Hirschsprung disease (HSCR) is a rare congenital disorder of the intestine, typically
diagnosed in neonates presenting with delayed passage of meconium and signs of
bowel obstruction. The gold-standard and life-saving procedure is the surgical
resection of the segment of the colon lacking ganglion cells. A notable number of
patients, nevertheless, experience long-term postoperative complications that
significantly affect quality of life. This review analyzes 315 studies published
between 2013 and 2025, focusing on complications after surgical treatment for
HSCR, especially fecal incontinence (reported in up to 83.3% of cases), constipation
(up to 66.7%), and Hirschsprung-associated enterocolitis (HAEC) (up to 48.5%).
Several factors influence treatment outcomes, including age at operation, type of
surgical procedure, length of aganglionosis, one-stage or multi-stage surgery, and
the presence of HAEC before the operation. Patients with associated syndromes,
e.g., Down syndrome, manifest a higher risk of bowel dysfunction. Children with
HSCR frequently experience a lower quality of life than their peers, specifically in
continence and psychosocial well-being. In contrast, adults report slightly more
favorable long-term quality of life, especially in psychological and social domains.
Differences in research plans, participant numbers, diagnostic criteria, and followup
methods contribute to discrepancies in complication rates. In conclusion,
although surgical intervention is essential, long-term multidisciplinary care and
standardized follow-up are key for optimizing patient outcomes and quality of life.
Keywords: Hirschsprung disease, fecal incontinence, constipation, postoperative
outcomes, HAEC
