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Adult Outcomes: Hirschsprung Disease
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
Finland has a social security number for all citizens that allows tracking of all patients and access to their medical history from national records.
There are very few controlled studies in adults who have been operated on for Hirschsprung disease in their childhood.
The study was a population-based cross-sectional study of patients operated at the institution between 1960 and 1986, with 143 eligible patients after excluding deaths and migrations.
86 matched controls without any previous surgery were used for comparison.
The operations performed were mainly Duhamel operations.
The average bowel function score in healthy adults is 1.1, where a low score means poor function and a high score means very good bowel function.
The GIQLI (Gastrointestinal Quality of Life Index) is a validated score for health-related quality of life that records physical and social function and emotional states. The average score in healthy adults is 125.8, with a maximum score of 144.
Constipation, soiling, accidents, and social problems were much more frequent in Hirschsprung patients than in controls.
The mean bowel function score of adult Hirschsprung patients was 17.1 compared to 19.1 in controls, and this difference was statistically significant.
25% of Hirschsprung patients scored the full 20 points on bowel function as opposed to 50% of controls.
13% of Hirschsprung patients reported frequent soiling, 2% had accidents, and 10% had complications that required treatment.
The bowel function of Hirschsprung patients is not at the same level as in healthy individuals.
The gastrointestinal quality of life is mostly at the same level as in the healthy population, though some individuals had worse outcomes.
22% of Hirschsprung patients had a GIQLI score lower than 110, which indicates poor gastrointestinal quality of life.
Increasing age was the only significant predictor of poor bowel function in adult Hirschsprung patients.
Age was inversely related to bowel function score in Hirschsprung patients but not in controls, suggesting patients may not do as well as they age.
Low bowel function score was the only predictor of poor gastrointestinal quality of life.
The study on total colonic aganglionosis included 25 patients treated between 1984 and 2013.
Five patients with aganglionosis extending very near the duodenojejunal junction remained on parenteral nutrition.
All patients with aganglionosis extending to the mid small bowel (4 patients) had ileal pouch procedures, but none were weaned from parenteral nutrition and two died from syndromic disease.
Patients with purely colonic aganglionosis (5 patients) all achieved bowel continuity and were weaned from parenteral nutrition.
In the ileal pouch–anal anastomosis group, all patients achieved voluntary bowel movements, with stooling frequency of 4 per 24 hours (range 1–10).
Four out of ten ileal pouch patients had bowel movements at nighttime.
Two ileal pouch patients had some degree of fecal soiling.
None of the ileal pouch patients suffered from constipation.
Most ileal pouch patients had at least one episode of enterocolitis, treated with oral antibiotics (metronidazole).
Obstructive episodes were common in ileal pouch patients, with some requiring more than two Botox injections (maximum 6).
Patients with aganglionosis extending beyond 50 cm of small bowel had poor survival and remained dependent on parenteral nutrition.
Patients with total colonic aganglionosis limited to less than 50 cm of small bowel had reassuring bowel function after ileal pouch–anal anastomosis.
Obstructive episodes and enterocolitis are frequent in total colonic aganglionosis patients after ileal pouch–anal anastomosis but are manageable with Botox and metronidazole.