December, 1969 P H Y S I O T H E R A P Y Page 3 Spina Bifida and M yelom eningocoele M I C H A E L K A T Z E N , M .B .B .C h . (R a n d ), F .R .C .S . (E d in .) P art-tim e P aed iatric S u rg eo n , T r a n sv a a l M em o ria l H o sp ita l for C h ild ren an d U n iv ersity o f th e W itw atersran d , J o h a n n esb u rg S p in a B ifida is a co n g e n ita l a b n o r m a lity c o n sis tin g o f a se p a r a tio n o f th e sp in e o f a verteb ra in to tw o halves. It m ay o c c u r in v a r io u s fo rm s d ep en d in g o n a sso c ia te d featu res. 1. S p ina Bifida O ccu lta T h is c o n sis ts o f th e b o n y d e fo r m ity a lo n e a n d is n o rm a lly p resen t in 5 -1 0 per c e n t o f p e o p le in the region o f L .5 .- S .1 . A p a rt from sk in b lem ish es o v e r the area it very rarely c a u se s a n y p a th o lo g ic a l sy m p to m s. / 2. M en in g o co elc In th is c o n d itio n th e sp in e o f th e verteb ra is bifid and b etw een th e tw o h a lv es a cyst o f th e m e n in g e s p rotru des t o fo rm a lu m p o n th e b ack co v e r e d by sk in . T h e cord is n o rm a l. T h e o n ly p rob lem is a c o s m e tic o n e a n d th is m ay b e co rrected at a n y c o n v e n ie n t tim e. 3 . M y elo m en in g o co elc T h is c o n sis ts o f a d efo rm e d sp in a l cord o v er several se g m e n ts su rroun d ed by a cy st o f the m en in g e s. T h is b u lges b etw een th e bifid sp in e s o f several verteb rae an d a p p ears a as m a ss o n th e b ack u su a lly u n covered by sk in in its cen tral p o r tio n . It m a y o c c u r in a n y a rea o f the sp in e b u t is m o st c o m m o n in th e lu m b ar area fo llo w e d by the sacral and th o r a c ic areas. It o c c u r s at lea st tw en ty tim es a s c o m m o n ly as m e n in g o c o e le , is th e c o m m o n e s t sin gle su rgical a b n o r m a lity seen o n th e first d a y o f life and o ccu rs in 2 -3 /1 0 0 0 liv e births, S in ce it is m y e lo m e n in g o c o e le w h ich c o n s titu te s th e m ajor th e ra p eu tic p ro b lem further rem ark s w ill be co n fin e d to this a b n o rm a lity . P A T H O L O G Y M y e lo m e n in g o c o e le has several se q u ela e : 1. B e c a u se it is u n co v ered b y sk in it is lia b le to in fe c tio n a n d u n treated ch ild ren d ie o f m en in g itis o r ven tri­ cu litis in a h igh p ercen ta g e o f ca ses. 2. In 80 per c e n t o f c a se s th e re is a n a sso cia ted h y d r o c e p h a lu s. T h is is d u e t o an a b n o r m a lity o f th e b ase o f th e b rain (th e A rn o ld -C h ia r i p h e n o m e n o n ) c o n sis tin g m a in ly o f h e r n ia tio n o f part o f th e cere­ b ellu m and m e d u lla th r o u g h th e fo ra m e n m a g n u m a n d a r a c h n o id a d h e sio n s p rev en tin g th e free c ircu la ­ tio n o f C .S .F . I f u n tre a ted , th e se ch ild ren d ev elo p m a ssiv e e n la rg em en t o f th e h ead and m a y b e m en ta lly d efectiv e . 3. T h e d e fo r m ity o f th e sp in a l co rd p ro d u ces parap legia b e lo w th e lev el o f th e le s io n w h ich in d u c e s n o t o n ly m o to r a n d se n so ry lo ss to th e lo w er lim b s o f vary in g d egree, but a ls o d efo rm ity . T h e parap legia a ls o p ro d u ces blad der p a ra ly sis w ith its se co n d a r y p ro b lem s o f in c o n tin e n c e o r se c o n d a r y in fe c tio n . T h is in fe c tio n m ay u ltim a te ly c a u se d eath after several years if u n relieved . T h e a n u s and rectum are also p aralysed in th e se ch ild ren p ro d u cin g n o t m erely in c o n tin e n c e b ut o fte n severe c o n stip a tio n b eca u se o f lo s s o f b o w e l se n sa tio n . M A N A G E M E N T T h e p resen t-d a y a p p r o a c h t o trea tm en t is a n ag g ressiv e o n e sta rtin g o n th e first d a y o f life. T h e sa c is su rg ic a lly c lo se d w ith in h o u rs o f birth in th e h o p e that th is w ill preven t m en in g itis. It h as a ls o b een sh o w n b y Sharrard e l a l 1 that early c lo su r e p rev en ts d e te r io r a tio n in th e p a ra p leg ia d u e to in fe c tio n o r dryin g o u t o f th e neural tub e. W h en this p h a se is o v e r , a w a tch is kept for th e d e v e lo p ­ m en t o f h y d ro cep h a lu s ju d g ed m a in ly b y in creased h ead circu m feren ce. If th is is n o te d to be p ro g ressiv e (a b o u t 5 0 per cen t o f ca ses) d ra in a g e o f the h y d r o c e p h a lu s is in stitu ted . T h is is a ch iev ed by v en tr ic u lo -a tr ia l sh u n t, w h ich c o n sis ts o f in ser tin g a tu b e th r o u g h a b u rr-h o le in th e sk u ll in to o n e o f th e lateral v en tricles an d th e n le a d in g th is sub- c u ta n e o u s ly in to th e ju g u la r v e in a n d th r ea d in g it in to the righ t atriu m o f th e heart. T h e ap p aratu s in c o r p o r a te s a pu m p w hich lies under th e sk in and ca n be p a lp a ted , a n d a v a lv e w h ich o p e n s at a sp ec ific pressu re an d a llo w s d ra in a g e o f C .S .F . in to th e b lo o d stream and p rev en ts reflux o f b lo o d in to the v en tricle. T h e c o m m o n v a lv e s in use are the P u d en z-H ey er a n d th e S p itz -H o lte r . T h ese v a lv es require freq u en t revision a s th e y m a y b lo ck d u e to d ebris o r c lo t at th e distal en d particu larly w h en th e y are w ith d ra w n from th e atrium in to the ju g u la r v e in w ith g ro w th o f th e c h ild . M o s t ch ild ren o n ly require th e se v a lv e s up to th e a g e o f tw o years w h en their h y d r o c e p h a lu s rem its sp o n ta n e o u sly b u t q u ite a few require th e m fo r the rest o f their liv es. A carefu l ch eck m u st be m a d e at regular intervals to a sc erta in th a t th e v a lv e is fu n c tio n in g . T h is can be d o n e b y p a lp a tin g th e p u m p and by h ead m ea su rem en t b efore th e su tu res are c lo s e d . O n c e th e su tu r e s are u n ited , th e o n ly sig n s o f n o n -fu n c tio n are th o s e o f in creased in tr a ­ cran ial pressure, e.g . h e a d a c h e , d ro w sin ess and v o m itin g . O n ce th e h y d ro cep h a lu s is c o n tr o lle d , a tte n tio n is turned to th e o r th o p a e d ic a n d u ro lo g ic a l a sp e c ts o f th e parap legia w h ich are d ea lt w ith in th e fo llo w in g articles. It is v ita lly im p o r ta n t that th e v a rio u s d isc ip lin e s c o n ­ cerned c o -o r d in a te in th e m a n a g e m en t o f th e se ch ild ren . T o this e n d sp ecia l c lin ic s m ust be esta b lish ed w h ere th e sp e c ia lists c o n cern ed c a n se e th e se p a tie n ts to g eth er and d iscu ss their in d iv id u a l m a n a g e m en t. S u ch a co m b in e d clin ic is held w eek ly at th e T ransvaal M em o ria l H o sp ita l for C h ild ren in J o h a n n e sb u r g a n d is run b y a p aed iatric su r g e o n , o r th o p a e d ic su rg e o n a n d u r o lo g ist. A n o p th a l- m o lo g is t a lso a tte n d s b eca u se o f th e h igh in cid en ce o f sq u in ts. A n o r th o p a e d ic tec h n ic ia n , so c ia l w ork er, e d u c a ­ tio n a l p sy c h o lo g ist a n d p h y sio th e r a p ists c o m p le te th e c o m p le m e n t. S in c e th e m a n a g e m en t is so c o m p le x , it is lik ely th a t, n o m atter h o w g o o d a h o m e th is ch ild c o m e s from , it is b est m a n a g e d e ith er a s an in -p a tien t o r an o u t-p a tie n t in an in stitu tio n sp e c ia lly geared t o its p ro b lem s and w h ere ex p ert n u rsin g, p h y sio th era p y , o c c u p a tio n a l th erap y, and s c h o o lin g c a n be p rovid ed u n d er o n e ro o f. T h e H o p e S c h o o l a n d H o m e s h a v e p roved ideal fo r th is p u rp o se in J o h a n n esb u rg . B e c a u se o f th e p r o fo u n d im p a ct su ch a crip p led ch ild m a k e s o n h is h o m e , th e v a r io u s social p ro b lem s sh o u ld be atten d ed t o b y a so c ia l w orker. R E S U L T S O F T R E A T M E N T If an e n erg etic p ro g ra m m e o f trea tm en t is in stitu ted from birth, a b o u t h a lf o f th e ch ild ren w ith m y e lo m e n in g o c o e le w ill su rvive and a b o u t 85 per cen t o f the su rv iv o r s w ill h a v e 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 4 P H Y S I O T H E R A P Y December 1969 rea so n a b le in te llig en ce. B e ca u se o f their severe d isa b ility , it is lik e ly that a la r g e p r o p o r tio n w ill require s o m e form o f sh eltered e m p lo y m e n t in th e future. T H E E T H IC A L P R O B L E M I t m ig h t b e arg u ed th a t th e se ch ild ren w o u ld b e better left u n tre a ted in th e h o p e that th e y w o u ld s o o n die. H o w e v e r , w e k n o w fr o m th e stu d ie s o f L a u re n c e2 in C ard iff th a t, if c o m p le te ly u n tre a ted , a b o u t 2 0 per cen t w ill su rvive a n y w a y . T h e s e w ill th e n be se v erely d isa b le d , m a n y w ith large h ea d s a n d tu m o u r s o n their b a ck s a n d w ith a h igh in cid en ce o f m en ta l d eficien cy . A c tiv e trea tm en t therefore p r o d u c e s m o r e c rip p les b ut few er m e n ta lly d eficien t ch ild ren w ith less d efo rm ity . A t p resent, the refo re, a c tiv e treatm en t a p p ea rs th e b est a ltern a tiv e p articu larly sin c e ad v a n ces, e .g . e le c tr o n ic a lly c o n tr o lle d b lad ders, etc. are a future p ro sp ec t. S e le c tio n o f c a se s fo r treatm en t d o e s n o t ap p ea r to be a ten a b le s o lu tio n sin c e there is n o m ea n s o f te llin g , a t birth, w h ich c a se s w ill e ith er su rvive o r d o w ell o n trea tm en t. S h o u ld o n e th e n h a v e left a ch ild u n tre a ted a n d it su rvives, o n e m ig h t h ave m isse d th e o p p o r tu n ity o f a c h ie v in g the best resu lt a s regard s r e h a b ilita tio n . T H E F U T U R E T h e e m b r y o lo g ic a l d efect in m y e lo m e n in g o c o e le o ccu rs w ith in th e first 6 w e e k s o f fo e ta l life a n d , th o u g h m any th e o r ie s e x ist, th e precise m ech a n ism is o b scu re. In m a n y c a se s th e a b n o r m a lity p ro b a b ly o ccu rs as a result o f s o m e p a th o lo g y in th e m o th er, e .g . viru s d ise ase ta k in g o f d rugs, e tc . th o u g h th is is n o t p r o v e n . H o w ev er’ it is certa in that in a high p r o p o r tio n o f c a se s a genetic fa c to r e x ists, th e tr a n sm issio n o f w h ich is ill-u n d erstood P aren ts sh o u ld be w arn ed that after the b irth o f a child w ith m y e lo m e n in g o c o e le the c h a n c e s o f h av in g a fu rth e r ch ild w ith th e a b n o r m a lity is a b o u t 1 :25. A fte r a se co n d ch ild is b o rn w ith th e a b n o r m a lity th e c h a n c e o f a defect in a n o th e r ch ild is a b o u t 1 :8. T h e ch a n c e s w o u ld o b v io u s ly be in crea sed t o o if there w a s a h isto ry o f any cen tral n e r v o u s sy stem d efect in th e im m ed ia te fam ily. S in ce there is a g e n e tic fa cto r in v o lv e d , th e lik elihood e x ists that m y e lo m e n in g o c o e le w ill b e c o m e m o r e prevalent in fu tu r e g en era tio n s. W e are u n lik e ly to b e a b le to prevent th e c o n d itio n o ccu rrin g in the fo r e se e a b le future a n d should th e r e fo r e m a k e str e n u o u s effo r ts t o in crease ou r therapeutic p o te n tia l in th e reh a b ilita tio n o f th e se u n fo rtu n a te children. R E F E R E N C E S \ 1. S h a r r a r d , W . J. W ., Z a c h a r y , R . B ., L o r b e r , J. and B r u c e , A . M . (1 9 6 3 ) A rch . D is . C h ild ., 3 8 :1 8 . 2. L a u r e n c e , K . M . (1 9 6 6 ), D e v . M ed . C h ild . N eu ro l S u p p l. N o . 11, p. 10. The M anagem ent of Urological Complications of M yelom eningocoele J. A . B L E L O C H , M .B .B .C h . F .C .S .S .A . U r o lo g is t, T r a n sv a a l M em o r ia l H o sp ita l fo r C h ildren T h e n erves w h ich su p p ly th e b ladder arise fro m the sacral p o r tio n o f th e sp in a l co rd . F a ilu r e o f d e v e lo p m e n t o f th is p o r tio n o f th e cord n ecessa rily resu lts in, a t least, so m e degree o f n e u r o g e n ic b lad d er d y sfu n c tio n . T h e resu ltan t sta s is o f urine in th e blad der to g e th e r w ith v esic o -u r e te r ic reflu x o f urine le a d s to persisten t in fe ctio n o f th e k id n e y s. T h e p y elo n e p h r itis w o u ld e v e n tu a lly d estroy th e ren a l su b s ta n c e i f m ea su res w ere n o t ta k e n t o p revent th is c h a in o f e v e n ts. C a u se o f d eath o f th e ch ild w ith a m y e lo m e n in g o c o e le , w h o su rv iv e s th e in itial c lo su r e o f th e d e fe c t a n d h y d r o c e p h a lu s, is c o m m o n ly renal fa ilu re d u e to p y elo n ep h ritis. T h e a p p r o a c h a d o p te d at th e S p inal D e fe c ts C lin ic, from th e u ro lo g ic a l a sp e c t, is esse n tia lly co n se rv a tiv e. T h e r eg im e c o n sis ts o f full u ro lo g ic a l in v e stig a tio n in the form o f clin ica l e x a m in a tio n a n d X -ra y stu d ies o f th e bladder a n d up p er urinary tract. T h e b ladder is filled w ith r a d io ­ o p a q u e d y e in o rd er to stu d y its o u tlin e a n d co n firm o r refu te th e p resen ce o f v e s ic o -u r e te r ic reflu x o f urin e. A n in tra­ v e n o u s p y e lo g r a m o u tlin e s th e ca ly c e s, p elv is and ureters to e s ta b lish th e fu n c tio n o f th e k id n e y s a n d th e d eg ree o f d a m a g e su sta in ed . T h e urine is ex a m in e d m ic r o sc o p ic a lly a n d cu ltured t o iso la te th e in fe c tin g o rg a n ism . T rea tm en t c o n s is ts o f clea rin g th e urinary tract in fe ctio n w ith a n a p p ro p ria te a n tib io tic , fo llo w e d by a lo n g -term trea tm en t w ith a urinary a n tise p tic su ch a s a so lu b le su lp h o n a m id e or m a n d e lic a cid . T h e urine is ex a m in ed for in fe c tio n at freq u en t in tervals. T h e m o st im p o r ta n t bu lw ark o f c o n se r v a tiv e treatm en t is su p ra p u b ic m a n u a l e x p r e ssio n o f th e b lad der. T h is re d u c e s th e resid ual urine and th e refo re the o p p o r tu n ity for in fe c tio n to g a in a fo o th o ld . T h e m o th er or n u rse is in stri c- ted to e m p ty the b ladder at 2 -h o u rly in tervals du rin g the day. W h en th e ch ild is o ld e r he is ta u g h t to e x p r e ss h is ow n bladder. X -r a y s o f th e blad der a n d k id n e y s are repeated at a p p r o x im a te ly 3 -m o n th ly intervals. I f th e in fe c tio n c a n n o t be c o n tr o lle d b y the m ea n s outlined or if there is d e te r io r a tio n o f th e k id n e y s a ssessed b y X -rays, th e ch ild is ad m itted to h o sp ita l fo r e n d o sc o p ic e x a m in a tio n . T h is e x a m in a tio n o fte n b rin gs t o lig h t a sso c ia te d uro­ lo g ic a l a b n o r m a litie s w ith o b str u c tiv e effe cts, e .g . urethral ste n o s is , urethral v a lv e s in b o y s o r p erh a p s th e p resence o f a ca lcu lu s. T r ea tm en t o f th e se le sio n s o fte n im proves the situ a tio n an d th e c o n se r v a tiv e reg im en o f 2 -h o u r ly exp res­ sio n and urinary a n tise p tic s is a g a in su ccessfu l. If, d e sp ite all th e a b o v e m easu res, in fe c tio n c a n n o t be c o n tr o lle d a n d there is d e te r io r a tio n in th e X -ra y appearance o f th e urinary tract, a c o n d u it is p erform ed. T h is e n ta ils is o la tio n o f a lo o p o f sm a ll in te stin e w ith its b lo o d su p p ly in ta c t. T h e ureters are separated from the blad der a n d im p la n ted in to o n e en d o f th e lo o p . T h e other e n d o f th e lo o p is b ro u g h t o u t t o th e sk in a s an ile o sto m y . U r in e is c o n sta n tly ex p ressed th r o u g h th e sto m a b y the h e a lth y , a c tiv e b o w e l. A rubber b a g is w o r n to co lle c t the urine. It is k ep t in p la c e w ith a d h e siv e s a n d a belt. T h er e are 39 c a se s w h o require u ro lo g ica l surveillan ce in th e c lin ic, 18 m a les a n d 21 fem a les. E lev en o f these c h ild ren h a v e n o t r e sp o n d e d to co n se r v a tiv e m a n a g e m e n t a n d c o n d u its h a v e b een fa sh io n ed to p ro v id e free, lo w p ressure d ra in a g e o f th e upper urinary tracts. 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. )