Hirschsprung disease-Bowel function beyond childhood

Area of Science:

• Gastroenterology
• Developmental biology
• Pediatric surgery

Background:

• Hirschsprung disease is a congenital disorder of the enteric nervous system, resulting in aganglionic segments of the distal colon.
• Impaired bowel function is a known complication, with previous studies focusing primarily on short-term outcomes.

Purpose of the Study:

• To review and synthesize current knowledge on bowel function outcomes in patients with Hirschsprung disease beyond childhood.
• To assess the long-term impact of Hirschsprung disease on bowel function in adolescents and adults.

Main Methods:

• Systematic review of controlled studies and relevant literature.
• Analysis of data on bowel function, quality of life, education, and occupation in patients with Hirschsprung disease.

Main Results:

• Bowel dysfunction, including constipation and fecal incontinence, persists into adolescence and adulthood in a significant proportion of patients.
• Despite impaired bowel function, most patients adapt, and the disease has a limited impact on educational and occupational achievements.
• Quality of life is negatively affected by bowel symptoms, though adaptation occurs.

Conclusions:

• Hirschsprung disease has significant long-term consequences for bowel function and quality of life into adulthood.
• While functional impairments are notable, educational and occupational trajectories are generally preserved, indicating patient adaptation.