Table 5

The Hirschsprung’s disease core outcome set

Core outcomeCore areaDefinitionScore 7–9 (%)Minimum age of measurement
Highest scoring outcomes in each OMERACT Filter 2.0 core area
Death with cause specified DeathDeath, with cause classified as due to
  1. A complication of treatment (excluding Hirschsprung-associated enterocolitis)

  2. Hirschsprung-associated enterocolitis

  3. An associated anomaly

  4. Other.

100%No minimum
Long-term faecal incontinence Life impactInvoluntary passage of faecal matter in an inappropriate place by a child aged 5 years or over. Severity of faecal incontinence should be graded as:*
  1. Occasionally (eg, once or twice per week), with or without social problems

  2. Every day, but without social problems

  3. Constant, with social problems.

100%Five years of age
Objective score of bowel function Pathophysiological manifestationsObjective score of bowel function, as measured by the Paediatric Incontinence and Constipation Score in children under 18 years of age, and the Gastrointestinal Quality of Life Index in adults over 18 years of age.94%No minimum
Unplanned reoperation, with indication specified Resource use/economical impact
adverse event category
Unplanned reoperation with indication classified according to NICE criteria as minor, intermediate or major/complex.
Unplanned is defined as any procedure not considered part of routine post-intervention practice.
This outcome should include any procedure that is performed as a direct result of the diagnosis or treatment of the participants HD, and any episodes of general anaesthesia that are required as a direct result of the diagnosis or treatment of the participant’s HD, regardless of whether an operative intervention is undertaken (eg, an examination under anaesthesia, or manual evacuation).
89%No minimum