Prognosis

Long-term outcomes for Hirschsprung's disease are generally good. Postoperative soiling and obstructive symptoms are relatively common but can usually be managed with proper care.[88][89][90]

Long-term outcome varies greatly depending on the severity of disease and the type of pull-through operation performed.[91] Children with delayed diagnosis of Hirschsprung's disease have more postoperative complications and may have worse long-term outcomes than patients diagnosed in infancy.[92][93] Patients with long-segment disease, particularly total colonic aganglionosis, are known to experience worse long-term outcomes than patients with short-segment disease.[4]​​​​​

Studies suggest children with Hirschsprung's disease have a significantly impaired long-term quality of life.[94][95][96]​​​​ The pooled prevalence of faecal incontinence was 20% in one meta-analysis, and unplanned re-operations were common (44%) in one prospective cohort study.[94][95]​​​ Soiling and obstructive symptoms improve with age, but adult patients with Hirschsprung's disease still report worse bowel function and quality of life than age-matched controls.[97] Transition of care programmes and adult providers familiar with the care of Hirschsprung's disease are important to preserve quality of life in older patients.[98]​​​

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