congenital ABNL of GI Flashcards
sx and associations of duodenal atresia
sx: polyhydraminos, bilious vomitting, “double bubble” sign on X-ray
- associated with Down syndrome
pyloric muscular propria hyperplasia causes what
congenital pyloric sphincter
omphalocele vs gastroschisis
Both: incomplete closure of abdominal muscle
omphalocele: abdominal contents herniate into ventral membranous SAC ; more associated with other congenital anomalies
gastrochisis: involves all layers of ab wall (peritoneum–>skin) and is herniation of GI content WITHOUT a sac
define true diverticulum
outpouching of all 3 layers of GI wall with lumen communication
why is it important to evaluate other organs when a congenital GI abnormality is found
because of simultaneous organ development in utero
what is the vitelline duct
connect GI tract to yolk sac for nutrient transfer in utero, should involute but if it doesn’t it causes a meckels diverticulum
what is a common abnormality caused by failure of cloacal diaphragm to involute
imperforate anus
details and unique traits of Meckels Diverticulum
- MOST COMMON TRUE diverticulum
- caused by failure of vitelline duct to involute
- located at the ileum
- sx: bleeding, volvulus, intussecpition, obstruction, infarction risk (can present at 2 yo)
- *RULES OF 2:
- 2% population
- 2x more in males
- within 2 feet of ileocecal valve
- 2 inches long
- sx present at 2yo
define diaphragmatic hernia
when abdominal contents herniate into mediastinum
pathogenesis of Hirshsprungs Disease and associated syndrome and gene
“congenital ganglionic mega colon”
- Abnormal neural crest cell migration to rectum or early NCC death leading to not Meissener (submucosal) or Auerback (muscularis) myenteric plexus which leads to impaired peristalsis and obstruction–> MEGA COLON
- associated with down syndrome
- RET gene mutation
what is gastric heterotopia
- type of GI ectopia
- when GI tissue is in Small or large intestine and can cause occult blood in stool (+FOBT)
details of esophageal atresia (most common site, sx, complications, associations)
- most commonly at/around tracheal bifurcation
- causes mechanical obstruction that presents with aspiration, non-bilious vomiting, abdominal distention
- complications: polyhydraminos, suffocation, pneumonia
- associated with TE fistula (fistula connection between esophaguses and trachea/bronchus)
most frequent site of GI ectopia and associated complication
upper 1/3 of esophagus (“inlet patch)
-acid released here can cause Barretts esophagus
*ectopic pancreatic tissue common in stomach and esophagus
what are the two most common types of atresia
esophageal and intestinal (duodenal)
when/how does pyloric stenosis present
3-6 weeks after birth with regurgitation, non bilious projectile vomit, increased “refeeding” demand, visible peristalsis, and olive-like abdominal mass
PS comes late in a letter, ps ED TURNER
