Hirschsprung’s disease is a condition that affects the large intestine, causing difficulties in passing stool. This issue is present from birth and occurs because the baby’s colon is missing some nerve cells in the muscles. The colon muscles need these nerve cells to move stool through the digestive system. Without them, stool can get backed up, leading to blockages in the bowel.
What Is Hirschsprung’s Disease In Children?
Babies with Hirschsprung’s disease are missing nerve cells in all or part of the large intestine. In most cases, only the end parts of the colon are affected. Without these nerve cells, the muscles can’t move food and waste through that part of the large intestine. Stool can’t move forward. It stays in the large intestine.
The intestine can become partly or fully blocked. It begins to grow larger than normal. This can cause constipation, swelling, pain, and infection.
In most cases, this condition requires surgery to bypass or remove the affected part of the colon. This relieves the blockages and restores normal bowel function.
What are the symptoms of Hirschsprung’s Disease in Children?
Each age group experiences different symptoms. Hirschsprung’s disease affects 80 percent of children within the first six weeks of their lives. However, children who only have a short segment of intestine that lacks normal nerve cells may not exhibit symptoms for several months or years. Constipation is the main symptom of their condition.
Each child may experience symptoms differently, but common symptoms in infants include:
- Not having a bowel movement in the first 48 hours of life
- Slow swelling or bloating of the belly
- Vomiting green or brown fluid
- Fever
It is possible for children who do not show early symptoms to develop the following signs of Hirschsprung’s disease as they grow up:
- Constipation that gets worse over time
- Loss of appetite
- Slow or delayed growth
- Small, watery, bloody stools
- Loss of energy
What causes Hirschsprung’s disease in a child?
The exact cause of Hirschsprung’s disease isn’t completely understood. Sometimes, it can run in families, and there’s a possibility that it’s linked to a genetic mutation in certain cases.
During pregnancy, a baby’s nerve cells form along the intestines. They begin in the mouth and end in the anus. In babies with Hirschsprung’s disease, the nerve cells don’t grow past a certain part of the large intestine.
Hirschsprung’s disease is more common in boys, occurring five times more frequently in them than in girls. Additionally, children with Down syndrome are at a higher risk of developing this condition.
How is Hirschsprung’s disease diagnosed in Children?
When you take your child to the doctor, they will conduct an examination and inquire about your child’s bowel movements. Based on their findings, the doctor might suggest a few tests to either confirm or rule out the possibility of Hirschsprung’s disease.
- Abdominal X-ray: This test allows the doctor to examine the abdomen for any signs of blockages or abnormalities.
- Barium Enema: In this X-ray exam, your child will be given a special metallic fluid called barium. This fluid coats the organs, making them visible on the X-ray. The barium is gently inserted into your child’s rectum using a tube, similar to how an enema is administered. The X-ray of the belly will then reveal if there are any narrow areas, blockages, or if the intestine is bulging above a blockage. It’s a painless way for the doctor to get a closer look and understand what might be going on inside.
- Anorectal Manometry: This test is most often used for older children. A small tube is put into the rectum to check how well the rectal muscles are working. If the muscles don’t relax, it may be a sign of Hirschsprung disease.
- Biopsy of the Rectum or Large Intestine: A small tissue sample is taken to check for the presence of nerve cells. This biopsy helps confirm the diagnosis by identifying whether the necessary nerve cells are present.
How is Hirschsprung’s Disease treated in a Child?
Hirschsprung’s disease treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is.
The common treatment for Hirschsprung’s disease involves surgical intervention to either bypass or remove the section of the colon lacking nerve cells. There are two ways this can be done: a pull-through surgery or an ostomy surgery.
Pull-through surgery: During this procedure, the affected portion of the colon is removed and the healthy section is brought down and connected to the anus. This is generally done by abdominal surgery (abdominal pull-through) or might be done laparoscopically in some cases. In some cases, the surgery can be performed per anally.
Ostomy surgery: A child who is very sick from Hirschsprung’s disease may first need ostomy surgery. A surgeon removes the abnormal portion of the colon and connects the healthy portion to an opening in the child’s abdomen. The stool leaves the body through an opening into a bag attached to the intestine that protrudes through the abdomen hole (stoma). In the subsequent surgery, the healthy normal colon is brought down to the anus and the stoma might be closed in the same surgery or an additional surgery might be required for it after few weeks.
Risk Factors of Hirschsprung’s Disease in Children
The following factors may increase Hirschsprung’s disease risk:
- Having a sibling who has Hirschsprung’s disease. Inheritance of Hirschsprung’s disease is possible. You may be at risk for the condition if you have one biological child with the condition.
- Gender. Hirschsprung’s disease is more frequently observed in males.
- Other Inherited Conditions: The presence of certain inherited conditions, such as Down syndrome, or other congenital abnormalities like congenital heart disease, is linked to an increased risk of Hirschsprung’s disease.
What are the complications of Hirschsprung’s Disease in a Child?
Hirschsprung’s disease is characterized by a lack of nerve cells in a section of the large intestine. It means that digested food and stool cannot pass through that section of the digestive tract. As a result, the large intestine becomes blocked with stool. There will be constipation in your baby, or he or she will not be able to pass bowel movements normally. When there is a blockage, pressure is created inside the intestines. As a result, parts of the intestinal wall wear thin. It is possible for the digestive tract to develop a bacterial infection called enterocolitis (Hirschsprung’s- Associated Enterocolitis- HAEC) over time. This is very serious.
Symptoms of Enterocolitis
Fever: A notable increase in body temperature, indicating the presence of an infection.
Abdominal Pain: Children with enterocolitis may experience discomfort or pain in the abdominal region.
Diarrhea: An increase in the frequency of loose or watery stools, often accompanied by urgency.
Vomiting: Nausea and vomiting can occur as a response to the inflammation and infection.
Bloody Stools: In some cases, the stools may contain blood, indicating internal irritation or damage to the digestive tract.
Dehydration: Reduced fluid intake due to symptoms like diarrhea and vomiting can lead to dehydration, requiring prompt attention and fluid replenishment. If you observe any indications of enterocolitis in your child, it is crucial to promptly contact your child’s healthcare provider.
How Can I Help My Child Live With Hirschsprung’s Disease?
After surgery, your child may experience changes in bowel function. Common long-term issues include challenges with bowel control, occasional stool leakage, constipation, and a risk of infections. The specific problems depend on the extent of the intestine lacking nerve cells and the amount removed during the surgery.
- Introduce High-Fiber Foods Gradually: If your child eats solids, include whole grains, fruits, and veggies while limiting low-fiber options. Go slow to avoid worsening constipation.
- Ask About Constipation-Relieving Formulas: For non-solid eaters, consult the doctor about formulas that can help. Some infants might require a feeding tube temporarily.
- Boost Fluid Intake: Ensure your child drinks enough water, especially if part of their colon was removed. Ample hydration can aid in managing constipation.
- Encourage Physical Activity: Daily aerobic activity promotes regular bowel movements, contributing to your child’s overall well-being.
- Initially, stools may be frequent and loose. To prevent skin irritation, be thorough in cleaning the anal area after bowel movements, and consider using diaper rash creams.
- Some kids may struggle to sense the need for a bowel movement. Encourage your child to spend 10 minutes on the toilet after meals to help with this.
Getting to know Hirschsprung’s Disease in Children is crucial for parents. Dr. Apoorva Kulkarni, a pediatrician in Thane, Bandra, Chembur, stresses early detection and treatment. From symptoms and diagnosis to surgical options, this guide empowers parents. Talk with your child’s healthcare provider about your child’s specific situation. Get expert care for your child’s “Hirschsprung’s Disease” with Dr. Apoorva Kulkarni.