Hirschsprung's Disease Flashcards
What is Hirschsprung’s disease?
Congenital condition where the nerve cells of the myenteric plexus are absent in the distal bowel and rectum
What is the myenteric plexus also called?
Auerbach’s plexus
What is the myenetric plexus (auerbach plexus)?
It forms the enteric nervous system- It is the brain of the gut.
What is the key pathophysiology finding in someone with hirschprung’s disease?
Absence of parasympathetic ganglion cells in part of the colon and rectum
Who is more commonly affected by hirschsprung’s disease?
Males
What genetic condition is strongly associated with Hirschsprung’s disease?
Down’s syndrome
What is the main presenting feature of hirschsprung’s disease in a neonate?
Delay in passing meconium (more than 24 hours)
What are the other presenting features of hirschsprung’s disease?
Chronic constipation since birth
Abdominal pain and distention
Vomiting
Poor weight gain and failure to thrive
What is the gold standard for the diagnosis of hirschprung’s disease?
Rectal biopsy
What are the other investigations that can be done for hirschsprung’s disease?
Abdominal x-ray
What is the initial management of Hirschsprung’s disease?
Rectal washouts/bowel irrigation
What is the definitive management for hirschsprung’s disease?
Surgical removal of the aganglionic section of bowel
What would you find on a rectal biopsy of someone with hirschsprung’s disease?
Absence of ganglionic cells.
