Hirschsprung’s disease is a condition where the large intestine makes it difficult for stool to pass through it. It’s usually found in babies and is present at birth.
Between the fourth and twelfth week of pregnancy, the foetus develops nerve cells in the digestive tract. When these cells don’t grow sufficiently past a specific point in the intestine, digested food and stool can’t move through that part of it.
After this happens, Hirschsprung’s Disease develops, and your baby becomes constantly constipated and unable to have normal bowel movements.
This condition is found in approximately one in five thousand births. Males are more vulnerable than females and children with Down syndrome are at high risk.
Symptoms of Hirschsprung’s Disease differ depending on the severity of the condition. Signs commonly show after birth but may also appear later.
Common symptoms in a newborn:
- Not having a bowel movement within 48 hours after being born
- A swollen stomach
- Vomiting
- Constipation or gas
How is it diagnosed?
Abdominal X-ray
This is to check if the baby’s bowel area is blocked.
Contrast enema
The large intestine is examined with an X-ray to check for abnormalities. The rectum is coated with a substance so that the intestine will be seen on the X-ray machine.
Rectal biopsy
For this procedure, a sample of the cells in the rectum is examined under a microscope. The aim of this exam is to check if the cells in the intestine have developed properly. This isn’t a painful process and a suction rectal biopsy can be done while your little one is in bed.
How is it treated?
As Hirschsprung’s Disease is a life-threatening one, it needs treatment, usually in the form of surgery. Surgery involves removing all or part of the colon and is called a colectomy.
There are different kinds of colectomies:
Pull-through procedure
The surgeon removes the part of the large intestine where nerve cells are missing and connects it to the healthy parts of the anus. This procedure is usually done very soon after a baby is diagnosed.
Ostomy surgery
This procedure changes the route that the stool takes to exit the body when a piece of the bowel is removed. This means that a part of the intestine is brought through the abdominal wall so that stool can leave the baby’s body without going through the anus. After surgery, a removable collection pouch, called an ostomy pouch is worn outside the baby’s body to collect stool. Parents will need to empty the pouch a few times a day. Ostomy surgery is often done with babies who have a severe case of the disease and need to get better before having the pull-through procedure.
Good to know:
- After having a colectomy, a baby will need time to heal.
- Stools may be loose and frequent at first after surgery. Clean the anal area carefully to remove any loose stools.
- Apply rash creams or lotions to help prevent irritation on your little one’s skin.
- For older children, they may struggle to understand when they need a bowel movement. Talk to your doctor about techniques that can be used to help them to become more aware of this.
References:
- https://www.stanfordchildrens.org/en/topic/default%3Fid%3DHirschsprung’ss-disease-90-P01999&sa=U&ved=0ahUKEwjd24bV6KHKAhWF2RoKHUASDtgQFggzMAU&usg=AFQjCNFXTzvi9ari_Xs42kJEphO1Hrgosg
- https://www.mayoclinic.org/diseases-conditions/Hirschsprung’s’s-disease/symptoms-causes/syc-20351556
- https://www.mayoclinic.org/diseases-conditions/Hirschsprung’s’s-disease/symptoms-causes/syc-20351556