What causes Aganglionic Megacolon?
Hirschsprung disease (HD) is a motor disorder of the gut, which is caused by the failure of neural crest cells (precursors of enteric ganglion cells) to migrate completely during intestinal development during fetal life. The resulting aganglionic segment of the colon fails to relax, causing a functional obstruction.
What is the pathophysiology of Hirschsprung disease?
Hirschsprung disease results from the absence of enteric neurons within the myenteric and submucosal plexus of the rectum and/or colon. Enteric neurons are derived from the neural crest and migrate caudally with the vagal nerve fibers along the intestine.
What does congenital megacolon mean?
In digestive system disease: Congenital megacolon. Aganglionic megacolon, or Hirschsprung disease, is a condition of unknown cause that is characterized by the absence of ganglion cells and normal nerve fibres from the distal (or lower) 3 to 40 cm (1 to 16 inches) of the large intestine.
Why is Hirschsprung disease called congenital megacolon?
Hirschsprung’s (HIRSH-sproongz) disease is a condition that affects the large intestine (colon) and causes problems with passing stool. The condition is present at birth (congenital) as a result of missing nerve cells in the muscles of the baby’s colon.
What is Aganglionic segment?
Listen to pronunciation. (ay-GANG-glee-AH-nik MEH-guh-KOH-lun) A condition in which certain nerve cells are missing from the muscle layers of part of the large intestine. This causes severe constipation or blockage of the large intestine.
What is the other name of Hirschsprung disease?
Hirschsprung’s disease is a rare congenital disorder that causes an obstruction (or blockage) of the intestine. This prevents normal bowel movements. You might hear doctors refer to this condition with other names, such as: Congenital megacolon or megarectum.
Is megacolon hereditary?
Although clinically chronic megacolon can occur in any age group, inherited types usually present in young patients, and acquired types usually present in older patients.
What does megacolon mean?
Megacolon, as well as megarectum, is a descriptive term. It denotes dilatation of the colon that is not caused by a mechanical obstruction. [1, 2] Although the definition of megacolon has varied in the literature, most researchers use the measurement of greater than 12 cm for the cecum as the standard.
What is the other name of megacolon?
