Ch. 62

Question 1

Abdominal wall defects occur during

Answer 1

The 1st trimester

Question 2

The midgut usually returns into the abdominal cavity by week

Answer 2

11-12

Question 3

When midgut fails to return to abdominal cavity __ is formed

Answer 3

abdominal wall defect

Question 4

Folding helps the embryo transform itself into __, this is a critical part of the process of _

Answer 4

a cylindrical shape

closing the abdominal wall

Question 5

Most common types of abdominal wall defect

Answer 5

Gastroschisis
Omphalocele
Umbilical hernia

Question 6

Abdominal defects other than gastrosch, omphal, and umb. hernia

Answer 6

Ectopia cordis
Bladder & cloacal exstrophy
Amniotic band syndrome
Limb-body-wall complex

Question 7

Omphalocele

Answer 7

Herniated loops of bowel normally return and rotate into position within abdominal cavity by 12th week of pregnancy. When bowel loops fail to return to abdomen, bowel-containing omphalocele occur

Question 8

An omphalocele develops when there is _ of the abdominal muscles, fascia, and skin that results in __

Answer 8

Midline defect

herniation of intraabdominal structures into the base of the umbilical cord

Question 9

With omphalocele, AFP may be

Answer 9

slightly elevated or normal

Question 10

2 types of omphalocele

Answer 10

1. contains liver in the sac

2. contains bowel without liver

Question 11

Bowel omphalocele has a higher risk for __abnormalities & other anomalies

Answer 11

Chromosomal

Question 12

Bowel omphalocceles develop b/c

Answer 12

the intestines fail to return to the abdomen (primitive stalk remains)

Question 13

Liver omphalocele represents

Answer 13

a developmental defect in abdominal wall closure

Question 14

Liver omphalocele affects the

Answer 14

abdominal wall muscles, fascia, and skin

Question 15

Liver omphaloceles may contain _, and demonstrate

Answer 15

bowel

relatively large abdominal wall defect

Question 16

Omphalocele prognosis varies depending on

Answer 16

extent of the primary defect and assoc. structural & chromosomal abnormalities

Question 17

Fetal mortality of omphalocele __ when more than 1 abnrmality exists

Answer 17

increases to 80%

Question 18

Mortality rate of omphalocele is __ with isolated omphalocele

Answer 18

10%

Question 19

With omphalocele, mode of delivery varies depending on

Answer 19

the type of omphalocele and other anomalies

Question 20

Sono appearance of omphalocele

Answer 20

Central abdominal wall defect with evisceration of bowel or combination of liver into base of umbilical cord.
Stomach may be involved.
Bowel omphaloceles appear echogenic & must be distinguished from umbilical hernia (normal cord insert suggests umbilical hernia).
Ascites may be seen.
Polyhydramnios is common
Diaphragmatic hernias may be present

Question 21

Anomalies assoc. w/ omphalocele

Answer 21

Complex cardiac disease
GI
Neural tube defects
Genitourinary tract anomalies

Question 22

Gastroschisis

Answer 22

Periumbilical defect
Opening in the layers of the abdominal wall with herniation of bowel.
Infrequently involves the stomach & genitourinary organs.
Rarely the liver

Question 23

Gastroschisis is almost always located

Answer 23

to the right of the umbilicus

Question 24

It is thought that gastroschisis is a consequence o

Answer 24

atrophy of the right umbilical vein or a disruption of the omphalomesentric artery.
Vascular accident

Question 25

Is gastroschisis know to be genetically transmitted

Answer 25

No

Question 26

With gastroschisis, defects are _ in size. Located _

Answer 26

Small, 2-4cm | Next to normal cord insertion (right side)

Question 27

With gastroschisis, _ is always found in the herniation. Other organs that may be involved:

Answer 27

small bowel ``` large bowel stomach portions of the genitourinary system liver GB bladder pelvic organs ```

Question 28

AFP levels with gastroschisis

Answer 28

signifiantly higher than w/ omphalocele because it lacks a membrane

Question 29

Gastroschisis is more common in

Answer 29

Males

Question 30

Prognosis w/ uncomplicated gastroschisis

Answer 30

excellent, surgery occurs hours after delivery

Question 31

With gastroschisis, able to detect after __ weeks gestation

Answer 31

12

Question 32

With gastroschisis, herniated bowel may be mildly

Answer 32

dilated with bowel wall thickening | Dilation may be seen in herniated portions of bowel &/or within fetal abdominal cavity

Question 33

With gastroschisis, notably dilated bowel may suggest

Answer 33

infarction or bowel atresia

Question 34

With gastroschisis, _ may be obsesrved

Answer 34

Hydronephrosis, bladder deviation & exstrophy

Question 35

W/ gastroschisi, consider _ when clefting of face &/or encephalocele is found

Answer 35

amniotic band syndrome amputations

Question 36

Omphalocele is associated with

Answer 36

chromosomal abnormalities

Question 37

Amniotic band syndrome

Answer 37

Rupture of the amnion which leads to the entrapment / entanglement of fetal parts

Question 38

Beckwith-Wiedemann syndrom

Answer 38

Rare sporadic group of disorders all having in common the coexistence of Omphalocele macroglossia visceromegaly

Question 39

Visceromegaly

Answer 39

enlargement of the organs inside the abdomen

Question 40

Beckwith-Wiedemann is characterized by (8)

Answer 40

``` ▪Macrosomia ▪Macroglossia ▪Visceromegaly ▪Embryonic tumors ▪Omphalocele ▪Neonatal hypoglycemia ▪Ear creases ▪Polyhydramnios [3rdtrimester] ```

Question 41

Bladder exstrophy

Answer 41

Defect in the lower abdoinal wall & anterior wall of the urinary bladder. Protrusion of the bladder through the abdominal wall defect

Question 42

With bladder exstrophy, __ bladder becomes exposed on the lower abdominal wall

Answer 42

everted

Question 43

Bladder exstrophy may be

Answer 43

``` mild or severe accompanied by omphalocele inguinal hernia cryptorchidism anal problems genital malformation ```

Question 44

Sono appearance of bladder exstrophy

Answer 44

Normal urinary bladder is not visible •Confluence of the rectum, vagina & urethra ▪A soft tissue mass that represents the exposed bladder mucosa may be seen on the surface of the lower abdominal wall

Question 45

Cloacal estrophy

Answer 45

``` Rare, complex malformation involving ▪Lower limb amnormalities ▪Spinal defects ▪Anal atresia ▪Lower abdominal wall defect below the cord insertion including exstrophy of the bladder and protrusion of intestines ```

Question 46

cloacal exstrophy occurs

Answer 46

early in development w/ involvement of primitive gut & persistent cloaca

Question 47

cloacal exstrophy results in

Answer 47

exstrophy of the bladder in which two hemibladders are separated by intestinal mucosa

Question 48

Sono appearance of cloacal exstrophy

Answer 48

Primary finding is anterior abdominal wall defect | abnormally low cord insert

Question 49

Pentalogy of Cantrel

Answer 49

``` Rare Is the association of these 5 defects: ▪cleft in distal sternum ▪Diaphragmatic defect ▪Midline anterior ventral defect ▪Defect of apical pericardium with communication into peritoneum ▪Internal cardiac defect ```

Question 50

With pentalogy of cantrell, _ is usually the primary finding

Answer 50

high/superumbilical omphalocele pericardial effusion may be present

Question 51

Pentalogy of cantrell is assoc. w/

Answer 51

``` ▪Cardiac defects [ectopic cordis] ▪Cleft lip/palate ▪Encephalocele ▪Exencephaly ▪Sirenomelia ▪Cystic hygroma [1sttrimester]▪Trisomy 13, 18 & 45X ```

Question 52

ectopic cordis

Answer 52

Ectopic heart

Question 53

with ectopic cordis, exposed heart presents

Answer 53

outside of the chest all through a cleft sternum

Question 54

Ectopic cordis is assoc. w.

Answer 54

▪Omphalocele ▪Cardiovascular malformations ▪Craniofacial defect

Question 55

Limb-Body-Wall Complex assoc. w/

Answer 55

▪Large cranial defects ▪facial cleft ▪Body wall complex defects involving thorax ▪Body wall complex defects involving abdomen ▪Limb defects ▪Scoliosis ▪Various internal malformation

Question 56

limb-body-wall complex occurs

Answer 56

with fusion of amnion & chorion

Question 57

with l-b-w complex, amnion does not

Answer 57

cover umbilical cord normally, but extends as sheet from the margin of the cord and is continuous with both the body wall & placenta

Question 58

With L-B-W complex, left-sided defects are __ times more common than right-sided

Answer 58

3

Question 59

Sono: L-B-W complex

Answer 59

large involve abdomen and thorax Eviscerated organs from complex, bizarre appearing mass entangled with membranes Umbilical cord is short & adherent to placental membranes absent