Gastrointestinal Flashcards
Gastroschisis
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
Prune Belly Syndrome or Eagle Barrett
congenital absence of the abdominal musculature
associated with severe renal and urinary tract abnormalities (more common in males)
Common GI abnormalities associated with polyhydramnios
duodenal atresia and esophageal atresia
Presence of copious oral secretions and inability to pass catheter to stomach
indicates esophageal atresia
presence of a large amount of amniotic fluid in the stomach at delivery indicates…
duodenal atresia
Until 30-32 weeks gestation, the greatest abdominal diameter is ______ than the head circumference; _____ from 32-36, and _______ after 36 weeks.
30-32 weeks - head > abdomen
32-36 weeks - head = abdomen
36 weeks + abdomen > head
The only obstruction that presents with abdominal distention at birth
meconium ileus
malrotation
results from abnormal fixation of the intestine in the abdomen
midgut volvulus
abnormal rotation of the bowel around the mesentary and subsequent obstruction of blood flow to the bowel.
an umbilical hernia is often seen with ___________.
hypothyroidism; usually resolve by 2 years of age
epigastric hernia
a small, firm nodule between the umbilicus and xiphoid process; surgical intervention usually required
omphalocele
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
Associated Syndomes: Duodenal Atresia
fetal hydantoin syndrome, trisomy 21
Associated syndromes: EA / TEF
Aperts, CHARGE, Trisomy 18, VACTERL
Associated syndromes: Hirschsprung’s Disease
DiGeorge, Smith-Lemli-Opitz, Trisomy 21
Associated syndromes: Intestinal Malrotation
Trisomy 13, 18
Associated syndromes: Imperforate Anus
VACTERL
Associated syndromes: Pyloric Stenosis
Aperts, Trisomy 13, 18
Associated syndromes: Umbilical Wall Defects
beckwith-widemann, fetal hydantoin syndrome (umbilical hernia), trisomy 13, 18, 21
Amount of Abdominal Distention directly correlates with level of obstruction; the ______ abdominal distention the more _______ the obstruction.
Greater abdominal distention = more distal obstruction
obstruction beyond the level of the ________ will always result in bilious emesis
ampulla of vader
EA / TEF presentation
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”
Most common type of EA/ TEF
EA with distal TEF
EA / TEF management
repogle to low continuous suction, elevate HOB, Avoid CPAP, try to comfort to avoid crying (gets more air in the abdomen)
Pyloric Stenosis Presentation
nonbilious emesis that progresses to projectile,
on palpation a small “olive” shaped mass in the right upper quadrant
Duodenal Atresia patho, diagnosis
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
Duodenal Atresia Presentation
bilious emesis within a few hours of birth, minimal abdominal distention, failure to stool
Ileal/Jejunal Atresia
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
Malrotation with Midgut volvulus presentation
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
Post-op gastroschisis feeding / nutrition
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
Hirschsprung’s Disease
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
Hirschsprung’s presentation
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
Hirschsprung’s management
rectal irrigation with warm saline x2 daily until surgery,
if irrigations succesfully pass stool, feedings can continue, but if no stool - no feeds
Meconium Ileus
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
Meconium Plug
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
Small Left Colon Syndrome
obstruction due to thick meconium, but site of obstruction is commonly the splenic flexure
50% of cases associated with IDM
NEC
bowel inflammation & necrosis; ,most common site terminal ileum and proximal ascending colon
NEC presentation on X-ray
pneumatosis intestinalis (indicates hydrogen gas in bowel wall from bacterial fermentation)
NEC Bell Staging - Stage 1
clinical signs and symptoms - inconclusive radiologic findings
NEC Bell Staging - Stage 2
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
NEC Bell Staging - Stage 3
advanced
3A critically ill infant with concern for impending perforation
3B critically ill infant with proven intestinal perforation with pneumoperitoneum on x-ray
NEC Stage 1 Management
- 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
NEC Stage 2 management
- 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
NEC stage 3 management
- supportive care
- treatment of coagulopathies
- treatment of hypotension
- surgical consultation (indicated if abdominal free air present)
NEC risk factors in term infant
CHD, polycythemia, hypotension, in utero cocaine exposure, IUGR
NEC Lab Indicators
thrombocytopenia, persistent metabolic acidosis, refractory hyponatremia
Ileocecal Valve function
slows down intestinal transit time to increase absorption
Short Bowel Syndrome
malnutrition and malabsorption resulting from a surgical loss of intestine; degree depends on amount removed and presence of ileocecal valve
Spontaneous Intestinal Perforation Presentation
isolated perforation not associated with necrosis or ischemia
sx: acute abdominal distention with bluish color over abdomen
abdominal xray shows pneumoperitoneum
