Part 15

Rickets and mollities ossium seem to differ also in this. In the latter there is seldom, if ever, any reparative action. The diseased process of deposit continues in the bone, the softening increases, and the patient ultimately perishes. Whereas, in rickets, the softened and yielding state of the bones is only temporary: after a time earthy matter is deposited in due quantity, and the bones become compact, firm, and solid, capable of supporting the weight of the body, though necessarily permanently bent and deformed, if proper means have not been employed during the softened condition. The thickness of the rickety bone, as Mr. Stanley has shown, takes place on the concavity, which is the situation where the greatest strength may be added with the least expense of new matter. In the same way the reparation of fractures not accurately adjusted goes on most actively in the concavity formed by their displacement.

Softening of the bones is met with at all ages, and in different degrees. It seems sometimes to be congenital, and combined with hydrocephalus. It often follows dentition, measles, hooping-cough, or other infantile diseases inducing debility. In females it seems to be produced, or at least often accompanied, by the debilitating effects of leucorrhœa, miscarriages, and floodings. Loss of blood, in any way, predisposes to it. Mercury, given in immoderate quantities, produces a softening of the bones; and, in some most remarkable instances on record, the free use of common salt was the only cause assigned. When the disease affects children, all the bones generally suffer, those of the extremities as well as those of the trunk; the limbs become bent in an extraordinary manner, and the heads of the bones are swollen, and appear to be much more so in consequence of the wasted and flabby state of the muscles. The child walks with difficulty, and in many cases the legs are utterly incapable of supporting the weight of the body, so that he cannot remain in the erect position. The chest and pelvis become deformed, breathing is oppressed, the digestive organs are deranged, and the belly is tumid. The bones of the limbs become flattened as well as bent, and in their concavities, as remarked in the preceding cut, new bone is effused, in order that the column of support may be thereby strengthened. The new deposit is of extremely dense consistence, and is effused in greater or less quantity, according to the degree of curvature.

The bones of rickety subjects are soft, cellular, and of a brown colour, contain a dark fluid, and are very deficient in earthy matter. As a simple proof of the latter circumstance, it may be mentioned that distortions of the pelvis can be, and often are, accurately imitated by soaking the bones for some time in acid, whereby the earthy matter is extracted. In many instances this component of the bones is almost entirely removed, and soft matter deposited instead; they then consist merely of an extremely thin external osseous shell, covered by thickened periosteum, and containing a pulpy substance resembling fatty matter. During the progress of the disease, the urine deposits, often in great quantity, a white sediment, which, on analysis, is found to be the phosphate of lime. In rickets the head is generally enlarged to a greater or less degree, and the bones of the cranium are thickened and spongy; not unfrequently the intellectual faculties remain acute. In adolescents and adults the limbs seldom become affected; the bones composing the spinal column are the seat of the disease, and, along with the distortion of the spine, the position of the ribs is necessarily altered. Certain rare cases have occurred, in which all the bones of the adult were softened to a very great degree. In one remarkable instance, the patient complained of an annoying sense of tightness in the limb most affected, and, on examination, the softened bone was found greatly depressed at that point, as if a strong ligature had been drawn tightly round it. Mr. Howship, who attended the patient, was so kind as to present me some years ago with a portion of the altered femur, which consists of a fatty-looking substance, and appears to contain little or no earthy matter.[13]

Though incurvation of the spine occurs in boys, and even in adults, still it is most frequently met with in young females; and in them it is often induced by their having assumed a bad habit by sitting long in one constrained and awkward posture, as in writing or drawing, without, perhaps, the bones being unnaturally soft in the first instance. It often follows affections of the lower limbs, as of the knee or hip-joint; and is also caused by shortening of a limb, which has been negligently or ineffectually treated after fracture during childhood, or by the patient being allowed to continue a custom of standing awkwardly on one leg. In a very remarkable specimen in my possession, the curvature seems to have resulted from the tremendous enlargement and consequent weight of the head. The whole skeleton (head, thorax, pelvis, and extremities) is deformed, flattened, and twisted. This may have arisen more readily in consequence of the atrophy of the bones, and retardation of their growth, produced by the long confinement to bed. The number of ossa triquetra in the lambdoidal suture was unusually great. The patient attained the age of twenty-five. The affection commenced from birth.

At first, during slight curvature from such causes, the spine can be brought into its original straight position by the voluntary action of its muscles. After some time, however, the curve cannot be remedied by any effort; interstitial absorption of the bodies of the vertebræ towards the concavity of the curve occurs; they become changed in form, and accommodated to their altered position, as shown in the accompanying sketch; the muscles also accommodate themselves to the new position, as do also the various ligaments connected with the spinal column. When the curvature is seated in the dorsal vertebræ it is generally to the right side; this shoulder is raised, and the chest is protruded, whilst the opposite side is depressed and flattened. The clothes hang loose, or fall off on the left side—the patient rests the weight of his body chiefly on the left leg—on stooping the right scapula projects, and, on examination, is found to be nearer to the spinous processes of the vertebræ than the left. The left cavity of the chest is diminished, and the ribs press upon the heart and lungs, causing difficulty of breathing. To preserve the balance of the body, a curvature occurs below the former, and in the opposite direction; and not unfrequently there is a third incurvation situated above the primary one.

The bones of the pelvis become distorted, and are twisted to one side; or, when the softening is great, and the patient confined to the recumbent position, the introitus of the cavity becomes diminished in the antero-posterior diameter; and, if the patient walk about, the ossa pubis are squeezed together, in consequence of the pressure of the ossa femora against the acetabula. The crests of the ilia are often bent inwards, in consequence of the pressure of steel apparatus injudiciously applied with a view of removing deformity. When the bones become consolidated after such distortion, they present most serious obstacles to parturition; and, most unfortunately, crooked and deformed women possess, it is said, “great aptitude for conception.” When, in such females, the untoward circumstance of pregnancy has occurred, it has been necessary, in some, to have recourse to the Cæsarean operation, and others have been delivered with the greatest difficulty and danger; notwithstanding which, many of these latter have, after recovering from a long and tedious illness, again become pregnant.

In some cases the softened ribs not only compress the organs of the chest, but are also pressed down upon the abdominal viscera, or even into the pelvis. The symptoms arising from such displacement are at first urgent, as can readily be imagined, and are often treated as inflammatory, to the detriment of the patient.

After some time, as the state of the patient’s health improves, the bones in some degree regain their original firmness, and the curvatures are rendered permanent. New bone is deposited in the concavity of the curve, at first in irregular masses, but afterwards becoming condensed, and assuming a more regular form, the column is thereby supported and strengthened.

Bending of the spine backward, with depression of the spinous processes, is extremely rare. But curvature forward, with projection of these processes, is by no means uncommon, and is generally supposed to be caused by caries of the bodies of the vertebræ; in many instances, however, it arises from interstitial absorption only.

Curvature from caries of the vertebræ, though not so frequent as the curvature from other causes, is met with pretty often. In adults, the curvature from ulcerative absorption is more common than that from softening of the bones. It is attended with the formation of purulent matter, which presents in the loins, at the top of the thigh, or near the anus; the bones may become affected secondarily, though much less frequently, in consequence of the formation and accumulation of purulent matter in their neighbourhood. There is pain in the loins; the patient walks in a stooping posture, and often complains of pain in the knee or thigh. The lower limbs sometimes become paralytic, as also the sphincters and extremities of the hollow viscera; this, however, may arise, without curvature, from softening of, or effusion on, the chord, or diseased thickening of its membranes. In some cases the palsy supervenes slowly; at first the patient has an awkward gait; he lifts his feet high to avoid stumbling, and afterwards puts them down clumsily and suddenly; the foot, in some cases, is extended, so that the patient is unable to plant the sole on the ground. Retention of urine occurs, and is followed by incontinence, with copious deposits.

In the _treatment_ of Rachitis, the chief indication to be fulfilled is to support and increase the powers of the system; and this may be accomplished by affording the patient a generous diet, keeping the bowels in good order, enjoining gentle exercise and exposure to pure air, by the assiduous use of frictions, and by supporting the softened bones by properly applied and light machinery. Much mischief may be done by clumsy and heavy apparatus which confine the movements of the patient; the muscles are wasted, consequently the spinal column is weakened, the general health is impaired, and the disease is aggravated. Some have recommended the internal administration of the phosphate and muriate of lime, but their efficacy is extremely doubtful. Preparations of iron seem to answer much better in the greater number of cases. In cases of curved spine, apparently arising from bad habit, the patient should be in no degree confined at writing, or drawing, or music; her posture while at work or play ought to be attended to, as well as her mode of walking or standing; and, if awkward, prohibited. Gymnastic exercises of the more gentle kinds ought to be enjoined, such as those with poles and light wooden clubs, the turning of a wheel, the exercise with balls, &c. Carrying weights on the head can only be applicable in certain cases. The shoulders, in some instances, ought to be kept back by means of a light back-board; and in aggravated cases the weight of the shoulders, and sometimes also of the head, must be taken off the spine by a light and well-contrived apparatus. Sea-bathing, good air, out-of-door exercise, and attention to diet, are of material importance. Frequently advantage will result from the patient’s sleeping on a hard mattress; and, in bad cases, from lying down, when tired, on an inclined plane.

In great softening, it will be necessary to confine the patient entirely to the recumbent position, and to support the head and shoulders by a light and firm machine. The causes, symptoms, appearances, and treatment of caries of the spine, in its different regions, with or without curvature, will be afterwards considered.

OF INFLAMMATORY AND OTHER AFFECTIONS OF THE ARTERIAL SYSTEM.

During inflammation of arteries, the actions of the vessels are accelerated and attended with pain; the internal coat is found to be of a red colour, from increase of its vascularity, and not from its being merely tinged with the colouring part of the blood; or it is of a yellowish hue and rough, from the deposition of lymph on its surface, whilst the external coat is thickened by the infiltration of serum and lymph. When bloodvessels are inflamed from mechanical irritation, lymph is secreted on their internal coat, becomes organised, and obliterates their calibre; if deficient in nervous influence and circulating fluid at a particular point, there ulceration of their coats occurs; if violently injured or completely isolated, their coats mortify; and these circumstances must all be calculated on in the surgical treatment of arteries. A universal inflammatory state of the arterial coats is said to have existed, and its symptoms have been minutely detailed; but its occurrence seems to be extremely rare, and the treatment is medical.

Inflammation is supposed to precede degeneration of the arterial coats. As a person advances in life the arteries lose elasticity, and the heart its balance with them; either the one or the other becomes dilated, their parietes are thickened, and the valves are altered in structure; the enlargement of the vessels is generally greatest towards their origin. The dilatation of arteries, more especially of the internal ones, is often very great; ultimately the internal coat gives way, and the external, with the surrounding tissues, yields in proportion as the blood diffuses itself. The internal tunic is occasionally burst in consequence of violent and sudden muscular exertion; and, even when the vessels are pretty limber and sound, effusion of lymph, and obliteration of the vessel ensue, or, more frequently, aneurism.

Previously to the rupture of the internal tunic, however, there is, in most cases, a morbid alteration in the texture of the vessel. The internal coat becomes dry; its textures is more dense and less elastic, and consequently more brittle. Morbid matter is deposited between the middle and internal coats, and this, by stretching the latter still further, diminishes the elasticity and cohesion of their texture. The deposit is at first to a slight extent and of soft consistence, somewhat resembling condensed fatty matter. Afterwards it increases in quantity and consistence, becoming, instead of soft and yielding, dense, hard, and incompressible; in short, calcareous.

Though the morbid deposit is at first confined, as above related, its limits are afterwards extended; calcareous matter is insinuated, either in minute particles or in broad laminæ, amongst the fibres of the middle coat, is also found external to it, and occasionally situated in the cellular coat. In fine, the various component parts of the parietes of the vessel degenerate, according to the degree of advancement which the disease has attained; and such a condition is the predisposing cause to ulceration of the internal coat, and subsequent effusion of blood. The steatomatous, ulcerated, earthy degeneration of the proper coats of an artery, as Scarpa, the celebrated professor of Pavia, has it, are well exhibited in the accompanying sketch.

During violent and sudden exertions the more brittle parts may burst, either at a certain point, or throughout the whole circumference of the artery; and on this such results will supervene as on ulceration of the internal tunic. Ecchymosis then takes place under the cellular coat, which becomes thickened, and incorporated with, and strengthened by, the surrounding tissues; this is the incipient state of an aneurismal tumour. The effusion of blood, gradually increasing, distends the cellular coat, forming the cavity into which it is poured, and produces a tumour of a size proportional to the distensibility of the tunic and the force of the effusion. Sometimes the external coat is separated from the others to a considerable extent by the insinuation of blood. An aneurism, however, may exist from simple dilatation of a portion of the vessel, gradually increasing, and forming a cavity in which the blood accumulates. At one time it was supposed that all spontaneous aneurisms were caused by simple dilatation of the canal; but such an opinion has been long shown to be incorrect, and the term of true aneurism is now confined by many to that tumour and accumulation of blood consequent on the giving way of the internal coat, and situated externally to the canal of the artery. It is true that dilatation may occur previously to the giving way of the coats, and thus the two causes are combined. The dilatation occurs from the calibre of the artery being considerably diminished, in the first instance, at the point where its coats have undergone the calcareous degeneration, and only acts as a predisposing cause to the failure of the coats when thus diseased. When there is mere dilatation, the tumour is generally of an oval form; but when the internal coat gives way, a lateral prominence is formed, and gradually increases in size. The shape of the true aneurism is various: sometimes the tumour is globular, with a narrow neck; and, from this being of considerable length, it becomes difficult, in some situations, as above the clavicle, to ascertain the particular artery which is the seat of disease, the globular extremity of the tumour presenting itself at some distance from the vessel with which its pedicle is connected. This is rare, however. At other times its form is very irregular, being most prominent at the part where the accumulation of the blood is least resisted. Pulsation in the tumour is distinct from the first, and is painful to the patient; and in the external aneurisms it is so strong as to be perceived by a bystander at a considerable distance. The tumour is at first compressible, and completely disappears on firm pressure being applied, either directly to the sac, or to the artery above, the sac being thereby emptied of its contents, or prevented from being filled. It may sometimes be difficult to form an accurate diagnosis, from the circumstance that tumours, not aneurismal, receive a pulsatory movement from an artery or from arteries immediately beneath them; such difficulty is obviated by attention to this simple test—that in an aneurism the pulsation is felt equally in all directions. Besides, if the tumour is moveable, it can be partially displaced, so as not to lie immediately over a large artery, and, if it be not aneurismal, it will then be found to possess no pulsation; if it be an aneurism, its pulsation will not be diminished by any change of position.

The blood contained within the aneurismal sac, being comparatively motionless, coagulates, and the coagulum is attached to the inner surface; at first it contains red globules, but it afterwards loses them, and becomes of a pale hue, consisting solely of fibrin. This coating gradually increases, and attains no small thickness, fresh portions of fibrin being superadded in concentric laminæ. These layers are chiefly deposited from the blood within the cavity, but they also appear to receive addition from lymph being effused by the vessels proper to the original parietes of the tumour. By such thickening, it can be easily conceived that the pulsation will be somewhat lessened. In large aneurisms the accumulation and deposit of fibrin may be much greater at some points than at others, and hence pulsation may be rendered “not equal in all directions.” It is not, however, diminished to any great extent; for absorption of one or more points occurs, and the coating is again attenuated.

In some rare cases the deposition of fibrin has gone on gradually accumulating, filled completely the aneurismal cavity, and thus effected a spontaneous cure, the remaining solid tumour imperceptibly diminishing by the action of the absorbents. After obliteration of the aneurismal cavity, the fibrin is generally deposited in so great quantity as to occupy the calibre of the vessel above and below the tumour, obstructing the progress of the blood, causing it to flow by the smaller and collateral branches, and effecting a spontaneous cure, somewhat similar to that produced by the artificial application of a ligature. Coagula are seldom formed in the dilated vessel, to whatever size it may be enlarged, unless there is fissure of the internal coat; for in no other way can a portion of the blood readily become stagnant, while the calibre of the vessel remains pervious. There is in my collection a preparation of dilated aorta, to the coats of which adheres a large firm coagulum. Occasionally, though rarely, a dilatation of the internal coats is met with accompanied by thinning of the external ones. Of this sort of diverticulum, there is also a good specimen in the collection here alluded to.

A spontaneous cure may also be accomplished from the original aneurism being compressed by one of a more recent origin, causing ultimate obliteration of the canal. Of this I recollect one remarkable instance; the patient was afflicted with an aneurism of the axillary artery, which had attained a large size, and the cure for the disease in this situation being then unknown or unattempted, the patient was considered as lost; but some time after the tumour began to diminish, and disappeared. The patient died; and the cause of death was found to be the giving way of an aneurismal tumour of the arteria anonyma, which was situated so closely to the aneurism of the subclavian as to have acted as a mechanical compress, causing obliteration of the vessel at that point.

When a cure has been effected, the vessel is found to be converted into a dense and impervious cord at the site of the tumour. The canal above is dilated; the coats are thickened, especially the middle; and from the thickening and increased action of the fibres, the internal coat becomes somewhat rugous, the rugæ being in a transverse direction.

The aneurismal tumour in general increases, and approaches the surface, involving and destroying all the intervening textures. If resisted in its enlargement by bone, even this is not sufficient to impede its progress; the bone is absorbed, and perhaps ulcerated, at the point where it is compressed by the tumour. The osseous is more liable to destruction from this cause than the cartilaginous tissue, contrary to what occurs from compression by abscess. Ultimately the sac gives way, and its contents are discharged either externally, or into an internal cavity or canal, in consequence of its parietes sloughing from the compression made by the tumour; and such termination is instantly fatal.