Alexis Thomson and Alexander Miles - Manual of Surgery

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[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 aet. 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 haemorrhages 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 fossae
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 vertebrae. 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 aet. 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 aet. 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 maxillae; these bones are
enlarged in all directions and project on the face, and the nasal fossae
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 foetal 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 aet. 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 aet. 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 oedema 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 haemorrhages, 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.