Ch. 62 - Fetal Anterior Abdominal Wall
an omphalocele herniation is covered by a membrane made of what 2 things ?
-amnion -peritoneum
what are the 2 possible causes for gastroschisis ?
-atrophy of the right umbilical vein -disruption of the omphalomesenteric artery
what 2 things are evaluated if there is bladder and/or cloacal exstrophy ?
-bladder -pelvis
what two things is it important to image when looking for abdominal wall defects ?
-cord insertion -anterior abdominal wall
with gastroschisis notably dilated bowel may suggest what 2 things ?
-infarction -bowel atresia
what 4 things can bladder exstrophy be accompanied by ?
-omphalocele -inguinal hernia -undescended testes -anal problems
what are the 3 most common abdominal wall defects ?
-omphalocele -umbilical hernia -gastroschisis
what 3 things are needed to have Beckwith Wiedemann Syndrome ?
-omphalocele -visceromegaly (organomegaly) -macroglossia
hydramnios is found in ______ of fetuses with an omphalocele
1/3
an omphalocele is most commonly associated with what trisomy(s) ?
13 & 18
it is considered bladder exstrophy if the normal bladder is not visible after ____ minutes
30
what week does folding happen ?
6
-umbilical vein drains the placenta, body stalk, and evolving abdominal wall -hepatic bud enlarges -right umbilical vein atrophies -SMA is formed from the right omphalomesenteric artery this fusion of the midline happens in what week ?
7
midgut herniation is normale between ____ to ____ weeks
8, 12
perinatal mortality rate approaches _____% with omphalocele when more than one fetal abnormality exists and almost all die when there is a chromosomal or major heart defect
80
-rare and occurs early in development with involvement of primitive gut -defect in lower abdominal wall and anterior wall of urinary bladder
Cloacal Exstrophy
also known as OEIS complex
Cloacal Exstrophy
defect in lower abdominal wall and anterior wall of urinary bladder
Cloacal Exstrophy
another name for trisomy 18
Edwards syndrome
Cloacal Exstrophy is also known as ?
OEIS complex
another name for trisomy 13
Patau syndrome
what is formed from the right omphalomesenteric ?
SMA
1. is a limiting membrane present? 2. what is the relation of the umbilical cord to the defect? 3. which organs are eviscerated? 4. is the bowel in normal appearance? 5. are other fetal malformations evident? questions that should be asked when evaluating what ?
abdominal wall
folding is a critical process in closing the ?
abdominal wall
amniotic band syndrome is when the ? ruptures
amnion
associated with anomalies of the limbs, cranium, face, thorax, spine, abdominal wall, and peritoneum
amniotic band syndrome
entrapment or entanglement of the fetal parts by the "sticky" chorion
amniotic band syndrome
rare, occurs in 7.8 per 10,000 what syndrome ?
amniotic band syndrome
what is the primary ultrasound finding of cloacal exstrophy ?
anterior abdominal wall defect
severe body wall defects may be seen in gastroschisis with secondary _______ ________
band formation
characterized by a defect in the lower abdominal wall and anterior wall of the urinary bladder
bladder exstrophy
may be accompanied by an omphalocele, inguinal hernia, undescended testes, and anal problems
bladder exstrophy
an omphalocele that contains only ________ have a higher risk for chromosomal anomalies and other anomalies
bowel
it is not good when the baby comes in contact with the AMNION or CHORION ?
chorion
omphaloceles that contain only bowel have a higher risk for ?
chromosomal anomalies
-rare and more complex than bladder exstrophy -occur early in development
cloacal exstrophy
results in exstrophy of the bladder in which two hemi-bladders are separated by internal mucosa
cloacal exstrophy
in an absent sternum is the heart COVERED or NOT COVERED by skin ?
covered
an omphalocele may occur concurrently with ?
diaphragmatic hernia
EARLY or LATE entrapment by the amniotic bands may lead to severe craniofacial defects and internal malformations
early
evisceration of the heart is called ?
ectopia cordis
Cloacal Exstrophy is also known as OEIS complex what does the E stand for ?
exstrophy
helps embryo to transform itself into a cylindrical shape what process ?
folding
transformation that is critical part in the closing of the abdominal wall
folding
-atrophy of the right umbilical vein -disruption of the omphalomesenteric artery causes for ?
gastroschisis
-herniated bowel may be mildly dilated with a bowel wall thickening -notably dilated bowel may suggest infarction or bowel atresia OMPHALOCELE or GASTROSCHISIS
gastroschisis
-hydronephrosis -bladder deviation -exstrophy OMPHALOCELE or GASTROSCHISIS
gastroschisis
associated with extremely elevated AFP levels OMPHALOCELE or GASTROSCHISIS
gastroschisis
coexisting anomalies are rare OMPHALOCELE or GASTROSCHISIS
gastroschisis
found to the right of the cord OMPHALOCELE or GASTROSCHISIS
gastroschisis
insertion of the umbilical cord is normal OMPHALOCELE or GASTROSCHISIS
gastroschisis
right paraumbilical defect of abdominal wall, rarely a left-side defect
gastroschisis
survival rate is excellent if uncomplicated (preterm delivery may be done to monitor fetus) OMPHALOCELE or GASTROSCHISIS
gastroschisis
the eviscerated bowel is not covered by a membrane OMPHALOCELE or GASTROSCHISIS
gastroschisis
thought to be a consequence of the atrophy of the right umbilical vein or a disruption of the omphalomesenteric artery
gastroschisis
with gastroschisis the survival rate is GOOD or BAD if uncomplicated
good
½ bladder; developmental anomaly in which the bladder is formed as two
hemi-bladder
normal cord insertion HERNIA or OMPHALOCELE
hernia
umbilical arteries are in a normal location HERNIA or OMPHALOCELE
hernia
-pentalogy of Cantrell -diaphragmatic hernia -ectopia cordis (evisceration of heart) -other heart defects HIGH (SUPRAUMBILICAL) or LOW omphalocele
high (supraumbilical)
primary finding of Pentalogy of Cantrell ?
high or super-umbilical omphalocele
Cloacal Exstrophy is also known as OEIS complex what does the I stand for ?
imperforate anus
EARLY or LATE entrapment by the amniotic bands leads to amputations or limb restrictions
late
when an omphalocele and scoliosis are found together what should be considered ?
limb-body wall (body stalk complex)
-bladder or cloacal exstrophy -anal atresia -spina bifida -lower limb defects HIGH (SUPRAUMBILICAL) or LOW omphalocele
low
bladder exstrophy is characterized by a defect in the UPPER or LOWER abdominal wall and anterior wall of the urinary bladder
lower
gastroschisis is more common in MALES or FEMALES
males
permits development of intra-abdominal organs
midgut herniation
does gastroschisis have a membrane ?
no
with bladder exstrophy is the normal bladder visible ?
no
an umbilical hernia will have a NORMAL or ABNORMAL cord insertion
normal
in an ectopia cordis is the heart COVERED or NOT COVERED by skin ?
not covered
2 types: -contain liver within the sac (may or may not contain bowel) -contain variable amount of bowel without liver
omphalocele
AFP slightly elevated or normal OMPHALOCELE or GASTROSCHISIS
omphalocele
Cloacal Exstrophy is also known as OEIS complex what does the O stand for ?
omphalocele
associated with chromosomal anomalies, most common trisomy 13 & 18 OMPHALOCELE or GASTROSCHISIS
omphalocele
central abdominal wall defect with evisceration of the bowel or a combination of liver and bowel into the base of the umbilical cord
omphalocele
cord goes through OMPHALOCELE or GASTROSCHISIS
omphalocele
develops where there is a midline defect of the abdominal muscles, fascia, and skin that results in herniation of the intraabdominal structures into the base of the umbilical cord
omphalocele
may occur concurrently with diaphragmatic hernia OMPHALOCELE or GASTROSCHISIS
omphalocele
often occur with chromosomal anomalies OMPHALOCELE or GASTROSCHISIS
omphalocele
prognosis for ? varies according to the extent of the primary defect and associated structural and chromosomal abnormalities
omphalocele
the umbilical hernia is a form of a ?
omphalocele
when scoliosis is found with ? consider limb-body wall complex
omphalocele
what 3 things does the umbilical vein drain ?
placenta, body stalk, and evolving abdominal wall
-ectopia cordis -pentalogy of Cantrell -limb-body wall complex -amnion rupture sequence -bladder and cloacal exstrophy are all COMMON or RARE abdominal wall defects ?
rare
cloacal exstrophy is more COMMON or RARE than bladder exstrophy
rare
is Pentalogy of Cantrell COMMON or RARE ?
rare
with gastroschisis the defect is located to the RIGHT ot LEFT of the umbilical cord ?
right
the SMA is formed from the ? artery
right omphalomesenteric
when ? is found with an omphalocele consider limb-body wall complex
scoliosis
gastroschisis always has free floating SMALL or LARGE bowel
small
what is always found in a gastroschisis herniation ?
small bowel
with bladder exstrophy a _______ __________ mass, representing the exposed bladder mucosa, may be seen on the surface of the lower abdominal wall
soft tissue
Cloacal Exstrophy is also known as OEIS complex what does the S stand for ?
spina bifida
ascites may occur with an omphalocele TRUE or FALSE
true
bladder exstrophy can be mild or severe TRUE or FALSE
true
what drains the placenta, body stalk, and evolving abdominal wall ?
umbilical vein
abdominal wall defects cause distortion of the normal contour of the _________ or __________ surface of the fetal abdomen
ventral, anterior
is there a membrane covering an omphalocele ?
yes
