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Timing for reconstructive surgery in Hirschsprung disease.

Alessio Pini Prato1, Enrico Felici2

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Optimal timing for Hirschsprung disease (HSCR) surgery balances patient health and complications. For healthy neonates, reconstructive surgery is recommended around 3 months, with urgent intervention for severe cases or ineffective conservative management.

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Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Clinical Strategy

Background:

  • Hirschsprung disease (HSCR) management involves complex decisions regarding surgical timing.
  • Existing literature presents varied approaches to optimizing reconstructive surgery in HSCR patients.
  • Hirschsprung Associated Enterocolitis (HAEC) significantly influences surgical timing considerations.

Purpose of the Study:

  • To propose a strategic framework for determining the optimal timing of reconstructive surgery in Hirschsprung disease.
  • To synthesize current evidence guiding surgical intervention in various HSCR patient groups.
  • To address the critical role of preoperative enterocolitis in surgical decision-making.

Main Methods:

  • Systematic literature review of PubMed and Embase databases.
  • Search focused on HSCR, preoperative enterocolitis, mortality, complications, and surgical timing.
  • Analysis of 10 relevant studies identified from 170 initial papers.

Main Results:

  • Hirschsprung Associated Enterocolitis (HAEC) is the primary factor influencing surgical timing.
  • A consensus suggests pull-through surgery around 3 months post-effective bowel decompression.
  • Prolonged bowel decompression should be avoided to mitigate complications.

Conclusions:

  • Healthy neonates with HSCR should undergo reconstructive surgery at approximately 3 months of age.
  • Urgent surgery (levelling enterostomy) is indicated for critically ill patients, Total Colonic HSCR, or failed conservative management.
  • Delayed surgery is feasible for older patients with low HAEC risk, avoiding prolonged rectal irrigations.