Manual of Surgery Volume I Part 50

_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 cuc.u.mber is deserved. In some cases the tibia is actually increased in length as well as in girth.

[Ill.u.s.tration: 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-sh.e.l.l 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 sc.r.a.ped 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.

CONSt.i.tUTIONAL DISEASES ATTENDED WITH LESIONS IN THE BONES

These include rickets, scurvy-rickets, osteomalacia, ost.i.tis deformans, osteomyelitis fibrosa, fragilitas ossium, and diseases of the nervous system.

RICKETS

Rickets or rachitis is a const.i.tutional disease a.s.sociated 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 cla.s.ses 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 a.s.similated. 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.

[Ill.u.s.tration: 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, princ.i.p.ally 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 att.i.tude 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--c.o.xa 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 att.i.tudes.

_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 p.r.o.nounced 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 a.s.sumes 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 (c.o.xa 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 r.e.t.a.r.ded, 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 cla.s.ses 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 ma.s.saged. In children of five or six and upwards, the prospect of natural straightening is a diminis.h.i.+ng 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 ma.s.sage 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 p.u.b.erty, 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 cla.s.ses. 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 s.h.i.+ny, and there may be some dema. These symptoms are due to a sub-periosteal haemorrhage, and a.s.sociated 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-s...o...b..tics 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 a.s.sociated with New Growths in the Skeleton._--When _secondary cancer_ is widely distributed throughout the skeleton, it is a.s.sociated 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 sh.e.l.l, and may break on the slightest pressure; the urine contains alb.u.mose, a substance resembling alb.u.men 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).

#Ost.i.tis 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 ca.n.a.l 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 a.s.sociated 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).

[Ill.u.s.tration: FIG. 134.--Changes in the Skull resulting from Ost.i.tis 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 att.i.tude 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.

[Ill.u.s.tration: FIG. 135.--Cadaver, ill.u.s.trating the alterations in the Lower Limbs resulting from Ost.i.tis Deformans.]