4. kuliah klarif kelainan kongenital mayor
TRANSCRIPT
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Kelainan Kongenital Mayor
Kuliah Klarifikasi
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1. SPINA BIFIDA
Failure of fusion of the vertebral arches.
This mesodermal defect may be associated with adefect of ectoderm and neuroectoderm.
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Spina Bifida
Incidence 2 – 2.5 / 1,000 births
enetic !redis"osition
10# incidence of "ositive family histories
5# incidence of $"ina %i&da / 'nce"halocele
in siblin( of an a)ected child
10*15# incidence if two siblin(s a)ected
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Causes & Risk factors
Causes Risk factors
• + o c t o r s a r e n t c e r t a i n
• - o m b i n a t i o n o f ( e n e t i c a n d e n v i r o n m e n t a l r i s f a c t o r s
• F a m i l y h i s t o r y o f n e u r a l t u b e d e f e c t s a n d f o l i c a c i d d e & c i e n c y
ace his"anics, whites
$e (irls boys
Family history
Folate de&ciency edication
+iabetes
3besity
Increased body tem"
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Types of spina bidaSpina BidaSymptoms
1. s"ina bi&daocculta
2. s"ina bi&dacystica enin(ocele
yelomenin(ocele
sym"toms are caused by com"licationsof s"ina bi&da
!eo"le with s"ina bi&da
occulta are almostalways com"letelyasym"tomatic
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The most common complications
4arious de(rees of le( "aralysis, s"inecurvature scoliosis6, hi", foot, and le(deformities, and "roblems with bowel andbladder control
Infection in the tissues surroundin( thebrain menin(itis6.
7ydroce"halus
3besity due to inactivity6 and urinary tractdisorders due to "oor draina(e6
rowth hormone de&ciency resultin( inshort stature
8lthou(h most "eo"le with s"ina bi&da
http://www.emedicinehealth.com/scoliosis/article_em.htmhttp://www.emedicinehealth.com/obesity/article_em.htmhttp://www.emedicinehealth.com/growth_hormone_deficiency/article_em.htmhttp://www.emedicinehealth.com/short_stature_in_children/article_em.htmhttp://www.emedicinehealth.com/short_stature_in_children/article_em.htmhttp://www.emedicinehealth.com/growth_hormone_deficiency/article_em.htmhttp://www.emedicinehealth.com/obesity/article_em.htmhttp://www.emedicinehealth.com/scoliosis/article_em.htm
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Management
• T h e r e i s n o c u r e f o r s " i n a b i & d a
• 8 c h i e v e t h e h i ( h e s t " o s s i b l e l e v e l o f f u n c t i o n a n d i n d e " e n d e n c e
• T r e a t m e n t s h o u l d a d d r e s s a n y d i s a b i l i t y , " h y s i c a l , e m o t i o n a l , o r e d u c a t i o n a l , t h a t
i n t e r f e r e s w i t h t h a t " e r s o n 9 s " o t e n t i a l
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Peran bidan
• :00 icro(ramfolic acid daily
• -ontinue for 1st
three months of"re(nancy
%umil
• +etesi dini
• !erawatan ;cele9• u
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Hidrosefalus
7ydroce"halus is the buildu" of >uid in the cavities
ventricles6 dee" within the brain
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Hydrocephalus
-erebros"inal >uid normally >ows throu(h theventricles and bathes the brain and s"inalcolumn
The "ressure of too much cerebros"inal >uid
associated with hydroce"halus can damagebrain tissues and cause a lar(e s"ectrum ofimpairments in brain function
7ydroce"halus is caused by an imbalance
between how much cerebros"inal >uid is"roduced and how much is absorbed into thebloodstream.
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Excess cerebrospinal fluid in the ventriclesoccurs for one of the following reasons
O b s t r u c t i o n • T h e m o s t c o m m o n " r o b l e m i s a " a r t i a l
o b s t r u c t i o n o f t h e n o r m a l > o w o f c e r e b r o s " i n a l > u i d
P o o r a b s o r p t i o
n • 3 f t e n r e l a t e d t o i n > a m m a t i o n o f b r a i n t i s s u e f r o m d i s e a s e o r i n
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Risk factors in newborns
8bnormaldevelo"ment of the
-=$
%leedin(within theventricles
"remature6
Infection in
the uterusdurin( a
"re(nancy
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Symptoms in infants
Changes in the head Physical symptoms
8n unusually lar(e head
8 ra"id increase in the si?e of the head
8 bul(in( or tense soft s"ot fontanel6on the to" of the head
4omitin(
$lee"iness
Irritability
!oor feedin( $ei?ures
'yes &ed downward sunsettin( of the
eyes6
+e&cits in muscle tone and stren(th,res"onsiveness to touch, and e"ected
(rowth
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Penatalaksanaan
Umum
!en(awasan suhu atau "ence(ahan
hi"otermi.
!ence(ahan infesi.
3bservasi ativitas,reasi dan
ran(san(an,serta adanya dilatasi "u"ilstrabismus.
Intae*out"ut.
!erawatan sehabis %8@ dan %8%.
husus
!en(uuran lin(ar e"ala, catat danbuat (ra&nya
!en(awasan dan "ence(ahan muntah
!en(awasan e
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Peran bidan
!enatalasa
naan umum
!enatalasa
naan husus
u
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Gastroschizis & Omphalocele
Intestines herniate throu(h the abdominal wall
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(astroschisis om"halocele
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Omphalocele
!astroschisis
"ncidence
Covering
Sac
#ascial$efect
Cord%ttach&
'()*+++,'+*+++
Present -may be
ruptured.
Small to large
Umbilical the sac
'(/+*+++,0+*+++
%bsent
Small -vascularcompromise.
%bd 1all
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Omphalocele Gastroschisis
HerniatedBowel
Other organs
IUGR
NEC
Protected
Liver often in sac
Less common
If sac is rptred
Edematos andmatted
Remain in a!d"
Common
#$ %
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How will &o manage this'
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Delivery Room Management:Gastroschisis
8%-9s of resuscitation Barm, saline*soaed la" s"on(es, "lastic
wra" or bowel ba( to cover theintestines
+ecom"ression of the bowel 8$8!
8void volvulus of the mesenteric vessels
8void tearin( bowel mesentery or
causin( unnecessary dama(e to bowel emember im"ortance of
thermore(ulation and controllin( >uidlosses
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Anus imperforata
the o"enin( to the anus is missin( or bloced.
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Macam letak
T i n ( ( i • r e t u m b e r a h i r d i a t a s m . C e v a t o r a n i ,
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Gejala
obstrusiusus
a"ertura anal*6
eoniumeluar dari
suatu ori&sium
abnormal
untah Dabdomen
embun(
@esuaran
defeasi
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Manajemen
@olostomidisesi "osterosa(ital atau
"lasti anoretal"osterosa(ital
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Peran bidan
+etesidini
!emerisa
an &siscermatcolo
dubur E*6
u
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Atresia esofagus
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Manifestasi pada kelainan ini
• " r e n a t a l " o l i h i d r a m n i o n 6
• s a a t l a h i r b u s a " a d a s e t e r o r a l d i s e r t a i d e n ( a n t e r s e d a
d a n s i a n o s i s 6
• T e r d a " a t 5 t i " e a t r e s i a
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Tipe atresia esofagus
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Gejala
$alivasi dan droolin( berlebihan
Ti(a tanda utama traeoesofa(eal &stulaHbatu, terseda, sianosis
8"nea
enin(atnya distress "ernafasan setelahfeedin(
+istensi abdomen
@ebiruan "ada ulit sianosis6 etia diberimaan
%atu, (a((in(, terseda etia diberi maan
$ulit untu diberi maan
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Penatalaksanaan
Termore(ulasi
Cewatan selan( oro(astri dan lauanas"irasi antun( untu men(hindari "nemoniaas"irasi
Infus
cairan nutrisi "arenteral u
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Hirschsprung’s disease
elainan bawaan "enyebab (an((uan "asase usus,
dindin( usus yan( menyem"it tida ditemuan(an(lion "arasim"atis
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• 8 ( a n ( l i o n " l . 2 e i s s n e r d a n 8 u r b a c h d a l a m l a " i s a n d i n d i n ( u s u s
• m u l a i d a r i s " i n ( t e r a n i i n t e r n u s e a r a h " r o s i m a l
• J 0 # r e t o s i ( m o i d , 1 0 # s a m " a i s e l u r u h o l o n s e i t a r n y a , 5 #
s e l u r u h u s u s s a m " a i " i l o r u s
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Gejala
• t i n
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Komplikasi & manajemen
@ o m " l l i a s i
• ' n t e r o o l i t i s n e r o t i a n s , " n e u m a t o s i s u s u s , a b s e s " e r i o l o n ,
" e r f o r a s i d a n s e " t i e m i a
2 a n a
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Peran bidan
• eoneum *6hin((a 2: