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.
The extremities begin to suffer at a later period than the ribs and
cranium. The opinion of Guérin, that the rachitical process begins in
the lower extremities and ascends gradually, is erroneous. It cannot
even be stated that the lower extremities are affected sooner than the
upper. There is no regularity at all; it is not even necessary that all
the osseous tissue should fall sick. But this can be taken as a fact,
that hands and feet, and particularly the phalanges, are the latest to
undergo the rachitical change. First in the line of morbid alteration
of the bones are the epiphyses, mainly of the tibia, fibula, radius,
and ulna. Their integument appears to be thin; now and then the
cutaneous veins are dilated. The periosteum of the diaphysis becomes
thick, softened, and painful to the touch and pressure, its compact
layer thin, the medullary space large, the whole bone flexible, at the
same time that the ligamentous apparatus of the joint becomes softened
and flabby. At this time babies are greatly admired and applauded for
the facility with which they introduce their feet into their mouths.
For at the same time the bones begin to curve under the influence of
the flexor muscles, which are always stronger, as they do in later
months under the weight of the body when the child begins to walk. The
curvature is not always a mere arching, but sometimes the result of
infraction (green-stick fracture), a complete fracture not being
accomplished because both of the softness of the osseous tissue and the
resistance on the part of the thickened and softened periosteum. Both
the legs and forearm bend on the external side, the resulting concavity
looking inward. The humerus bends in a direction opposite to that of
the forearm; the thigh, usually outward and forward.
The attempts at locomotion are often the causes of quite preposterous
anomalies; creeping, sliding, walking, turn the extremities in such
unexpected directions that talipes valgus, genu valgum, and now and
then double curvatures, are the results. These, however, may not always
be very marked, but there is one change in the rachitical bone which is
constant--viz. the impairment of longitudinal growth. In every case the
diaphyses remain abnormally short, and the proportion of the several
parts of the body are thereby disturbed. Chiari measured parts of the
skeleton of a rachitical woman of twenty-six years who was nine years
old before she could walk. Her height was 116 centimeters, the length
of the lower extremities 42, femur 23, tibia 15, fibula 20, humerus 16,
right radius 12.5, left radius 11, right ulna 15, left ulna 14
centimeters. In a second case the parts of the skeleton were measured
after they had {156} been extended with great care. The right arm from
the acromion to the middle finger (incl.) was 39 centimeters, the left
38; the right lower extremity from the trochanter to large toe (incl.)
39, the left 41.
The skin participates in the general nutritive disorder. It is soft and
flabby. In those infants who become rachitical gradually while proving
their malnutrition by the accumulation of large quantities of fat, it
exhibits a certain degree of consistency. When rachitis develops in the
second half of the first year or later, with the general emaciation the
skin appears very thin, flabby, unelastic. The veins are generally
large. Complications with eczema and impetigo are very frequent; where
they are found the glandular swellings of the neck and below are still
more marked than in uncomplicated cases. Circumscribed alopecia is
sometimes found (not to speak of the extensive baldness of the
occiput). It is not attended with or depending on the microsporon
Audouini, but the result of a tropho-neurosis. In the hair Rindfleisch
found fat-globules between its inferior and central third. Then it
would break, the axial evolution would cease, and the end become
bulbous by the new formation of cells.
Acute Rachitis.
There is a form of rachitis which may be, and has been, called multiple
epiphysitis or multiple periostitis of the articular ends of the long
bones. The changes which in the usual form of rachitis require months
to develop take place in a very short time. Not infrequently the
children were quite well before they were taken with this peculiar
affection. Cases have been known to occur between the fourth and
twenty-fourth months of life, and to last from two to six weeks, or
just as many months. They have been known to get well, or a few of them
terminate fatally. They are accompanied with fever and rapid pulse,
perspiration, now and then with diarrhoea, with eager or reduced
appetites. At the same time the epiphyses swell very rapidly, and are
painful. The same is true of the diaphyses and the flat bones of the
head. Many authors do not recognize this form as an independent
variety. Some call it an acute initial stage of certain cases of
rachitis, as they are not infrequently found in infants which exhibit a
very rapid growth. Some have taken it as an independent disease,
developed on the basis of a constitutional disposition; some look upon
it as a very intense acute form of rachitis; others, as an intense
growth of the osseous tissue only. Others call it an inflammation of
the bone. Some refer it to hereditary syphilis, and a few to the
influence of malaria. That the disease is epiphysitis and periostitis
there is no doubt. I do not hesitate to claim it as rachitis, for
epiphysitis and periostitis of early age not of rachitical basis are
not apt to run such a favorable course as this form frequently does.
The cases complicated with subperiosteal hemorrhages are claimed as
scurvy by Th. Barlow.
The differences of opinion would probably not have been so great if
every author had seen all the cases of the other observers. It will not
do to judge of unobserved cases by the light shed by a single case
under one's own observation. I have seen cases of acute rachitis which
were {157} the initial stages of general rachitis, and have observed
those of local or multiple epiphysitis, mainly after infectious fevers,
which were diagnosticated as such. They are, however, very uncommon.
But even without a preceding infectious fever, such as scarlatina or
more frequently typhoid fever, there are unexplained cases of rachitis
and deformity. Thus, R. Barwell had some before the Pathological
Society of London,[18] which are positive proofs that some forms of
ostitis may occur and result in the most formidable deformities without
being rachitical. A girl of seventeen years was perfectly well formed
up to the age of two and a half or three years. After that time the
deformities began to develop, and did not change after she was
thirteen, at which time the author saw her the first time.
Her left humerus measured 7-3/4 inches from shoulder to elbow;
distance 6-1/4
Her right humerus measured 7-1/2 inches from shoulder to elbow;
distance 4-1/4
Her left tibia measured 10 inches from knee to ankle; distance 7-1/4
Her right tibia measured 9-1/2 inches from knee to ankle;
distance 4-1/2
Her bones were always very brittle. When she was between nine and
thirteen she broke her arms four times and her lower limbs on several
occasions. A male patient of twenty-two years, who was born healthy and
well formed, continued thus until five years of age, when he was
attacked with a fever, after which his bones became soft and bent.
Osteotomy was performed on him, and the femora were found to be mere
thin shells of bones surrounding cavities containing great quantities
of medulla, which flowed out of the wound as oil; five ounces were
discharged at once. In both cases there appeared to be a hypertrophy of
the medulla at the expense of the bone-substance--a condition which
Barwell proposes to call eccentric atrophy. "While these subjects are
still youthful very little bone-earth is deposited, or at least remains
in the very thin layer of osseous tissue that subsists. The
relationship between infantile ostitis and extreme development of the
intraosseous fat, though well known, is still occult; neither should we
lose sight of the possibility that the softening process of ostitis may
be due to a fatty acid. Now, fatty ostitis usually occurs in epiphyses.
In these cases the shafts were affected."
[Footnote 18: _Trans._, xxxiv., 1883, pp. 203-208.]