169824294 perthes disease

7/23/2019 169824294 Perthes Disease

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BAB I

PENDAHULUAN

Penyakit Legg-Calv-Perthes adalah kelainan idiopatik pada pinggul yang

mengakiatkan nekrosis serta iskemik pada kaput !emur yang sedang dalam proses

pertumuhan "#im$ %&'0()

BAB II *IN+AUAN

PU,*A#A

%)' De!inisi

Penyakit Perthes adalah gangguan pada kaput !emur yang terdiri dari

proses nekrosis, kolaps$ peraikan dan remodeling yang mun-ul pada dekade

pertama kehidupan$ khususnya pada anak laki-laki. Penyea dari penyakit ini

dihipotesiskan seagai satu atau eerapa ke.adian vaskular yang diikuti olehrevaskularisasi.,elan.utnya hal ini dapat mengakiatkan kesulitan dan nyeri pada

agian pinggul) Penyakit Perthes pertama kali dipulikasikan se-ara independen

oleh +a-/ues Calv$ Arthur *) Legg and 0eorg Perthes pada tahun '1'&)

Perthes merupakan suatu penyakit degenerati! pada sendi panggul$ dimana

kaput !emur mengalami kematian$ mengakiatkan in!lamasi dan iritasi dan

-enderung ditemukan pada anak laki-laki) Prognosis pada penyakit ini aik .ika

ditangani se-ara tepat "htt p23 3444)orthopedi-moti on)-om3 gloss ar y)htm(.

Penyakit Perthes adalah penyakit sendi pinggul pada anak5anak) ,endi

pinggul terdiri dari dua agian utama, yaitu kaput !emur dan a-etaulum) Pada

penyakit Perthes$ kaput !emur kehilangan suplai darah dan agian terseut akan

mati) *ulang men.adi lemah dan akan mengalami !raktur yang mengakiatkan

kaput !emur terseut tidak lagi sesuai dengan soket yang ada. ,etelah eerapa
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ulan aliran darah akan kemali dan akan ter.adi remodeling "Hamilton Health

,-ien-es$ %&'%()

Penyakit Perthes .uga diseut seagai ,indrom Legg-Calve-Perthes yangmerupakan suatu kondisi sistem skeletal dimana kaput !emur mengalami proses

degenerasi setelah ter.adinya episode in!ark karena gangguan pada suplai aliran

darah) 6ani!estasi penyakit ini ersi!at unilateral pada leih dari 7& persen kasus

yang ada.

"http23 3 4 4 4)liv)a - )uk3Human A n a to m y 3phd 3r e s e ar - h3pe r thes3p e rthpd)html(

%)% Epidemiologi

Penelitian penyakit perthes di Inggris mengindikasikan ah4a terdapat insidensi

yang leih esar di agian North 8est) Penyakit ini memiliki insidensi yang tinggi

pada keluarga menengah kea4ah di Liverpool) 6eskipun hal ini masih elum

sepenuhnya dipahami sea akiatnya,ada kaitan erat antara pengangguran dan

minimnya pendapatan) *erdapat pula daerah-daerah tertentu yang memiliki angka

ke.adian leih tinggi$ seperti daerah perkotaan "%9 ke.adian per '&0$&&&

diandingkan dengan rata-rata nasional yaitu : ke.adian per '&0,000(.Penelitian

yang sedang erlangsung mengindikasikan ah4a insidensi yang tinggi

menun.ukkan ah4a kedua genetika dan lingkungan merupakan !aktor penyea

yang penting untuk kondisi terseut)

(ht t p2 3 344 4 )l i v)a- )uk3Human A n a to m y 3phd 3r e s e ar - h3pe r thes3p e rthpd)htm l(

Insidensi per '&&)&&& orang ervariasi pada eerapa agian di Inggris

"Northern Ireland2 '');< Liverpool2 '9);< 8esse=2 9)9< *rent2 :);( > '? > % ? > @? > ?

and 4orld4ide "Nor4ay2 1)%< ,outh India2 ) to ')7< ,outh #orea2 @)7(. > 9?

> ; ? > :? prevalensi di Amerika ,erikat adalah ' dari '%&& anak-anak) Penyakit

Perthes leih sering ter.adi pada eerapa populasi seperti pada orang Eskimo$

+epang dan pada eerapa ras di Eropa *engah. Penyakit ini leih .arang ter.adi

pada orang erkulit hitam$ penduduk asli Australia$ ras Indian Amerika dan

Polinesia.

Angka ke.adian per '&0.000 populasi di A!rika ,elatan ervariasiantara

'&$7 pada orang kulit putih$ '$: pada keturunan -ampuran dan &$9 pada orang

kulit hitam) #ondisi ini leih umum ter.adi pada daerah uranisasi dan terdapat

huungan yang konkrit antara kondisi ini dan rendahnya tara! sosial ekonomi

seseorang.
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Penyakit Perthes iasa ter.adi pada anak5anak usia sampai 7 tahun

dengan usia rata-rata : tahun$ meskipun tidak tertutup kemungkinan penyakit ini

menyerang anak usia antara % sampai '% tahun) Penyakit Perthes ersi!at ilateral

pada '& sampai %& persen kasus.

%)@ Etiologi

Etiologi dari penyakit Perthes tidak diketahui se-ara pasti "Hamilton Health

,-ien-es$ %&'%(.Penelitian di Liverpool telah menun.ukkan ah4a mungkin ada

huungan dengan asupan makanan dari mangan dan ke.adian Penyakit Perthes$

meskipun ini adalah penelitian -urrrently erlangsung dan hasilnya tidak

sepenuhnya .elas

"htt p23 3444)li v)a-)uk3HumanAn atom y3phd3r esear -h3perthes3perthpd)html()

6eskipun etiologi dari penyakit ini elum .elas, keadaan patologis yang

ada diseakan oleh nekrosis avaskular dari epi!isis kaput !emoralis) Proses in!ark

dan peraikan selan.utnya ertanggung .a4a terhadap sindrom yang dikenal

seagai Legg-Calve-Perthes "uku a.ar edah()

,euah studi nasional penyakit Perthes di Nor4egia dilakukan selama

periode lima tahun se.ak +anuari '11;. Ada %9 pasien yang terda!tar, yang

merupakan ke.adian tahunan rata-rata 1$% per '00000pada suyek di a4ah '9

tahun$ dan tingkat ter.adinya '2:' untuk negara se-ara keseluruhan.Ada variasi

regional ditandai. Angka ke.adian terendah ditemukan di 4ilayah utara "9) per

'&& 000 per tahun( dan tertinggi di 4ilayah tengah dan arat "'&$7 dan ''$@per

'&& 000per tahun$ masing-masing() Ada ke-enderungan insiden yang leih tinggidi perkotaan "1$9 per '00 000 per tahun( diandingkan dengan daerah

pedesaan "7$1 per '&& 000per tahun() Usia rata-rata saat onset adalah 9$7 tahun

"',@-'9$%( dan laki-laki2 perempuan adalah rasio @)@2')

#ami memandingkan &% pasien dengan kelompok kontrol -o-ok non5

terkena anak "n ' 0%9 19%( dari Birth egistry Nor4egia 6edis dan data

dianalisis iu "usia saat melahirkan$ paritas$ durasi kehamilan($ pan.ang dan erat

lahir$ presentasi lahir $ lingkar kepala$ indeks Ponderal dan adanya anomali
http://www.liv.ac.uk/HumanAnatomy/phd/research/perthes/perthpd.htmlhttp://www.liv.ac.uk/HumanAnatomy/phd/research/perthes/perthpd.htmlhttp://www.liv.ac.uk/HumanAnatomy/phd/research/perthes/perthpd.html


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kongenital. Anak-anak dengan penyakit Perthes se-ara signi!ikan leih

pendek saat lahir dan memiliki peningkatan !rekuensi anomali kongenital.

6enerapkan log-normal 6odel ,art4ell tentang periode inkuasi untuk

distriusi usia saat onset penyakit Perthes menun.ukkan -o-ok dengan kurva log-

normal) *emuan kami mengarah ke penyea tunggal$ aik genetik atau

lingkungan, ertindak seelum lahir dalam etiologi

penyakit Perthes )

"htt p23 3444).. )oneand.oi nt)org)uk3-ontent3 775 B3 13'%':)astra-t(

Penyea dari penyakit Perthes dihipotesiskan seagai suatu ke.adian

vaskular yang diikuti oleh revaskularisasi dan ersi!at multi!aktorial) *idak adapola genetik yang teridenti!ikasi pada penderita dan !rekuensi diantara keluarga

terilang rendah)

#aput !emur ergantung pada pemuluh epi!isis lateral untuk suplai darah

saat usia sampai : tahun) Penyea dari in!ark kaput !emur masih kontroversial

dan isa dikarenakan oleh arteri itu sendiri oleh karena tromosis venosus .,uplai

darah arteri pada sisi yang terkena dapat melemah$ dengan ostruksi pada arteri

kapsular superior atau arteri medial sirkum!le=. Dalam pemerian kontras vena

pada kaput !emur merupakan kalier medium$ sama dengan vena kutaneus atau

vena sereral) Hipertensi vena telah ditemukan pada pasien dengan penyakit

Perthes) 6eskipun demikian$ masih elum dapat dipastikan apakah tromosis

merupakan hal utama atau hanya erkontriusi dalam penyakit ini seagai

kominasi dari etiologi lainnya.Anatomi vaskular pada anak laki-laki usia antara

samapi 7 tahun memuat mereka leih rentan terhadap keadaan hipoerkoagulael

dan !aktor-!aktor lainnya. #e.adian protromotik pada keadaan hiperkoagulael

dapat mengakiatkan tromosis dan in!ark dari kaput !emur)

*romosis vaskular .arang ter.adi di usia muda, namun dapat ter.adi

dikarenakan de!ek genetik$ seperti resistensi terhadap vitamin C teraktivasi)

Protein C adalah protein protromotik dependen terhadap vitamin # yang

mengakiatkan teratasnya enim5enim prokoagulan$ !a-tor5!aktor Fa dan

tromin melalui !aktor G dan GIII. aktor Leiden G diimplikasikan pada proses

protromik$ kandungannya yang memlokade ikatan antara protein C teraktivasi
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kepada !aktor) Belum ada ukti konkrit apakah de!isiensi ini diakiatkan oleh

konversi atau resistensi dari entuk teraktivasi) Bagaimanapun .uga$ de!isiensi

protein C dapat menyeakan tromosis pada vena dengan kalier medium yang

mengakiatkan iskemik tulang dan kartilago)

Anak5anak penderita penyakit Perthes menun.ukkan kemampuan !ungsi

yang erkurang, anak-anak terseut tidak mampu erdiri seagaimana mestinya)

>@9? Pemuluh darah epi!isis lateral yang er.alan di retina-ula dan rentan

terhadap peregangan dan tekanan dalam hal e!usi) >%:? >%7? >%1? huungan

kausati! antara sinovitis transien dan penyakit Perthes elum isa ditetapkan

karena ,inovitis transien pada dasarnya adalah seuah penyakit .inak dan kadang5kadang anak5anak dengan ge.ala erlarut-larut eresiko terkena penyakit Perthes )

>@;? >@:? 6ungkin ada peningkatan terkait dalam intra5artikular tekanan$ dengan

ke.adian pemuluh darah ersamaan) >%?

,euah !enotipe tertentu yang -enderung terdiri dari ertuuh ke-il, usia tulang

tertunda dan pra-puertas penangkapan skeletal. Hal ini telah menyeakan

hipotesis ah4a endo-rinopathy mendasari mungkin hadir) Anak-anak ini$

agaimanapun$ memiliki pera4akan normal '% sampai '9 tahun) >'? >@7? >@1?

somatomedin Elevated !aktor pertumuhan A atau insulin-like "I0( % tingkat

menun.ukkan ah4a Perthes mungkin men.adi penyakit transisi pertumuhan)

>&? >'? >%? Namun,somatomedin C "I0'( tingkat normal pada pasien ini.>@?

,euah esar$ -ross-se-tional$ studi longitudinal klinis anak euthyroid dengan

penyakit Perthes menemukan tingkat tinggi se-ara signi!ikan eas * dan *@

diandingkan dengan kontrol normal, terutama pada pasien dengan tingkat yang

leih esar keterliatan kepala !emoral) >';? >? ,euah turnover tulang

erkurang .uga men-atat, meskipun tidak .elas apakah ini adalah penyea atau

e!ek) >9?

Penyakit Perthes leih sering ter.adi pada pasien dengan displasia skeletal)

*erdapat huungan antara penyakit Perthes dan ADHD) Perokok pasi! atau

ri4ayat iu yang merokok selama masa kehamilan dapat men.adi !aktor yang

erkontriusi pada penyakit ini. Penyakit Perthes adalah kondisi non traumatik$

meskipun demikian ri4ayat trauma minor dapat ditemukan pula)


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%)@ aktor isiko

Penyakit Perthes ter.adi pada anak5anak dengan rentang usia sampai '&

tahun) Penyakit ini ter.adi empat kali leih sering pada laki-laki diandingkan

dengan perempuan) Dengan demikian risiko LCPD pada anak-anak perokok

pasif lebih dari lima kali lebih tinggi dibandingkan pada anak-anak yang

tidak terpapar asap rokok. Tampaknya bahwa merokok pasif merupakan

faktor langsung maupun tidak langsung berhubungan dengan LCPD

(ata et al., !000". Penyakit #uami bilateral P$D$ !0 persen khususnya

pasien (%uku a&ar %edah".

Risk factorsshow all

Strong

male se=

Perthes disease is aout or 9 times more -ommon in males than

!emales) *he uni/ue vas-ular anatomy o! oys et4een to 7 years

makes them parti-ularly vulnerale in the presen-e o! hyper-

-oagulale states and other !a-tors.>%:? >%7?>%1?

age to 7 years

*ypi-ally$ Perthes disease a!!e-ts -to 7-year-olds 4ith a mean

age o! : years$ although it -an a!!e-t -hildren et4een % to '% years

o! age and -an also a!!e-t adoles-ents)

so-io5e-onomi- deprivation

Poverty and so-ial deprivation are asso-iated 4ith Perthes disease)

hyper5-oagulale states

Gas-ular thromosis is un-ommon at a young age ut may o--ur

due to a geneti- de!e-t,> %%? su-h as resistan-e to a-tivated

protein C) Protein C is a vitamin #-dependent prothromoti-

protein that
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leads to -urtailment o! pro-oagulant enymes$ !a-tors Fa and

thromin$ via !a-tors G and GIII.> @&? a-tor G Leiden is

impli-ated in the prothromoti- pro-ess y virtue o! a sustitution

that lo-ks the inding o! a-tivated protein C to !a-tor G.> @'?

>@ % ? It is not -lear 4hether the de!i-ien-y is due to -onversion or

resistan-e to the a-tivated !orm) Ho4ever$ protein C de!i-ien-y

-auses thromosis in medium5-alire veins resulting in one and

-artilage is-haemia.> %'? >%%?>%@? >%?>%9? >@@?>@?

Weak

uran population

*he in-iden-e o! Perthes disease is higher in inner -ity populations)

> '? >% ? >@ ? > ? >9 ? >:? >7 ? >'&? >' % ? >' @ ?

Eskimos$ +apanese$ some -entral European ra-es

*he disease is more -ommon in some populations su-h as Eskimos$

+apanese and in some Central European ra-es) It is mu-h less

-ommon in la-k people$ native Australian$ Ameri-an Indian and

Polynesian ra-es) *he in-iden-e per '&0$&&& in ,outh A!ri-a varies

et4een '&)7 in 4hite people$ '): in those o! mi=ed an-estry and

&)9 in la-k people)

transient hip synovitis

Although some have reported this to e the !irst symptom o!Perthes disease$ this has not een proven$ and the asso-iation may

e in-idental.> @;? >@ : ? *here may e an asso-iated in-rease in

intra5arti-ular pressure,4ith a -on-omitant vas-ular event.> %?

hip .oint e!!usion

*he !emoral head relies on lateral epiphyseal vessels !or its lood

supply et4een and : years o! age) *hese vessels run in the
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retina-ula and are sus-eptile to stret-hing and pressure in the event

o! an e!!usion.> %:? > %7? >%1?

passive smoking

Passive smoking in the household and maternal smoking during

pregnan-y may e -ontriutory !a-tors.> :? > ' ' ? > %'? >% 9 ? > 7 ?

skeletal dysplasias

Perthes disease is more -ommon in patients 4ith skeletal

dysplasias)

trisomy %'

*his genotype has an in-reased in-iden-e o! -on-urrent hip

pathologies in-luding Perthes and slipped -apital !emoral

epiphysis)> 9@?

attention de!i-it hypera-tivity disorder "ADHD(

*here is an asso-iation et4een Perthes disease and ADHD.> ;?

> @1?

endo-rinopathy

A parti-ular phenotype that is asso-iated is a -hild 4ith small

stature$ delayed one age and pre-puertal skeletal arrest$ leading to

a hypothesis that an underlying endo-rinopathy may e present)

Elevated somatomedin A or insulin-like gro4th !a-tor "I0( %

levels suggest that Perthes may e a disease o! gro4th transition)

> &? > ' ? > % ? Ho4ever$ somatomedin C "I0'( levels are

normal in these patients.> @?

A large$ -ross-se-tional$ longitudinal study o! -lini-ally euthyroid

-hildren 4ith Perthes disease !ound signi!i-antly elevated levels o!

!ree * and *@ -ompared 4ith normal -ontrols$ parti-ularly in
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9/23

patients 4ith a greater e=tent o! !emoral head involvement.> ';?

> ?

A redu-ed one turnover is also noted$ although it is not -lear

4hether this is the -ause or e!!e-t.>9?

%)9 Pato!isiologi

,uplai darah ke !emur proksimal diperoleh dari arteri sirkum!leksia

!emoralis media) Pemuluh darah ini mementuk -in-in anastomosis pad aasis

kolum !emoralis) Dari -in-in ini, arteri retinakular posteroin!erior danposterosuperior melintasi kolum !emoralis untuk memperdarahi pusat osi!ikasi

sekunder pada epi!isis kaput !emoralis) Caang dari arteri sirkum!leksia !emoralis

lateral memperdarahi agian trokanter mayor. klusi total atau seagian dari

kelompok pemuluh darah ini mengakiatkan eragai dera.at nekrosis pusat

osi!ikasi sekunder "uku a.ar edah(.

+ika iskemia mengakiatkan in!ark tulang$ pertumuhan normal dari

epi!isis tulang erhenti untuk sementara 4aktu$ sedangkan kartilago yang

mendapatkan nutrisinya dari di!usi -airan synovial tetap tumuh) Daerah ke-il

kartilago yang erdekatan dengan epi!isis tulang dan tidak mendapatkan suplai

darah akan mengalami nekrosis .uga)

Epi!isis tulang akhirnya mendapatkan kemalia aliran darah) ,elama masa

revaskularisasi ini, penderita iasanya tidak menun.ukkan ge.ala. Bila .aringan

granulasi menyerang tulang nekrotik$ traekula yang mati tetap mengalami

sustitusi ertahap "penggantian ertahap dari tulang mati dengan tulang yang

masih hidup() ,elama !ase penyemuhan ini$ epi!isis tulang dan kartilago

diatasnya rentan terhadap de!ormasi dan hilangnya s!erisitas$ terutama .ika

terdapat distriusi anormal dari tenaga transartikular dari dari pinggul)

Children 4ith Perthes Disease have een !ound to e=hiit gro4th delay

a!!e-ting oth their skeletal maturity as 4ell as their anthropometri- status)

esear-h in Liverpool has also sho4n that the !eet are small in the su!!erer 4hen
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10/23

-ompared to their peer group) ther gro4th paramenters ehiit a tenden-y !or

smallness 4hen -ompared to normal una!!e-td -hildren

"htt p23 3444)li v)a-)uk3HumanAnatom y3phd3r esear -h3perthes3perthpd)html(

Penyakit Perthes melalui tahap dalam kurun 4aktu % sampai tahun se.ak a4al

kemun-ulannya,yaitu2

*ahap '$ iskemia2 agian kaput !emur terliat$ tergantung keparahannya.

Bagian epi!isis !emur erhenti tumuh pada periode ini yang erlangsung ;

sampai '% ulan) Pemeriksaan radiologi dapat menghasilkan gamaran

normal$ namun dengan kartilago artikular yang terus tumuh dan meneal

sehingga menyeakan peruahan$ meskipun tidak signi!ikan. Hal ini

dieut seagai tanda 8aldenstrom$ yaitu peningkatan rongga sendi dan

gamaran pseudosuluksasi ringan pada =-ray.

*ahap %$ resorpsi$ !ragmentasi$ revaskularisasi$ and peraikan2 !raktur

sukondral dapat terlihat pada tahap a4al di daerah dan hal ini diseut

seagai tanda ,alter atau Ca!!rey.ragmen traekular yang mati diresorsi

dan digantikan dengan .aringan !irosa yang akan mengalami kalsi!ikasi)

hilangnya dukungan stru-tural dikarenakan oleh resorpsi yang ter.adi pada

tulang -an-ellous pada agian proksimal dari epi!isis !emur.Hal ini akan

menyeakan de!ormasi pada epi!isis ketika dierikan ean normal

melalui sendi pinggul)tulang direvaskularisasi dengan lamella aru diatas

traekula yang mati$ hal ini akan memuar agian nu-leus terlihat

!ragment.. > 9&? esorpsi iasanya selesai setelah '% sampai '7 ulan)

#ista mun-ul di meta!isis !emur proksimal< keadaan yang semakin parah

dapat menyeakan osteolisis pada agian superolateral kaput !emur)

*ahap @$ reossi!ikasi dan resolusi2 re5ossi!i-ation typi-ally starts at the

epiphyseal margin "paraphyseal ossi!i-ation() --asionally, re-ossi!i-ation

through the physis results in a ony ridge leading to gro4th arrest in the

!emoral ne-k. > 1? esolution is usually -omplete 4ithin ; to %

months resulting in healing$ or a residual de!ormity in more severe -ases.

> 9'?
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11/23

*ahap $ remodeling2 kolapsnya kaput !emur selama remodeling dapat

mengakiatkan !lattening dan distorsi dari kaput) ive -lasses o! stru-tural

hip .oint -hanges$ ased on the spheri-ity o! the !emoral head and

asso-iated a-etaular -ongruen-e, have een des-ried. > 9%? *hese

-lasses are -losely linked to the !inal prognosis o! degenerative -hanges in

the hip .oint and demonstrate that spheri-ity is not as important a

prognosti- variale as the hip .oint -ongruen-e. An aspheri-al !emoral

head 4ith a good -ongruen-e o! the .oint 4ill at 4orst lead to a moderate

hip osteoarthritis) Ho4ever$ an aspheri-al in-ongruous .oint 4ill more

o!ten lead to severe osteoarthritis at a younger age.

%) Pemeriksaan

Pada pemeriksaan radiogra!i 1 pasien dengan penyakit Perthes dalam

tahap !ragmentasi dan .uga 8e revie4ed the radiographs o! 1 patients 4ith

Perthes disease at the stage o! !ragmentation and also a!ter the end o! skeletal

gro4th to assess the value o! the lateral pillar -lassi!i-ation o! Herring) *heaverage age o! the patients at diagnosis 4as : years ; months and the mean !ollo45

up 4as % years) *en o! the '' Herring group-A hips sho4ed good re-onstru-tion

o! the !emoral head) *here 4ere good results in group-B hips 4hen the patients

4ere less than nine years o! age at diagnosis) All '' group-C patients sho4ed hip

de!ormity at !ollo4-up) *he Herring -lassi!i-ation provides a valid long-term

prognosis in Perthes disease, although age at diagnosis is also an important

prognosti- !a-tor) *he -lassi!i-ation is relatively easy to apply, is reliale$ and

re/uires only an anteroposterior radiograph taken during the !ragmentation stage

o! the disease."htt p23 3444).. )oneand.oi nt)org.u k3-ontent3: :-B393 :@1)as tra-t()

History & examination

Key diagnostic factorsshow all

presen-e o! risk !a-tors "-ommon(
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12/23

isk !a-tors in-lude male se=$ age to 7 years$ so-io5e-onomi-

deprivation and a hyper--oagulale state.

8hen girls are a!!e-ted$ involvement o! the !emoral head is more

severe -ompared 4ith that in oys o! the same age.

*he age range -an vary !rom % to '% years and -an also rarely a!!e-t

adoles-ents) Higher the age at onset$ more severe is the

involvement as there is less re-modelling potential$ a higher e=tent

o! in-ongruity at the end o! the natural history and 4orse prognosis)

emales also tend to have a more severe involvement than males at

a later age.

limp "-ommon(

*he gait is a painless limp that -an e des-ried as a gluteus medius

lur-h$ though may e antalgi- during a-utely pain!ul episodes)

limited range o! motion at the hip .oint "-ommon(

*here is a !le=ion de!ormity o! the hip in the a-ute setting) 8ithprogression o! the disease addu-tion in !le=ion$ internal rotation$

and adu-tion in e=tension are limited due to impingement lesions)

*he later stage o! the disease may e -hara-teried y a gloal

redu-tion in all ranges o! motion 4ith asso-iated pain indi-ative o!

.oint arthritis)

Other diagnostic factorsshow all

short stature "-ommon(

6ost o! the -hildren are shorter than peers in height) Ho4ever$ they

attain puerty and a normal !inal height y the mid teens)

mus-le 4asting "-ommon(

8asting o! gluteal mus-les and /uadri-eps may e evident)


13/23

hypera-tivity "-ommon(

Children 4ith this disease tend to e hypera-tive and indulge in

several sporting a-tivities$ unless symptomati-)

*rendelenurgs sign "-ommon(

*his may e positive due to the mus-le 4asting)

synovitis "-ommon(

,ome studies have reported synovitis to e the !irst symptom$

although this has not een proven$ and the asso-iation may e

in-idental.> @;? >@:?

knee pain "un-ommon(

*he pain may have a varied radiation ased on the di!!erent nerves

that innervate the hip .oint< it may radiate to the suprapatellar

portion o! the knee through the !emoral nerve) *his -an lead tomistaken evaluation o! the knee and a delay in diagnosis)

thigh pain "un-ommon(


that innervate the hip .oint< it may radiate to the medial aspe-t o!

the thigh y 4ay o! the oturator nerve) *his -an lead to a delay in

diagnosis)

groin3utto-k pain "un-ommon(


that innervate the hip .oint< it may radiate to the utto-ks y 4ay o!

the s-iati- nerve) *his may o--asionally lead to a delay in

diagnosis)
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14/23

asymmetri- lim length "un-ommon(

*he presen-e o! an addu-tion -ontra-ture may a--ount !or an

apparent de-rease in lim length$ although there may also e true

shortening !rom loss o! !emoral head height as -ollapse progresses)

minor trauma "un-ommon(

*here may e an asso-iated history o! trauma at presentation)

Perthes disease is a non-traumati- -ondition$ although a history o!

minor trauma may e noted)

Diagnostic tests

1st tests to ordershow all

Test Result

ilateral hip =-rays

Anteroposterior and !rog lateral vie4s should e

taken) Gie 4 im a geGie 4 i mage Helps determine

the stage o! the disease pro-ess)

!emoral head -ollapse

and !ragmentation

Tests to considershow all

Test Result

BC normal
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15/23

Consider in the a-ute phase to e=-lude other

-onditions)

serum E,


-onditions)

may e rea-tively

in-reased in the

symptomati- phase o!

the disease or may

indi-ate an alternative

pathology

serum C-rea-tive protein


-onditions)

may e rea-tively

in-reased in the

symptomati- phase o!

the disease or may

indi-ate an alternative

pathology

one s-intigraphy

Helps in the diagnosis during the is-haemi-

stage 4hen radiographs may e normal)

-old spot in the a!!e-ted

hip early in the disease

pro-ess

6I o! hips

,hould e -onsidered i! radiographs appear

normal) It is also a use!ul ad.un-t in the early

stages o! diagnosis$ and has een !ound to e a

sensitive modality in diagnosing Perthes

disease. > 9? I! per!ormed ; months a!ter

disease onset$ an 6I -an a--urately

demonstrate the degree o! epiphyseal

involvement.> 99? A!ter reossi!i-ation$ 6I o!

the hips may also e

use!ul in assessing the e=tent o! the damage in

one or oth hips.Gie 4 imag e Gie 4 im a ge

!emoral head -ollapse

and !ragmentation
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16/23

Differential diagnosis

ConditionDifferentiating

signs/symtoms

Differentiating tests

, e pt i - a rth r it is

A-ute onset)

,ystemi- symptoms)

Unilateral

involvement)

A-ute pain in the hip)

6arked restri-tion in

movement)

Elevated 8BC

-ount)

Positive lood

-ultures)

F-ray sho4s

4idened .oint spa-e.

+oint e!!usion on

ultrasound

e=amination)

+oint aspiration may

-on!irm the

in!e-tion)

*ransient synovitis

o! the hip

A-ute onset)

Prior history o! viral

illness)

6ild systemi-

!eatures)

Unilateral

involvement)

estri-ted motion 4ith

minimal pain)

Normal 8BC

-ount)

Negative lood

-ultures)

Normal =-rays)

Ultrasound

e=amination sho4s

.oint e!!usion)

+oint aspiration is
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17/23

,pontaneous

improvement over %

to 7 hours)

sterile.

, i-kle -e ll dise a se

A-ute adominal pain)

,ystemi- symptoms)

Unilateral involvement

4ith asso-iated long

one osteomyelitis)

,i-kling test

positive.

+ uv e ni l e id i op a th i -

a rth r it is

A-ute or -hroni-

presentation)

,ingle or multiple

.oint involvement)

,ystemi- symptoms)

E!!usion o! the

a!!e-ted .oints)

e-urren-e o! a-ute

episodes)

High 8BC -ount in

a-ute phase.

heumatoid !a-tor

positive.

+oint aspirate sho4s

4hite -ells$ no

-rystals or

organisms)

F-rays may sho4

de-reased .oint

spa-e.

6ultiple epiphyseal

dysplasia "6eyerJs

disease(

Bilateral epiphyseal

dysplasia 4ith oth

hips at the same stage

and 4ith the same

degree o! involvement)

Although Perthes

disease -an e

ilateral$ it is more

likely to e se/uential

,e/uential !ilms

sho4 -ontinued

disease e/uality and

other .oint

epiphyseal

involvement)
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18/23

than simultaneous)

,pondyloepiphyseal

dysplasia

Bilateral Perthes

disease 4ith spine

involvement$ kno4n

as platyspondyly.

,pine radiographs

demonstrate

!eatures o!

platyspondyly$

4hi-h are a

!lattened verteral

ody shape 4ith

redu-ed distan-e

et4een end plates)

%)9 Penatalaksanaan

Penatalaksanaan dari penyakit Perthes didasarkan pada prinsip

meminimalisasikan kerusakan dari sendi pinggul dan kaput !emur) +ika

memungkinkan$ maka diusahakan untuk men.aga entuk senormal mungkin pada

dua tulang di sendi terseut sehingga memungkinkan untuk mendapatkan hasil

!ungsional yang normal agi pasien likely to e as normal a !un-tional hip as

possile. 8hile traditional splints and removal o! 4eight earing have een

employed 4idely, generally, surgeons today pre!er to undertake an operation

on the !emur or hip in order to maintain the internal one shapes 4ithin the .oint)

*his also minimalises time a -hild might loose in s-hooling and other a-tivities)

It is very important to note that ea-h -ase must e -onsidered in its o4n -onte=t

y the teating orthopaedi- surgeon and that the treatment undertaken is seen as

est !or that individual)As the -ondition seems sel!-limiting$ re-overy$ 4hile

variale !rom -ase to -ase,is usually -omplete$ although i! there is damage to the

hip$ it is possile that !urther teatment may e re/uired in later li!e. *his is a

su.e-t eing


19/23

resear-hed at present)" ht t p2 3 344 4 )l i v)a- )uk3

HumanAnatomy3phd3resear-h3perthes3perthpd)html)

%); #omplikasi

Comlications Timeframe !ikelihood

osteoarthritis

s e e our - omp r e h e nsive - ov e ra g e o !

steoarthrit is

Containment o! the hip$ spheri-ity o! the !emoral

head and -ongruen-e o! the hip .oint in!luen-e

development o! .oint arthritis) +oint

anormalities stem !rom a -omination o!

altered iome-hani-s and the initial vas-ular

insult to the arti-ular -artilage.

long term medium

lim length ine/uality

keterliatan dari epi!isis !emur proksimal dan

gangguan suplai pada plat pertumuhan !emur

proksimal dapat mengakiatkanpremature

-losure.Hal ini dapat menyeakan

pemendekan tungkai pada agian yang terkena

penyakit.

long term medium

kaku dan hilangnya !ungsi rotasi

*he position that the hip is held in during the

disease pro-ess determines the degree o!

sti!!ness and loss o! hip rotation) 6aintaining

hip moility throughout the disease pro-ess

helps prevent this -ompli-ation) ,e-torial

variale high
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20/23

!emoral head damage -ould -ontriute to the

loss o! rotation)

%): Prognosis

*his nation4ide prospe-tive study 4as designed to determine prognosti- !a-tors

and evaluate the out-ome o! di!!erent treatments o! PerthesJ disease.

,eanyak %7 rumah sakit di Nor4ay diinstruksikan untuk melaporkan semua

kasus aru dari penyakit Perthes dalam periode 9 tahun dan seanyak %9A total

o! %7 hospitals in Nor4ay 4ere instru-ted to report all ne4 -ases o! PerthesJ

disease over a period o! !ive years and %9 patients 4ere reported and !ollo4ed !or

!ive years) ! these,@;7 4ith unilateral disease 4ere in-luded in the present study)

*he hips 4ere -lassi!ied radiologi-ally a--ording to a modi!ied t4o-group

Catterall -lassi!i-ation and the lateral pillar -lassi!i-ation) A total o! @97 patients

"1:K( attended the !ive-year !ollo4-up$ 4hen a modi!ied three-group ,tulerg

-lassi!i-ation 4as used as a radiologi-al out-ome measure.or patients over si=years o! age at diagnosis and 4ith more than 9&K ne-rosis o! the !emoral head

"'9% patients($ the surgeons at the di!!erent hospitals had -hosen one o! three

methods o! treatment2 physiotherapy "99 patients($ the ,-ottish ite adu-tion

orthosis "%;(, and pro=imal !emoral varus osteotomy ":'(. ! these hips$ ';

"1;K( 4ere availale !or the !ive-year !ollo4-up)

*he strongest predi-tor o! out-ome 4as !emoral head involvement o! more or less

than 9&K "odds ratio "( :):;$ 19K -on!iden-e interval "CI( %)7% to %')@:($

!ollo4ed y age at diagnosis " &)17$ 19K CI &)1% to &)11( and the lateral

pillar -lassi!i-ation " &);%$ 19K CI &)& to 0)17() In -hildren over si= years at

diagnosis 4ith more than 9&K o! !emoral head ne-rosis$ pro=imal !emoral varus

osteotomy gave a signi!i-antly etter out-ome than orthosis "p 0.&&'( or

physiotherapy "p 0.0&'() *here 4as no signi!i-ant di!!eren-e et4een the

physiotherapy and orthosis groups "p &)@;($ and 4e !ound no di!!eren-e in

out-ome a!ter any o! the treatments in -hildren under si= years "p &):@(.


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Garus osteotomi !emur proksimal direkomendasikan pada anak5anak usia diatas ;

tahun ketika terdiagnosis dengan nekrosis kaput !emur leih dari 9& persen) *he

adu-tion orthosis should e aandoned in PerthesJ disease.

"http23 3 4 4 4)..)one a nd.o i nt)or g )uk3-ont e nt 3 10-B 3 '&3'@;)astr a- t(

BAB III

#E,I6PULAN

Perthes merupakan suatu self limiting diseaseJ pada kaput !emur yang

terdiri dari nekrosis, kolaps$ peraikan dan remodeling yang ter.adi pada de-ade

pertama kehidupan$ khususnya leih sering ter.adi pada anak laki-laki) Pasien

iasanya memiliki !enotip karakteristik pera4akan pendek, usia tulang tertunda$

dan hiperaktivitas.Perthes iasanya ersi!at unilateral$ ke-uali pada '0-%0persen

kasus yag ditemui.0e.ala klinis meliputi gaya er.alan pin-ang dan nyeri pinggul

sering men.alar ke paha$ lutut$ pangkal paha$ pantat atau perut) Nyeri akan
http://www.bjj.boneandjoint.org.uk/content/90-B/10/1364.abstracthttp://www.bjj.boneandjoint.org.uk/content/90-B/10/1364.abstracthttp://www.bjj.boneandjoint.org.uk/content/90-B/10/1364.abstracthttp://www.bjj.boneandjoint.org.uk/content/90-B/10/1364.abstract


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semakin memuruk dengan aktivitas) Penatalaksanaan yang dilakukan ergantung

pada usia pasien.Pilihan yang ada meliputi termasuk moilisasi dan pemantauan,

penahanan tanpa pemedahan atau operasi$ serta prosedur penyelamatan.

DA*A PU,*A#A

Hamilton Health ,-ien-es) %&'%) Perthes Disease.6-6asterJs Children Hospital)

6ata, ,0)$ Ai-ua, EA)$ ve.ero$ AH., 0rande, 66) %&&&) Legg-Calve-

PerthesDisease and Passive ,moking.+ournal o! Pediatri- rthopaedi- %%"@(2 @%;-

@&) http23 3.ournals)l44.-om3 pedorthopaedi -s3Astra-t3 %&&&3&9&&&3 Le ggM

CalvP erthesMDiseaseM andMP assi veM, moki ng.' ')asp=. >diakses pada2 %

+anuari %&'@?.

#im$ H#8. %&'&) Legg-Calv-Perthes Disease.Journal of American

Academy of Orthopedic Surgeons '7"''(2 ;:;-7;)

ht t p2 3 34 4 4). aa os)o r g 3 - ontent3'73

''3;:;)astra-t) >diakses pada2 %@ +anuari %&'@?.
http://journals.lww.com/pedorthopaedics/Abstract/2000/05000/Legg_%20Calv__Perthes_Disease_and_Passive_Smoking.11.aspxhttp://journals.lww.com/pedorthopaedics/Abstract/2000/05000/Legg_%20Calv__Perthes_Disease_and_Passive_Smoking.11.aspxhttp://journals.lww.com/pedorthopaedics/Abstract/2000/05000/Legg_%20Calv__Perthes_Disease_and_Passive_Smoking.11.aspxhttp://journals.lww.com/pedorthopaedics/Abstract/2000/05000/Legg_%20Calv__Perthes_Disease_and_Passive_Smoking.11.aspxhttp://journals.lww.com/pedorthopaedics/Abstract/2000/05000/Legg_%20Calv__Perthes_Disease_and_Passive_Smoking.11.aspxhttp://journals.lww.com/pedorthopaedics/Abstract/2000/05000/Legg_%20Calv__Perthes_Disease_and_Passive_Smoking.11.aspxhttp://journals.lww.com/pedorthopaedics/Abstract/2000/05000/Legg_%20Calv__Perthes_Disease_and_Passive_Smoking.11.aspxhttp://journals.lww.com/pedorthopaedics/Abstract/2000/05000/Legg_%20Calv__Perthes_Disease_and_Passive_Smoking.11.aspxhttp://journals.lww.com/pedorthopaedics/Abstract/2000/05000/Legg_%20Calv__Perthes_Disease_and_Passive_Smoking.11.aspxhttp://journals.lww.com/pedorthopaedics/Abstract/2000/05000/Legg_%20Calv__Perthes_Disease_and_Passive_Smoking.11.aspxhttp://journals.lww.com/pedorthopaedics/Abstract/2000/05000/Legg_%20Calv__Perthes_Disease_and_Passive_Smoking.11.aspxhttp://journals.lww.com/pedorthopaedics/Abstract/2000/05000/Legg_%20Calv__Perthes_Disease_and_Passive_Smoking.11.aspxhttp://www.jaaos.org/content/18/http://journals.lww.com/pedorthopaedics/Abstract/2000/05000/Legg_%20Calv__Perthes_Disease_and_Passive_Smoking.11.aspxhttp://journals.lww.com/pedorthopaedics/Abstract/2000/05000/Legg_%20Calv__Perthes_Disease_and_Passive_Smoking.11.aspxhttp://journals.lww.com/pedorthopaedics/Abstract/2000/05000/Legg_%20Calv__Perthes_Disease_and_Passive_Smoking.11.aspxhttp://www.jaaos.org/content/18/


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