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THE ORTHOPAEDIC ASPECTS of ONE HUNDRED CASES of SPINA BIFIDA by T

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THE ORTHOPAEDIC ASPECTS of ONE HUNDRED CASES of SPINA BIFIDA by T Postgrad Med J: first published as 10.1136/pgmj.32.366.201 on 1 April 1956. Downloaded from 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 .;3. ·-· ;··, :::::: ··;·.··I :liiiii:==== ======'....= ...·..'"':4 jL I iaii; l·~sil is.. ;·:: .·I,·:..-·· ·.*i .-.-:,iiii.··:.; by copyright. ·,. FIG. I FIG. 2 Of the many papers on the subject of spina TABLE I bifida few are concerned mainly with the ortho- Associated Anomalies in One Hundred Patients it Macnab has I. Lumbo-sacral anomalies such as vertebrae of paedic problems presents. (I954) transitional type and vertebrae with ab- indicated that, particularly since the advent of normally shaped bodies, spinous processes antibiotics, the early mortality rate is not as high as and neural arches 32 formerly, so that although the treatment of associ- 2. Scoliosis (i8 congenital, 2 idiopathic) 20 http://pmj.bmj.com/ ated is still not more 3. Congenital fusion of vertebrae, involving hydrocephalus satisfactory, from 2-12 segments 18 patients may survive to require orthopaedic care. 4. Congenital lumbo-sacral spondylolisthesis 5 It is my purpose to describe some of the problems 5. Hypoplasia of sacrum 5 encountered in one hundred patients with spina 6. Abnormal ribs (fusion, absence or deformity) 7 bifida who advice at the National 7. Syndactyly of fingers 2 sought Royal 8. Sacro-coccygeal dermoid I Orthopaedic Hospital. Many had already been 9. Cafe-au-lait type ofpigrnentation of trunk and under treatment at other hospitals for some years, limbs 5 and this made some degree of selection of patients Io. Osteochondritis juvenilis (hip 3, spine 3) 6 on September 25, 2021 by guest. Protected inevitable. of these were I. Congenital absence of pectoralis major 2, Forty-two patients male, thyroglossal cysts 3, congenital absence of and fifty-eight were female; four had a family vagina i, imperforate anus 2, and macro- history of the condition. Only a few had mild mastia I 9 static hydrocephalus. Quite apart from the well- known sequelae of the neurological lesions such as Total I o club foot and trophic ulceration (Fig. i), and related conditions such as dysplasia of the hipjoint matosis, but no caseresembling those described by (Fig. 2), a wide variety of associated anomalies was him was encountered. found (Table i). In the five patients with cafe-au-lait type of skin The Spinal Lesion pigmentation no other evidence of neurofibro- For the purpose of describing the spinal lesion matosis was found. Kessel (I95 ) has discussed the patients have been divided.into .two grups:-- or the association of lateral.intrathoracic extrapleural (I) Twenty-three. patients....with .-nyelocele.:. meningocele..with spinal deformity. in. neurofibm- ..".. :',men.ngoQele..'menin' .oe ...,... ....: Postgrad Med J: first published as 10.1136/pgmj.32.366.201 on 1 April 1956. Downloaded from 202 POSTGRADUATE MEDICAL JOURNAL April 1956 (2) Seventy-seven patients with spina bifida lesion was again lumbo-sacral and the smaller occulta. defects were usually placed centrally (see Tables 6 to 9). GROUP I: Myelocele and Meningocele All but two of the had Analysis of the Defects in Seventy-seven cases of Spina twenty-three patients Bifida Occulta extensive involvement of the nervous system. TABLE 6 Most of them had had operations on the myelocele Number of vertebrae involved Number ofpatients or meningocele within a few months of birth but *i 28 there was no definite evidence of neurological 2 II in 3 7 improvement any case. The neural arch defect 4 4 usually involved five or more segments and in one 5 9 patient no less than twelve arches were defective. 6 7 Both laminae were often completely absent, and the 7 5 lesion was most 8 3 site of the frequently lumbo-sacral. 9 2 When the defect was small it was nearly always II I centrally 2 to *This was the first sacral segment in 2I of the 28 placed (Tables 5). patients. Analysis of the Defects in Twenty-three cases of Myelocele and Meningocele TABLE 7 TABLE 2 Size of neural arch defect Number of vertebrae involved Number of vertebrae involved Number ofpatients I. Less than i cm. in I 2 width on the radio- 2 3 graph 141 4 2 2. Subtotal 8 5 4 3. Total 113 6 4 7 2 TABLE 8 8 3 Site of lesion Number of patients 9 2 Cervical 2 by copyright. 12 I Cervico-thoracic and sacral i Cervical and lumbo-sacral I TABLE 3 Thoracic 2 Size of neural arch defect Number of vertebrae involved Thoraco-lumbar I I. Less than I cm. in Thoraco-lumbo-sacral 3 width on the radio- Lumbar 7 graph 33 Lumbo-sacral 37 2. Subtotal 14 Sacral 23 3. Total 8I TABLE 9 TABLE 4 Site of Number of vertebrae involvedhttp://pmj.bmj.com/ Site of lesion Number of patients small neural arch defects Cervical I I. Central II8 Thoraco-lumbo-sacral 2 2. Right laminar 12 Lumbar 4 3. Left laminar II Lumbo-sacral 15 Sacral I Cutaneous and subcutaneous changes were in over cent. of the TABLE 5 present just fifty per patients Site Number vertebrae involved with spina bifida occulta (Table IO); in the of of on September 25, 2021 by guest. Protected small neural arch defects remainder the skin and subcutaneous tissues of the I. Central 30 back were normal, although in five there was a 2. Right laminar I to be 3. Left laminar 2 bony gap large enough easily palpable. TABLE I 0 GROUP II: Spina Bifida Occulta Cutaneous and subcutaneous evidence In this group half of the patients showed in- of spina bifida occulta in 39 patients Number ofpatients of one or Hairy patch 22 volvement only two vertebrae while the Haemangioma and telangiectasis I6 remainder showed defects as extensive as those Palpable bony defect i6 found in myelocele and meningocele. When only Scarring 9 one segment was involved it was the first sacral in Lipoma 8 cent. of cases. The Lumbar linear scleroderma 3- seventy-five per laminar defects Dimples 3 were smaller in a higher proportion of the patients Pigmentation of skin I than in Group I, but in the extensive lesions the laminae were usually completely absent, just as in Of the seventy-seven patients in Group I1- fifty the myelocele group. The commonest site of the had neurological lesions. The presence either of April 1956 CARR: The Orthopaedic Aspects of One Hundred Cases of Spina Bifida 203 Postgrad Med J: first published as 10.1136/pgmj.32.366.201 on 1 April 1956. Downloaded from cutaneous and subcutaneous changes or of ex- was incomplete; certain muscles tended to tensive laminar defects indicated that involvement escape and various patterns of paralysis thus of the nervous system was likely, although they emerged. were occasionally present when the central nervous system was normal. Of those cases without Paralysis Affecting the Hip superficial evidence of spina bifida fifty per cent. Twenty-one patients had paralysis of muscles had neurological lesions (Tables i and I2). controlling the hip. In five it was unilateral, slight and accompanied by a similar degree of TABLE II in the rest of the limb. In sixteen the Number of patients paresis Cutaneous and subcutaneous changes paralysis was severe, bilateral and accompanied by with neurological lesion .. .. 3 extensive paralysis more distally. Cutaneous and subcutaneous changes Three main groups were observed:- with normal C.N.S. .. 8 I. Paralysis affecting all muscles uniformly but No superficial changes with neuro- in from case to case. logical lesion ..... 19 varying severity No superficial changes with normal 2. Paralysis in which certain muscles were strong C.N.S. .. .. .. I9 and others weak. TABLE 12 3. Paralysis associated with dysplastic changes Number of Number of patients in the hip (Table I3). vertebrae with neurological Number of patients involved lesion with normal C.N.S TABLE 13 Condition of the hip in 21 patients Number of hips 1 14 14 Normal 5 2 4 7 Uniformn hip paralysis 14 3 7 o with muscle imbalance I2 4 4 o Paralysis 5 8 Paralysis with dysplastic changes io 6 6 I 41 7 3 2 (One patient had a disarticulation through the hip at the 8 2 I of fourteen for a flail 9 2 0 age years limb) II O I The first group showed three main clinical by copyright. Tables 6 and 12 indicate that about fifty per pictures:- cent. of the patients with spina bifidaocculta ofthe (a) Slight general weakness, all muscles being first sacral segment had neurological lesions. graded about 4 on the M.R.C. scale. These patients This high figure is of course the result of the either had no complaints or felt that the affected selection of patients to which I have already hip was somewhat weaker than the other. referred. By contrast Campbell Golding (1950) (b) A moderate degree of paralysis causing a has found spina bifida occulta of the first sacral Trendelenberg type of limp from abductor segment in nineteen per cent. of adult patients weakness. http://pmj.bmj.com/ subjected to routine radiological examination. (c) Severe paralysis with the joint completely or almost completely flail. In none of five such hips Neurological Lesions was dislocation present. Neurological examination of all the hundred In the second group paralysis was again variable patients showed the following features:- in severity and the striking feature was the existence I. Nervous involvement was usually more severe of muscle imbalance. In two patients the flexors and more extensive in the myelocele and were normal, the extensors weak and the other meningocele group but equally severe dis- muscles fairly evenly balanced.
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