CHAPTER XXXII.. Surgical Diseases of the Osseous System.

SURGICAL DISEASES OF THE OSSEOUS SYSTEM.

At the outset of a study of surgical diseases of the osseous system it is necessary to emphasize a fact which students and young practitioners are liable to forget, namely, that bone, even the densest, is a tissue, and that as such it is liable to infection, suppuration, gangrene, etc., just as is any other tissue; that all infectious processes are identical in general character, their gross manifestations varying only by virtue of the peculiar characteristics of the tissue in which the infection occurs. Bone is vascular, and even that exceedingly hard variety, which is met with in the petrous portion of the temporal, or the ivory exostosis, has sufficient connection with the vascular system to permit of its proper nutrition. The firmest and hardest bone will bleed when divided or injured, and any tissue which will thus bleed can react injuriously to various irritants.

All bone-marrow begins as red marrow, with 1 or 2 per cent. of fat, and ends by becoming yellow, with 60 or 70 per cent. of fat, and whether this change shall take place suddenly or rapidly depends upon diverse conditions. Many years ago it was claimed by Bourgery that bone is simply a large cavernous arrangement where stagnation of the blood current favors the deposition of fat. Fatty alteration progresses from periphery to centre, and the bones of the hands and feet undergo fatty alterations before those of the trunk and pelvis. In other words, the truncal skeleton remains as “red bone” longer than the balance of the osseous system, and he whose sternum has become a “yellow bone” should have reached a ripe old age. In long bones distal extremities first become fatty. Individual peculiarities seem to govern these changes. Thus the neck of the femur will sometimes be fatty and friable at the fortieth year, or reasonably firm and still red at the eightieth. This fatty condition is not to be confounded with true osteoporosis or rarefaction in bone, though it is often associated with it. When the two conditions are combined we have osteoporosis adiposa. Into this condition immobilized limbs pass more easily than those which are used. Their weeks have been equal to years of ordinary inactivity. Red bone seems to be too highly vascular to be a favorite site for tubercle, and distinctly yellow bone too non-vascular. Consequently bone tuberculosis is less often seen at the extremes of life. White bone, as those who make anatomical preparations call it, is most favorable for tuberculous infection on account of its minimum contents of blood and fat. These bones come from phthisical subjects.

ACUTE OSTEOMYELITIS.

This condition was never accurately recognized until described by Chassaignac, in 1853, and even he missed many of its distinctive features, although he gave to it a most descriptive name, “typhus of the limbs.”

=Pathology.=--The disease is a distinctly infectious process, limited sometimes to the bone-marrow and internal portion of the bone, sometimes apparently involving every particle of the osseous structure. Its onset is sudden, its manifestations acute and serious, and its ravages, when not promptly checked, most extensive. The following more or less distinct varieties may be distinguished:

The staphylococcus;

The streptococcus;

The pneumococcus;

The tuberculous;

Miscellaneous infections, including the colon bacillus, the typhoid bacillus, etc.

It is known that the virulence of cocci growing under pressure is thereby much enhanced; hence the extreme rapidity of some of these disease processes may be thereby better explained.

Acute Osteomyelitis, showing Purulent Foci and Accompanying Disturbance (Kocher.)]

Typhoid infection of bone; focus in rib. (Lexer.)]

The mechanism of the infection and the lesions produced by the organism are essentially similar, and may be described together. These consist of rapid thrombosis, coagulation necrosis, and suppuration, along with the local destruction incident thereto, and with unlimited possibilities in the way of auto-intoxication from the local lesions and from the disturbance of the general economy and interference with excretion. Every severe case is accompanied by more or less of general septic intoxication, presumably from the ptomaine produced by the bacteria, while in many instances, particularly those where the bacteria at fault seem extremely virulent, the intoxication is overwhelming and the course a rapidly fatal one. Death has been known to follow within thirty-six hours after the first symptom of an acute osteomyelitis. For the average case three more or less distinct stages can usually be distinguished: first, a period of purulent infiltration, with the formation of local foci in the bone-marrow and speedy secondary involvement of the periosteum and synovial membrane; second, a period of sequestration or formation of a sequestratrum inside of an abscess cavity; third, the stage of repair.

=First Stage.=--During this period there occurs violent inflammatory infiltration, localized areas becoming at first hyperemic, then infiltrated with hemorrhagic exudate, whose rapidity of production will indicate the intensity of the infection. Often at the same time are found enlargement of the spleen and hemorrhagic exudations in distant serous cavities, such as the pleura and pericardium. The locally infected areas of bone-marrow break down into collections of pus, which spread either toward the epiphyseal line or else along the Haversian canals toward the periosteum, which becomes both infiltrated and loosened. The loosening is particularly marked about the shafts rather than the joint ends, while, as a rule, that end of the bone toward which the nutrient artery is directed is the one whose epiphyses are first loosened. Nevertheless about the knee it would seem as though the lower end of the femur and upper end of the tibia are the particularly predisposed localities.

In many instances obliteration of nutrient vessels and thrombosis are early features. The area of separation of the periosteum is usually an index of the extent of deep destruction. From the periosteum the infection may extend toward the covering of the soft parts, in which case there may be a parosteal abscess, or it may perforate toward the joint cavity, leading quickly to pyarthrosis and destruction of joint structures. It would appear in children, particularly, that the epiphyseal cartilage often forms a barrier to the advancement of the lesion in the direction of the joint, and thus it happens that we have acute necrosis of the shaft of a long bone, with perforation through the periosteum at both of its ends. In adults this takes place less often, the joint ends being often primarily involved. Softening and separation of cartilages are usually secondary to the other processes. It is possible even to have the primary infection in the joint end proper, and extension therefrom to the epiphyses permitting of epiphyseal separation and extrusion of this fragment as a sequestrum. This separation occurs in many instances rapidly and before the attendant is aware of what has happened.

=Second Stage.=--The second stage includes, coincidently with the occurrence of suppuration, the proliferation of considerable granulation tissue, by which more or less protection is afforded; also, when time is afforded, the rapid formation of new bone, whose effect is to wall off the scene of conflict and death from the surrounding tissue, by which event prognosis, so far as the patient’s life is concerned, is improved. Intra-osseous abscesses may quickly coalesce, and the result may be one long tubular abscess extending through the shaft. At other times both bone-marrow and the cancellous tissue are bathed in pus, while if the periosteum have been totally separated the consequence will be a sequestrum whose dimensions correspond with those of the shaft. When periosteum is not loosened the necrosis will probably be central and more or less circumscribed. (See Plate XXXV.)

=Third Stage.=--The third stage is the period of efforts at spontaneous repair. There is a natural effort toward elimination of the sequestrum by the process of softening or liquefaction in the direction of least resistance. This process may extend over months, when surgical relief has been delayed, and may be accompanied by so much other disturbance as to completely ruin a bone or limb for further use. In neglected cases several sinuses may lead down toward the central sequestrum. On the other hand, once this sequestrum of eliminated an extraordinary amount of activity is usually displayed in the direction of repair (Fig. 224).

Acute necrosis of tibia, with formation of cloacæ for affording opportunity for escape of sequestra. Illustrating also the extensive openings which necrotomy may necessitate. (Lexer.)]

=Symptoms.=--In a general way the signs and symptoms of acute infectious lesions in bone are strikingly similar, and are significant when construed aright. Patients complain usually first of exhaustion, followed by pain, which may become agonizing. This is often accompanied by an introductory chill with high fever, after which the general character of the disease assumes the typhoid aspect. Evening temperature may rise high and be followed by some morning remission. The spleen is usually enlarged, the primæ viæ disturbed, and often we have to do with a fetid diarrhea. In the young the sensorium is early affected and children soon become delirious. The pain, at first vague, quickly focuses in the particular bone or bones most involved, and as it increases in intensity there is a significant tenderness. Ordinarily there appear early reddening and swelling of the affected parts. With all these evidences there is also a characteristic muscle spasm, by which certain posture signs will be produced, varying with the bone involved. Pain is always intensified by the slightest degree of disturbance. In consequence the limbs (for it is the limbs which are usually involved) are contracted, and every effort to overcome the contractures is followed by aggravated pain. The more acute the pain the more vivid the external evidences of inflammation and the edema of the parts, especially below and about the lesion. Thus it may happen that within forty-eight hours there may be swelling and edema of the part involved, which should be regarded as pathognomonic.

A little later, superadded to the other signs of inflammation, there is fluctuation if parosteal abscesses have formed, or possibly the evidences of epiphyseal loosening or complete separation. When the disease is primary in an epiphysis the corresponding joint will be early involved, and the joint symptoms will assume the type of an acute purulent synovitis, but with more pain. It is probable that under few circumstances is complaint of pain more serious or aggravating than in cases of acute osteomyelitis of the fulminating type.

So far only local symptoms have been described. To these there should be added the list of those pertaining to thrombosis and metastatic infection, with their septic and disastrous consequences. The disease is frequently so acute and rapid that even within the first day or two not only are added extensive thrombosis in and along the bones, with rapid purulent degeneration and thrombi, but soon that even more serious general condition to which these lesions so easily give rise--i. e., unmistakable pyemia.

The general symptoms are common to the disease, no matter what bone be involved. Local symptoms will change in accordance with their location. While not so common, the flat bones, like the pelvis, cranium, and sternum, may be involved in active manifestations of this disease. The same is true even of the vertebræ, but, as a rule, it is in the long bones of the extremities that its ravages are most frequently seen.

=Prognosis.=--The prognosis depends upon the early recognition of the disease and prompt surgical relief. There is perhaps no disease less amenable to purely medicinal treatment, and if bones are to be saved in their entirety early and free incision is called for. Consequently when the case is seen late it almost invariably entails necrosis, with more or less disturbance of function, or possibly such a serious condition as to call for amputation. The fulminant cases when not early recognized and promptly operated often prove fatal, and death has been known to follow within thirty-six hours after the onset of the first symptom, the fatal result being due to overwhelming septic infection, with thrombosis, etc. Almost every case, however, if seen sufficiently early can be saved.

=Complications.=--The complications are to be divided into the constitutional and the local. The former refer rather to the spread of septic infection and its more or less disastrous and remote ravages. Metastatic infections may produce serious or fatal complications, while, when less acute, important functions may suffer a serious impairment. Among the local sequels are to be considered mainly the results of destruction of bone tissue and neighboring joint structures. When the disease occurs in young and rapidly growing children partial or complete arrest of development in the bone involved is not infrequent. This may lead to inequalities in length of the femora or humeri. It may lead also to compensatory hypertrophy of bone, with perhaps considerable distortion during subsequent growth.

An entirely distinct consequence of osteomyelitis is bone abscess, in which the acuteness of symptoms has long since subsided, but in which a distinct local focus remains.

=Etiology.=--The disease is an infection from the beginning, but the source of the infection is not always easy to trace. Two distinct causes seem to conspire to produce the majority of these bone infections--microörganisms of more than ordinary virulence, and a predisposing condition of the system, due sometimes to constitutional weakness or inherited taint, or to the results of exposure and fatigue. The causes of suppuration have been discussed in Chapter III. It is a fact, however, that the majority of cases occur in children and after a combination of exposure and fatigue--as, for instance, sitting upon the ice after being exhausted by skating--all of which would be inoperative to produce an infection were not the germs at hand ready to assail every tissue whose resistance is thus temporarily lowered.

The infection may occur from within or from without--from within perhaps through the alimentary canal or the respiratory tract, probably from the tonsils and the pharynx. Infection from without may occur through an abrasion or scratch, a blister upon the foot made by an ill-fitting shoe or by a skate-strap. These cases occur generally in the young, more often in boys than in girls, probably because in the former more opportunities for infection are permitted. Bone infections, however, are possible even in the newborn, in which case the infection may occur through the pharynx or through the umbilicus, while the local resistance may have been lowered by the injury due to mechanical delivery, turning, etc. In elderly people the disease is almost unknown.

=Diagnosis.=--The disease for which this is most commonly mistaken is acute rheumatism. There may have been some excuse for this in the past because of the lack of general knowledge of bone infections; now there is none. The majority of cases of necrosis following osteomyelitis which have come under the writer’s observation were the result of errors in diagnosis.

Rheumatism is never followed by suppuration and seldom produces a septic type of disease; its painful lesions are rarely so painful as those due to osteomyelitis. Lesions of rheumatism are usually multiple; those of bone infection are mostly single. The first complaint of pain in the latter is generally along the shaft of a bone than at the joint end, while this is not true of rheumatism. Moreover in acute osteomyelitis the disease assumes from the outset a seriousness which is seldom approximated by acute inflammatory rheumatism.

=Treatment.=--The treatment for acute osteomyelitis is essentially surgical. Anodynes may be necessary for relief of pain, but no time should be lost, when once the diagnosis is made, in making incisions to expose the bone involved, and then opening to its interior to relieve tension and to remove septic products. The incision over the femur or tibia, for instance, may be ten or twelve inches in length. The tissues will invariably be found edematous or infiltrated, with evidence of the proximity of pus; the periosteum will be thickened and infected, and between it and the bone, as well as outside of it, there may be collections of pus. If seen late the characteristic muscle appearances already described may be noted. The periosteum should be incised to the bone throughout the length of the incision, and then an ordinary bone drill may be used to perforate the bone for exploratory purposes. From the punctures in the bone thus involved will exude purulent fluid, often sanious, thus indicating the condition within. A deep groove or channel should now be cut, opening into the marrow cavity, in which numerous foci will be found, or in which all distinctive structure of bone-marrow may be lost, the cavity being filled with pus. The pus cavity should be scraped and disinfected with hydrogen peroxide and cauterized with zinc chloride or its equivalent, and then packed, the wound being left open. Even this may not be sufficient, but if there be epiphyseal separation, or evidences of joint infection, the neighboring joints should be explored under aseptic precautions; if pus be found they should be opened, washed out, and drained. Meanwhile if in the soft tissues exposed by the incision the parosteal veins are found filled with septic thrombi, they should be opened as far as exposed and their contents removed.

These operations are often severe, but nothing in the way of operative treatment can be so severe nor so serious as the disease itself when left unoperated; the rule is stringent that every infected tissue, and especially every infected bone interior, should be exposed and cleaned out. Only in this way can lives be saved. Moreover, it is necessary to carry out this treatment in the fulminant cases as early as possible; and errors in diagnosis by which it may be postponed until metastatic infection or grave pulmonary and cardiac complications have set in are unfortunate. So long as the local indications are as above described, surgical treatment is desirable, whether the systemic complications are pronounced or not. The immediate effect of the operation having passed the relief thus afforded will often be so pronounced that within twenty-four hours patients may be out of danger.

Total necrosis of humerus, as seen by aid of the cathode rays. (Lexer.)]

The results of this operation are a wound which will discharge at first freely, and which so soon as septic material is out of the way will begin to granulate. Ordinarily no attempt should be made to close such a wound, though much may be done to favor rapidity of granulation. While some antiseptic dressing is always employed, it will be of advantage occasionally to change the character of the same, and to alternate between various antiseptics, the effect of any one drug being apparently lost after it has been used for some time.

There are some cases where an entire diaphysis or bone shaft will be found separated from one or both epiphyseal terminations, lying in a subperiosteal abscess cavity, bathed in pus, and dead beyond possibility of repair. This is total necrosis of the shaft from an acute infectious process, and is to be treated by complete removal of all dead and dying tissue. In the case of the forearm or leg it may be that the remaining bone, when only one is involved, as is usual, will be sufficient to maintain the integrity of the limb until new bone can be reproduced within the periosteal bed occupied by the old one. More or less complete regeneration of bone is possible, particularly in the young, and in connection with compensatory hypertrophy of the parallel bone will permit the restoration of the leg to partial or complete usefulness. On the other hand, should this later prove a complete failure, amputation and substitution of an artificial limb may be required.

When the disease has involved the articular side of an epiphyseal line, and when there is complete epiphyseal separation with consequent pyarthrosis, the probable consequence will be necessity for a complete or partial resection of the joint and the probability of subsequent ankylosis. Patients may find later that a modern artificial limb with its possibilities will be preferable to such a condition, and may readily consent later to an amputation which they would at first refuse.

=Acute Infectious Periostitis.=--This is an infection of the same general character and type as the osteomyelitis just described, but refers to those cases where the disease apparently is confined to the periosteum and the outermost layer of the bone. In its possibilities for harm it is scarcely less serious, although in its tendency to spontaneous perforation and escape of pus it is less likely to prove fatal.

=Causes.=--The causes and the general clinical manifestations are practically identical. The disease is perhaps less grave in its acute manifestations, the localization of pain more exact, with ordinarily less tendency to joint complications. Local tenderness is exquisite, and particularly in those bones which lie near the surface--e. g., the tibia--and early recognition of fluctuating areas is easy. It may be localized over a small area, or the entire periosteum of the shaft may be involved; in which case, so soon as pus forms and the periosteum is separated from the bone, there is probability of acute necrosis of the shaft. Here, again, there may be a tendency to mistake at least the first signs of the disease for acute rheumatism, from which it must necessarily be early differentiated as above.

=Treatment.=--Here also there is the same necessity for immediate intervention, if possible before pus be formed, in order that there may be little or no periosteal separation and encouragement to necrosis. Anesthesia is necessary, with prompt incision, the use of the sharp spoon, and disinfecting agents: no attempt should be made to close the wound, but drainage should be favored in every way. The intensity of the pain is promptly relieved and the whole clinical picture immediately changed by such a procedure.

The ordinary bone felon upon a terminal phalanx is practically an expression of this type of disease, and experience corroborates the wisdom of deep and early incision, even in the case of so small a bone entity as a phalanx.

=Acute Epiphysitis.=--This is a term applied rather indiscriminately to a form of acute osteomyelitis involving primarily and especially the epiphyseal lines, or to a condition of hyperemia and neurovascular excitement at epiphyseal junctions stopping short of suppuration, but giving rise to intense pain, muscle contraction, joint tenderness, etc. It is often seen at the upper end of the tibia. Sympathetic disturbance may extend even to serous effusion into a joint, although this is not necessarily the case. The limbs are early drawn up, and every attempt to extend them simply aggravates the distress. So long as there are no evidences of suppuration, it is sufficient in these cases to apply a sufficient degree of traction to overcome muscular contracture and to straighten the limbs. This should be applied first under anesthesia, and the patient kept under anodynes for a few hours thereafter. So soon, however, as the muscles are tired out by the steady traction, pain subsides, and the intensity of the condition may be thus relieved within forty-eight hours or less. It would be well to continue physiological rest and traction as long as there remains the slightest tenderness. Should evidences of suppuration at any time supervene, incision and evacuation of pus and exudate should be practised. Should epiphysitis occur in one of two parallel bones, there may result such failure of growth of that bone as shall cause marked deformity in the attacked hand or foot. In some of these cases, should operation be required on one bone, the other may be shortened at the time, or later, by exsection of a portion of the shaft, or even of the epiphyseal junction.

Osteogenesis and osteosclerosis in slow infective processes. (Buffalo Museum.)]

=Periostitis Albuminosa.=--This is a rare manifestation of bone disease, only given an identity of its own since 1868, when Ollier first distinguished it, since which time it has been the subject of considerable controversy. The name refers to a condition less acute than the infectious periostitis just described, almost always localized in a single bone, necessitating incision and evacuation of a fluid which is gelatinous or mucoid in appearance rather than purulent. It is because of the peculiarity of the subperiosteal collection of fluid that it received the name periostitis albuminosa, and it was not generally regarded until recently as a variety of the infectious form of periostitis. It is, however, now conceded as being a mitigated form of infection, in which the products of exudation assume the serous rather than the purulent type. In some instances it appears to be the tubercle bacilli which are at fault. At all events, the organisms which produce the disease are more or less virulent, else the clinical form of the disease would be less serious than it really is. Cultures made from these subperiosteal collections have in almost all recent instances revealed the presence of some one of the numerous pyogenic organisms. Quite recently Dor has described a polymorphic microbe, in instances of this kind, which he has called the Bacillus cereus citreus, with which he claims to have been able to reproduce the disease in animals.

=Chronic and Latent Osteomyelitis.=--As in the lungs, however, chronic lesions are met with, and as in the lungs, again, it is possible for collections of microörganisms to become more or less encapsulated and for a long time to lie latent until some provoking cause excites them again into activity. In this way are to be explained the numerous instances of recurring abscesses within the bone necessitating repeated operations, often at long intervals. (See Plate XXXVI.)

=Possible Consequences of Any and All of the Bone Infections.=--Bone is a living tissue, calcified and stiffened by inorganic material for the purpose of giving it strength; it may suffer remotely from the consequences of local infections, the same as other tissue. Thus it may have its nutrition impaired so as to produce atrophy on one hand, or increased so as to lead on the other to hypertrophy, either regular or irregular in outline. Again in its texture it may be altered to a wide extent between the sponginess or porosity on one side (osteoporosis), or to the density attained by ivory (osteosclerosis) on the other. Similar changes are also noted in cases of bone tuberculosis, which is to be considered by itself. The densest bone has sufficient vitality to permit its nutrition and life, and may assume dimensions much larger than that of the original, and a hardness which will defy the best steel instruments should it become necessary to operate upon it. The other extreme of osteoporosis includes a condition where the bone has barely sufficient inorganic material to permit it to retain its shape and ordinary proportions. Such bone is fragile in the extreme and scarcely serviceable as a supporting tissue. The principal portion of its bulk is constituted by marrow tissue, which makes it extremely vascular, but far from strong. When spongy it is ordinarily unserviceable for its proper function. Astonishing pictures of osteosclerosis and osteoporosis side by side are present in many instances of disease, the latter being often evidence of more or less ossification of new-formed granulation tissue. This is often a happy combination, because the bone, which has been sadly weakened by disappearance of its calcareous material by liquefaction and by absorption, is reinforced along some of its lines by a pillar of osteosclerotic tissue, by means of which it still functionates as a more or less useful support (Fig. 226).

The operating surgeon should familiarize himself with the density of normal bone in various locations, as in many operations upon the deeper bones he detects healthy bone rather by the sense of touch and of hearing, and the resistance which it offers to his instruments, than by sense of sight.

TUBERCULOSIS OF BONE.

In Chapter IX, on Tuberculosis in general, we entered into considerable detail in regard to the nature of tuberculous lesions, which were stated to be essentially the same whether occurring in hard or soft tissue, the active agent being the now well-known Bacillus tuberculosis, which, finding lodgement, for instance, in the osseous tissue, acts as a specific irritant, and so provokes the production, first, of a typical tubercle, and, later, of typical granulation tissue, by whose ravages the distinctive signs of bone tuberculosis are produced. This process, then, is in no respect different in bones from similar lesions in other parts, though modified to a slight extent pathologically, to a greater extent clinically, by the dense environment. Nevertheless, trifling or most extensive destruction of bone substance is produced by this tissue, while by continuity or by metastasis there is more or less involvement of the adjoining textures, either parosteal or articular. It is by granulation tissue that so-called caries is produced, and it is by the same tissue that distinct portions of bone are sometimes completely segregated from their vascular surroundings and shut off from nutrition, so that they die and form what are known as sequestra. Necrosis may then be the result of tuberculous disease.

Tuberculous Disease of Hip-joint and Pelvis, involving the Muscles (rare). (Lannelongue.)

o, rarefying ostitis (i. e., osteoporosis); f, fungus granulation tissue.]

So long as the process is active, this granulation tissue tends to enlarge its boundaries, and, like pus, to spread in the direction of least resistance. When produced in the shaft of a long bone this may lead to involvement of the entire shaft, or there may be liquefaction and absorption of dense bone and the formation of a sinus from the marrow cavity to the periosteum, beneath which the granulation tissue will spread, and through which it will sooner or later perforate, to resume its progress toward the surface, always in the direction of least resistance. In this progress tendon sheaths or bursæ may be involved, or dense aponeuroses may turn the granulation column aside, causing it to perforate toward the surface at some remote point; while it may spread out more or less beneath the skin before finally causing its destruction. Sooner or later, if uninterrupted by treatment, this escape will occur, and then we have the condition of a tuberculous ulcer of the skin, from which leads down, by a devious path, a sinus toward the original focus.

When this original focus has been juxta-epiphyseal there is involvement of the epiphyseal cartilage and a pathological diastasis, which may early lead to spontaneous or pathological luxation. Or, again, a focus having once originated at an epiphyseal extremity, tends usually to perforate quickly into a joint cavity, after which a considerable length of time is usually expended in filling up this joint cavity with exuberant granulation tissue. This is the material so often found in tuberculous joints, and is well characterized by the name given to it by the Germans, fungous tissue, they calling such joint affections fungous joint inflammations. (See previous chapter.)

Seen thus in joints, after it has been long exposed to friction and to more or less pressure, it may have lost some of its original luxuriant features. It is best seen when it is freshest and has been exposed to least disturbance. Under these circumstances it is vascular, dark red in appearance, friable, and easily removed from the tissue upon which it has grown. Ordinarily it is infectious, and by its inoculation into animals is capable of reproducing the disease.

=Pathology.=--The pathology of tuberculosis of bone may then be virtually summed up in saying that it consists of the ravages produced by the presence of this granulation tissue, with the irritative hyperplasia of surrounding tissues which its presence always excites, even though they be not actively infected. This is the explanation for the majority of cases of caries, of tumor albus, of Pott’s disease, of spina ventosa, and of the condition which has been known under many other names.

=Varieties.= =Acute Miliary Tuberculosis of Bone.=--This corresponds to a similar invasion of the lungs. It might be fittingly described as an acute tuberculous form of osteomyelitis. It may run its destructive course within a short time and cause such involvement of structures as to necessitate amputation of a limb, or it may appear in the truncal skeleton as a primary disease, spreading rapidly therefrom and involving the viscera or the cerebrospinal membranes, and causing an early death, perhaps within a few weeks after its onset. This condition has been more prevalent than is generally understood, and has not even yet received the attention it deserves. It is less painful than the pyogenic forms of osteomyelitis, and may assume less of the septic and more of the typhoid or meningeal type of disease. The pain also may be less severe, though reflex symptoms, especially muscle spasm, will be an early and marked feature of these cases. When a limb is involved the case may not be hopeless; but when involving the cranium, spine, or trunk it is fatal, and little can be accomplished by treatment. The operative treatment for parts which are accessible is given under Acute Osteomyelitis.

=Chronic Tuberculous Osteomyelitis.=--This is the ordinary form of the disease, and is exceedingly common. In some sections it constitutes nearly one-third of the diseases necessitating surgical treatment in clinics and hospitals. This is particularly so in the thickly settled portions of the European continent. In Buffalo it constitutes from 15 to 20 per cent. of cases found in my wards and in my clinic. The proportion some years has been larger.

=Symptoms.=--The essential symptoms of bone tuberculosis are muscle atrophy, muscle spasm and pain, direct or referred, and upon the existence of these, coupled with local tenderness and local swelling, a diagnosis can almost always be made. Muscle atrophy is distinct, and is not alone that of disuse, but is a distinctive evidence of the tuberculous process. It involves the parts above and below the lesions.

Muscle spasm is never lacking, but is most noticeable about the spine and the joints of the extremities. In Pott’s disease, for instance, the condition causes a stiffening of the back and an inflexibility of the spine. About the joints it leads gradually to fixation, usually in the condition of more or less flexion, the flexor muscles being ordinarily stronger than the extensors in all parts of the body. Thus we see the knee and the elbow drawn up, and most other joints in a condition of flexion so far as it may be permitted.

It is characteristic also that muscle spasm is frequently exaggerated, usually in a reflex way, by which pain is always augmented. These sudden but brief contractures occur more often during sleep than during the waking hours, and give rise to the so-called starting pains, usually nocturnal, which are noted in nearly every case of this kind.

The pain is in large measure the result of contracted muscles pulling tender joint surfaces together, and is consequently augmented during the muscle spasms just described to an extent causing the patient to cry out even during sleep. There is also usually a more or less deep-seated and constant pain or soreness, manifested in increasing degree as the lesion advances. These pains are also often referred, lesions in the upper ends of long bones usually giving rise to pain which patients refer to the lower ends. In hip-joint disease pain is often referred to the knee, and in Pott’s disease to the anterior part of the trunk. Slight but slowly increasing disturbance of function of a joint inaugurated by trifling muscle spasm, with complaint of aching pain, is significant and needs careful examination, it being a mistake to anesthetize patients for this purpose, as by the anesthetic the pathognomonic muscle spasm is abolished and mistakes in diagnosis favored.

Tuberculous disease of the hip. (Buffalo Museum.)]

Healed tuberculosis of the spine. (Buffalo Museum.)]

It will be seen that these features are also met with in tuberculous-joint disease, the fact being the conditions are not only allied but often associated.

=Treatment.=--The treatment of tuberculosis of bone is constitutional and local. The former consists in the best possible hygiene and in those measures which are everywhere recognized as helpful in similar conditions. I believe in the internal use of benzosol, or its equivalents, in doses sufficiently large to influence the tissues. In addition the tonics and evacuants should be judiciously used. But it is mainly with local treatment that we shall here have to deal.

The local treatment may be divided into the non-operative and the operative. The former consists in enforcing the general principles of physiological rest, which is done partly by orthopedic apparatus proper and partly by the general principles of traction, and is resorted to mainly in a class of cases treated of under Orthopedic Surgery, the best methods for the purpose, apparatus, etc., being found in the next chapter.

Aside from this a hopeful method has been that suggested by Bier, consisting of making an artificial chronic congestion, it having been long known that tubercles do not thrive when bathed in much blood. The congestion is secured by wearing an elastic bandage above the point involved, elastic constriction being made to a degree as great as may be comfortably borne. The result is venous congestion, possibly edema of the parts below, which to be made effective should be carried nearly to the tolerable extreme. Constriction may be at first enforced for only a short time, but can be later borne for longer periods, until a time is reached when the patient can wear a bandage almost continuously. Marked improvement in many cases follows this method.

The operative treatment consists in ignipuncture, curettage, or formal extirpation. Ignipuncture is the insertion into the bone focus of the glowing point of the thermocautery. It should be practised under an anesthetic, and when the bone is superficial the cautery should be plunged through the skin, making it burn its way into the depth of the bone. This is not difficult when the cancellous tissue is that at fault. If the bone be deep an incision may be made down to it, after which the cautery is applied as above. The result in almost every instance is relief from pain.

This effect seems to be brought about partly by relief of tension, partly by destruction of diseased tissue, and by the acute congestion which is the result of vigorous counterirritation. It need occasion no fear nor difficulty, and is applicable to all accessible bones. It must not be expected to cure every case, but is a measure which may be confidently expected to relieve pain and to do good.

The radical form of treatment is necessary when it can be determined that the carious process is advancing or that pus or caseated deposits are present. This is made known in various ways; but when reasonably sure of their presence it is best to begin the operation as an exploration, going as far as the findings may justify. This may include scraping out of a small focus, or it may entail removal of a large portion of a bone or resection of a joint, or even amputation, according to the severity of the deep lesion. It is best to do whatever may be necessary, and to do it all at once. The operator should not rest content with mere operative attack, but should carefully disinfect the entire tract, cutting away or removing with the spoon the sinus wall and fungous tissue, which he should follow wherever it may lead, disinfecting freely with hydrogen peroxide or caustic pyrozone, and then using an active caustic, like zinc chloride or the actual cautery, unless caustic pyrozone has already been used. In this way material may be destroyed which has escaped the instruments used, and absorbents are eared or closed and protection afforded. My personal preference is for a packing made of bismuth subiodide gauze, soaked in a mixture of balsam of Peru containing 10 per cent. of guaiacol, which I find more advantageous than anything I have used. There should be added to these measures, however, whatever may be necessary in the way of after-treatment, both local and constitutional, and the surgeon should be prepared to operate once or twice again should latent foci subsequently manifest themselves or should there be recrudescence of the active disease.

BONE ABSCESS.

Bone abscess is a term applied to deep and circumscribed collections of pus within the bone, mainly within the shafts of long bones. They are due either to the acute ravages of pyogenic cocci or to the slower lesions produced by the tubercle bacillus. They are frequently evidences of return of disease in its acute type after a long period of latency. The manifestations are usually localized, in this respect differing from those of acute osteomyelitis. The pain is deep-seated and boring, while there is local tenderness, often with considerable enlargement of the overlying bone. The lesion occurs more often in the tibia than in all of the other bones together--at least under those clinical conditions which entitle it to be called bone abscess. The pain is frequently nocturnal or osteoscopic, and patients may endure it for weeks or months before seeking relief.

The surgeon may always expect to find a layer of condensed, sometimes extremely hard bone around these local foci, and it is due to this that they do not either perforate or diffuse and cause extensive trouble.

=Treatment.=--Treatment is always operative; it should consist in anesthesia, exposure of the bone, effective exploration by means of the bone drill, as the hypodermic needle would be used for exploration in the soft parts, and then the free use of the bone chisel or other instruments by which the area may be widely exposed. The density and firmness of the bone under these conditions will sometimes almost defy the best-tempered instruments. Care should be taken to make the external opening nearly the size of the deep focus, in order that the surface may not heal too readily and before the deeper part is filled. The same directions with regard to cauterization and packing the cavity obtain as given before.

SYPHILIS OF BONE.

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

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.

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 extirpation--i. 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;

2. Pathological--i. e., the result of disease; or

3. Toxic, due to the presence of specific poisons in the system.

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.

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

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.

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.

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).

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.

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.

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, 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.)

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.

Osteoarthropathy. (Marie.)]

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.

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 Chapter XXVI. There are few of the true tumors which may not be met with in bone, including the periosteum.

Multiple enchondromas of fingers.]

=Fibromas.=--Fibromas may spring from the periosteum, especially about the jaws and from the base of the skull, from which latter place they may project into the nasopharynx and interfere with the welfare of the patient. Some of these tumors are soft and succulent, as well as extremely vascular, and I have seen death occur upon the table in an endeavor to remove a growth of this kind, hemorrhage being uncontrollable.

Multiple ecchondroses and exostoses.

Skeleton in the museum at Lyons. (Poncet.)]

Multiple ecchondroses and exostoses. (Lexer.)]

Cancellous osteomas springing from the diploë. (Musée Dupuytren.)]

Sarcoma of femur. (Buffalo Clinic.)]

Fungating osteosarcoma of cranium. (Pemberton.)]

=Cartilaginous Tumors.=--Cartilaginous tumors, as stated in Chapter XXVI, are not often found outside of the bony skeleton. They may spring from cartilaginous extremities of growing bones, from epiphyseal cartilages, or from the interior of long and short bones, where their origin is probably due to inclusion of cartilaginous elements, as comprehended in Cohnheim’s theory. In young children they are often multiple and involve various parts of the body. Occurring in adults they are less often multiple, but may attain considerable size. (See Fig. 243.) They are found usually about the ribs, sternum, pelvis, and femora. If the entire structure of a given bone be involved in a growth of this kind, its eradication--that is, amputation--will probably be necessary.

Exostosis bursata. (Orlow.)]

When otherwise, complete removal with careful cauterization of the base of the growth or surface from which it sprang will usually be sufficient. These cartilaginous tumors tend on one hand to mucoid softening and cystic formation, and on the other to calcification or ossification, by which the original cartilaginous character of the growth may be concealed.

=Osteomas.=--Osteomas are by some writers made to include exostoses and hyperostoses. In accordance with the system followed in this work only those growths are considered as tumors which are of no physiological usefulness, and it is preferable to maintain a distinction between osteomas and the exostoses or bone hypertrophies, which pertain either to evolutionary relics or to constitutional affections.

There is, however, a peculiar form of exostosis which becomes covered by an adventitious bursa, whose walls become in time quite thick, which is called exostosis bursata. In the cavity of this bursa may frequently be found rice-grain or other fibrinous concretions. This lesion is common in the neighborhood of joints, and the new bursa frequently communicates with the joint cavity (Fig. 249).

=Myxomas.=--Myxomas are rare in bone, and are seen usually only as degenerated forms of cartilaginous bony or malignant growths. They lead to cystic degeneration. A primary growth of this kind has for its origin the bone-marrow.

=Sarcoma.=--As already described, sarcoma of bone should not be confused with osteosarcoma. (See Sarcoma.) The former refers to sarcoma springing from the true osseous tissue or periosteum. When central the bony walls are expanded and form a shell. Osteosarcoma refers to a tumor springing from the original connective tissue which holds the bony elements together, and contains osseous tissue scattered through it. Sarcoma occurs usually in the long bones, although none are exempt; mostly single, it nevertheless may be multiple. It occurs frequently in the young, is seen even at birth, and in these instances is supposed to take its origin usually from epiphyseal structures. No period of life is, however, exempt. Tumors attain sometimes enormous size. Marsh has recently described such a tumor weighing thirty-three pounds. Microscopically these tumors may assume any of the varieties, endothelioma, angiosarcoma, etc., those of the most rapid growth being found rather of the round-cell type, while those of slow growth are usually myeloid or contain giant cells.

Sarcomas frequently arise from the periosteum. Commencing in the interior of a bone, they develop for the most part very slowly, and expand the bone more or less symmetrically, in distinction to those growths of external origin which are in evidence on one or another aspect of the bone involved (Figs. 247 and 248).

Sarcoma not infrequently has its origin from the callus of a delayed bone union, and I have had repeatedly to amputate for this sequel of fracture. (See Fig. 252.)

As the disease advances there is increase of pain, usually with increasing cachexia, while augmentation in size of such a tumor may make a limb not only useless, but the source of greatest annoyance and difficulty in management of the case.

=Treatment.=--There is but one treatment in cases which will permit it--amputation of limbs, extirpation of tumors from certain bones, or excision of entire bones. Thus for sarcoma of the scapula we extirpate the entire bone; for sarcoma of the skull we make extensive resections of the same, removing the underlying dura when involved; for sarcoma of the lower or upper jaw we remove it in whole or in part. Sarcoma of the spine is inoperable, that of the pelvis almost equally so. In absolutely inoperable cases treatment by the toxins of erysipelas may be tested. In all cases where pain is severe opiates should be administered, which under these circumstances are anodyne, stimulant, and almost nutritive. Patients in this condition should not be allowed to suffer, and opium in assimilable form should always be administered to any amount necessary.

Sarcoma of periosteum of humerus. (Pemberton.)]

Bone cyst of tibia. (Buffalo Clinic.)]

=Myeloma (Kahler’s Disease).=--Collins reports the tenth recorded case in this country. The disease was first described by Bence Jones in connection with a peculiar proteid found in the urine. It is characterized by changes in the bones, with pain in the chest, back, and loins. In the urine albumose appears, which seems to be pathognomonic when taken in connection with such symptoms as those above. On section numerous small tumors are seen in the bones. The disease has hitherto been regarded as an expression of osteomalacia. All the bones of the skeleton may be involved without any tendency to metastasis in other tissues. On minute examination the myelomatous tumors met with seem to be found alike in the bone substance and the marrow, and to be cell proliferations of myeloid tissue. The matter is still left somewhat in doubt as to what should be meant by the term myeloma, this being a feature to be cleared up later. It is seen more often in males than in females, and in the later part of life. Aside from constant malaise, with pain in the back and side, there occur progressive weakness, with anemia, and such final softening and fragility of bones as to lead to spontaneous fractures, or to the projection of tumors, which may be especially noted about the ribs, with deformity of the vertebræ. On close inspection the urine will be found turbid and albumose is detected. The disease is usually regarded as hopeless; there is no information regarding its successful treatment.

Medical Record, April 29, 1905.

Sarcoma developing in callus. (Haberen.)]