Restorative proctocolectomy with J-pouch ileoanal anastomosis for total colonic aganglionosis among neonates and infants

Background: No consensus exists on the optimal surgical management of total colonic aganglionosis (TCA). Outcomes after restorative proctocolectomy (RPC) as the initial reconstructive procedure among neonatal and infant TCA patients have not been evaluated previously.

Methods: Medical records of patients with Hirschsprung disease (HD) who underwent RPC during infancy between 1997 and 2012 (n=8) were reviewed. Bowel function and satisfaction with operative results were assessed in a follow-up interview.

Results: Median age at RPC was 1.1 months, and covering loop ileostomies were closed 3.7 months later. No operative complications occurred. Hospitalizations for enterocolitis and obstruction occurred each in 50% of patients postoperatively. Enterocolitis-associated outlet obstruction occurred in one third of patients, most of whom responded well to intersphincteric botulinum toxin (botox) injections. No pouchitis or elevated fecal calprotectin levels (median 51 μg/g) were observed. At last follow-up 3.2 years after ileostomy closure, the median 24-hour stooling frequency was 3.5. None had socially limiting fecal incontinence or problems in holding back defecation. Parent satisfaction with operative results was high.

Conclusions: The rate of postoperative enterocolitis was similar to other procedures, but a better functional outcome was achieved. Botox injections were effective for postoperative functional outlet obstruction. Short-term results following RPC among neonates and infants are promising.