Alexis Thomson and Alexander Miles - Manual of Surgery

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Female members of haemophilia families sometimes show a tendency to
excessive haemorrhage, but they seldom manifest the characteristic
features met with in the male members.

Sometimes the haemorrhage takes place apparently spontaneously from the
gums, the nasal or the intestinal mucous membrane. In other cases the
bleeding occurs into the cellular tissue under the skin or mucous
membrane, producing large areas of ecchymosis and discoloration. One of
the commonest manifestations of the disease is the occurrence of
haemorrhage into the cavities of the large joints, especially the knee,
elbow, or hip. The patient suffers repeatedly from such haemorrhages, the
determining injury being often so slight as to have passed unobserved.

There is evidence that the tendency to bleed is greater at certain times
than at others--in some cases showing almost a cyclical
character--although nothing is known as to the cause of the variation.

After a severe haemorrhage into the cellular tissue or into a joint, the
patient becomes pale and anaemic, the temperature may rise to 102 or
103 F., the pulse become small and rapid, and haemic murmurs are
sometimes developed over the heart and large arteries. The swelling is
tense, fluctuating, and hot, and there is considerable pain and
tenderness.

In exceptional cases, blisters form over the seat of the effusion, or
the skin may even slough, and the clinical features may therefore come
to simulate closely those of an acute suppurative condition. When the
skin sloughs, an ulcer is formed with altered blood-clot in its floor
like that seen in scurvy, and there is a remarkable absence of any
attempt at healing.

The acute symptoms gradually subside, and the blood is slowly absorbed,
the discoloration of the skin passing through the same series of changes
as occur after an ordinary bruise. The patients seldom manifest the
symptoms of the bloodless state, and the blood is rapidly regenerated.

The _diagnosis_ is easy if the patient or his friends are aware of the
family tendency to haemorrhage and inform the doctor of it, but they are
often sensitive and reticent regarding the fact, and it may only be
elicited after close investigation. From the history it is usually easy
to exclude scurvy and purpura. Repeated haemorrhages into a joint may
result in appearances which closely simulate those of tuberculous
disease. Recent haemorrhages into the cellular tissue often present
clinical features closely resembling those of acute cellulitis or
osteomyelitis. A careful examination, however, may reveal ecchymoses on
other parts of the body which give a clue to the nature of the
condition, and may prevent the disastrous consequences that may follow
incision.

These patients usually succumb sooner or later to haemorrhage, although
they often survive several severe attacks. After middle life the
tendency to bleed appears to diminish.

_Treatment._--As a rule the ordinary means of arresting haemorrhage are
of little avail. From among the numerous means suggested, the following
may be mentioned: The application to the bleeding point of gauze soaked
in a 1 in 1000 solution of adrenalin; prolonged inhalation of oxygen;
freezing the part with a spray of ethyl-chloride; one or more
subcutaneous injections of gelatin--5 ounces of a 2.5 per cent.
solution of white gelatin in normal salt solution being injected at a
temperature of about 100 F.; the injection of pituitary extract. The
application of a pad of gauze soaked in the blood of a normal person
sometimes arrests the bleeding.

To prevent bleeding in haemophilics, intra-venous or subcutaneous
injections of fresh blood serum, taken from the human subject, the
sheep, the dog, or the horse, have proved useful. If fresh serum is not
available, anti-diphtheritic or anti-tetanic serum or trade
preparations, such as hemoplastin, may be employed. We have removed the
appendix and amputated through the thigh in haemophilic subjects without
excessive loss of blood after a course of fresh sheep's serum given by
the mouth over a period of several weeks.

The chloride and lactate of calcium, and extract of thymus gland have
been employed to increase the coagulability of the blood. The patient
should drink large quantities of milk, which also increases the
coagulability of the blood. Monro has observed remarkable results from
the hypodermic injection of emetin hydrochloride in 1/2-grain doses.

THROMBOSIS AND EMBOLISM

The processes known as thrombosis and embolism are so intimately
associated with the diseases of blood vessels that it is convenient to
define these terms in the first instance.

#Thrombosis.#--The term _thrombus_ is applied to a clot of blood formed
in the interior of the heart or of a blood vessel, and the process by
which such a clot forms is known as _thrombosis_. It would appear that
slowing or stagnation of the blood-stream, and interference with the
integrity of the lining membrane of the vessel wall, are the most
important factors determining the formation of the clot. Alterations in
the blood itself, such as occur, for example, in certain toxaemias, also
favour coagulation. When the thrombus is formed slowly, it consists of
white blood cells with a small proportion of fibrin, and, being
deposited in successive layers, has a distinctly laminated appearance on
section. It is known as a _white thrombus_ or laminated clot, and is
often met with in the sac of an aneurysm (Fig. 72). When rapidly formed
in a vessel in which the blood is almost stagnant--as, for example, in a
pouched varicose vein--the blood coagulates _en masse_, and the clot
consists of all the elements of the blood, constituting a _red thrombus_
(Fig. 66). Sometimes the thrombus is _mixed_--a red thrombus being
deposited on a white one, it may be in alternate layers.

When aseptic, a thrombus may become detached and be carried off in the
blood-stream as an embolus; it may become organised; or it may
degenerate and undergo calcification. Occasionally a small thrombus
situated behind a valve in a varicose vein or in the terminal end of a
dilated vein--for example in a pile--undergoes calcification, and is
then spoken of as a _phlebolith_; it gives a shadow with the X-rays.

When infected with pyogenic bacteria, the thrombus becomes converted
into pus and a localised abscess forms; or portions of the thrombus may
be carried as emboli in the circulation to distant parts, where they
give rise to secondary foci of suppuration--pyaemic abscesses.

#Embolism.#--The term _embolus_ is applied to any body carried along in
the circulation and ultimately becoming impacted in a blood vessel. This
occurrence is known as _embolism_. The commonest forms of embolus are
portions of thrombi or of fibrinous formations on the valves of the
heart, the latter being usually infected with micro-organisms.

Embolism plays an important part in determining one form of gangrene, as
has already been described. Infective emboli are the direct cause of the
secondary abscesses that occur in pyaemia; and they are sometimes
responsible for the formation of aneurysm.

Portions of malignant tumours also may form emboli, and their impaction
in the vessels may lead to the development of secondary growths in
distant parts of the body.

Fat and air embolism have already been referred to.

ARTERITIS

_Pyogenic._--Non-suppurative inflammation of the coats of an artery may
so soften the wall of the vessel as to lead to aneurysmal dilatation. It
is not uncommon in children, and explains the occurrence of aneurysm in
young subjects.

When suppuration occurs, the vessel wall becomes disintegrated and gives
way, leading to secondary haemorrhage. If the vessel ruptures into an
abscess cavity, dangerous bleeding may occur when the abscess bursts or
is opened.

_Syphilitic._--The inflammation associated with syphilis results in
thickening of the tunica intima, whereby the lumen of the vessel becomes
narrowed, or even obliterated--_endarteritis obliterans_. The middle
coat usually escapes, but the tunica externa is generally thickened.
These changes cause serious interference with the nutrition of the parts
supplied by the affected arteries. In large trunks, by diminishing the
elasticity of the vessel wall, they are liable to lead to the formation
of aneurysm.

Changes in the arterial walls closely resembling those of syphilitic
arteritis are sometimes met with in _tuberculous_ lesions.

#Arterio-sclerosis# or #Chronic Arteritis#.--These terms are applied to
certain changes which result in narrowing of the lumen and loss of
elasticity in the arteries. The condition may affect the whole vascular
system or may be confined to particular areas. In the smaller arteries
there is more or less uniform thickening of the tunica intima from
proliferation of the endothelium and increase in the connective tissue
in the elastic lamina--a form of obliterative endarteritis. The
narrowing of the vessels may be sufficient to determine gangrene in the
extremities. In course of time, particularly in the larger arteries,
this new tissue undergoes degeneration, at first of a fatty nature, but
progressing in the direction of calcification, and this is followed by
the deposit of lime salts in the young connective tissue and the
formation of calcareous plates or rings over a considerable area of the
vessel wall. To this stage in the process the term _atheroma_ is
applied. The endothelium over these plates often disappears, leaving
them exposed to the blood-stream.

Changes of a similar kind sometimes occur in the middle coat, the lime
salts being deposited among the muscle fibres in concentric rings.

The primary cause of arterio-sclerosis is not definitely known, but its
almost constant occurrence, to a greater or less degree, in the aged
suggests that it is of the nature of a senile degeneration. It is
favoured by anything which throws excessive strain on the vessel walls,
such as heavy muscular work; by chronic alcoholism and syphilis; or by
such general diseases as tend to raise the blood-pressure--for example,
chronic Bright's disease or gout. It occurs with greater frequency and
with greater severity in men than in women.

Atheromatous degeneration is most common in the large arterial trunks,
and the changes are most marked at the arch of the aorta, opposite the
flexures of joints, at the mouths of large branches, and at parts where
the vessel lies in contact with bone. The presence of diseased patches
in the wall of an artery diminishes its elasticity and favours
aneurysmal dilatation. Such a vessel also is liable to be ruptured by
external violence and so give rise to traumatic aneurysm. Thrombosis is
liable to occur when calcareous plates are exposed in the lumen of the
vessel by destruction of the endothelium, and this predisposes to
embolism. Arterio-sclerosis also interferes with the natural arrest of
haemorrhage, and by rendering the vessels brittle, makes it difficult to
secure them by ligature. In advanced cases the accessible arteries--such
as the radial, the temporal or the femoral--may be felt as firm,
tortuous cords, which are sometimes so hard that they have been aptly
compared to "pipe-stems." The pulse is smaller and less compressible
than normal, and the vessel moves bodily with each pulsation. It must be
borne in mind, however, that the condition of the radial artery may fail
to afford a clue to that of the larger arteries. Calcified arteries are
readily identified in skiagrams (Fig. 65).

[Illustration: FIG. 65.--Radiogram showing Calcareous Degeneration
(Atheroma) of Arteries.]

We have met with a chronic form of arterial degeneration in elderly
women, affecting especially the great vessels at the root of the neck,
in which the artery is remarkably attenuated and dilated, and so friable
that the wall readily tears when seized with an artery-forceps,
rendering ligation of the vessel in the ordinary way well-nigh
impossible. Matas suggests infolding the wall of the vessel with
interrupted sutures that do not pierce the intima, and wrapping it
round with a strip of peritoneum or omentum.

The most serious form of arterial _thrombosis_ is that met with _in the
abdominal aorta_, which is attended with violent pains in the lower
limbs, rapidly followed by paralysis and arrest of the circulation.

THROMBO-PHLEBITIS AND THROMBOSIS IN VEINS

#Thrombosis# is more common in veins than in arteries, because slowing
of the blood-stream and irritation of the endothelium of the vessel wall
are, owing to the conditions of the venous circulation, more readily
induced in veins.

Venous thrombosis may occur from purely mechanical causes--as, for
example, when the wall of a vein is incised, or the vessel included in a
ligature, or when it is bruised or crushed by a fragment of a broken
bone or by a bandage too tightly applied. Under these conditions
thrombosis is essentially a reparative process, and has already been
considered in relation to the repair of blood vessels.

In other cases thrombosis is associated with certain constitutional
diseases--gout, for example; the endothelium of the veins undergoing
changes--possibly the result of irritation by abnormal constituents in
the blood--which favour the formation of thrombi.

Under these various conditions the formation of a thrombus is not
necessarily associated with the action of bacteria, although in any
of them this additional factor may be present.

The most common cause of venous thrombosis, however, is inflammation of
the wall of the vein--phlebitis.

#Phlebitis.#--Various forms of phlebitis are met with, but for practical
purposes they may be divided into two groups--one in which there is a
tendency to the formation of a thrombus; the other in which the
infective element predominates.

In surgical patients, the _thrombotic form_ is almost invariably met
with in the lower extremity, and usually occurs in those who are
debilitated and anaemic, and who are confined to bed for prolonged
periods--for example, during the treatment of fractures of the leg or
pelvis, or after such operations as herniotomy, prostatectomy, or
appendectomy.

_Clinical Features._--The most typical example of this form of phlebitis
is that so frequently met with in the great saphena vein, especially
when it is varicose. The onset of the attack is indicated by a sudden
pain in the lower limb--sometimes below, sometimes above the knee. This
initial pain may be associated with shivering or even with a rigor, and
the temperature usually rises one or two degrees. There is swelling and
tenderness along the line of the affected vein, and the skin over it is
a dull-red or purple colour. The swollen vein may be felt as a firm
cord, with bead-like enlargements in the position of the valves. The
patient experiences a feeling of stiffness and tightness throughout the
limb. There is often oedema of the leg and foot, especially when the limb
is in the dependent position. The acute symptoms pass off in a few days,
but the swelling and tenderness of the vein and the oedema of the limb
may last for many weeks.

When the deep veins--iliac, femoral, popliteal--are involved, there is
great swelling of the whole limb, which is of a firm almost "wooden"
consistence, and of a pale-white colour; the oedema may be so great that
it is impossible to feel the affected vein until the swelling has
subsided. This is most often seen in puerperal women, and is known as
_phlegmasia alba dolens_.

_Treatment._--The patient must be placed at absolute rest, with the foot
of the bed raised on blocks 10 or 12 inches high, and the limb
immobilised by sand-bags or splints. It is necessary to avoid handling
the parts, lest the clot be displaced and embolism occur. To avoid
frequent movement of the limb, the necessary dressings should be kept in
position by means of a many-tailed rather than a roller bandage.

To relieve the pain, warm fomentations or lead and opium lotion should
be applied. Later, ichthyol-glycerin, or glycerin and belladonna, may be
substituted.

When, at the end of three weeks, the danger of embolism is past,
douching and gentle massage may be employed to disperse the oedema; and
when the patient gets up he should wear a supporting elastic bandage.

The _infective_ form usually begins as a peri-phlebitis arising in
connection with some focus of infection in the adjacent tissues. The
elements of the vessel wall are destroyed by suppuration, and the
thrombus in its lumen becomes infected with pyogenic bacteria and
undergoes softening.

_Occlusion of the inferior vena cava_ as a result of infective
thrombosis is a well-known condition, the thrombosis extending into the
main trunk from some of its tributaries, either from the femoral or
iliac veins below or from the hepatic veins above.

Portions of the softened thrombus are liable to become detached and to
enter the circulating blood, in which they are carried as emboli. These
may lodge in distant parts, and give rise to secondary foci of
suppuration--pyaemic abscesses.

_Clinical Features._--Infective phlebitis is most frequently met with in
the transverse sinus as a sequel to chronic suppuration in the mastoid
antrum and middle ear. It also occurs in relation to the peripheral
veins, but in these it can seldom be recognised as a separate entity,
being merged in the general infective process from which it takes
origin. Its occurrence may be inferred, if in the course of a
suppurative lesion there is a sudden rise of temperature, with pain,
redness, and swelling along the line of a venous trunk, and a rapidly
developed oedema of the limb, with pitting of the skin on pressure. In
rare cases a localised abscess forms in the vein and points towards the
surface.

_Treatment._--Attention must be directed towards the condition with
which the phlebitis is associated. Ligation of the vein on the cardiac
side of the thrombus with a view to preventing embolism is seldom
feasible in the peripheral veins, although, as will be pointed out
later, the jugular vein is ligated with this object in cases of
phlebitis of the transverse sinus.

VARIX--VARICOSE VEINS

The term varix is applied to a condition in which veins are so altered
in structure that they remain permanently dilated, and are at the same
time lengthened and tortuous. Two types are met with: one in which
dilatation of a large superficial vein and its tributaries is the most
obvious feature; the other, in which bunches of distended and tortuous
vessels develop at one or more points in the course of a vein, a
condition to which Virchow applied the term _angioma racemosum venosum_.
The two types may occur in combination.

Any vein in the body may become varicose, but the condition is rare
except in the veins of the lower extremity, in the veins of the
spermatic cord (varicocele), and in the veins of the anal canal
(haemorrhoids).

We are here concerned with varix as it occurs in the veins of the lower
extremity.

_Etiology._--Considerable difference of opinion exists as to the
essential cause of varix. The weight of evidence is in favour of the
view that, when dilatation is the predominant element, it results from a
congenital deficiency in the number, size, and strength of the valves of
the affected veins, and in an inherent weakness in the vessel walls.
The _angioma racemosum venosum_ is probably also due to a congenital
alteration in the structure of the vessels, and is allied to tumours of
blood vessels. The view that varix is congenital in origin, as was first
suggested by Virchow, is supported by the fact that in a large
proportion of cases the condition is hereditary; not only may several
members of the same family in succeeding generations suffer from varix,
but it is often found that the same vein, or segment of a vein, is
involved in all of them. The frequent occurrence of varix in youth is
also an indication of its congenital origin.

In the majority of cases it is only when some exciting factor comes into
operation that the clinical phenomena associated with varix appear. The
most common exciting cause is increased pressure within the veins, and
this may be produced in a variety of ways. In certain diseases of the
heart, lungs, and liver, for example, the venous pressure may be so
raised as to cause a localised dilatation of such veins as are
congenitally weak. The direct pressure of a tumour, or of the gravid
uterus on the large venous trunks in the pelvis, may so obstruct the
flow as to distend the veins of the lower extremity. It is a common
experience in women that the signs of varix date from an antecedent
pregnancy. The importance of the wearing of tight garters as a factor in
the production of varicose veins has been exaggerated, although it must
be admitted that this practice is calculated to aggravate the condition
when it is once established. It has been proved experimentally that the
backward pressure in the veins may be greatly increased by straining, a
fact which helps to explain the frequency with which varicosity occurs
in the lower limbs of athletes and of those whose occupation involves
repeated and violent muscular efforts. There is reason to believe,
moreover, that a sudden strain may, by rupturing the valves and so
rendering them incompetent, induce varicosity independently of any
congenital defect. Prolonged standing or walking, by allowing gravity to
act on the column of blood in the veins of the lower limbs, is also an
important determining factor in the production of varix.

Thrombosis of the deep veins--in the leg, for example--may induce marked
dilatation of the superficial veins, by throwing an increased amount of
work upon them. This is to be looked upon rather as a compensatory
hypertrophy of the superficial vessels than as a true varix.

_Morbid Anatomy._--In the lower extremity the varicosity most commonly
affects the vessels of the great saphena system; less frequently those
of the small saphena system. Sometimes both systems are involved, and
large communicating branches may develop between the two.

The essential lesion is the absence or deficiency of valves, so that
they are incompetent and fail to support the column of blood which bears
back upon them. Normally the valves in the femoral and iliac veins and
in the inferior vena cava are imperfectly developed, so that in the
erect posture the great saphena receives a large share of the backward
pressure of the column of venous blood.

The whole length of the vein may be affected, but as a rule the disease
is confined to one or more segments, which are not only dilated, but are
also increased in length, so that they become convoluted. The adjacent
loops of the convoluted vein are often bound together by fibrous tissue.
All the coats are thickened, chiefly by an increased development of
connective tissue, and in some cases changes similar to those of
arterio-sclerosis occur. The walls of varicose veins are often
exceedingly brittle. In some cases the thickening is uniform, and in
others it is irregular, so that here and there thin-walled sacs or
pouches project from the side of the vein. These pouches vary in size
from a bean to a hen's egg, the larger forms being called _venous
cysts_, and being most commonly met with in the region of the saphenous
opening and of the opening in the popliteal fascia. Such pouches, being
exposed to injury, are frequently the seat of thrombosis (Fig. 66).

[Illustration: FIG. 66.--Thrombosis in Tortuous and Pouched Great
Saphena Vein, in longitudinal section.]

_Clinical Features._--Varix is most frequently met with between puberty
and the age of thirty, and the sexes appear to suffer about equally.

The amount of discomfort bears no direct proportion to the extent of
the varicosity. It depends rather upon the degree of pressure in the
veins, as is shown by the fact that it is relieved by elevation of the
limb. When the whole length of the main trunk of the great saphena is
implicated, the pressure in the vein is high and the patient suffers a
good deal of pain and discomfort. When, on the contrary, the upper part
of the saphena and its valves are intact, and only the more distal veins
are involved, the pressure is not so high and there is comparatively
little suffering. The usual complaint is of a sense of weight and
fulness in the limb after standing or walking, sometimes accompanied by
actual pain, from which relief is at once obtained by raising the limb.
Cramp-like pains in the muscles are often associated with varix of the
deep veins.

The dilated and tortuous vein can be readily seen and felt when the
patient is examined in the upright posture. In advanced cases, bead-like
swellings are sometimes to be detected over the position of the valves,
and, on running the fingers along the course of the vessel, a firm
ridge, due to periphlebitis, may be detected on each side of the vein.
When the limb is oedematous, the outline of the veins is obscured, but
they can be identified on palpation as gutter-like tracks. When large
veins are implicated, a distinct impulse on coughing may be seen to pass
down as far as the knee; and if the vessel is sharply percussed a fluid
wave may be detected passing both up and down the vein.