Chapter XIII

. So far as the manifestations in the bones are concerned it is a constitutional dystrophy caused by improper deposition of calcareous material in the softened and somewhat perverted fetal cartilages. It is a condition, however, pertaining rather to postnatal life, and while inconspicuous at birth becomes more and more marked as the child develops. It is essentially a disease of malnutrition, and consequently may be seen in all walks of life, as well in the bottle-fed babies of the wealthy as in the best-nourished children of the poor. The subject should be studied also in connection with the facts set forth in the chapter on the Status Lymphaticus, which bear on the relation of the ductless glands to tissue growth, and especially to rickets. The lesions are widely distributed. The disease is divided by some writers into three periods: (_a_) _Rarefaction of bone tissue_; (_b_) _softening of same_; (_c_) _re-ossification_.

The first stage is the intra-uterine part; the second and third stages are postnatal. To fetal rarefaction have been attributed intra-uterine fractures, even by Hippocrates.

The general dyscrasia and visceral alterations of rachitis interest us here less than deformities of the various bones. The head is disproportionately large, the vertex flattened, the frontal and parietal eminences pronounced; the anterior fontanelle closes very late. To the atrophic alterations of the head have been given the name _craniotabes_. The face is disproportionately small, the lower jaw assuming a polygonal shape. The palatal vault is of the Gothic type, dentition irregular and retarded. In the thorax the clavicular curves are exaggerated, by which the bones are shortened and the shoulders made narrow. The costochondral junctions are enlarged, the result being the so-called _rachitic rosary_. The sternum projects and gives the peculiar appearance known as _pigeon-breast_. The pelvis is often deformed, and frequently distorted to such an extent as in after years to make normal delivery impossible. The spinal column may either be distorted early or is likely to undergo alterations of curvature, due to the combined results of pressure and traction upon softened vertebræ. The joint ends of the long bones are enlarged or clubbed, this being true even of the phalanges. Joint movements are often accompanied by crepitation. The axes of the long bones are distorted, and more or less marked deviations and curvatures result, giving rise to such deformities as knock-knee, bow-leg, etc. (See pp. 161 and 162.)

=Osteomalacia.=--As rickets is essentially a disease of early childhood, osteomalacia is practically confined to adults. The name implies a peculiar _softening of the bones_, by which their resistance and rigidity are weakened and deformity permitted. The disease is common to man and to animals in confinement, and is frequently noted among wild animals dying in zoölogical gardens. It commonly occurs in _pregnant women_, where it would appear as if the mineral elements needed for the growing fetus were abstracted from the mother’s bones rather than from the food ingested. It is brought about also by _starvation_, possibly by _lactation_, especially among those who nurse their children for unusual periods.

[Illustration: FIG. 238

Osteomalacia: celebrated case of Moraud, 1753. (Skeleton now in Musée Dupuytren.)]

_Spontaneous fractures_, especially of the long bones, are frequent. These may refuse to unite properly and false joints may result. The urine will under these circumstances contain an excess of mineral salts, carbonates, phosphates, and oxalates, and when these are discovered in the urine of those suffering from fractures it should always be a warning to administer calcium salts and mineral acids, preferably phosphoric, internally, and to carefully watch the excretions. The progress of the disease is slow, yet steady, and often not easily checked, if at all affected, by mineral acids. Occurring in pregnant women, it may be checked after delivery, especially if the child be not allowed to nurse from the mother. In some instances it occurs with each successive confinement in the same patient, and makes distinct advance with each fresh attack.

=Prognosis.=--The prognosis is therefore unfavorable, least so in puerperal cases.

An _infantile form_, as well as a _fetal form_, have been noted, but it is doubtful whether these forms really come under the same category, and whether they are not manifestations of rickets. A _senile form_ has also been described which affects most frequently the sternum and thorax, which is characterized by excess of nervous excitability and by bone pains, as well as by liability to multiple fracture upon the slightest provocation. This form, however, differs but little from the osteoporosis of advanced years, and scarcely deserves distinct consideration. Certain writers have also mentioned a _symptomatic form_--cancer, syphilis, scurvy, etc.--which, however, is unnecessary, since the fractures occurring in cases of cancer or syphilis are due to secondary lesions of the same character, while those occurring during scurvy are simply an expression of starvation and weakening, even of the bones. Cases of cancer, for instance, where bones have broken without being previously weakened by secondary growths, are exceedingly rare.

Under the name of _osteogenesis imperfecta_ has been described the “fragilitas ossium” of certain writers. The condition has also been known as congenital fetal rickets. These cases may usually be recognized in infancy, in that the extremities are more or less bent and deformed, and the bones very fragile. Sometimes intra-uterine fractures occur, which may be recent or old, and united with more or less callus and deformity. The spinal column will be soft and friable, with marked divisions, and the ribs are often fractured. The clavicle shows lesions of this kind more frequently than any other single bone. Bones so affected will be found extremely fragile and delicate, and sometimes so thin that they may be crushed between the fingers. They are defective in every respect of structure. But these changes pertain mostly to the shafts of the long bones, and do not concern the cartilages. They are to be distinguished from chondrodystrophia fetalis, in which the extremities are shortened, the skin thickened, and the subcutaneous tissues extremely fatty or edematous.

The condition is to be distinguished from rickets, as there is no enlargement of rib ends or epiphyses and no disturbances of the alimentary or nervous systems. Osteomalacia usually occurs after puberty. Hereditary syphilis, in very rare instances, is a factor, but should give additional evidences in other parts of the body. At present there is no satisfactory explanation as to the cause of the condition.

[Illustration: FIG. 239

Osteopsathyrosis. (Blanchard’s case.)]

=Treatment.=--The treatment for all these conditions should be removal of the cause if discoverable and the administration of calcium salts in accessible shape, as in cases of rickets, combined with thymus or pituitary extract.

=Osteopsathyrosis, or Fragility of Bones.=--This is a condition distinct from osteomalacia and is due to trophic nerve disturbance. The condition seems to be hereditary, often extending through several generations. It is characterized by fracture of long bones upon the slightest provocation, and is common to all ages. While apparently congenital in origin, it persists often throughout life, no impression being made upon the condition by medication. It is not characterized by distinctive histological changes, and all theories heretofore advanced toward its cause are disappointing. It is seen, at least in this country, most often in _paretics_ and _inmates of insane asylums_. The ease with which the bones of such patients are broken has given rise to repeated charges of violence or homicide. From one case in which this charge was made I secured specimens of the ribs, which were so fragile that they could be crumbled between the fingers. Such patients might easily sustain serious fractures when undergoing necessary restraint, even of the gentlest nature. Allegations of undue violence are frequently made in these cases, which, especially in asylums, may be most unjust and difficult to prove or disprove.

The _relationship of osteomalacia to exophthalmic goitre_ furnishes another illustration of the peculiar and mysterious influences which the thyroid exercises upon nutrition. The conditions have a similar geographical distribution, as well as being coincidental in the same individual. Honicke, who has recently studied the subject, believes the bone condition to be an expression of thyroidal disorder, the more so in that castration does not remedy the disease, thus proving that the genital glands are not at fault.

The peculiar relationship between the bone and the thyroid in these cases is probably one of disturbance of the elaboration of the phosphorus compounds which are necessary for the proper development of bone, these compounds being excreted rather than utilized.

Osteopsathyrosis of this congenital type is perhaps best illustrated by a case reported by Blanchard,[35] of Chicago, in the case of a woman twenty-seven years of age at the time of his report, who up to that time had sustained over one hundred fractures. In her case it was sufficient to merely gently slide from the sofa to the floor to break some bone. Treatment in her case had been of no avail. (See Fig. 239.)

[35] Trans. Amer. Orthopedic Assoc.

=Senile Fragility of Bones.=--This means weakening of the bones which is incident to advanced age in either sex, due to and comprised under the term _osteoporosis_. Added to this, in certain places is a positive change in shape, also characterizing the senile condition--_e. g._, the neck of the femur. Under these circumstances bones will break with a minimum of violence and without invoking any theory of osteomalacia, osteopsathyrosis, or the like. As bone disappears under these circumstances fat usually takes its place, so that while the volume of the bone may not be particularly diminished, its weight and density are materially altered. (See introductory remarks to this chapter.)

=Atrophic Elongation.=--This is a term first applied by Ollier, and refers to a distinct type of alteration in long bones by which their actual volume is relatively diminished, although they _increase in length_. It is produced largely by lack of pressure, and is seen in many amputated stumps, in which it has much to do with the conicity of the same. It is seen in certain cases of typhoid fever or in forced confinement of the young in bed, where the bones appear to grow at a much more rapid rate than normal. It may also be due to unequal amounts, or defects, of nutritive supply, especially that furnished by the periosteum, and in certain other cases seems to be a purely reflex or trophoneurotic change which is always inexplicable. Frequently accompanying it is muscular wasting, which is to be explained rather by reflex action through the cord, produced perhaps through the mechanism of the terminal filaments of the articular nerves.

=Ostitis Deformans.=--Ostitis deformans is often called _Paget’s disease of the bones_, and is a condition found alike in long and flat bones, the osseous tissue being condensed in texture and increased in amount, or at other times the osseous tissue becoming quite porous and the spongy tissue rarefied without alteration in the marrow. It is due to the unknown causes which may be summed up in the expression trophoneurotic, a _painful_ and a _painless_ form having been described, the former the more frequent. It produces deformities, disfigurements, and hypertrophies of the long bones. It is distinguished from _arthritis deformans_, described in the previous chapter, which is a distinct malady.

In the skull it is usually the face bones which are most involved, although the disease often commences in the cranial bones. The skull proper may be thickened even to 3 Cm. The thorax becomes globular or cubic in form, the arms are relatively too long, and there is usually dorsal kyphosis; the pelvis is thickened and distorted; the ribs are augmented in size and the femora irregularly curved; the patellæ enlarged; the tibiæ more massive and their curves exaggerated. The disease is essentially _symmetrical_, commonly commencing in the cranium and radius. Fractures are rare, because the bones become stronger rather than weaker.

In many instances these changes are accompanied by severe pains, which may be exaggerated by pressure. The malady is usually regarded as rheumatism, but it may be said that even were accurate diagnosis made early it would scarcely avail in treatment, since there is none for it. It may require to be distinguished from hereditary syphilis, in which the tibiæ have more of the saber shape; from _acromegaly_ or _leontiasis_, which begin in the bones of the face and involve the cranium only secondarily.

=Osteoarthropathie Hypertrophiante Pneumique.=--Under this title, which has no exact equivalent in English, was described, in 1890, by Marie, a peculiar affection, often wrongly spoken of in this country as Marie’s disease. This is in large part a pulmonary affection accompanied by enlargement of the extremities. There is reason to believe that there are present microörganisms, giving rise to products that are absorbed into the general circulation, the result of whose presence is an irritative hypertrophy of certain parts, particularly the joints and ends of the fingers, the elbow-, shoulder-, and knee-joints, and often the wrist. There is also ordinarily dorsolumbar kyphosis, which in acromegaly is usually cervicodorsal. The cranium remains intact; the borders of the jaw are sometimes involved.

=Acromegaly.=--Acromegaly is so named from its tendency to increase the volume of the bone extremities or apices. The first case of this disease was published by Marie in 1885. It is characterized by progressive increase in weight, by enlargement of all the extremities, bones and soft tissues alike; but the most characteristic involvement is that of the lower jaw, the upper jaw being little if at all affected. The lower jaw assumes enormous size and projects so that its teeth are far in front of those of the upper. The supra-orbital ridges enlarge, as do also the sternal ends of the clavicles and costal cartilages. As the disease progresses the ribs are widened and the scapulæ enlarged, the vertebræ and the intervertebral cartilages thickened and fused together, causing usually cervicodorsal kyphosis. The long bones of the limbs suffer later, especially at the lowermost joint ends--_i. e._, hands and feet. The viscera are rarely affected, but there is a peculiar and characteristic enlargement, usually of the thyroid and pituitary bodies. The lower cervical ganglion of the sympathetic is also sclerosed; the mucous membrane of the nose is usually hypertrophied; the uvula is enlarged and the larynx often

## participates in the changes. Acromegaly is essentially symmetrical, and

for each change upon one side of the body is noticed a corresponding alteration upon the other. Particular features are observed in individual cases, but the above are practically common to all.

[Illustration: FIG. 240

Osteoarthropathy. (Marie.)]

[Illustration: FIG. 241

Acromegaly. (Original.)]

The underlying pathological condition is as yet undetermined, though most indications point to late alterations along the original _craniopharyngeal tract_ of the young embryo, whose remains are best known in the pituitary body and the thyroid. On this account there is reason for trying the treatment by extract of the pituitary body, or even of the thyroid. The greatest complaint usually is of headache, which is difficult of relief. The disease is steady, progressive, unaffected by treatment, and the prognosis bad, though its course is slow.

=Leontiasis.=--A diffuse bilateral, symmetrical hypertrophy of the bones of the face and later of the cranium, described first by Virchow, the real origin appearing to be in the superior maxillæ, the result being a peculiar leonine appearance of the face, hence the name given to the disease. There is no distinct tumor formation in the bone, but rather the entire structure of the bones involved is affected. As it advances function of the parts is interfered with, mastication becomes impossible, headache and pain are constant. The special senses are disturbed because of involvement of their nerves, and patients die usually from inanition, because no longer able to chew and swallow food. It is distinguished from Paget’s disease, because it shows no tendency to involve the rest of the skeleton; from acromegaly, in which the general shape of the jaw is preserved, though its dimensions are magnified; from tumors of the jaw or face, because of its symmetrical enlargement. Its pathogeny is as obscure as that of the other bone affections mentioned in this list, and its treatment as unsatisfactory.

[Illustration: FIG. 242

Leontiasis: skull of a Chinese woman. (U. S. A. Museum, No. 10,620.)]

TUMORS OF BONE.

As between the various hypertrophic conditions of the bones above noted should be distinguished the true neoplasms, which answer all the requirements of the definition given in