perthes disease - by abdul karim

7/24/2019 Perthes Disease - By Abdul Karim

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Perthes disease

ByDr. Abdul Karim

Postgraduate Resident Orthopedic Surgery

PGMI!G". !A"OR#$ PAKIS%A&.


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'IRS% D#S(RIB#DB) LEGG* A&D

WALDENSTORMI& +,-,* A&D B)PERTHESA&DCALVEI& +,+-


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D#'I&I%IO&

!egg(al/0Perthes

disease 1!(PD2 is thename gi/en toidiopathicosteonecrosis o3 the

capital 3emoralepiphysis in a child.


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Increased Joint

space

Smaller head

Denser headNormal

joint


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#pidemiology

Prevalence4


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#pidemiology

Race: Caucasiansare a33ectedmore 3re5uently than persons o3other races.

Sex: Malesare a33ected !" #imesmoreo3ten than 3emales.

A$e: !(PD most commonly is seen inpersons aged %!&' (ears* 6ith amedian age o3 7 years.


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B!OOD S8PP!)


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(auses

#9act causeun:no6n.

Pro)ose*

#+eories. Inherited protein (

andor S de3iciency. ;enous thrombosis Arterial occlusion Raised intra

osseous pressure


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(auses

Pro)ose* #+eories. #9cessi/e 3emoral anti/ersion. Syno/itis. Generali


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Causes


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Pa#+o)+(siolo$( %he capital 3emoral epiphysisal6ays is in/ol/ed. In +=

>-?o3 patients 6ith !(PD* in/ol/ement is bilateral.

%he blood supplyto the capital 3emoral epiphysis is

interrupted.

Bone in3arctionoccurs* especially in the subchondralcortical bone* 6hile articular cartilage continues togro6. 1Articular cartilage gro6s because its nutrients

come 3rom the syno/ial 3luid.2

Re/asculari


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Pa#+o)+(siolo$(

At this point* a percentage o3 patients de/elop!(PD* 6hile other patients ha/e normal bonegro6th and de/elopment.

!(PD is present 6hen a subchondral 3ractureoccurs. %his is usually the result o3 normalphysical acti/ity* not direct trauma to the area

(hanges to the epiphyseal gro6th plate occursecondary to the subchondral 3racture.


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Pa#+o$enesis

A/ascular necrosis %emporary cessation o3 gro6th

Re/asculari


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Clinical

His#or(: Symptoms usually ha/e beenpresent 3or 6ee:s.

"ip or groin pain* 6hich may be

re3erred to the thigh Mild or intermittent pain in anterior

thigh or :nee

!imp 8sually no history o3 trauma


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Clinical

P+(sical: Pain3ul gait

Decreased range o3 motion1ROM2*particularly 6ith internal rotation andabduction

Atrophy o3 thigh musclessecondary to

disuse Muscle spasm !eg length ine5ualitydue to collapse


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Clinical

Short stature4 (hildren 6ith !(PDo3ten ha/e delayed bone age.

Roll test @ith patient lying in the supine position*the e9aminer rolls the hip o3 the a33ectede9tremity into e9ternal and internal

rotation. %his test should in/o:e guarding or

spasm* especially 6ith internal rotation.


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Di,,eren#ials

-nila#eral Septic hip %o9ic syno/itis

Slipped 3emoralcapital epiphysis

Spondyloepiphysealdysplasia

Metaphysealdysplasia !ymphoma

.ila#eral "ypothyroidism Multiple epiphyseal

dysplasia Spondyloepiphyseal

dysplasia Sic:le cell disease


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Wor/u)

La0 S#u*ies:

(B(

#rythrocyte sedimentation rate Maybe ele/ated i3 in3ection present


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Wor/u)

1ma$in$ S#u*ies: Plain 9rays o3 the hipare e9tremely use3ul in

establishing the diagnosis. 'rog leg /ie6s o3 the a33ected hip are /ery help3ul.

Plain radiographs ha/e a sensiti/ityo3 ,7?and aspeci3icityo3 7?in the detection o3 !(PD

Multiple radiographic classi3ication systemse9ist*based on the e9tent o3 abnormality o3 the capital3emoral epiphysis.

@aldenstrom* (atterall* Salter and %hompson* and"erringare the most common classi3icationsystems.

&o agreement has been reached as to the bestclassi3ication system.


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Radiographic stages

'i/e radiographic stagescan be seenby plain 9ray. In se5uence* they areas 3ollo6s4


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Radiographic stages

1.Cessation of growth at

the capital femoral

epiphysis; smallerfemoral head epiphysis

and widening of

articular space on

affected side.


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Radiographic stages

>. Subchondral3racture$ linear

radiolucency 6ithinthe 3emoral headepiphysis


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Radiographic stages

C. Resorption o3 bone


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Radiographic stages

. Reossi3ication o3 ne6 bone


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Radiographic stages

=. "ealed stage


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(atterall classi3ication

Ca##erall Grou) 14 In/ol/ement only o3 the an#eriorepiphysis 1there3ore seen only on the 3rog lateral 3ilm2

Ca##erall Grou) 114 Cen#ral segment 3ragmentation

and collapse. "o6e/er the lateral rim is intact and thusprotects the central in/ol/ed area. Ca##erall Grou) 1114 %he la#eralhead is also in/ol/ed

or 3ragmented and only the medial portion is spared.%he loss o3 lateral support 6orsens the prognosis.

Ca##erall Grou) 1V4 %he en#ire +ea*is in/ol/ed. (atteralls classi3ication has a signi3icant inter and intra

obser/er error.


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(atterall classi3ication

Grou)s 1 an* 11had a goodprognosis1in ,-?2 and re5uired nointer/ention.

Grou)s 111 an* 1Vhad a poorprognosis1in ,- ?2 and re5uiredtreatment.

%he classi3ication is applied to the 3roglateral and AP 3ilm during the3ragmentation phase


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Salter and %hompson (lassi3ication

Salter and %hompson recognihead in/ol/ed.

Sal#er 2 T+om)son Grou) .4 More than+>head in/ol/ed.

Again the main di33erence bet6een theset6o groups is the integrity o3 the lateralpillar.


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1"erring2 !ateral Pillar(lassi3ication

La#eral Pillar Grou) A:%here is no lossin heighto3the lateral +C o3 the head and minimal densitychange. 'ragmentation occurs in the central segmento3 the head.

La#eral Pillar Grou) .:%here is lucency and loss o3height in the lateral pillar but not more that =-?o3the original 1contralateral2 pillar height. there may besome lateral e9trusion o3 the head.

La#eral Pillar Grou) C:%here is greater than =-?

lossin the height o3 the lateral pillar. %he lateral pillaris lo6er than the central segment early on.


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1n#rao0server an* in#ero0server relia0ili#( o,Ca##erall3 Herrin$3 Sal#er!T+om)son an* S#ul0er$classi,ica#ion s(s#ems in Per#+es4

Conclusions4 %he results o3 our studysuggest the use o3 (atterall and Salter%hompson systems prior to treatment and the

Stulberg system at the end o3 the treatmentat s:eletal maturity."o6e/er* e/aluation o3the patients during the treatment period isstill a dilemma and necessitates a ne6 morereliable classi3ication system. Eournal o3 Pediatric Orthopaedics B. +C1C24+FF+F,* May >--.

Agus, Haluk a; Kalenderer, Onder a; Eryanlmaz, Gurkan b[latin

dotless i]; Ozcalabi, Isa Turkay a


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Unilateral erthes with entire head in!ol!ement and fragmentation. "he

reossification phase has not yet #egun.


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Unilateral erthes disease with widening of the medial joint space$ #lurring of

the physis$ increased density of the head and lucency #etween the medial and

central 1%&'s of the head corresponding to early fragmentation phase.


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Unilateral erthes in the

reossification phase with

a !isi#le su#chondral line

similar to (aldenstrom's

sign. )owe!er

(aldenstrom's

su#chondral fracture is

seen !ery early in the

disease process$ #efore

fragmentation. In this

case the lateral pillar hasmaintained some

integrity.


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Hea* a# ris/ si$nsH

+. Ga$e5s si$n. a ; shaped lucency in thelateral epiphysis.

>. la#eral calci,ica#ion1lateral to the

epiphysis2 1implies loss o3 lateral support2C. la#eral su0luxa#ion o, #+e +ea*.

1implies loss o3 lateral support2

. A +ori6on#al $ro7#+ )la#e.1implies agro6th arrest phenomenon andde3ormity2


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Wor/u)

%echnetium ,, bonescan "elp3ul indelineating the e9tento3 a/ascular changesbe3ore they are e/ident

on plain radiographs. %he sensiti/ity o3

radionuclide scanningin the diagnosis o3 !PDis ,?* and thespeci3icity is ,=?.

Dynamic arthrographyAssesses sphericity o3the head o3 the 3emur.


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8ltrasonographyin transientsyno/itis and early Perthes disease

8ltrasonography may pro/ide signi3icant

diagnostic clues to di33erentiate earlyPerthes 3rom transient syno/itis.

% 'utami* ) Kasahara* S Su


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(% Scan Staging determined

by using plainradiographic3indings is

upgraded in C-? o3patients.

&ot as sensiti/e asnuclear medicine or

MRI. (% may be used 3or

3ollo6up imagingin patients 6ith!PD.


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MRI

It allo6s more preciselocali


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Ou#come varia0les

Age #9tent o3 in/ol/ement

Duration Remodeling potential Premature physeal closure

%ype o3 treatment Stage o3 disease at treatment.


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Trea#men#

Goals o, #rea#men# Achie/e and maintain ROM Relie/e 6eight bearing (ontainment o3 the 3emoral epiphysis

6ithin the con3ines o3 the acetabulum %raction


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Ra#ional 0e+in* 8con#ainmen#8

Salter has demonstrated the biologicplasticity"o3 the 3emoral head in pigs3ollo6ing a /ascular insult.

(ontainment o3 the head 6ithin theacetabulum is reported to encouragespherical remodelling during thereossi3ication and subse5uent phases.

"o6e/er i3 there is total head in/ol/ement

and the lateral pillar collapses then the e33ecto3 containment is probably less. %here3ore it seems that the e9tent o3

in/ol/ement o3 the head is the critical 3actorand containment simply optimi


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Me*ica#ions Medical treatment does not stop or re/erse

the bony changes. Appropriate analgesic medication should be

gi/en. onsteroidal anti!in"lammatory drugs

Ibupro3en Adult dose4 >---- mg PO 5Fh$ not to e9ceed C.> gd. Pediatric dose4 F months to +> years4 >-- mg:gd PO di/ided

tid or 5id$ start at lo6er end o3 dosing range andtitrate up6ard$ not to e9ceed >. gdJ+> years4 Administer as in adults.


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Non sur$ical con#ainmen#


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Scotish Rite abduction brace


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Japenes modification ofpetrie abduction cast


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Surgical containment


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Grea#er #roc+an#ericover$ro7#+

%he trochanteric o/ergro6th can be dramatic onradiographs but se/eral studies ha/e sho6n thata %rendelenberg gait does not al6ays occur.

I3 it does occur* and is signi3icant* thentrochanteric ad/ancementmay impro/e thegait.

An alternati/e is to per3orm a trochantericarrestat an earlier date but this assumes thatthe 3irst statement 6ill not apply to theparticular child.


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R#(O&S%R8(%I;# S8RG#R)

1ND1CAT1ONS4 Hin$e a0*uc#ion4 /algus subtrochanteric

osteotomy. Mal,orme* ,emoral +ea*

in late group III or residualgroup I;. Garceaus cheilectomy.

Coxa ma$na4 shel3 augmentation A lar$e mal,orme*

,emoral +ea* 7i#+ la#eralsu0laxa#ion4 (hiaris pel/icosteotomy.

Ca)i#al ,emoral )+(sealarres#4 trochanteric

ad/ancement or

arrest.


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A S%8D) A% (I&(I&&A%II&S%I%8%#

Hin$e a0*uc#ion an* 9oin# s#i,,ness in)er#+es *isease: E,,ec# o, me*ial so,# #issue release an* )e#rie

cas#in$ )rior #o ,emoral +ea* con#ainmen#4

"ypothesis4 (orrect hinge abduction Impro/e motion &ormali


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(O&(!8SIO&

Medial capsulotomy and dynamicpositiong e33ecti/ely corrects hingedabduction.

ROM 6as impro/ed in all parameters. "igh degree o3 patient and parent

satis3action.


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S-MMAR

'or patients less that F yearsold theprognosisis good3or the maLority.

I3 they are sti33 or pain3ul they respondto bed rest* traction and pain relie/ingantiin3lammatory medication.

%here is no e/idence that abduction

splints or surgical inter/ention is6arranted in the maLority o3 theseyounger patients.


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S-MMAR

'or patients bet6een F and yearsbut 6ith abone age less than F and an intact lateral pillar1"erring A and B2 the prognosisis similar tothat 3or the 3irst group and obser/ation is as

goodas surgical inter/ention 3or the maLority. I3 they ha/e bone ages greater than F years

and "erring lateral pillar classi3ication B thencontainment o3 the head 6ithin theacetabulum seems to be 6arranted.

%his may be done by abduction bracing*3emoral /arus osteotomy or a pel/ic osteotomy.


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S-MMAR

I3 they are bet6een F and and are inlateral pillar group (then the result o3inter/ention are e5ui/ocal.

(hildren presenting 6ith Perthes disease atage , or oldero3ten ha/e lateral pillar B or(and a poor prognosis.

%he trend is to6ards early containmento3

these hips although sti33ness can be aproblem 3ollo6ing early pel/ic 1Salters2osteotomy.


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;ollo7!u)

Initially* close 3ollo6upis re5uired todetermine the e9tent o3 necrosis.

Once the healing phasehas beenentered* 3ollo6up can be e/ery Fmonths.

!ongterm 3ollo6upis necessary to

determine the 3inal outcome.


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Com)lica#ions

;emoral Shortening

sti33ness Malrotation !imp Positi/e

trendelenburg

Pelvic !enghtening Sti33ness (hondrolysis 'ailure o3

containment


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Pro$nosis

%he youngerthe age o3 onset o3 !(PD*the betterthe prognosis.

(hildren older than +- yearsha/e a

/ery high ris: o3 de/elopingosteoarthritis.

Most patients ha/e a 3a/orable outcome.

Prognosis is proportional to the degreeo3 radiologic in/ol/ement.


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perthes disease - by abdul karim

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