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what helps the embryo transform into a cylindrical shape
folding (aids in closing ab wall)
When does folding happen
6th week
what are the three layers of embryonic disk at the fifth week
mesoderm, ectoderm, endoderm
when does fusion of the midline begin and end
begins 7th week, ends 8th week
why do the intestines herniate into umbliic
because the midgut grows faster than the ab cavity
what is the incidence of omphaloceles
1:400 live births
what is the incidence of gastroschisis
12 per 10:000
what causes a bowel containing ompahlocele
when hernated bowel does not return to abdomen
what are two types of omphaloceles
bowel and liver
will omphalocele or gastroschisis have high AFP
gastroschisis (no membrane)
will bowel or liver gastro have higher risk of anomlies
bowel
what is mortality rate when more than one fetal abnormality exists w/omphalocele
80%
what is mortality rate of omphalocele without other anomalies
10%
what % is risk of recurrence of gastroschisis
3-5%
sono of omphalocele
echogenic, central ab wall defect, evisceration of bowel and/or liver into umbilical cord
what can help distinguish umbilical hernias from liver omphalocele
a normal cord insertion suggests hernia
what may coexist with omphalocele
ascites and hydramnios
what other issues may be associated w/omphalocele
aneuploidy, cardiac, GI, genitourinary, neural tube
what are most common tirsomies associated w/omphaloceles
13 and 18
what umbilical cord finding can be used to differentiate omphalocele from gastroschisis
cord goes through an ompalocele, gastroschisis is to right of cord
what ab wall abnormality should be considered if there is scoliosis and/or cranial defects
limb body wall complex
what ab wall abnormality should be considered if there is amniotic bands
amniotic band syndrome (milder limb body wall complex)
what ab wall abnormality should be considered if there is large omphalocele, diaphragmatic hernia and pericardium, ectopia cordis, sternal cleft
Pentalogy of Cantrell
what ab wall abnormality should be considered if there is a low omphalocele
bladder or cloacal exstrophy
what ab wall abnormality should be considered if there is organomegaly and macroglossia
beckwith-weidemann syndrome
what is gastroschisis
periumbilical defect to right of umbilicus
what is occurrence of gastroschisis
1.75-2.5 : 10:000 live births
Gastroschisis is more common in M or F
M
at what week can gastro be detected by US
12 gestation weeks
sono of gastro
free floating bowel outside ab cavity in area of cord (to right)
what is amniotic band syndrome
rupture of amnion entrapts fetal parts by “sticky” chorion
what are common findings with amniotic band syndrome
facial clefts, asymmetric encephaloceles, clubfoot
what is occurance of amniotic band syndrome
7.8 per 10:000
what do Bechwith-Wiedemann Syndromes have in common
omphalocele, macrglossia, visceromegaly
what is cloacal exstrophy
involves primitive gut and persistent cloaca
what are signs of pentalogy of cantrell
omphalocele, sternal cleft, anterior diaphragmatic hernia, ectopia cordis, defects of diaphragmatic pericardium
what is ectopia cordis
heart on outside of chest through cleft sternum
what are signs of beckwith-wiedemann syndrome
organomegaly, macroglossia, macrosomia, omphalocele
what rare defect occurs early in primitive gut and results in exstrophy of bladder that has two hemi-bladders
cloacal exstrophy
what is usually primary finding w/pentalogy of cantrell
high or superumbilical omphalocele
what’s another name for Turner’s syndrome
45X
what anomolies are most common w/ectopia cordis
omphalocele, cardiovascular malformations, craniofacial defects
what side is limb body wall complex more common
left - 3x more common
how do limb body wall defects happen
when the amnion and chorion fuse and the amnion doesn’t cover the UC normally but extends as a sheet that is continuous w/both body wall and placenta
sono findings of limb body wall defect
large ab and thorax defects, eviscerated organs, bizarre mass entagled w/membranes, short cord adhered to placental membranes
what trisomies are associated w/pentalogy of cantrell
13, 18, 45x