Operative strategies for Hirschsprung disease presenting in adults: A systematic review

To systematically review the surgical management of Hirschsprung disease in patients diagnosed or undergoing definitive surgical treatment in adulthood, and to identify factors influencing operative decision-making in elective and emergency settings.

PubMed, Embase, Scopus, and Web of Science were searched. Studies reporting surgical management of histologically confirmed Hirschsprung disease in patients aged 18 years or older were included. Operative strategies and determinants of surgical decision-making were extracted.

A total of 207 adult patients from 113 studies were included. Adult-specific factors were reported to influence treatment selection in 24 patients (12%). Elective surgical management was reported in 136 patients (66%) and included staged repair (48 [35%]), myectomy (37 [27%]), and one-stage repair (37 [27%]), whereas permanent stoma was rare (2 [1%]). Emergency surgery was required in 62 patients (30%). Operative strategy was strongly influenced by diagnostic recognition: when Hirschsprung disease was recognized preoperatively, stoma-based decompression was favored, whereas lack of recognition was associated with a higher rate of subtotal or total colectomy (39% vs 8%, P = 0.01). Restoration of intestinal continuity after extensive resection was uncommon.

Adult Hirschsprung disease represents a distinct clinical context in which operative decision-making is shaped by adult-specific factors and surgeon experience, and differs between elective and emergency settings. Failure of diagnostic recognition is associated with a higher likelihood of inappropriate extensive bowel resection, potentially limiting subsequent reconstructive options.