T2-GI Disorders: Hirschprung Disease Flashcards
What is another name for Hirshprung disease?
Congenital aganglionic magacolon
Hirshprung disease is the most common cause of _____
Neonatal obstruction
Hirshprung disease is ___ more common in males
4x
Is this familial?
In some cases
Is this disease associated with other diseases?
Yes, Down Syndrome
Hirshprung disease is the absence of _____ in the bowel.
Ganglion cells
Where is this most often in?
Rectosigmoid region
What does Hirsphrung disease result in?
Portion of the colon not having motility
Hirshprung disease causes accumulation of _____
Intestinal contects
What is proximal to the defect?
Bowel distention
Clinical manifestation: Failure to pass meconium within ____ of birth
24-48 hours
What are some other clinical manifestations of Hirschsprung’s Disease?
- FTT
- Poor feeding
- Refusal to feed
- Chronic constipation
- Abdominal distention
- Bile stained vomitus
- Intestinal obstruction (may have explosive stools)
What would a bowel x-ray show?
Dilated loops of bowel
What with a biopsy of the intestine show?
Absence og ganglion cells
What will an anorectal manometry show?
Failure of the internal sphincter to relax
