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congenital malformations of the GI system Flashcards

1
Q

tracheoesophageal fistula and esophageal atresia clinical features

A

air in stomach
milk in lungs
polyhydraminos
vomiting

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2
Q

defects associated with esophageal atresia or tracheaesophageal fistula

A

VATER or VACTERL

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3
Q

narrowing of lumen of esophagus

A

congenital esophageal stenosis- usually mid esophagus

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4
Q

when is esophageal tenosis diagnoses

A

by 2 years usually

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5
Q

common presenting symptoms of esophageal stenosis

A

dysphagia, food impaction, respiratory symptoms

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6
Q

what may be confused with esophageal stenosis

A

eosinophilic esophagitis

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7
Q

double aortic arch can cause

A

vascular compression of esophagus

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8
Q

double aortic arch is associated with

A

Digeorge syndrome

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9
Q

aberrant right subclavian artery causes

A

compression of esophagus due to subclavian running behind esophagus

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10
Q

aberrant right subclavian may be seen when

A

Down syndrome
reduced BP in right upper extremity

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11
Q

achalasia is caused by

A

failure to relax the lower esophageal sphincter
disordered esophageal motility

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12
Q

achalasia causes

A

progressive dysphagia, regurgitation, chest pain, weight loss
increased risk for esophageal squamous cell carcinoma

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13
Q

diagnosis of achalasia

A

barium swallow “bird beak sign”

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14
Q

treatment of achalasia

A

surgery or botulinum toxin injection

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15
Q

what causes hypertrophic pyloric stenosis

A

when neural crest cells fail to migrate to pyloric muscle

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16
Q

when do hypertrophic pyloric stenosis symptoms occur and what causes it

A

2-8 weeks after birth seen with nonbilious vomiting
higher incidence in infants treated with erythromycin

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17
Q

causes of duodenal atresia

A

intrinsic- failed recanalization
extrinsic compression

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18
Q

signs of duodenal atresia

A

bilious vomiting without abdominal distention
polyhydraminios
“double bubble sign”

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19
Q

biliary atresia

A

obliteration of intrahepatic ducts or extra hepatic ducts due to failed recanalization

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20
Q

symptoms of biliary atresia

A

progressive neonatal jaundice
white clay colored stool
dark urine
100% mortality

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21
Q

patent accessory pancreatic duct

A

not an abnormality

22
Q

pancreatic divisum

A

patients prone to pancreatitis

23
Q

annular pancreas

A

duodenal obstruction or stenosis caused by pancreas wrapping around duodenum

24
Q

signs of annular pancreas

A

bilious vomiting if annulus is inferior to bile duct

25
Q

cystic fibrosis of the pancreas

A

inflamed pancreas due to digestive enzymes not being flushed from pancreatic duct

26
Q

severity of the CFTR mutations is graded by

A

their effect on the pancreas

27
Q

meconium ileum at birth is seen in what disorder

A

cystic fibrosis

28
Q

omphalocele will have

A

persistent herniation through umbilicus
peritoneal covering
trisomy 13 or 18
bowel encased within umbilicus, will appear relatively normal

29
Q

omphacele cause

A

failed lateral body folding or inability of body wall to form normally

30
Q

gastroschisis

A

midgut herniated through abdominal wall
no peritoneal covering
no known chromosomal abnormalities

31
Q

cause of gastroschisis

A

premature recession of right umbilical vein

32
Q

signs of gastroschisis

A

second or third trimester ultrasound and elevated alpha-fetoprotein

33
Q

umbilical hernia

A

segment of midgut herniated through umbilicus
- skin covers herniation

34
Q

meckels diverticulum

A

persistence of vitelline duct

35
Q

problems caused by meckels diverticulum

A

risk of volvulus
risk of intussusception

36
Q

rule of 2s for mockers diverticulum

A

2% of incidence
2 times more common in. males
2 percent of symptoms
2 years of age
2 inches long

37
Q

non rotation of midgut loop

A

2nd and 3rd rotations fail to occur (retraction phase)

38
Q

non rotation of midgut loop causes

A

left sided colon and increased risk of volvulus

39
Q

reversed rotation of midgut loop

A

only second and 3rd rotations are reversed
transverse colon is secondarily retroperitoneal
duodenum can dress against transverse colon and cause blockage

40
Q

volvulus causes

A

malrotation of gut
ladd’s bands

41
Q

effects of volvulus

A

intestinal ischemia or necrotic or ischemic bowel
vomiting, GI bleed, acute abdominal pain

42
Q

intestinal atresia or stenosis

A

due to failed recanalization of midgut or vascular accidents (section does not get enough perfusion)

43
Q

sources of diaphragm

A

septum transversum
pleuroperitoneal membrane
mesoderm of body wall
esophageal mesenchyme

44
Q

congenital diaphragmatic hernia is seen where

A

commonly on left side

45
Q

types of congenital diaphragmatic hernias

A

bochdalek hernias: worse hernias
Morgan hernias: less common

46
Q

effects of congenital diaphragmatic hernia

A

pulmonary hypoplasia

47
Q

intussusception causes

A

most commonly idiopathic
in children: lymphoid hyperplasia
in adults: tumors are the most common

48
Q

common cause of bowel obstruction in children under 2

A

intussusception

49
Q

abnormal formation of the urogenital septum

A

fistula causing incomplete separation of urogenital sinus and anorectal canal

50
Q

imperforate anus

A

persistent anal membrane commonly seen in VACTERL and Down syndrome and fistula

51
Q

classifications of imperforate anus

A

low, intermediate, high

52
Q

Hirschsprungs disease

A

absence of ganglionic plexus due to absence of caudal migration of vagal neural crest cells (primary)
- colonic distention (megacolon)
associated with trisomy 21

gi (7 decks)

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