2 F I S I O T E R A P I E DESEMBER 1974 This work has been supported in part by a grant fro m the Medical Research Council o f South Africa, and by the University o f Pretoria. Understanding Scoliosis by G. F. D O M M ISSE M .D ., Ch.M ., F.R.C.S.(Edin.) Department o f Orthopaedics, University of Pretoria, Pretoria. Scoliosis is a deformity, not a disease. The cause o f the deformity is totally unknown, and in about 60 per cent of cases the child is in all other respects healthy at the time of the development o f the abnorm al curve. In the remaining 40 per cent o f cases, other factors such- as congenital m alform ations (about 17,5 p ercen t), paralytic lesions (about 15 per cent), arid Von Recklinghausen’s neurofibrom atosis (about 3,5 per cent), are concom itant diseases. The m anner in which they operate in producing a curved spine is not always clear. A turning point in the management o f scoliosis came when the natural history of the untreated curvature was recorded ‘by Jam es ‘(1954), who conducted a follow-up study of two hundred and forty-one m ature cases of idiopathic scoliosis, and-of. sixty-seven cases in which skeletal maturity had not yet been attained. The disastrous results of the untreated deformities were no longer in doubt, and the vital need for early diagnosis was clear. In every' instance there had been a stage in which the deformity was mild and at which effective treatment could have been easily rendered. The salient features which enable early recognition have been described by the author elsewhere (Dommisse, 1970).' John C obb (1948), described an ‘outline for the study of scoliosis’, and a method for measuring the curve (Fig. 1), which he attributed to Ferguson. T he method enjoys universal use and has added immeasurably to the study of the problem. It affords an easily comprehensible index of progress, and of the results o f treatm ent as well as o f the prognosis in the individual case. A ttem pts at operative correction achieved little success until 1931, when Hibbs, Risser and Ferguson described a technique o f posterior spinal fusion which today still forms the foundation on which surgical treatm ent is based. The next m ajor step was by H arrington (1962) who intro­ duced a system of instrum entation which not only perm itted instant correction of the deformity, but also provided a surgical im plant which acted as an internal splint. When combined with H ibbs’ posterior fusion, the H arrington method of operative treatm ent became the standard method o f surgical approach. In spite o f more recent surgical advances which include the anterior approach o f Dwyer & Schafer (1974), the backbone o f treatm ent remains non-operative. It is by conservative measures only that a return to norm al may be anticipated, and it is only in the case o f early diagnosis that conservative of Ferguson, as described by Cobb (1948). R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. ) measures offer a prognosis which is good. The routine scanning o f children during their tender years at school is niandatory, and in some regions is already being applied. DECEMBER, 1974 Fig. 2: T H E STRU C TU R AL CURVES IN T H E MACERATED S P IN E O F A SEVERE T H O R A C IC SCOLIOSIS. (Photograph by courtesy of the publishers. Messrs. E. & S. L IV IN G ST O N E LTD ., L O N D O N , M67, and of • the Jauthor. R E FE R E N C E : Monograph entitled ‘SC O L IO SIS’. A U T H O R : J . I. P . .TAM E S . (N ote: The black m arker dots have been added by the present author.) THE NATURE O F T H E BASIC D E FO R M IT Y : The structural curve in the m acerated spine (Fig. 2), illustrates graphically the distortion o f the bony cage of the chest and also o f the abdom inal cavity. It needs little 'Tiagination to appreciate the degree to which the viscera ( I compressed. -T 'h e complicated nature o f the 3-dimensional curves is apparent, and a clear understanding o f each com ponent of the deformity is essential to an appreciation of the problem. In the first instance, the lateral bend is most obvious, and it constitutes a physical mal-alignment o f the spine. There is a translation of the vertebrae in the curve, away from the middle line o f the trunk. The occipital protruberance, which in the norm al erect posture is placed directly over the middle of the sacrum, is displaced towards the side o f the convexity of the m ajor curve. The intervertebral discs are no longer horizontally aligned, but are oblique in varying degree. The force o f gravity which is directed vertically when the indivi­ dual stands erect, exerts a shearing force on the discs which is harmful and which leads to degenerative changes. In a nutshell, there is a state o f disequilibrium in the scoliotic spine, which is quite contrary to nature, and which results in physical spinal disability. There is'a second com ponent which is an integral part of the curve, namely rotation o f the vertebral bodies on a vertical axis. Consider the thoracic curve (Fig. 2), and observe the position of the b lack d o t placed at the middle point o f the anterior surface of the vertebral body. At the apex of the curve, the black dot is maximally displaced towards the convexity and the vertebral.body is maximally rotated. 3 The effect of the rotation deformity on the thoracic cage is devastating when severe (Fig. 3). The pleural cavity on the side of the convexity is effectively obliterated and the ribs are closely applied to the bodies of the vertebrae. The rib hump, which in severe cases forms a sharp edge and is known as a ‘razor back’, adds a distressing cosmetic disability and indicates an irreversible change. The diaphragm is embarrassed on the same side because the origin and- insertion o f the muscle are closely apposed; in effect, the diaphragm lies in loose folds on one side and is stretched on the other, adding to the functional loss. Each com ponent o f the scoliotic deformity is o f a structural, not functional nature which means that the patient is unable to restore or reverse the curve by voluntary effort. A third com ponent is hyper-extension of the spine into a position o f lordosis in the normally kyphotic thoracic spine, and o f hyper-lordosis in the lum bar region. When a black dot is placed at mid-point on each anterior vertebral surface, then a series of dots when linked together will be seen to outline the convex arc o f the curve (Fig. 3). Expressed in other words, the line joining the black dots runs parallel with the margins of the anterior longitudinal ligament, which is stretched across the arc o f the curve, and serves to emphasize the fact th at the spine is indeed hyper-extended. The effect of the lordotic curve in the thoracic spine is devastating in severe cases, for the vertebral bodies are literally pushed forw ard to approxim ate the sternum and the mediastinal space is reduced o r obliterated (Fig. 3). Fig. 3: Diagrammatic representation o f a bird’s eye - view showing a silhouette of the skull o f a patient with severe thoracic scoliosis, convex to the right side, (a) Vertebral body rotated towards the side of the convexity of the curve, (b) The rib hump, (c) The obliterated pleuralcavity on right, (d) The aorta, (e) The unicameral pleural cavity which contains left and right lungs and mediastinal contents including the heart. . (f) The sternum. P H Y S I O T H E R A P Y R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. ) 4 F I S I O T E R A P I E OESEMBER 1974 Cardiac em barrassm ent and c a rd io p u lm o n a ry insuffi­ ciency are unfortunate and more serious complications which account for prem ature death during the third or fourth decade in an appreciable percentage of cases. W inter colds and flu are a special hazard. In lum bar curves the effects are less serious and the life of the patient is not threatened. The com plaint in these cases is o f lum bar backache which increases to intolerable proportions and may necessitate operation for spinal fusion. There is loss o f vertebral alignment and degeneration o f the intervertebral discs, leading to instability (Fig. 4). Fig. 4 : Radiograph of a forty-eight-year-old white female with lumoar scoliosis. Instability is severe at L.3/L.4 level on account of intervertebral disc degeneration due to shearing strain. Lordo-scoliosis is much more com m on than kypho­ scoliosis, and is the typical deform ity seen in idiopothic cases. Kypho-scoliosis is relatively rare and the kyphotic or clasp-knife com ponent is the significant part of the curvature, not the scoliosis. From the point of view o f management, the scoliotic com ponent may be ignored. Kypho-scoliosis is usually the result o f a congenital anom aly such as an absent vertebral body, o r a congenital hemi-vertebra in which the anterior half is missing and the spinal colum n is ‘buckled’ forward, or in which there is congenital failure of segm entation (which means fusion), of two o r more vertebral bodies. In the latter event, the grow th centre for the vertebral body is absent, while the growth centres for the neural arches are present. T he latter continue to grow and to produce a progressive deformity. Kypho-scoliosis is associated with a grave risk o f cord compression and paraplegia, because there is kinking and narrow ing o f the spinal canal at the level o f the lesion. In lordo-scoliosis by contrast, paraplegia is an uncommon spontaneous com plication, as the spinal canal is widened in the region of the curve. Less com m on causes o f kyphoscoliosis are neurofibro­ matosis and paralytic lesions o f all etiological types. In the latter there is collapse o f the ‘floppy spine’, brought about by sitting erect. In both the lordotic and the kyphotic deformities, there is a compromise of lung function, and the vital capacity is reduced. An additional factor in the reduction is the effect o f the spinal deformity on the ribs. O n the convex side (Fig. 2), the ribs are more obliquely placed and the costo­ transverse and costo-vertebral joints are mal-aligned. On the concave side, the ribs are crowded and the rib-vertebral body angle is increased. Each o f these joints is a synovial lined joint cavity, in which the range o f movements is adversely affected. In paralytic cases, the intercostal muscles are generally involved to the extent that there is paradoxical breathing. In non-paralytic cases, the intercostal muscles and the diaphragm are functionally paralysed because of the defor­ mity o f the rib cage. N o r is respiratory insufficiency the only disability a s s ^ ciated with scoliosis. R ather, the severe case should be regarded as a ‘triple cripple’, who suffers from cardio­ pulm onary, cosmetic and physical (spinal) disability. There is yet a further, more sinister complication which is that of neurological loss and paraplegia. TREA TM EN T: Conservative measures rem ain the background of all treat­ ment o f scoliosis, and can be effectively applied when early diagnosis is made. The Milwaukee brace has became standard practice, and the balanced traction of Cotrel, which is applied only at night, has evoked increasing attention in recent years. Perhaps the most im portant aspect is the team-work which is implied. The physiotherapist is an im portant member of a team which includes a physician, surgeon, anaesthetist, pathologist, and nursing sister in the intensive care unit, the operating room, the ward and the out-patient department. There are the occupational therapist, the social worker and the orthotist. T here are the radiographer and the clinical photographer. There are the parents and the school teacher whose understanding and co-operation are vital. Last but not least there are the registrar, the house doctor and the plaster room assistant. The role of each member of the team is im portant in the; end-result to be gained. The team is only as strong as weakest member. T he attention o f the physiotherapist is directed at and a t the strengthening of such muscles as are weak disuse or paralysis. The joints of the spine and limbs are to be put their norm al range of movements at frequent intervals e during the pursuit o f the child’s norm al activities. The la instruction must of course bend to the rules o f reason. C ontractures o f joints are to be prevented, and recognised when already present. Efforts at their correction are made in consultation with the surgeon. Pulm onary function in the more severe cases receives top priority and the progress o f the child is recorded periodically So that the child may not be given false encouragem ent by at kind, soft-hearted clinician who is lenient in his or her interpretation o f the tests for respiratory efficiency, the writff offers a single test which cannot be faulted nor the resulli altered. T he test consists o f the under-water holding o f the breath for as long as the child is able, after being allowed to hyper ventilate for as long as desired. T he subm ersion test can b( performed only in selected instances, but it is m ore valuable and m ore significant than the standard tests which are valid only in so far as the patient is co-operative. In carefully recorded cases, the fluctuations in respiratofl efficiency are often rem arkable, and when retrogression I R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. ) DECEMBER, 1974 P H Y S I O T H E R A P Y 5 noted it may be attributable to non-co-operation and lack of discipline. Operative procedures are ineffective in improving respiratory function. The value of exercises in controlling an d /o r correcting scoliotic curves is no longer a contentious m atter. There has been no exercise devised which improves or corrects scoliosis. Nevertheless, exercises are valuable for the scoliotic just as for the healthy individual, and the physiotherapist includes exercises in her repertoire o f treatm ent, along w ith other modalities. In the pre-operative and post-operative phases o f surgical care, the physiotherapist plays a vital role not only in breathing exercises but also in the application o f Inter­ mittent Positive Pressure R espiration (l.P .P .R .) and in the management o f endo- an d intra-tracheal intubation. The controversial schoolbook bag an d suitcase deserve mention, in order th at m isunderstanding may be allayed. When a norm al healthy child is overloaded, the effect is to sag and collapse which is an intolerable situation. An immediate reduction in the load is m andatory. Alternately, with a load which is tolerable, the individual will respond not fey sagging o r collapsing, but by an unconscious bracing 't/I the muscles so th a t the part under load straightens up rather than collapses. Van N iekerk (1970) showed th a t a load o f 100 kilogram s placed on the head of a porter causes the norm al curves of the neck to straighten out, provided the porter is able. A heavy suitcase when carried in one hand causes the shoulder to brace on th a t side an d to become elevated, while the tru n k is bent over to the other side in order to maintain equilibrium . A heavy book bag slung between the shoulders causes a child to lean forw ard in order to continue his or her forw ard progress. If to o heavy, it will cause the child to fall over backw ards—which is an intol­ erable situation. The im portant question is w hether or n o t the bearing of a load which the child can manage, but which exceeds physio­ logical limits, causes harm . There are many factors involved including the age and weight o f the child, the period over which the load is carried, the m anner in which it is borne, the physical condition o f the child. N ewm an (1974) quoted a series of military trainees who developed a stress fracture of the pars inter-articularis o f a lum bar vertebra after carrying a pack weighing 56 lbs. over a distance o f 40 miles. Lamy, K raus and Bazergui (1974) showed that mechanical loads applied to the vertebrae o f fresh cadaver specimens in a manner which resembled loading in the living individual, while the spine was forw ard flexed, resulted in the same lesion quoted by Newman. Schultz and co-workers (1974), also King Liu and associates (1974) studied methods and .'■esults of compression an d shear loads on the intervertebral fis c s and yielded evidence th at excessive compression forces '^Sause radial tears o f the bony end plates o f the vertebral bodies. They showed th at the bony end plates are more susceptible than the cartilage rings, and th at a much greater force is needed to produce compression fracture o f the verte­ bral bodies. Shear forces are more harm ful th an compression forces. The question o f injury to the cartilaginous epiphyseal rings of the thoracic and lum bar spine in the growing child under conditions o f loading which exceed physiological limits has not yet received detailed attention and once again there are many variable factors. If for instance the child bends forw ard by utilising the hip joints, and at the same time m aintains a straight (rigid) spine, then it is unlikely that there will be any damage. At this stage o f our knowledge, an d bearing in mind the various ages, sizes and requirem ents o f school children, it is necessary to rule th at in all cases the weight of book bags and satchels be within physiological limits irrespective of whether they are borne in the hand o r on the shoulders. The conclusion is th at in healthy children, book bags and suitcases do not cause harm , provided the weight is within physiological limits. In the scoliotic child, and in paralytic or other abnorm alities, the carrying o f school bags should be controlled and limited, under d octor’s instruction. C O N C L U S IO N S : The results o f neglect o f scoliosis are disastrous and often fatal. T he neglected patient is subject to triple crippledom , and a fourth m ore sinister disability may be paraplegia. E arly recognition o f the case is m andatory, and treatm ent must be instituted from the mom ent of diagnosis. Conservative treatm ent may be effectively applied with relative ease in the early case and offers the only chance of ‘resolution’, th a t is, return to norm al. Surgery in scoliosis is largely a salvage procedure, the ‘second best’, and a m odality which has a bone-grafted, fused spine as the final objective. Clearly a fused spine is not as good as a mobile one. Physiotherapeutic modalities of treatm ent along guided lines are vital to the welfare o f the patient, and the physio­ therapist is a key member o f th e team along with other medical and param edical colleagues. The routine exam ination o f school children is recom m en­ ded, an d where already carried ou t has enabled early diagnosis in many cases. REFE R E N C E S 1. COBB, J. R . (1948): O utline for the Study o f Scoliosis. The American Academy o f O rthopaedic Surgeons. Instructional C ourse Lectures. Vol. V, 261. 2. C O T R EL , Y. (1971): T he E .D .F . Technique. 3rd A nnual, P ost-graduate C ourse on the M anagem ent & C are o f the Scoliosis Patient. New Y ork O rthopaedic H ospital, Colum bia-Presbyterian Medical Centre. 3. D O M M ISSE , G . F. (1970): ‘The M anagem ent of Scoliosis’. G .P. Review Article. South African Medical Journal, Vol. 44, 1331-1335. 4. D W Y E R , A. F . and SC H A F E R , M. F. (1974): A nterior A pproach to Scoliosis. R esults o f T reatm ent in fifty-one Cases. Journal o f Bone & Joint Surgery, 56B, 2, 218. 5. H A R R IN G T O N , P. R . (1962): Correction and Internal Fixation by Spine Instrum entation. Journal of Bone and Joint Surgery. 44-A, 4, 591. 6. HIBBS, R . A., R ISSER, J. C. & F E R G U S O N , A. B. (1931): Scoliosis T reated by the Fusion O peration. An E nd Result Study o f Three H undred and Sixty Cases. The Journal o f Bone and Joint Surgery, X III, 1, 91. 7. JA M E S, J. I. P. (1954): Idiopathic Scoliosis. The Prognosis, Diagnosis and O perative Indications related to Curve Patterns and the Age o f Onset. The Journal of Bone an d Joint Surgery. 36-B, 36. 8. K IN G L IU , Y „ R A Y , G. & H IR S C H , C. (1974): ‘The Resistance o f the L um bar Spine to D irect Shear’. Paper read at F irst Meeting o f International L um bar Spine Society, M ontreal. 9. LA M Y , C., K R A U S, H . & B A Z E R G U I, A. (1974): ‘Deflection o f the N eural Arch D uring Forw ard Flexion. Paper read at F irst M eeting of International L u m b ar Spine Society, M ontreal. 10. N EW M A N , P. (1974): D uring discussion o f lum bar spine mechanics, at F irst M eeting o f L um bar Spine Society, M ontreal. 11. SC H U L TZ , A., K U L A K , R „ B EL Y TSC H K O , T. and G A L A N T E , J. (1974): ‘Biochemical Characteristics of Vertebral M otion Segments and Intervertebral Discs’. P aper read at First Meeting o f International L um bar Spine Society, M ontreal. 12. VAN N IE K E R K , J. (1970): Personal Com m unication. THA NK S AND A CK N O W L ED G E M E N T S: To the D irector o f H ospital Services, Transvaal Provincial A dm inistration, D r. H . A. G rove ; the superintendent, H . F. Verwoerd H ospital, O rthopaedic Section, D r. E. van W yngaard; the D ean o f the Faculty o f Medicine, University o f Pretoria, Professor H . W. Snym an; the H ead o f the D epartm ent o f O rthopaedics, H . F. Verwoerd H ospital, Pretoria, D r. I. S. de W et; the Principal Photographer, O rthopaedic D epartm ent, Pretoria, Mrs. T. T euben; my secretary, Mrs. S. Eagar. R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. )