Alexis Thomson and Alexander Miles - Manual of Surgery

A >> Alexis Thomson and Alexander Miles >> Manual of Surgery

Pages:
1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 | 11 | 12 | 13 | 14 | 15 | 16 | 17 | 18 | 19 | 20 | 21 | 22 | 23 | 24 | 25 | 26 | 27 | 28 | 29 | 30 | 31 | 32 | 33 | 34 | 35 | 36 | 37 | 38 | 39 | 40 | 41 | 42 | 43 | 44 | 45 | 46 | 47 | 48 | 49

_In the septum of the nose, the nasal bones, and the hard palate_,
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 (ozoena), 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 taenia
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 vertebrae, 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 haemorrhages,
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 oedema. These symptoms are due to a sub-periosteal haemorrhage, 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. Haemorrhages 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 vertebrae, 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 vertebrae 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 diploe 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.