D e c e m b e r , 1969 P H Y S I O T H E R A P Y Page 5 The O rthopoedic M anagem ent of M yelom eningocoele J. E. H A N D E L S M A N , M.Ch. Orth., F.R.C.S. Principal O rth o p o e d ic Su rgeon, G eneral H ospital, Transvaal M e m o ria l H o sp ita l for C hild ren and U n iv e rsity of the W itw ate rsra n d , Johannesburg. In the 4 th C en tu ry B .C ., H ip p o c r a te s m a d e th e first written referen ce to sp in a b ifid a in L ib e r G lan du lis b u t it was n o t u n til th e 16th C en tu ry th a t V e s a liu s a ccu rately d escribed a b o y w ith m u ltip le d e fo r m itie s o f th e lo w er lim bs a n d a n a sso c ia te d h y d ro cep h a lu s. T h e p a th o lo g y o f m y e lo m e n in g o c o e le , a ss o c ia te d h y d r o ­ cep h alu s an d renal c o m p lic a tio n s is fu lly d ea lt w ith elsew h ere in this S y m p o siu m , b u t it is w o r th e m p h a sisin g the im portan ce o f early su rg ic a l c lo su r e o f th e sp in a l d e fe c t in order to p reserve e x is tin g n e u r o lo g ic a l tissu e. T H E S P I N A L D E F E C T T h e degree o f p a ra p leg ia se en in th e m y e lo m e n in g o c o e le patient d ep en d s prim arily o n th e lev el o f th e le sio n , a n d in general term s, th e h igh er th e d efect th e m o r e p r o fo u n d is the p aralysis a n d se n so r y d eficit in th e lo w er lim b s. A t a n y lev el, h o w e v e r , co rd a n d n erv e r o o ts m a y be fu n ctio n in g in sp ite o f th e sp in a l le s io n a n d h ern ia tio n but this fu n c tio n w ill b e lo st u n le ss th e e x p o se d sa c is covered b y sk in w ith in th e first few h o u r s o f life . A c h ie v in g closure o fte n p resen ts a fo r m id a b le su rg ic a l p ro b lem because o f d eficien t so ft tissu es a n d th e w id e n e d bifid p osterior sp in o u s p ro cesses. R e c e n tly a te c h n iq u e h as b een d ev elo p ed w h ereb y u n w a n ted b o n e is r e m o v e d fro m a ro u n d the nerve r o o ts, a n d e v e n w h o le verteb ral b o d ie s e x cised (Sharrard, 1968). T h is greatly fa cilita tes sk in clo su r e , and is o f particular v a lu e w h e n a g ib b u s is p resen t. T h is deform ity o c c u r s in o n e in eig h t p a tie n ts, a n d c o r r e c tio n in the n e o n a ta l p e r io d p rev en ts th e p e r p e tu a tio n o f a g ro tesq u e d eform ity that m a y su b se q u e n tly req u ire c o r r e c tio n by a difficult sp in a l o s te o to m y (F ig . 1). A S S O C I A T E D D E F O R M I T I E S T h e a ss o c ia tio n o f d islo c a tio n o f th e h ip , eq u in o -v a r u s, valgus a n d c a lc a n e u s d e fo r m itie s o f th e f o o t h a v e lo n g been r eco g n ised in m y e lo m e n in g o c o e le . E a r ly w ork ers regarded th e se a s id io p a th ic b u t u n c o n n e c te d co n g e n ita l a b n o rm a lities, b u t m o r e critica l o b se r v a tio n h a s revealed specific pattern s o f d e fo r m ity d e p e n d in g u p o n th e level o f the sp in a l lesio n . T h e c o m m o n e s t ty p e o f m y e lo m e n in g o c o e le o b liter a tes all se g m en ts from th e fo u r th lu m b a r n e u r o lo g ic a l level d istally. T h e a ttitu d e o f su ch a c h ild at b irth is ch a ra c­ teristic. F ix e d fle x io n d e fo r m ity o f th e h ip s is c o n sta n t a n d d islo c a tio n o f th e h ip s is freq u en tly p resen t at b irth. T h e k n ees are h eld e x te n d e d a n d ex te r n a lly r o ta te d . D e fo r m itie s o f the feet vary, b u t are u su a lly c a lc a n e o - o r eq u in o -v a r u s (F ig. 2). O n th e o th e r h a n d ch ild ren w ith a sp in a l le s io n in the lu m b o -d o rsa l r e g io n n ev er p resen t w ith fix ed co n tr a c ­ tures, d islo c a tio n s o r d e fo r m itie s at birth. F u r th erm o re, low lu m b ar a n d sacral le s io n s affect th e fe e t o n ly . It is th u s ap p aren t that d e fo r m ity is a resu lt o f p artial in n e r v a tio n o f the low er lim b rather th a n to ta l a b se n c e , a n d certa in ly is n o t id io p a th ic in n atu re. It is r e a so n a b le t o infer th a t m uscle im b a la n ce is th e fu n d a m e n ta l c a u se o f th e a sso c ia te d d efo rm ities. B e c a u se th is im b a la n c e c o m m e n c e s d u rin g intra-uterine life, th e d e fo r m itie s a p p ea r “ c o n g e n ita l” . S E N S O R Y C H A N G E S U n lik e p o lio m y e litis, w h e r e m u sc le im b a la n c e m a y lead to sim ilar d e fo r m itie s, th e ch ild w ith a m y e lo m e n in g o c o e le h as lo st n o rm a l sk in se n s a tio n in th e d istr ib u tio n o f the nerve r o o ts in v o lv e d . B e c a u se p r o te c tiv e p a in a p p r e c ia tio n is a b se n t, trea tm en t th a t in v o lv e s c o rre ctiv e pressure h o w e v e r g e n tle , is p reclu d ed . A n a sp e c t o f th e se n so r y d eficien cy n o t alw a y s a p p recia ted is th e lo s s o f p r o p r io c e p tio n . B e c a u se th e se p a tie n ts d o n o t k n o w w h ere their lo w er lim b s are in sp a ce , r e h a b ilita tio n is d ifficult. S o m e tim e s cerebral d a m a g e , p r o d u c e d b y th e h y d r o c e p h a lu s, c o m p o u n d s th e la ck o f a d e q u a te jo in t a n d m u sc le se n sa tio n . E v e n w ith the m o s t a ctiv e reh a b ilita tiv e p ro g r a m m e , w a lk in g m a y b e d ela y ed fo r so m e years. A S S E S S M E N T E s tim a tio n o f th e d eg ree o f p a ra ly sis a n d se n s o r y lo ss at th e ea rliest o p p o r tu n ity e n a b le s th e p ra ctitio n er t o p la n for th e fu tu re, a n d ju d g e th e e ffe ct o f im m e d ia te c lo s u r e o f th e sp in a l d efect. I n a n ew -b o rn c h ild a ss e ssm e n t o f m u s c le p o w e r a n d se n s a tio n is e x trem ely d ifficult. H o w e v e r , d e fo r m ity is rea d ily a p p a ren t, a n d th e ra n g e o f m o v e m e n t o f e a c h j o in t m a y be a c cu ra tely o b se r v e d a n d r eco r d e d . B o th th e se features g iv e a g o o d in d ic a tio n o f th e type o f m u scle im b a la n c e th a t is alrea d y p resen t a t birth a n d it is the p ra ctice a t th e S p in a l D e fe c ts C lin ic o f th e T r a n sv a a l M e m o r ia l H o s p ita l fo r C h ild ren to ch a rt th e se clin ica l sig n s a t th e first v isit. E v e n in th e very y o u n g p a tie n t a fair a ss e ssm e n t o f m u sc le p o w er m a y b e m a d e . A w a rm a n d w a k efu l c h ild w ill m o v e a ll n o rm a l lim b s a n d sim p le o b se r v a tio n w ill reveal a m u sc le gro u p th a t is n o t fu n c tio n in g a d e q u a te ly . S k in stim u la tio n m a y p r o v id e a n in d ic a tio n o f th e se n so r y lo ss. W ith e a c h v isit th e e x a m in a tio n sh o u ld b e rep ea ted an d e v e n tu a lly a c o m p le te p ictu r e o f th e m o to r a n d se n so r y d eficit w ill em erge. B y stu d y in g th e n erve r o o t in n e r v a tio n o f th e lo w er lim b m u sc le s, in fe ren ces ca n b e d raw n w h ere a c o m m o n sp in al level ca ters fo r m o r e than o n e grou p . F o r e x a m p le th e m ed ia l h a m strin g m u sc le s m a y b e e a sily e x a ­ m in e d , a n d w h en p a ra ly se d , it m a y b e d ed u ced th a t th e h ip a b d u c to r s, w h ich are n o t ea sy to ex a m in e w ill a lso b e p a ra ly se d (Sharrard, 1964). T h is ea rly a sse ssm e n t p r o v id e s a g o o d g u id e to p o ss ib le fu tu re d e fo r m ity , th e p o te n tia l fo r w a lk in g u n a id e d o r the n e e d fo r a ssista n c e w ith su ita b le a p p lia n c e s at a later d a te. P R I N C I P L E S O F M A N A G E M E N T In th e p a st a large p r o p o r tio n o f m y e lo m e n in g o c o e le ch ild ren h a v e b een c o n d e m n e d to a w h eel-ch a ir e x isten ce. T h e a p p a ren tly “ c o n g e n ita l” d e fo r m itie s resisted th e usu al fo r m s o f trea tm en t b e c a u se th e u n d erly in g ca u se— m u sc le im b a la n c e — w a s n eith e r a p p recia ted n o r co rre cted . T h e m o d e r n su r g e o n find s th is a p p ro a ch u n ten a b le. H e e x p e c ts to reb a la n ce th e lo w er lim b s s o th a t e v ery p a tie n t m a y learn to sta n d a n d w a lk in d ep en d e n tly . T o a c h ie v e th is, th e p a tie n t m u st h a v e p la n tig r a d e feet, e x te n d e d k n ees and sta b le h ip s, a n d a b a la n c e d u p righ t trunk. C o rre ctio n o f th e feet ta k e s p reced en ce b e c a u se o n c e a p la n tig ra d e b a se is e x ta b lish e d , sta n d in g a n d w a lk in g w ith a ssista n c e is p o s s ib le a n d reh a b ilita tio n m a y c o m m e n c e . W ith th e feet co rre cted , a p p lia n c e s m a y b e fitted to o v e r c o m e w e a k n e ss o f th e k n e e s a n d h ip s. A lth o u g h fle x io n c o n tr a c ­ tures a n d d islo c a tio n o f th e h ip s ten d to tip th e tru nk fo rw a rd s, in crea sin g th e lu m b a r lo r d o sis m a y c o m p e n sa te th is te n d e n c y su fficien tly t o m a k e w a lk in g fe a sib le . T h e im p o r ta n c e o f a c h ie v in g th e u p righ t p o stu r e a s ea r ly a s p o ss ib le a n d tra in in g th e b a la n c e m ech a n ism s c a n n o t b e o v er-em p h a sised . 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. ) Page 6 P H Y S I O T H E R A P Y December, 1969 1a 2b 1b L a c k o f n o r m a l p r o t e c t i v e sk in s e n s a t i o n i m p o s e s a d if fe re nt t h e r a p e u t i c a p p r o a c h t o t h e c o r r e c t i o n o f d e f o r m i t y . E v e n t h e sli ght es t p r e s s u r e a p p l i e d by a p a d d e d p l a s te r o f P a r i s c a s t re a d i l y p r o d u c e s p r e s s u r e n e c r o s i s o f t h e skin . T h e s u b s e q u e n t p e n e t r a t i n g ul ce rs m a y t a k e m o n t h s a n d e v e n y e a r s t o heal , a n d if o s te iti s s u p e r v e n e s a m p u t a t i o n m a y be t h e s e q ue l. F o r t h es e r e a s o n s t h e u se o f c a l i p e r s o r s p l i n t s a s c o r r e c t iv e m e a s u r e s a r e in effective a n d d a n g e r o u s , a s a r e f r a m e s a n d t r a c t i o n . O n c e c o r r e c t i o n h a s b e e n a c h i e v e d by s u r g e r y h o w e v e r , c a l i p e r s a n d n i g h t s p l i n t s m a y b e u se d t o m a i n t a i n c o r r e c t i o n b u t m u s t be r e m o v e d f r e q u e n t l y d u r i n g t h e c o u r s e o f a d a y so t h a t e a r l y sk i n e r y t h e m a is n o t m iss ed . M a n i p u l a t i o n s u n d e r a n a e s t h e s i a a r e e q u a l l y u n s a t i s ­ f a c t o ry . E v en w h e n d e f o r m i t i e s a r e c o r r e c t e d by thi s m e t h o d , t h e u n d e r l y i n g m u s c l e i m b a l a n c e will p r o d u c e a r a p i d r e c u r r e n c e o f t h e o r i g i n a l d e f o r m i t y . F u r t h e r m o r e , t h e lo w er li m b b o n e s o f th es e p a t i e n t s a r e a b n o r m a l a n d f r a c t u r e r e a d i l y — o f t e n s p o n t a n e o u s l y . F o r thi s r e a s o n , m a n i p u l a t i o n is n o t o n l y in effective b u t d a n g e r o u s . T h u s fixed d e f o r m i t y r e q u i r e s s ur g ic al i n t e r v e n t i o n for s a t i s f a c t o r y c o r r e c t i o n . O p e r a t i o n s m a y b e c o m m e n c e d a s s o o n as t h e c h i l d ’s g e n e r a l c o n d i t i o n p e r m i ts . P a r t i c u l a r l y F ig. 1: A severe lumbar gibbus in a 5 -y ea r old boy (a and b) treated by spinal o steo to m y with e x cisio n o f o n e-and -a-half vertebral bodies and sta p le fixation (c). F ig . 2 : T h e typical attitu de o f an infant with a m id-lum bar m yelom en in gocoele. T h e hips are dislocated , fixed in flexion and ex tern a l rotation, and the kn ees exten d ed (a ). T h e left foot is held in equino-varus and the right show s a hind-foot equinus deform ity (a and b). 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, 1969 P H Y S I O T H E R A P Y Page 7 wi th r e g a r d to th e fo o t , c o r r e c t i o n s h o u l d b e a c h i e v e d as ea r l y a s p o s s i b l e if n o r m a l b o n y a n a t o m y is to be m a i n t a i n e d . T h e s i m p l e di vi si o n o f ti g h t so f t t iss ues a n d l e n g t h e n i n g o f s h o r t e n e d t e n d o n s will n o t a c h i e v e a l o n g - t e r m c o r r e c t i o n . T h e o r i g in a l m u s c l e i m b a l a n c e , h o w e v e r s li g h t, will c a u s e i r r e v o c a b l e r el ap se . It is es se n t i al t h e r e f o r e t o r e b a l a n c e a j o i n t by t r a n s f e r r i n g a re lat iv ely o v e r - a c t i v e t e n d o n t h a t is p r o d u c i n g d e f o r m i t y t o a s i t u a t i o n w h e r e it will h a v e the o p p o s i t e effect. U n l i k e th e m u s c l e i m b a l a n c e in p o l i o ­ myel it is, it is a c c e p t a b l e to t r a n s f e r q u i t e w e a k m u s c le s in o r d e r t o r e m o v e a d e f o r m i n g f o rc e , h o w e v e r slight. F u r t h e r ­ m o r e , in t h e n e w p o s i t i o n t h e t r a n s f e r r e d t e n d o n will o ft en p r o v i d e a te n o d e s i s effect a n d d o e s n o t st r e t c h , b e c a u s e th e m u s c l e bellies in t h e m y e l o m e n i n g o c o e l e a r e f re q u e n tl y fi br ou s. W h e r e t h e r e is n o d e f o r m i t y b u t o n l y w e a k n e s s o r p a r a ­ lysis, it is r ar e ly p o s s i b l e t o a c h i e v e s t a b il i t y by m u s c le b a l a n c i n g . I n t h e s e s i t u a t i o n s , a n e x t e r n a l a p p l i a n c e s u c h as a c a l i p e r is r e q u i re d . T h e m o r e c o m p l i c a t e d h i p s u r g e r y is f r e q u e n t l y d el ay ed u n t i l m a x i m u m d e v e l o p m e n t o f s p a r e d m u s c le s h a s be en a c h i e v e d , a n d r e h a b i l i t a t i o n is well u n d e r way . T H E M A N A G E M E N T O F S P E C I F I C D E F O R M I T I E S T he F eet B e si de s p r o v i d i n g t h e p a t i e n t w i t h p l a n t i g r a d e feet be f o re w a l k i n g c o m m e n c e s , ea r l y su r g e r y i m p r o v e s t h e p r o g n o s i s . A se v er e fixed f o o t d e f o r m i t y at b i r t h r e p r e s e n t s a d i s l o c a t i o n r a t h e r t h a n d i s t o r t i o n o f a c t u a l b o n e s t r u c t u r e . W i t h p e r s i s t e n c e o f t h e d e f o r m i t y , h o w e v e r , a l t e r a t i o n in s h a p e o f t h e t a r s a l b o n e s o c c u r s . T h e s e h a v e little c a p a c i t y fo r r e m o d e l l i n g a n d b e c a u s e t h e u l t i m a t e p r o g n o s i s fo r t h e f oo t d e p e n d s u p o n m a i n t a i n i n g a n o r m a l b o n y c o n f i g u r a t i o n , c o r r e c t i o n as e a r l y as p o s s i b l e is i m p e r a t i v e . S ur gi c al c o r r e c t i o n m a y b e c o m m e n c e d a t t h e ag e o f t h r e e m o n t h s o r less in a fit ch i l d . T h e specific f o o t d e f o r m i t y se en d e p e n d s u p o n th e p a r t i c u l a r m u s c l e i m b a l a n c e p r e s e n t a n d it is n o t e w o r t h y t h a t sli g h t re si d u a l f u n c t i o n in o n e m u s c l e g r o u p in a n a p p a r e n t l y flail f o o t is e n o u g h t o p r o d u c e a se ve re fixed c o n t r a c t u r e . T h u s a f u n c t i o n i n g t r ic e p s s u r a e b u t re latively w e a k f o o t e x t e n s o r s will p r o d u c e a n e q u i n u s d e f o r m i t y o f th e c a l c a n e u m , w h e r e a s th e o p p o s i t e f o r m o f m u s c le i m b a l a n c e will i r r e v o c a b l y d r a w th e heel i n t o ca l c a n e u s . F o r e f o o t a d d u c t i o n a n d v a r u s u s u a l ly go t o g e t h e r a n d ar e lar gel y a f u n c t i o n o f re l a t i v e o v e r - a c t i o n o f t h e tibialis p o s t e r i o r . W h e n t he p e r o n e a l t e n d o n s p r e d o m i n a t e va lg u s a n d ev en a b d u c t i o n o f t h e f o r e f o o t will r e su l t . A n is o la te d i n t r i n s i c m u s c l e p a r a l y s i s se en w i t h le s io n s a t t h e SI level, m a y p r o d u c e c l a w i n g o f t h e t o e s a t a ver y ea r l y ag e a n d c a v u s o f t h e f o o t by ad o l e s c e n c e . The Equino-Varus Foot T h e c o m m o n e s t f o o t d e f o r m i t y in t h e m y e l o m e n i n g o c o e l e ch i l d is e q u i n o v a r u s . T h i s cl os ely r e s e m b l e s t h e c o m m o n c o n g e n i t a l c l u b f o o t a n d h a s in t h e p as t led to t h e beli ef t h a t its o c c u r r e n c e w a s a s e p a r a t e c o n g e n i t a l a n o m a l y . T h e su r g ic al re l e a se f o llo w s t h e p r i n c i p le s a p p l i e d to the c o m m o n c l u b f o o t ( S o l o m o n a n d H a n d e l s m a n , 1967) b u t a t e n d o n t r a n s f e r is a l w a y s i n c l u d e d . T h e f o o t is a p p r o a c h e d f r o m t h e m e d i a l a s p e c t a n d e l o n g a t i o n o f t h e t e n d o - c a l c a n e u s , flex or h al l u c i s l o n g u s a n d flex or d i g i t o r u m l o n g u s t e n d o n s a r e c a r r i e d o u t . In a d d i t i o n , d i v i s i o n o f t h e p o s t e r i o r c a p s u l e o f t h e a n k l e j o i n t a n d t he m e d i a l t a r s a l c a p s u l e s es p ec ia ll y a r o u n d t h e n a v i c u l a r c o m p l e t e s t h e p r o c e d u r e . T h e re le as e a l o n e will n o t p r e v e n t a r el ap se , so b a l a n c e is s o u g h t . T h e tib ialis p o s t e r i o r t e n d o n is d i v i d e d at its i n s e r t i o n i n t o t h e n a v i c u l a r at t h e t i m e o f th e re lea se o p e r a t i o n a n d t r a n s p o s e d t h r o u g h t h e i n t e r o s ­ s e o u s m e m b r a n e t o t h e d o r s u m o f t h e f o o t , p r o v i d i n g o p p o s i t i o n t o t h e e q u i n o - v a r u s t e n d e n c y ( Fi g . 3). The Equino- Valgus Foot W h e n t h e f o r e f o o t is re lat iv ely flail, b u t t h e t e n d o - c a l c a n e u s s h o r t , t h e p a t i e n t ’s ef fo rt t o a c h i e v e a p l a n t i g r a d e 3a 3 b 3c p o s i t i o n will f o rc e t h e f o r e f o o t i n t o a v a l g u s p o s i t i o n . I n th e s e ca s es a s i m p l e e l o n g a t i o n o f t h e t e n d o - c a l c a n e u s w i t h re le as e o f the p o s t e r i o r c a p s u l e o f t h e a n k l e j o i n t m a y suffice. In m o s t i n st an c es , h o w e v e r , t h e f o r e f o o t v a l g u s is th e re su l t o f r el at iv e o v e r - a c t i o n o f th e p e r o n e a l m u sc le g r o u p in r e l a t i o n t o t h e tibialis p o s t e r i o r . W h e n this i m b a l a n c e is se v e re a n d t h e e q u i n u s m a r k e d t h e f o o t b e c o m e s “ b r o k e n ” a r o u n d t h e h e a d o f t he t a l u s w hi ch is d r a w n i n t o a v er ti ca l p o s i t i o n by t h e h i gh c a l c a n e u m . T h e f o o t t h e n a s s u m e s a r o c k e r - b o t t o m s h a p e , t h e p a t i e n t w a l k s o n t h e d i s l o c a t e d h e a d o f t a l u s r a t h e r t h a n the c a l c a n e u m a n d a F ig . 3 : T he com m on equino-varus deform ity (a ), treated b y a m edial and posterior capsular release and tendon elongation. T he tibialis posterior tendon has been transposed to the lateral sid e o f the foot and is held, in a tunnel m ade in the tarsal bones, by a silk suture tied over a g au ze pad which is le ft undisturbed for 6 w eek s (b). A sta b le p lantigrade fo o t is the result (c). 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. ) Page 8 P H Y S I O T H E R A P Y December, 1969 d i s a b l i n g a n d ugly d e f o r m i t y en s u e s . T h e a p p e a r a n c e s o f this f o o t a r e v i r tu a ll y i d e n t i c a l to the e x t r e m e f o r m o f c o n g e n i t a l flat f o o t o r c o n g e n i t a l ver ti cal t a l u s a n d th e t r e a t m e n t f o l lo w s s i m i l a r lines. S ur g i c a l c o r r e c t i o n s h o u l d be c a r r i e d o u t a t th e e a r l i e s t o p p o r t u n i t y a n d t h e p r i n c i p le s a r e t h e s a m e as t h o s e e m p l o y e d f o r t h e e q u i n o - v a r u s d e f o r m i t y . T h e f o o t is a p p r o a c h e d in t hi s i n s t a n c e f r o m th e l at er al side. T h e t e n d o - c a l c a n e u s is e l o n g a t e d , th e p o s ­ t e r i o r c a p s u l e o f t h e a n k l e j o i n t d i v i d e d , a n d th e heel r e s t o r e d t o its n o r m a l p o s i t i o n , t h u s c a r r y i n g t h e ver tical 4a t a l u s i n t o a m o r e h o r i z o n t a l s i t u a t i o n . In o r d e r to r e d u c e t h e n a v i c u l a r o n t o th e h e a d o f t h e t a l u s , h o w e v e r , it is n e c e s s a ry t o e l o n g a t e p e r o n e u s lo n g u s , t e r t i u s a n d o f t e n the e x t e n s o r t e n d o n s o f t h e f o o t ; t o d i v i d e t h e t a r s a l c a p s u l a r l i g a m e n t s a r o u n d t h e l at er al si d e o f t h e f o ot a n d t o re lease p e r o n e u s b r e v i s f r o m its in s e r t i o n . R e l a p s e will be the i n e v i t a b l e se q u el if thi s f o o t is n o t b a l a n c e d , a n d it is t he d e t a c h e d p e r o n e u s b re v is t h a t is u se d fo r thi s p u r p o s e . T h i s t e n d o n is p a s s e d t h r o u g h t h e i n t e r o s s e o u s m e m b r a n e t o the m e d i a l si d e a n d in se rt e d i n t o t h e t e n d o n o f ti b ial is p o s t e r i o r to a u g m e n t its w e a k e n e d a c t i o n a n d m a i n t a i n th e m ed i al a r c h o f t h e f o o t . T h e w h o l e p r o c e d u r e is id en t i ca l to o n e d e s c r i b e d f o r t h e m a n a g e m e n t o f c o n g e n i t a l ver tical t a l u s ( H a n d e l s m a n , 1968). I s o la te d V a ru s a n d V algus F o o l D e fo r m itie s I n a p r o p o r t i o n o f p a t i e n t s , t h e h i n d f o o t m a y be n e u t r a l w i t h r e g a r d t o e q u i n u s a n d c a l c a n e u s , b u t p u ll ed i n t o p u r e v a r u s o r v a l g u s as t h e f o r e f o o t d i s t o r t s f o l lo w in g m u s c l e 4 b i m b a l a n c e . A p u r e v a r u s d e f o r m i t y is u n u s u a l a n d c a n be r e b a l a n c e d by a ti bi al is p o s t e r i o r t r a n s f e r . A v a l g u s fo o t , o n th e o t h e r h a n d , is c o m m o n l y se en . W h e n t r e a t e d ea rl y, t r a n s f e r o f t h e p e r o n e u s br evi s t o t h e m e d i a l sid e o f t h e f oo t o r i n t o t h e ti b ial is p o s t e r i o r t e n d o n m a y b e all t h a t is r e q u i r e d , b u t c a u t i o n m u s t b e ex er ci se d w h e n s o m e f u n c t i o n r e m a i n s in th e ti b ia li s p o s t e r i o r o r o v e r a c t i o n m a y sw in g th e f o o t f r o m v a l g u s t o v a r u s . I n t h e o l d e r ch i l d , h o w e v e r , a t e n d o n t r a n s f e r m a y n o t be suff ici ent to c o r r e c t th e d e f o r m i t y , p a r t i c u l a r l y w h e n t h e heel v a l g u s is se vere. I t is u se fu l in th e s e i n s t a n c e s t o d o a n e x t r a - a r t i c u l a r f us io n o f th e s u b t a l a r j o i n t so t h a t t h e c a l c a n e u m m a y be held in a v er ti ca l p o s i t i o n by t h e s t a b l e t a l u s . F u s i o n s in t h e feet o f sm a ll c h i l d r e n a r e n o t u s u a l ly p r a c t i c a b l e o r d e s i r a b l e , b u t in thi s i n s t a n c e , filling t h e s i n u s ta rs i w i t h a b l o c k o f b o n e ( G r i c e , 1952) will p r o v i d e a f u s i o n w i t h o u t i n t e r f e r i n g w it h a r t i c u l a r su r f a c e s . A s i m p l e r a n d m o r e s a t i s f a c t o r y m e t h o d o f a c h i e v i n g th i s f u s i o n is to d r i v e a r es ec te d l e n g t h o f t h e 4 p a t i e n t ’s f i b u la r s h a f t ( w h i c h s o o n r e g e n e r a t e s ) t h r o u g h a h o l e d ri lle d in t h e ne ck o f t h e t a l u s a n d i n t o t h e c a l c a n e u m , us i n g a sm a ll i n c i s i o n o n t h e d o r s u m o f t h e fo o t . ( B r o w n , 1968, S e y m o u r a n d E v a n s , 1968). I n t h e p a r a l y t i c v al gu s fo o t , a s u b t a l a r a r t h r o d e s i s a l o n e will n o t m a i n t a i n c o r r e c t i o n a s t h e f u s i o n will e i t h e r m e l t a w a y o r d i s t o r t w i t h th e p a s s a g e o f ti m e , b u t a s a n a u g m e n t a t i o n p r o c e d u r e t o a r e b a l a n c i n g o p e r a t i o n it se rv es a ve r y u s e fu l p u r p o s e . T h i s p r i n c i p l e h o l d s t r u e in o t h e r a s p e c t s o f t h e m a n a g e m e n t o f d e f o r m i t y in m y e l o m e n i n g o c o e l e . T h e C a lc a n e u s F o o l P a r a l y s i s o f t h e t r ic e p s s u r a e is n o t u n c o m m o n in thes e c h i l d r e n a n d l e a d s t o a g r o t e s q u e d e f o r m i t y . T h e c a l c a n e u m e l o n g a t e s a n d g r o w s ve r t i c a l l y d o w n w a r d s , t e r m e d a p t l y th e “ pi st o l g r i p h e e l ” ( F i g . 4 a). U s u a l l y t h e lo n g t o e f lex ors a r e s p a r e d a n d t h e i r c o n s t a n t a c t i o n i n a n a t t e m p t o n th e F ig . 4 : T h e typical “ pistol grip h e e l” (a) is w ell illustrated in this 1 4 -year old boy with a n eglected ca lca n eo -v a lg u s 4d deform ity. T ib ia lis anterior (b) and other tendons w ere inserted into the ten do-calcan eu s which w a s then shortened, and bony correction achieved by e x c isin g a w ed ge o f calcan eu m and talus posteriorly. T h e sta p le provided fix a tio n until sub-talar fusion w as firm (c). T h e surgical correction is on the left fo o t, which previously resem bled the d eform ity still present on the right sid e (d). S u b sequently this fo o t w as sim ilarly treated with good result. 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, 1969 P H Y S I O T H E R A P Y Page 9 patient’s part to b rin g th e fo r e fo o t to th e g r o u n d , co m b in e d with the slin g in g effect o f th e fu n c tio n in g p e r o n e a l te n d o n s and T ib ia lis g r o u p , resu lts in a n eq u a lly u n sig h tly c a v u s contracture that c o m p o u n d s th e d e fo r m ity (F ig . A d). A c o m b in a tio n o f c a lc a n e u s a n d v aru s is u n u su a l w h ereas the ca lca n eo -v a lg u s f o o t is r ela tiv ely c o m m o n . If left untreated th e d e fo r m ity is n o t o n ly u n sig h tly b u t disabling b eca u se th e se ch ild ren c a n n o t b rin g th e f o o t to th e ground w ith o u t b e n d in g th e k n ees w h ich s o o n d e v e lo p fixed flexion co n tractu res. T h e g en eral p r in c ip le s o f reb a la n cin g apply to th e c a lca n eu s f o o t. In e v ery in sta n c e th e tib ia lis anterior ten d o n a n d u su a lly th e p e r o n e u s tertiu s are rerouted into the te n d o -c a lc a n e u s w h ich is co n c u r r e n tly sh o rten ed (Fig. Ab). T h e rem a in in g e x te n so r d ig ito r u m lo n g u s p r o v id e s adequate su b seq u en t f o o t e x te n sio n . W h en v a lg u s is associated, o n e o f th e d e fo r m in g te n d o n s su ch a s the p e r o ­ neus brevis m a y a lso b e tran sferred to th e te n d o -c a lc a n e u s or to the c a lca n eu s itse lf, a s m a y be tib ia lis p o sterio r, should varus be p resen t. In the o ld er c h ild m a rk ed b o n y d e fo r m ity b e c o m e s a feature, an d te n d o n tran sfers a lo n e w ill n o t suffice. A m o r e normal a p p earan ce o f th e h eel a n d m u c h im p r o v e d fu n c tio n is, how ever, q u ite ea s ily a c h ie v e d b y e x c is in g a w e d g e o f bone from the b ack o f th e c a lc a n e u m a n d ta lu s to in clu d e the subtalar jo in t. T h e d o w n w a r d p o in tin g h eel m a y then be brought up in to a n a n a to m ic a l p o s itio n a n d su b talar fusion esta b lish ed b y a p p o s in g th e resected b o n y su rfa ces and fixing them w ith a sta p le (F ig . Ac). Intrinsic M u sc le P a r a ly s is o f th e F oot T he m ain d isa b ility fo llo w in g p a ra ly sis o f th e in te r o ss e o u s and lum brical m u s c le s is t h e ' lo s s o f flex o r p o w e r o f th e m etatarso-phalangeal jo in ts . In a tte m p tin g to c o m p e n sa te for this lo ss, th e lo n g flex o rs a n d e x te n so r s o f th e to e s overact and u ltim a tely th e p r o x im a l p h a la n g e s su b lu x a n d later d islo ca te d o r sa lly o v e r th e m eta ta rsa l h ea d s a n d fixed flexion con tractures o f th e in te r -p h a la n g e a l jo in ts d e v e lo p . A cavu s or c a v o -v a r u s d e fo r m ity o f th e f o o t in v a ria b ly follow s. T he co n d itio n is a m e n a b le to r e b a la n cin g b y d e ta c h in g th e short to e flexors fro m their in se r tio n s, re-ro u tin g th e tendons a lo n g sid e th e p r o x im a l p h a la n g e s a n d in ser tin g them into the c o m m o n e x te n so r te n d o n s. In th is n ew situation they rep lace th e in tr in sic m u s c le s, a n d fu n c tio n a s flexors o f the m eta ta r so -p h a la n g e a l jo in ts a n d e x te n so r s o f the inter-p h alan geal j o in ts. W h en p erfo rm ed su fficien tly early, this flexor to e x te n s o r te n d o n tran sfer w ill p rev en t any fixed d efo rm ity . In th e a d o le sc e n t o r o ld e r p a tie n t, how ever, fixed b o n y d e fo r m ity h as occu rred a n d it m a y th e n be necessary t o a u g m e n t reb a la n c in g p r o c e d u r e s w ith a wedge ex cisio n a n d fu s io n in th e m id -ta rsa l re g io n to correct th e ca v u s, o r a v a lg u s o s t e o t o m y o f th e c a lc a n e u m when the cavu s is m ild a n d th e h eel in varus. The C o m p letely F la il F oot W hen th e m y e lo m e n in g o c o e le is h ig h , p a ra ly sis in th e fo o t is usually a b so lu te a n d b eca u se there is n o im b a la n ce, deform ities d o n o t o c c u r e x c e p t a s a resu lt o f g ra v ity or the w eight o f b ed c lo th e s, w h ich ca n p r o d u c e eq u in u s. T h e se are readily p reven ted b y p h y sio th e r a p y a n d s im p le sp lin ta g e. Surgery in th e se p a tie n ts is rarely in d ica ted . B e c a u se it is unusual for th e m t o h a v e a d e q u a te c o n tr o l o f th e k n ees or hips, calipers rem ain n e cessa ry e v e n i f th e feet are stabilised. It d o e s o c c a s io n a lly h a p p e n , h o w e v e r , th a t a flail fo o t m ay be th e o n ly in d ic a tio n fo r a n a p p lia n c e a n d in these patients a trip le a r th r o d e sis o f th e f o o t m a y b e w o r th ­ while. In general term s f o o t sta b ilisa tio n p ro ced u res in the m y elo m en in g o co ele ch ild are u n n ecessa ry a n d o fte n d a n ­ gerous because, o n c e th e f o o t is in a fix ed p o s itio n , w e ig h t­ bearing m ay o ccu r p r e d o m in a n tly in a lo c a lise d area a n d with deficient sk in se n s a tio n , a pressu re so r e is d ifficult to avoid. The Knees C om p lete p aralysis o f th e h a m strin g m u sc le s a llo w in g un op posed q u ad ricep s fu n c tio n is c o .n m o n w ith th e mic1- lu m b a r sp in a l le s io n . T h e k n e e s are h e ld in e x te n sio n , o fte n w ith s o m e d eg ree o f recu rv a tu m , a n d th e ra n g e o f fle x io n m a y b e very lim ite d s o o n after b irth . W ith sittin g , h o w e v e r , th is u su a lly im p r o v e s a n d s e ld o m req u ires trea tm en t. O n o c c a s io n it m a y b e n e cessa ry to e lo n g a te th e q u a d r ic e p s te n d o n a b o v e th e k n e e a n d very rarely a su p ra co n d y la r o s t e o t o m y fo r se v ere recu rv a tu m m a y b e req u ired . F le x io n c o n tr a c tu r e s o f th e k n e e are m u c h less c o m m o n , a n d are se en in ch ild ren w h o are a b le to w a lk b ut suffer fro m a se v ere c a lc a n e u s d e fo r m ity . W h en th e fixed e x te n sio n o f th e fe e t is c o r r e c te d , sp o n ta n e o u s c o r r e c tio n o f th e k n ee d e fo r m ity u su a lly o c c u r s a n d th e trea tm en t o f th e se k n ees is the trea tm en t o f th e d e fo r m ity o f th e f o o t. T h e w h e e l-c h a ir c r ip p le m a y d e v e lo p a p o stu r a l fle x io n c o n tr a c tu r e o f th e k n e e , b ut th is u su a lly r e sp o n d s t o g en tle stretch in g . S u rg ery is o n ly o c c a s io n a lly n e cessa ry fo r th e fle x io n c o n tr a c tu r e . W h en req u ired , d e ta c h m e n t o f th e sh o r te n e d h a m str in g s a n d r e -in se r tio n in to th e b a ck o f th e lo w e r e n d o f th e fe m u r a n d a p o ste r io r c a p s u lo to m y o f th e k n ee j o in t w ill u su a lly se cu re c o r r e c tio n . In th e very rare c a s e a c o r r e c tiv e su b tr o c h a n te r ic o s t e o t o m y m a y be in d ica ted . It is n o te w o r th y h o w e v e r , th a t in fix ed d e fo r m ity a m u sc le m a y ta k e o n a fu n c tio n o p p o s it e to n o r m a l. F o r e x a m p le , in a k n ee fix ed in fle x io n , th e ilio -tib ia l tract, w h ich u su a lly e x te n d s th e k n e e , m a y b e c o m e a flex o r. S im ilarly in th e recu rved k n ee th e sa r to r iu s a n d g r a c ilis, n o rm a lly fle x o r s, b e c o m e e x te n so r s in a c tio n a n d th u s p e r p e tu a te the d e fo r m ity . F o r th is r e a s o n su rg ic a l c o r r e c tio n m u st n o t be d e la y e d w h e n th e d e fo r m itie s are fixed . T h e H ip s B y th e a g e o f 3 y ea rs, h a lf th e ch ild ren su fferin g from m y e lo m e n in g o c o e le w ill h a v e d islo c a te d h ip s. A p p r o x im a te ly o n e third o f th is n u m b er w o u ld h a v e b e e n d islo c a te d from b irth. In all th e se ch ild r e n , th e sp in a l le s io n is in the m id -lu m b a r reg io n , th e h ip flex o rs a n d a d d u c to r s are spared b ut e x te n so r s a n d a b d u c to r s are p a ra ly se d . T h is m u sc le im b a la n c e p r o d u c e s, a p art fro m a fle x io n , a d d u c tio n a n d extern al r o ta tio n c o n tr a c tu r e at th e h ip j o in t , a d e fo r m a tio n o f th e n e c k o f th e fem u r b e c a u se o f a n u n o p p o s e d p u ll o n th e lesser tro c h a n te r . E x p er im en ta l stu d ie s o n a s o fte n e d fem u r h a v e e s ta b lish e d th a t a v a lg u s a n d a n te v e r sio n d e fo r m ity o f th e fe m o r a l n eck is th e c o n s e q u e n c e o f th is fo rm o f m u sc le im b a la n c e (B r o o k e s a n d W ard le, 1962). T h e c h a n g e in a ttitu d e o f th e fem o r a l n eck c o m b in e d w ith a n a b d u c to r w e a k n e ss, p r o d u c e s an in e v ita b le d islo c a tio n o f th e h ip . U n lik e th e c o n g e n ita l ty p e, w h en th e d islo c a tio n is p resen t fr o m b irth or s o o n th e rea fter, th e a c e ta b u lu m m a in ta in s a sa tisfa c to r y sh a p e a n d d e p th fo r several years (F ig . 5a). T h e su b lu x in g h ip o n th e o th e r h a n d , p r o d u c e s a slo p in g a c e ta b u lu m . In th e p a st, th e h ip d is lo c a tio n o f th e m y e lo m e n in g o c o e le ch ild h a s u su a lly b een a b a n d o n e d a s a n u n co rrecta b le c o n d itio n , o r treated b y v a ru s an d r o ta tio n fe m o r a l o s t e o ­ to m ie s , o p e n r e d u c tio n s a n d s h e lf o p e r a tio n s to d e e p e n the a c e ta b u lu m . In th e fa c e o f m u s c le im b a la n c e , h o w e v e r , th e d e fo r m itie s recur a n d r e -d islo c a tio n is in e v ita b le . F o r th e sa m e r e a so n , so ft tissu e relea ses su c h a s th e S o u tte r slid e o p e r a tio n a n d a d d u c to r te n o to m ie s p r o d u c e p o o r resu lts. M o r e rece n tly Sharrard (1 9 6 4 ) h a s p r o d u c e d a r e v o lu tio n in th e m a n a g e m e n t o f th e se d islo c a te d a n d c o n tr a c te d h ip s b y c o m b in in g a m u sc le b a la n c e o p e r a tio n w ith a n o p e n r e d u c tio n a n d r elea se p ro ced u re. T h e m a in d e fo r m in g fo r c e in th e se y o u n g p a tie n ts is th e p o w e r fu l ilio p s o a s m u sc le , w h ic h a lso p r o v id e s a c o n v e n ie n t r e p la cem en t fo r th e p a ra ly se d a b d u c to r g r o u p . In h is o p e r a tio n , its te n d o n o f in se r tio n is re m o v e d fro m th e lesser tro ch a n ter , r o u te d th r o u g h a h o le m a d e in th e ilia c b la d e, a n d re-in serted in to th e b a ck o f th e greater tr o c h a n te r . O n c e th e ilio p s o a s is d e ta c h e d fr o m th e lesser tro ch a n ter , h ip r e d u c tio n b e c o m e s q u ite ea sy . N e v e r th e le s s th e a c e ta b u lu m sh o u ld a lw a y s be o p e n e d a n d clea red o f fib r o u s tissu e. In its n e w p o sitio n the ilio p s o a s m a y w ell fu n c tio n a s a n a b d u c to r a n d e x te n so r o f th e h ip , b ut e v e n w h e r e it d o e s little b e sid e p r o v id e a te n o d e sis effe ct, th is is a lw a y s su fficien t to en su r e c o n tin u e d 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. ) Page 10 P H Y S I O T H E R A P Y December, 1969 l o c a t i o n o f t h e h i p j o i n t a n d p r e v e n t r e c u r r e n c e o f the flexion c o n t r a c t u r e . T h i s o p e r a t i o n is q u i t e ex te n si v e but well w o r t h w h i l e ( Fi g. 56 a n d c). B e ca u se t h e a c e t a b u l u m u su a l ly r e m a i n s well d e v e l o p e d in t he m y e l o m e n i n g o c o e l e ch i l d , in m o s t ca s es t h e r e is no h a r m in d e l a y i n g t h e p r o c e d u r e unt il th e c h i l d ’s ge n er al h e a l t h is g o o d a n d r e h a b i l i t a t i o n well e s t a b l is h e d . O n c e the h i p is r e d u c e d it is n o t e w o r t h y t h a t t h e v al g u s a n d a n t e - v e r s i o n d e f o r m i t y o f t h e f e m o r a l ne ck g r a d u a l l y d i s a p p e a r 5a ev en w h e n p a t i e n t s a r e a l r e a d y in t h e i r ea rl y tee ns . I n the o l d e r p a t i e n t , t h e f e m o r a l ne ck m a y h a v e to be c o r r e c t e d by a s u i t a b l e o s t e o t o m y . In t h e s u b l u x i n g t y p e o f h i p w h e r e a c e t a b u l a r s h a p e is u n s a t i s f a c t o r y , t h e i l i o p s o a s t r a n s f e r o p e r a t i o n m a y c o n ­ v e n ie nt ly be c o m b i n e d w i t h a pelvic o s t e o t o m y ( S a l te r, 1961) o r s h e l f o p e r a t i o n d e s i g n e d e i t h e r t o sw in g th e a c e t a b u l u m o v e r t h e f e m o r a l h e a d o r t o d e e p e n it su i t a b l y . A f t e r a n i l i o p so a s t r a n s f e r o r c o m b i n e d o p e r a t i o n , the p a t i e n t is k e p t in a h i p sp i c a w i t h t h e o p e r a t e d li m b held a b d u c t e d a n d i n t e r n a l ly r o t a t e d f o r 3 i t o 5 w ee k s a n d t h e r e ­ a f t e r a l l o w e d free t o r e h a b i l i t a t e . T h e r es u lt s h a v e been very s a t i s f a c t o r y in r e p o r t e d series a n d c o n f i r m e d by the 16 o p e r a t i o n s p e r f o r m e d at t h e S p i n a l D e f e c t s C l i n i c o f th el T r a n s v a a l M e m o r i a l H o s p i t a l f o r C h i l d r e n . T h e Fl ail L o w e r L im b W h e r e t h e r e is c o m p l e t e p a r a l y s i s m u s c le i m b a l a n c e c a n n o t exist. T h e h i p s ne v e r d i s l o c a t e a n d c o n t r a c t u r e s ar e a l w a y s s e c o n d a r y t o p o s t u r a l o r o u t s i d e fo rce s a n d r e s p o n d to r e g u l a r p a s si v e s t r e t c h i n g . P h y s i o t h e r a p y t o m a i n t a i n full k n e e e x t e n s i o n a n d p l a n t i g r a d e feet will a l l o w th e us e o f 5 b l o ng - le g c a l i p e r s a n d pelvic b a n d s . E v e n w h e n l i m ite d t o a t r i p o d g ai t, c r u t c h w a l k i n g in th e s e p a t i e n t s is us u a l ly very s a t i s f a c t o r y . S P O N T A N E O U S F R A C T U R E S A p p r o x i m a t e l y 25 p e r c e n t o f o u r m y e l o m e n i n g o c o e l e p a t i e n t s h a v e s u s t a i n e d s p o n t a n e o u s f r a c tu r e s . U s u al l y th es e f o l lo w p e r i o d s o f i m m o b i l i s a t i o n in a h i p sp i c a af t er s u r g e r y . T h e f r a c t u r e s o c c u r c o m m o n l y t h r o u g h t he m e t a ­ p h y s e a l r e g i o n a n d a r e a l w a y s p ai n l es s, b e i n g d i a g n o s e d by a b n o r m a l m o b i l i t y a n d sof t tiss ue swelling. It is well r e c o g n i s e d t h a t i m m o b i l i s a t i o n in t h e s u p i n e p o s i t i o n p r o d u c e s d e c a l c i f i c a t i o n , n e v e r t h e l e s s c h i l d r e n with p o l i o m y e l i t is u n d e r g o i n g s i m i l a r o p e r a t i v e p r o c e d u r e s an d i m m o b i l i s a t i o n r ar e ly d e v e l o p s p o n t a n e o u s f rac tu re s. F u r t h e r m o r e , in s o m e in st a n c e s, f r a c t u r e s ha ve o c c u r r e d s p o n t a n e o u s l y in t h e m i d - s h a f t o f a b o n e w i t h o u t a pe r i o d o f i m m o b i l i s a t i o n a n d w i t h n o t r a u m a . T h e s e p a t i e n t s u s u a l ly p r e s e n t w i t h q u i t e g r o s s sw el lin g o f t h e af fe ct ed leg o r t h i g h , m i l d p y r e x i a , local he a t a n d r ed n es s , b u t w i t h no s y s t e m i c t ox ic signs. A d i a g n o s i s o f a c u t e o s t e o m y e li t i s is p r e s u m p t i v e un t i l t h e X - r a y r ev ea ls m a r k e d ea r l y ca l l u s in a ver y l ar ge s u b - p e r e o s t e a l h a e m a t o m a . T h e o b v i o u s b l e e d i n g u n d e r th e p e r i o s t e u m is s i m i l a r to s c u rv y , a n d it h a s be en s u g g e s t e d ( M c K i b b e n a n d P o r t e r , 1967) t h a t th e s e p a t i e n t s h a v e a n a b n o r m a l V i t a m i n C m e t a b o l i s m , r e s e m b l i n g a c h r o n i c def iciency. T h e f r a c t u r e s a l w a y s h eal r a p i d l y a n d d o n o t a p p e a r to 5c p r e s e n t a n y l on g t e r m p r o b l e m b u t it w o u l d s e e m r e a s o n a b l e to p r o v i d e a d d i t i o n a l V i t a m i n C f o r t he se p a t i e n t s a n d to a v o i d u n d u e i m m o b i l i s a t i o n p a r t i c u l a r l y in th e s u p i n e p o s i t i o n . R E H A B I L I T A T I O N I n t h e a b s e n c e o f se v e re m e n t a l defic ienc y, a l m o s t every m y e l o m e n i n g o c o e l e p a t i e n t c a n be t a u g h t to w a l k . O n ce d e f o r m i t i e s a r e c o r r e c t e d a n d t he c h il d is u p r i g h t , the li m i ti n g f a c t o r s a r e th e d e g r e e o f m o t i v a t i o n in p at ie n t, p a r e n t s a n d a s s o c i a t e d p e r s o n n e l . F ig . 5 : B i l a t e r a l di slo ca tio n o f the hips in a 5 - y e a r old girl (a) we re co r re c t e d by open r ed uc t i on a n d il iopsoas transf er s. T h e hole th r o u g h which th e t r a n s f e r r e d il iopsoas p as se d can be se en in t he left iliac b l a d e an d t h e po int o f i ns e rti on o f the tr a n s p o s e d ten d on is still visible in th e rig h t g r e a t e r t r o c h a n t e r (b). An up ri g h t p o s t u r e an d as si st e d wa l ki n g is now possible (c). 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, 1969 P H Y S I O T H E R A P Y Page 11 A p p lia n c e s are req u ired to h old u n sta b le jo in ts b u t m a y n ever b e u sed to a tte m p t c o r r e c tio n o f a fix ed d efo rm ity . W h en this e x ists, it is better to c o n to u r th e a p p lia n c e to the d efo rm ity th a n to e n d e a v o u r to b en d th e lim b to a rigid ly d esig n ed sp lin t. C a lip ers n eed t o b e ca r e fu lly m a d e to a v o id pressu re o n in se n s itiv e sk in a n d it is e sse n tia l that p a tie n ts a n d p aren ts b e ta u g h t t o r e m o v e a p p lia n c e s and b o o ts se v era l tim e s a d ay to in sp e c t fo r sig n s o f sk in irrita tio n . B o o t s w ith la c e s th a t e x te n d to th e to e s are a v a lu a b le sa fety p r e c a u tio n , s o th a t in se n sitiv e feet m a y be c o rre ctly situ a te d u n d er v isio n . B e lo w th e k n e e , th e u se o f in sid e a n d o u ts id e iro n s, T -stra p s, fro n t a n d b a ck s to p s all h a v e th e u su a l ro le to p lay in c o n tr o llin g v a r io u s p a ra ly se s o f th e f o o t. A sim p le straight c a lip er is u su a lly b est fo r th e u n sta b le k n ee b u t as the c h ild g r o w s o ld e r it is a d v isa b le to fit a k n e e h in g e to fa cilita te sittin g at a s c h o o l d esk . T h e im p o r ta n c e o f p h y sio th e r a p y an d o c c u p a tio n a l th erap y c a n n o t b e o v e r -e m p h a s ise d . C e n tr a lisa tio n o f th e se se rv ices a llo w s for g r o u p th e ra p y th a t in its e lf p r o v id e s an im p o r ta n t b o o s t fo r th e p a tie n t. In th is regard w e are fo r ­ tu n a te in h a v in g th e fa c ilitie s o f th e H o p e S c h o o l a n d H o m e s . 'V e r y g o o d p h y sica l a m e n itie s e x ist, a n d sta ff en th u sia sm and ex p e r tise are o u ts ta n d in g . T h e ch ild r e n p ro g ress ste a d ily fro m th e p o o l to p a ra llel bars a n d u ltim a te ly t o in d e p e n d e n t w a lk in g . T o ta l p a tie n t r e h a b ilita tio n m u st n o t b e n eg le c te d . A d e q u a te s c h o o lin g is e s se n tia l a n d th e p r o v isio n o f w o r k ­ sh o p a n d o th e r fa c ilitie s th a t c a n eq u ip h a n d ic a p p e d p a tie n ts w ith a practical tra in in g w ill e n a b le th e m t o ta k e their rightful p la c e in so c ie ty . J o h n G a llsw o r th y h a s o b se r v e d “ A n ic h e o f u se fu ln e ss a n d a se lf-resp e ct e x is ts fo r e v ery m a n h o w e v e r h a n d ic a p p e d , b u t th a t n ic h e m u st be fo u n d for h im .” S U M M A R Y T h e m a n a g e m e n t o f m y e lo m e n in g o c o e le c a lls fo r an en erg etic a n d c o -o r d in a te d trea tm en t reg im e th a t in v o lv e s several d isc ip lin e s. T h e o r th o p a e d ic su r g e o n m u st r e c o g n ise and a sse s s fu n c tio n . In s e n sitiv e sk in p reclu d es th e u se o f m a n ip u la tio n s, p la ster c a sts an d sp lin ts, a n d th e trea tm en t is su rg ic a l in th e first in sta n ce. D iv is io n a n d e lo n g a tio n o f tig h t stru ctu res w ill reverse o th e r w ise p ro g ressiv e c o n tr a c ­ tures a n d j o in t d is lo c a tio n s , and se le c te d m u sc le reb a la n cin g b y te n d o n tran sfers c a n p rev en t r ela p ses. J o in t a rth ro d esis m a y p la y a part b u t o n ly in c o n ju n c tio n w ith reb a la n cin g o p e r a tio n s. A su b s e q u e n t u n ified p r o g r a m m e o f reh a b ilita tio n e n a b le s th e m a jo r ity o f th e se p a tie n ts t o w alk in d e p e n d e n tly an d m a n y w ill lead u sefu l liv es. R E F E R E N C E S 1. B r o o k e s, M . a n d W a rd le, E . M . (1 9 6 2 ): M u sc le a c tio n a n d th e S h a p e o f th e F e m u r . J o u rn a l o f Bone a n d J o in t S u rg e r y . 4 4 B . 398. 2 . B r o w n , A . (1 9 6 8 ): A S im p le M e th o d o f F u s io n o f th e S u b ta la r J o in t in C h ild ren . J o u rn a l o f B on e a n d J o in t S u rg e r y . 50B . 364. 3. G r ic e , D . S. (1 9 5 2 ): A n E x tra -A rticu la r A r th r o d e sis o f th e S u b a stra g u la r J o in t fo r C o r r e c tio n o f P a ra ly tic F la t F e e t in C h ild ren . J o u rn a l o f B o n e a n d J o in t S u rg e r y . 3 4 A . 9 2 7 . 4. H a n d e lsm a n , J. E . (1 9 6 8 ): T r ea tm en t o f C o n g e n ita l V ertica l T a lu s . Jo u rn a l o f B o n e a n d J o in t S u rg e r y . 50B . 4 3 9 . 5. M c K ib b e n , B. a n d P o rter , R . W . (1 9 6 7 ): T h e in cid en ce o f V ita m in C D e ffic ie n c y in M e n in g o m y e lo c e le . D e v e lo p . M e d . C h ild N e u r o l. 9. 338. 6. S a lte r, R . B. (1 9 6 1 ): I n n o m in a te O s te o to m y in th e T r e a tm e n t o f C o n g e n ita l D is lo c a tio n a n d S u b lu x a tio n o f th e H ip . J o u rn a l o f B o n e a n d J o in t S u rg e ry . 4 3 B . 518. 7. S e y m o u r , N . a n d E v a n s, D . K . (1 9 6 8 ): A M o d ific a tio n o f th e G r ic e S u b -T a la r A r th r o d e sis. J o u rn a l o f B o n e a n d J o in t S u r g e r y . 5 IB . 372. 8. S h arrard , W . J. W . (1 9 6 4 ): P o ste r io r Ilio p s o a s T r a n s­ p la n ta tio n in th e T r e a tm e n t o f P a r a ly tic D is lo c a tio n o f th e H ip . J o u rn a l o f B o n e a n d J o in t S u rg e r y . 4 6 B . 4 2 6 . 9. Sharrard , W . J. W . (1 9 6 4 ): T h e S e g m e n ta l In n e r v a tio n o f th e L o w e r L im b M u sc le s in M a n . A n n a ls o f th e R o y a l C o lle g e o f S u rg e o n s o f E n g la n d . 35 (2). 106. 10. Sharrard , W . J. W . (1 9 6 8 ): S p inal O ste o to m y fo r C o n g e n ita l K y p h o s is in M y e lo m e n in g o c o e le . Jo u rn a l o f B o n e a n d J o in t S u rg e r y . 5 0 B . 4 6 6 . 11. S o lo m o n , L . a n d H a n d e lsm a n , J. E. (1 9 6 7 ): T h e T r ea t­ m e n t o f C lu b F o o t . S o u th A fric a n J o u rn a l o f S u rg e ry . 5 ( 1 ) . 31. The Social W o rke r in the Spinal Defects Team M R S . N . S H N E I E R S o c ia l W o rk er, T r a n sv a a l M e m o r ia l H o sp ita l fo r C h ild ren , J o h a n n e sb u r g T h e so c ia l w ork er a tta ch ed t o th e S p in a l D e f e c t s C lin ic is in v o lv e d w ith th e p erso n a l p r o b le m s o f th e p a tie n ts and their p a ren ts. S o m e o f th e se p r o b le m s ex isted b e fo r e th e ch ild w a s b o r n , a n d n o w b e c o m e u n b ea ra b le, b u t m a n y o th e rs are th e resu lt o f h a v in g a h a n d ic a p p e d c h ild in th e fam ily. S o m e fa m ilie s fin d th e a d d itio n a l stra in t o o great, an d th e re is a th r ea te n e d c o lla p se o f th e m arria g e. In te n siv e c a sew o rk a n d c o u n se llin g w ith th e se p a ren ts m u st th e n b e u n d erta k en in a n effort t o so r t o u t fe e lin g s and c o m e to so m e s o lu tio n fo r th e fu tu re. O n e o f th e c o m m o n e s t c a u se s o f ill-feelin g is g u ilt a n d th e d ifficu lty o f a p p o r tio n in g b lam e. T h e p h y sica l e ffe c ts o f m y e lo m e n in g o c o e le e x te n d righ t through th e b o d y . B e sid e s th e q u e stio n o f in crea se in h ead siz e, th e re m a y b e w e a k n e ss in th e leg s, th e b o w e ls a n d th e bladder. S o m e ch ild r e n m a y h a v e a ll o f th e se d ifficu lties; so m e p erh a p s, o n ly o n e , a n d th a t in a very slig h t degree' T o th e p aren ts, o n th e w h o le , th e o n e w h ich is m o s t d is­ turbin g e m o tio n a lly is in crea se in th e siz e o f th e h ea d , th o u g h th e o n e th a t c a u se s m o st tr o u b le p ra ctica lly is w e a k ­ ness o f b o w e ls or b lad der. B u t c erta in ly m o re im p o r ta n t to m o st p e o p le th a n a n y p h y sic a l h a n d ic a p is w h eth er o r n o t th e c h ild is n o rm a l in in te llig e n c e . A s a ru le, a c c e p ta n c e c o m e s m o r e e a s ily i f it is. F o r m o s t p a ren ts th e first c o n ta c t w ith th e h o sp ita l is in th e w a rd , a s m o st ch ild ren are a d m itte d fo r in v e stig a tio n an d trea tm en t s o o n a fter b irth . M a n y p e o p le k n o w n o th in g at all o f th e c o n d itio n , a n d t o m e e t it fo r th e first tim e in their o w n c h ild is a n in te n s e ly a la rm in g situ a tio n , a n d o n e w h ich is lia b le t o e v o k e v ery str o n g fe e lin g s o f g u ilt. N o r are th e y h e lp e d , in s o m e c a se s, b y lo c a l d o c to r s a n d n u rses w h o h a v e n o fa ith in th e m o d e r n d e v e lo p m e n t o f trea tm en t fo r c h ild r e n ; a n d t h o s e w h o h a v e b een to ld th a t th e b e st th e y ca n h o p e fo r is a n ea r ly d ea th , m a y b e fa c e d w ith th e p a in fu l retu rn o f h o p e fo r a ch ild a lrea d y m o u r n e d . A fte r th e first sh o c k is o v e r , m o s t p e o p le are v ery a n x io u s fo r a s m uch in fo r m a tio n a s th e y c a n g e t. S y m p a th e tic a n d ca refu l e x p la n a tio n b y th e m ed ica l a n d n u r sin g sta ff a t th is sta g e d o e s a g rea t d e a l t o m a k e th in g s le ss m y ste r io u s a n d less fr ig h ten in g . Id e a lly , all th e p aren ts s h o u ld b e se e n b y th e so c ia l 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. )