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Hirschprung's Disease-Cause-Signs and Symptoms-Treatment

Hirschprung's Disease:
Hirschsprung's disease is a congenital disease, characterized by the absence of myenteric and submucosal ganglion cells in the distal Gastrointestinal tract (GIT); resulting in loss/decrease of motility in the affected bowel segment. The neuronal connections are necessary for proper contraction and reflexes of the colon. For example in a normal person when intestinal contents reach the distal colon, the walls of colon stretch. This stretch stimulates the myenteric plexus present in the colon wall. This stimulation results in strong peristaltic movement and the person feel the need for defecation.
In Hirschsprung's disease the neuronal circuit in the distal colon is absent and when intestinal contents reach the distal colon, but in this case, the stretch in the intestinal wall doesn't lead to peristalsis. Accumulation of contents distend the distal colon which is referred to as megacolon
Hirschsprung's disease, in other words, is "congenital aganglionic megacolon"

hirschprung's disease signs-barium anema
Hirschprung's disease. Radio-graph - taken after
giving the barium 

Hirschprung's disease results from the absence of parasympathetic ganglion cells in the myenteric and submucosal plexus of the rectum and/or colon.
Ganglion cells, which are derived from the neural crest, migrate caudally with the vagal nerve fibres along the intestine.
Arrest in migration leads to an aganglionic segment.

Clinical Signs and Symptoms
Newborns :

  • Failure to pass meconium within the first 48 hours of life 
  • Abdominal distension that is relieved by rectal stimulation or enemas 
  • Vomiting 
  • Neonatal enterocolitis 
Symptoms in older children and adults include the following:

  • Severe constipation 
  • Abdominal distension 
  • Bilious vomiting 
  • Failure to thrive

Children who have Hirschsprung's disease require surgery to remove the area of the large intestine that has no nerve cells. Sometimes a baby will need enemas to remove stool from the intestine until surgery can be performed.

In most cases, surgery is done within the first months after birth. During surgery, the affected portion of the intestine is removed. Two surgeries may be needed to remove the affected area of the large intestine:
Colostomy: In which the affected part of the colon is removed by surgically opening the abdominal cavity, but re-anastomosis of normal parts of the large intestine is not yet done
rather a tube is attached to the end of the proximal part and this allows the faeces to leave the intestine through the tube into a disposable container. This procedure gives times to the normal part of the intestine to heal. Then after a few weeks, a second surgery is done in which the normal parts of the intestine are re-attached
Laparoscopy: In which the affected part of the colon is removed with the help of a lighted instrument called laparoscope.
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