Gastrointestinal

Question 1

Gastroschisis

Answer 1

a defect in the abdominal wall through which the viscera protrude, not covered with sac, usually to the right of the midline, lower incidence of associated anomalies with gastroschisis than omphalacele

Question 2

Prune Belly Syndrome or Eagle Barrett

Answer 2

congenital absence of the abdominal musculature
associated with severe renal and urinary tract abnormalities (more common in males)

Question 3

Common GI abnormalities associated with polyhydramnios

Answer 3

duodenal atresia and esophageal atresia

Question 4

Presence of copious oral secretions and inability to pass catheter to stomach

Answer 4

indicates esophageal atresia

Question 5

presence of a large amount of amniotic fluid in the stomach at delivery indicates…

Answer 5

duodenal atresia

Question 6

Until 30-32 weeks gestation, the greatest abdominal diameter is ______ than the head circumference; _____ from 32-36, and _______ after 36 weeks.

Answer 6

30-32 weeks - head > abdomen
32-36 weeks - head = abdomen
36 weeks + abdomen > head

Question 7

The only obstruction that presents with abdominal distention at birth

Answer 7

meconium ileus

Question 8

malrotation

Answer 8

results from abnormal fixation of the intestine in the abdomen

Question 9

midgut volvulus

Answer 9

abnormal rotation of the bowel around the mesentary and subsequent obstruction of blood flow to the bowel.

Question 10

an umbilical hernia is often seen with ___________.

Answer 10

hypothyroidism; usually resolve by 2 years of age

Question 11

epigastric hernia

Answer 11

a small, firm nodule between the umbilicus and xiphoid process; surgical intervention usually required

Question 12

omphalocele

Answer 12

herniation of the bowel contents through the umbilical cord; contents contained within a translucent sac; thought to result from failure of the bowel to reenter the abdomen after its normal extrusion into the cord before the 10th week of gestation

Question 13

Associated Syndomes: Duodenal Atresia

Answer 13

fetal hydantoin syndrome, trisomy 21

Question 14

Associated syndromes: EA / TEF

Answer 14

Aperts, CHARGE, Trisomy 18, VACTERL

Question 15

Associated syndromes: Hirschsprung’s Disease

Answer 15

DiGeorge, Smith-Lemli-Opitz, Trisomy 21

Question 16

Associated syndromes: Intestinal Malrotation

Answer 16

Trisomy 13, 18

Question 17

Associated syndromes: Imperforate Anus

Answer 17

VACTERL

Question 18

Associated syndromes: Pyloric Stenosis

Answer 18

Aperts, Trisomy 13, 18

Question 19

Associated syndromes: Umbilical Wall Defects

Answer 19

beckwith-widemann, fetal hydantoin syndrome (umbilical hernia), trisomy 13, 18, 21

Question 20

Amount of Abdominal Distention directly correlates with level of obstruction; the ______ abdominal distention the more _______ the obstruction.

Answer 20

Greater abdominal distention = more distal obstruction

Question 21

obstruction beyond the level of the ________ will always result in bilious emesis

Answer 21

ampulla of vader

Question 22

EA / TEF presentation

Answer 22

depends on type of defect, but may include excessive oral secretions, drooling, coughing, choking, regurgitation of undigested milk,

recurrent pneumonia with coughing / choking during feeds in the infant that a catheter be passed think “H Type”

Question 23

Most common type of EA/ TEF

Answer 23

EA with distal TEF

Question 24

EA / TEF management

Answer 24

repogle to low continuous suction, elevate HOB, Avoid CPAP, try to comfort to avoid crying (gets more air in the abdomen)

Question 25

Pyloric Stenosis Presentation

Answer 25

nonbilious emesis that progresses to projectile, on palpation a small "olive" shaped mass in the right upper quadrant

Question 26

Duodenal Atresia patho, diagnosis

Answer 26

results from failure of vacuolization at 5-6 weeks GA and recanalization at 8-10 weeks shows on U/S as dilated stomach and duodenum, "double bubble" may also be polyhydramnios

Question 27

Duodenal Atresia Presentation

Answer 27

bilious emesis within a few hours of birth, minimal abdominal distention, failure to stool

Question 28

Ileal/Jejunal Atresia

Answer 28

more commonly occurs as an intrauterine accident where the blood supply is cut off (after 12 weeks gestation) U/S findings: dilated bowel loops w/ absence of air in the distal bowel proximal jejunal atresia shows up as "triple bubble" Presentation: failure to stool, abdominal distention, bilious emesis in the first 24 to 36 hours Contrast enema is gold standard for diagnosis

Question 29

Malrotation with Midgut volvulus presentation

Answer 29

sudden onset of bilious emesis in an otherwise healthy infant who has been stooling and feeding normally; majority present within first week and remaining present within first month gold standard for diagnosis is Upper GI that shows abnormal position of the ligament of treitz

Question 30

Post-op gastroschisis feeding / nutrition

Answer 30

feeding advancement may be challenging due to the prolonged ileus, low osmolality feedings preferred; soy or elemental formulas may be needed for continued feeding intolerance or absorption

Question 31

Hirschsprung's Disease

Answer 31

congenital absence of the ganglion cells of a segment of the colon resulting in functional obstruction; because bowel is unable to relax; more commonly affects the rectum or rectosigmoid colon; **most common cause of large bowel obstruction** males affected 4x more than females

Question 32

Hirschsprung's presentation

Answer 32

failure to stool by 48 hours of life, increased abdominal distention and bilious emesis, rectal stimulation often results in passage of stool rectal biopsy is the gold standard for diagnosis, barium enema is suggestive of hirschsprungs if barium is retained for greater than 24 hours

Question 33

Hirschsprung's management

Answer 33

rectal irrigation with warm saline x2 daily until surgery, if irrigations succesfully pass stool, feedings can continue, but if no stool - no feeds

Question 34

Meconium Ileus

Answer 34

mechanical obstruction of the distal ileum due to thick, tenacious meconium 90% have CF due to an autosomal recessive gene defect that results in altered function of the chloride channel transporter gold standard for diagnosis is a water-soluble contrast enema showing a microcolon with pelets of meconium at the site of distal obstruction. on abdominal xray the distal intestine has a “soap bubble” appearance

Question 35

Meconium Plug

Answer 35

mechanical obstruction of the **distal lumen of the colon** due to thick, tenacious meconium in the absence of enzymatic deficiency or abnormal ganglion cells results from colon dysmotility commonly associated with: maternal diabetes due ot increased fetal glycogen production, maternal magnesium therapy, neonatal CNS disease with hypotonia, neonatal sepsis, prematurity

Question 36

Small Left Colon Syndrome

Answer 36

obstruction due to thick meconium, but site of obstruction is commonly the splenic flexure 50% of cases associated with IDM

Question 37

NEC

Answer 37

bowel inflammation & necrosis; ,most common site terminal ileum and proximal ascending colon

Question 38

NEC presentation on X-ray

Answer 38

pneumatosis intestinalis (indicates hydrogen gas in bowel wall from bacterial fermentation)

Question 39

NEC Bell Staging - Stage 1

Answer 39

clinical signs and symptoms - inconclusive radiologic findings

Question 40

NEC Bell Staging - Stage 2

Answer 40

definitive clinical signs and symptoms present + pneumatosis or portal venous gas on xray 2A - moderately ill infant 2B moderately ill infant with systemic clinical signs

Question 41

NEC Bell Staging - Stage 3

Answer 41

advanced 3A critically ill infant with concern for impending perforation 3B critically ill infant with proven intestinal perforation with *pneumoperitoneum* on x-ray

Question 42

NEC Stage 1 Management

Answer 42

* Bowel rest * fluid resuscitation * replogle to continuous suction * monitoring labs (CBC, electrolytes) * blood culture with broad spectrum antibiotics to cover gut flora * abdominal x-rays q4-8

Question 43

NEC Stage 2 management

Answer 43

* Bowel rest * fluid resuscitation * replogle to continuous suction * monitoring labs (CBC, electrolytes) * blood culture with broad spectrum antibiotics to cover gut flora - consider addition of anaerobic agent * abdominal x-rays q4-8 - + **lateral decubitus imaging for increased risk of pneumoperitoneum**

Question 44

NEC stage 3 management

Answer 44

* supportive care * treatment of coagulopathies * treatment of hypotension * surgical consultation (indicated if abdominal free air present)

Question 45

NEC risk factors in term infant

Answer 45

CHD, polycythemia, hypotension, in utero cocaine exposure, IUGR

Question 46

NEC Lab Indicators

Answer 46

thrombocytopenia, persistent metabolic acidosis, refractory hyponatremia

Question 47

Ileocecal Valve function

Answer 47

slows down intestinal transit time to increase absorption

Question 48

Short Bowel Syndrome

Answer 48

malnutrition and malabsorption resulting from a surgical loss of intestine; degree depends on amount removed and presence of ileocecal valve

Question 49

Spontaneous Intestinal Perforation Presentation

Answer 49

isolated perforation not associated with necrosis or ischemia sx: acute abdominal distention with bluish color over abdomen abdominal xray shows pneumoperitoneum