Hirschsprung’s disease or megacolon is a genetic disorder that is classified by an absence of nerve cells in the bottom portion of the 5′ large colon. These ganglia nerves are responsible for allowing muscles to contract (peristalsis), which moves digested food through the colon to the anus. In other words, a bowel movement will form, within the intestines, but the child will not be able to expel it from their rectum. Instead, the food will become blocked in the colon, which causes many secondary problems such as pain and enterocolitis ( infection of the intestines).
This disorder is more common in male newborns than females and effects 1 of every 5,000 live births (Nationwide Children’s).
What is Hirschsprung’s Disease
The sigmoid colon is most often involved with Hirschsprung’s disease, which is the area of the large colon right before the anus. A bowel resection surgery is most often required, in order to restore function to the colon. The sphincter muscles and anal canal, so the child can maintain the continence mechanism.
During the surgery a temporary colonoscopy is performed, so the child can continue to have normal bowel movements, which will give the colon time to completely heal.
Hirschsprung’s disease may be linked to genetics and heredity, although it has not been completely determined.
Symptoms of Hirschsprung’s Disease
Most children will exhibit with symptoms, within the first 48 hours of life, because they will not have a bowel movement. Others with a short colon segment involvement will not see an onset of symptoms for several months or years.
- Abdominal distension (bloating)
- Bile vomitus (greenish/yellow)
- Sepsis (infection that spreads through the bloodstream)
- Failure to thrive
- Decreased appetite
- Loose stools or constipation
Each child is different and may or may not exhibit all symptoms.
Hirschsprung’s Disease in Children
If children are able to make it through the first years of their life, without complications of Hirschsprung’s, they will need to undergo a series of testing, in order to determine their condition.
- Colon biopsy
- Anorectal manometry (determines sphincter muscle contractility)
Prognosis of Hirschsprung’s Disease
If the condition is diagnosed and treated early, the newborn or child will be able to live a normal life, with normal bowel movements.