Notes in Hirschsprung’s Disease

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Published	10/08/2024	{{c1::Hirschsprung’s disease}} is a congenital condition where nerve cells of the {{c2::myenteric plexus}} are absent in the {{c3::distal bowel}} a…
Published	10/08/2024	The {{c2::myenteric plexus/Auerbach’s plexus}}, forms the {{c1::enteric nervous system}} i.e. brain of the gut. It is responsible for stimul…
Published	10/08/2024	The key pathophysiology in Hirschsprung’s disease is the absence of {{c1::parasympathetic ganglion cells}}. During fetal development these cel…
Published	10/08/2024	When the entire colon is affected in Hirschsprung's Disease, this is called {{c1::total colonic aganglionosis}}. The aganglionic section of co…
Published	10/08/2024	A {{c1::family history}} of Hirschsprung’s greatly increases the risk
Published	10/08/2024	Hirschsprung’s disease usually occurs in isolation, however it is associated with a number of other syndromes, including: • {{c1::Down’s}} syndrom…
Published	10/08/2024	Presentation of Hirschsprung’s Disease It can present with {{c1::acute intestinal obstruction}} shortly after birth or more gradually developin…
Published	10/08/2024	{{c1::Hirschsprung-associated enterocolitis (HAEC)}} is inflammation and obstruction of the intestine occurring in around 20% of neonates with Hirs…
Published	10/08/2024	{{c1::Abdominal xray}} can be helpful in diagnosing intestinal obstruction and demonstrating features of HAEC
Published	10/08/2024	{{c1::Rectal biopsy}} is used to confirm the diagnosis of Hirschsprung’s Disease. The bowel histology will demonstrate an absence of {{c2::ganglion…
Published	10/08/2024	Management of Hirschsprung Disease Unwell children and those with enterocolitis will require initial {{c1::fluid}} resuscitation and management …
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