Large Intestine Flashcards
What is the occurrence rate of Hirschsprung’s disease?
Occurs in 1 out of every 5000 live births
What are the increased risk factors for Hirschsprung’s disease?
Positive family history and Down syndrome
What is the most affected site in Hirschsprung’s disease?
Rectosigmoid (75%)
What characterizes Hirschsprung’s disease pathologically?
Absent ganglion cells in the myenteric and submucosal plexus with hypertrophy
What is one type of neurocristopathies associated with Hirschsprung’s disease?
Consequence of defective migration of neural crest cells to colonic mucosa
What causes functional obstruction in Hirschsprung’s disease?
Muscular spasm of the distal colon and internal anal sphincter
What is the bowel condition in Hirschsprung’s disease?
Abnormal bowel is the contracted distal segment, normal bowel is the proximal, dilated portion
What are the signs in neonates that suggest Hirschsprung’s disease?
Delayed passage of meconium beyond the first 24 hours of life, abdominal distension following feeds
What is the gold standard for diagnosing Hirschsprung’s disease?
Rectal biopsy
What does a rectal biopsy demonstrate in Hirschsprung’s disease?
Aganglionosis and hypertrophic nerve trunks
What clinical presentation in infants may indicate Hirschsprung’s disease?
Gross abdominal distension, chronic constipation, failure to thrive
What is the utility of anorectal manometry in Hirschsprung’s disease?
Useful as a screening test; rectoanal inhibitory reflex is absent
What should be suspected in otherwise healthy children with rapid fecal expulsion?
Short segment Hirschsprung disease
What treatment may be sufficient for short segment Hirschsprung disease?
Extended myectomy removing a strip of rectal wall up to the area where normal ganglion cells start
What is a common treatment method for patients with Hirschsprung’s disease?
Repeated tube decompression and gentle rectal washouts with normal saline
What does a water-soluble contrast enema indicate in Hirschsprung’s disease?
The length and site of the involved intestine
What are important positive findings in radiology for Hirschsprung’s disease?
Coning down of transition zone, irregularity in mucosa, abnormal contraction of intestine
What is the treatment approach for long segment Hirschsprung’s disease?
Temporary colostomy followed by definitive procedures
Fill in the blank: Hirschsprung’s disease is associated with _______ of the rectal wall.
contracted
True or False: Fecal soiling is a common feature of Hirschsprung’s disease.
False
