Alexis Thomson and Alexander Miles - Manual of Surgery

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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 diploe of the skull, the pelvis,
vertebrae, 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 diploe 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 aet. 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 oedematous, 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 aet. 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 Haematoma# 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 vertebrae, 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.

CHAPTER XXI

DISEASES OF JOINTS

Definition of terms--Ankylosis. DISEASES: Errors of
development--Bacterial diseases: _Pyogenic_; _Gonorrhoeal_;
_Tuberculous_; _Syphilitic_; _Acute rheumatism_--Diseases
associated with certain constitutional conditions: _Gout_; _Chronic
articular rheumatism_; _Arthritis deformans_;
_Haemophilia_--Diseases associated with affections of the nervous
system: _Neuro-arthropathies_; _Charcot's disease_--Hysterical or
mimetic affections of joints--Tumours and cysts--Loose bodies.

#Definition of Terms.#--The term _synovitis_ is applied to any reaction
which affects the synovial membrane of a joint. It is usually associated
with effusion of fluid, and this may be serous, sero-fibrinous, or
purulent. As the term synovitis merely refers to the tissue involved, it
should always be used with an adjective--such as gouty, gonorrhoeal, or
tuberculous--which indicates its pathological nature.

The terms _hydrops_, _hydrarthrosis_, and _chronic serous synovitis_ are
synonymous, and are employed when a serous effusion into the joint is
the prominent clinical feature. Hydrops may occur apart from
disease--for example, in the knee-joint from repeated sprains, or when
there is a loose body in the joint--but is met with chiefly in the
chronic forms of synovitis which result from gonorrhoea, tuberculosis,
syphilis, arthritis deformans, or arthropathies of nerve origin.

_Arthritis_ is the term applied when not only the synovial membrane but
the articular surfaces, and it may be also the ends of the bones, are
involved, and it is necessary to prefix a qualifying adjective which
indicates its nature. When effusion is present, it may be serous, as in
arthritis deformans, or sero-fibrinous or purulent, as in certain forms
of pyogenic and tuberculous arthritis. Wasting of the muscles,
especially the extensors, in the vicinity of the joint is a constant
accompaniment of arthritis. On account of the involvement of the
articular surfaces, arthritis is apt to be followed by ankylosis.

The term _empyema_ is sometimes employed to indicate that the cavity of
the joint contains pus. This is observed chiefly in chronic disease of
pyogenic or tuberculous origin, and is usually attended with the
formation of abscesses outside the joint.

_Ulceration of cartilage_ and _caries of the articular surfaces_ are
common accompaniments of the more serious and progressive forms of joint
disease, especially those of bacterial origin. The destruction of
cartilage may be secondary to disease of the synovial membrane or of the
subjacent bone. When the disease begins as a synovitis, the synovial
membrane spreads over the articular surface, fuses with the cartilage
and eats into it, causing defects or holes which are spoken of as
ulcers. When the disease begins in the bone, the marrow is converted
into granulation tissue, which eats into the cartilage and separates it
from the bone. Following on the destruction of the cartilage, the
articular surface of the bone undergoes disintegration, a condition
spoken of as _caries of the articular surface_. The occurrence of
ulceration of cartilage and of articular caries is attended with the
clinical signs of fixation of the joint from involuntary muscular
contraction, wasting of muscles, and starting pains. These _starting
pains_ are the result of sudden involuntary movements of the joint. They
occur most frequently as the patient is dropping off to sleep; the
muscles becoming relaxed, the sensitive ulcerated surfaces jar on one
another, which causes sudden reflex contraction of the muscles, and the
resulting movement being attended with severe pain, wakens the patient
with a start. Advanced articular caries is usually associated with some
abnormal attitude and with shortening of the limb. It may be possible to
feel the bony surfaces grate upon one another. When all its constituent
elements are damaged or destroyed, a joint is said to be _disorganised_.
Should recovery take place, repair is usually attended with union of the
opposing articular surfaces either by fibrous tissue or by bone.

#Conditions of Impaired Mobility of Joints.#--There are four conditions
of impaired mobility in joints: rigidity, contracture, ankylosis, and
locking. _Rigidity_ is the fixation of a joint by involuntary
contraction of muscles, and is of value as a sign of disease in
deep-seated joints, such as the hip. It disappears under anaesthesia.

_Contracture_ is the term applied when the fixation is due to permanent
shortening of the soft parts around a joint--muscles, tendons,
ligaments, fasciae, or skin. As the structures on the flexor aspect are
more liable to undergo such shortening, contracture is nearly always
associated with flexion. Contracture may result from disease of the
joint, or from conditions outside it--for example, disease in one of
the adjacent bones, or lesions of the nerves.

_Ankylosis_ is the term applied when impaired mobility results from
changes involving the articular surfaces. It is frequently combined with
contracture. Three anatomical varieties of ankylosis are
recognised--(a) The _fibrous_, in which there are adhesions between
the opposing surfaces, which may be in the form of loose isolated bands
of fibrous tissue, or may bind the bones so closely together as to
obliterate the cavity of the joint. The resulting stiffness, therefore,
varies from a mere restriction of the normal range of movement, up to a
close union of the bones which prevents movement. Fibrous ankylosis may
follow upon injury, especially dislocation or fracture implicating a
joint, or it may result from any form of arthritis. (b) _Cartilaginous
ankylosis_ implies the fusion of two apposed cartilaginous surfaces. It
is often found between the patella and the trochlear surface of the
femur in tuberculous disease of the knee. The fusion of the
cartilaginous surfaces is preceded by the spreading of a vascular
connective tissue, derived from the synovial membrane, over the
articular cartilage. Clinically, it is associated with absolute
immobility, (c) _Bony ankylosis_ or _synostosis_ is an osseous union
between articulating surfaces (Figs. 154 and 155). It may follow upon
fibrous or cartilaginous ankylosis, or may result from the fusion of two
articular surfaces which have lost their cartilage and become covered
with granulations. In the majority of cases it is to be regarded as a
reparative process, presenting analogies with the union of fracture.

[Illustration: FIG. 154.--Osseous Ankylosis of Femur and Tibia in
position of flexion.]

The term _arthritis ossificans_ has been applied by Joseph Griffiths to
a condition in which the articular surfaces become fused without evident
cause.

The occurrence of ankylosis in a joint before the skeleton has attained
maturity does not appear to impair the growth in length of the bones
affected; ankylosis of the temporo-maxillary joints, however, greatly
impairs the growth of the mandible. When there is arrest of growth
accompanying ankylosis, it usually depends on changes in the ossifying
junctions caused by the original disease.

To differentiate by manipulation between muscular fixation and
ankylosis, it may be necessary to anaesthetise the patient. The nature
and extent of ankylosis may be learned by skiagraphy; in osseous
ankylosis the shadow of the two bones is a continuous one. In fibrous as
contrasted with osseous ankylosis mobility may be elicited, although
only to a limited extent; while in osseous ankylosis the joint is
rigidly fixed, and attempts to move it are painless.

[Illustration: FIG. 155.--Osseous Ankylosis of Knee in the flexed
position following upon Tuberculous Arthritis.

(Anatomical Museum, University of Edinburgh.)]

The _treatment_ is influenced by the nature of the original lesion, the
variety of the ankylosis, and the attitude of the joint. When there is
restriction of movement due to fibrous adhesions, these may be elongated
or ruptured. Elongation of the adhesions may be effected by
manipulations, exercises, and the use of special forms of
apparatus--such as the application of weights to the limb. It may be
necessary to administer an anaesthetic before rupturing strong fibrous
adhesions, and this procedure must be carried out with caution, in view
of such risks as fracture of the bone--which is often rarefied--or
separation of an epiphysis. There is also the risk of fat embolism, and
of re-starting the original disease. The giving way of adhesions may be
attended with an audible crack; and the procedure is often followed by
considerable pain and effusion into the joint, which necessitate rest
for some days before exercises and manipulations can be resumed.

_Operative treatment_ may be called for in cases in which the bones are
closely bound to one another by fibrous or by osseous tissue.

_Arthrolysis_, which consists in opening the joint and dividing the
fibrous adhesions, is almost inevitably followed by their reunion.

_Arthroplasty._--Murphy of Chicago devised this operation for restoring
movement to an ankylosed joint. It consists in transplanting between the
bones a flap of fat-bearing tissue, from which a bursal cavity lined
with endothelium and containing a fluid rich in mucin is ultimately
formed.

Arthroplasty is most successful in ankylosis following upon injury; when
the ankylosis results from some infective condition such as tuberculosis
or gonorrhoea, it is liable to result in failure either because of a
fresh outbreak of the infection or because the ankylosis recurs.

When arthroplasty is impracticable, and a movable joint is desired--for
example at the elbow--a considerable amount of bone, and it may be also
of periosteum and capsular ligament, is resected to allow of the
formation of a false joint.

When bony ankylosis has occurred with the joint in an undesirable
attitude--for example flexion at the hip or knee--it can sometimes be
remedied by osteotomy or by a wedge-shaped resection of the bone, with
or without such additional division of the contracted soft parts as will
permit of the limb being placed in the attitude desired.

Bony ankylosis of the joints of a finger, whether the result of injury
or disease, is difficult to remedy by any operative procedure, for while
it is possible to restore mobility, the new joint is apt to be
flail-like.

_Locking._--A joint is said to lock when its movements are abruptly
arrested by the coming together of bony outgrowths around the joint. It
is best illustrated in arthritis deformans of the hip in which new bone
formed round the rim of the acetabulum mechanically arrests the
excursions of the head of the femur. The new bone, which limits the
movements, is readily demonstrated in skiagrams; it may be removed by
operative means. Locking of joints is more often met with as a result of
injuries, especially in fractures occurring in the region of the elbow.
In certain injuries of the semilunar menisci of the knee, also, the
joint is liable to a variety of locking, which differs, however, in many
respects from that described above.