Timing for reconstructive surgery in Hirschsprung disease.

Introduction: Debate exists regarding the ideal timing for surgery in Hirschsprung disease (HSCR) in various groups of age. The aim of this paper was to suggest a possible strategy to determine the optimal timing for reconstructive surgery in patients affected by HSCR.

Evidence acquisition: A systematic literature search of papers published on PubMed and Embase during the last decade, addressing "Hirschsprung," "preoperative enterocolitis," "preoperative mortality," "complications," and "timing" in all possible combinations, was performed.

Evidence synthesis: A total of 10 out of 170 identified papers addressed this issue in detail and were subsequently assessed for in-depth analysis. Our review confirmed that the most important issue to guide surgical timing is represented by HSCR Associated Enterocolitis (HAEC). Most authors suggest performing pull-through at around 3 months of age after effective bowel decompression, which should not be continued indefinitely to avoid complications.

Conclusions: Based on this systematic review we suggest the following: 1) healthy neonates should undergo surgical reconstruction at 3 months of age; 2) urgent surgery (levelling enterostomy) might be required in critically unwell patients, those with Total Colonic HSCR, or those in whom nursing proved to be ineffective; 3) surgery can be safely postponed only in older patients with a lower likelihood of HAEC (i.e. without previous HAEC occurrences) always avoiding long-lasting rectal irrigations.