CHAPTER XIII.
SCURVY AND RICKETS.
SCURVY.
Scurvy is placed among the so-called surgical diseases, since it manifests many distinctly surgical features and is possibly of parasitic character, although this feature of its existence has not been incontrovertibly established. It is a starvation disease, its principal characteristic being that of malassimilation, accompanied by profound anemia. Well-marked cases are seen during long sieges, like that of Paris, in 1871, or during long imprisonment, as in Andersonville prison. It has certain points of resemblance to that condition of multiple neuritis met with in warm climates, and known usually as beriberi. The former is apparently due to the absence of a vegetable regimen, while beriberi is largely due to the absence of an animal regimen, nature having intended that man’s diet should be mixed, and having ordained that suffering and disease always follow confinement to one or the other.
Pathology.
—The pathology of scurvy is obscure. It has been shown that gastric digestion is seriously at fault, that there is much intestinal putrefaction, that the urine shows great absorption of toxins, that the hematopoietic function is incomplete: that scurvy is a toxemic or chronic ptomain poisoning, which may in part or at times be due to the use of tainted food. Morphological changes are, however, neither distinct nor pathognomonic. It has been described as a disease of diet and occupation rather than of race, age, sex, or season. The ease with which hemorrhagic effusions occur, the degeneration of muscles and other tissues, the frequent detachment of cartilages, can be accounted for by conditions thus summarized, for which, however, we have no minute explanation. Scurvy may so complicate various other diseases, and usually does when occurring in large bodies of men—as in armies, prisons, among convicts, etc.—that it is hard to dissociate morbid phenomena and assign to each its proper place.
Symptoms.
—The disease begins by a condition of generalized prostration, with an icteric tint of the skin, malaise, mental torpor, loss of appetite, insomnia, etc. The first recognizable or distinctive local appearances occur about the margins of the gums. Here, in the intervals between the teeth, the gums become livid, friable, and bleed easily, while the breath assumes a characteristic fetid odor. The skin becomes dry and brittle, and covered with minute prominences, which give it the popular name “goose-flesh.” These appearances are followed by local pains, diversified and sometimes excessive, and extravasations of blood in the skin and under the visible mucous membranes, causing small ecchymoses, which by themselves would be considered as simple purpura hæmorrhagica. These pass through the usual phases of extravasations, while it is made evident by pain, nodular masses, etc., and by postmortem examination, that similar hæmorrhages occur in the deeper tissues, especially in the muscles, even in the bones and epiphyses. So easily do hemorrhages occur in advanced stages that there is often external bleeding, particularly from the gums and mucous membranes, while from points thus involved pyogenic infection may proceed internally. Near the close the victim presents a picture apparently of an animated corpse, with surface discolored and mottled, often appearing bruised, with ulcerations where extravasations have failed to resolve, and where infection has occurred, possibly with epiphyses loosened, and necrosis of the bones of the extremities. In such cases death results from marasmus and sepsis.
Treatment.
—As long as the patient is not in the desperate condition just described the prognosis and outlook for treatment are promising, as all the milder manifestations of scurvy can be dispersed by suitable feeding and medication. Loss of teeth and cicatrices of ulcers leave permanent traces, but function can be restored. The purpura is but one expression of the scorbutic condition. Nearly all cases of scurvy will present purpuric manifestations, but all cases of purpura are not necessarily scorbutic. The course of treatment may be summed up in proper diet and in the administration of certain drugs. Proper diet should be prescribed at once, but administered, especially in severe cases, with extreme caution. The food selected should be given in small quantities, but frequently. It should consist in large measure of fresh fruits and vegetables, while cranberries and lime-juice figure largely among the former. Buttermilk is excellent, and cider may be allowed; also lemonade, with but little sugar.
PLATE XI
FIG. 1
Rickets. Rib. Very low power. (Gaylord and Aschoff.)
FIG. 2
Rickets. Flat Bone of Skull (Craniotabes). (Karg and Schmorl.)
For the local condition in the mouth an antiseptic mouth-wash containing a fair proportion of hydrogen dioxide is advisable. Alcoholic stimulants are called for, at least up to a certain point. Strychnine and cinchona preparations will give force to the heart’s action, and the horizontal position, for a time at least, will prevent sudden heart failure. Compound syrup of the hypophosphites, with meat preparations, will supply lacking material, while the hemorrhagic manifestations are best controlled by the fluid extract of ergot and aromatic sulphuric acid, separately or combined. Particular attention should be given to cleanliness and fresh air.
Infantile Scorbutus.
—Infantile scorbutus sometimes furnishes the surgeon with very young patients who are brought to him especially for disability of the limbs, with pain and fretfulness, leading to immobility, followed by enlargement of the lower ends of the femurs (due to subperiosteal hemorrhages) with fixation by muscle spasm; this may be followed by “spontaneous” fractures. The gums will show the same changes as are seen in adults, while subcutaneous hemorrhages and infiltrated muscles, with foul breath, ashen pallor, listlessness and apathy, and perhaps several swollen joints, will complete an unmistakable picture.
Fresh milk with orange-juice in small amounts between feedings, combined with more strictly surgical measures if needed, will secure good results in these little patients.
RICKETS.
Rickets, or rachitis, is another of the diathetic conditions, in this instance not yet considered of parasitic origin, most commonly occurring in infancy and early childhood, although its resulting lesions may persist throughout life. It is characterized by nutritional disturbances and organic irregularities.
Pathology.
—Rickets is generally referred to as “fetal” or “congenital,” according to whether the infant presents characteristic markings at birth or whether they develop later. The most marked constitutional defect seems to be in the supply of calcium salts, which leads apparently to formation of bone which has not sufficient compact tissue to make it strong. Especially along the line of junction between bone and cartilage do we see the most marked expressions of rachitic lesions. Here the cartilage is evidently actively growing, while the bone formation proceeds with difficulty, and the proportion of vascular tissue is excessive. The result is prolongations of soft vascular into the cartilaginous tissue, by which the latter becomes more or less absorbed, and this essentially interferes with ossification. In severe cases it may be lacking. At epiphyseal lines one may see a layer of osteoid tissue which is not cartilage and will not become bone. Because of its yielding nature it warps under the mechanical strain to which the bones of the extremities in young children are constantly subjected.
The obscure but unmistakable relations existing between rickets and the status lymphaticus will be referred to in Chapter XIV.
The osseous lesions of rickets differ from those seen in osteomalacia, since in the latter the softened tissue is practically decalcified bone, while in the former case most of the affected tissue has never gone so far as genuine bone formation, but is arrested in its perverted state.
The result of rickety changes in the skeleton is a thickening of the shafts of the long bones, of the outer table of flat ones, of the epiphyseal extremities of shafts, and frequently a stunting of their development, so that they do not attain their normal length. The periosteum is also affected in rickets, with the result that when the changes occur, mostly subperiosteally, there are warpings and curvings of the bone shafts, while so long as the disturbance is epiphyseal more or less abrupt curvatures and angular deformities will be produced as the result of muscle action. So marked are the changes in some instances that it has been stated that bones may even lose three-fourths of their calcium salts. When rachitic bones are so soft as to be easily cut with a knife, marked deformities occur as the result of muscular activity. (See Plate XI.)
In the extremities we see bow-legs, knock-knees, clubbing of the ends of the long bones, bending of the neck of the femur, flat-foot, club-foot, etc.; while the clubbing of the bone ends also may be well marked in the bones of the upper extremity, where, however, marked deformity is less common, because the upper extremity does not bear the weight of the growing body. In the skull the bones remain soft and yielding to pressure, with a tendency to return to their original membranous condition, and this is the condition comprised under the term craniotabes rachitica. The fontanelles always remain open for an undue time; the sutures are broad and membranous. The bones of the face grow less rapidly, giving to the face a disproportionately small size; dentition is delayed and the teeth decay easily. The upper incisors often project far over the lower.
In the thorax there are enlargements of the sternal ends of the ribs, causing a row of nodules referred to as the rachitic rosary. The ribs tend to sink in, the sternum to be protruded forward, and the deformity known as pigeon-breast becomes often pronounced. Curvatures of the spinal column, especially kyphosis, are common, and distinct degrees of lateral curvature are frequently begun as rachitic deformities, to be magnified by perverted muscle action as the child grows older. In the pelvis the innominate bones approach each other, causing the pelvic cavity to become contracted, or the sacral promontory projects too far, or in various other ways the normal pelvic diameters are so far compromised that rachitic deformities of the pelvis constitute the most common and serious obstacles to normal labor in adult women, and are frequently the cause of major obstetric operations.
While the rachitic changes in the osseous system are the most distinctive and easily recognized, numerous other organs and tissues of the body are more or less seriously compromised. Ventricular dilatation, leading to chronic hydrocephalus, is one of the common results of rachitis of the skull, which may be followed by convulsions and terminate fatally. Porencephalon and cerebral sclerosis may also ensue. Disturbances of digestion are common in rickety children—the liver may decrease in size or become much enlarged; the spleen often enlarges, sometimes to enormous dimensions. In various other parts of the body there are the same expressions of malnutrition as are met with in tuberculous disease. Rickety children perspire easily, particularly at night, when the head will often be found bathed in perspiration. They are fretful and irritable, as a rule, and difficult to control. A child with protuberant belly, due to enlargement of liver and spleen, as well as to crowding of pelvic organs, with relaxation of abdominal walls, and a contracted and distorted thorax, the skull flattened on the top, clubbed bone ends, a history of resting badly at night and sweating profusely, constitute a clinical picture of rachitis so marked that it can be recognized at a glance. Between this picture in its worst forms and the slightest deviation from the ideal type there may be met all degrees in manifestations of rickets in the children of the rich or the poor, while in adults may often be seen evidences of that which prevailed during early childhood. In order that all these features may be made out the child should be stripped and examined from head to foot.
Laryngismus stridulus is a frequent accompaniment. It may be followed by general convulsions and tetany. (See Chapter XIV.) While rickets may be a very acute disease, it is as a rule chronic, and children dying essentially from this disease die rather from cerebral or other manifestations which may be regarded as in some degree accidental. Scurvy and other nutritive disturbances may be associated with rickets.
Treatment.
—The treatment for the condition consists mainly in proper nutrition. Mothers’ milk is certainly preferable to any other, and should be demanded. If feeding must be artificial, it should be in accordance with the best precepts of modern therapeutics. Cod-liver-oil emulsions are of advantage; compound syrup of the hypophosphites is a remedy of great virtue. Minute doses of phosphorus seem to be of value—1 Mg. pro die. It is a mistake to let rickety children begin to walk or even to creep too early. They should be kept upon the back in their cribs.
The modern opotherapy of rickets includes the employment of thyroid and pituitary extracts. The dose should be graduated to the age of the patient, based upon 30 Cg. for an adult, and given thrice daily. This will not preclude the necessity for a careful regulation of diet, etc., but will constitute a valuable adjunct in treatment.
The deformities due to rickets are so numerous as to constitute a large part of those to which special or orthopedic surgery is addressed. The mechanical and operative treatment of these cases will be referred to in their appropriate place.