Overview

Hirschsprung’s disease is a condition that affects the large intestine (colon) and causes problems with passing stool this disease is also known as congenital megacolon. This condition is present at birth (congenital) as a result of missing nerve cells in the muscles of the baby’s colon. Without these nerve cells stimulating gut muscles to help move contents through the colon, the contents can back up and cause blockages in the bowel. If the baby has Hirschsprung’s disease, poop moves through their intestines until it reaches the section that’s missing nerve cells. Once the poop reaches that point, it moves slowly or stops (constipation). There’s no cure for Hirschsprung’s disease, but surgical treatment often results in a positive outcome. There are two types of surgeries to treat Hirschsprung’s disease: a pull-through procedure and an ostomy. Without treatment, Hirschsprung’s disease can cause serious complications. If everything goes well, your baby will feel much better a few days after surgery. No one can prevent Hirschsprung’s disease. If you have the disease or a family history of it, you may want to meet with genetic counselors before starting a family.

Symptoms

Some babies with Hirschsprung’s disease have blocked intestines at birth. There’s a chance your baby has Hirschsprung’s disease if they don’t poop within 48 hours after birth.

Other symptoms of Hirschsprung’s disease in babies may include:

• Swollen belly.
• Constipation.
• Vomiting.
• Diarrhea.
• Lack of appetite (refusing to eat) and poor weight gain.
• Delayed growth.

Causes

It’s not clear what causes Hirschsprung’s disease. It sometimes occurs in families and might, in some cases, be associated with a genetic mutation. During fetal development, neural crest cells typically grow from the top of the small intestine through the large intestine to the anus. In children with Hirschsprung disease, these nerve cells stop growing in the large intestine before reaching the anus.

Diagnosis and Tests

The physician  will check the baby belly to see if it’s swollen and painful. Then they’ll check your baby’s rectum for backed-up poop, also perform one or more of these tests:

• X-ray: Abdominal X-rays can show a blockage in your baby’s intestine.
• Contrast enema: A healthcare provider inserts a catheter (a thin tube) through your baby’s rectum. The catheter fills their intestine with contrast, a safe liquid. A technician takes X-rays as the contrast travels through your baby’s intestine. This exam shows if there are any blockages or narrowing in your child’s intestines.
• Biopsy: Your baby’s healthcare provider uses a special device to remove a small amount of tissue (biopsy) from your baby’s rectum. A pathologist then looks at the tissue under a microscope to check for nerve cells. This procedure isn’t painful and doesn’t require anesthesia.

Treatment

There are two types of surgeries to treat Hirschsprung’s disease: a pull-through procedure and an ostomy.

Pull-through procedure

A surgeon removes the section of your baby’s large intestine that’s missing nerve cells. Then they connect the healthy part of your baby’s large intestine to their anus. The surgeon may use laparoscopic or traditional surgery to perform the pull-through procedure.

The pull-through procedure is the most common surgery for Hirschsprung’s disease and has the best outcome for recovery.

Ostomy surgery

Your baby may need a colostomy (large intestine) or ileostomy (small intestine) before, or at the same time as, a pull-through procedure.

During ostomy surgery, surgeons connect the large or small intestine to the skin outside your baby’s belly. Ostomy surgery allows poop to leave your baby’s body through an opening (stoma) outside of your baby’s anus, usually around their belly. The poop goes into an ostomy bag attached to your baby’s body.

Additional treatments

Some nonsurgical treatments work well in addition to surgery. They include:

• Bowel management: A routine involving medicines and/or enemas to make sure your child’s pooping habits are healthy.
• Sacral nerve stimulation: A surgeon inserts a tiny device near your lower spine to control when you pee and poop.
• Biofeedback: Therapy that involves learning strategies to have more control over involuntary bodily functions — in this case, pooping.

After surgery, some babies with Hirschsprung’s disease may still have constipation, pooping accidents and colon infections. But with long-term follow-up care, most children can manage pooping without becoming severely constipated or developing fecal incontinence.

Conclusion

If you come across any of your known friend, neighbour or loved one suffering from any disease unaffordable or untreatable at your country please feel free to contact us via email query@gtsmeditour.com or you can whatsapp us  on +91 9880149003 and get second medical opinion from our professional healthcare providers abroad. further we shall assist in getting the appropriate treatment.

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