7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 1/21

DANDY WALKERSYNDROMEDibimbing oleh : dr. Jefry, Sp. A.

Di!!n oleh : Y"min M"ri" S"n#oo $%&'()&*')&*+

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 2/21

An"#omi

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 3/21

De-nii

Kel"in"n ongeni#"l ber!p" m"l/orm"ip"d" erebel!m

'001  S!##on, em!di"n di2el""n

lebih rin3i oleh Dandy d"n 4l"3 5"np"d" #"h!n

 6"h!n '78%  oleh 6"g"r# d"n Walker 

Ahirny" p"d" #"h!n '7+8, 4end"men"m""n peny"i# ini eb"g"i DandyWalker.

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 4/21

D"ndy W"ler Syndrome

pembe"r"n 9en#riel eemp"#

"genei "#"! hipopl"i" 9ermierebel!m

pembe"r"n p"d" /o" po#erior

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 5/21

Epidemiologi

' d"ri (&.&&& el"hir"n

eremp!"n ; L"i)l"i

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 6/21

E#iologi d"n 5"#or Riio

De/e romoom y"ng mempeng"r!hiperemb"ng"n o#" /e#!

<n/ei 9ir"l p"d" ib! ""# h"mil

"p"r"n #oin "#"! ob"#)ob"#"n p"d"b"yi y"ng bel!m l"hir

M"#ern"l di"be#e

S!mb"#"n p"d" /or"men M"gendie "#"!L!3h" ""# embriogenei

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 7/21

=e2"l" Klini

eriode e"rly in/"n3y "#"! ""# men2el"ng!i" ' #"h!n :

eremb"ng"n mo#ori y"ng l"mb"#

embe"r"n ep"l" "#"! #engor" y"ngprogrei/ 

De9elopmen#"l del"y p"d" "pe

mo#ori d"n em"mp!"n b"h"" dim"n" "n" !li# !n#! d!d!, ber2"l"n,d"n berbi3"r"

Ke2"ng

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 8/21

=e2"l" Klini

ening"#"n #e"n"n in#r"r"ni"l

<ri#"bili#"

M!n#"h

 6"nd")#"nd" di/!ngi erebel"r

ening"#"n ling"r ep"l"

4!lging p"d" b"gi"n po#erior ep"l"

="ngg!"n ner9! y"ng meng"#!r m"#",>"2"h, d"n leher

ol" pern"p""n y"ng "bnorm"l.

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 9/21

=e2"l" Klini

enon2ol"n d"er"h oipi#"l $b!lging

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 10/21

Di"gnoi

MR< : mod"li#" pilih"n !n#!mendi"gnoi DWM

?6 S3"n : mendi"gnoi DWM er#"

menge9"l!"i /!ngi shunt p"d" p"ien y"ng

#erp""ng shunt.

@S= : melih"# "bnorm"li#" p"d" 9ermierebel!m d"n i#" /o" po#erior

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 11/21

MR<

T2 weighted MRI : kista CSF besar di fossaposterior, hipoplasia heisfer serebel!"winged appearan#e$

T% wighted MRI : &entri#!loegalydan displa#ed posterior fossa #yst

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 12/21

Di"gnoi

Dandy Walker #ople' : D"ndy W"ler M"l/orm"#ion $DWM

D"ndy W"ler 9"ri"n# $DW

Meg" 3i#ern" m"gn"

Ki#" "r"3hnoid /o" po#erior

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 13/21

D"ndy W"ler "ri"n#

DW : ben#! y"ng lebih ring"n d"riDWM

5o" po#erior : norm"l "#"! edii#

membe"r en#riel < : dil"#"i ring"n "mp"i

ed"ng

Bemi/eri!m 3erebelli : norm"l er#"hipopl"i $b!"n "genei 9ermi3erebelli

 J"r"ng di2!mp"i "d"ny" hydrocephalusp"d" el"in"n ini.

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 14/21

D"ndy W"ler "ri"n#

D"ndy W"ler)9"ri"n# p"d"p"ien "n" peremp!"n !i"'( #"h!n deng"n "genei3orp! 3"llo!m d"nhipopl"i" 9ermi in/erior

$en#riel emp"# membe"r,n"m!n /o" po#eriornorm"l

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 15/21

Meg" ?i#ern" M"gn"

5o" po#erior y"ng be"r "ib"#pembe"r"n 3i#ern" m"gn".

ermi 3erebelli d"n 9en#riel < :

norm"l.

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 16/21

Meg" ?i#ern" M"gn"

Meg" ?i#ern" M"gn" p"d" MR< 6)' >eigh#ed $3"ir"nre#roerebel"r deng"n 9en#rielemp"# d"n 9ermi y"ng norm"l

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 17/21

Ki#" Ar"noid

Ki#" 2in" y"ng #er2"di di 3erebropin"le "r"h membr"n "r"3hnoid d"n #id"berh!b!ng"n deng"n i#em 9en#riel

4erii 3"ir"n bening #id" ber>"rn" Ki#" "r"noid re#ro3erebell"r e2"#i  

mende9i"i 9en#riel < d"n 3erebell!m

e "r"h "n#erior

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 18/21

Ki#" Ar"noid

Ki#" Ar"3hnoid 5o"o#erior p"d" p"ien "n"peremp!"n !i" '+ b!l"n

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 19/21

 6"#"l""n"

 6ind""n bed"h"r"/ :en#ri3!loperi#on

e"l Sh!n# $Sh!n#

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 20/21

D"/#"r !#""

N"#ion"l <n#i#!#e o/ Ne!rologi3"l Diorder "nd S#roe $N<NDS. N<NDS D"ndy)W"ler yndromein/orm"#ion p"ge. C3i#ed %&'( Sep#ember &(. A9"il"ble /rom:h##p:>>>.nind.nih.go9diorderd"ndy>"ler.h#m.

Agr">"l S F 6h"!r . <m"ge in D"ndy)W"ler m"l/orm"#ion. 4MJ ?"e Repor#. C3i#ed %&'( Sep#ember&(. A9"il"ble /rom: h##p:3"erepor#.bm2.3om3on#en#%&&7b3r.&1.%&&0.&++7./!llGidH(88337/") %*e*)8%e")"'bb)eddd1137b3e+.h#ml

Ad"m A, DiIon AK, =!nny RS, ?hong WK. edi"#ri3 ne!rology. <n: Ad"m A, DiIon AK, ed. Ad"m =r"ingerF Allion di"gno#i3 r"diology. +#h edn. hil"delphi": Ele9ier ?h!r3hill Li9ing#one, %&&0: '*++)*.

Kliegm"n RM, 4ehrm"n RE, Jeon B4, S#"n#on 45, John#on M, Kinm"n S. ?ongeni#"l "nom"lie o/ #he3en#r"l ner9o! y#em. <n: Kliegm"n RM, 4ehrm"n RE, Jenon B4, S#"n#on 45, ed. Nelon #eI#boo o/pedi"#ri3. '1#h edn. hil"dephi": W4 S"!nder, %&&8: '77'.

<n3e! L. <m"ging in D"ndy)W"ler m"l/orm"#ion. C3i#ed %&'( Spe#ember &(. A9"il"ble /rom:h##p:emedi3ine.med3"pe.3om"r#i3le8&0&+7) o9er9ie>.h#m

4"ro9i3h AJ, K2o 4O, Norm"n D, Ed>"rd MS. Re9ied ?l"i-3"#ion o/ po#erior /o" 3y# "nd 3y#liem"l/orm"#ion b"ed on #he re!l# o/ m!l#ipl"n"r MR im"ging. Am J Roen#genol. '707 '+(: '%07)(&&.

Ry"n M, =renier E, ?"#ro A, Nemerof ?4. Ne>)one# py3hoi "o3i"#ed >i#h d"ndy)>"ler 9"ri"n# in

"n "dole3en# /em"le p"#ien#. J Ne!ropy3hi"#ry ?lin Ne!ro3i. %&'% %8$%: %8')*. 4end" ?E. 6he D"ndy)W"ler yndrome or #he o)3"lled "#rei" o/ #he /or"men M"gendie. J Ne!rop"#hol

EIp Ne!rol. '7+8 '( '8)%7

Simin F ="ill"rd 5. D"ndy)W"ler 3on#in!!m. C3i#ed %&'( Spe#ember &(. A9"il"ble /rom:h##p:r"diop"edi".org"r#i3led"ndy)>"ler3on#in!!m.h#ml

7/21/2019 Dandy Walker Syndrome

http://slidepdf.com/reader/full/dandy-walker-syndrome-56da6c9576923 21/21

 6ER<MA KAS<B