Ch. 62 Flashcards

1
Q

Abdominal wall defects occur during

A

The 1st trimester

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2
Q

The midgut usually returns into the abdominal cavity by week

A

11-12

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3
Q

When midgut fails to return to abdominal cavity __ is formed

A

abdominal wall defect

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4
Q

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

A

a cylindrical shape

closing the abdominal wall

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5
Q

Most common types of abdominal wall defect

A

Gastroschisis
Omphalocele
Umbilical hernia

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6
Q

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

A

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

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7
Q

Omphalocele

A

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

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8
Q

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

A

Midline defect

herniation of intraabdominal structures into the base of the umbilical cord

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9
Q

With omphalocele, AFP may be

A

slightly elevated or normal

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10
Q

2 types of omphalocele

A
  1. contains liver in the sac

2. contains bowel without liver

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11
Q

Bowel omphalocele has a higher risk for __abnormalities & other anomalies

A

Chromosomal

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12
Q

Bowel omphalocceles develop b/c

A

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

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13
Q

Liver omphalocele represents

A

a developmental defect in abdominal wall closure

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14
Q

Liver omphalocele affects the

A

abdominal wall muscles, fascia, and skin

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15
Q

Liver omphaloceles may contain _, and demonstrate

A

bowel

relatively large abdominal wall defect

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16
Q

Omphalocele prognosis varies depending on

A

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

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17
Q

Fetal mortality of omphalocele __ when more than 1 abnrmality exists

A

increases to 80%

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18
Q

Mortality rate of omphalocele is __ with isolated omphalocele

A

10%

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19
Q

With omphalocele, mode of delivery varies depending on

A

the type of omphalocele and other anomalies

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20
Q

Sono appearance of omphalocele

A

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

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21
Q

Anomalies assoc. w/ omphalocele

A

Complex cardiac disease
GI
Neural tube defects
Genitourinary tract anomalies

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22
Q

Gastroschisis

A

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

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23
Q

Gastroschisis is almost always located

A

to the right of the umbilicus

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24
Q

It is thought that gastroschisis is a consequence o

A

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

25
Q

Is gastroschisis know to be genetically transmitted

A

No

26
Q

With gastroschisis, defects are _ in size. Located _

A

Small, 2-4cm

Next to normal cord insertion (right side)

27
Q

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

A

small bowel

large bowel
stomach
portions of the genitourinary system
liver
GB
bladder
pelvic organs
28
Q

AFP levels with gastroschisis

A

signifiantly higher than w/ omphalocele because it lacks a membrane

29
Q

Gastroschisis is more common in

A

Males

30
Q

Prognosis w/ uncomplicated gastroschisis

A

excellent, surgery occurs hours after delivery

31
Q

With gastroschisis, able to detect after __ weeks gestation

A

12

32
Q

With gastroschisis, herniated bowel may be mildly

A

dilated with bowel wall thickening

Dilation may be seen in herniated portions of bowel &/or within fetal abdominal cavity

33
Q

With gastroschisis, notably dilated bowel may suggest

A

infarction or bowel atresia

34
Q

With gastroschisis, _ may be obsesrved

A

Hydronephrosis, bladder deviation & exstrophy

35
Q

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

A

amniotic band syndrome amputations

36
Q

Omphalocele is associated with

A

chromosomal abnormalities

37
Q

Amniotic band syndrome

A

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

38
Q

Beckwith-Wiedemann syndrom

A

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

39
Q

Visceromegaly

A

enlargement of the organs inside the abdomen

40
Q

Beckwith-Wiedemann is characterized by (8)

A
▪Macrosomia
▪Macroglossia
▪Visceromegaly
▪Embryonic tumors
▪Omphalocele
▪Neonatal hypoglycemia
▪Ear creases
▪Polyhydramnios [3rdtrimester]
41
Q

Bladder exstrophy

A

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

42
Q

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

A

everted

43
Q

Bladder exstrophy may be

A
mild or severe
accompanied by
omphalocele
inguinal hernia
cryptorchidism
anal problems
genital malformation
44
Q

Sono appearance of bladder exstrophy

A

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

45
Q

Cloacal estrophy

A
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
46
Q

cloacal exstrophy occurs

A

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

47
Q

cloacal exstrophy results in

A

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

48
Q

Sono appearance of cloacal exstrophy

A

Primary finding is anterior abdominal wall defect

abnormally low cord insert

49
Q

Pentalogy of Cantrel

A
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
50
Q

With pentalogy of cantrell, _ is usually the primary finding

A

high/superumbilical omphalocele

pericardial effusion may be present

51
Q

Pentalogy of cantrell is assoc. w/

A
▪Cardiac defects [ectopic cordis]
▪Cleft lip/palate
▪Encephalocele
▪Exencephaly
▪Sirenomelia
▪Cystic hygroma [1sttrimester]▪Trisomy 13, 18 & 45X
52
Q

ectopic cordis

A

Ectopic heart

53
Q

with ectopic cordis, exposed heart presents

A

outside of the chest all through a cleft sternum

54
Q

Ectopic cordis is assoc. w.

A

▪Omphalocele
▪Cardiovascular malformations
▪Craniofacial defect

55
Q

Limb-Body-Wall Complex assoc. w/

A

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

56
Q

limb-body-wall complex occurs

A

with fusion of amnion & chorion

57
Q

with l-b-w complex, amnion does not

A

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

58
Q

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

A

3

59
Q

Sono: L-B-W complex

A

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