Hirschsprung's disease is a disorder that affects the large intestine (colon) and produces diarrhea. The problem is present at birth (congenital) due to a lack of nerve cells in the baby's colon muscles. If nerve cells do not stimulate gut muscles to assist transport contents through the colon, the contents can back up and produce bowel obstructions.
In the days following delivery, an infant with Hirschsprung's illness normally cannot have a bowel movement. The disease may not be identified until later in infancy in moderate cases. Hirschsprung's illness is seldom identified in adulthood.
The therapy involves surgery to bypass or remove the affected portion of the colon.
WHAT ARE THE SYMPTOMS OF HIRSCHSPRUNG'S DISEASE?
The signs and symptoms of Hirschsprung's disease differ depending on the severity of the disorder. Signs and symptoms usually develop immediately after delivery, although they might appear later in life as well.
The most visible indicator is usually a newborn's inability to make a bowel movement within 48 hours of delivery.
Other newborn signs and symptoms may include:
- Swollen stomach
- Vomiting, particularly vomiting a green or brown liquid
- Constipation or gas, which can make a baby irritable.
- Diarrhea
- Delayed passage of meconium – the first bowel movement of a baby
Signs and symptoms in older children may include:
- Gas
- Inability to thrive
- Swollen stomach
- Constipation that persists
- Fatigue
WHAT ARE THE CAUSES OF HIRSCHSPRUNG'S DISEASE?
It is unknown what causes Hirschsprung's illness. It can occur in families and, in some situations, be linked to a genetic mutation.
Hirschsprung's illness develops when nerve cells in the colon do not fully mature. Colon nerves regulate muscular contractions that carry food through the intestines. Stool remains in the large intestine in the absence of contractions.
WHAT ARE THE RISK FACTORS FOR HIRSCHSPRUNG'S DISEASE?
The following factors may raise the chance of Hirschsprung's disease:
- Having a sibling suffering from Hirschsprung's disease: Hirschsprung's disease is heritable. Future biological siblings may be at danger if you have one kid with the illness.
- Being male: Hirschsprung's illness affects more men than women.
- Having other inherited diseases: Hirschsprung's disease is linked to genetic disorders such as Down syndrome and other birth defects such as congenital heart disease.
WHAT ARE THE COMPLICATIONS OF HIRSCHSPRUNG'S DISEASE?
Hirschsprung's disease patients are predisposed to a dangerous intestinal infection known as enterocolitis. Enterocolitis can be fatal and must be treated right away.
HOW IS HIRSCHSPRUNG'S DISEASE DIAGNOSED?
Your child's doctor will examine him or her and ask questions regarding his or her bowel motions. To diagnose or rule out Hirschsprung's disease, he or she may suggest one or more of the following tests:
- Taking a sample of colon tissue for testing (biopsy): This is the most certain approach to diagnose Hirschsprung's illness. A suction device can be used to obtain a biopsy sample, which can then be inspected under a microscope to identify whether nerve cells are absent.
- Abdominal X-ray using a contrast dye: A special tube put into the rectum is used to deliver barium or another contrast dye to the bowel. The barium fills and covers the gut lining, forming a distinct silhouette of the colon and rectum.
The X-ray will frequently reveal a striking contrast between the nerveless short portion of intestine and the normal but frequently enlarged stretch of bowel behind it.
- Measuring control of the muscles around the rectum (anal manometry): Manometry tests are often performed on older children and adults. A balloon is inflated inside the rectum by the doctor. As a consequence, the surrounding muscle should relax. If it does not, Hirschsprung's illness may be the blame.
WHAT ARE THE TREATMENTS FOR HIRSCHSPRUNG'S DISEASE?
Hirschsprung's illness is usually treated with surgery to bypass or eliminate the section of the colon that lacks nerve cells. There are two options for doing this: a pull-through surgery or an ostomy operation.
Pull-through surgery
The lining of the diseased section of the colon is removed during this treatment. The regular part is then withdrawn from the interior of the colon and connected to the anus. This is normally accomplished through the anus utilizing minimally invasive (laparoscopic) techniques.
Ostomy surgery
In the case of very unwell children, surgery may be performed in two stages.
First, the aberrant piece of the colon is removed, and the upper, healthy portion of the colon is attached to a hole in the child's belly made by the surgeon. Stool subsequently exits the body through the aperture into a bag that is attached to the end of the intestine that protrudes through the abdominal hole (stoma). This gives the bottom section of the colon time to recover.
Following the healing of the colon, a second treatment is performed to seal the stoma and link the healthy segment of the intestine to the rectum or anus.
Surgery outcomes
Most children can pass stool through the anus after surgery.
Possible issues that may resolve themselves over time include:
- Diarrhea
- Constipation
- Leaking stool (fecal incontinence)
- Toilet training delays
Children are also at risk of acquiring a bowel infection (enterocolitis) following surgery, particularly in the first year. If any of the following signs and symptoms of enterocolitis appear, contact your doctor right away:
- Rectum bleeding
- Diarrhea
- Fever
- Abdominal swell
- Vomiting
HOME REMEDIES FOR HIRSCHSPRUNG'S DISEASE?
If your kid develops constipation after Hirschsprung's disease surgery, talk to your doctor about trying any of the following:
- Serve meals high in fiber: Include high-fiber meals if your youngster consumes solid foods. Limit white bread and other low-fiber foods in favor of whole grains, fruits, and vegetables. Because a rapid increase in high-fiber meals might aggravate constipation, introduce high-fiber foods gradually into your child's diet.
If your kid isn't yet eating solid meals, talk to your doctor about formulas that may help ease constipation. Some newborns may require a feeding tube for a period of time.
- Increase your fluid intake: Make it a point to encourage your youngster to drink more water. If some or all of your child's colon was removed, he or she may have difficulty absorbing enough water. Drinking extra water will keep your kid hydrated, which can help relieve constipation.
- Promote physical exercise: Aerobic activity on a daily basis promotes regular bowel motions.
- Laxatives (as prescribed by your child's doctor): If your kid does not react to or tolerates extra fiber, water, or physical activity, some laxatives (medications used to induce bowel movements) may help relieve constipation. Ask your doctor if you should give your kid laxatives, how frequently, and what the dangers and benefits are.