gummatous disease causes ulceration, which, beginning in the mucous
membrane, spreads to the bones, and being complicated with septic
infection leads to caries and necrosis. In the nose, the disease is
attended with stinking discharge (ozœna), the extrusion of portions of
dead bone, and subsequently with deformity characterised by loss of the
bridge of the nose; in the palate, it is common to have a perforation,
so that the air escapes through the nose in speaking, giving to the
voice a characteristic nasal tone.
_Syphilitic disease of the tibia_ may be taken as the type of the
affection as it occurs _in the long bones_. Gummatous disease in the
periosteum may be localised and result in the formation of a
well-defined node, or the whole shaft may become the seat of an
irregular nodular enlargement (Fig. 132). If the bone is macerated, it
is found to be heavier and bulkier than normal; there is diffuse
sclerosis with obliteration of the medullary canal, and the surface is
uneven from heaping up of new bone--hyperostosis (Fig. 131). If a
periosteal gumma breaks down and invades the skin, a syphilitic ulcer is
formed with carious bone at the bottom. A central gumma may eat away the
surrounding bone to such an extent that the shaft undergoes pathological
fracture. In the rare cases in which it attacks the articular end of a
long bone, gummatous disease may implicate the adjacent joint and give
rise to syphilitic arthritis.
[Illustration: FIG. 131.--Syphilitic Hyperostosis and Sclerosis of
Tibia, on section and on surface view.]
_Clinical Features._--There is severe boring pain--as if a gimlet were
being driven into the bone. It is worst at night, preventing sleep, and
has been ascribed to compression of the nerves in the narrowed Haversian
canals.
The _periosteal gumma_ appears as a smooth, circumscribed swelling which
is soft and elastic in the centre and firm at the margins, and shades
off into the surrounding bone. The gumma may be completely absorbed or
it may give place to a hard node. In some cases the gumma softens in the
centre, the skin becomes adherent, thin, and red, and finally gives way.
The opening in the skin persists as a sinus, or develops into a typical
ulcer with irregular, crescentic margins; in either case a probe reveals
the presence of carious bone or of a sequestrum. The health may be
impaired as a result of mixed infection, and the absorption of toxins
and waxy degeneration in the viscera may ultimately be induced.
A _central gumma_ in a long bone may not reveal its presence until it
erupts through the shell and reaches the periosteal surface or invades
an adjacent joint. Sometimes the first manifestation is a fracture of
the bone produced by slight violence.
In radiograms the appearance of syphilitic bones is usually
characteristic. When there is hyperostosis and sclerosis, the shaft
appears denser and broader than normal, and the contour is uneven or
wavy. When there is a central gumma, the shadow is interrupted by a
rounded clear area, like that of a chondroma or myeloma, but there is
sclerosis round about.
_Diagnosis._--The conditions most liable to be mistaken for syphilitic
disease of bone are chronic staphylococcal osteomyelitis, tuberculosis,
and sarcoma; and the diagnosis is to be made by the history and progress
of the disease, the result of examination with the X-rays, and the
results of specific tests and treatment.
_Treatment._--The general health is to be improved by open air, by
nourishing food, and by the administration of cod-liver oil, iron, and
arsenic. Anti-syphilitic remedies should be given, and if they are
administered before there is any destruction of tissue, the benefit
derived from them is usually marked.
Radiograms show the rapid absorption of the new bone both on the surface
and in the marrow, and are of value in establishing the therapeutic
diagnosis.
In certain cases, and particularly when there are destructive changes in
the bone complicated with pyogenic infection, specific remedies have
little effect. In cases of persistent or relapsing gummatous disease
with ulceration of skin, it is often necessary to remove the diseased
soft parts with the sharp spoon and scissors, and to gouge or chisel
away the unhealthy bone, on the same lines as in tuberculous disease.
When hyperostosis and sclerosis of the bone is attended with severe pain
which does not yield to blistering, the periosteum may be incised and
the sclerosed bone perforated with a drill or trephine.
#Lesions of Bone in Inherited Syphilis.#--_Craniotabes_, in which the
flat bones of the skull undergo absorption in patches, was formerly
regarded as syphilitic, but it is now known to result from prolonged
malnutrition from any cause. _Bossing of the skull_ resulting in the
formation of Parrot's nodes is also being withdrawn from the category of
syphilitic affections. The lesions in infancy--epiphysitis, bossing of
the skull, and craniotabes--have been referred to in the chapter on
inherited syphilis.
_Epiphysitis or Syphilitic Perichondritis._--The first of these terms is
misleading, because the lesion involves the ossifying junction and the
shaft of the bone, and the epiphysis only indirectly. The young bone is
replaced by granulation tissue, so that large clear areas are seen with
the X-rays. The symptoms are referred to the joint, because it is there
that the muscles are inserted and drag on the perichondrium when
movement occurs; swelling is most marked in the vicinity of the joint,
and it may be added to by effusion into the synovial cavity. The baby,
usually under six months, is noticed to be feverish and fretful and to
cry when touched. The mother discovers that the pain is caused by moving
a particular limb, usually the arm, as the humerus, radius, and ulna are
the bones most commonly affected; the limb, moreover, hangs useless at
the side as if paralysed, and the condition was formerly described as
_syphilitic pseudo-paralysis_.
The lesions met with later correspond to those of the tertiary period of
the acquired disease, but as they affect bones which are still actively
growing, the effects are more striking. Gummatous disease may come and
go over periods of many years, with the result that the external
appearance and architectural arrangement of a long bone come to be
profoundly altered. In the tibia, for example, the shaft is bowed
forward in a gentle curve, which is compared to the curve of a
sabre--"sabre-blade" deformity (Fig. 132). The diffuse thickening all
round the bone obscures the sharp margins so that the bone becomes
circular in section and the anterior and mesial edges are blunted, and
the comparison to a cucumber is deserved. In some cases the tibia is
actually increased in length as well as in girth.
[Illustration: FIG. 132.--Sabre-blade Deformity of Left Tibia in
Inherited Syphilis.
(From a photograph lent by Sir George T. Beatson.)]
The contrast between the grossly enlarged and misshapen tibia and the
normal or even attenuated fibula is a striking one.
_Treatment_ is carried out on lines similar to those recommended in the
acquired disease. When curving of the tibia causes disability in
walking, the bone may be straightened by a cuneiform resection.
_Syphilitic dactylitis_ is met with chiefly in children. It may affect
any of the fingers or toes, but is commonest in the first phalanx of the
index-finger or of the thumb. Several fingers may be attacked at the
same time or in succession. The lesion consists in a gummatous
infiltration of the soft parts surrounding the phalanx, or a gummatous
osteomyelitis, but there is practically no tendency to break down and
discharge, or to the formation of a sequestrum as is so common in
tuberculous dactylitis.
The finger becomes the seat of a swelling, which is more evident on the
dorsal aspect, and, according to the distribution and extent of the
disease, it is acorn-shaped, fusiform, or cylindrical. It is firm and
elastic, and usually painless. The movements are impaired, especially if
the joints are involved. In its early stages the disease is amenable to
anti-syphilitic treatment, and complete recovery is the rule.
HYDATID DISEASE
This rare disease results from the lodgment of the embryos of the tænia
echinoccus, which are conveyed to the marrow by the blood-stream. The
cysts are small, usually about the size of a pin-head, and they are
present in enormous numbers scattered throughout the marrow. The parts
of the skeleton most often affected are the articular ends of the long
bones, the bodies of the vertebræ, and the pelvis.
As the cysts increase in number and in size, the framework of the bone
is gradually absorbed, and there result excavations or cavities. The
marrow and spongy bone first disappear, the compact tissue then becomes
thin, and pathological fracture may result. The bone becomes expanded,
and the cysts may escape through perforations into the surrounding
cellular tissue, and when thus freed from confinement may attain
considerable dimensions. Suppuration from superadded pyogenic infection
may be attended with extensive necrosis, and lead to disorganisation of
the adjacent joint.
_Clinical Features._--The patient complains of deep-seated pains. In
superficial bones, such as the tibia, there is enlargement, and it may
be possible to recognise egg-shell crackling, or unequal consistence of
the bone, which is hard in some parts, and doughy and elastic in others.
The disease may pursue an indolent course during months or years until
some complication occurs, such as suppuration or fracture. With the
occurrence of suppuration the disease becomes more active, and abscesses
may form in the soft parts and in the adjacent joint. In the vertebral
column, hydatids give rise to angular deformity and paraplegia. In the
pelvis, there is usually great enlargement of the bones, and when
suppuration occurs it is apt to infect the hip-joint and to terminate
fatally.
Examination with the X-rays shows the characteristic excavations of the
bone caused by the cysts. The disease is liable to be mistaken for
central tumour, gumma, tuberculosis, or abscess of bone.
The _treatment_ consists in thorough eradication of the parasite by
operation. The bone is laid open and scraped or resected according to
the extent of the disease, and the raw surfaces swabbed with 1 per cent.
formalin. In advanced cases complicated with spontaneous fracture or
with suppuration, amputation affords the best chance of recovery.
The lesions in the bones resulting from _actinomycosis_ and from
_mycetoma_, have been described with these diseases.
CONSTITUTIONAL DISEASES ATTENDED WITH LESIONS IN THE BONES
These include rickets, scurvy-rickets, osteomalacia, ostitis deformans,
osteomyelitis fibrosa, fragilitas ossium, and diseases of the nervous
system.
RICKETS
Rickets or rachitis is a constitutional disease associated with
disturbance of nutrition, and attended with changes in the skeleton.
The disease is most common and most severe among the children of the
poorer classes in large cities, who are improperly fed and are brought
up in unhealthy surroundings. There is evidence that the most important
factors in the causation of rickets are ill-health of the mother during
pregnancy, and the administration to the child after its birth of food
which is defective in animal fat, proteids, and salts of lime, or which
contains these in such a form that they are not readily assimilated. The
occurrence of the disease is favoured, and its features are aggravated,
by imperfect oxygenation of the blood as the result of a deficiency of
fresh air and sunlight, want of exercise, and by other conditions which
prevail in the slums of large towns.
_Pathological Anatomy._--The most striking feature is the softness
(malacia) of the bones, due to excessive absorption of osseous tissue,
and the formation of an imperfectly calcified tissue at the sites of
ossification. The affected bones lose their rigidity, so that they are
bent under the weight of the body, by the traction of muscles, and by
other mechanical forces.
The _periosteum_ is thick and vascular, and when detached carries with
it plates and spicules of soft porous bone. The new bone may be so
abundant that it forms a thick crust on the surface, and in the flat
bones of the skull this may be heaped up in the form of bosses or ridges
resembling those ascribed to inherited syphilis.
In the epiphysial cartilages and at the ossifying junctions, all the
processes concerned in ossification, excepting the deposition of lime
salts, occur to an exaggerated degree. The cartilage of the epiphysial
disc proliferates actively and irregularly, so that it becomes softer,
thicker, and wider, and gives rise to a visible swelling, best seen at
the lower end of the radius and lower end of the tibia, and at the
costo-chondral junctions where the series of beaded swellings is known
as the "rickety rosary."
The ossifying zone is increased in depth; the marrow is abnormally
vascular; and the new bone that is formed is imperfectly calcified. The
result is that the bones may never attain their normal length, and they
remain stunted throughout life as in rickety dwarfs (Fig. 133), or the
shafts may grow unequally and come to deviate from their normal axes as
in knock-knee and bow-knee.
[Illustration: FIG. 133.--Skeleton of Rickety Dwarf, known as
"Bowed Joseph," leader of the Meal Riots in Edinburgh, who died in 1780.
(Anatomical Museum, University of Edinburgh.)]
These changes are well brought out in skiagrams; instead of the
well-defined narrow line which represents the epiphysial cartilage,
there is an ill-defined, blurred zone of considerable depth.
In the shafts of the long bones, owing to the excessive absorption of
bone, the cortex becomes porous, the spongy bone is rarefied, and the
bones readily bend or break under mechanical influences. When the
disease is arrested, a process of repair sets in which often results in
the bones becoming denser and heavier than normal. In the flat bones of
the skull, the absorption may result in the entire disappearance of
areas of bone, leaving a membrane which dimples like thin cardboard
under the pressure of the finger--a condition known as _craniotabes_.
_Changes in the Skeleton before the Child is able to walk._--The
fontanelles remain open until the end of the second year or longer, and
the frontal and parietal eminences are unduly prominent. There is
sometimes hydrocephalus, and the head is characteristically enlarged.
The jaws are altered so that while the upper jaw is contracted into the
shape of a #V#, the lower jaw is square instead of rounded in outline,
and the teeth do not oppose one another. In the _thorax_, the chief
feature may be the beading at the costo-chondral junctions, principally
of the fifth and sixth ribs or its walls may be contracted,
particularly if respiration is interfered with as a result of bronchial
catarrh or adenoids. The contraction may take the form of a vertical
groove on each side, or of a horizontal groove at the level of the upper
end of the xiphi-sternum; when the sternum and cartilages form a
projection in front, the deformity is known as "pigeon-breast."
The _spine_ may be curved backwards--_kyphosis_--throughout its
whole extent or only in one part; or it may be curved to one
side--_scoliosis_.
In the _limbs_, the prominent features are the deficient growth in
length of the long bones, the enlargements at the epiphysial junctions,
and the bending, and occasional greenstick fracture, of the shafts. The
degree of enlargement of the epiphysial junctions is directly
proportionate to the amount of movement to which the bone is subjected
(John Thomson). The curves at this stage depend on the attitude of the
child while sitting or being carried--for example, the arm bones become
bent in children who paddle about the floor with the aid of their arms;
and in a child who lies on its back with the lower limbs everted, the
weight of the limb may lead to curvature of the neck of the femur--coxa
vara. The clavicle or humerus may sustain greenstick fracture from the
child being lifted by the arms; the femur, by a fall. From the extreme
laxity of the ligaments, the joints can be moved beyond the normal
limits, and the child is often observed to twist its limbs into abnormal
attitudes.
_In Children who have walked._--In these children the most important
deformities occur in the spine, pelvis, and lower extremities, and
result for the most part from yielding of the softened bones under the
weight of the body. Scoliosis is the usual type of spinal curvature, and
in extreme cases it may lead to a pronounced form of hump-back. The
pelvis may remain small (_justo-minor pelvis_), or it may be contracted
in the sagittal plane (_flat pelvis_); when the bones are unusually
soft, the acetabular portions are pushed inwards by the femora bearing
the weight of the body, and the pelvis assumes the shape of a trefoil,
as in the malacia of women. The shaft of the femur is curved forwards
and laterally; the bones of the leg laterally as in bow-leg, or
forwards, or forwards and laterally just above the ankle. The
deformities at the knee (genu valgum, genu varum, and genu recurvatum),
and at the hip (coxa vara), will be described in the volume dealing with
the Extremities.
The majority of cases seen in surgical practice suffer from the
deformities resulting from rickets rather than from the active disease.
The examination of a large series of children at different ages shows
that the deformities become less and less frequent with each year. Those
who recover may ultimately show no trace of rickets, and this is
especially true of children who grow at the average rate; in those,
however, in whom growth is retarded, especially from the fifth to the
seventh year, the deformities are apt to be permanent. It may be noted
that the scoliosis due to rickets has little tendency towards recovery.
_Treatment._--The treatment of the disease consists in regulating the
diet, improving the surroundings, and preventing deformity. Phosphorus
in doses of 100th grain may be given dissolved in cod-liver oil, and
preparations of iron and lime may be added with advantage. To avoid
those postures which predispose to deformities, the child should lie as
much as possible. In the well-to-do classes this is readily accomplished
by the aid of a nurse and the use of a perambulator. In hospital
out-patients the child is kept off its feet by the use of a light wooden
splint applied to the lateral aspect of each lower extremity, and
extending from the pelvis to 6 inches beyond the sole.
When deformities are already present, the treatment depends upon whether
or not there is any prospect of the bone straightening naturally. Under
five years of age this may, as a rule, be confidently expected; the
child should be kept off its feet, and the limbs bathed and massaged. In
children of five or six and upwards, the prospect of natural
straightening is a diminishing one, and it is more satisfactory to
correct the deformity by operation. In rickety curvature of the spine,
the child should lie on a firm mattress, or, to allow of its being taken
into the open air, upon a double Thomas' splint extending from the
occiput to the heels; the muscles acting on the trunk should be braced
up by massage and appropriate exercises.
#Late Rickets# or #Rachitis Adolescentium# is met with at any age from
nine to seventeen, and is generally believed to be due to a
recrudescence of rickets which had been present in childhood. The
disease is not attended with any disturbance of the general health; the
pathological changes are the same as in infantile rickets, but are for
the most part confined to the ossifying junctions, especially those
which are most active during adolescence, for example at the knee-joint.
The patient is easily tired, complains of pain in the bones, and, unless
care is taken, deformity is liable to ensue. There can be no doubt that
adolescent rickets plays an important part in the production of the
deformities which occur at or near puberty, especially knock-knee and
bow-knee.
#Scurvy-Rickets# or #Infantile Scurvy#.--This disease, described by
Barlow and Cheadle, is met with in infants under two years who have been
brought up upon sterilised or condensed milk and other proprietary
foods, and is most common in the well-to-do classes. The hæmorrhages,
which are so characteristic of the disease, are usually preceded for
some weeks by a cachectic condition, with listlessness and debility and
disinclination for movement. Very commonly the child ceases to move one
of his lower limbs--pseudo-paralysis--and screams if it is touched; a
swelling is found over one of the bones, usually the femur, accompanied
by exquisite tenderness; the skin is tense and shiny, and there may be
some œdema. These symptoms are due to a sub-periosteal hæmorrhage, and
associated with this there may be crepitus from separation of an
epiphysis, rarely from fracture of the shaft of the bone. X-ray
photographs show enlargement of the bone, the periosteum being raised
from the shaft and new bone formed in relation to it. Hæmorrhages also
occur into the skin, presenting the appearance of bruises, into the
orbit and conjunctiva, and from the mucous membranes.
The _treatment_ consists in correcting the errors in diet. The infant
should have a wet nurse or a plentiful supply of cow's milk in its
natural state. Anti-scorbutics in the form of orange, lemon, or grape
juice, and of potatoes bruised down in milk, may be given.
#Osteomalacia.#--The term osteomalacia includes a group of conditions,
closely allied to rickets, in which the bones of adults become soft and
yielding, so that they are unduly liable to bend or break.
One form occurs in _pregnant and puerperal women_, affecting most
commonly the pelvis and lumbar vertebræ, but sometimes the entire
skeleton. The lime salts are absorbed, the bones lose their rigidity and
bend under the weight of the body and other mechanical influences, with
the result that gross deformities are produced, particularly in the
pelvis, the lumbar spine, and the hip-joints.
_Neuropathic_ forms occur in certain chronic diseases of the brain and
cord; in some cases the bones lose their lime salts and bend, in others
they become brittle.
_Osteomalacia associated with New Growths in the Skeleton._--When
_secondary cancer_ is widely distributed throughout the skeleton, it is
associated with softening of the bones, as a result of which they
readily bend or break, and after death are easily cut with a knife. In
the disease known as _multiple myeloma_, the interior of the ribs,
sternum, and bodies of the vertebræ is occupied by a reddish gelatinous
pulp, the structure of which resembles sarcoma; the bones are reduced to
a mere shell, and may break on the slightest pressure; the urine
contains albumose, a substance resembling albumen but coagulating at a
comparatively low temperature (140° F.), and the coagulum is
re-dissolved on boiling, and it is readily precipitated by hydrochloric
acid (Bence-Jones).
#Ostitis Deformans--Paget's Disease of Bone.#--This rare disease was
first described by Sir James Paget in 1877. In the early stages, the
marrow is transformed into a vascular connective tissue; its bone-eating
functions are exaggerated, and the framework of the bone becomes
rarefied, so that it bends under pressure as in osteomalacia. In course
of time, however, new bone is formed in great abundance; it is at first
devoid of lime salts, but later becomes calcified, so that the bones
regain their rigidity. This formation of new bone is much in excess of
the normal, the bones become large and bulky, their surfaces rough and
uneven, their texture sclerosed in parts, and the medullary canal is
frequently obliterated. These changes are well brought out in X-ray
photographs. The curving of the long bones, which is such a striking
feature of the disease, may be associated with actual lengthening, and
the changes are sometimes remarkably symmetrical (Fig. 135). The bones
forming the cranium may be enormously thickened, the sutures are
obliterated, the distinction into tables and diploë is lost, and, while
the general texture is finely porous, there may be areas as dense as
ivory (Fig. 134).
[Illustration: FIG. 134.--Changes in the Skull resulting from Ostitis
Deformans.
(Anatomical Museum, University of Edinburgh.)]
_Clinical Features._--The disease is usually met with in persons over
fifty years of age. It is insidious in its onset, and, the patient's
attention may be first attracted by the occurrence of vague pains in the
back or limbs; by the enlargement and bending of such bones as the tibia
or femur; or by a gradual increase in the size of the head,
necessitating the wearing of larger hats. When the condition is fully
developed, the attitude and general appearance are eminently
characteristic. The height is diminished, and, owing to the curving of
the lower limbs and spine, the arms appear unnaturally long; the head
and upper part of the spine are bent forwards; the legs are held apart,
slightly flexed at the knees, and are rotated out as well as curved; the
whole appearance suggests that of one of the large anthropoid apes. The
muscles of the limbs may waste to such an extent as to leave the large,
curved, misshapen bones covered only by the skin (Fig. 135). In the
majority of cases the bones of the lower extremities are much earlier
and more severely affected than those of the upper extremity, but the
capacity of walking is usually maintained even in the presence of great
deformity. In a case observed by Byrom Bramwell, the patient suffered
from a succession of fractures over a period of years.
[Illustration: FIG. 135.--Cadaver, illustrating the alterations in the
Lower Limbs resulting from Ostitis Deformans.]
The disease may last for an indefinite period, the general health
remaining long unaffected. In a considerable number of the recorded
cases one of the bones became the seat of sarcoma.
#Osteomyelitis Fibrosa.#--This comparatively rare disease, which was
first described by Recklinghausen, presents many interesting features.
Because of its causing deformities of the bones and an undue liability
to fracture, and being chiefly met with in adolescents, it is regarded
by some authors as a juvenile form of Paget's disease. It may be
diffused throughout the skeleton--we have seen it in the skull and in
the bones of the extremities--or it may be confined to a single bone,
usually the femur, or, what is more remarkable, the condition may affect
a portion only of the shaft of a long bone and be sharply defined from
the normal bone in contact with it.
[Illustration: FIG. 136.--Osteomyelitis Fibrosa affecting Femora in a
man æt. 19. The curving of the bones is due to multiple fractures.]
On longitudinal section of a long bone during the active stage of the
disease, the marrow is seen to be replaced by a vascular young
connective tissue which encroaches on the surrounding spongy bone,
reducing it to the slenderest proportions; the formation of bone from
the periosteum does not keep pace with the absorption and replacement
going on in the interior, and the cortex may be reduced to a thin shell
of imperfectly calcified bone which can be cut with a knife. The young
connective tissue which replaces the marrow is not unlike that seen in
osteomalacia; it is highly vascular and may show hæmorrhages of various
date; there are abundant giant cells of the myeloma type, and
degeneration and liquefaction of tissue may result in the formation of
cysts, which, when they constitute a prominent feature, are responsible
for the name--_osteomyelitis fibrosa cystica_--sometimes applied to the
condition.
It would appear that most of the recorded cases of _cysts of bone_ owe
their origin to this disease, while the abundance of giant cells with
occasional islands of cartilage in the wall of such cysts is responsible
for the view formerly held that they owed their origin to the
liquefaction of a solid tumour, such as a myeloma, a chondroma, or even
a sarcoma. Although the tissue elements in this disease resemble those
of a new growth arising in the marrow, they differ in their arrangement
and in their method of growth; there is no tendency to erupt through the
cortex of the bone, to invade the soft parts, or to give rise to
secondary growths.
_Clinical Features._--The onset of the disease is insidious, and
attention is usually first directed to it by the occurrence of fracture
of the shaft of one of the long bones--usually the femur--from violence
that would be insufficient to break a healthy bone. Apart from fracture,
the great increase in the size of one of the long bones and its uneven
contour are sufficiently remarkable to suggest examination with the
X-rays, by means of which the condition is at once recognised. A
systematic examination of the other long bones will often reveal the
presence of the disease at a stage before the bone is altered
externally.
Symmetrical bossing of the skull was present in the case shown in
Figs. 136 and 137, and there were also scattered patches of brown
pigmentation of the skin of the face, neck, and trunk, similar to those
met with in generalised neuro-fibromatosis. Apart from fracture, the
disease is recognised by the thickening and usually also by the curving
of the shafts of the long bones. It is easy to understand the curvature
of bones that have passed through a soft stage and also of those that
have been broken and badly united, but it is difficult to account for
the curvatures that have no such cause; for example, we have seen
marked curve of the radius in a forearm of which the ulna was quite
straight. The curvature probably resulted from exaggerated growth in
length.
[Illustration: FIG. 137.--Radiogram of Upper End of Femur showing
appearances in Osteomyelitis Fibrosa.]
The X-ray appearances vary with the stage of the malady, not estimated
in time, for the condition is chronic and may become stationary, but
according to whether it is progressive or undergoing repair. The shadow
of the bone presents a poor contrast to the soft parts, and no trace of
its original architecture; in extreme cases the shadow of the femur
resembles an unevenly filled sausage (Fig. 137); there is no cortical
layer, the interior shows no trabecular structure, and some of the many
clear areas are probably cysts. The condition extends right up to the
articular cartilage, or, in the case of adolescent bones, up to the
epiphysial cartilage.
_Prognosis._--The condition does not appear to affect the general
health. The future is concerned with the local conditions, and,
especially in the case of the femur, with its liability to fracture; so
far as we know there is no time limit to this.
_Treatment_ is confined to protecting the affected bone--usually the
femur--from injury. Operative treatment may be required for lameness due
to a badly united fracture.
#Neuropathic Atrophy of Bone.#--The conditions included under this
heading occur in association with diseases of the nervous system.
Most importance attaches to the fragility of the bones met with in
general paralysis of the insane, locomotor ataxia, and other chronic
diseases of the brain and spinal cord. The bones are liable to be
fractured by forces which would be insufficient to break a healthy bone.
In _locomotor ataxia_ the fractures affect especially the bones of the
lower extremity, and may occur before there are any definite nerve
symptoms, but they are more often met with in the ataxic stage, when the
abrupt and uncontrolled movements of the limbs may play a part in their
causation. They may be unattended with pain, and may fail to unite; when
repair does take place, it is sometimes attended with an excessive
formation of callus. Joint lesions of the nature of Charcot's disease
may occur simultaneously with the alterations in the bones. In
_syringomyelia_ pathological fracture is not so frequent as in locomotor
ataxia; it is more likely to occur in the bones of the upper extremity,
and especially in the humerus. In some cases of _epilepsy_ the bones
break when the patient falls in a fit, and there is usually an
exaggerated amount of comminution.
In these affections the bones present no histological or chemical
alterations, and the X-ray shadow does not differ from the normal. It is
maintained, therefore, that the disposition to fracture does not depend
upon a fragility of the bone, but on the loss of the muscular sense and
of common sensation in the bones, as a result of which there is an
inability properly to throw the muscles into action and dispose the
limbs so as to place them under the most favourable conditions to meet
external violence.
#Osteogenesis Imperfecta#, #Fragilitas Ossium#, or #Congenital
Osteopsathyrosis#.--These terms are used to describe a condition in
which an undue fragility of the bones dates from intra-uterine life. It
may occur in several members of the same family. In severe cases,
intra-uterine fractures occur, and during parturition fresh fractures
are almost sure to be produced, so that at birth there is a combination
of recent fractures and old fractures united and partly united, with
bendings and thickenings of the bones. Large areas of the cranial vault
may remain membranous.
After birth the predisposition to fracture continues, the bones are
easily broken, the fractures are attended with little or no pain, the
crepitus is soft, and although union may take place, it may be delayed
and be attended with excess of callus. Cases have been observed in which
a child has sustained over a hundred fractures.
The bones show a feeble shadow with the X-rays, and appear thin and
atrophied; the medullary canal is increased at the expense of the
cortex.
In young infants in whom multiple fractures occur the prognosis as to
life is unfavourable, and no satisfactory treatment of the disease has
been formulated. If the patient survives, the tendency to fracture
gradually disappears.
#Hypertrophic Pulmonary Osteo-Arthropathy.#--This condition, which was
described by Marie in 1890, is secondary to disease in the chest, such
as chronic phthisis, empyema, bronchiectasis, or sarcoma of the lung.
There is symmetrical enlargement and deformity of the hands and feet;
the shafts of the bones are thickened, and the soft tissues of the
terminal segments of the digits hypertrophied. The fingers come to
resemble drum-sticks, and the thumb the clapper of a bell. The nails are
convex, and incurved at their free ends, suggesting a resemblance to the
beak of a parrot. There is also enlargement of the lower ends of the
bones of the forearm and leg, and effusion into the wrist and
ankle-joints. Skiagrams of the hands and feet show a deposit of new bone
along the shafts of the phalanges.
TUMOURS OF BONE
New growths which originate in the skeleton are spoken of as _primary
tumours_; those which invade the bones, either by metastasis from other
parts of the body or by spread from adjacent tissues, as _secondary_. A
tumour of bone may grow from the cellular elements of the periosteum,
the marrow, or the epiphysial cartilage.
Primary tumours are of the connective-tissue type, and are usually
solitary, although certain forms, such as the chondroma, may be multiple
from the outset.
_Periosteal tumours_ are at first situated on one side of the bone, but
as they grow they tend to surround it completely. Innocent periosteal
tumours retain the outer fibrous layer as a capsule. Malignant tumours
tend to perforate the periosteal capsule and invade the soft parts.
_Central_ or _medullary tumours_ as they increase in size replace the
surrounding bone, and simultaneously new bone is formed on the surface;
as this is in its turn absorbed, further bone is formed beneath the
periosteum, so that in time the bone is increased in girth, and is said
to be "expanded" by the growth in its interior.
#Primary Tumours--Osteoma.#--When the tumour projects from the surface
of a bone it is called an _exostosis_. When growing from bones developed
in membrane, such as the flat bones of the skull, it is usually dense
like ivory, and the term _ivory exostosis_ is employed. When derived
from hyaline cartilage--for example, at the ends of the long bones--it
is known as a _cartilaginous exostosis_. This is invested with a cap of
cartilage from which it continues to grow until the skeleton attains
maturity.
An exostosis forms a rounded or mushroom-shaped tumour of limited size,
which may be either sessile or pedunculated, and its surface is smooth
or nodulated (Figs. 138 and 139). A cartilaginous exostosis in the
vicinity of a joint may be invested with a synovial sac or bursa--the
so-called _exostosis bursata_. The bursa may be derived from the
synovial membrane of the adjacent joint with which its cavity sometimes
communicates, or it may be of adventitious origin; when it is the seat
of bursitis and becomes distended with fluid, it may mask the underlying
exostosis, which then requires a radiogram for its demonstration.
[Illustration: FIG. 138.--Radiogram of Right Knee showing Multiple
Exostoses.]
_Clinically_, the osteoma forms a hard, indolent tumour attached to a
bone. The symptoms to which it gives rise depend on its situation. In
the vicinity of a joint, it may interfere with movement; on the medial
side of the knee it may incapacitate the patient from riding. When
growing from the dorsum of the terminal phalanx of the great
toe--_subungual exostosis_--it displaces the nail, and may project
through its matrix at the point of the toe, while the soft parts over it
may be ulcerated from pressure (Fig. 107). It incapacitates the patient
from wearing a boot. When it presses on a nerve-trunk it causes pains
and cramps. In the orbit it displaces the eyeball; in the nasal fossæ
and in the external auditory meatus it causes obstruction, which may be
attended with ulceration and discharge. In the skull it may project
from the outer table, forming a smooth rounded swelling, or it may
project from the inner table and press upon the brain.
The diagnosis is to be made by the slow growth of the tumour, its
hardness, and by the shadow which it presents with the X-rays (Fig. 138).
An osteoma which does not cause symptoms may be left alone, as it ceases
to grow when the skeleton is mature and has no tendency to change its
benign character. If causing symptoms, it is removed by dividing the
neck or base of the tumour with a chisel, care being taken to remove the
whole of the overlying cartilage. The dense varieties met with in the
bones of the skull present greater difficulties; if it is necessary to
remove them, the base or neck of the tumour is perforated in many
directions with highly tempered drills rotated by some form of engine,
and the division is completed with the chisel.
[Illustration: FIG. 139.--Multiple Exotoses of both limbs.
(Photograph lent by Sir George T. Beatson.)]
#Multiple Exostoses.#--This disease, which, by custom, is still placed
in the category of tumours, is to be regarded as a disorder of growth,
dating from intra-uterine life and probably due to a disturbance in the
function of the glands of internal secretion, the thyreoid being the one
which is most likely to be at fault (Arthur Keith). The disorder of
growth is confined to those elements of the skeleton where a core of
bone formed in cartilage comes to be encased in a sheath of bone formed
beneath the periosteum. To indicate this abnormality the name
_diaphysial aclasis_ has been employed by Arthur Keith at the suggestion
of Morley Roberts.
Bones formed entirely in cartilage are exempt, namely, the tarsal and
carpal bones, the epiphyses of the long bones, the sternum, and the
bodies of the vertebræ. Bones formed entirely in membrane, that is,
those of the face and of the cranial vault, are also exempt. The
disorder mainly affects the ossifying junctions of the long bones of the
extremities, the vertebral border of the scapula, and the cristal border
of the ilium.
_Clinically_ the disease is attended with the gradual and painless
development during childhood or adolescence of a number of tumours or
irregular projections of bone, at the ends of the long bones, the
vertebral border of the scapula, and the cristal border of the ilium.
They exhibit a rough symmetry; they rarely attain any size; and they
usually cease growing when the skeleton attains maturity--the conversion
of cartilage into bone being then completed. While they originate from
the ossifying junctions of the long bones, they tend, as the shaft
increases in length, to project from the surface of the bone at some
distance from the ossifying junction and to "point" away from it. They
may cause symptoms by "locking" the adjacent joint or by pressing upon
nerve-trunks or blood vessels.
In a considerable proportion of cases, the disturbance of growth is
further manifested by dwarfing of the long bones; these are not only
deficient in length but are sometimes also curved and misshapen, which
accounts for the condition being occasionally confused with the
disturbances of growth resulting from rickets. In about one-third of the
recorded cases there is a dislocation of the head of the radius on one
or on both sides, a result of unequal growth between the bones of the
forearm.
[Illustration: FIG. 140.--Multiple Cartilaginous Exostoses in a
man æt. 27. The scapular tumour projecting above the right clavicle has
taken on active growth and pressed injuriously on the cords of the
brachial plexus.]
In early adult life, one of the tumours, instead of undergoing
ossification, may take on active growth and exhibit the features of a
chondro-sarcoma, pressing injuriously upon adjacent structures (Fig. 140)
and giving rise later to metastases in the lungs.
The _X-ray appearances_ of the bones affected are of a striking
character; apart from the outgrowths of bone or "tumours" there is
evident a widespread alteration in the internal architecture of the
bones, which suggests analogies with other disturbances of ossification
such as achondroplasia and osteomyelitis fibrosa. The condition is one
that runs in families, sometimes through several generations; we have
more than once seen a father and son together in the hospital
waiting-room.
As regards _treatment_, there is no indication for surgical interference
except when one or other tumour is a source of disability as by pressing
upon a nerve-trunk or by locking a joint, in which case it is easily
removed by chiselling through its neck.
[Illustration: FIG. 141.--Multiple Cartilaginous Exostoses in a
man æt. 27, the same as in Fig. 140.]
_Diffuse Osteoma, Leontiasis Ossea._--This rare affection was described
by Virchow, and named leontiasis ossea because of the disfigurement to
which it gives rise. It usually commences in adolescence as a diffuse
overgrowth first of one and then of both maxillæ; these bones are
enlarged in all directions and project on the face, and the nasal fossæ
and the maxillary and frontal sinuses become filled up with bone, which
encroaches also on the orbital cavities. In addition to the hideous
deformity, the patient suffers from blocking of the nose, loss of smell,
and protrusion of the eyes, sometimes followed by loss of sight. The
condition is liable to spread to the zygomatic and frontal bones, the
vault of the skull, and to the mandible. The base of the skull is not
affected. The disease is of slow progress and may become arrested; life
may be prolonged for many years, or may be terminated by brain
complications or by intercurrent affections. In certain cases it is
possible to remove some of the more disfiguring of the bony masses.
A less aggressive form, confined to the maxilla on one side, is
sometimes met with, and, in a case of this variety under our own
observation, the disfigurement, which was the only subject of complaint,
was removed, after reflecting the soft parts, by paring away the excess
of bone; this is easily done as the bone is spongy, and at an early
stage, imperfectly calcified.
A remarkable form of _unilateral hypertrophy and diffuse osteoma of the
skull_, following the distribution of the fifth nerve, has seen
described by Jonathan Hutchinson and Alexis Thomson.
#Chondroma.#--Cartilaginous tumours, apart from those giving rise to
multiple exostoses, grow from the long bones and from the scapula,
ilium, ribs, or jaws. They usually project from the surface of the bone,
and may attain an enormous size; sometimes they grow in the interior of
a bone, the so-called _enchondroma_.
The hyaline cartilage composing the tumour frequently undergoes
myxomatous degeneration, resulting in the formation of a glairy,
semi-fluid jelly, and if this change takes place throughout the tumour
it comes to resemble a cyst. On the other hand, the cartilage may
undergo calcification or ossification. The most important transition of
all is that into sarcoma, the so-called _malignant chondroma_ or
_chondro-sarcoma_, which is associated with rapid increase in size,
and parts of the tumour may be carried off in the blood-stream and give
rise to secondary growths, especially in the lungs.
Cases have been met with in which certain parts of the skeleton--only
those developed in cartilage--were so uniformly permeated with cartilage
that the condition has been described as a "chondromatosis" and is
regarded as dating from an early period of fœtal life. Unlike the
condition known as multiple cartilaginous exostoses, it is a malignant
disease.
[Illustration: FIG. 142.--Multiple Chondromas of Phalanges and
Metacarpals in a boy æt. 10 (cf. Fig. 143).]
The chondroma is met with as a slowly growing tumour which is specially
common in the bones of the hand, often in a multiple form (Figs. 142 and
144). The surface is smooth or lobulated, and in consistence the tumour
may be dense and elastic like normal cartilage, or may present areas of
softening, or of bony hardness. The skin moves freely over it, except in
relation to the bones of the fingers, where it may become adherent and
ulcerate, simulating the appearance of a malignant tumour. Large tumours
growing from the bones of the extremities may implicate the main
vessels and nerves, either surrounding them or pressing on them.
Portions of a chondroma, which have undergone calcification or
ossification, throw a dark shadow with the X-rays; unaltered cartilage
and myxomatous tissue appear as clear areas.
[Illustration: FIG. 143.--Skiagram of Multiple Chondromas shown
in Fig. 142.]
_Treatment._--It is necessary to remove the whole tumour, and in
chondromas growing from the surface of the bone, especially if they are
pedunculated, this is comparatively easy. When a bone, such as the
scapula or mandible, is involved, it is better to excise the bone, or at
least the part of it which bears the tumour. In the case of central
tumours the shell of bone is removed over an area sufficient to allow of
the enucleation of the tumour, or the affected portion of bone is
resected. Should there be evidence of malignancy, such as increased rate
of growth, a tube of radium should be inserted, and in advanced cases
with destruction of tissue, amputation may be called for.
[Illustration: FIG. 144.--Multiple Chondromas in Hand of boy æt. 8]
In multiple chondromas of the hand in young subjects, it was formerly
the custom to amputate the limb; an attempt should be made to avoid this
by shelling out the larger tumours individually, and persevering with
the application of the X-rays or of radium to inhibit the growth of the
smaller ones.
Chondromas springing from the pelvic bones usually arise in the region
of the sacro-iliac joint; they project into the pelvis and press on the
bladder and rectum, and on the sciatic and obturator nerves; sometimes
also on the iliac veins, causing œdema of the legs. They are liable to
take on malignant characters, and rarely lend themselves to complete
removal by operation.
#Fibroma# is met with chiefly as a periosteal growth in relation to the
mouth and pharynx, the _simple epulis_ of the alveolar margin and the
_naso-pharyngeal polypus_ being the most common examples. We have met
with a fibroma in the interior of the lower end of the femur of an
adult, causing expansion of the bone with decided increase in girth and
liability to pathological fracture; it is possible that this represents
the cured stage of osteomyelitis fibrosa.
_Myxoma_, _lipoma_, and _angioma_ of bone are all rare.
#Myeloma.#--The myeloid tumour, which is sometimes classified with the
sarcomas, contains as its chief elements large giant cells, like those
normally present in the marrow. On section these tumours present a
brownish-red or chocolate colour, and, being highly vascular, are liable
to hæmorrhages, and therefore also to pigmentation, and to the formation
of blood cysts. Sometimes the arterial vessels are so dilated as to
impart to the tumour an aneurysmal pulsation and bruit. The enlargement
or "expansion" of the bone results in the cortex being represented by a
thin shell of bone, which may crackle on pressure--parchment or
egg-shell crackling.
The myeloma is most often met with between the ages of twenty-five and
forty in the upper end of the tibia or lower end of the femur. It grows
slowly and causes little pain, and may long escape recognition unless an
examination is made with the X-rays. Although these tumours have been
known to give rise to metastases, they are, as a rule, innocent and are
to be treated as such. When located in the shaft of a long bone,
pathological fracture is liable to occur.
_Diagnosis and X-ray Appearances of Myeloma._--The early diagnosis of
myeloma is made with the aid of the X-rays: the typical appearance is
that of a rounded or oval clear area bounded by a shell of bone of
diminishing thickness (Fig. 145). The inflammatory lesions at the ends
of the long bones--tubercle, syphilitic gumma, and Brodie's abscess,
that resemble myeloma, are all attended with the formation of new bone
in greater or lesser amount. The myeloma is also to be diagnosed from
chondroma, from sarcoma, and from osteomyelitis fibrosa cystica.
[Illustration: FIG. 145.--Radiogram of Myeloma of Humerus.
(Mr. J. W. Struthers' case.)]
_Treatment._--In early cases the cortex is opened up to give free access
to the tumour tissue, which is scraped out with the spoon. Bloodgood
advises the use of Esmarch's tourniquet, and that the curetting be
followed by painting with pure carbolic acid and then rinsing with
alcohol; a rod of bone is inserted to fill the gap. In advanced cases
the segment of bone is resected and a portion of the tibia or fibula
from the other limb inserted into the gap; a tube of radium should also
be introduced.
The coexistence of diffuse myelomatosis of the skeleton and albumosuria
(Bence-Jones) is referred to on p. 474. Myeloma occurs in the jaws,
taking origin in the marrow or from the periosteum of the alveolar
process, and is described elsewhere.
#Sarcoma# and #endothelioma# are the commonest tumours of bone, and
present wide variations in structure and in clinical features.
Structurally, two main groups may be differentiated: (1) the soft,
rapidly growing cellular tumours, and (2) those containing fully formed
fibrous tissue, cartilage, or bone.
(1) The _soft cellular tumours_ are composed mainly of spindle or round
cells; they grow from the marrow of the spongy ends or from the
periosteum of the long bones, the diploë of the skull, the pelvis,
vertebræ, and jaws. As they grow they may cause little alteration in the
contour of the bone, but they eat away its framework and replace it, so
that the continuity of the bone is maintained only by tumour tissue, and
pathological fracture is a frequent result. The small round-celled
sarcomas are among the most malignant tumours of bone, growing with
great rapidity, and at an early stage giving rise to secondary growths.
(2) The second group includes the _fibro-_, _osteo-_, and
_chondro-sarcomas_, and combinations of these; in all of them fully
formed tissues or attempts at fully formed tissues predominate over the
cellular elements. They grow chiefly from the deeper layer of the
periosteum, and at first form a projection on the surface, but later
tend to surround the bone (Fig. 150), and to invade its interior,
filling up the marrow spaces with a white, bone-like substance; in the
flat bones of the skull they may traverse the diploë and erupt on the
inner table. The tumour tissue next the shaft consists of a dense,
white, homogeneous material, from which there radiate into the softer
parts of the tumour, spicules, needles, and plates, often exhibiting a
fan-like arrangement (Fig. 151). The peripheral portion consists of soft
sarcomatous tissue, which invades the overlying soft parts. The
articular cartilage long resists destruction. The ossifying sarcoma is
met with most often in the femur and tibia, less frequently in the
humerus, skull, pelvis, and jaws. In the long bones it may grow from the
shaft, while the chondro-sarcoma more often originates at the
extremities. Sometimes they are multiple, several tumours appearing
simultaneously or one after another. Secondary growths are met with
chiefly in the lungs, metastasis taking place by way of the veins.
[Illustration: FIG. 146.--Periosteal Sarcoma of Femur in a young
subject.]
[Illustration: FIG. 147.--Periosteal Sarcoma of Humerus, after
maceration.
(Anatomical Museum, University of Edinburgh.)]
_Clinical Features._--Sarcoma is usually met with before the age of
thirty, and is comparatively common in children. Males suffer oftener
than females, in the proportion of two to one.
In _periosteal sarcoma_ the presence of a swelling is usually the first
symptom; the tumour is fusiform, firm, and regular in outline, and when
it occurs near the end of a long bone the limb frequently assumes a
characteristic "leg of mutton" shape (Fig. 146). The surface may be
uniform or bossed, the consistence varies at different parts, and the
swelling gradually tapers off along the shaft. On firm pressure, fine
crepitation may be felt from crushing of the delicate framework of new
bone.
[Illustration: FIG. 148.--Chondro-Sarcoma of Scapula in a man æt. 63;
removal of the scapula was followed two years later by metastases and
death.]
In _central sarcoma_ pain is the first symptom, and it is usually
constant, dull, and aching; is not obviously increased by use of the
limb, but is often worse at night. Swelling occurs late, and is due to
expansion of the bone; it is fusiform or globular, and is at first
densely hard, but in time there may be parchment-like or egg-shell
crackling from yielding of the thin shell. The swelling may pulsate, and
a bruit may be heard over it. In advanced cases it may be impossible to
differentiate between a periosteal and a central tumour, either
clinically or after the specimen has been laid open.
Pathological fracture is more common in central tumours, and sometimes
is the first sign that calls attention to the condition. Consolidation
rarely takes place, although there is often an attempt at union by the
formation of cartilaginous callus.
[Illustration: FIG. 149.--Central Sarcoma of Lower End of Femur,
invading the knee-joint.
(Museum of Royal College of Surgeons, Edinburgh.)]
[Illustration: FIG. 150.--Osseous Shell of Osteo-Sarcoma of Upper Third
of Femur, after maceration.]
The soft parts over the tumour for a long time preserve their normal
appearance; or they become œdematous, and the subcutaneous venous
network is evident through the skin. Elevation of the temperature over
the tumour, which may amount to two degrees or more, is a point of
diagnostic significance, as it suggests an inflammatory lesion.
The adjacent joint usually remains intact, although its movements may be
impaired by the bulk of the tumour or by effusion into the cavity.
Enlargement of the neighbouring lymph glands does not necessarily imply
that they have become infected with sarcoma for the enlargement may
disappear after removal of the primary growth; actual infection of the
glands, however, does sometimes occur, and in them the histological
structure of the parent tumour is reproduced.
To obtain a reasonable prospect of cure, the _diagnosis_ must be made at
an early stage. Great reliance is to be placed on information gained by
examination with the X-rays.
[Illustration: FIG. 151.--Radiogram of Osteo-Sarcoma of Upper Third
of Femur.]
_X-ray Appearances._--In periosteal tumours that do not ossify, there is
merely erosion of bone, and the shadow is not unlike that given by
caries; in ossifying tumours, the arrangement of the new bone on the
surface is characteristic, and when it takes the form of spicules at
right angles to the shaft, it is pathognomic.
In soft central tumours, there is disappearance of bone shadow in the
area of the tumour, while above and below or around this, the shadow is
that of normal bone right up to the clear area. In many respects the
X-ray appearances resemble those of myeloma. In tumours in which there
is a considerable amount of imperfectly formed new bone, this gives a
shadow which barely replaces that of the original bone, in parts it may
even add to it--the resulting picture differing widely in different
cases; but it is usually possible to differentiate it from that caused
by bacterial infections of the bone and from lesions of the adjacent
joint.
[Illustration: FIG. 152.--Radiogram of Chondro-Sarcoma of Upper End of
Humerus in a woman æt. 29.]
Skiagraphy is not only of assistance in differentiating new growths from
other diseases of bone, but may also yield information as to the
situation and nature of the tumour, which may have important bearings on
its treatment by operation.
When fracture of a long bone takes place in an adolescent or young adult
from comparatively slight violence, disease of the bone should be
suspected and an X-ray examination made.
In difficult cases the final appeal is to exploratory incision and
microscopical examination of a portion of the tumour; this should be
done when the major operation has been arranged for, the surgeon waiting
until the examination is completed.
The _prognosis_ varies widely. In general, it may be said that
periosteal tumours are less favourable than central ones, because they
are more liable to give rise to metastases. Permanent cures are
unfortunately the exception.
_Treatment._--When one of the bones of a limb is involved, the usual
practice has been to perform amputation well above the growth, and this
may still be recommended as a routine procedure. There are reasons,
however, which may be urged against its continuance. High amputation is
unnecessary in the more benign sarcomas, and in the more malignant forms
is usually unavailing to prevent a fatal issue either from local
recurrence or from metastases in the lungs or elsewhere. Following
the lead of Mikulicz, a considerable number of permanent cures have been
obtained by resecting the portion of bone which is the seat of the
tumour, and substituting for it a corresponding portion from the tibia
or fibula of the other limb. In a cellular sarcoma of the humerus of a
boy we resected the shaft and inserted his fibula ten years ago, and he
shows no sign of recurrence. When resection is impracticable, a
subcapsular enucleation is performed, followed by the insertion of
radium.
#Pulsating Hæmatoma# or #Aneurysm of Bone#.--A limited number of these
are innocent cavernous tumours dating from a congenital angioma. The
majority would appear to be the result of changes in a sarcoma,
endothelioma, or myeloma. The tumour tissue largely disappears, while
the vessels and vascular spaces undergo a remarkable development. The
tumour may come to be represented by one large blood-containing space
communicating with the arteries of the limb; the walls of the space
consist of the remains of the original tumour, plus a shell of bone of
varying thickness. The most common seats of the condition are the lower
end of the femur, the upper end of the tibia, and the bones of the
pelvis.
The _clinical features_ are those of a pulsating tumour of slow
development, and as in true aneurysm, the pulsation and bruit disappear
on compression of the main artery. The origin of the tumour from bone
may be revealed by the presence of egg-shell crackling, and by
examination with the X-rays.
If the condition is believed to be innocent, the treatment is the same
as for aneurysm--preferably by ligation of the main artery; if
malignant, it is the same as for sarcoma.
#Secondary Tumours of Bone.#--These embrace two groups of new growth,
those which give rise to secondary growths in the marrow of bones and
those which spread to bone by direct continuity.
_Metastatic Tumours._--Excepting certain cancers which give rise to
metastases by lymphatic permeation (Handley), the common metastases
arising in the bone-marrow reach their destination through the
blood-stream.
[Illustration: FIG. 153.--Epitheliomatous Ulcer of Leg with direct
extension to Tibia.
(Lord Lister's specimen. Anatomical Museum, University of Edinburgh.)]
Secondary cancer is a comparatively common disease, and, as in
metastases in other tissues, the secondary growths resemble the parent
tumour. The soft forms grow rapidly, and eat away the bone, without
altering its shape or form. In slowly growing forms there may be
considerable formation of imperfectly formed bone, often deficient in
lime salts; this condition may be widely diffused throughout the
skeleton, and, as it is associated with softening and bending of the
bones, it is known as _cancerous osteomalacia_. Secondary cancer of bone
is attended with pain, or it suddenly attracts notice by the occurrence
of pathological fracture--as, for example, in the shaft of the femur or
humerus. In the vertebræ, it is attended with a painful form of
paraplegia, which may involve the lower or all four extremities. On the
other hand, the disease may show itself clinically as a tumour of bone,
which may attain a considerable size, and may be mistaken for a sarcoma,
unless the existence of the primary cancer is discovered.
The cancers most liable to give rise to metastasis in bone are those of
the breast, liver, uterus, prostate, colon, and rectum; hyper-nephroma
of the kidney may also give rise to metastases in bone.
_Secondary tumours derived from the thyreoid gland_ require special
mention, because they are peculiar in that neither the primary growth in
the thyreoid nor the secondary growth in the bones is necessarily
malignant. They are therefore amenable to operative treatment.
_Secondary sarcoma_, whether derived from a primary growth in the bone
or in the soft parts, is much rarer than secondary cancer. Its removal
by operation is usually contra-indicated, but we have known of cases
terminating fatally in which the _section_ revealed only one metastasis,
the removal of which would have benefited the patient.
In all of these conditions, examination of the bones with the X-rays
gives valuable information and often disclose unsuspected metastases.
_Cancer of Bone resulting from Direct Extension from Soft Parts._--In
this group there are also two clinical types. The first is met with in
relation to _epithelioma of a mucous surface_--for example, the palate,
tongue, gums, antrum, frontal sinus, auditory meatus, or middle ear.
They will be described under these special regions.
The second type is met with in relation to _epithelioma occurring in a
sinus_, the sequel of suppurative osteomyelitis, compound fracture, or
tuberculous disease. The patient has usually had a discharging sinus for
a great number of years: we have known it to last as many as fifty. The
epithelioma originates at the skin orifice of the sinus, and spreads to
the bone and into its interior, where the progress of the cancer is
resisted by dense bone, which obliterates the medullary canal. Although
its progress is slow, the infiltration of the bone is usually more
extensive than appears externally. It is recognised clinically by the
characteristic cauliflower growth at the orifice of the sinus, and by
the offensive nature of the discharge. A similar epithelioma may arise
in connection with a _chronic ulcer of the leg_. The cancer may infect
the femoral lymph glands. The operative treatment is influenced by the
extent of the disease in the soft parts overlying the bone, and consists
in wide removal of the diseased tissues and resection of the bone, or in
amputation.
#Cysts of Bone.#--With the exception of hydatid cysts, cysts in the
interior of bone are the result of the liquefaction of solid tissue;
this may be that of chondroma, myeloma, or sarcoma, but more commonly of
the marrow in osteomyelitis fibrosa.