The sternomastoid "tumor" of infancy.

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    ST ERNOM ASTO ID TUM OUR AND M USCULAR TORT ICOLL IS

    DO N ALD M ACDO N ALD , SYD NEY , A U STRA L IAF rom the R oya l H osp ita lfo r S ick C h ild ren , B ris to l, E ng la ,zd

    To rtico llis due to con trac tu re o f th e ste rn om asto id m usc le is a w e ll reco gn ised en tity ,bu t the re seem s little ag reem en t a s to e tio lo gy , treatm en t o r even te rm in o log y . T he d efo rm ityis la rg e ly cosm etic and the resu lts o f trea tm en t g ra tify ing . T h e grea test in teres t lie s in thecausa tion o f th is cond ition , w ith re fe ren ce in pa rticu lar to ste rn om asto id tum our. T hepa thog enesis rem a ins unce rta in b u t app ea rs un iq ue , th ere b ein g n o know n c lin ical o rh isto log ica l p ara llel in any o th er d isea se p rocess. Th e ob jec ts o f th is p ap er a re to c la rify th erela tionsh ip of s te rn om asto id tum our to to rtico llis , to p resen t som e observ ation s o n the tw ocond itions an d to d is cu ss the re su lts o f trea tm en t.

    ET IO LOGY AND PATHOGENES IS

    D esp ite sev era l com preh en sive rev iew s , no tab ly by C hand le r and A ltenbe rg (1 944 ) an dL idge , B ech to l and L am bert (1957 ), the re is little ag reem en t abo u t the e tio logy of ste rnom as to idtum ou r or m u scu la r to rtico llis . Chand le r and A ltenb erg reg arded th e p ro gress ion from on eto the o th er a s so ce rta in th at they recomm end ed exc isio n of a ll tum ou rs at th e ea rlies t ag e.B y con tra st, H u lbe rt (1 950 ) an d C ov en try and H arris (1 959 ) con tended tha t th e m ajo rity o ftum ou rs reso lve com p le te ly w ithou t ac tive trea tm en t. G ray (19 35 ) and B ianco (1 958 ) c laim edtha t m os t ch ild ren p re sen tin g w ith to r tico llis g ive n o h is to ry o f a s ternom asto id tum our, bu tCoven try and H arris (1 959 ) s tated tha t in h alf the ir ca se s the p resen ce o f a tum ou r w as m issedby paren ts an d on ly de tected on rou tine paed ia tric exam ina tion .

    I t is now w ell e stab lished tha t a ste rn om asto id tum ou r is frequen tly p reced ed by a breech ,fo rcep s or p r im iparo us b ir th (W itze l 18 83 , F itz sim m on s 1933 , C hand le r and A ltenbe rg 1 944 ,H u lbe rt 1 950 , C ov en try and H arr is 1 959 ). It is tem p ting to assum e th at th e com m on fac to r istraum a . S trom ey er (18 38) postu lated th at rup tu re o f th e m usc le d uring p artu r ition p rod uced atum oro us haem a tom a , and tha t f ib rous rep lacem en t caused subsequ en t to rtico llis . O f f ifteenneonatal or stillbirth necropsies with sternomastoid haematomata, Spencer (1893) found thattw elv e w ere b y breech or fo rceps d e live ry . S an erk in an d E dw ards (1 96 6) dem on stra ted ex tensiv epos t-m o rtem m u sc le fib re d am age , haem orrhag e and ischaem ic chang e afte r a b reech d eliv ery .F itz simm ons (1 933 ) rev iew ed fif ty -fou r case s o f E rb s pa lsy and found an assoc ia tedste rnom asto id tum ou r in elev en . Th is h e con stru ed as fu rthe r ev id en ce th at the tum ou r w asdu e to b ir th in ju ry . Expe rim en tally , M idd leto n (19 30) p roduced h is to log ica lly sim ila r tum oursby lig atio n of v ein s d ra in ing th e sar to r iu s m uscle in do gs, an d in ferred tha t v en ous occ lusiondu ring p artu r ition w as th e cause in b ab ies . H ow ev er , th e strik ing h isto lo g ical fea tu re o fste rnom asto id tum our, desc ribed as ea rly as 18 75 by Tay lo r, is p ro fuse fib ro us rep lacem en t o fm usc le (F ig s. I and 2) w ithou t a trace o f h aem oside rin , even in spec im ens exc ised as early asth ree w eek s a fte r b ir th (C hand le r 19 48). R eye (19 51) exam in ed fo ur nec ro psy specim en s a tag es be tw een fou r and tw en ty w eeks, an d conc lud ed tha t the appea rances sug gested alm ostan overgrowth of tendon at the expense of muscle. Kiesewetter, Nelson, Palladino and Koop(19 55) , in a stud y o f th irty - tw o su rg ica l sp ec im en s, fou nd ve ry sm all am oun ts o f h aem o side rinin eig h t o n ly . Th ese le sions w ere n o t con sisten t w ith m assiv e haem a tom a ta , an d th e tum ourm uch m o re c lose ly re sem bled fib ro sa rcom a, fib rom a to sis o f p alm ar o r p lan ta r fascia , o rdesmoid tumours. Finally, birth injury cannot have been a factor in the n ow num erou spu b lish ed instances o f ste rn om asto id tum ou r fo llow ing C aesarian sec tion (S ch loessm ann 1911 ,Stern 1924, Rossi 1928 , C h and le r and A lte rnbe rg 19 44 , K iesew e tter et a !. 1 955 ).43 2 TH E JOURNA L O F BON E AN D JO IN T SURGERY

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    F I G . 1 F I G . 2F ig ur e 1 -H is to lo gi ca l sec tion from th e edg e o f a ste rnom asto id tum ou r in a 4 -w eek-o ld bo y . N o tethe sw ollen fragm ented m usc le fib res in the low er righ t hand corner, and the ex ten sive fib ro us tissuerep lacem en t in th e rem aind er o f the f ie ld . N o h aem osiderin can b e seen . (H aem atoxy lin and eo sin ,

    70 .) F igu re 2-H igh pow er v iew of the sam e affec ted m usc le fib res. (H aem atoxy lin and eos in , 3 50 .)

    ST ER NO MA S TO ID T UM OU R A N D M USCULA R TO RT ICO LL IS 43 3

    vo i . 51 B , NO . 3, AUGUST 1969

    It is c onceivable, as suggested by Coventry and Harris (1959), that intra-uterine torticollispred isposes to a h igh in cidence of b reech o r fo rceps de live ries. T he sub sequen t s te rn om asto idtum our cou ld th en be a fo rm of m u sc le dysp lasia, or in deed a re spo nse to traum a o f a lreadyab norm al m u sc le . V an Roonhy sen (1670) firs t pos tu la ted abno rm al u ter in e p ressu re as acau se o f to rtico llis. S ippe l (192 0) an d Chand ler and A ltenb erg (19 44) dem ons tra ted aco rre latio n be tw een th e b reech p osition and the side o f a subsequ en t tum our. S chm id t (189 0)and C hand le r (194 8) n o ted asymm etry o f th e p inna an d face to ge the r w ith to r tico llis

    imm ed iate ly af ter b irth an d be fo re the deve lopm en t o f a tum our. A sid e from pure lyenv iro nm en ta l fac to rs , gene tic in flu en ce as firs t pos tu la ted b y Joach im sth al (19 05) can no t bed ism issed . V on L ack um (1929 ) qu o ted a fam ily of th ree s ib lin gs w ith to rtico llis, and S tev en s(1 948 ) p ub lish ed an unusu al ca se o f a rig h t-s ided to rtico llis sym m etrica lly a ffec ting iden ticaltw ins .

    A curious accom pan im en t o f e stab lished to rtico llis is the w idesp read fac ial a sym m etry o rh em ia trop hy so succ inc tly desc rib ed by G old in g -B ird (18 90) thou gh he m istaken ly asc r ibedit to inc ip ien t po liom yelitis . M idd leto n (19 30) d ocum en ted ex tensiv e bo ny ch anges in th esku ll and fac ia l bones , and it is d if ficu lt to accoun t fo r a ll the observed chan ges pu re ly on theb as is o f re lative sh orten ing of the ste rn om asto id m u scle during grow th . N o really sa tisfacto ryexp lan ation of th is ph en om enon ex ists .

    The developmental and anatomical peculiarity of the muscle has received insufficientem phasis . S trau s and H ow ell (1936) em phas ised tha t the ste rnom asto id m u sc le is un ique inits doub le inn erva tion , and deve lopm en ta lly it m ay b e prim arily v isce ra l in o rig in w ith la te rsom a tic com ponen ts ad ded . L ast (196 3) po in ted o u t tha t th e m usc le rea lly co nsists o f fou rpa rts , and Jahss (19 36) d iffe ren tiated be tw een ste rn al to rtico llis and clav icu lar to rtico llis ,rela ting the d istin ctio n to the re su lts o f trea tm en t. F o rm erly the b lo od sup p ly o f the m usc lesw as b elieved , by N ov#{233} -Josse ran d and V ian nay (1906), to be segmental via end arteries,o cc lu sion of w hich resu lted in segm en ta l in fa rc tion . In app ly ing to hum ans h is ex pe rim en talev idence from dogs , M idd leto n (193 0) a ssum ed th at the v en ous d ra inage of the m usclefo llow ed an id en tica l p atte rn . H ow eve r, sub sequen t in jectio n stu d ie s , no tab ly b y C hand lerand A ltenb erg (1944) , h av e show n tha t th ere is an ab und an t a rte r ial and v en ous su pp ly w hichfo llow s no regu lar o r segm en ta l p atte rn , and th is ca sts dou b t on bo th th ese h ypo theses.

    TERM INO LOGY AND CLA SS IF ICA T IONT he term congen ita l to r tico llis , o r w ry n eck , is w ide ly used b u t seem s inapprop ria te

    since th e de fo rm ity is se ldom , if ev er , p resen t a t b irth . C apu t o bstip um (H o llow ay 1931), an

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    43 4 D . M ACD ON ALDo ld title u sed in m uch of th e G erm an lite ratu re , em phasise s th e obs tetr ic backgrou nd bu to therw ise has little m ode rn s ign if ican ce . T he co nd itio n has been ca lled in fan t i le by A dam s(1 967 ) tho ugh cases m ay presen t a t an y age th roug h ch ild hood or indeed in ado le scence.A nde rso n (18 93) refe rred sim p ly to sternomasto id to rtico llis , and on the w ho le th e term muscu l a r to r tico llis (C hand ler 1 948 ) seem s prefe rab le as it un derlin es th e basic pa th o log ica lp rocess .

    A p ar t from diffe rences in te rm ino log y , d oub ts have been ra ised as to th e un ifo rm ity ofa ll ca se s. A nde rso n (18 93) d isting u ish ed be tw een to rtico llis o f a tru ly cong en ital na tu re andth at a ris in g so le ly as a re su lt o fb ir th traum a . H u lb e rt (1 950 ) desc r ibed a tran sien t postu ralty pe , and a muscu l a r ty pe presum ed due to a p rev io us s ternom asto id tum our. F ina lly ,bo th H u lbe rt (195 0) an d C oven try and H arr is (195 9) show ed th at m os t ste rnom asto id tum ou rsreso lve com ple te ly and d id n o t p rog re ss to m u scu la r to rtico llis . T h is has led som e to q uestionwhether the tw o a re n o t in fac t sepa rate p ath o log ica l en tities .

    CLIN ICAL M ATER IALBetween 1950 and 1965 , 1 52 ch ild ren w ith to r tico llis w ere seen a t th e B risto l H osp ita l

    fo r Sick C h ild ren , the B risto l R oy al In f irm ary and the W info rd O rtho paed ic H osp ita l. T he rew ere th ree gro ups of pa tien ts. Tw en ty -s ix bab ie s p re sen ted a t o r sho rtly a fte r b ir th w ithtransien t po stu ra l to r tico llis w h ich se ttled w ith a m in im um o fcon se rva tive trea tm en t. F ifty -on ebab ies p resen ted w ith in the f irs t w eeks of life w ith a ste rnom as to id tum our. S ev en ty -fivech ild ren presen ted a t va riou s ages w ith m u scu la r to rtico llis .

    O f the fif ty -one bab ie s w ith a s te rnom asto id tum our, o ne w as asso cia ted w ith m u ltip lecong en ital de fo rm itie s an d w as lef t ou t o f th e ser ies . O f th e rem a inde r , th irty -on e w erefo llow ed up and ex am in ed persona lly , and a fu rthe r n ine teen case no tes w ere ava ilab le , thusm ak ing fifty in a ll ava ilab le fo r s tudy .

    O f the seven ty -f ive ch ild ren pre sen ting w ith m uscu la r to r tico llis , tw en ty -n ine w ere ex clu dedbecause of inadequa te d ocum enta tion . O ne w as assoc ia ted w ith E ng elm anns d isease an d w asthe re fo re lef t o u t. O f th e rem a in ing fo rty -f ive , th ir ty -fou r w ere fo llow ed up and exam in edpe rsona lly and the case reco rds w ere ava ilab le in a fu rth er e leven . To th is num ber w ere addedseven p atien ts from the s ternom asto id tum our g rou p w ho su bsequ en tly deve lop ed m uscu la rto r tico llis . T h is m ade fif ty - tw o cases ava ilab le fo r stud y .

    T he tw en ty -six bab ie s w ith pos tu ral to rtico llis w ere n o t con side red re lev an t to th is stu dyand w ere no t inc lud ed . In th ese c ase s the e tio lo gy w as no t c lear , bu t in m any cases pos ition in g in u te ro w as su spec ted . The ea rly o nse t at o r so on a fte r b irth p reclu ded an oph tha lm iccause which, according to Duke-Elder (1949), is not seen u n til e igh teen m onths o f ag e w henit i s commonly associated with a non-concomitant squint.

    PAT IENT S PRESENTING W ITH A STERNOM ASTO ID TUM OURIn fif ty b ab ies a s te rnomas to id tumour (Fig. 3) was palpable between one and fifteen

    weeks o f ag e, the m ean be ing th ree w eeks. Th irty w ere in boys and tw en ty in g ir ls . T h e b ir thh isto ry is summ arised in T ab le I. O ve r ha lf the ch ild ren had b reech or fo rceps b irth , and ofthe remainder over half again w ere firs tb o rn . O ne case occurred in a ch ild w ith an id en tica ltwin (unaffected) and in two others a second degree re la tive w as kn ow n to have had to rtico llis .

    The tumour occurred on the r igh t sid e in th ree -qu a rte rs o f a ll ca se s, and w hen the b ir thhad been by the breech, the preponderance was even higher (Tab le II). Ma ny of the babiesh ad asso cia ted d efo rm itie s , m a in ly m in or in na tu re (T ab le III). N ea rly on e-qu arte r show edtransient to r t ico l l is which subs ided w ith th e tum ou r. P lag iocepha ly w as reco rded inapproximately one-fifth of all cases.

    THE JOURNA L OF BON E AND JO IN T SU RG ERY

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    FIG . 3

    F IG . 4

    STERN OMA STO ID TUM O UR A N D M USCU LAR TO RT ICO LL IS 435

    VOL . 51 B, NO . 3, AUGUST 1969

    M ost o f the bab ies rece ived no trea tm en t excep t fo r sim p le in stru c tions to th e m othe r.A few w ere g iven stre tc h in g exe rcis es, supe rv ised by a p hys io th erap y dep artm en t. N o o therm easures w e re taken .

    Tw o-th irds o f the pa tien ts w e re fo l lowed up fo r b etw een tw o and six te en years w ith anav erage o f s ix years , bu t the len g th o f fo llow -up in on e-th ird was one year or less . The c lin ica l

    Figure 3-Pho to graph of a boy agedI m onth w ith a r ig h t s te rnom asto idtum our. H e had had a breech b ir th .F ig u re 4 -Pho to g rap hs o f the sam ech ild as sh ow n in F igu re 3 , when 8years o f age . S om e tigh tness andth icken in g o f the c lav icu la r head andvery sligh t fac ia l asymm etry can be

    seen .

    sta te a t fo llow -up is summ arised in T ab le IV . Seven ou t o f f ifty p atie n ts sub seq uen tly developedm uscu la r to r tico llis. T he rem ainder had no com pla in t bu t o n care fu l exam ina tio n m inord egrees o f asymm etry or tigh tn ess cou ld be d em ons tra ted in approx im ate ly o ne-quarte r(F ig . 4 ) .

    PAT IENT S PRESENTING W ITH M USCULAR TORTICO LL ISF if ty -tw o ch ild ren presen ted w ith m u scu la r to rtico llis (F ig . 5) . Tw enty -n in e w ere boys

    and tw en ty -th ree g ir ls . T he ir ages a re show n in F ig ure 6 . T he ch ild ren presen ted at a ll ages w itha fa irly ev en spread th rou gh the fir st d ecade , a fte r w h ich spo rad ic cases w ere seen in ado lescen ce.H ow ever, re trospec tive assessm en t o f the age of on se t o f the con trac tu re revea led tha tapprox im ate ly one-th ird comm enced in the firs t year o f life .

    In ten cases th ere w as a h is to ry of p ro ven o r p robab le ste rnom asto id tum our a t b ir th .M oreov er , the b ir th h isto rie s o f the w ho le g rou p (T ab le V ) show ed a rem ark ab ly sim ilarpa tte rn to those o f bab ies w ith ste rnom as to id tum our . O ver ha lf had been born b y b reech o rfo rceps de live ry and , o f the rem aind er, appro x im ate ly one-ha lf w ere firs tb o rn . O ne ch ildhad an iden tica l tw in w ho w as n o t a ffec ted . Tw o w ere sing ly affec ted m em bers o f b ino vu la rtw in s. F our h ad sim ila rly a ffec ted first degree re la tives and four had s im ila rly a ffec ted seco ndd egree re la tiv es .

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    Number of babies

    R ight L eft T ota l

    W hole ser ies . 37 13 50

    B reech b irths . 15 2 17

    Number o fAssociated defo rmit ie s casesT ra ns ie nt to rtic ollis . . 1 2P lag iocepha ly . . . . ICeph a lo h aema toma . . 2P ylo ric ste no sis . . . IC ong en ita l d is loca tion o f h ip . I

    O utcom e fo llow in g ste rnom asto id Numbe rtumourNo rma l . . . . . . 22M in im al tig h tness o r band . . 5M inim al asym metry o r p lag ioceph aly . 7Tor t ico l l i s . . . . . 7Too recen t o r unk now n . . . 9

    Numbe r o f b ab ies

    R igh t L eft Tota l

    W ho le se ries . 31 21 52B reech b irths . 14 8 22

    Facia l asymm etry N um berPresen t . . . . 36A bsen t o r very s ligh t . . 7Unrecorded . . . 9

    S ite o f con tractu re N um berM ain ly ste rna l head . . 8M ain ly c lav icu la r head . 19B oth h ead s . . . 25

    TH E JOURNA L OF BON E AND JO IN T SURGERY

    436 D . M ACD ON ALD

    TABLE IF IF TY BA BI ES W IT H ST ER NO MA ST OI D T UM OU R:

    B IR TH H Is ro Rl Es

    B irth h is tor y Numbe rBreech . . . . 17F o r c e p s . . . . 12P rim igrav ida norm al . . 12M ultig rav ida norm al . . 5Unknown . . . . 4

    TABLE IIIF IF TY BA B IE S W IT H ST ER NO MA ST OI D TU MO UR :

    A SSO CIA TED D EFO RM IT IE S

    TABLE VF IF T Y- TW O C HI LD RE N WI TH MU SC UL AR

    T OR TI CO LL IS : B IR TH HI Sr OR IE S

    B irth h isto ry Numbe rBreech . . . . 22Fo rcep s . . . . 9P rim igrav ida norm al . . 14M ultig rav ida n orm al . . 4U n k n o w n . . . . 3

    TABLE IIFIFTY BAB IE S W ITH STERNOM ASTO ID TUM OUR :

    SID E OF LES IO N

    TABLE IVFI FT Y BA BI ES WI TH ST ER NO MA ST OI D TU MO UR :

    AV ER AG E FO LL OW -U P W AS SI X Y E A R S

    TABLE VIF IFTY -TW O CH ILD REN W ITH M U SCULAR

    T O R T I C O L L I S : S IDE O F L ESIO N

    TABLE VIIF IF T Y- TW O CH IL D RE N WI TH ID IO PA TH IC

    TO RT IC OL LI S : F AC IA L AS YM M ET RY

    TABLE VIIIF IF TY -T WO C HI LD RE N WI TH

    T0 RT IC O LL IS : S IT E OF C O NT RA CT UR E

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    STERNOM A STO II) TUM OU R AN D MUSCULAR TO RT ICO LL IS 437

    VOL . 51 B , NO . 3, AUGUST 1969

    The con trac tu re w as m ore frequen t on the righ t side , and in ch ild ren bo rn b y the b reechth is p repo nderance w as app rox im ately tw o to on e (T ab le V I) . F acia l asymm etry (F ig . 5) wa sassociated w ith m o st o f the con trac tu res bu t w as by no m eans in variab le (T ab le V II). T h es ite o f the con trac tu re w ith in the m u sc le w as no ted in m ostcases: the clav icu la r head w as usua lly in vo lved , o ften prin -c ip ally (T ab le V III). In on e case th is w as assoc iated w ith abony an om aly w ith in th e c lav icu lar a ttachm en t.

    DISCUSSIONT here is a stro ng sim ila rity be tw een the assoc ia tion of

    ear ly stern om asto id tum our in ch ild ren b orn by the breech ,afte r fo rc eps de liv ery , and in the firs tb o rn , and the assoc ia -tion of ch ild ren presen tin g la te r w ith to r tico llis a fte r s im ila r . -obste tric ev en ts. O n the o th er hand , on ly o ne baby in sevenw ith a s ternom asto id tum our sub sequen tly deve loped m us-cu lar to rtico llis. In the rem a inde r the tum ou r re so lved , #{149}tho ugh care fu l exam in a tion in som e ch ild ren la te r in life revea led in te rm ed ia te res id ua , su ch as a band in the m usc le T ortico llis in FIG. 5 aged 6 . T h eo r s ligh t restric tion of m ov em en t. T he re lationsh ip is fu rthe r ch ild w as f ir st seen a t 2 monthsunderlined by th e strik ing righ t-sided p repond eran ce of bo th lesio ns fo l low in g b reech b ir th s , an o bse rva tion prev io usly months .recorded by Coven try an d H arris (195 9) . It sugg es ts th atthe cause of bo th lesion s m ay be re la ted to an tena ta l po sition ra th er than to b irth traum a.T h is is su pported by th e find in g o f p lag iocepha ly in one o u t o f five bab ies w ith ste rnom as to idtum our, an inc id en ce tw ice tha t fo un d in th e norm a l popu la tion unde r one y ea r o f ag e byW ynne-D av ies (19 68). F am ilia l fac to rs , a lthou gh no t p repo nde ran t, m ay p lay a pa rt. In n in e

    I J AGE AT COM MENCEM ENT25NUMBER2#{176} lAGE AT PRESENTAT ION

    OF 15CA SES 10

    iIfl lhil. I. i J . iAGE IN YEARS

    FIG . 6D iagram to sh ow th e age of onse t o f m uscu la r to rtico llis in f i f ty - two

    ch ild ren , and the age w hen th ey presen ted fo r m ed ical trea tm en t.

    o f a com b in ed 100 cases a firs t o r seco nd d egree rela tiv e w as a ffe cted . O ne pa tie n t withste rnom asto id tum our su ffered also from congen ita l p y lo ric stenos is. T hree such cases w ererecorded by Chand le r and A lten berg (1944) .

    The rela tion sh ip b etw een th e tw o cond itions is th ere fo re com p lex . W ha teve r the chang ein th e m uscle it seem s like ly tha t it is p resen t a t o r b efo re b irth and has th ree incons tan t andvariab le seque lae . It m ay reso lve com ple te ly : it m ay c lin ica lly becom e m an ifes t as aste rnom asto id tum ou r: o r it m ay rem ain c lin ica lly la ten t, subsequen tly underg o ing a variab ledegree of c ica trisa tion to produce to rtico llis.

    In estab lish ed to rtico llis the con trac tu re is m ore comm on in the c lav icu la r h ead asdescrib ed by Jahss (1936 ). In one case th is w as assoc ia ted w ith a bony anom aly in the c lav icu la r

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    438 D . M A CD ON A LD

    THE JOURNAL O F BON E AND JO INT SU RGER Y

    head , like th at p rev iou sly reco rd ed b y M idd le ton (19 30). F ac ial asymm etry w as g en era lly ,thou gh no t in va riab ly , p re sen t b u t it w as no t pos sib le to co rrela te its seve rity w ith tha t o f th eto rtico llis a s sug gested by H ough (1934). T he re latio nsh ip is no t n ecessa r ily a d irect on e.

    TREATMENT O F MU SCULAR TORT ICO LLISA ccord in g to Hu lbe rt (19 50) su bcu taneou s ten o tom y w as prac tised in R om an tim es , bu t

    the f ir st reco rd ed in stance w as tha t by Isaac M innus in 1685 . T he m eth od has been advo ca tedsub sequen tly by H ow e ll ( I 929 ), E lm slie ( I 94 3) an d H ulb ert

    (19 50). W illiam C hese lden (1749) w as the f irs t to desc rib e anopen op eration , a techn ique subsequen tly u sed b y M eyerd ing(1 92 1) and H ough (19 34). Jahss (193 6) d escr ibed a v a ria tioninvo lv ing m edia l tran sp lan ta tion of the clav icu la r be lly .H ellstad ius (192 7) and Soeur (194 0) repo rted the re su lts o f ado ub le app ro ach usin g a low er sub cu taneous teno tom y and an

    i upper open d iv is ion . C hand le r and A ltenbe rg (1944 ) adv ised b lock exc ision a t the earlie st s tage of frank ste rnom as to id tum ours o r c ica trised m usc le . The use o f a p la ste r co lla r to m a in ta in correc tion afte r opera tion w as adv oca ted by M eye rd ing (19 21), H ow e ll (1 92 9), H ough (1 934 ) an d Soeur

    (1940) .O f th e fifty - tw o ch ild ren w ith m uscu la r to r tico llis , th irty -

    six ex am ined a t fo llow -u p had been treated by open d iv isio n# { 1 4 9 }- # { 1 4 9 } of the low er a ttachm ent o f th e m u sc le (F ig . 7 ). Tw o- th irds o f

    F IG 7 th ese opera tions w ere perfo rm ed by one surgeo n and theP ho to graph of the sam e ch ild as rem ain ing th ird by a n um ber o f su rgeon s us ing the sam e tech -show n in F igure 5 af te r open n ique . T h e age a t opera tion and the len g th o f fo llow -u p ared iv is io n of b o th heads of theste rnom asto id m usc le . A t 14 sh ow n in F igure 8 .years o f ag e th ere w as a good O pe rative tec hn iq ue -In a ll cases a low transverse inc ision w asscar bu t sligh t residu a l fac ia l . . .asymm etry w as presen t. used d iv id ing sk in and p la tysm a . In m ost instances b o th heads

    of the m u sc le w ere d iv ided , and in m any th e un derly ing deepfasc ia (T ab le IX ). C lo su re w as p erfo rm ed by su tu ring the p la tysm a and the sk in in tw o layers ,though o ccas io na lly sk in on ly w as c lo sed . A fte r opera tion a dressing w as app lied and imm edia tephys io the rapy begun . In o n ly five cases w ere s tretch ing exerc ises con tinu ed fo r longer thanfour m on ths, and in m any the dura tion w as m uch less.

    2

    AG E

    IN i 1 YEARS J iiJ111 iH _____ _____ _____ _

    IND IV IDUAL CASESFIG . 8

    D iagram of the age o f op eration in th irty -s ix cases o f m u scu la rto rtico llis, and th e tim e of fo llow -u p in years in each case .

    R E S U L T SA ll p a tien ts w ere p leased w ith the co rrec tion an d non e presen ted fo r fu rthe r trea tm en t.

    H ow ever, a t fo llow -up string en t c rite ria w ere used to revea l m ino r cosm etic b lem ishes(T ab le X ).

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    E xten t o f op en op eration N um ber

    S te rna l head on ly . . 2

    C lav icu la r head o n ly .B oth h eads . . . 19Bo th heads and deep fasc ia 1 4

    Tota l . . . 36

    STERN OMA STO ID TUM O UR A ND M USCU LA R TO RT ICO LL IS 43 9

    VOL . 51 B , NO . 3, AUGUST 1969

    M uscu lo -fasc ia l b and s-T hese w ere g en era lly on ly dem on strab le a t ex trem es of ro ta tion andla te ra l flex ion of the n eck (F ig . 9 ) . T h ey stre tch ed from the occ ip ita l a ttachm en t o f th e m usc leto the clav ic le and w ere invariab ly a ttach ed w ell la te ra l to the n orm a l inse r tion of the c lav icu la rh ead . T he bands som etim es cons isted of tigh t fasc ia and sk in on ly , bu t m ore freq uen tly theyco nta ined a ctiv ely con tra ctile m uscle . T hey appea red to re su lt f rom anom alous rea ttachm en t

    TABLE IX TABLE XTH IRTY -S ix CH ILD REN W ITH T0RT IC 0Lu s: TH IR TY -S IX CH ILD REN W ITH T 0R TIC0LL IS :

    EXTEN T OF OPEN SECTION FO LLOW -U P AFTER O PERA TION

    Residu a l b lem ishes N um berR e si du a l t or ti co ll is -s li gh t_ 4

    ________________________________ R esidua l fac ia l asymm etry 1 2M uscu lo -fasc ia l bands . 23R estric tion o f m ovem ent . I 2A b sence of s te rna l co lum n 14D isfig u ring scar . . 2R e ve rs e t or ti co ll is . . I

    of the seve red c lav icu la r head as su ggested b y Jah ss (19 36 ). A lthou gh pre sen t in tw o-th ird sof a ll cases, th ey w ere se ld om an im portan t cosm etic defec t, tho ugh in genera l th ey causedsom e term in al re stric tio n of m ovem en t.R estr ic tio n of m ovem en t-T h is occu rred only at th e ex trem es of ro ta tion or la tera l f lex ion .It se ldom affected m ore th an the la st 15 deg rees o f ran ge . U sua lly , th ough no t inv ariab ly , itw as du e to a m uscu lo -fasc ia l band .A bsen ce o f th e sternom asto id co lum n -Ab sen ce o f the norm al v er tica l co n tour o f theste rn om asto id m usc le in th e neck re su lted in a qu ite no ticeab le cosm e tic de fec t (F ig . 10 ) injust o ve r one -th ird o f cases.R ev erse to r tico llis-In on e ca se (F igs. 1 1 an d 12) th e s te rnom asto id co lum n w as com ple telyabsen t and a rev ersa l o f th e to r tico llis h ad occurred , p re sum ab ly due to m uscu la r im ba lan ce .D isfig u rin g scar -T he neck scar cons titu ted a cosm etic defec t to som e ex ten t in all cases ,pa rtic u la rly in the g ir ls . T he scars f requen tly show ed broaden ing of the m edia l end (F ig . 10),bu t in tw o cases on ly d id th is con stitu te a d is figu rem en t su f ficien t to w arran t fu rthe r su rg ica lmeasu re s .R esidua l fac ia l a sym metry-Fa cia l a sym m etry o f vary in g d egre e w as re co rded b efo re op era tio nin tw en ty -f iv e of the th ir ty -six cases . A t fo llow -up res id ua l asymm etry w as de tec ted in tw elve ,tha t is in app rox im ate ly ha lf, and the de tails o f these cases a re show n in F igure 13 . R es id ua lfa cia l asymm etry w a s generally unnoticed by pa tien t an d p aren t a like (F ig . 7 ). It w as m ostreadily detected from the pos ition o f the eyebrow . It is no tew orthy tha t in all b u t one of th etw elve cases w ith res id ua l asymm etry there w ere o th er residua o f the to rtico llis (F ig . 13). Th reeof th e pa tien ts w ere treated in th e first y ear o f life ye t s till re ta in ed asymm etry w h en seenseven , n in e and ten y ears la ter . It seem s lik e ly tha t the persis tence of bands afte r su rgery inth e young ch ild m ay preven t com plete res to ra tio n of symm etry . F urthe r teno tom y o f theseb an ds is the re fo re p robab ly ind ica ted .

    DISCUSSIONIn th e tre atm ent of th is la rge ly cosm etic p rob lem m any m eth ods are op en to the su rgeon

    an d a lm os t a ll cla im equa lly sa tisfac to ry resu lts . T he m ajo r d efo rm ity is no t d iff icu lt to co rrec t

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    F IG . 9Tw o exam ples o f m uscu lo -fasc ia l b an ds afte r op en d iv ision of bo th heads of th e m usc le.

    F I G . 10A b sence of the ste rnom as to id co lum n on th e le ft s id e of the neck afte r opend iv is io n o f the m u sc le . N o te also b roaden ing of th e m edia l end of th e sca r.

    44 0 D . M ACD ON ALD

    THE JOUR NA L O F BON E A ND JO INT SU RGERY

    and atten tion m ust b e fo cused on the re lative ly m inor cosm etic defec ts th at p recede or fo llowsurgery . T he most com m on c rit ic ism o f the op en opera tion is the re su l tan t s car . In th is se rieson ly tw o pa tien ts w ere con side red to have a d isfigu ring scar. O n th e o th er hand , the m etho d

    w as sa fe and posed no th rea t to the neurov ascu la r struc tu res in the neck . It cou ld b e re liedon to correc t the princ ipa l de fo rm ity w ith ou t the u se of su bseq uen t sp lin tage , bu t anom alou sla te ra l reattachm ent o f the severed c lav icu la r head led to a h ig h inc idence of m u scu lo -fasc ialbands . These w ere p rev iou sly no ted by H ough (19 34), an d to preven t th is com p lica tion

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    A

    m ovem en t. T he re w as som e ev id en ce .25

    20

    AG E IN YEARS I

    IND IVIDUALCASES

    F I G . 13D iagram o f the cau ses o f tw e lve cases o fresidua l asymm etry , w ith the ag e of o pera -tion and tim e o f fo llow -up in years.

    VOL . 51 B , NO . 3. AUGUST 1969

    STERNOM ASTO ID TUM OU R AN D M USCU LAR TO RT ICO L IIS 44 1

    FIG . 11 F IG . 12F ig ure lI-F ive-year-o ld g irl show n b efo re opera tion w ith righ t-s ided to rtico llis . F igu re 12-T he sam e ch ildaged 8 years . R ev erse to rtico llis has deve loped fo llow in g open d iv is ion o f b o th heads and deep fasc ia .

    N o te com p le te res to ra tion o f fac ia l symm etry d esp ite th is.

    Jahss (193 6) d esc r ibed a variation of the techn ique in vo lv ing m ed ial tran sp lan ta tion of th ec lav icu la r be lly . In th is se r ies these b ands w ere se ldom a se rious cosm e tic b lem ish , th ough ingeneral they c aused som e term ina l re stric tio n o fhow eve r , th at the ir pe rsis tence w as re la ted to thepe rsis tence o f slig h t d eg rees o f fac ia l asymm etry .H ough (1 934 ) an d Soeur (19 40) sta ted th a t co rrectio no f asymm etry cou ld b e exp ec ted if su rg ery w aspe rfo rm ed und e r the ag e o f fou rteen yea rs . In th isse r ies , h ow eve r, re so lu tion d id n o t a lw ays o ccur ,ev en afte r treatm en t in the ea rly yea rs o f life . Inall bu t o ne of these re sidua l ca se s b an ds w erep resen t .

    T h e m o st n o ticeab le co sm e tic de fec t w as losso f the n orm a l co n to u r o f the neck , a lso p rev ious lyn o ted b y H ough (19 34). In on e case d iv isio n of them usc le had b een so com ple te tha t rev erse to r tico llisre su l ted , p resum ably from m uscular im balanc e. Itwas w o rth no tin g tha t in th is ca se the facia l a sym -m e try d isapp ea red com ple tely ov e r a fo llow -u ppe riod of th ree yea rs (F ig . 1 2 ).

    BANDS#{149}

    T iGHTNESS # {149 }11r RESIDUALt2T 1OO L L #{149}

    SUMM ARY AND CONCLU SIO N SI. A pa ralle l study has b een m ad e of fifty pat ien ts pre sen tin g w ith a ste rn om asto id tum ou rand fif ty - tw o p atien ts p re sen ting w ith m uscu la r to r tico llis .2 . In the b ir th h isto rie s o f bo th th ese g rou ps the re w as a h igh inc idence of breech , fo rceps an dpr imiparous b irth s . Th e d istrib u tion of each w as str ik ing ly sim ila r .

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    44 2 D . M AC DO NALD

    3 . S ternom asto id tum ours w ere r igh t-s id ed in th ree -q ua rte rs o f a ll case s and in an even h ighe rp ro portion of th e b reech b irth s . Th ere w as tw ice the ex pec ted in c idence of p lag io ceph a ly .O n ly one in seven proceeded to m uscu la r to rtico llis , bu t in som e of th e rem a inde r m inorresidua cou ld be de tec ted .4 . M uscu la r to r tico llis p resen ted a t an y ag e, b u t one -th ird comm en ced in the firs t yea r o f life .O n ly on e in five gav e a h isto ry o f p rev iou s ste rnom as to id tum our. The con trac tu re show ed apred ilec tion fo r the c lav icu la r head , an d w as gene ra lly a ssoc ia ted w ith som e d eg ree o f fac ia la symmet ry .5. N ine of a com b ined 102 cases had a firs t o r second d egree sim ilar ly affec ted rela tive .6 . I t is conc lud ed th at w ha teve r the cond ition in the m usc le at b irth , it has th ree incons tan tand va riab le seq ue lae. T he to rtico llis m ay reso lve com p lete ly ; it m ay becom e c lin ica llym an ifes t as a tum our; o r it m ay rem a in clin ica lly la ten t, su bsequen tly un de rg o ing a v ariab ledeg ree o f c ica trisa tion to p ro duce to rtico llis .7 . T he trea tm en t o f estab lish ed to rtico llis by o pen d iv ision is desc rib ed and the fo llow -u pin th ir ty -s ix cases reco rded .8 . Th is op era tio n can be re lied on to cu re the p rinc ipa l d efo rm ity , b u t is accom pan ied b y anum ber o f m ino r cosm etic d efects . O f th ese th e m os t str ik ing a re tigh t band s app aren tly d ueto an om alous rea ttachm ent o f th e clav icu la r head , and lo ss o f the ste rnom asto id co lum n ofthe neck .9 . Th e m e tho d co u ld no t be re lied on to cu re facia l a symm etry com ple te ly , even in the ea rlyyea rs of life . H ow eve r, the re w as som e ev idence to sug gest tha t p ersis tence o f a sym m etry w asallied to p ersis tence o f o th er re sid ua of th e to r tico llis , fo r ex am ple fascia l bands .

    M y th anks are du e to th e su rgeons of the B ris to l a rea , and in p ar ticu la r to M r A . L . E yre -B ro ok under w hosecare m o st o f the cases w ere , an d to M r A . H . C . R a tlif f fo r m uch cr itic ism and encouragem ent.

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    L td .ANDERSON , W . (1893): C lin ica l L ec tu re o n S te rn o-m asto id T o rtico llis. Lancet. I, 9.B IA NCO , A . J ., Jun . (195 8): C ongen ita l M uscu la r T ortico llis . T hesis , G radua te S ch oo l, U nivers ity o f M inneso ta .CHANDLER , F . A . (1 948 ): M u scu lar T ort ico llis . Journal o fBone and Jo int Surge ry, 30 -A , 56 6 .CHANDLER , F . A ., an d ALTENBERG , A . (19 44): Congen i ta l M u sc ula r T ortic ollis . Journal o fthe Amer ican

    M ed ica l A ssoc ia tion , 125 , 476 .CHESELDEN , W . (1749): In The O peration s in Surg ery by F . W . L e D ran , w ith rem arks , p la tes o f the op erations

    an d a sett o f in strum en ts b y W illiam C hese lden , p . 454 . T rans la ted by Thom as G ataker. L ondon : C . H itchan d R . D od sle y.

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    D U K E - E L D E R , W . 5. (19 49): T ex tbook of Ophthalm ology . V olum e 4 , p . 4050 . London : H en ry K imp to n .ELMSLIE , R . C . (1943) : O pera tions on T endons . In M oder,, O pera tiv e Surg ery. Th ird ed itio n , vo lum e 1 , p . 154 .

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    o f M ed ic in e, 209 , 66 .G O LD IN G -B IRD , C . H . (1890 ): Congen ita l W ry-neck . W ith R em arks o n F ac ia l H em ia tro phy . G uy s H osp ita l

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    o f the Am erican M ed ica l Assoc ia tion , 157 , 12 81 .LAST , R . J . (1963) : A na to my , R eg io na la nd Ap pli ed . Th ird ed itio n , p . 520 . L ondon : J . & A . Chu rch ill.L IDG E , R . T. , BECHTOL , R . C ., and LAMBERT , C . N . (1957): C ongen ita l M uscu lar T ortico llis . Jou rnal o f B on e

    and Jo int Surge ry, 39 -A , 1 165 .MEYERD ING , H . W . (1921): C ongen ital T o rtico llis. Jou rnalo fO r thopa edic Surg ery , 3 , 91 .MIDDLETON , D . S . (1 930 ): Th e Pa tho log y o f C ongen ita l T or tico llis. Br itish Jou rna l o fSurgery, 18 , 188 .MINNUS , I. (168 5): C ited by B ick , E . M . (1 948): Sou rce Book ofO rth opaed ics . Seco nd ed ition , p . 55 . Bal t imore :

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    a lletiog en es i de l T orcico llo .) Riv is ta d os tetr icia e gin eco log ia p ratica , 10 , 277 .SANERKIN , N . G ., an d EDWARDS , P . (1 966 ): B ir th In jury to th e S ternom asto id M usc le . Jou rna l o f Bone and

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    v o t. 51 B, NO . 3, AUGUST 1969