Primary Endorectal Pull-Through for Hirschsprung's Disease
Direct Recommendation
Primary transanal endorectal pull-through (TEPT) should be the first-line surgical approach for children with rectosigmoid Hirschsprung's disease, as it offers equivalent or superior functional outcomes compared to traditional transabdominal approaches while providing significant advantages in operative time, hospital stay, and cosmetic results. 1, 2
Technical Approach and Patient Selection
Optimal Candidates for Primary TEPT
- Children with classic rectosigmoid Hirschsprung's disease are ideal candidates for primary single-stage TEPT without preliminary colostomy. 3, 4
- Primary TEPT can be safely performed across all age groups, including neonates as young as 7 days old, though neonatal cases require awareness of specific complications. 5, 3
- Patients with long-segment aganglionosis, severe preoperative enterocolitis, or prominently dilated colon are NOT good candidates for primary TEPT and should undergo staged repair with initial colostomy. 3
Technical Refinements That Improve Outcomes
The following modifications have been shown to reduce complications and improve results: 1
- No preoperative bowel preparation is necessary - this simplifies perioperative management without compromising safety 1
- Combined general anesthesia with regional sacral block provides optimal operative conditions 1
- Use only a single purse-string suture in the rectal mucosa before transanal submucosal dissection 1
- Avoid retractors and electrocautery during submucosal dissection - this reduces thermal injury and subsequent stricture formation 1
Comparative Outcomes: TEPT vs. Traditional Approaches
Perioperative Advantages
TEPT demonstrates clear superiority in several perioperative metrics: 2
- Operating time is significantly shorter (133 minutes vs. 204 minutes for transabdominal approach, p<0.001) 2
- Hospital stay is significantly reduced (9.8 days vs. 17.7 days, p<0.001) 2
- Time to full oral feeding is faster (2.8 days vs. 4.4 days, p=0.005) 2
- Lower wound infection rates due to absence of abdominal incisions 1
- No visible abdominal scars 5
Long-Term Functional Outcomes
The functional outcomes of TEPT are equivalent to or better than traditional transabdominal pull-through procedures: 1, 2, 4
- Overall success rates (normal or good bowel function) range from 76-93% in patients over 4 years of age 2, 4
- Continence rates are comparable between TEPT and traditional approaches, with 93.8% achieving excellent or good outcomes 2
- One important caveat: soiling tends to be slightly more severe after TEPT compared to transabdominal approaches, though this difference may not reach statistical significance in all studies. 2
Age-Specific Considerations
Neonatal Primary Pull-Through
Primary TEPT in neonates is feasible and safe but requires awareness of specific complications: 5
- Perianal skin rash occurs more frequently in neonates (67% vs. 36% in older children) but typically resolves within 6 weeks 5
- Postoperative anastomotic dilatations are required more often in neonatal cases (40% vs. 9% in older children) 5
- Hospital stay and time to full feeding are equivalent across age groups 5
- Despite these minor differences, neonatal TEPT avoids the morbidity and cost of maintaining a colostomy for months 5
Stooling Pattern Evolution
Understanding normal postoperative stooling patterns prevents unnecessary intervention: 3
- In young infants, stooling frequency declines rapidly from 10.5±3.2 to 4.4±1.6 bowel movements per day in the first 3 months 3
- Most infants achieve regular bowel movements (1-2 times daily) by 1 year postoperatively 3
- At 2 years follow-up, median bowel frequency is 2 movements per day (range 1-6) 4
Complications and Management
Early Complications
The most common early complications include: 1, 5
- Perianal dermatitis (23%) - more common in neonates, usually self-limited 1
- Anastomotic strictures (8.6%) - managed with dilatation regimen 1
- Anastomotic leakage (rare, approximately 3%) - may require temporary diversion 3
- Prolapse of pulled-through colon (rare) - usually reducible without further intervention 5
Postoperative Enterocolitis
Postoperative enterocolitis occurs in 10-21% of patients and is more common in those with preoperative enterocolitis history: 3, 4
- Most episodes are mild and resolve spontaneously or with outpatient management 5, 3
- Severe cases requiring hospitalization occur in approximately 7% of patients 5
- Patients with severe preoperative enterocolitis should be considered for staged repair rather than primary pull-through 3
Major Complications Requiring Reoperation
Major surgical complications occur in approximately 8% of cases: 3
- Anastomotic leakage requiring diversion 3
- Colon perforation 3
- Delayed colovesical fistula formation 3
- Ischemic stricture (particularly in salvage procedures after failed previous operations) 4
Laparoscopy-Assisted Approach
Primary laparoscopy-assisted endorectal pull-through (LAERPT) is an alternative that combines benefits of minimally invasive abdominal mobilization with transanal anastomosis: 3
- Provides excellent visualization for proximal dissection in cases where transanal mobilization alone may be challenging 3
- Continence results are excellent, with 56% normal and 44% good outcomes in children over 3 years 3
- Same contraindications apply: avoid in long-segment disease, severe enterocolitis, or markedly dilated colon 3
Critical Pitfalls to Avoid
Do not attempt primary TEPT in patients with long-segment aganglionosis - these patients require staged repair 3
Do not ignore severe preoperative enterocolitis - this predicts higher postoperative enterocolitis rates and may warrant staged approach 3
Do not use electrocautery during submucosal dissection - this increases stricture risk 1
Do not mistake normal early postoperative stooling frequency (10+ times daily) for pathology - this improves spontaneously over 3 months 3
Do not perform TEPT as salvage after failed previous pull-through without careful consideration - ischemic stricture risk is higher in this setting 4
In neonates, anticipate and counsel families about temporary perianal dermatitis and need for dilatations - these are expected and manageable 5