🍼💩 Hirschsprung’s Disease in Children: A Parent-Friendly Guide

✅ Hirschsprung’s disease is a condition a baby is born with where a part of the large intestine (colon) is missing nerve cells that help push stool forward.
That section becomes “stuck” and stool can’t pass normally, causing severe constipation or bowel blockage.
It is treatable—most children improve greatly after surgery.

1) 🧾 Quick “At-a-glance” box (top of page)

✅ Condition name: Hirschsprung’s disease
Common names: Congenital aganglionic megacolon

Plain-language summary (2–3 lines):
Hirschsprung’s happens when a segment of the colon lacks the nerve cells that tell the bowel to relax and push stool. Stool backs up behind the blocked area. Surgery removes the affected segment and reconnects healthy bowel.

Who it affects (typical ages):
Most often recognized in newborns (first days of life), but milder cases may present later in infancy/childhood.

✅ What parents should do today:

• If your newborn hasn’t passed stool in the first 24–48 hours, call your doctor urgently
• Watch for belly swelling and vomiting
• Follow the surgical team’s plan carefully after diagnosis
• Learn signs of a serious complication called enterocolitis

⚠️ Red flags that need urgent/ER care:

• Green (bilious) vomiting
• Very swollen belly with poor feeding
• Fever with diarrhea (possible Hirschsprung-associated enterocolitis)
• Extreme sleepiness, weakness, or dehydration

🟡 When to see the family doctor/clinic:

• Chronic severe constipation (especially since birth)
• Poor growth or feeding difficulties
• Recurrent belly swelling or vomiting
• Stool accidents in a previously trained child with severe constipation history

2) 🧠 What it is (plain language)

The bowel moves stool forward using coordinated muscle contractions, guided by nerve cells.

In Hirschsprung’s disease:

• a segment of the colon is missing those nerve cells
• the affected segment stays tight and does not relax
• stool cannot pass easily → buildup and swelling upstream

What part of the body is involved? (small diagram required)

Common myths vs facts

• Myth: “My baby is constipated because of formula or something I ate.”
  Fact: Hirschsprung’s is present from birth due to missing nerve cells.
• Myth: “All constipation is Hirschsprung’s.”
  Fact: Most constipation is functional; Hirschsprung’s is less common but important to recognize.
• Myth: “Surgery means lifelong disability.”
  Fact: Many children do very well, though some need ongoing bowel management.

3) 🧩 Why it happens (causes & triggers)

Cause

• During pregnancy, nerve cells normally migrate down the bowel.
• In Hirschsprung’s, nerve cells do not reach the last part of the colon.

Risk factors

• more common in boys
• can be associated with genetic conditions (for example, Down syndrome)
• family history increases risk

Triggers that worsen symptoms

• dehydration
• infections
• delayed recognition leading to bowel obstruction

4) 👀 What parents might notice (symptoms)

Newborn clues (high yield)

• not passing meconium (first stool) in the first 24–48 hours
• swollen belly
• vomiting (especially green)
• poor feeding

Infant/child clues (milder forms)

• chronic severe constipation from early life
• slow growth
• belly distension
• explosive stool after rectal exam (sometimes)

Serious complication: Hirschsprung-associated enterocolitis (urgent)

• fever
• foul-smelling diarrhea (sometimes with blood)
• worsening belly swelling
• lethargy, dehydration

Symptom tracker

• stool frequency and consistency
• belly size changes
• vomiting (especially green)
• fever episodes
• feeding and weight gain

5) 🏠 Home care and what helps (step-by-step)

✅ Hirschsprung’s is treated surgically. Home care supports safe feeding, stooling routines, and early detection of enterocolitis.

First 24–48 hours after diagnosis (what to expect)

✅ Do this now:

• Your team may decompress the bowel (rectal irrigations) before surgery
• Baby may need IV fluids and careful feeding plan
• Surgical plan discussed:
  • pull-through procedure (most common definitive surgery)

After surgery: early home care

• follow feeding plan
• watch hydration and wet diapers
• track stool output and skin care
• follow surgeon’s guidance on diaper rash prevention

6) ⛔ What NOT to do (common mistakes)

• Don’t ignore fever + diarrhea + bloating (possible enterocolitis).
• Don’t delay urgent evaluation for green vomiting.
• Don’t use random laxatives unless advised post-op (plans vary).
• Don’t assume ongoing stool issues mean surgery failed—many need gradual bowel retraining.

7) 🚦 When to worry: triage guidance

🔴 Call 911 / Emergency now

• baby very floppy/unresponsive
• trouble breathing
• signs of shock (cold, pale, weak)

🟠 Same-day urgent visit

• green vomiting
• worsening belly swelling with poor feeding
• fever + diarrhea (possible enterocolitis)
• blood in stool with illness signs

Example: “My child has fever and diarrhea and belly is getting bigger.”

🟡 Book a routine appointment

• constipation or stooling pattern issues after surgery
• diaper rash or skin breakdown
• questions about bowel training plan

🟢 Watch at home

• child stools regularly, feeding well, no fever, normal belly size

8) 🩺 How doctors diagnose it (what to expect)

What the clinician will ask

• stooling history since birth
• timing of first meconium
• vomiting and belly distension history
• growth and feeding

Physical exam basics

• belly distension
• rectal exam findings (team-guided)

Possible tests (and why)

• abdominal x-ray (signs of blockage)
• contrast enema (shows transition zone)
• rectal biopsy (confirms missing nerve cells — gold standard)

What tests are usually not needed

• repeated imaging if biopsy confirms diagnosis and plan is set

9) 🧰 Treatment options

✅ The definitive treatment is surgery.

First-line treatment

• stabilize baby (fluids, decompression if needed)
• pull-through surgery (removes affected segment and connects healthy bowel)

If not improving (next steps)

• evaluate for strictures, infection, or motility problems
• bowel management program (diet, medications, sometimes irrigations)

Severe cases (hospital care)

• enterocolitis treatment (urgent antibiotics and bowel decompression)
• dehydration management
• rare need for temporary ostomy depending on disease extent and stability

10) ⏳ Expected course & prognosis

Typical timeline

• after surgery, stooling can be frequent initially
• gradual improvement over months
• some children have constipation or stool accidents that improve with a structured plan

What “getting better” looks like

• comfortable belly
• normal feeding and growth
• regular stooling pattern over time

What “getting worse” looks like

• recurring bloating
• fever + diarrhea episodes
• feeding refusal
• dehydration

11) ⚠️ Complications (brief but clear)

Common/manageable

• diaper rash (due to frequent stools early)
• constipation or stool accidents
• mild strictures (sometimes)

Serious complications

• Hirschsprung-associated enterocolitis
• bowel obstruction
• severe dehydration

12) 🛡️ Prevention and reducing future episodes

• follow surgical and bowel management plans
• aggressive diaper/skin care early post-op
• early recognition and treatment of enterocolitis
• maintain hydration and consistent routines

13) 🌟 Special situations

Infants

Hydration and weight monitoring are critical.

Teens

Some may have chronic bowel management needs; support routines and privacy.

Kids with Down syndrome

Higher vigilance for bowel issues and growth monitoring.

Neurodevelopmental differences/autism

Visual schedules and caregiver-led toileting routines help.

Travel considerations

Carry medical summary and “enterocolitis red flag” instructions.

School/daycare notes

• bathroom access plans
• support for accidents if needed
• hydration encouragement

14) 📅 Follow-up plan

• pediatric surgery follow-up
• bowel management clinic or GI follow-up if needed
• growth and nutrition checks
• urgent plan for fever + diarrhea + bloating

15) ❓ Parent FAQs

“Is it contagious?”

No.

“Can my child eat ___?”

Most children can eat normally. Some need fiber, fluids, and structured routines.

“Can they bathe/swim/exercise?”

Yes, once healing is complete and surgeon approves.

“Will they outgrow it?”

Many improve over time, but some need long-term bowel management strategies.

“When can we stop treatment?”

Follow-up continues until stooling is stable and growth is normal.

16) 🧾 Printable tools (high-value add-ons)

🧾 Printable: Hirschsprung’s One-Page Action Plan

Daily:

• Track stool frequency
• Track belly size
• Track hydration (wet diapers / urine output)
• Skin care plan followed

Call clinic if:

• constipation pattern worsens
• frequent accidents persist
• poor growth

Urgent/ER if:

• green vomiting
• fever + diarrhea + bloating (enterocolitis concern)
• blood in stool with illness
• extreme sleepiness or dehydration

🧾 Printable: Enterocolitis Warning Sheet

⚠️ Possible enterocolitis: fever + foul diarrhea + bloating + lethargy.
Action: seek urgent medical care the same day.

🧾 Printable: Stool & Belly Tracker

Date: ______

• Stools: ______
• Belly: normal / mildly bloated / very bloated
• Vomiting: none / yes (green?)
• Fever: yes/no
• Notes: _______________________

🧾 Printable: “Red Flags” Fridge Sheet

⚠️ Urgent: green vomiting, fever + diarrhea + bloating, severe lethargy, dehydration.

17) 📚 Credible sources + last updated date

Trusted references:

• Children’s hospital pediatric surgery Hirschsprung’s family guides
• Pediatric GI bowel management resources from major centers

Last reviewed/updated on: 2025-12-30
Local surgical approaches and bowel management plans may differ.

🧡 Safety disclaimer

This guide supports—not replaces—medical care. If you are worried about your child, trust your instincts and seek urgent medical assessment.

This guide was fully developed & reviewed by Dr. Mohammad Hussein, MD, FRCPC ROYAL COLLEGE–CERTIFIED PEDIATRICIAN & PEDIATRIC GASTROENTEROLOGIST Board-certified pediatrician and pediatric gastroenterologist (Royal College of Physicians and Surgeons of Canada) with expertise in inflammatory bowel disease, eosinophilic gastrointestinal disorders, motility and functional testing, and complex nutrition across diverse international practice settings.

To book an online assessment Email Dr. Hussein’s Assistant Elizabeth Gray at: Elizabeth.Gray@pedsgimind.ca
In the email subject, please write: New Assessment Appointment with Dr. Hussein

Important: This appointment is completely online as Dr. Hussein is currently working overseas. This service is not covered by OHIP