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.