Hirschsprung’s Disease

What is Hirschsprung’s Disease?
A Congenital disorder which causes the blockage of large Intestine due to contractions or poor muscle movement in the bowel which affects in excretion of fecal matter or passing stool.This is caused due to missing nerve cells in the muscles of part or all of the baby’s colon.

Hirschsprung’s Disease is a congenital which means by birth, new borns are more effected by this disease.It occurs one in every 5000 new borns.This Disease is uncommon and tends to run in families(familial),this disease causes 25 to 30% of all new born intestinal blockages,risk factor is more in boys.

Symptoms of Hirschsprung’s Disease?
For infants

  • Not having a bowel movement in the first 48 hours of life
  • Swollen belly
  • Vomiting
  • Fever
  • Mild or intermittent constipation often with bouts of mild diarrhea

For older infants

  • Constipation
  • Swollen belly
  • Loss of appetite
  • Gas

Hirschsprung’s Disease Diagnosis:
Hirschsprung’s Disease may not be diagnosed until later in infancy, how ever it is important to make correct diagnosis at early infancy.
Barium enema:This is used to investigate disorders of the lower GI Tract,large intestine and rectum to know the abstraction,but it will not give accurate diagnosis of congenital mega colon in new born babies.
Rectal biopsy: Removal of small amount of tissue for microscopic analysis.
Proctoscopy: Inspection of the rectum with a viewing instrument.

The longer the disease gets untreated,greater the chances of toxic enterocolitis with small intestinal overgrowth of bacteria and production of bacterial toxins
The consequent Fulminant, catastrophic diarrhea results in massive fluid loss and may rapidly result in dehydration and death.

Hirschsprung’s Disease Treatment:
Colostomy: Creation of an artificial outlet for the colon through the abdominal wall.Fluid replacement and antibiotics are important before performing colostomy.Surgical removal of narrowed portion of intestine and rejoin with normal portions of intestine.
Ileostomy. This procedure involves removing the entire colon and excretion is done through small intestine.

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