hydrocephalus & spina bifida

8/20/2019 Hydrocephalus & Spina Bifida

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HYDROCEPHALUS (H) &

SPINA BIFIDA (SP BI)SAM LAZUARDI


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SPINA BIFIDA (SP BI)

• Spina bifida (split spin) ! in"#$plt "l#s% #f$b'#ni" n%al t%b #l'in* tba %n+

f%sd & #pn

abn p#ti#n #f sp "#d sti", #%tt-#%*- #pnin* in b#ns

• Sp bi . "at*#is ! sp bi #""%lta sp bi "'sti"a($'l#$nin*#"l) $nin*#"l lip#+

$nin*#"l• L#"ati#n ! l%$ba & sa"al

• /'l#$nin*#"l disabilit'

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• Sp bi "an b "l#sd s%*i"all' b%t da$a* is p$annt

• Sp bi in"idn" "an b 1 23 4 b' dail' f#li" a"idinta, bf# "#n"pti#n

• Sp bi l%$ba s'$pt#$s -ip l* ,n f##t$%s"ls & s"#li#sis

• Sa"al f##t $%s"ls b#5l+ bladd p#bl$s"ti#n

• Oftn -'d#"p-al%s al' intll"t%al p#bl$s

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• 9t-d sp "#d s' (9SC) as a s%lt f#$ s"a

tiss% f#ll#5in* s%*' t# "l#s #pn df"t sp bi

$'l#$nin*#"l al' D: & 9- : bf# p$annt da$a*

• 9SC s'$pt#$s ! l#5 b#d' pain l* 5a,nss

in"#ntinn" s"#li#sis n%$bnss

• 26 4 Sp bi all*' t# lat; (*l#s "at-t t")

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• Sp bi #""%lta (-iddn) ! n# #pnin* b%t #%t pat

#f tba n#t "#$pltl' "l#sd s,in n#$al :

s#$ -ai *#5in* di$pl in s,in lip#$a #d$al sin%s bit-$a,

• /a' s%ff 9SC s' f#$ diast$at#$'lia #

lip#$a in lip#$'l#$nin*#"l sp "#d

tappd bl#5 aff"td ll #f *#5in* spin l*s & bladd s'$pt#$s

• Ba", pain 5#s 5it- sp bi #""%lta

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• /nin*#"l # $nin*al "'st (last "#$$#n)

• Sp bi "'sti"a $#st "#$$#n & si#%s

• Unf%sd p#ti#n #f sp "#ll%$n all#5s sp "#d p#t%d t-#%*- #pnin* in #l'in* tb

• /nin*al $$ban "#in* sp "#d f#$ a sa"n"l#sin* spinal l$nts

• Sp bi = $'l#s"-i>is $#st s ! plat+li, $ass#f n#%s tiss% 5it- n# #l'in* s,in p#nt# inf"ti#ns

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• Dpndin* #f l#"ati#nsdf#$itis #f -ip

,ns ft l#ss #f $%s"l t#n s#$ d*

#f paal'sis l#ss #f snsati#n bl#5 ll #fspinal df"t bladd @ b#5l "#nt#l

p#bl$s

• Ass#"iatd abn#$alitis ! An#ld "-iai II("bll%$ $alf#$ati#n) $'l#$+

nin*#"l -'d#"p-al%s

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• Pat-#p-'si#l#*' !n%al t%b "l#s% 0<

da's p#stftili>ati#n

• Ca%s ! $%ltipl *n & ni#n$ntal

fa"t#s (anti"#n%lsant antidiabti" d%*s)

• #$n 5it- is, fa"t# -i*- d#s f#li"

a"id (. $*:d) 6 $# bf# "#n"pti#n + 0

5,s d%in* p*nan"' (n#$al 3. $* : d)


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• F#li" a"id in laf **is 5-#l *ains f%its& bans

• P*nan"' s"nin* ! AFP(alfa ft#p#+ tinf# D#5n s' SP BI N%al 9%b Df"t) USa$ni#"ntsis *nti" "#%nslin*

• In"idn" @ 0 p 333 bit-s

• 9at$nt ! s%*' (sp "#d & n ##ts p%t ba", int# spinal "anal & "#d 5it-$nin*s) & s-%nt f# -'d#"p-al%s

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OBESE O/EN

• Obs 5#$n si*nifi"antl' $# li,l' t#

-a "-ildn 5it- bit- df"t s%"- as

spina bifida -at df"ts "lft lip t-ansli$ $#t-s

• An sti$atd #n in si; p*nant 5#$n in

Bitain is #5i*-t (3 Fb 033?)

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Gita$in B0 Dfi"in"' Can Lad

9# C-ild-##d Bain Da$a*• #$n 5it- l#5 lls #f Gita$in B0 %st bf#

# aft "#n"pti#n a $# li,l' t# *i bit- t#

"-ildn 5it- n%al t%b df"ts (Pdiati"sFb%a' 033?)

• 9- st%d' a%t-#s 5#t t-at it is n#t ,n#5n -#5

B0 and f#lat $i*-t inta"t t# infl%n" t-

f#$ati#n #f t- n%al t%b

• $an' fis- and s-llfis- a a$#n* t- t#p s#%"s

#f ita$in B0

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HYDROCEPHALUS (H)

• H'd#"p-al%s 5at -ad

• Abn CSF a""%$%lati#n in nti"ls J IC

pss% in s,%ll p#*ssi -ad nla*+$nt "#n%lsi#ns & $ntal disabilit'

• N#$all' CSF "i"%lats t-#%*- bain

nti"ls spinal "#d & dains int#"i"%lat#' s'st$

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HYDROCEPHALUS (H)

• H "a%sd b' #p#d%"ti#n #f CSF "#n*n $alf bl#",in* "#$pli"ati#n #f ta%$a "apitis #infla$$ati#n bl#",a* in nti"ls

• An' "a%s #f CSF a""%$ in nti"ls bain"#$pssi#n "#n%lsi#n & tadati#nsp"iall' #ld infants

• Y#%n* infants abn la* -ad & d#5n5ad*a>

• 9-: s-%ntin* ;"ss CSF t# pit#n%$

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HYDROCEPHALUS (H)

• Intnal H ! .t- f#a$ina # "balaK%d%"t bl#",d CSF a""%$ in n+

ti"ls (bain st$ t%) J IC pss isibl bain da$a*

• E;tnal H ! CSF a""%$%lati#n in s%baa"-spa"

• H "a%s ! CSF fl#5 #bst%"ti#n abs#p+ti#n ;"ssi p#d%"ti#n

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HYDROCEPHALUS (H)

• Obst%"ti#n ($#st "#$$#n) ! aK%d%"t

stn#sis f#a$n /#n# stn#sis d% t# t%

infla$$ati#n -$#-a* "#n*nital$alf#$ati#n

• 0Op#d%"ti#n #f CSF ! "-##id pl;%s

papill#$a• H ! "#$$%ni"atin*

0 N#n+"#$$%ni"atin* (#bst%"ti)

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HYDROCEPHALUS (H)

• 1.Communicating H ! i$paid CSF abs#pti#nf%n"ti#nal i$pai$nt #f aa"-n#idal*an%lati#ns in s%p sa*ital sin%s b' s%baa"-n :IGH $nin*itis "-iai $alf "#n*n absn" #fPa"-i#nis *an%lati#ns N#$al Pss% H(NPH) H ;a"%# (nla*$nt #f nti"ls &s%baa"- spa" b' bain at#p-' in d$ntia

s"-i>#p-nia)

• NPH ! int$ittnt J ICP D : "#ntin%#%s inta+nti"%la pss% "#din*

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NOR/AL PRESSURE

HYDROCEPHALUS @ NPH• NPH ! Ha,i$ tiad *ait instabilit'

%ina' in"#ntinn" d$ntia

• F#"al si*ns ! n8 pals' *a> pals' (Paina%d

s') d% t# "#$pssi#n #f K%adi*$inal

plat

• 9- in"asd #l%$ #f CSF pss d#5n &da$a* bain tiss%

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RIS FAC9ORS OF NPH

• S%baa"-n#idal -$#-a*

• Cl#sd -ad i%'

• /nin*itis

• S%*' #f t- bain ("ani#t#$')

• NPH a""#%nt f# 7 4 #f all d$ntias• S'$pt#$s b*in sl#5l'

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6 SY/P9O/S OF NPH

• al,in* diffi"%ltis (*ait apa;ia) ft

-ld 5id apat s-%fflin* & %nstadinss

• 0Sl#5in* #f $ntal f%n"ti#n !

f#*tf%lnss diffi"%lt' pa'in* attnti#n &

apat-'

• 6Uina' in"#ntinn" & s#$ti$s f"alin"#ntinn"

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EMA/S AND 9ES9S OF NPH

• S%ddn falls 5it-#%t a l#ss #f "#ns"i#%snss

# d#ps atta",s $a' #""% al' in t- illnss

• al,in* "-an*s & in"asd dp tnd#nfl;s in t- l#5 l*s

• L%$ba p%n"t% 5it- "af%l tstin* #f

5al,in* bf# & aft spinal tap• Had C9+s"an # /RI #f t- -ad

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9at$nt #f NPH

Gnti"#pit#nal s-%nt

S#$ i$p# b%t $an' d# n#t

• it-#%t tat$nt s'$pt#$s #ftn *t 5#s and"#%ld lad t# dat-

• P#pl 5it- $ini$al s'$pt#$s -a t- bst

#%t"#$

• P#ssibl "#$pli"ati#ns ! inf"ti#n bldin*

d$ntia t-at b"#$ 5#s in%' f#$ falls

s-#tnd lif span

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HYDROCEPHALUS (H)

• Noncomm H / obstructive H "a%sd b' CSF#bst%"ti#n !

• F#a$n /#n# #bst%"ti#n ("#ll#id "'st)• 0AK%d%"t S'li ( atsia t% -$#- infla$)

• 6.t- nti"l #bst%"ti#n

• . F#a$n L%s"-,a & /a*ndi #bst (Dand'

al, $alf)• 7S%ba"-n spa" s%#%nd bainst$ #bst%"ti#n

(-$#-a* infla$$ati#n)

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HYDROCEPHALUS (H)

• C#n*nital H ! IGH in p$at% infants infla$

t'p 0 An#ld C-aii $alf Dand' al, $alf

aK%d%"t atsia• S'$pt#$s ! p## fdin* #$itin* apatis

d#5n5ad 's is%al d%"ti#n 5a,

$#$nts

•


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CHAIRI /ALFOR/A9ION+

C/• 9- indntd b#n' spa" at t- l#5 a #f

t- s,%ll is s$all t-an n#$al + "bl+l%$

& bainst$ is p%s-d d#5n5ad 9- s%ltin* pss% #n t- "bll%$ "an bl#", t- fl#5

#f "sf & "a%s di>>inss $%s"l 5a,nss

n%$bnss isi#n p#bl$s -ada"- and

p#bl$s 5it- balan" and "##dinati#n 9-a t- pi$a' t'ps #f C/

0<


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C/

• /#st "#$$#n is 9'p I + n# s'$pt#$s &

f#%nd b' a""idnt d%in* ;a$inati#n f#

an#t- "#nditi#n 9'p II (An#ld+C-iai $alf)is a""#$panid b' $'l#$nin*#+"l+a f#$

#f Sp Bi + patial # "#$plt paal'sis bl#5

t- spinal #pnin* 9'p III +$#st si#%s f#$

+ s n%#l#*i"al df"ts Oftn ass#"iatd5it- -'d#"p-a+l%s s'in*#$'lia and spinal

"%at%

0?


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63

Arnold-Chiari malformation, blockingfuid fow between the ventricles and ...www2.warwick.ac.uk


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DANDY+ALER SY

• Dand'+al, S'nd#$ is a "#n*nital bain

$alf in#lin* t- "bll%$ and t- fl%id+

filld spa"s a#%nd it + nla*$nt #f t-f#%t- nti"l a patial # "#$plt absn" #f

t- aa #f t- bain bt5n t- t5# "blla

-$isp-s ("blla $is) and "'st

f#$ati#n na t- intnal bas #f t- s,%llIn"as si> #f t- fl%id spa"s s%#%ndin* t-

bain & in"as in pss% + -'d#"p-al%s

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Dandy Walker isknown by many

names: DandyWalker Syndrome,Dandy WalkerMalformation,Dandy WalkerCyst, DandyWalker Variant


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6.

A sagittal T1-weighted magnetic resonance imaging (MRI) scan in a 5-year-old girl (alsosee the following 2 images) shows a large posterior fossa cyst elevating the torclarherophili and sins rects (short arrow)! The hypoplastic vermis is everted over theposterior fossa cyst (long arrow)! The cere"ellar hemispheres and "rainstem (") arehypoplastic! Thinned occipital s#ama is seen (arrowheads)!


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An a$ial T2-weighted MRI scan

that shows hydrocephals% a largecere"rospinal flid cyst in theposterior fossa% thinned occipital"one (arrows)% and hypoplasticcere"ellar hemispheres with awinged appearance (c)!


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HYDROCEPHALUS (H)

• A"K%id H "a%sd b' CNS infla$bain t%

$nin*itis ta%$a "apitis IC -$#-

• S'$pt#$s #f J IC pss ! Ha"- na%sa#$it papild$a slpinss "#$a

• J IC pss %n"al : "blla -niati#n

bain st$ "#$pssi#n lift-atnin*

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HYDROCEPHALUS (H)

• H s'$pt#$s ! lanin* disabilit' is%al p#bl$s

"l%$ps' p"#"i#%s p%bt' pilps'

• D: C9+s"an• 9- : nti"%la "at- int# nti"ls dain int#

b#d' "aitis (pit#n%$ i*-t ati%$ pl%a)

• S-%nt "#$pli"ati#n! $alf%n"ti#n infla$$ati#n

fail% -a"- na%sa #$it p-#t#p-#bia

si>%s

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hydrocephalus & spina bifida

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