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Late Rickets or Rachitis Adolescentium is met with at any age from nine to seventeen, and is generally believed to be due to a recrudescence of rickets which had been present in childhood. The disease is not attended with any disturbance of the general health; the pathological changes are the same as in infantile rickets, but are for the most part confined to the ossifying junctions, especially those which are most active during adolescence, for example at the knee-joint. The patient is easily tired, complains of pain in the bones, and, unless care is taken, deformity is liable to ensue. There can be no doubt that adolescent rickets plays an important part in the production of the deformities which occur at or near puberty, especially knock-knee and bow-knee.
Scurvy-Rickets or Infantile Scurvy.—This disease, described by Barlow and Cheadle, is met with in infants under two years who have been brought up upon sterilised or condensed milk and other proprietary foods, and is most common in the well-to-do classes. The hæmorrhages, which are so characteristic of the disease, are usually preceded for some weeks by a cachectic condition, with listlessness and debility and disinclination for movement. Very commonly the child ceases to move one of his lower limbs—pseudo-paralysis—and screams if it is touched; a swelling is found over one of the bones, usually the femur, accompanied by exquisite tenderness; the skin is tense and shiny, and there may be some œdema. These symptoms are due to a sub-periosteal hæmorrhage, and associated with this there may be crepitus from separation of an epiphysis, rarely from fracture of the shaft of the bone. X-ray photographs show enlargement of the bone, the periosteum being raised from the shaft and new bone formed in relation to it. Hæmorrhages also occur into the skin, presenting the appearance of bruises, into the orbit and conjunctiva, and from the mucous membranes.
The treatment consists in correcting the errors in diet. The infant should have a wet nurse or a plentiful supply of cow's milk in its natural state. Anti-scorbutics in the form of orange, lemon, or grape juice, and of potatoes bruised down in milk, may be given.
Osteomalacia.—The term osteomalacia includes a group of conditions, closely allied to rickets, in which the bones of adults become soft and yielding, so that they are unduly liable to bend or break.
One form occurs in pregnant and puerperal women, affecting most commonly the pelvis and lumbar vertebræ, but sometimes the entire skeleton. The lime salts are absorbed, the bones lose their rigidity and bend under the weight of the body and other mechanical influences, with the result that gross deformities are produced, particularly in the pelvis, the lumbar spine, and the hip-joints.
Neuropathic forms occur in certain chronic diseases of the brain and cord; in some cases the bones lose their lime salts and bend, in others they become brittle.
Osteomalacia associated with New Growths in the Skeleton.—When secondary cancer is widely distributed throughout the skeleton, it is associated with softening of the bones, as a result of which they readily bend or break, and after death are easily cut with a knife. In the disease known as multiple myeloma, the interior of the ribs, sternum, and bodies of the vertebræ is occupied by a reddish gelatinous pulp, the structure of which resembles sarcoma; the bones are reduced to a mere shell, and may break on the slightest pressure; the urine contains albumose, a substance resembling albumen but coagulating at a comparatively low temperature (140° F.), and the coagulum is re-dissolved on boiling, and it is readily precipitated by hydrochloric acid (Bence-Jones).
Ostitis Deformans—Paget's Disease of Bone.—This rare disease was first described by Sir James Paget in 1877. In the early stages, the marrow is transformed into a vascular connective tissue; its bone-eating functions are exaggerated, and the framework of the bone becomes rarefied, so that it bends under pressure as in osteomalacia. In course of time, however, new bone is formed in great abundance; it is at first devoid of lime salts, but later becomes calcified, so that the bones regain their rigidity. This formation of new bone is much in excess of the normal, the bones become large and bulky, their surfaces rough and uneven, their texture sclerosed in parts, and the medullary canal is frequently obliterated. These changes are well brought out in X-ray photographs. The curving of the long bones, which is such a striking feature of the disease, may be associated with actual lengthening, and the changes are sometimes remarkably symmetrical (Fig. 135). The bones forming the cranium may be enormously thickened, the sutures are obliterated, the distinction into tables and diploë is lost, and, while the general texture is finely porous, there may be areas as dense as ivory (Fig. 134).
Fig. 134.—Changes in the Skull resulting from Ostitis Deformans.
(Anatomical Museum, University of Edinburgh.)
Clinical Features.—The disease is usually met with in persons over fifty years of age. It is insidious in its onset, and, the patient's attention may be first attracted by the occurrence of vague pains in the back or limbs; by the enlargement and bending of such bones as the tibia or femur; or by a gradual increase in the size of the head, necessitating the wearing of larger hats. When the condition is fully developed, the attitude and general appearance are eminently characteristic. The height is diminished, and, owing to the curving of the lower limbs and spine, the arms appear unnaturally long; the head and upper part of the spine are bent forwards; the legs are held apart, slightly flexed at the knees, and are rotated out as well as curved; the whole appearance suggests that of one of the large anthropoid apes. The muscles of the limbs may waste to such an extent as to leave the large, curved, misshapen bones covered only by the skin (Fig. 135). In the majority of cases the bones of the lower extremities are much earlier and more severely affected than those of the upper extremity, but the capacity of walking is usually maintained even in the presence of great deformity. In a case observed by Byrom Bramwell, the patient suffered from a succession of fractures over a period of years.
Fig. 135.—Cadaver, illustrating the alterations in the Lower Limbs resulting from Ostitis Deformans.
The disease may last for an indefinite period, the general health remaining long unaffected. In a considerable number of the recorded cases one of the bones became the seat of sarcoma.
Osteomyelitis Fibrosa.—This comparatively rare disease, which was first described by Recklinghausen, presents many interesting features. Because of its causing deformities of the bones and an undue liability to fracture, and being chiefly met with in adolescents, it is regarded by some authors as a juvenile form of Paget's disease. It may be diffused throughout the skeleton—we have seen it in the skull and in the bones of the extremities—or it may be confined to a single bone, usually the femur, or, what is more remarkable, the condition may affect a portion only of the shaft of a long bone and be sharply defined from the normal bone in contact with it.
Fig. 136.—Osteomyelitis Fibrosa affecting Femora in a man æt. 19. The curving of the bones is due to multiple fractures.
On longitudinal section of a long bone during the active stage of the disease, the marrow is seen to be replaced by a vascular young connective tissue which encroaches on the surrounding spongy bone, reducing it to the slenderest proportions; the formation of bone from the periosteum does not keep pace with the absorption and replacement going on in the interior, and the cortex may be reduced to a thin shell of imperfectly calcified bone which can be cut with a knife. The young connective tissue which replaces the marrow is not unlike that seen in osteomalacia; it is highly vascular and may show hæmorrhages of various date; there are abundant giant cells of the myeloma type, and degeneration and liquefaction of tissue may result in the formation of cysts, which, when they constitute a prominent feature, are responsible for the name—osteomyelitis fibrosa cystica—sometimes applied to the condition.
It would appear that most of the recorded cases of cysts of bone owe their origin to this disease, while the abundance of giant cells with occasional islands of cartilage in the wall of such cysts is responsible for the view formerly held that they owed their origin to the liquefaction of a solid tumour, such as a myeloma, a chondroma, or even a sarcoma. Although the tissue elements in this disease resemble those of a new growth arising in the marrow, they differ in their arrangement and in their method of growth; there is no tendency to erupt through the cortex of the bone, to invade the soft parts, or to give rise to secondary growths.
Clinical Features.—The onset of the disease is insidious, and attention is usually first directed to it by the occurrence of fracture of the shaft of one of the long bones—usually the femur—from violence that would be insufficient to break a healthy bone. Apart from fracture, the great increase in the size of one of the long bones and its uneven contour are sufficiently remarkable to suggest examination with the X-rays, by means of which the condition is at once recognised. A systematic examination of the other long bones will often reveal the presence of the disease at a stage before the bone is altered externally.
Symmetrical bossing of the skull was present in the case shown in Figs. 136 and 137, and there were also scattered patches of brown pigmentation of the skin of the face, neck, and trunk, similar to those met with in generalised neuro-fibromatosis. Apart from fracture, the disease is recognised by the thickening and usually also by the curving of the shafts of the long bones. It is easy to understand the curvature of bones that have passed through a soft stage and also of those that have been broken and badly united, but it is difficult to account for the curvatures that have no such cause; for example, we have seen marked curve of the radius in a forearm of which the ulna was quite straight. The curvature probably resulted from exaggerated growth in length.
Fig. 137.—Radiogram of Upper End of Femur showing appearances in Osteomyelitis Fibrosa.
The X-ray appearances vary with the stage of the malady, not estimated in time, for the condition is chronic and may become stationary, but according to whether it is progressive or undergoing repair. The shadow of the bone presents a poor contrast to the soft parts, and no trace of its original architecture; in extreme cases the shadow of the femur resembles an unevenly filled sausage (Fig. 137); there is no cortical layer, the interior shows no trabecular structure, and some of the many clear areas are probably cysts. The condition extends right up to the articular cartilage, or, in the case of adolescent bones, up to the epiphysial cartilage.
Prognosis.—The condition does not appear to affect the general health. The future is concerned with the local conditions, and, especially in the case of the femur, with its liability to fracture; so far as we know there is no time limit to this.
Treatment is confined to protecting the affected bone—usually the femur—from injury. Operative treatment may be required for lameness due to
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