Hirschsprung Disease in Babies: Signs & Surgery

Dr. Tanmay Motiwala
Pediatric Surgeon, Raipur · Updated 30 Sep 2026 · 6 min read

Every healthy newborn baby should pass their first black, sticky stool (meconium) within the first 24 to 48 hours of life. So if your baby has not passed any stool for more than 2 days, and the tummy looks swollen, a doctor needs to check. One possible cause is Hirschsprung disease.

I always look for this condition in such a baby. The name sounds hard, but the idea behind it is simple. And so is the answer once we confirm it.

What Is Hirschsprung Disease?

Think of the bowel as a long tube. For stool to move through it and come out, the bowel wall needs to squeeze and push. For example, think of how you squeeze a tube of toothpaste from one end to the other.

Tiny nerve cells inside the bowel wall send the signal to squeeze. In a baby with Hirschsprung disease, these nerve cells are missing from the lower part of the bowel. The signal never arrives. So that part of the bowel stays stiff and tight, like a blocked pipe, and stool cannot get through. This is not the parents’ fault. It happens during the baby’s growth in the womb, before birth.

Signs of Hirschsprung Disease in Babies

The most common sign in a newborn is simple: no stool in the first 48 hours, with a swollen, tight-looking tummy.

In slightly older babies, the signs look different. These babies have had trouble passing stool since the very beginning, from the first weeks of life. Also, their tummy is always a little bloated. Changes in diet or simple medicines do not help. Sometimes they pass a sudden large burst of very watery stool after many days with nothing. But this is not diarrhoea. It is stool leaking around a blockage.

How is this different from ordinary constipation? First, ordinary constipation in babies does not start from day one of life. Second, it usually gets better with small changes. Hirschsprung disease does not.

How Does the Doctor Confirm It?

An X-ray with a dye: First, we gently put a small amount of safe dye into the back passage. On the X-ray, we can then see where the narrow, blocked part of the bowel ends and the normal, wider bowel begins. This tells us how much of the bowel has the problem.

A small tissue test (biopsy): Next, we take a tiny piece of tissue, smaller than a grain of rice, from just inside the back passage. This needs no operation and takes a few minutes. Then a lab checks whether the nerve cells are there or missing. This is the surest way to confirm the diagnosis.

Surgery for Hirschsprung Disease

The treatment is surgery, because no medicine can replace the missing nerve cells. In the operation, I remove the part of the bowel that has no nerve cells. Then I bring the healthy bowel down and join it just above the bottom opening. So stool can pass normally.

For most babies with the common type, we do this as a single surgery through the bottom passage itself. The child does not need a permanent bag.

But sometimes the baby is very unwell on arrival, or the blockage has been there a long time. Then we first make a small opening in the tummy to let stool out safely. This opening is always temporary. We close it later, once the baby has recovered.

What Happens After Surgery?

In the first few months after surgery, the baby will pass loose stool often. This is completely normal, because the bowel is still settling down. It usually improves within 6 to 9 months.

After surgery, we teach parents to gently pass a small smooth stick into the bottom opening twice a day. This keeps the opening from becoming too tight as it heals. Most parents manage it well once we show them.

About long-term bowel function — an honest picture:

Most children improve steadily through childhood. Puberty is when the final improvement usually happens, not necessarily school age. By their mid-teens, many children say they have a good quality of life and cope normally day to day.

However, the numbers are worth knowing. Studies show that 10 to 30% of children have some ongoing soiling after surgery. Some get constipation again. This is because the inner muscle at the bottom can stay tighter than normal, even after a well-done operation. It often improves with a simple procedure (an injection to relax the muscle). But it may need care more than once.

Even in adults who have had a good outcome, research shows that bowel function scores remain lower than in people who never had the condition. Also, some adults find that their control slowly gets harder with age. That is why long-term follow-up with a surgeon matters.

I do not say this to scare you. Most children live full, normal lives. But this condition needs honest check-ups and follow-up, not just surgery and discharge.

One Warning That Every Parent Must Know

Sometimes, a child with Hirschsprung disease can get a serious bowel infection. This can happen before or after surgery. Watch for these signs:

Sudden, very watery and foul-smelling stool
A very swollen, hard tummy
Fever
The baby looks very unwell — pale, not feeding, very sleepy

If you see these signs, go to a hospital immediately. Do not wait. This is a medical emergency that needs treatment within hours.

When Should I See a Pediatric Surgeon?

Come to a children’s surgery centre without delay if:

Your newborn has not passed any stool in 48 hours and the tummy looks swollen
Your baby has had difficulty passing stool since the very first weeks of life
Your baby’s tummy always looks bloated, even after passing stool
A child with known Hirschsprung disease suddenly develops a swollen tummy, watery stool, and fever

Watch: Dr. Tanmay Motiwala explains Hirschsprung Disease in Hindi

📋 This article is part of Dr. Motiwala’s Pediatric Colorectal & Anorectal Surgery in Raipur services — see the full range of conditions treated, what to expect, and when to see a pediatric surgeon.

Baby Not Passing Stool Since Birth? It Could Be Hirschsprung Disease