What is Hirschsprung Disease? Hirschsprung Disease symptoms, diagnosis and treatment

Hirschsprung disease is a disease of the large intestine that causes severe constipation or intestinal obstruction. There are 1/50000 children suffers from this disorder in the US. If your new infant has just rare bowel movements, and his abdomen seems bloated, your pediatrician will examine him to determine whether recalled stool is swelling the abdomen while the rectum is empty.

What are the symptoms of Hirschsprung Disease

Eighty percent of children with Hirschsprung disease show symptoms in the first 6 weeks of life. Infants suffering from the disorder ordinarily become symptomatic during the first 24 to 48 hours of life. Nonetheless, children with just a short section of intestine that lacks normal nerve cells may not show symptoms for many months if not years. While individuals experience a variety of symptoms, the following are the most typical:

  • Not having a bowel movement in the first 48 hours of life
  • Slow onset of vomiting
  • Slow noticeable swelling of the abdomen
  • Temperature

Kids who do not have early symptoms may present with all the following like Constipation that worsens over time, Sepsis , Lack Of hunger and Delayed increase.

Diagnosis of Hirschsprung Disease

Careful physical examination is needed, and physical findings are dependent upon the age at presentation as well as the intensity of the problem. Establishing the investigation also has experiencing several diagnostic studies. These include the following:
Anorectal manometry. This determines whether regular reflexes involving the rectum as well as the anus are present. Used only in older children, the test can be executed at the bedside.

Abdominal X-ray. This may indicate a bowel blockage. This study only allows doctors to guess the investigation, not definitively diagnose it.

An X ray of the abdomen will reveal a narrowed colon, obstruction and dilated (exceptionally enlarged) intestine above the obstruction.

Rectal biopsy. This procedure will establish the diagnosis of Hirschsprung disease. A sample of the cells in the rectum is taken and then looked at under a microscope.

Confirmation of Hirschsprung is based on the absence of ganglion cells along with the existence of nonmyelinated nerves in the biopsy section.

Contrast enema. This really is a procedure performed to examine the large intestine (colon) for abnormalities. A contrast agent is provided to the rectum so that you can coat the interior of organs so they’ll arrive on an X ray. That is the most valuable radiologic study for confirming the diagnosis.

How to treat Hirschsprung Disease

Hirschsprung disease is treated with operation. Left untreated, it can lead to life threatening complications, so make sure to bring early constipation that happens only after birth or in early infancy to your own pediatrician’s consideration.