Chapter IX: Front Matter (9)

For the direct cause of rachitis Glisson looked to the inequality of nutrition by the arterial blood, and for that of the curvature of the long bones to their superabundant vascularization. He found the disease mainly amongst the well-to-do classes, not unlike a modern American writer, who declares infantile paralysis to be the result of the nervousness of the better classes of the American people! John Mayow (1761) held a disturbance of the innervation responsible; Zeviani (in the same year), improper food in general, and particularly prolonged lactation; and Selle (1791), a peculiar diathesis (acrimonia rachitica). About that time a defective nutrition with abnormal function of the lymph-ducts was looked upon as the cause of rachitis by many--by others, an undue production of acid, and the softening of the osseous tissue thereby. This result was attributed by some to the influence of milk (Veirac, De Krzowitz). Attention was directed at an early time to phosphoric acid and lime, with the view that variations in the elimination of these substances might explain the occurrence of rachitis. A large quantity of both was found in some urines (Malfatti); a superabundance of phosphoric acid was presumed to prevail in the whole system (Wendt, Fourcroy); while symptoms resembling rachitis were found in animals fed upon small doses of phosphoric acid by Caspari (1824). Chossat fed young animals on food deprived of lime, and claimed to produce softening of the bones and death, a result which was denied by Friedleben. Guérin claimed to produce rachitis by feeding young animals on meat in place of their mother's milk, a result equally denied by Tripier, who, like Friedleben, found the bones under such circumstances more liable to fractures, but not rachitical. Wildt and Weiske found the bones uninfluenced by withholding lime from food; Forster, however, and Roloff claimed to notice a marked influence, and the latter {141} stated that animals, after having been rendered sick by depriving them of lime, recovered when they were again supplied with it. Wegner, in his numerous experiments with phosphorus, found that in growing animals it increases the growth and firmness of both long and flat bones; after the growth of the animal has been completed it renders epiphyses and vertebræ denser. There is no change, however, in the relative chemical composition of those parts. He found at the same time that results similar to those caused by the administration of phosphorus were obtained when food deprived of its phosphate of lime was given. But he met with no rachitical changes proper during these several procedures. Teissier having found an increase in the urine of rachitis after the administration of lactic acid, and lactic acid having been frequently found in the urine of rachitical patients by Ragsky, Morehead, Simon, and Lehmann, C. Heitzmann fed with lactic acid both carnivorous and herbivorous animals, found the cortical layer of the bones softened and the medullary substance hyperæmic, and claimed to produce rachitis in the former and osteomalacia in the latter. Both of these assertions were denied by Tripier and Toussaint, who insist upon Heitzmann's having selected animals which have a peculiar disposition to suffer from rachitis. Again, Milne Edwards and Boussaingault found the bones softened when they withdrew both phosphoric acid and lime from the food, without restoring the bone's consistency by administering powdered bone. But, lately, Ad. Baginsky states that he produced rachitis by withholding lime, and increased the effect by introducing lactic acid. By so doing, however, he changed only the relation of the mineral to the organic substances, without interfering with the normal proportions to each other of the constituents of the ashes. Beneke, finding oxalic acid in the urine in many cases of rachitis, attributes to it the want of calcification in rachitis, and Senator suggests that what impedes the deposition of bone might be formic, acetic, and lactic acids, which are also found in the young osseous tissue.[6]

[Footnote 6: L. Fürth, _Path u. Ther. d. Rachitis_, Wiener Klinik, 1882.]

Of these statements many are uniform, others contradictory. Thus far, they are not convincing except in one way--viz. that both withholding and introducing certain ingredients, mainly lime, influence the growth of the bone considerably. This may prove nothing else but that lime is of paramount importance in the building up of bone, and that bone in the period of rapid development is amenable to a great many influences.

It is in the period of rapid development that rachitis is observed. Thus it occurs in every stage of intra-uterine and infant life. It is met with in the foetus in very early intra-uterine life; it is found as a congenital affection, continuing to develop after birth when it has originated in the latter half of foetal existence; there is, thirdly, the rachitis of early infancy; and, lastly, that of advanced infancy and childhood. Of 624 cases of rachitis enumerated by A. Baginsky, there were 256 less than a year old, 313 in the second, and 63 in the third year. After this time rachitis is rare, as far as the active symptoms of the disease are concerned. But still, a retarded form of rachitis (r. tardiva) has been described by some authors. It is said to occur about puberty, and to exhibit local changes in the bones of genuine rachitical character, but to be wanting in all the other symptoms required for the diagnosis of general rachitis. Such cases have been described {142} by C. Lucas.[7] He found it complicated, now and then, with albuminuria. The occurrence of the latter at that time of life had been referred to by Moxon.[8] The principal symptoms described by Lucas are scoliosis, talipes valgus, and genu valgum. The epiphyses were slightly thickened; there were pain in the limbs, languor, and pallor. In some of the cases there were also rachitical deformities dating from infancy. He believes rachitis of adolescence to exhibit more symptoms belonging to relaxation of the ligaments than to softening of the bones.

[Footnote 7: _Lancet_, June 9, 1883.]

[Footnote 8: _Guy's Hosp. Rep._, 1878.]

A case of rachitis of undoubtedly congenital nature has been reported by Chiari. There were but twelve teeth. There were no other alveoli, nor was there any intimation of the formation of alveoli in the shape of the jaw, which resembled very much the usual senile form of retrograde metamorphosis.

Twenty years ago I described the lesions in part of a rachitical cranium removed from an infant who lived up to her eleventh day. She was born at full term with hernia of the brain, about one-sixth of which protruded through the small fontanel. Only the cranium could be studied with regard to rachitis, and but small portions of the frontal and the anterior half of the parietal bones surrounding the large fontanel could be removed. In these few square inches of bone there were between twenty-five and thirty openings of the usual craniotabic nature, nothing but a transparent membrane being left. The bony edges of these thin portions were partly sloping off gradually, partly very steeply, and somewhat thickened. They were distributed over the whole part of the skull removed; some were found in the immediate neighborhood of the points of ossification. No recent deposits of soft rachitical bone had taken place under the periosteum. Thus, evidently, the process was of rather an early date of intra-uterine life, and had at birth run the full course of its usual development without having had an opportunity to terminate in the restitution of the normal bone.[9]

[Footnote 9: _Amer. Jour. Obst._, Nov., 1870.]

In a case reported by Dr. F. A. Burrall[10] the infant (female) was cyanosed at birth, and had a small head and feeble general development. The respiration was shrill and piping from birth, as though from congenital laryngismus; in a few days it became raucous. The post-mortem examination proved the larynx normal, with no obstructive growths. She was pigeon-breasted, and the last phalanx of her right finger wanting.

[Footnote 10: _Trans. N.Y. Path. Soc._, vol. i. p. 81.]

In the meeting of June 27, 1883, of the Société de Chirurgie of Paris, Guéniot presented a newly-born baby with well-pronounced rachitis of the extremities which had healed at the time of birth. The bones had recovered their firmness, and the characteristic deformities remained. In the meeting of December 19th he could report that the child had exhibited neither symptoms of rachitis nor of syphilis since. In regard to the latter, a very rigorous examination of the baby's whole family, made by Guéniot and Fournier, resulted in the existence of no trace of syphilis.[11]

[Footnote 11: _Rev. Mens. des Mal. de l'Enfance_, Janv., 1884.]

Kassowitz has examined many still-born infants, and also children dying at an early age, at the foundling hospital of Vienna. In a large majority of the cases he found rachitical changes in the ends of the bones. In {143} many of those who lived several weeks he found rachitis developed to such an extent that the presumption of its intra-uterine origin became conclusive. Here nothing is left but the conclusion that the cause of congenital rachitis has to be looked for in the condition of the maternal blood.

Thus, the foetal and congenital occurrence of rachitis cannot be doubted. Both forms are represented in literature. Neither requires the presence of rachitis in one or both of the parents. But the cause of the intra-uterine disease has not been found. Perhaps a disease of the mother with considerable nutritive disorders or a defective placentar supply may be found responsible. The foetal form runs its course long before the normal termination of pregnancy; the congenital may have run its full course at birth or complete it afterward. The bones are found of characteristic nature, the diaphyses suffering more than the epiphyses; even a rachitical pelvis has been met with by Fischer. Early foetal rachitis is probably dependent upon a defective development of the very first cartilaginous deposits and the first osseous nuclei; thus, many of the congenital synostoses find a ready explanation. Besides these, abnormal circulation is accounted for. For periosteal proliferation at that early period contracts the foramina carrying the blood-vessels, and, while interfering with the size of the bones, the foramen magnum also. Thus, a certain class of cretinism appears to be due to foetal rachitis, mainly of the base of the cranium, which results in early ossification of the synchondroses, particularly of the sphenoid bone. But lately I have seen a case of this description, which, however, had not terminated at the time of birth. For after birth the rachitical process developed further, and in addition to the rachitical deformity of the base of the cranium there were afterward thickening of the epiphyses, pigeon breast, and thoracic grooving and flattening.

Rachitis is found in city and country, less on mountains than in valleys. Still, it is met with at elevations of two thousand feet. In the tropical regions it is almost unknown. Why it should have been considered quite a new disease in England but a few centuries ago, or whether it did not exist before that time, it is difficult to say. It is certain, however, that deformities have been described in antiquity which we are accustomed to attribute to rachitis.

As the disease is one that occurs during the period of rapid growth, and is a developmental disease, everything that interferes with normal growth and development is apt to change physiological functions into pathological conditions and to produce rachitis. In the pregnant mother her ill-nutrition and the defective cell-material used in the building up of the embryo and foetus, or a defective placenta, may come in for the explanation of foetal and congenital rachitis, although the case of Klein's, who reports twins, of which one was normal and one rachitic, is rather difficult to explain on that basis only. Even rachitis of early infancy is not easily accounted for otherwise, for its first symptoms show themselves at a very early period; thus constipation, adiposity, and afterward craniotabes and thoracic grooving.

The common form, and that which is the usual subject of the text-books and monographs, has, however, in most cases a well-marked preparatory stage in the shape of diseases or ailments reducing sanguification {144} and nutrition. Some cases are ushered in by, or follow the course of, acute exanthems or acute gastric disorders or the presence of entozoa. A larger number appear to result from insufficient oxygenation resulting from lung diseases, with a long chronic ailment following the acute stage. Even acute pneumonia, with its direct influence on general nutrition, stands often for the proximate cause of rachitis. Bad air alone, even swamp air, does not appear to be a sufficient cause. When it seems so, it is complicated with the main cause of rachitis; that is, bad, insufficient, improper food, with its immediate result--viz. intestinal catarrh. Cow's milk, particularly when acid, starchy food administered too early or in too large quantity or too exclusively, early weaning followed by improper artificial food, insufficient mother's milk or such as is either too thin or too caseinous, lactation protracted beyond the normal limit,--may all alike be causes of intestinal disturbances and rachitis.

Is rachitis hereditary? A number of women who were rachitical themselves have been known to have rachitical children. But it has been said that the process runs its full course during infancy, and that therefore a direct inheritance of rachitis from mother to child is an impossibility. Still, we must not forget that the consecutive conditions of the parents may, or will, influence the general condition of the infant and result in similar disturbances. No rule, however, exists. Dyscrasic parents may have healthy children, and healthy parents sickly or dyscrasic ones. But the probability is greater that diseased children should come from dyscrasic parents than from healthy ones. Tuberculosis in the parents has frequently been accused of being the cause of rachitis in the infant--not directly, but in consequence of general impairment of the tissues. Gout has also been accused of being the cause of rachitis, but it is a peculiar fact that the poor have but little gout and a great deal of rachitis. In all of these cases it is better to look upon rachitis as only one of the forms of general mal-nutrition, and to speak of inheritance of the disposition rather than of the disease. Thus it was that about the end of the eighteenth century Portal spoke of scrofulous, syphilitic, scorbutic, rheumatic, arthritic, and exanthematic rachitis. Particularly has syphilis been accused of being the main cause of rachitis by some, and even the only cause by others. Thus it was looked upon by Boerhaave. In modern times Parrot maintained, from 1872 up to the time of his death, which occurred recently, that every case of rachitis is of syphilitic origin. As his proof he relied mainly on the condition of the teeth and the bones. But those appearances in the teeth, the thin and ragged edges, the friability and the grooving, either horizontal or vertical, which have been considered characteristic of syphilis by Hutchinson and others, have no such dignity, and moreover they are not observed in the temporary teeth at all, but in the permanent only; the rachitical softening of the bones also is not found in syphilis at all. Particularly are there no curvatures in syphilis and no infractions. It is true that marasmus is found in both rachitis and syphilis, but it is met with in all sorts of diseases. The changes in the bones of syphilis are found at birth; in rachitis they usually develop in later months. When a baby is syphilitic and rachitic at the same time, the syphilis may last very much longer than the rachitis, which meanwhile has healed. The internal organs in rachitis do not exhibit any such {145} changes as are known to occur in very many cases of syphilis. No gummata are ever found in rachitis, and the interstitial inflammation of the internal organs in syphilis is not met with to the same degree in rachitis. What Parrot claimed as a desquamative syphilide of the tongue--that is, red insulated spots, denuded of their epithelium, small in the beginning, later extending backward and increasing in size--is by no means always syphilitic, but is found in a great many cases where there is no suspicion of syphilis. It is mainly Kassowitz and Bouchut who have taken the stand against Parrot. The former, taking rachitis for a peculiar inflammatory process, admits that syphilis can be one of the causes. The latter directs attention mainly to the fact that by changing food in certain ways rachitis may be produced in dogs, but that they cannot be made syphilitic. There is no doubt, however, that syphilis may give rise to rachitis by its general influence on nutrition, and in this fact lies the key to the connection of great nutritive disorders with each other. Syphilis will undoubtedly change nutrition to such an extent as to result in rachitis. Rachitis will affect the glands; the caseous and suppurative degeneration of the glands will lead to metastatic processes, to acute tuberculosis, and so on.

Malaria been claimed as the main cause of rachitis by Z. Oppenheimer,[12] or, rather, rachitis is presumed by him to be the form in which malaria makes its appearance in young infants. After disposing of other alleged causes of rachitis, none of which is proved to give rise to every case, and referring to the anatomical belief that the peculiar hyperæmia and inflammation of rachitical bones is created by the embryonic condition of the growing osseous tissue, he points to the prodromi, amongst which he emphasizes chronic diarrhoea and the nocturnal crying. The latter, with its perspiration and subsequent sleep, he claims as evidence of malaria, and as a substitute for the intermittent neuralgia of adults, the more so as he believes he finds the spleen tumefied. The persistent diarrhoea of these infants is said to be paroxysmal--to take place in the morning, contrary to what is seen in the usual form of intestinal catarrh; the discharges are said to be serous, not tinged with bile; the appetite to be good through the rest of the day; the weight of the body not to be lessened, but anæmia to develop gradually, and fever to occur occasionally. In other cases infants have cold hands and feet and blue lips toward evening; the skin is pale, the spleen enlarged; otherwise there are perhaps no symptoms, but the infants try to get uncovered, and have an increase of temperature of from 1° to nearly 3° F., and a perspiring head in the morning. After a while the rachitical symptoms belonging to the bones and the general system become apparent. After all of the author's ingenious and emphatic assertions and deductions, it becomes evident that malaria--in the severe forms in which it has been found by Arnstein, Browicz, and Henck to cause bone diseases--may give rise to rachitis, but it is also clear that he tries to prove too much. The long series of attempts at proving that every form and case of rachitis depends upon a single and uniform cause have proved futile. The physiological hyperæmia of the bones and the rapid growth of all the infant tissues are shaped into the complex ailment which we call rachitis by more than a single disease or a single nutritive disturbance.

[Footnote 12: _D. Arch. f. klin. Med._, xxx., 1881.]

{146} SYMPTOMS.--Before entering upon a more accurate and elaborate enumeration of the symptoms of rachitis, I mean to dwell upon peculiar differences which take place according to the age in which the disease makes its appearance. Very young babies--that is, infants of a month or two--develop rachitis in such a manner that many cases are overlooked until it is too late to relieve them in time. This occurrence takes place when there are no prominent causes, such as diarrhoea or other nutritive disorders, nor any premonitory symptoms. Such infants appear to be perfectly well; they have the average weight, and even more; they have plenty of adipose tissue, and look well. The only anomaly appears to be an undue degree of paleness. Without pain or flatulency they are constipated. This constipation is not congenital, as it always is when the colon is unusually long even for an infant, and when the sigmoid flexure is of double or even treble length, but makes its first appearance about the end of the first or the beginning of the second month. It is relieved only when the increasing muscular power of the intestine results in more effective peristalsis. The second symptom is the thoracic groove, to which I shall allude later, and a gradual thickening of the costo-cartilaginous junctures, with or without periosteal pain on pressure. About the same time the cranial softening, craniotabes, with its hyperæmia and perspiration of the entire scalp, and baldness, and the first symptoms of maxillary rachitis, become perceptible. During all this time the epiphysial swellings and the diaphysial curvatures develop but very slowly; but at a very early time chronic bronchial catarrh, with a loose cough, begins to be troublesome. When rachitis begins at a late period--say, about the sixth or eighth month--the aspect of the case is different. The infant has suffered before either from bronchitis and broncho-pneumonia, or in most cases from indigestion and intestinal catarrh. There is some degree of emaciation; the skin does not fit the limbs, as it were--is loose, thin, flabby, and rather dry. The tendency to diarrhoea continues to prevail. The epiphyses, particularly of the lower extremities, are thickened at an early time, curvatures of the tibiæ become apparent, and all the rest of the bones participate in the process, with the exception, sometimes, of those of the head.

The head, however, is liable to exhibit symptoms of rachitis at a very early period of life. It is large, or appears to be so,[13] mostly for the reason that the face is proportionately small. The forehead is large, the frontal protuberances very prominent, as are also those of the parietal bones. Thus, the head is more or less square. Dilated veins are visible in and through the pale skin; there is but little hair, on the occiput less than on the rest of the head. Sometimes the occiput is quite bald, the hair having been rubbed off on the pillow. The scalp feels warm, except during perspiration. The latter is very copious, particularly on the occiput--to such an extent, indeed, that the pillow is drenched--and will remain so for months. The sebaceous follicles are often still larger and more numerous than they normally are at that age, and seborrhoea is {147} often, though not always, met with. The sutures are wide, sometimes one or two centimeters; the posterior fontanel remains open; the large anterior fontanel is very large, being sometimes several inches long and wide. The pulse is felt very distinctly through it. The systolic cerebral murmur, which was first found by Fisher of Boston in 1833, and considered to be a positive symptom of rachitis (which certainly it is not, as it is found in almost every healthy baby with a patent fontanel), is very audible. The fontanel and sutures remain open for a long period. Instead of closing, as they do normally at the fourteenth or fifteenth month, the former ossifies about the end of the second or third year, or later. Gerhardt reports a case in which it persisted to the ninth year. The cranial bones appear to be thin, and give way under the pressure of the finger. Ordinarily, it is true, the cranial bones of every baby, even if perfectly healthy, are movable under pressure, but they are so only along the sutures, where they may retain this mobility, in some instances, a long time. Indeed, it appears that sometimes about the middle of the first year the occipital bone becomes thinned out in apparently quite healthy children. Moreover, even in the skulls of infants who were taken to be in good health small defects in the bones were found (Friedleben), with no uncomfortable symptoms at all. Therefore it is rather difficult to draw the exact boundary-line between the healthy and the morbid condition; thus it is possible that some of those cases which exhibited apparently morbid local changes without morbid symptoms may not have been diseased after all. In those, however, in which rachitis is really developed in the cranium a peculiar condition is found. In the posterior half or third of the parietal bones, either the right or the left side being more marked, there are in the tissue of the bone distinct spots in which the osseous material is not only thinned out, but has entirely disappeared. In fact, the bone is perforated, the edges of the holes being rather steep, sometimes slightly thickened, and the scalp separated from the brain only by a thin, transparent membrane, the remnant of the periosteum. These holes can be easily found through the integument. The finger, though ever so gently pressing down upon it, moves the cranium, if any be left, before it; the bone feels like paper, and the sensation as if it could be easily broken through is quite distinct and embarrassing. Such perforations are usually quite numerous; from five to twenty or more can often be counted. They are surrounded by normally hard bone, and thereby can be recognized from the flexible part of the cranium extending along the sagittal and lambdoid sutures. Where these results of rachitical softening, craniotabes, are most prominent--that is, on the part on which the infant is mostly reclining--the bone is flattened, and may remain so for life, though in the majority of cases the asymmetry will disappear. The flattening and perforations result from the same causes--viz. softening of the bones and pressure upon the bone between the pillow outside and the brain inside. With it go, hand in hand, thick rachitical deposits under the hyperæmic periosteum of other portions of the skull. Where craniotabes is largely developed on the occipital portion, the frontal and the parietal bones (in their anterior halves) are usually thus thickened. A cross-section with a knife will reveal a diameter of the new osteoid material between the periosteum and bone of one-half to one centimeter in thickness. It is very hyperæmic--even more so than the bone itself, {148} which, when cut into, exhibits an unusual amount of blood. Sometimes the deposits are still larger, and are apt to change the appearance and weight of the skull considerably after recovery has taken place and eburnation and sclerosis have taken the place of the normal osseous tissue.

[Footnote 13: Boötius (1649), quoted by Haller (_Bibl. Med. pract._, 1779): "Infantibus caput grandescit, reliquum corpus contabescit, ossa in articulis tument, dextrum hypochondrium tumore æquali prominet; hoc malum multis millibus infantum molestum est" ("The infant head grows large, the rest of the body emaciates, the articular bones swell, the right hypochondrium is raised by a uniform tumor; this malady is a sore affection in many thousands of infants").]

Such a case of rachitical cranial sclerosis I have described in the _Amer. Med. Monthly_ of 1861. It was, however, by no means a mate of the case related by E. Huschke. The latter skull was that of a girl of seventeen years, and weighed 4117 grammes instead of the normal weight of 600 grammes. The medullary (Havers') canaliculi were large and very numerous on the surface, narrow and very few in the interior of the sclerotic bones, and the osseous canaliculi were more spherical and irregular in site and shape. The chemical composition was also abnormal, phosphate of lime being 65.59, carbonate of lime 11.12, sulphate of magnesia 1.14, cartilage and fat (very little), etc. 22.15 per cent. No fluorate of lime was found. Most of the bones were exceedingly hard, but fragile when tried in small pieces; very white inside, yellowish on the surface, the latter color being the remnant of extravasated blood or other pigmentous matter. Another skull, in Huschke's possession, and moderately sclerotic, weighed (lower jaw excluded) 1075 grammes; a third, in the museum of the University of Jena, is that of a young baboon,[14] in which all the bones covering the hemispheres had become sclerotic.

[Footnote 14: Baboons suffer from rachitis very extensively. In the _Transactions of the Pathological Society of London_ (xxxiv., 1883, pp. 310, 312) I. B. Sutton gives the description of two baboons, one of which was six months, the other one year and six months old, when they died. The careful description of the specimens exhibited leaves no doubt as to the rachitical nature of the changes in both the periosteum and the tissue of all the bones of the body.]

Of undoubted total cranio-sclerosis Huschke reports but ten cases--those of Malpighi (1697), Cuvier (1822), Ribalt (1828), J. Forster and Bojanus (1826), Ilg (1822), Kilian (1822), Otto (1822), Vrolik (1848), Albers (1851), Huschke (1858). The disease does not affect the auditory bones, the condyles of the maxillary and occipital bones, nor the styloid process of the temporal bone. It is recognizable in the posterior part of the cranium and basis cranii, but affects mostly the bones of the face and the frontal, parietal, and cribroid bones. Thus, the disease takes its origin in the anterior portion of the skull, particularly in the superior maxilla, and proceeds upward and backward, terminating in the basis cranii in the neighborhood of the infundibulum and appendices. But two of all the cases were observed during life. In all the disease was traced back to early life. The chemical composition of the bones was greatly changed in all. Instead of the normal proportion of earthy to organic material = 2.1 (or 1.5):1, it was from 3.5 to 4.4:1. Particularly the carbonate of lime was greatly increased.

The brain and its meninges participate, in many respects, in the changes worked by rachitis, and mainly in the abnormal vascularization of the bones. They are very much congested, and succulent. A section through the brain shows a great many large and small blood-points. This hyperæmia may give rise to over-nutrition, which assumes the character of real hypertrophy of the brain. When that hyperæmia, however, becomes excessive, effusion will take place into the cavities, the tissue of the arachnoid, and the substance of the cerebrum, which latter looks {149} peculiarly brilliant, elastic, and sometimes white, in consequence of the blood-vessels being emptied by the pressure on the part of the enlarged mass of the cerebrum upon the blood-vessels. Thus, instead of cerebral hyperæmia there may be anæmia. Every form of hydrocephalus may follow the rachitical process. Afterward, when the craniotabes has healed, the secondary effusions will generally also disappear, but not a few cases of hydrocephalus may be traced to rachitis occurring during the first half year of life. When that occurs, the intellectual faculties may suffer, while, on the contrary, complete recovery not infrequently exhibits an unusual degree of mental development, for the same reason which improves the chances of the development of the bone. The degrees of physiological and pathological nutrition and over-nutrition are very variable in their nature and results.

This condition of the cranial contents is not the only one brought about by rachitis. The softness of the cranial bones permits a direct pressure on the brain. The side on which the infant for the most part reposes gets flattened, and the brain is also compressed. The skull consequently bulges out in the opposite direction. This anomaly, as stated above, is sometimes visible through life, though in the large majority of cases after recovery from rachitis has taken place this asymmetry will gradually disappear. Before that can occur, however, the infant is liable to suffer from the rachitical changes. Convulsions are by no means rare. Vogel has, however, been able to produce an attack of convulsions by pressing upon the softened spots of the cranium. Permanent or temporary contractures of the fingers and toes I have seen in several instances. Gerhardt looks upon rachitis as one of the causes of tetany.

A frequent symptom of the cerebral changes which take place during, and in consequence of, craniotabes is the crowing inspiration, or laryngismus stridulus, of infants. It may be mild or severe. The mild form is very frequent, and consists in the occurrence of a shrill inspiratory sound while the baby is either quite placid or excited or crying. It is frequently overlooked entirely, is usually overcome after a number of months, and gives rise to serious trouble in but very few instances. The severe form is of a different nature. While the baby is awake or asleep, without any premonitory symptoms, while playing or crying, placid or excited, all at once respiration will cease. This will take place, usually, after expiration. The limbs are hanging down, as it were lifeless, the face turns pale, then purple, and slight convulsive twitching may set in for ten or twenty seconds. There appears to be a complete paralysis, and death from apnoea seems to be imminent. All at once, a long, deep crowing inspiration will be heard, respiration will commence again, and the whole terrible attack is overcome. It may return a number of times every day, or sometimes not for several days, during a period of many weeks or several months. The attacks which set in after inspiration are apt to be more dangerous. In such an one, but also in the other kind which sets in after the expiratory movement, death may occur suddenly, or the attack may be followed by a convulsion which may terminate fatally like any other eclamptic seizure. In this manner it is that the majority of cases of rachitis perish which terminate fatally during the active progress of the morbid process. In this connection, however, it may be well to add that craniotabes is not the only cause of laryngismus, particularly when the {150} latter is found in the second year of life, or even later. But almost every case, without any exception, which is observed during the first eight or nine months is due to that very cause; and a good many cases occurring later, when the craniotabic bones have become normal, arise from the effects, either meningeal or encephalic, of the rachitic process. Still, complications of craniotabes with a large size of the thymus gland may occur, and enlargements of the tracheal and bronchial lymphatic glands are quite frequent, as we shall see below.[15]

[Footnote 15: Z. Oppenheimer prefers the name rachitic asthma in place of laryngismus, and suggests an explanation of the symptoms from a strictly anatomical point of view. If not correct, it is at all events interesting, as everything this ingenious writer proposes. He points to the ligament situated between the spinæ intrajugulares of the temporal and occipital bones, which, as long as it is of normal consistency, separates the jugular vein from the pneumogastric nerve. As it is covered with periosteum and dura, it is apt to ossify, and forms an osseous partition in the foramen jugulare, which participates in all the changes taking place in the periosteum. As this becomes softened and succulent, so will the ligament, either on both sides or on either. Its influence on the neighborhood depends on its size or succulence (as also on the difference in width of the foramen jugulare or lacerum, which corresponds with the difference in size of the transverse sinuses). The irritation of the pneumogastric is perhaps easily explained thereby, but in very exceptional cases only the accessory nerve would be affected. As, however, the latter controls the sterno-cleido mastoid and trapezius, and also the laryngeal muscles, and is apt to provoke cardiac paralysis during diastole, the occurrence of sudden death would be best accounted for.]

While the size of the cranium is normal, or sometimes more than normal, the face undergoes some changes which result in absolute or relative diminution of size. These depend mostly on a reduction in the volume of the jaws. Glisson knew of it, and therefore looked for the cause of rachitis in the process of dentition. Now, both maxillæ are liable to become rachitical at an early date, as early indeed as the bones of the cranium. Rachitical deposits and softening take place in them very generally. The lower maxilla is flat anteriorly, it loses its rounded outline, is shorter in longitudinal direction, while the rami are thick and clumsy; the whole bone is shorter than normal, and sometimes asymmetric. Its changed appearance is greatly due to the effect the muscles, with their powerful insertions, produce on the softened bone; mainly the masseter, also the mylohyoid, which draws the lateral portions inward, and the geniohyoid, which pulls at the central portion. Of the latter, the lower portion is drawn out, the inner and the alveolar part inward. Thus, the teeth, mainly the incisors, of the lower jaw are turned inward to such an extent that, as those of the upper look outward, the two rows of teeth do not touch but cover each other. Besides, the periosteal proliferation around the alveoli is excessive, sometimes so much so as not only to crowd the teeth into irregular positions, but even to absorb and annihilate alveolar processes in the course of the morbid changes. The cases in which the number of teeth are actually diminished by rachitis are not at all rare. In the superior maxilla the last-described anomaly is also observed. Periosteal thickening is mainly noticed about the intermaxillary bone--sometimes to such an extent that above and behind it a considerable impression takes place. The shape of the upper jaw is more spherical than normal, and the cheek-bones become very prominent.

The belief that maxillary rachitis is now and then met without any other symptom of rachitis I do not share. What I said of craniotabes is also valid in regard to this form.

{151} Irregular teething is a constant companion of maxillary rachitis, but is also present where the latter is not well, or not at all, marked. As a rule, the first teeth protrude late, about the ninth or tenth or twelfth month. That the first year and more should elapse without any tooth is of frequent occurrence in rachitis. Cases in which the first teeth do not come before the second year is completed are not very uncommon; in some there are none even when the child is much older. In most cases the retardation of dentition goes hand in hand with very marked retardation in the development of the rest of the bones and in the closure of the cranial fontanel. But not in every case of rachitis is there a retardation in the process of teething. In some a few teeth appear at the regular period (at the completion of the seventh or eighth month), or even at a very early age (in the fourth or fifth month); after which there is an interruption in the protrusion of teeth for an indefinite period. Evidently, the period in which rachitis is developed exerts its influence on the teething process. When it exists at a very early age, it will retard teething until recovery takes place. Still, it is possible that a moderate amount of periosteal and osteal hyperæmia and over-irritation matures the teeth abnormally. In all those cases, however, in which rachitis does not occur before the second half of the first year, the first teeth will appear at the normal time, and a long period will follow in which no teeth at all will make their appearance. Then, again, when the whole process comes to a standstill, and recovery takes place with solidification of the bones, and even eburnation, the teeth will come in rapid succession. Whether they will, as is frequent, decay almost as soon as formed, or whether they will be unusually hard, solid, and yellowish, depends on the stage of the disease in which they made their appearance, and on the complications aggravating the case. Of very grave import in this respect are digestive disorders before and during the course of the disease.

The vertebral column suffers also. In the normal infant it is straight, but in the rachitic it exhibits a kyphotic deformity very soon. When such a baby of three or six months is sitting up, the middle portion of the back is protruding, as in Pott's disease. In almost every case, however, this kyphosis is but apparent and the result of muscular debility. In order to arrive at a diagnosis at once, it is sufficient to place the patient on his face and support the head, and raise the lower extremities and pelvis in the air. If the kyphosis is but functional, the prominence disappears at once. By nothing can the muscular insufficiency of early rachitis be better demonstrated than by this little experiment. But actual deformity is also found in rachitis. It softens both the vertebræ and intervertebral cartilages, and either their anterior or posterior portion may be irregularly developed, and be either too high or too low. Besides, the articulating surfaces are sometimes too convex. Thus the causes of both kyphosis and scoliosis are amply furnished, and complications of the two are quite frequent, and the deformities resulting therefrom quite formidable. Scoliosis is mostly to the left; kyphosis generally complicated with lordosis, and sometimes the vertebral column exhibits a spiral shape.

The ribs of the convex half are prominent and divergent, those of the concave side flattened and parallel. The two halves of the chest are therefore very unequal indeed. Muscular traction, atmospheric pressure, {152} the elastic traction of the lungs, the presence of pulmonary complications, and the pressure from below on the part of the enlarged viscera of the abdominal cavity, come also in for a considerable share in the completion of the deformity.

The ribs and the sternum aggravate it considerably. Even without any affection of the vertebral column they suffer seriously from the general affection. The manubrium is thickened and drawn inward, the ensiform process protuberant, the sternum often swelled and painful to the touch. The ribs are sensitive to the touch on one or both sides. The child cries when taken up or when fearing to be taken up. The costo-cartilaginous junctures are thickened, mainly so from the fourth to the eighth ribs. The insertion of the diaphragm becomes soon perceptible by a deep groove around the chest. The anterior portion of the ribs is flattened, posteriorly they are inserted at acute angles. Thus the intrathoracic space becomes narrow, the sternum with the costal cartilages is pressed forward (pigeon breast, pectus carinatum), the thorax is deprived of its elliptical shape and becomes triangular, the dorsal aspect being flattened, and the distance between the vertebral column and the sternum increased. Below the diaphragmatic groove the thorax expands, the liver and other abdominal organs crowding the ribs outward. All sorts of changes are experienced by the ribs in these conditions. Parts of them are flattened, parts undergo infraction, parts are even concave; they are bent and twisted, now and then to such an extent as to turn the concave side out, the convex surface in. In addition to all this, the scapula is big and clumsy and protuberant, the clavicle considerably bent and frequently infracted, and not rarely covered with genuine callus.

That the respiratory and circulatory organs must suffer from such anomalies, though they be not excessive, is certain. The heart is crowded by the flattening of the ribs and the contraction of the thoracic cavity. Its beat is visible over a large surface, and its percussion dulness is extended over its normal space, though no enlargement have taken place. This, however, is very apt to occur after some time by overexertion. The latter is increased by the condition of the respiratory organs. The ribs being flexible, the chest contracted and compressed, the diaphragm raised, the respiratory muscles feeble, respiration is insufficient, even without the presence of any further complications; thus dyspnoea and a certain amount of cyanosis are frequently met with in consequence of the anatomical changes only. In addition to this, there is from the beginning a tendency to catarrhal and inflammatory conditions. Even without any deformity the rachitical process is accompanied from an early time with bronchial and tracheal catarrh. A chronic cough in an infant, with very little or no fever, disappearing and returning, mostly with copious secretion--which, however, is swallowed as soon as it reaches the pharynx--rouses the suspicion of general rachitis. It is often complicated with extensive dulness over the manubrium sterni, due (to rachitical thickening of this bone and) mostly to the persistence of a large size of the thymus gland; and also with enlargement of the bronchial and tracheal glands, the latter of which are often accessible to recognition by percussion. They are to be looked upon as a frequent occurrence in rachitis, though no associated diseases leading to their enlargement have been noticed. They and the chronic tracheo-bronchial {153} catarrh are closely dependent upon each other. They are each other's both cause and effect. Neither of them, however, remain uncomplicated. Catarrh grows into broncho-pneumonia, with frequent returns. Atelectasis, interstitial pneumonia, dilatation of bronchi, and pulmonary consumption are often traceable to such apparently slight catarrhs, which, when not recognized as depending on their constitutional cause, cannot be removed. Nor are the cases of miliary tuberculosis, resulting from caseous degeneration of rachitical glands, very exceptional.

The anatomical changes in the abdominal viscera may be due to the preparatory diseases or the complications of rachitis; but, at all events, the abdomen yields a number of changes visible through the whole duration of rachitis. It is very large; its size is due to the contraction of the thoracic cavity and the downward pressure of the chest-wall upon the contents of the abdominal cavity. It is also due to the changes wrought by rachitis in the pelvis. Softening of bones and synchondroses, torsion, the weight of the trunk, and the pressure of the femora from below produce the change of the pelvis so well known and much feared in the parturient female. The promontory and sacrum are pushed in, the arcus pubis is large, the pelvis asymmetric; the small pelvis is contracted, the large pelvis broader. Thus, the small pelvis has no room for viscera, which, then, are crowded upward. The digestive disorders which gave rise to, or formed the first stage of, rachitis result in the accumulation of gas; the scrobiculus cordis is greatly expanded. The liver[16] is large, congested, and in fatty degeneration. The latter is the more frequent the more a certain degree of fatty condition is a normal attribute of every infant liver. When the liver is found but small in post-mortem examination, it is so because of the general anæmia and emaciation. Sometimes it is amyloid, as are also the spleen (mostly hyperplastic only), the kidneys, and the arteries of the intestines in many instances.

[Footnote 16: Dr. Norman Moore presented a cast and drawing to the Pathological Society of London (_Trans._, vol. xxxiv., 1883, p. 185) showing how considerable may be the digressions of the diaphragm and local pressure upon the liver in a case of rickets. Three large beads caused as many projections from the under side of the diaphragm, and corresponded with local thickenings of the capsule of the liver, probably produced by the continued pressure through the diaphragm of the beads, which were on the seventh, eighth, and ninth ribs, and the largest of which was equal in size to a hazel-nut.]

The alimentary tract is the seat of many changes recognizable during life. The tonsils are often large. The tongue is seldom coated to an unusual degree. On it are found little islands, red, marginated, deprived of epithelium. They will increase in size and number and extend backward. They will heal and reappear. They are by no means syphilitic, as Parrot would have it, and correspond exactly with the erosions near the solitary glands and those of Lieberkühn in the intestinal part, which mean nothing else but a nutritive disorder of the epithelia, and give rise to nothing worse than incompetency of absorption in that locality and abnormal secretion. The stomach is in a condition of chronic catarrh, sometimes dilated. Acid dyspepsia is frequent. Anorexia and bulimia will alternate. Feces contain an abnormally large amount of lime. Diarrhoea and constipation will follow each other in short intervals. The former owes its origin to faulty ingesta or chronic catarrh; the latter, sometimes to improper food, but more generally to muscular insufficiency. {154} This condition has not been estimated at its proper value. Besides myself,[17] nobody but Bohn has paid the attention to it which it deserves. Here, again, I have to insist that rachitis is a disease of the whole system, and not exclusively of the bones. Indeed, the muscular system is amongst the first to suffer. In the same way in which the voluntary muscles are not competent to raise and support the head or to allow a baby to sit up without a functional kyphosis, the involuntary muscles of the intestine are too feeble for normal peristalsis. The infant of a month or two months of age may have had normal and sufficiently numerous evacuations; gradually, however, constipation sets in; the feces become dry, but are perhaps not much changed otherwise. If no other cause be apparent, the suspicion of rachitical constipation is justified. Seldom, however, after it has lasted some time--and only after some time has elapsed relief will be sought--it will remain alone. Other symptoms of rachitis will turn up and the case be easily recognized. This constipation is an early symptom, as early as thoracic grooving or craniotabes. Very often it precedes both--is, in fact, the very first symptom--and ought therefore be known and recognized in time.

[Footnote 17: _Jour. Obst., etc._, Aug., 1869.]

The kidneys have been mentioned above. They are often found rather large. Though the fact has been alluded to before, I will here again state that it has always been the general impression that the amount of lime eliminated in the urine of rachitic children is excessive. The reverse of that is true. Seemann and Lander have proved beyond dispute that in most stages of rachitis there is less than the normal amount of lime in the urine. Thus, the theory that lime is eliminated by an excess of acids in the blood is proven to be incorrect. But it is a fact that the rachitical bone contains a proportionately small amount of lime. The conclusion is, then, that its introduction must have been diminished. On the other hand, every article of food contains a large amount of lime, which might be introduced into the circulation if digestion be not at fault. The fact is, that a large amount of lime introduced is not utilized, and is eliminated with the feces.

In connection with these facts the following will be found very interesting. It has been found by Bunge that when potassium, with the exception of chloride of potassium, meets chloride of sodium, the two will exchange their acids, so as to form chloride of potassium and phosphate of sodium. They will be found in the blood also, will be eliminated as such, and result in a comparative absence of chloride of sodium from the serum of the blood. Now, comparative absence of chloride of sodium diminishes the possibility of the development of hydrochloric acid. Thus, it is not a surplus of acid, but a lack of hydrochloric acid, which results from such chemical combinations. If such be the case, calcium salts are not absorbed sufficiently. Thus, they will appear in the feces, and not even be absorbed in the intestines, because of the alkalinity of the intestinal secretion, by which the lime cannot be dissolved. The more lime, then, is introduced under these circumstances, the greater the incumbrance to digestion.

The correct proportion between chlorine, phosphorus, potassium, and sodium is certainly exhibited in woman's milk. There is lime enough in even the poorest article of that kind. But indigestion brought on by {155} woman's milk in a disordered condition or by any other cause will prevent the absorption of lime when a superabundance of phosphorus and potassium disturbs the formation of hydrochloric acid. In these cases not only the development of the bones, but also that of the muscles, is disturbed. The latter is of great importance in regard to circulation, because a large part of the circulation depends on the pressure on the part of the muscular fibres exerted on the small blood-vessels. These facts have been the reason why I insist upon the addition of chloride of sodium to the food of infants and children, particularly those who are fed on cow's milk; for cow's milk and vegetables contain a relative superabundance of potassium compared with sodium. Even adults will find cow's milk very much more digestible by adding table-salt to it.