Pott's disease, its pathology and mechanical treatment, with remarks on rotary lateral curvature

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UNITED STATES OF AMEEIOA.

Chapter I. Pathology

THE opportunities for making post-mortem examinations, with a view to ascertain the pathological condition of the vertebral column in the earliest stage of Pott's disease — that is, before deformity occurs — are certainly very rare. If the lesion presented at this period, an acute stage, during which the patient might die, our pathological opportunities would be increased, and many points still in dispute regarding its etiology would be definitely settled. But I have never seen, nor obtained the history of, an idiopathic, acute spondylitis. That which has ordinarily been described to me as such, I am convinced, has been nothing more than an exacerbation of an insidiously progressive, but semi-latent chronic state. And many of the so-called acute and sub-acute attacks which ultimate in a completely disintegrated joint, are generally found as part of the history of every progressive, chronic, osseous lesion of the articulations. On the other hand, the frequent occurrence of an idiopathic, acute inflammation of some of the structures entering into the formation of the larger articulations, and especially the synovial membrane and fibrous capsule, has suggested to many surgeons that this acute condition may pass by gradual stages into a chronic one. But no one has, so far as I am aware, attempted to trace a chronic spondylitis to a definite, acute, non-traumatic origin. In every sense this disease may be considered a typically chronic one, from its very inception to its ultimate result. Indeed, the disease is so typically chronic, that it is always a difficult matter to decide in each case, as it presents for examination, at just what time the lesion began : nor is it always clear, especially in the latter stages of the chronic lesions of the spine, hip or knee, as to just what tissue was originally attacked.

If we question intelligent and observing persons who have carefully watched the patient, regarding the early development of the symptoms in a case of Pott's disease, we find, in many instances, that the child has shown an indifference to his ordinary amusements for an indefinite time, which preceded, however, the appearance of pain or deformity. This condition resembles the incipient stage of chronic osteitis affecting the larger articulations, and it points both in chronic epiphysitis of the larger bones of the extremities, and in chronic spondylitis, to a peculiar condition of the system, which, whether hereditary or acquired, places the patient in a state where a slight injury may act as an exciting cause. This peculiar tendency to develop chronic osteitis, either in the vertebral column or the larger epiphyses, upon slight provocation, accounts in great measure, for the readiness with which parents and nurses assign a traumatic origin to the disease.*

I have never had an opportunity to examine, post-mortem, the pathological condition of a vertebral column in the stage alluded to in the first paragraph of this paper, viz., before the appearance of deformity. Even if such examinations were on record, it would require accurate microscopic descriptions of many cases to establish facts regarding the changes which primarily occur, and their most frequent seat.

We have Barwell's conclusions f as to the condition of the joint ends in strumous children, which are, I believe, generally accepted as correct. In the absence, among the authors I have consulted, of other important facts bearing upon this point, and based upon actual and very early pathological exploration, I propose, with the aid I have derived from them and from my own experience, both pathological and clinical, to consider the lesion from these standpoints.

Pott's disease, or chronic spondylitis, as I have preferred to call it, may be divided into four stages.

The first (the one before alluded to) may be very ill-defined, and easily escape the notice of the careless observer. I saw a case recently, for example, where the mother was certain that the first symptom she noticed was " a pain in the back.'' This was " a few days ago." The appearance of the child

* Guersant [Surgical Diseases of Children, Dunglison's Translation, 1873, page 107,) says, "An important observation we may make is, that traumatic arthritis, properly treated, rarely passes into a chronic condition, a fact which we have had many opportunities to verify, although often fearing to the contrary. The cure is assured if the patients are not predisposed to a scrofulous constitution."

\ Barwell on Diseases of the Joints, American edition, page 227.

and the deformity did not indicate a disease of long duration, but closer questioning elicited the fact that a few months before the appearance of the pain in the back, he had had " malarial fever." An examination developed an abscess in the sheath of the psoas muscle, which, undoubtedly, was formed during the " malarial fever." A careful examination — without intimating its object to the person most familiar with the history of the patient — will very frequently develop early symptoms which are as insidious, as is the development, in many cases, of the more apparent signs of the disease. This stage may continue for many months, or it may exist for a few days only, and in some cases it is, apparently, absent. Its existence was first brought to my attention by voluntary statements on the part of those having charge of patients, and it may be stated as the result of my own experience in the matter that, in chronic spondylitis, the first symptoms noticed by intelligent people, in the majority of cases, are those which can only be described as occurring in the prodromal stage. I have found that this condition exists with great frequency, and is sufficiently well marked to warrant its introduction as one of the stages of the disease.

The second stage is that of pain. But this stage, like the first, is sometimes absent, for as Billroth remarks of chronic inflammation of the joints, so clinical experience demonstrates we may say of chronic spondylitis : — " there are cases where the bone is extensively destroyed without any pain."* I have frequently seen cases where the kyphotic curve was the only evidence of a progressive, fungous, non-suppurative osteitis of

* Hackley's Billroth, page 482.

the vertebral bodies. Chronic osteitis of the hip joint also may go on to an actual shortening of the femur, with an entire absence of pain, so far as its oral expression is concerned. Pain, however, is present in the great majority of cases of chronic spondylitis, and it is generally accompanied, and not infrequently preceded, by a marked reflex spasm of some of the spinal muscles. This pain, which, in the earlier stages of the disease, is most frequently experienced at the periphery of the spinal nerves, probably arises from an irritation of these nerves as they pass through their foramina ; but in many cases the inflammatory focus does not involve those parts which are immediately contiguous to the foramina, and are we prepared to say that in all cases the " gastralgia," so-called, arises from this cause and no other ?

The reflex spasm of the muscles does not form the subject of any voluntary complaint on the part of the patient. As I first pointed out,* this reflex muscular spasm in joint inflammation is due to an irritation, peculiar in its character, of the peripheral nerves which actually supply the diseased or inflamed structure. It is accompanied, however, by an apprehensive feeling which is very difficult to define, but which is certainly very distressing to the patient. This sensation, oftentimes merging into violent paroxysms of pain, is not limited to any particular region, and on account of the intimate relation existing between the minute nerve filaments terminating in the vertebrae, and the great nerve centres, evinces itself in many ways, involuntarily. This spasm, which in many cases

* Reflex Muscular Contraction and Atrophy in Chronic Joint Disease. Archives of Clinical Suigery, June 15th, 1877.

might be called tetanoid, is present night and day, and after many experiments in diseases of the larger joints, I have found that it will yield to profound anaesthesia only. Certain it is, that the causes which produce this reflex, tetanoid spasm leave upon the patient, after a comparatively short space of time, evidences of severe suffering — evidences which do not follow chronic lesions of the synovial membrane or cartilage.

The third stage is that of deformity, and is due to some loss of substance in one or more of the bodies of the vertebrae. Sometimes suppuration ensues rapidly, in others it ocqurs later, while, not rarely, a morbid process, which has been aptly termed caries sicca by German pathologists, occurs, which ultimates in a very pronounced deformity without any evidences whatever of pyogenetic action. When abscess occurs it forms the fourth stage. Much might be written regarding this stage of the disease, but the limits of this paper will not permit any extended remarks. I will merely say that the absence of suppuration or any of its symptoms, together with an apparently arrested deformity, not unfrequently serves as a mask to the inherent chronicity of the lesion. Nor does this apparently favorable condition by any means insure the arrest of the destructive inflammation, nor the really progressive kyphosis. There are indications by which the permanent arrest of the morbid action may be demonstrated. But they need not detain us here. The clinical fact to which I wish to invite attention, especially, is that if the symptoms one would naturally expect to find associated with this disease are not present, it does not afford positive evidence upon which to make a favorable prognosis. On the contrary, the absence of suppuration after the disease has existed for many months, points, in many cases, and especially in the fungous caries, to an unfavorable prognosis in point of time. The nearer a chronic spondylitis approaches an acute disease in character the more favorable, and of course, the more quickly obtained, is the result.

Again quoting Billroth, we may say of spinal caries, as of any other chronic joint lesion, — " a good nutritive condition is the most important point for a favorable prognosis, which would not be very greatly affected by an early and extensive suppuration." An early suppuration, even if it be extensive, has, in my own experience, been indicative of a timely and favorable result. (See Case V.) In Pott's disease the formation of pus is, as a rule, marked by exacerbations of pain, by an evening rise of temperature, by a peculiar nocturnal cry, which I have named the osteitic, and sometimes — though it is very difficult to obtain this part of the history in children — by slight chilly sensations in the early stage. The rise in temperature, like that which occurs in the suppurative stage of chronic osteitis of the hip or other joints, does not usually exceed io2°-io3° in the evening, (I have seen it as high as 1050) and in the morning the mercury drops back to 99°-ioo°. If the disease occur in that region of the spine which affords attachment for the psoas magmis, this muscle, as well as the iliaats interims, may become the seat of reflex spasm. Whenever, in chronic spondylitis, the psoas resists when placed upon the stretch, it is well to explore, by palpation, the pelvic fossae and the course of the contracted muscle. There are some patients who will, involuntarily, make the abdominal muscles tense when this examination is attempted. Under these circumstances, I administer ether, after the plan of Dr. Packard,* of Philadelphia, and, during the primary stage of anaesthesia, which in several of my cases has been brought about in less than sixty seconds, I have been enabled to examine for thirty or forty seconds, without meeting any muscular resistance, the abdomen and pelvic fossae. In this way I have been enabled to recognize the existence of an abscess at my first examination, and before any external signs whatever of it existed. As it is always important to know whether pus exists or not in these cases, I can confidently recommend this plan, which I first tested in St. Luke's Hospital, with Dr. Andrew H. Smith, in a case where considerable difficulty in making the pelvic and abdominal examinations led to the adoption of this simple, useful procedure.

I have attempted to make an intelligent pathological division of the various phenomena of Pott's disease. I cannot avoid recognizing that it is impossible in the present state of our pathological knowledge to do much more than make an arbitrary classification of the disease into different stages.

In a typical case, we find the stages I have attempted to describe clearly marked. In many cases we find them all present at the first examination : viz., malaise, pain, deformity and suppuration, with no intelligent history as to their sequence. Again, I have seen several cases where profuse suppuration preceded the appearance of deformity, others where paraplegia occurred before the curvature, and many cases where pain, or rather the oral expression of pain, was wholly absent, though the kyphosis was marked. I was tempted to make a fifth, or exceptional stage, namely, paraplegia ; but this condition is so clearly symptomatic,

* Am. Jour. Med. Sciences, July, 1S77.

and, in reality, occurs so rarely under intelligent treatment, that I have classed it among the symptoms of any of the stages. The irregularity, however, with which the various prominent phenomena appear, has also been accompanied by other peculiarities which have added much to the clinical and probable pathological aspects of the subject. They have, in the main, served to strengthen, rather than weaken the division I have made.

As before stated, chronic spondylitis may be divided into two classes, viz., that in which suppuration is a comparatively early symptom, and the non-suppurative, or that in which caries sicca occurs. As Billroth forcibly expresses it, " The fungous articular inflammations are often accompanied by caries sicca ; the affection may go on for years without the formation of abscess, especially in adults otherwise healthy ; there may be extensive destruction in the cartilage and bones, with the consecutive dislocations already mentioned under caries, without a drop of pus." * This extremely interesting condition I have seen very often, clinically, both in the spine and larger joints, and on sevoccasions after amputation and excision of the larger joints. For reasons which I shall state hereafter, I think it very rare that this condition of caries sicca is seen in the vertebral column after death. Billroth's description of the pathological appearances of this fungous caries is brief and practical. I will condense and quote it here : " If you examine the granulations in the joint and bone, you will find them firmer than usual, and occasionally of cartilaginous consistence, like granulations about to atrophy or cicatrize. Indeed, they do partly atrophy, but the proliferation goes on again, and the bone is destroyed : the

IO POTT'S DISEASE.

process is analagous to cirrhosis ; the more luxurious the proliferation of the granulations, the more extensive the destruction." *

In the vertebral column, the initial lesion may be, among other conditions, an osteitis i?iterna fungosa seu granulosa ; an osteitis interna caseosa ; a caries super ficialis, following a periostitis, or a caries interna necrotica. These descriptive titles, taken from different pathologists, express some of the conditions that may be met with clinically, and pathological investigations prove that the original lesion may be termed, in all but the exceptional cases, a chronic osteitis, to which we may add the various qualifying terms to express the exact pathological condition. The German authors use many names to describe the several conditions found in chronic articular lesions, and while subdivisions of the two general classes — fungoid and suppurative, (the latter frequently taking the atonic form) may be made with advantage pathologically, the terms caries and chronic osteitis are used synonymously by many modern writers, the former signification of caries, " ulceration accompanied by suppuration, open ulcers of the bone," being, to a great extent, ignored. It is thus that we speak of a fungous osteitis, where the inflammatory neoplasia proliferate and then contract and cicatrize, but which may degenerate under certain circumstances into an ichorous, flocculent discharge, which marks the atonic form of caries. A very practical remark of Billroth will bear repeating here : " Pathological anatomists who see caries in the dissecting room only, seldom know the ganulofungous variety well, or regard it the less frequent one. But

* Op. Cit., page 476.

PO TT'S DISEA SE. I I

any one who has frequent opportunities of examining pieces of carious bone during life, especially the resected joints of children, where the process is in a state of active development, will form a very different opinion/' \ The general failure of the vital force which precedes death, produces an effect upon these fungous granulations which results in a rapid breaking down of the neoplastic proliferations of the dry caries — the. result is the formation of a degenerate pus, which is discovered at the autopsy. Hence it is that some observers have concluded that "all spinal caries is suppurative."

In a former paragraph I used the phrase, " comparatively early suppuration." I mean by this, when speaking of chronic spondylitis, a suppuration which progresses rapidly enough to appear as a " cold abscess " within, we will say, a year from the first, specific expression of the disease. As a rule, and depending on the site of the initial lesion, the suppurative form of the disease is characterized by the early appearance of pain at the periphery of the irritated spinal nerve, and an abrupt curvature which is apt to result from the rapid loss of bone and fibro-cartilage. Reflex muscular spasm is not so marked in the suppurative as it is in the fungous form — and suppuration, in spinal osteitis as in caries of the larger joints, seems to exert a modifying influence upon the reflex spasm. The peculiar deformity which results, in many cases, from the suppurative form of the lesion, has been for a long time known under the solecism, "Angular Curvature." In the majority of cases, in my own experience, but few vertebrae are involved in this active, suppurative process, though as the disease progresses

% Billroth's Surg. Path., Sydenham Ed., Vol. II., page 15S.

and involves other vertebrae, the abruptness of the projection may be lost. The caries sicca, on the other hand, more frequently involves a considerable number of the vertebrae primarily, and the resulting deformity partakes more of the character of a true curvature, sometimes, especially in the adult, like the excurvated spine of the old writers.

In the early history of the dry caries the pain partakes of an apprehensive character, which, however, may become acute under slight traumatic influence, and as in the granular caries of the large articulations, the intense muscular, spasm shows the profound impression which the fungous proliferations make upon the nerve filaments distributed to the spongy and vascular osseous tissue. Another fact which I have noticed in connection with these two important conditions — the dry and humid forms of the disease — is that while no portion of the spinal column is exempt from either, the non-suppurative variety occurs more frequently in the dorsal region — more frequently indeed than in the cervical and lumbar regions combined.

The atonic variety of suppurative spondylitis occurs very frequently among strumous children, and is usually described as the typical, strumous Pott's disease. It is, as a rule, accompanied by several of the commonly accepted evidences of scrofula, while on the other hand, I have frequently remarked that the dry caries may exist to a very considerable extent, and progress to marked deformity and loss of osseous tissue, without any marked evidence of dyscrasia in the patient. I have a patient now in my ward at St. Luke's Hospital, whose health is apparently good. There are no evidences whatever of struma, unless we except the joint disease and a slightly tinged sclerotic, and yet he has had a fungoid osteitis of the hip joint for two years. The thigh muscles are atrophied, the limb is shortened an inch, and the joint is practically immobilized by the most intense and persistent reflex muscular spasm. The analogue of this condition in the spine, I have seen very frequently, where all evidences of suppuration were wanting, and the excellent general condition of the patient gave delusive hopes that the kyphos was permanently arrested. This non-suppurative variety of spondylitis, however, is found more frequently, in proportion to the whole number of cases, among adults. It may, however, and following a failure of the general health, it frequently does, assume a suppurative phase. The transformation of the fungoid into the atonic form of the disease is ordinarily attended with urgent symptoms, and in the adult is followed, as a rule, by death. Septicaemia and amyloid degeneration are the most frequent causes of death in the suppurative variety, while tubercular meningitis has only too frequently, in my own experience, followed upon the dry caries, both in public and private practice.

Brodie first called attention to the fact that adults sometimes experience a non-suppurative form of spinal disease. The granular caries was apparently, not known as such to him, and as in many cases, he found a rheumatic history, this eminent writer considered the condition to be of rheumatic origin. In whatever light, however, we view the dry, granulo-fungoid osteitis of the vertebral bodies, clinical experience demonstrates that it is the most tedious disease, either in children or adults, that can affect the articulations : that it is the most difficult to control, and the typical rotary scoliosis alone excepted, the most insidiously progressive.

H POTT'S DISEASE.

I have already referred to the reflex muscular spasm, which is not only an early symptom of chronic spondylitis, but also of chronic epiphysitis. This muscular spasm is a prominent symptom of the dry form of spondylitis and when once fairly developed, it is a pretty sure indication that the best efforts in the way of treatment, both constitutional and mechanical, will not suffice to prevent some increase in the spinal curvature. On the other hand, in the more favorable forms of suppurative spondylitis, where the pus approaches the laudable in character, this spasm is not so marked, as I have before stated, and the result, other things being favorable, should be good. Of the atonic variety I need only say that the ichorous, flocculent discharge tells its own story, and that too much should not be expected of an already overburdened constitution.

There are some forms of spinal deformity or curvature, the etiology of which, in the absence of pathological exploration, is quite obscure. I cannot stop here to mention all these conditions, or to attempt to classify them. I may simply say that they seem to have one symptom in common at some stage of their development, viz., a painless contraction or spasm of some of the spinal muscles. In rotary lateral curvature, for example, there is, seemingly, no direct evidence of muscular contraction in the ec^-liest stage of the lesion, though I have found resistance to lateral flexion quite marked in many cases. If the intervertebral articulations were the perfect analogues of the larger joints, such conditions might correspond, in their incipient stage, to the chronic synovial inflammations of the latter, which, as I first pointed out,* are not accompanied to any

* Loc. cit. and Seguin's American Clinical Lectures, Vol. Ill, No. VI.

POTT'S DISEASE. I 5

great extent by a reflex muscular spasm, and which are also, for a physiological reason, practically painless. I have seen many cases of varied and great deformity of the spine, where the chief agency or factor in the production of the deformity was a painless, but very persistent muscular contraction, and where the apparent condition was, from an ultimate cause yet to be ascertained, an absolute motor paresis of some of the spinal muscles, followed by a shortening of the unbalanced antagonists. In many of these cases also, there are neural symptoms, or even the history of a severe lesion of one of the nerve centres. Such, for example, was the case of H. M., a boy of 14 years. This case was referred to me by Dr. T. M. Markoe, and was seen in consultation with Dr. B. F. Dexter, in November, 1877. In the fall of 1876, the patient, after severe mental exertion at school, had an attack of spinal meningitis, which greatly imperilled his life. He recovered, however, and became, instead of the strong, boy he was formerly, a very delicate and effeminate lad. Soon a slight lordosis of the spinal column was noticed. This became slowly progressive, until at the time of the consultation,, the anterior curvature of the spinal column was very marked, — resembling indeed the position of the spine in extreme opisthotonos. There was little or no muscular rigidity of the spinal muscles in the erect position. The abdominal muscles were tense ; by supporting the abdomen in front, the spine could be gradually straightened, but this the patient could not accomplish unaided.

There was no pain whatever after forcible tests. The lordosis disappeared almost completely when the patient assumed a prone position. The malposition was quite easily corrected with apparatus, but as the anterior curve disappeared under treatment, a typical lateral curvature appeared, accompanied by rotation of the bodies and rigidity, to a considerable extent, in lateral flexion. This is the boy's condition at present, though the scoliosis is under control. There are other cases where, after an insidious prodromal stage, the symptoms partake more of an apparently sub-acute character. The latter stage is generally short, pain being more or less marked. This is followed by a painless, reflex spasm of the muscles. The condition described occurs most frequently in the cervical region ; or, at least, it is the best studied in the neck, and the muscular phenomena present some very curious and anomalous conditions : for example, a marked contraction of the right sterno-mastoid existing with rotation of the chin to the same side ; an apparent anatomical contradiction. This was the condition in a patient, a girl of 12, whom I saw in consultation with Dr. Henry B. Sands in January, 1878, and where we subsequently 'February, 1879, one and one-half years after the first symptoms appeared), demonstrated that the muscular contraction was reflex, all muscular resistance disappearing under ether. The lesion in this case was a spondylitis sicca in the upper cervical region, and yet there has been no pain present for over a year, and the patient possesses an apparently normal degree of muscular power in flexing, extending or circumducting the head within the limits of the contraction, just as patients with rotary scoliosis possess, in many instances, a considerable degree of strength in the spinal muscles, the exercise of which does not produce pain. It certainly is a remarkable fact which I have seen demonstrated many times, that a prolonged reflex spasm of certain muscles either of the spine or limbs (yielding wholly under ether) may exist without any history of pain. Another fact, that I have seen too often illustrated to regard as a coincidence, is that in all, or nearly all these cases, the muscular atrophy and spasm could be traced to an osseous lesion, and particularly to the bodies of the vertebrae or the epiphyses.

That muscular contractions occur in the typical, rotary scoliosis is very apparent, and that these contractions are reflex, or due to some specific cause, is equally evident when their nature is studied. These cases form, however, a distinct group, yet the ultimate muscular condition . reminds me of that which exists in the lower extremities after infantile paralysis. There are other cases where there is a distinct and readily traced paralysis of some of the spinal muscles. The case of H. D. P. will illustrate this condition. The patient was six years old and was placed under my care by Drs. W. H. Draper and E. C. Seguin. The original lesion was a poliomyelitis. Dr. Seguin has furnished me with a memorandum of the muscles primarily affected. Those partially paralyzed (which recovered wholly under Dr. Seguin's treatment) were the muscles of the neck, arm and thigh (left side). Those wholly paralyzed — and which did not recover — were the left s&rratus magnus, the left transversalis and obliquus externus, and the supra and infra spinati of the same side. The vertebral column presented an inflexible dorsal curvature toward the paralyzed side, with the usual compensatory (?) curve in the lumbar region. The most marked rotation that I have ever seen occurred in this case, though, as above stated, the boy was only six years old. There was marked contraction of the unparalyzed

I 8 POTT'S DISEASE.

antagonists on the right side. I have seen several of this class, also, and one patient, a girl of thirteen, is now under observation at the Orthopaedic Hospital. I mention these cases and describe their condition, because I am convinced, after studying over three hundfed cases of the typical, rotary lateral curvature— and especially the development of the muscular resistance— that no analagous muscular contraction exists, in any condition, without some central or marked reflex cause. At any rate, I am prepared to state that rotary lateral curvature has a specific, pathological cause — not merely a mechanical etiology. The absolute demonstration of this statement and the location of the lesion, of course, lie in post-mortem exploration. On another occasion I shall dwell more particularly upon this interesting and important subject.

I have at least demonstrated from a clinical standpoint that a simple, non-specific loss of muscular equilibrium cannot explain the phenomena of a typical, rotary lateral curvature. In other words, the typical, progressive scoliosis does not develop from a simple alteration of the pelvic plane — where an unequal length of the lower extremities causes, in locomotion, a primary lateral curvature in the lumbar region as a matter of compensation. I have carefully noted the conyparative length of the lower extremities in over one hundred cases of the typical, progressive, rotary lateral curvature, and I have found only three where the difference in the length of the limbs amounted to three-eighths of an inch, or more. In view of recent statistics regarding the comparative length of the lower extremities,* this, certainly, is a small percentage, as the patients