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