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Definitive surgery for Hirschsprung Disease between 3 and 12 months achieves best outcomes: A systematic review with
Oliver Sowulewski1, Julia Leszkowicz1, Marlena Sakowska1
1Department of Paediatrics, Gastroenterology, Allergology and Paediatric Nutrition, Medical University of Gdańsk, Nowe Ogrody 1-6, 80-803 Gdańsk, Poland.
Insights
Optimal timing for definitive pull-through surgery in Hirschsprung disease (HD) is crucial. Early surgery (<3 months) increases complication risks like enterocolitis and incontinence, while later surgery shows better outcomes.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Clinical Outcomes Research
Background:
- The optimal age for definitive pull-through surgery in Hirschsprung disease (HD) remains debated.
- Current guidelines lack clear recommendations on timing, impacting short- and long-term patient outcomes.
Purpose of the Study:
- To systematically review and synthesize evidence on the association between the timing of definitive pull-through surgery and postoperative outcomes in pediatric patients with HD.
- To identify optimal surgical timing to minimize complications and improve patient outcomes.
Main Methods:
- A systematic review was conducted following PRISMA guidelines, including 19 studies (3,980 patients) published between 1998 and 2025.
- Patient age at surgery was stratified into predefined groups (neonatal, <3 months, <6 months, <12 months, <4 years, and above).
- Short- and long-term outcomes were extracted, and risk of bias was assessed using the Newcastle-Ottawa Scale.
Main Results:
- Neonatal surgery and surgery before 3 months were associated with higher rates of Hirschsprung-associated enterocolitis (HAEC) and anastomotic leakage.
- Long-term follow-up revealed increased rates of fecal incontinence and constipation in patients operated during the neonatal period or before 3 months of age.
- Surgery performed later in infancy demonstrated more balanced short- and long-term outcomes compared to early interventions.
Conclusions:
- The timing of definitive pull-through surgery significantly impacts postoperative outcomes in Hirschsprung disease.
- Neonatal and early infancy (<3 months) surgical timing are linked to increased short-term complications and long-term functional deficits.
- Infancy surgery appears to offer more favorable outcomes, suggesting a need for revised surgical timing guidelines.
Introduction:
Optimal age for definitive pull-through in Hirschsprung disease (HD) is debated. Both early and delayed interventions may influence short- and long-term outcomes, but current guidelines do not provide a clear recommendation.
Methods:
A systematic review was conducted following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Nineteen studies published between 1998 and 2025, including 3980 pediatric patients with HD undergoing definitive pull-through surgery, were included. Age at surgery was stratified using predefined thresholds (neonatal, <3 months, <6 months, <12 months, <4 years, and above). Short- and long-term outcomes were extracted and synthesized. Risk of bias was assessed using the Newcastle-Ottawa Scale.
Results:
Short-term outcomes were reported in 15 studies. Hirschsprung-associated enterocolitis (HAEC) was the most frequently reported complication (0-69.1 %), occurring more often in neonates than in non-neonates (22.4 %, 78/348 vs. 15.5 %, 125/808) and in patients operated before 3 months of age compared with those operated later (20.7 %, 68/328 vs. 13.6 %, 60/442). Neonatal surgery was also associated with higher rates of anastomotic leakage (7.1 %, 10/140 vs. 1.5 %, 6/412). Long-term outcomes were assessed in 14 studies. Patients operated during the neonatal period had higher rates of fecal incontinence (30.0 %, 55/183 vs. 17.8 %, 56/314) and constipation (25.2 %, 39/155 vs. 12.8 %, 43/336). Similarly, surgery performed before 3 months of age was associated with increased rates of fecal incontinence (18.0 %, 36/200 vs. 8.6 %, 20/232) and constipation (23.5 %, 47/200 vs. 13.8 %, 32/232) at long-term follow-up.
Conclusions:
Timing of definitive surgery in Hirschsprung disease is associated with differences in postoperative outcomes. Neonatal surgery and surgery performed during early infancy (<3 months) were more often associated with higher complication rates, whereas procedures performed during infancy showed more balanced results.
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