increase, but on the contrary, they become extremely brittle, which gives rise to frequent fractures, which however heal quickly with the formation of abundant callus. A tendency to osteomyelitic processes is also noted. The disease is usually discovered accidentally during X-ray examination undertaken for fractures or osteomyelitis. The increase in bone mass occurs mainly from the side of the endosteum, so that the outer contours of the bones are not disturbed. However, in some places the periosteum may also participate in the process, causing limited thickenings of the exostosis type. This is particularly often observed in the region of the sella turcica, as a result of which some atrophy of the hypophysis is often obtained, and at the edges of the cranial foramina, the narrowing of which can lead to compression of the nerves passing through them (atrophy of the optic nerve is not uncommon).- As for the details of the histological structure of the bones in this disease, due to the extremely limited number of cases in which microscopic examination has been performed, on the one hand, and rather contradictory results of these studies on the other, it is not yet possible to establish anything definite and characteristic in this respect. The same must be said about the state of the endocrine system, as well as the calcium and phosphorus metabolism, in the disturbance of which many thought to find the primary cause of all changes. The disease, especially in its later stages, is almost always accompanied by more or less pronounced changes in the blood of the type of secondary anemia, which are interpreted by most authors as consecutive, depending on the narrowing of bone marrow spaces and atrophy of hematopoietic tissue. The anemic symptom brings Albers-Schönberg disease very close to the so-called osteosclerotic anemia (see Osteosclerosis), distinguished by Assmann as a separate form in the same year (1907) in which the first fundamental work of Albers-Schönberg also appeared. Some researchers tend to identify these forms, but others point to a number of differences, although not of fundamental importance, however in combination giving each of these diseases its own special physiognomy.. Thus, in Albers-Schönberg disease, the changes in the bones with their clearly expressed eburnation and "great brittleness" stand in the foreground of the clinical picture. These changes almost always begin in childhood, usually early, in some cases even in the embryonic period. The latter is proved not only by the possibility of very early detection of sclerosis (in one case in a three-week-old child, a fully expressed "marble" appearance of the entire skeleton was established radioscopically), but also by the sometimes observed presence of the most marked compaction in the area of ossification nuclei, as well as the absence (non-development) of air cavities in the bones of the skull, not excluding the maxillary cavity, which is normally formed by the 4th month of embryonic life. The anemic symptom is usually not sharply expressed, has a distinctly secondary character and usually develops in later periods of the disease, and in some cases is completely absent. Enlargement of the spleen is rarely noted. The disease, although not of hereditary nature, however often has a distinctly familial character, that is, it is observed in several members of one family or in close relatives. This gives grounds to consider the disease a genotypic disease, caused by a recessive autosomal gene (Gertzenberg and Levit). In osteosclerotic anemia, on the contrary, the main symptom of the disease is more or less severe lesions of the blood and hematopoietic organs of the aplastic anemia, aleukemia or even the so-called Frank's myelophthisis type, while the changes in the skeleton recede into the background, as they do not reach very great degrees, do not manifest themselves by any antemortem signs (absence of fractures, atrophy of the optic nerve, etc.) and are usually discovered only at autopsy. The spleen is usually enlarged. The disease develops in mature or even in old age and is interpreted by most observers as primary myelogenous anemia with secondary proliferation of bone tissue, similar to what is sometimes observed in leukemias. Some authors (M. Schmidt) distinguish these diseases only as two types (childish and adult) of the same pathological form. During the last decade (starting from 1922) several cases of characteristic "marbleness", limited to the bones of one limb or even part of it (osteosclerosis eburnisans monomelica, melorheostosis), have been described. Most of these cases also refer to childhood or adolescent age, which in connection with the typical X-ray picture gives grounds to assume here a variety of Albers-Schönberg disease, especially understandable if one takes as the main moment a developmental defect which concerns here not the entire skeleton, but only a large part of it.- The group O. may also include progressive osteomyopathy (usually known under the name myositis ossificans multiplex progressiva), since in it we are dealing with a primary disease (possibly a developmental defect) of connective and bone tissue, in particular apparently the bone-forming layer of the periosteum (see Myositis).

M. Skvortsov,