012315 congenital defects of bowel Flashcards Preview

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Flashcards in 012315 congenital defects of bowel Deck (29):
1

esophageal atresia

the esophagus has a dead end
can have fistulas to the trachea

more than 50% association w other major congenital anomalies (VACTERL)--vertebral, anorectal, cardiac, tracheoesophageal fistula, renal, limb

2

when should bilirubin begin to decrease in newborns?

by day 7

3

when is jaundice abnormal in newborn?

beyond 2-3 weeks of age
direct bilirubin over 2
total bilirubin over 12 (term)
total bilirubin over 14 (preterm)

4

what is a possible cause of direct hyperbilirubinemia in infant?

biliary atresia

5

possible causes of neonatal bile duct obstruction

biliary atresia
choledochal cyst
others (rare)

6

when does biliary atresia typically present?

4-6 wks

7

what can biliary atresia cause in the end?

cirrhosis

8

pathogenesis of biliary atresia

likely an in utero biliary epithelial injury leading to inflam
etiology unknown

9

in biliary atresia, bile duct becomes

fibrotic

10

choledochal cyst

dilatation of bile duct

11

pathogenesis of choledochal cyst

-disordered organogenesis
-pancreaticobiliary malunion
-acquired weakness
-distal obstruction

12

when does choledochal cyst usually present

over 1 year old (75%)

13

bilious (any shade of yellow or green) emesis

surgical emergency

14

developmental defect in malrotation

narrow mesenteric base permits abnorma mobility of the small bowel, allowing the mesentery to twist (midgut volvulus and entire midgut can die)

Ladd's bands (attach cecum to abd wall), which cross the duodenum, can lead to obstruction

15

diagnostic test for malrotation

upper GI test used to look for location of ligament of treitz (duodenojejunal jxn), which should be to L of spine

16

double bubble

duodenal atresia

17

Meckel's diverticulum

TRUE diverticulum of the small bowel

results from failure of involution of the vitelline duct, the embryologic connection btwn lumen of the deveoping intestine and the yolk sac

18

rule of 2's for Meckel's diverticulum

2 types of abnormal lining in it (stomach and pancreas)

usually symptomatic by 2 years of age

19

symptoms of Meckel's diverticulum

bleeding into the intestine
blockage of the intestine
inflammation

20

massively dilated colon

Hischsprung's disease

21

Hirschsprung's disease

congenital defect in intestinal innervation
can be associated w Trisomy 21

normal migration of neural crest cells from small intestine is disrupted, so a segment of distal colon exists w/o nerve cells (both Meissner and Auerbach's)

most common in rectosigmoid, but can be entire colon

22

RET mutations

commonly occur in Hirschprung's disease
RET is a tyrosine kinase that transduces growth for tissues including neural crest cells

23

clinical presentation of Hirschprung's dis

failure to pass meconium
functional obstruction/bilious emesis
enterocolitis (stasis of stool leading to bacterial overgrowth/sepsis)

24

if feeding fine before but has problem now, think

necrotizing enterocolitis or
sepsis w/ ileus

25

necrotizing entercolitis is most common in

premature infants

26

pathophysiology of necrotizing enterocolitis

not well known

prematurity--possibly abnormal bacteria in gut
dysbiosis
formula feeding
impaired mucosal defense (b/c premature)

27

end result of necrotizing enterocolitis

ischemia, necrosis, bacterial translocation into systemic circulation

28

diagnosis of necrotizing enterocolitis

abdominal distension
feeding intolerance/emesis
rectal bleeding
abdominal wall erythema
temperature instability/apnea/bradycardia
penumatosis on XR (air in abdominal wall-implies there's migration of bacteria)

29

anorectal malformations

no opening or opening to wrong spot