Fig. 229

Syphilitic gummas of head and face. (After Jullien.)

Fig. 230

Syphilitic ostitis and osteosclerosis.

 

Syphilis of bone may assume the type of gummatous involvement of the periosteum or of the bone itself or of syphilitic caries and necrosis. The former appears usually as a distinct tumor, ordinarily tender and exceedingly painful, especially at night, it being characteristic of almost all cases of bone syphilis that the pain, however great during the day, is exaggerated at night. The true syphilitic gumma, or syphiloma, of bone is but little different from gumma in other tissues, which may become secondarily infected and then suppurate with the formation of sinuses, etc. Suppuration, however, is rare. Central gumma, like central osteosarcoma, is possible, and may lead to expansion of the surrounding bone. Syphilitic necrosis, so far as the bone lesion is concerned, scarcely differs from the other varieties. It is, however, almost always of the slow form, and involves more often the flat than the long bones. It is especially seen in the cranium and the sternum. Syphilis of bone is often mistaken for rheumatism or pseudorheumatism because of the deep-seated and somewhat indolent pain. Syphilitic disease of bone permits occasional spontaneous fracture, the bone affected with this disease being always more friable than natural. There is also another form of bone syphilis—namely, the hereditary. It leads either to bone enlargement or to caries and necrosis, the latter usually upon the cranium, where extensive ulceration and sequestrum formation may be observed, even the dura being exposed by breaking down of the fungous tissue.

Fig. 231

Caries of lower end of femur. (Buffalo Clinic.)

Hereditary bone syphilis is also characterized by osteophytic formation, by the substitution of gelatinous for spongy bone tissue in the neighborhood of epiphyses, and by early and easy epiphyseal separations. It is characterized also by irregularity of ossification of cartilage and consequent deformity of bone ends, especially about the phalanges and the metacarpal and metatarsal bones. In almost every case where doubt would in other respects arise the other evidences of congenital or acquired syphilis are so plain as scarcely to permit uncertainty (Fig. 230).

The possible combination of syphilis and tuberculosis in the same subject may occur, the lesions partaking of one or the other character according as the tuberculous or syphilitic taint may predominate.

There is urgent necessity in all cases of syphilis in bone, whether operated on or not, for the combination of suitable internal treatment with surgical intervention. Only by this combination can the efforts of the surgeon be crowned with success. In failure to appreciate this fact operation often seems to be almost futile.

CARIES.

Caries is a term applied to infiltration, and substitution in healthy bone of granulation tissue, which has been in use for many centuries, from a time long before the pathology of the condition was understood. Caries never occurs except in the presence of a specific irritant, which, in general, is tuberculous and sometimes syphilitic in character. The pure type of caries is connected entirely with the formation of granulation tissue, and the slow ravages connected with its presence in and substitution for the original bone. As long as septic infection (pyogenic) is avoided it assumes the dry type, as it used to be known, called by the older writers caries sicca. When the fungous tissue is invaded by putrefactive or pyogenic organisms suppuration takes place, and then occur the moist forms of caries, the caries humida of our forefathers, connected with the presence of pus. When closed areas of bone, small or large, being thus shut off from nourishment, die as the result of its presence the complicated condition used to be known as caries necrotica. Occurring under any circumstances, caries is a result and not a cause, and is to be dealt with accordingly.

Peculiar alterations and markings in bone are the consequence of carious changes, and bones are given a fantastic and peculiar appearance in consequence. The surface is almost always irregular, tunnels or canals are formed, and the bone is often honeycombed, as it were, by the excavations just made. Along with the process of osteoporosis and disappearance of bone at one point may be seen osteosclerosis in an adjoining area, and the bone, which is apparently much weakened by the destructive process, is strengthened in a compensatory way by the artificial density of the tissue undestroyed.

The clinical evidences of caries are those of joint and bone tuberculosis or syphilis, which have been already discussed, and its operative treatment consists always in surgical attack with bone chisel and sharp spoon, according to the rules already laid down. The bone which is completely carious calls for extirpationi. e., usually amputation. In the carpus and tarsus resection will often suffice, and also when the disease is limited to joint ends. Occurring in the pelvis, ribs, sternum, or cranium, more or less extensive resections of flat bones are necessary, in the latter place leading to exposure of the dura (of which one need have no fear). The same rules with regard to cleansing and packing the wound should be observed as in operation on tuberculous bones.

NECROSIS OF BONES.

Necrosis corresponds to gangrene of soft parts, and the term, when used by itself, is limited to death of bone tissue. Necrosis by itself is a distinct disease, but indicates the termination of some preceding disease process. It may be considered as:

1. Traumatic Necrosis.

—Traumatic necrosis is due to the discontinuance of the blood supply by accident or by separation of the whole or a part of a bone in the same way. Thus in consequence of multiple fractures fragments occasionally die and require removal. The same result has been ascribed to traumatic or non-traumatic embolism of the principal nutrient artery of a bone, but the possibility of this condition is doubtful, bone being too well supplied by its surrounding periosteum. Necrosis in connection with fracture is rare except in compound fractures, and, when a detached fragment can be seen, may be anticipated by removal of the same.

2. The Pathological Form.

—The pathological form is due to the preëxistence either of tuberculosis, syphilis, or an acute infection, such as osteomyelitis. It may also be the result of acute infectious periostitis, where the periosteum is completely loosened from the shaft of a long bone. These conditions are connected either with the slow ravages produced by granulation tissue, or with the acute septic processes by which infected exudates shut off large areas from sufficient blood supply, or by which in consequence of septic thrombosis a similar condition results. In consequence there may be met bone dying in small visible particles, or the entire shaft of a long bone or several smaller ones may be involved in the destructive processes.

The portion which dies is known as the sequestrum, which may assume irregular and unusual shapes, varying entirely with the area involved. The general character and size of a sequestrum will depend upon the nature of the cause. In acute osteomyelitis it is either a bone shaft or an epiphysis which thus suddenly dies. In the slower processes the fragments may be of almost any imaginable size and form—irregular with jagged ends, or long, extending completely through a bone, either from end to end or from side to side.

3. The Toxic Forms Of Necrosis.

—The toxic forms of necrosis are due mainly to two substances used in the arts—mercury and phosphorus—whose use seems to be inseparable from the manufacture of many modern industrial products.

Mercurial necrosis may come either from the volatilization of the metal in factories where mirrors are made or from refineries where amalgam is distilled. It also occurs from the internal use of the drug. Its effects are seen more frequently in the alveolar portion of the lower and upper jaw than elsewhere. It is through some unknown peculiarity that the jaws are the bones commonly involved in both of these forms.

Phosphorus necrosis, on the other hand, manifests itself almost entirely in the lower jaw, and occurs usually among the young, in factories where matches are made. It is due to the vapors of phosphorus, which cause a form of nearly distinct maxillary necrosis—a fact which has been so widely recognized as to lead to State legislation preventing the employment of the young in such work.

Phosphorus necrosis begins as a periostitis with the production of osteophytes, and is completed as a nearly total necrosis of the entire bone.

Treatment of the Toxic Forms.

—The preventive treatment should consist of supervision of the teeth, the use of alkaline mouth-washes, inhalation of terebinthinate vapors, which neutralize those of phosphorus, and the ventilation of establishments devoted to match-making. The curative treatment consists of buccal antisepsis, opening of abscesses, and the removal of diseased bone, especially of dead bone, upon the first provocation. The occurrence of fistulas should always be regarded as pathognomonic of diseased bone. In aggravated cases, such as are rarely if ever seen since legislation has been brought to bear upon the subject, practically complete necrosis of the lower jaw, either en masse or in portions, was far from unknown, and the possibility of regeneration of the bone was for a long time discredited, until the late James R. Wood, of New York, exhibited a specimen, both at home and abroad, which proved its possibility. Since then we have learned that it is possible for bone thus to regenerate, the cause of the disturbance having been removed.

PLATE XXXVII

Necrosis of Shaft of Femur with Sequestra. (Life size.)

Fig. 232

Phosphorus necrosis of the lower jaw. (Musée Dupuytren.)

Sequestrum Formation.

—To the portion of bone which dies is given the name sequestrum, while multiple sequestra are by no means uncommon. The sequestrum is white and ivory-like in hardness when it consists of original compact structure. It is rare to find a distinct sequestrum of spongy tissue, as this yields so readily to the presence of granulation tissue and of pyogenic infection. A sequestrum may include an entire bone shaft, or epiphysis, or only a small fragment. A portion of the bone having lost its vitality becomes a foreign body which the surrounding tissues endeavor to extrude or to wall off and surround. The extrusive effort is the one which is usually seen. This is done by the continued presence of granulation tissue, which gradually perforates the surrounding bone at places of least resistance, the result being the slow formation of a sinus or several sinuses, ultimately connecting with the surface, and in which in neglected cases the dead fragment of bone can be seen or felt, or from which it can be withdrawn almost without operation. While this weakening of bone is going on in certain portions a corresponding strengthening process is also being put into effect; and the result is a quantity of new bone, which is often wrapped around the sequestrum and is simply the effort to atone for its pathological weakness and to strengthen it. This new osseous tissue which so often surrounds the sequestrum is called the involucrum, and in many instances it is necessary to remove more or less of the involucrum before the sequestrum can be lifted out of its bed or removed. (See Plate XXXVII.)

The whole necrotic process is intelligible if read aright as an endeavor on the part of Nature to get rid of dead and irritating material. When this effort is properly interpreted the natural efforts can be seconded by the interference of the surgeon at a time when disturbance is limited to the minimum and before external sinuses have had opportunity to form. On the other hand, ignorance and neglect may lead to the extreme condition, and most fantastic arrangements of sequestra and involucra are seen in all pathological museums, some of which seem to partake almost of the perplexities of Chinese puzzles. The explanation, however, is always as above afforded. (See Figs. 233, 234 and 235.)

Treatment.

—The treatment should be surgical, and consist in removal of the dead portions and restoration of the parts to a condition favoring rapid regeneration. It should always be radical, but is sometimes made difficult by the inaccessibility of the fragment or by the density of the involucrum and the necessity for large external openings in order to remove the sequestrum.

Large and powerful forceps and strong and well-tempered bone chisels are usually necessary, while, after making the necessary opening for removal of the sequestrum, the sharp spoon should be used thoroughly to scrape away all the lining material of cavities in which fragments have been lying or all fungous tissue which may fill sinus tracks. It will be well after this to thoroughly cauterize the wall of the cavity, after which it is to be packed.

Fig. 233

Fig. 234

Fig. 235

 

Central necrosis of the tibia, long central sequestrum.

Sequestrum inside of a core of new-bone tissue, arranged much like a puzzle.

Necrosis of tibia, showing sequestra after removal. (All three specimens from the Buffalo Museum.)

 

The packing of old bone cavities is of importance, and operators should appreciate the reason for so treating them. The packing is essentially a foreign material which the tissues will naturally endeavor to extrude as they did the sequestrum. The method of extrusion is by filling up beneath and around it with granulation tissue, which later may ossify. The packing is therefore a constant provocation to the formation of this tissue, which is now desirable, and is used mainly for this purpose. It is antiseptic material, and will serve to prevent decomposition of the pyoid material which would otherwise fill such a cavity as the result of waste—Nature’s effort at formative material gone to waste. A number of years ago Gunn suggested the use of wax for this purpose, wax being plastic and incapable of absorption. A piece of white wax was heated in hot water, molded with the fingers to fit the cavity, where it served the purpose of a packing, and was reduced in size with each dressing, as was necessary to permit it still to remain. It is not now used as much as it deserves to be. (See p. 431.)

In favorable cases it may be possible to so thoroughly cleanse the bone cavity without the use of caustics as to justify the attempt, after rigid asepsis, of allowing it to fill with blood, which will coagulate and organize into connective tissue. When this effect is desired the wound should be covered with green silk protective, over which the other dressing may be snugly applied. This healing by the aseptic blood clot is the ideal method when possible.

The extent to which regeneration of bone is possible is often amazing, especially in the young. Thus after removal of the entire shaft of a tibia there may result, in time, not a complete restoration to former integrity, but, in addition, the formation of so much new osseous material as to restore a great degree of strength, and which shall, with the compensatorily hypertrophied fibula, make the leg as useful as ever. In the thigh, however, complete necrosis of the femur means amputation, as it will also in the arm unless the necrotic portion is but a small proportion of the length of the humerus. The treatment of necrosis of the skull, or, in fact, of any bone in the body which is accessible, is based practically on the principles already laid down.

BONE TRANSPLANTATION AND TRANSFERENCE.

In the effort to atone for extensive loss of bone many experiments have been tried, first on animals and afterward on men, success with the former having lent much prospect to the latter. It has been learned, for instance, that portions of living bone can be removed from some of the lower animals and transferred into a bed of more or less healthy sterile human tissues, often with the result that a fragment thus transplanted becomes vitalized and incorporated, and serves the purpose for which it was intended; still these efforts do not in all instances succeed. However, experience has led to the effort to utilize some portion of the patient’s own osseous system. This becomes more easily possible in the case of the forearm or leg where, especially in the latter, a small or less important bone can be utilized to take the place of the greater. Thus, when the entire shaft of the tibia has been removed for necrosis resulting from acute osteomyelitis, the fibula has been sawed across, opposite the site of the ends of the lacking tibial shaft, and transplanted into the trough-shaped depression, thus making it functionate for the lost tibia. Huntington has recently reported a case in which not only was this done, but later the upper and lower ends of the fibula attached to the tibia, with good bony union and with an almost perfect functional result. This will illustrate what elsewhere may be done in this direction.

FILLING OF BONE CAVITIES.

Our methods for removal of sequestra and cleaning out of infected bone cavities are now simplified and made safe. The difficulty which is still universal is to secure a rapid filling or closure of these cavities. If we could be certain of cleaning out every particle of infected tissue and the removal of every germ which might excite putrefaction, then we might resort to Schede’s plan and allow even a large cavity to fill with blood clot and await its organization, but no complicated and infected cavity in such tissue as bone-marrow can ever be so treated to a theoretical degree of perfection. Therefore disappointment often follows this attempt. Senn endeavored to improve upon the plan by the insertion of chips of decalcified bone, but this method is open to the same objection. Dentists have the advantage of surgeons because they deal with small cavities, and in tissues which can usually be thoroughly sterilized. Other things being equal, the methods to which they resort could, with advantage, be imitated by surgeons. In 1903, Mosetig-Moorhof suggested a mass containing iodoform 60 parts, spermaceti 40 parts, and oil of sesame 40 parts. When this mixture is slowly heated to 100° C. and allowed to cool, there remains a soft material which, when desired for use, is melted, being constantly stirred to keep the iodoform properly suspended, while it is poured into the cavity, where it immediately solidifies. It is claimed that its physical properties permit of its gradual absorption and replacement by granulation, and finally by new bone, as has been shown by a series of skiagrams. A cavity in which this preparation is used should be prepared as dentists prepare theirs. It is successful in proportion to the absolute disinfection of the same. For this purpose wide opening and ready access are necessary in order to dry and cleanse. Should oozing be persistent strands of catgut may permit of escape of the blood which enters the cavity. It would probably be best to use the elastic bandage and bloodless method, and to protect for a few moments the solidifying mass before allowing the blood to return to the limb. The originator uses, in his own clinic, a hot-air blast. The air is heated by an electric contrivance, and both dries and disinfects the cavity. After the cavity is thus filled the tissues are closed over it and a sterile dressing applied. It is serviceable in chronic cases and after thorough work. In acute osteomyelitis it is scarcely to be thought of because of the acute character of the infection.

OTHER PARASITIC AFFECTIONS OF BONES.

These are mainly of two varieties—hydatid disease and actinomycosis.

Fig. 236

Achondroplasic skeleton. (Porak.)

Hydatid Disease of Bone.

—Hydatid disease of bone consists in the development of hydatid cysts, which may be either of primary or secondary origin. Almost all the bones of the skeleton are liable to cyst formation, except the short bones of the carpus, tarsus, and digits. In the long bones they occur most frequently in the region of the epiphyses. The particular vascularity of this region is the main factor in their location at this point. The cysts may be unilocular or multilocular, and around them may be a thin or a large area of infiltration. In other words, their boundaries may be abrupt or not. Their volume is exceedingly variable, unilocular cysts sometimes attaining considerable size and distending the bone beyond its normal proportions. (See Chapter XXVI for further reference to the pathology of hydatid cysts.)

Treatment.

—The treatment is purely operative. The contents of the cysts should be evacuated and its walls radically destroyed by caustic, spoon, etc. All sequestra should be removed; in the limbs amputation is sometimes necessitated by the extent of the affection.

Actinomycosis.

—The general character of this parasitic disease has already been considered. (See Chapter VIII.)

The peculiar fungus may be found in the periosteum, in the compact outer layers of the bone, or within its more spongy depths. When the lesion is sufficiently large to be recognizable to the naked eye it assumes, for all practical purposes, the appearance of caries, like that due to tuberculous or leprous diseases, while in the pus or debris discharged from the same or contained within the invaded bones the characteristic yellow, cheesy, or calcareous particles will always be recognized. In this disease there never seems to be the slightest tendency to encapsulation nor to protect against further spreading by any process of repair. The diseased area constantly enlarges its dimensions, involving everything as it spreads, it being limited by no membrane or tissue of the body. Occurring in the bones, it is usually a secondary or metastatic infection, and may be found in any part of the body.

The symptoms will be those of osteoperiostitis, first occurring frequently in the jaws, as it nearly always does in cattle, and often in man; this is accompanied by loosening of the teeth and involvement of the submaxillary tissues. The course of the disease is slow, with little or no tendency toward spontaneous recovery.

TROPHONEUROTIC DISEASES OF THE BONES.

Under this heading it is proposed to group a number of diseases whose clinical manifestations are distinct or classic, but whose underlying causes are more or less obscure.

Achondroplasia.

—This is a lesion of intra-uterine life which includes a softening of primary cartilaginous structures and curvature or malformation of the bones which should be formed from them. It belongs to that period of fetal life between the third and sixth months. It is sometimes referred to as intra-uterine rickets. Under this name it was first described by Müller, in 1860, and since then under various names, most commonly as fetal rickets. It appears that in this disease the fetal cartilage contains mucus abnormally collected, quite generally, in minute cavities or cells just at its borders. The chondroblasts and osteoblasts are not regularly dispersed, and the development of the growing bone is thereby much interfered with. The periosteum appears to have nothing to do with this condition. In consequence the cartilage does not do its proper duty. The long bones fail to attain their proper proportionate length, but become thicker than normal, the periosteum being unaltered. On the other hand, those bones into whose formation cartilage enters but slightly, such as the clavicle and the ribs, retain their normal proportions—the consequence is a peculiar malformation and disproportion of the whole skeleton (Fig. 236).

These deformities are symmetrical, and pertain mostly to the bones at the base of the skull and to the long bones of the limbs; therefore the distinctive appearance may be recognized even at the birth of the child. The head is disproportionately large, the spinal column short, the lumbar curvature exaggerated, all of which is rather the reverse of the ordinary rachitic manifestations. The disease is not common (Fig. 237).

Fig. 237

Achondroplasia. (Lugeol.)

Prognosis.

—The prognosis is unfavorable, because it seems impossible to undo the faults of the intra-uterine condition. The disease, however, is not incompatible with a long life.

Rachitis.

—This also is a constitutional condition, and has been described in 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.

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.

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.

Fig. 240

Osteoarthropathy. (Marie.)

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.