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OB2
Two MOST COMMON abdominal wall defects
omphalocele
gastroschisis
When does fusion of the midline occur?
during the seventh week and is completed by the eighth week
When should the midgut herniation be completed?
by the end of the 12th week gestation
The midgut herniation is primordium of
small intestines
cecum
vermiform appendix
ascending colon
right half to two thirds of the transverse colon
Important to image to evaluate the following for presence of defects
cord insertion
fetal anterior abdominal wall
bladder and cloacal exstrophy
Omphalocele
when bowel loops fail to return to the abdomen from the midgut herniation
AFP slightly elevated
encased in a membrane consisting of peritoneum and amnion
2 types of omphaloceles
those that contain liver within the sac
those that contain a variable amount of bowerl but no liver
Omphaloceles that contain bowel only have a higher risk for
chromosomal abnormalities (35-65%)
other anomalies
Anomalies associated with omphaloceles
complex cardiac disease
gastrointestinal
neural tube
genitourinary tract anomalies
Limb-body wall complex
lethal disorder
acrania
encephalocele
facial clefts
extensive abdominal wall defect of the chest
abdomen and limb defects
Amniotic band syndrome
abnormal fusion of the amnion and chorion extends as a sheet from the cord and adheres to the fetus and the placenta
entangles or amputates fetal parts
Common findings with amniotic band syndrome
facial clefts
asymmetric encephaloceles
constriction or amputation defects of the extremities
clubfoot deformities
Bladder exstrophy
bladder outside of the pelvis
Anomalies associated with bladder exstrophy
anal atresia
spina bifida
lower limb defects
Pentalogy of Cantrell
large omphalocele
sternal cleft
anterior diaphragmatic hernia
ectopia cordis
defects of the diaphragmatic pericardium
Pentalogy of Cantrell may be associated with
cardiac defects
cleft lip/ palate
encephalocele
exencephaly
sirenomelia
trisomies 13, 18 and 45X
Beckwith-Wiedemann syndrome is suspected if these are found
organomegly
macroglossia
macrosomia
omphalocele
embryonic tumors
neonatal hypoglycemia
ear creases
Perinatal mortality approaches ____% when more than one fetal anomaly exists
80
Mortality rate of an omphalocele when it is isolated
10%
Gastroschisis
opening in the layers of the abdominal wall with evisceration of the bowel
A gastroschisis is almost ALWAYS to the _______ of the umbilical cord insertion
right
A gastroschisis is thought to be the consequence of:
atrophy of the right umbilical vein
disruption of the omphalomesenteric artery
Gastroschisis knows:
young mothers
males more than females
poor prognosis
higher AFP
only detected after 12 weeks
Gastroschisis sono findings
right paraumbilical defect of the abdominal wall
free-floating herniated small bowel
large bowel, stomach, gallbladder, urinary bladder, pelvic organs may be involved
bowel wall thickening
hydronephrosis
Common defects associated with amniotic band syndrome
limbs
cranium
face
thorax
abdominal wall
perineum
Bladder exstrophy
bladder becomes exposed on the lower abdominal wall
Cloacal exstrophy
results in exstrophy of the bladder which two hemi-bladders are separated by intestinal mucosa
Cloacal
becomes the rectum, urethra, and vagina
if misforms causes a split penis in boys
Ectopic cordis
exposed heart presents outside the chest wall through a cleft in the sternum
heart may present outside the thoracic cavity when a portion of all of the heart protrudes through a defect in the sternum
Ectopic cordis is associated with
omphalocele
cardiovascular malformations
craniofacial defects
Cleft sternum
may be either partial or absent without ectopia cordis and is typically a superior or total cleft
Associated with vascular malformations, including cavernous hemangiomas and omphaloceles
This side is MORE COMMON with a limb body wall complex
left side is 3x more common