orthopaedic aspects hundred spina bifida · analysis ofthe defectsin twenty-three cases ofmyelocele...

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201 THE ORTHOPAEDIC ASPECTS OF ONE HUNDRED CASES OF SPINA BIFIDA By T. L. CARR, F.R.C.S., Aberdeen, Scotland. From the Institute of Orthopaedics and the Royal National Orthopaedic Hospital, London :::::: ...·.. :liiiii: ==== ======= '.... '"':4 I iaii; l·~sil is.. FIG. I .;3. ·-· ;··, ··;·.··I jL ..-·· ;·:: .·I,·: ·.*i .··:.; .-.-:,iiii ·,. FIG. 2 Of the many papers on the subject of spina bifida few are concerned mainly with the ortho- paedic problems it presents. Macnab (I954) has indicated that, particularly since the advent of antibiotics, the early mortality rate is not as high as formerly, so that although the treatment of associ- ated hydrocephalus is still not satisfactory, more patients may survive to require orthopaedic care. It is my purpose to describe some of the problems encountered in one hundred patients with spina bifida who sought advice at the Royal National Orthopaedic Hospital. Many had already been under treatment at other hospitals for some years, and this made some degree of selection of patients inevitable. Forty-two of these patients were male, and fifty-eight were female; four had a family history of the condition. Only a few had mild static hydrocephalus. Quite apart from the well- known sequelae of the neurological lesions such as club foot and trophic ulceration (Fig. i), and related conditions such as dysplasia of the hipjoint (Fig. 2), a wide variety of associated anomalies was found (Table i). In the five patients with cafe-au-lait type of skin pigmentation no other evidence of neurofibro- matosis was found. Kessel (I95 ) has discussed the association of lateral.intrathoracic extrapleural meningocele..with spinal deformity. in. neurofibm- TABLE I Associated Anomalies in One Hundred Patients I. Lumbo-sacral anomalies such as vertebrae of transitional type and vertebrae with ab- normally shaped bodies, spinous processes and neural arches 32 2. Scoliosis (i8 congenital, 2 idiopathic) 20 3. Congenital fusion of vertebrae, involving from 2-12 segments 18 4. Congenital lumbo-sacral spondylolisthesis 5 5. Hypoplasia of sacrum 5 6. Abnormal ribs (fusion, absence or deformity) 7 7. Syndactyly of fingers 2 8. Sacro-coccygeal dermoid I 9. Cafe-au-lait type ofpigrnentation of trunk and limbs 5 Io. Osteochondritis juvenilis (hip 3, spine 3) 6 I. Congenital absence of pectoralis major 2, thyroglossal cysts 3, congenital absence of vagina i, imperforate anus 2, and macro- mastia I 9 Total I o matosis, but no caseresembling those described by him was encountered. The Spinal Lesion For the purpose of describing the spinal lesion the patients have been divided. into .two grups:-- (I) Twenty-three. patients with -nyelocele.:. or ". menin' .oe ... . ,.. .. . :',men.ngoQele..' .... . . ....: copyright. on June 17, 2020 by guest. Protected by http://pmj.bmj.com/ Postgrad Med J: first published as 10.1136/pgmj.32.366.201 on 1 April 1956. Downloaded from

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Page 1: ORTHOPAEDIC ASPECTS HUNDRED SPINA BIFIDA · Analysis ofthe Defectsin Twenty-three cases ofMyelocele andMeningocele TABLE2 Number of vertebrae involved Numberofpatients I 2 2 3 4 2

201

THE ORTHOPAEDIC ASPECTS OFONE HUNDRED CASES OF SPINA BIFIDA

By T. L. CARR, F.R.C.S., Aberdeen, Scotland.From the Institute of Orthopaedics and the Royal National Orthopaedic Hospital, London

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Of the many papers on the subject of spinabifida few are concerned mainly with the ortho-paedic problems it presents. Macnab (I954) hasindicated that, particularly since the advent ofantibiotics, the early mortality rate is not as high asformerly, so that although the treatment of associ-ated hydrocephalus is still not satisfactory, morepatients may survive to require orthopaedic care.It is my purpose to describe some of the problemsencountered in one hundred patients with spinabifida who sought advice at the Royal NationalOrthopaedic Hospital. Many had already beenunder treatment at other hospitals for some years,and this made some degree of selection of patientsinevitable. Forty-two of these patients were male,and fifty-eight were female; four had a familyhistory of the condition. Only a few had mildstatic hydrocephalus. Quite apart from the well-known sequelae of the neurological lesions such asclub foot and trophic ulceration (Fig. i), andrelated conditions such as dysplasia of the hipjoint(Fig. 2), a wide variety of associated anomalies wasfound (Table i).

In the five patients with cafe-au-lait type of skinpigmentation no other evidence of neurofibro-matosis was found. Kessel (I95 ) has discussedthe association of lateral.intrathoracic extrapleuralmeningocele..with spinal deformity. in. neurofibm-

TABLE I

Associated Anomalies in One Hundred PatientsI. Lumbo-sacral anomalies such as vertebrae of

transitional type and vertebrae with ab-normally shaped bodies, spinous processesand neural arches 32

2. Scoliosis (i8 congenital, 2 idiopathic) 203. Congenital fusion of vertebrae, involving

from 2-12 segments 184. Congenital lumbo-sacral spondylolisthesis 55. Hypoplasia of sacrum 56. Abnormal ribs (fusion, absence or deformity) 77. Syndactyly of fingers 28. Sacro-coccygeal dermoid I9. Cafe-au-lait type ofpigrnentation of trunk and

limbs 5Io. Osteochondritis juvenilis (hip 3, spine 3) 6I. Congenital absence of pectoralis major 2,

thyroglossal cysts 3, congenital absence ofvagina i, imperforate anus 2, and macro-mastia I 9

Total I o

matosis, but no caseresembling those described byhim was encountered.

The Spinal LesionFor the purpose of describing the spinal lesion

the patients have been divided.into .two grups:--(I) Twenty-three. patients with -nyelocele.:. or". menin' .oe ....,.... . :',men.ngoQele..' .... . . ....:

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202 POSTGRADUATE MEDICAL JOURNAL April 1956

(2) Seventy-seven patients with spina bifidaocculta.

GROUP I: Myelocele and MeningoceleAll but two of the twenty-three patients had

extensive involvement of the nervous system.Most of them had had operations on the myeloceleor meningocele within a few months of birth butthere was no definite evidence of neurologicalimprovement in any case. The neural arch defectusually involved five or more segments and in onepatient no less than twelve arches were defective.Both laminae were often completely absent, and thesite of the lesion was most frequently lumbo-sacral.When the defect was small it was nearly alwayscentrally placed (Tables 2 to 5).Analysis of the Defects in Twenty-three cases of Myelocele

and MeningoceleTABLE 2

Number of vertebrae involved Number of patientsI 22 34 25 46 47 28 39 2

12 I

TABLE 3Size of neural arch defect Number of vertebrae involvedI. Less than I cm. in

width on the radio-graph 33

2. Subtotal 143. Total 8I

TABLE 4Site of lesion Number of patients

Cervical IThoraco-lumbo-sacral 2Lumbar 4Lumbo-sacral 15Sacral I

TABLE 5Site of Number of vertebrae involved

small neural arch defectsI. Central 302. Right laminar I3. Left laminar 2

GROUP II: Spina Bifida OccultaIn this group half of the patients showed in-

volvement of only one or two vertebrae while theremainder showed defects as extensive as thosefound in myelocele and meningocele. When onlyone segment was involved it was the first sacral inseventy-five per cent. of cases. The laminar defectswere smaller in a higher proportion of the patientsthan in Group I, but in the extensive lesions thelaminae were usually completely absent, just as inthe myelocele group. The commonest site of the

lesion was again lumbo-sacral and the smallerdefects were usually placed centrally (see Tables6 to 9).Analysis of the Defects in Seventy-seven cases of Spina

Bifida OccultaTABLE 6

Number of vertebrae involved Number of patients*i 282 II3 74 45 96 77 58 39 2

II I*This was the first sacral segment in 2I of the 28

patients.TABLE 7

Size of neural arch defect Number of vertebrae involvedI. Less than i cm. in

width on the radio-graph 141

2. Subtotal 83. Total 113

TABLE 8Site of lesion Number of patients

Cervical 2Cervico-thoracic and sacral iCervical and lumbo-sacral IThoracic 2Thoraco-lumbar IThoraco-lumbo-sacral 3Lumbar 7Lumbo-sacral 37Sacral 23

TABLE 9Site of Number of vertebrae involved

small neural arch defectsI. Central II82. Right laminar 123. Left laminar II

Cutaneous and subcutaneous changes werepresent in just over fifty per cent. of the patientswith spina bifida occulta (Table IO); in theremainder the skin and subcutaneous tissues of theback were normal, although in five there was abony gap large enough to be easily palpable.

TABLE I 0Cutaneous and subcutaneous evidenceof spina bifida occulta in 39 patients Number ofpatientsHairy patch 22Haemangioma and telangiectasis I6Palpable bony defect i6Scarring 9Lipoma 8Lumbar linear scleroderma 3-Dimples 3Pigmentation of skin I

Of the seventy-seven patients in Group I1- fiftyhad neurological lesions. The presence either of

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April 1956 CARR: The Orthopaedic Aspects of One Hundred Cases of Spina Bifida 203

cutaneous and subcutaneous changes or of ex-tensive laminar defects indicated that involvementof the nervous system was likely, although theywere occasionally present when the central nervoussystem was normal. Of those cases withoutsuperficial evidence of spina bifida fifty per cent.had neurological lesions (Tables i and I2).

TABLE IINumber of patients

Cutaneous and subcutaneous changeswith neurological lesion .. .. 3

Cutaneous and subcutaneous changeswith normal C.N.S. . .. 8

No superficial changes with neuro-logical lesion ..... 19

No superficial changes with normalC.N.S. .. .. .. I9

TABLE 12Number of Number of patientsvertebrae with neurological Number of patientsinvolved lesion with normal C.N.S

1 14 142 4 73 7 o4 4 o5 86 6 I7 3 28 2 I9 2 0

II O I

Tables 6 and 12 indicate that about fifty percent. of the patients with spina bifida occulta ofthefirst sacral segment had neurological lesions.This high figure is of course the result of theselection of patients to which I have alreadyreferred. By contrast Campbell Golding (1950)has found spina bifida occulta of the first sacralsegment in nineteen per cent. of adult patientssubjected to routine radiological examination.

Neurological LesionsNeurological examination of all the hundred

patients showed the following features:-I. Nervous involvement was usually more severe

and more extensive in the myelocele andmeningocele group but equally severe dis-turbances were found occasionally in spinabifida occulta.

2. The lower lumbar and sacral segments of thecord were most frequently involved.

3. Sensory and motor paralysis usually occurredtogether but either could be present alone.

4. Both the sensory and the motor paralysis wereusually bilateral. In the unilateral cases thenumbers of right and left lower limbs affectedwere equal.

5. Motor paralysis was usually of the lower motorneurone type but in thirty per cent. of thepatients signs of pyramidal tract involvementwere present. In most patients the paralysis

was incomplete; certain muscles tended toescape and various patterns of paralysis thusemerged.

Paralysis Affecting the HipTwenty-one patients had paralysis of muscles

controlling the hip. In five it was unilateral,slight and accompanied by a similar degree ofparesis in the rest of the limb. In sixteen theparalysis was severe, bilateral and accompanied byextensive paralysis more distally.Three main groups were observed:-

I. Paralysis affecting all muscles uniformly butvarying in severity from case to case.

2. Paralysis in which certain muscles were strongand others weak.

3. Paralysis associated with dysplastic changesin the hip (Table I3).

TABLE 13Condition of the hip in 21 patients Number of hipsNormal 5Uniformn hip paralysis 14Paralysis with muscle imbalance I2Paralysis with dysplastic changes io

41(One patient had a disarticulation through the hip at the

age of fourteen years for a flail limb)The first group showed three main clinical

pictures:-(a) Slight general weakness, all muscles being

graded about 4 on the M.R.C. scale. These patientseither had no complaints or felt that the affectedhip was somewhat weaker than the other.

(b) A moderate degree of paralysis causing aTrendelenberg type of limp from abductorweakness.

(c) Severe paralysis with the joint completely oralmost completely flail. In none of five such hipswas dislocation present.

In the second group paralysis was again variablein severity and the striking feature was the existenceof muscle imbalance. In two patients the flexorswere normal, the extensors weak and the othermuscles fairly evenly balanced. Flexion de-formity of the hip ranging from 50 to 60 degieeswas present. In the remaining ten hips in thisgroup the flexors and especially the adductorswere stronger than their antagonists-a causeofspontaneous dislocation of the hip described byWatson-Jones (I926) and Hart (I928). In five ofthese hips the joint was dislocated (Fig. 3). Infour of the other five the short rotator muscleswere not severely paralysed, their grading beingbetween 3 and 4. This suggests that these musclesnormally may play an important part in main-taining the head ofthe femur within the acetabulum,

In the third group dysplasia of the hip waspresent as well as paralysis, four presenting the

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4 POSTGRADUATE MEDICAL JOURNAL April 1956

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FIG. 3.-Bilateral paralytic dislocation of the. hip.

typical appearance of congenital dislocation andsix having a shallow socket with or without mal-formation of the acetabular rim.The type of paralysis was again that of muscle

imbalance of variable severity. Fig. 2 shows asubluxated hip with an inadequate socket in apatient with almost complete paralysis of thelower limbs. Fig. 4 shows the hips of a girl ofsix years who had a large myelocele and severeparalysis of the hip muscles. The flexors andadductors were just a little stronger than their

antagonists and the acetabula were well formed.Five years later (Fig. 5) the left hip was begin-ning to subluxate and the acetabulum had becomemalformed. In five of the dysplastic hips dislo-cation had occurred and subluxation was presentin two.

Blundell Jones (-'954) has recently discussed therole of coxa valga in various types of paralyticdislocation of the hip. He found that an increaseof the angle between the neck of the femur and theshaft was invariable and that it was most severewhen paralysis was present in the first two years oflife. Piersol (1930) has stated that the normalangle ranges from IIo to I44 degrees in adults,the average being I25 degrees. At birth the angleof the neck may be I60 degrees, diminishing to itspermanent angle by puberty. Blundell Jones(I954) found that the major part of the reductionin the angle took place during the first five yearsof life.

I have been unable to confirm his finding thatcoxa valga is " always present." In the thirty-sixhips of this series in which varying degrees ofparalysis were present, fifteen showed definitecoxa valga, the others being within the normalrange. Of the twelve dislocated and subluxatedhips six showed coxa valga, which was only severein four instances. Paralysis had, of course, beenpresent in all these patients since birth.

Fig. 6 shows the radiograph of the hip of a

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FIG. 4.-The radiograph at the age of six years of achild with an extensive lumbo-sacral spinabifida and severe paralysis of the lower limbs.The acetabula are well formed, but there issome degree of coxa valga.

FIG. 5.-The radiograph of the same child at theage of eleven years now shows a defective roof..to the left acetabulum and early subluxation ofthe hip joint.

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April 1956 CARR: The Orthopaedic Aspects of One Hundred Cases of Spina Bifida 205

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FIG. 6.-A radiograph of a child with extensive lumbo-sacral spina bifida and severe paralysis. There wasconsiderable imbalance of the muscles controllingthe left hip joint with complete paralysis ofthe shortrotators. The left acetabulum is shallow and thecoxa valga is severe. The hip has already dislocated.

boy with a large meningo-myelocele. This patientdemonstrated all three of the factors which Ibelieve tend to produce dislocation of the hip inthese patients:-(a) Severe paralysis; the flexors and adductors

were stronger than their antagonists, whilst theshort rotators were completely paralysed.

(b) A shallow acetabulum.(c) Severe coxa valga.The Knee and the Leg

Nineteen patients presented deformities of theknee joint; flexion deformity was by far the mostfrequent, and was bilateral in half of these patients.In the others the opposite knee was either recurved,flail or normal (Table I4).

TABLE 14Condition of the knee joints in 9 patients

Flexion deformity .. .. .. 27Back knee .. .. .. .. 4Flail .. .. .. .. 4Normal .. .. .. .. 3

The amount of flexion deformity varied fromIO to 60 degrees, 30 degrees being frequent. Inthe majority the hamstrings and calf were strong

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FIG. 7.-The right lower limb of a young. girl to show amarked external rotation deformity of the foot inrelation to the femur (see text). Several yearspreviously a gross equino-varus deformity had beencorrected by radical tarsectomy. Next a fixedflexion deformity of the knee joint was corrected bycomplete tenotomy of the hamstrings. The scarjust above the patella marks the site of a painlesspressure sore from the post-operative plaster. Theleft leg was completely flail, and when a fracture ofthe femur occurred it was promptly amputatedthrough the site of fracture.

and the quadriceps weak; occasionally thehamstrings were spastic. In a few the maincause of deformity was capsular contracturefollowing years of nursing with the joint flexed.Only one of the four instances of back knee wassevere. As might be expected, minor degrees.ofexternal rotation deformity of the lower limb werefrequent in patients with flexion deformities ofthe hip and knee. Fig. 7 shows a severe deformity

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206 POSTGRADUATE MEDICAL JOURNAL April 1956

in a girl with a very strong biceps femoris and atensor fasciae femoris contracture.

The FootOf all deformities, those of the feet were the

commonest (Table I5).TABLE I5

Bilateral symmetrical deformity .. 30Bilateral asymmetrical deformity.. 6Unilateral deformity ... . 27

Total 63

Equino-varus was much the commonest de-formity and was frequently accompanied bysecondary cavus. A few patients had a cavo-varusdeformity only, some equinus being masked by theinversion of the hind foot. A smaller numberpresented with pes cavus alone; calcaneo-cavus,simple valgus and total paralysis were less common(Table i6).

TABLE 16Condition of I26 feet in 63 patients Number offeet

Equino-varus 48Varus only 9Cavus 23Calcaneo-cavus 6Valgus 3Flail foot o1Normal 27

Pes Equino-varus. The patients with equino-varus showed a wide range of the degree ofparalysis. As a rule all muscle groups wereinvolved to some extent, though the calfsometimes escaped completely. The posteriorleg muscles were much stronger than those in theanterior compartment and the invertors alwaysstronger than the peronei, which were usuallyseverely paralysed. In most of the patientstibialis posticus was stronger than tibialis anticus.The deforming factors therefore are the strongcalf, which itself tends to increase varus of the footin the presence of equinus, the weak peronei andthe relatively stronger tibial muscles. The mostsevere deformities occurred when the calf andtibialis posticus were powerful. Adduction of theforefoot was less severe than in congenital clubfootand the heel seldom so poorly developed.Many of the patients had been under treatment

for some time when they were reviewed. Correc-tion of the deformity by conservative measuressuch as strapping, splints and repeated plastercasts had frequently been regarded as successful,but cessation of treatment was often followed byrapid relapse according to the degree of muscleimbalance present. Transplantation of the tendonof the tibialis anticus to the outer side of the footwas performed in ten instances; in one foot therewas over-correction, while all the others still

showed equino-varus. In twenty instances aSteindler operation had been performed and nofurther treatment was contemplated unless de-formity recurred. In a third of the patients withequino-varus stabilisation of the foot by some typeof triple arthrodesis was eventually performed.Half of these patients finally had feet of good shapeand function and were able to wear ordinary shopshoes; in the other half some deformity remained,especially varus, due either to inadequate correc-tion or to the continued action of deformingmuscles.A number of patients with mild equino-varus

deformities had no treatment apart from insideirons with outside T-straps, varus wedges to thesoles and heels and surgical boots. Neverthelesssome deformity remained in most of the patientswho had attended hospital. The infrequency ofthe attempts to balance the foot by suitable tendontransplantation after closed or open correction ofthe deformity suggested that the part played bymuscle imbalance had not been sufficientlyrecognised.

Pes Cavus. Pes cavus was much less frequentthan equino-varus. The incidence of bilateraland unilateral deformity was equal. Half of thesepatients complained of pain under the metatarsalheads, of weakness of the foot and of inability towear shop shoes. Most of those with pain hadfixed hyperextension of the metatarso-phalangealjoints, rigid clawing of the toes and callositiesunder the metatarsal heads. The severity of thecavus was very variable and even in those with nosymptoms and normal sensibility some very severedeformities were observed. Electrical reactionsshowed evidence of denervation in only fifty percent.

Other deformities of the foot were uncommon.Only six patients had the calcaneo-cavus deformityso frequent in poliomyelitis, and three had a valgusdeformity caused by weak peronei in otherwiseflail feet. Complete flaccid paralysis below theknee was present in ten instances.

Impairment of Sensibility and its SequelaeFifty patients showed loss of sensibility. In the

myelocele group sensory loss was severe andwidespread; the lower lumbar and sacraldermatomes showed a similar frequency ofinvolvement and the higher lumbar segmentswere also sometimes affected. In patients withspina bifida occulta the fifth lumbar and firstsacral dermatomes were affected far more fre-quently than any other.The impairment usually involved every type of

both exteroceptive and proprioceptive sensation.As a rule, pain, touch and thermosensibility wereequally affected. Vibration sensibility was fre-

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Page 7: ORTHOPAEDIC ASPECTS HUNDRED SPINA BIFIDA · Analysis ofthe Defectsin Twenty-three cases ofMyelocele andMeningocele TABLE2 Number of vertebrae involved Numberofpatients I 2 2 3 4 2

April 1956 The Orthopaedic Aspects of One Hundred Cases of Spina Bifida 207

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FIGS. 8 and 9.-The clinical and radiographic appearances of a patient with neuropathic arthritisof the left ankle and tarsal joints.

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quently diminished and even lost if the extero-ceptive loss were severe and widespread. Diminu-tion of deep pressure and pain sensibility wasfrequent, but complete loss was rare. Loss ofpostural sensibility was uncommon, nearly alwaysconfined to the toes and feet, and accompanied byextensive-motor paralysis and severe loss of otherforms of sensibility. When the sensory dis-turbance was severe, trophic changes in the softtissues, bones and joints were frequent. Theskin was thin, often shiny, and either dry orhyperhidrotic. The nails were either brittle,ridged or short.

Ulceration. Trophic ulceration recurring overperiods of years had been present in fifty per cent.of the patients with sensory loss. The commonsites were the postero-lateral aspect of the heel,

the outer side of the foot, the dorsum of the toesand the buttocks.

Perforating ulcers under the fourth or fifthmetatarsal heads were present in six patients withequino-varus or cavo-varus deformities and severesensory disturbances. They had all been presentfor years, healing slowly during periods of restin bed and recurring on renewed activity.Complete correction of the deformity and theprovision of suitable sponge rubber insoles wasthe only successful line of treatment, excision of themetatarsal heads and necks without correction ofdeformity being followed by early recurrence.Fig. i shows a patient with severe bilateralequino-varus deformity, a large trophic ulcer onthe right foot and a perforating ulcer under theleft fourth metatarsal head. The prognosis in

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208 POSTGRADUATE MEDICAL JOURNAL April I956

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FIc. io.-The radiograph of a boy aged four years toshow excess callus formation after a spontaneousfracture of the upper end of the femur (see text)

perforating ulcers was bad; in fifty per cent.amputation became necessary because of increasingdeformity, or of failure of attempts to correct it,or because of chronic sepsis.

Neuropathic Bones and Joints. Neuropathicarthritis of the ankle and tarsal joints developed inonly one patient (Figs. 8 and 9). Spontaneousand almost painless fractures occurred in fourpatients. In two union took place normally;in the other two the fractures were notable for thedevelopment of excess callus. Fig. io shows theradiograph of a boy of four who had a severelyparalysed limb and a dysplastic hip. After thefailure of conservative methods, open reductionof the hip and later a rotation osteotomy of thefemur had been performed. After removal of theplaster he developed a supracondylar fracturewhile lying in bed and later a subtrochantericfracture with a great mass of callus. Furtherfractures followed and the limb was eventually

amputated. Histological examination showednormal callus and the " chondroid tissue"described by Baker (1946) in the hyperplasticcallus of osteogenesis imperfecta was not observed.

Excessive callus in fractures associated withspina bifida has been reported by Gillies andHartung (1938) who described two instances offracture of the upper tibia with sclerosis, frag-mentation and excessive callus formation; therewas widening of the epiphyseal line and lateraldisplacement of the head of the fibula. Theircases bear a close resemblance to one case in thisseries. A young girl with spina bifida, severelyparalysed lower limbs and gross sensory impair-ment came to hospital complaining of swelling ofthe upper tibial region of the left leg. A radio-graph showed the changes described by Gillies andHartung but no fracture (Fig. 1). The Wasser-mann and Kahn reactions were negative. Oneyear later the tibia had become almost normalagain (Fig. 12). These changes are probably theresult of trauma in a limb with decreasedsensibility.Alterations in the Neurological Symptomsand SignsThe neurological deficit in spina bifida has been

said to increase with growth, and Ingraham (1943)has stated that this occurs frequently between theages ofsix and ten years. This is thought to be due-apart from the rare development of benign andmalignant neoplasms or of increased growth oflipomata already present-to tethering of the cordand cauda equina at the site of myelodysplasia,with traction on the cord above resulting from adifference in the rate of growth between theneural and skeletal axes, that is to say, the samemechanism as for the Arnold-Chiari malformation.The onset or increase of neurological change isaccepted by many as an indication for explorationof the cord with a view to freeing the nervoustissue and thus preventing further deterioration.An increase in deformity is not necessarily dueto further involvement of the nervous system anda perforating ulcer may be due only to increasingactivity or weight bearing. I found definiteevidence of an increase in the neurological deficitsuch as the onset of urinary symptoms or thedevelopment of paralysis in a muscle previouslyknown to be normal, in only five patients whoseages ranged from four to seventeen years.Summaryi. One hundred patients with spina bifida have

been examined from the orthopaedic point of:view and the associated anomalies enumerated.

2. The spinal lesions in these patients are con-sidered in detail.

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Page 9: ORTHOPAEDIC ASPECTS HUNDRED SPINA BIFIDA · Analysis ofthe Defectsin Twenty-three cases ofMyelocele andMeningocele TABLE2 Number of vertebrae involved Numberofpatients I 2 2 3 4 2

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FIG. I .-The radiographs of a young girl with spina bifida and gross sensory loss in both legs.A painless swelling of the upper tibia developed. Note the sclerosis of bone with appearancesof fragmentation and new bone formation. The epiphyseal line is widened and the head of thefibula is displaced laterally.

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FIG. I2.-A radiograph of the same patient one year later showing spontaneous resolution of thechanges. After a further year the films showed almost normal bone structure.

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2 POSTGRADUATE MEDICAL JOURNAL April 1956

3. A description of the motor paralysis in thelower limbs and its relation to various de-formities is given.

4. The impairment of sensibility, its incidenceand sequelae are described.

5. Progression of neurological changes is discussed.

PostscriptI wish to record my thanks to Mr. H. J. Seddon

for arousing my interest in this subject and for hishelpful criticism, to Mr. K. I. Nissen and Mr.J. I. P. James for their continuous encouragementand advice, and to all those members of theConsultant Staff of the Royal National OrthopaedicHospital who kindly allowed me to examine theirpatients. I wish also to record my appreciation ofthe ever ready aid of the Medical Records Depart-ment of the Royal National Orthopaedic Hospital,

the Department of Medical Photography and theLibrarian of the Institute of Orthopaedics.

BIBLIOGRAPHYBAKER, S. L. (1946), 'Hyperplastic Callus Simulating Sarcoma in

two cases of Fragilitas Ossium,' Journal of Pathology andBacteriology, 58, 609.

GILLIES, C. L., and HARTUNG, W. (1938), 'Fracture of theTibia in Spina Bifida Vera,' Radiology, 31, 621.

GOLDING, F. C. (I950), Discussion on the significance of con-genital abnormalities of the lumbo-sacral region, ProceedingsRoyal Society of Medicine, 43, 636.

HART, V. L. (1928), 'Spontaneous dislocation of the Hip Jointduring early life: report of twenty-eight cases,' Archives ofSurgery, 17, 587.

INGRAHAM, F. D., and SWAN, H. (I943), 'Spina Bifida and'Cranium Bifidum: survey of 546 cases.' New England Journalof Medicine, 228, 559.

JONES, G. BLUNDELL (1954), 'Paralytic dislocation of the Hip,'Journal of Bone and Joint Surgery, $6-B, 375.

KESSEL, A. W. LIPMANN (x951), Intrathroacic meningocelesoinal deformity, and multiple neurofibromatosis,' Journal ofBone and Joint Surgery, 33-B, 87.

MACNAB, G. H. (1954), Spina Bifida Cystica,' Annals of the RoyalCollege of Surgeons of England, 14, 124.

PIERSOL, G. A. (1930), Human Anatomy, ninth edition, 359.WATSON-JONES, R. ( 926), 'Spontaneous Dislocation ofthe Hip,'

British 'ournal of Surgery. 14. 36.

OBSTETRICS & GYNAECOLOGY(Postgraduate Medical Journal, July, 1954)

Price: 3s. 10d., post free

INTRODUCTORY PELVIC THROMBOSISCharles D. Read, F.R.C.S., F.R.C.O.G. J. Stallworthy, F.R.C.S., F.R.C.O.G.

RESPIRATORY HAZARDS IN THE THE EARLY DIAGNOSIS OF GENITALPREMATURE INFANT CANCER BY CYTOLOGYAlbert E. Claireaux, M.D., M.R.C.P. Erica Waechtel, M.D.

POSTMATURTY THE MENOPAUSES. G. Clayton, M.D., M.S., F.R.C.O.G. G. I. M Swyer D.M., M.R.C.P.

THE PLACE OF ULTRA-RADICAL SUR-THE RELATIVE MERITS OF THE VARIOUS GERY IN ADVANCED MALIGNANTBIOLOGICAL TESTS FOR PREGNANCY DISEASE IN THE PELVISH. P. Ferreira, M.D. J. B. Blaikley, F.R.C.S., F.R.C.O.G.

Published byTHE FELLOWSHIP OF POSTGRADUATE MEDICINE

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