169824294 perthes disease
TRANSCRIPT
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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
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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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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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-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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*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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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)
-
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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(
*he pain may have a varied radiation ased on the di!!erent nerves
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(
*he pain may have a varied radiation ased on the di!!erent nerves
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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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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Consider in the a-ute phase to e=-lude other
-onditions)
serum E,
Consider in the a-ute phase to e=-lude other
-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
Consider in the a-ute phase to e=-lude other
-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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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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,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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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
-
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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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!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 %&'@?.
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