Hirschsprung Disease Surgery
What is Hirschsprung Disease Surgery ?
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Why do you need Hirschsprung Disease Surgery ?
Hirschsprung disease typically presents in newborns or infants with symptoms like failure to pass meconium within 48 hours, severe constipation, abdominal distension, or vomiting. It results from the absence of ganglion cells in the distal colon (most commonly the rectosigmoid region), leading to a functional obstruction. Surgery is indicated for:
- Confirmed Hirschsprung disease: Diagnosed via rectal biopsy showing absent ganglion cells.
- Symptomatic cases: Chronic constipation, enterocolitis, or bowel obstruction.
- Long-segment or total colonic HD: Rare cases where the entire colon or extensive segments are affected.
The goal is to remove the aganglionic segment, restore bowel continuity, and prevent complications like enterocolitis or megacolon.
Why Do Costs Vary in Philippines?
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Hirschsprung Disease Surgery Procedure
The procedure is tailored to the child’s age, extent of aganglionic bowel, and overall health:
- Before Surgery Evaluation:
- Diagnosis is confirmed via:
- Rectal suction biopsy: Shows absent ganglion cells and nerve hypertrophy.
- Contrast enema: Reveals a transition zone (narrow aganglionic segment to dilated proximal bowel).
- Anorectal manometry (in older children): Detects abnormal sphincter relaxation.
- Blood tests assess nutrition, infection, or electrolyte imbalances, common in obstructed infants.
- Imaging (e.g., abdominal X-ray, ultrasound) checks for obstruction or enterocolitis.
- Pre-surgery management may include rectal irrigations or laxatives to relieve obstruction, and IV fluids/antibiotics for enterocolitis.
- Parents receive counseling on the procedure, potential stoma, and post-operative care.
- Diagnosis is confirmed via:
- Surgical Techniques:
- Single-Stage Pull-Through Procedure:
- Preferred for stable infants with short-segment HD (rectosigmoid involvement), typically performed at 1–6 months of age.
- Performed under general anesthesia, lasting 2–4 hours, often laparoscopically or transanally.
- Common techniques:
- Soave (endorectal) pull-through: Removes the aganglionic mucosa, pulling healthy colon through a muscular cuff to the anus.
- Swenson pull-through: Removes the full thickness of the aganglionic rectum, connecting healthy colon directly to the anus.
- Duhamel pull-through: Creates a side-to-side anastomosis between healthy and aganglionic bowel, preserving some rectal function.
- The aganglionic segment is resected, and healthy, ganglionic bowel is pulled down and anastomosed near the anus.
- Intraoperative frozen-section biopsies confirm ganglion cells in the pulled-through segment.
- Staged Procedure:
- Used for long-segment HD, total colonic HD, or unstable infants (e.g., with enterocolitis or obstruction).
- Stage 1: A temporary colostomy or ileostomy is created to divert stool, allowing the child to stabilize and grow (performed in newborns or infants).
- Stage 2: Pull-through procedure is performed at 6–12 months, resecting the aganglionic segment and connecting healthy bowel.
- Stage 3 (if needed): Colostomy/ileostomy reversal after healing.
- Open surgery is common, though laparoscopic assistance is used in some centers.
- Laparoscopic-Assisted Pull-Through:
- A minimally invasive approach using 3–4 small incisions, a laparoscope, and transanal dissection.
- Offers less pain, smaller scars, and faster recovery, suitable for short-segment HD in stable patients.
- Transanal Pull-Through:
- A scarless approach for short-segment HD, performed entirely through the anus, minimizing abdominal incisions.
- Increasingly popular in centers like Apollo Children’s Hospital.
- Single-Stage Pull-Through Procedure:
- After Surgery:
- Patients are monitored in the pediatric ICU for 1–3 days to manage pain, fluids, and bowel function.
- Hospital stay lasts 3–7 days for single-stage procedures or 5–10 days for staged/open surgeries.
- IV antibiotics prevent infection; a nasogastric tube (if used) is removed once bowel function resumes (2–5 days).
- Parents are trained on anal dilatations (if needed) to prevent anastomotic strictures.
- Pathology reports confirm complete removal of aganglionic tissue.
Recovery After Hirschsprung Disease Surgery
Recovery focuses on restoring bowel function, monitoring for complications, and supporting growth:
- Hospital Stay:
- Single-stage pull-through: 3–7 days, with 1–2 days in the ICU.
- Staged procedures: 5–10 days per stage, with 1–3 days in the ICU.
- Post-Surgery Care:
- Pain is managed with medications (e.g., paracetamol, opioids initially).
- IV fluids and gradual feeding (breast milk, formula, or soft foods) resume within 2–5 days as bowel function returns.
- Antibiotics treat or prevent enterocolitis; rectal irrigations may be used if stooling is delayed.
- Parents are taught anal dilatation (if prescribed) to maintain anastomotic patency, typically starting 2–3 weeks post-surgery.
- Activity and Diet:
- Infants resume normal feeding as tolerated, with breast milk or formula preferred for nutrition.
- Older children follow a soft, high-fiber diet (e.g., pureed fruits, vegetables) to promote regular bowel movements.
- Strenuous activities (e.g., sports for older children) are avoided for 4–6 weeks.
- Stoma care is critical for staged procedures, with training on bag changes and skin protection.
- Follow-Up:
- Follow-up visits at 1–2 weeks, 4–6 weeks, and 3–6 months assess bowel function, growth, and complications.
- Rectal exams or contrast studies check for strictures or residual aganglionosis.
- Stoma reversal (if applicable) is planned after 6–12 months.
- Long-term follow-up monitors for enterocolitis or bowel dysfunction (up to 5–10 years).
Most children achieve normal or near-normal bowel function within 3–6 months, with 80–90% continent by school age. Success rates are 85–95% for short-segment HD, though long-segment cases may have more complications.
Risks and Complications
Hirschsprung disease surgery is complex, with potential risks:
- Surgical Risks:
- Bleeding, infection, or wound dehiscence at the incision or stoma site.
- Anastomotic leak (2–5%), causing peritonitis or abscesses.
- Post-Operative Complications:
- Hirschsprung-associated enterocolitis (HAEC, 10–30%), a serious infection causing diarrhea, fever, or distension, requiring antibiotics or irrigations.
- Anastomotic stricture (5–15%), treated with dilatations or reoperation.
- Fecal incontinence (5–20%), more common in long-segment HD or if sphincter damage occurs.
- Stoma-Related Complications:
- Stoma prolapse, retraction, or skin irritation, requiring revision or care adjustments.
- General Risks:
- Adverse reactions to anesthesia.
- Adhesions causing bowel obstruction, needing surgery.
- Long-Term Issues:
- Chronic constipation or soiling, particularly in long-segment HD or if residual aganglionic tissue remains.
- Growth delays if nutrition is compromised pre- or post-surgery.
Prompt reporting of symptoms like fever, diarrhea, or no stool output ensures timely management.
Frequently Asked Questions (FAQs)
What causes Hirschsprung disease?
It’s a congenital condition due to failure of neural crest cells to migrate, forming ganglion cells in the colon, often genetic (e.g., RET gene mutations).
Can Hirschsprung disease be treated without surgery?
No, surgery is required to remove the aganglionic segment. Non-surgical measures (e.g., irrigations) provide temporary relief but don’t address the underlying defect.
Is the colostomy permanent in Hirschsprung surgery?
Colostomy is usually temporary in staged procedures and reversed after pull-through. Single-stage procedures often avoid stomas.
How soon can my child resume normal activities?
Feeding resumes in 2–5 days; normal activities (e.g., play) resume in 4–6 weeks, depending on the procedure.
Is Hirschsprung disease surgery covered by insurance in India?
Most insurance plans cover it as a congenital condition. Confirm with your provider.
What are the signs of complications post-surgery?
Fever, diarrhea, abdominal distension, no stool output, or stoma issues require immediate medical attention.
Will my child have normal bowel function after surgery?
Most children (80–90%) achieve normal or near-normal bowel function, though long-segment HD may cause persistent issues like incontinence or constipation.
What lifestyle changes are needed post-surgery?
Ensure a high-fiber diet, adequate hydration, regular follow-ups, and prompt treatment of enterocolitis symptoms to support bowel health and growth.
Conclusion
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Looking for Best Hospitals for Hirschsprung Disease Surgery
Note: we are not promoting any hospitals
We do not promote any hospitals. All details are sourced from Google and hospital websites. Please verify independently. Start with your surgery—here’s the list of government, budget, and superspecialty hospitals accredited by the Department of Health (DOH), Philippine Health Insurance Corporation (PhilHealth), and Joint Commission International (JCI)
- click on your city and schedule your surgery now done in good hands.
| City | Minimum Price (₱) | Maximum Price (₱) |
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Angeles City |
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Antipolo |
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Bacolod |
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Bacoor |
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Baguio |
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Butuan |
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Cagayan de Oro |
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Caloocan |
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Cebu City |
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Dasmariñas |
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Davao City |
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General Santos |
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General Trias |
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Iligan |
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Iloilo City |
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Las Piñas |
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Makati |
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Malolos |
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Manila |
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Muntinlupa |
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Parañaque |
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Pasay |
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Pasig |
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Puerto Princesa |
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Quezon City |
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San Fernando |
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San Jose del Monte |
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Taguig |
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Valenzuela |
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Zamboanga City |
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Disclaimer
This information is sourced from open platforms and is for general awareness only. Costs may vary based on individual cases. Please consult a qualified doctor for medical advice and explore options within your budget before deciding on the procedure.