Perioperative and long-term functional outcomes of neonatal versus delayed primary endorectal pull-through for children with Hirschsprung disease: A pediatric colorectal and pelvic learning consortium study

Background/purpose: the timing of endorectal pull-through for Hirschsprung disease (HD) is controversial. Neonatal primary endorectal pull-through theoretically prevents preoperative enterocolitis. Delayed primary endorectal pull-through offers the surgeon the benefit of more robust perineal anatomy and allows primary caregivers the time to emotionally process the diagnosis and to gain experience with rectal irrigations. We hypothesized that delayed primary endorectal pull-through would be associated with equivalent perioperative morbidity compared to the neonatal repair and would lead to improved long-term functional outcomes.

Methods: we analyzed all patients in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry who had a primary endorectal pull-through for HD and at least three-and-one-half years of follow up in a specialty colorectal clinic. We evaluated patient demographics, operative outcomes, perioperative episodes of enterocolitis, and long-term functional outcomes for the neonatal (<31 days) and delayed (≥31 days) pull-through cohorts.

Results: eighty-two patients were identified of whom 49 were operated upon in the neonatal period and 33 in a delayed fashion. The median age at operation was 11 days [IQR 7 - 19 days] for the neonatal cohort and 98 days [IQR 61 - 188 days] for the delayed cohort. Thirty-four (69.4%) of the neonatal and 22 (66.7%) of the delayed cohort patients had rectosigmoid transition zones. Four of 49 patients (8.1%) in the neonatal cohort were diagnosed with enterocolitis preoperatively compared to two of 33 (6.0%) in the delayed cohort (p = 0.89). Eighteen of 49 patients (36.7%) in the neonatal cohort and 16 of 33 (48.5%) in the delayed cohort had at least one postoperative episode of enterocolitis (p = 0.38). Fifteen of 49 patients (30.6%) in the neonatal cohort were receiving bowel management for fecal incontinence at most recent follow up compared to five of 33 (15.2%) in the delayed cohort (p = 0.13).

Conclusion: delayed primary endorectal pull-through offers a safe alternative to operation in the neonatal period and appears to have, at least, equivalent functional outcomes.

Level of evidence: Level III, retrospective comparative study.