Hirschsprung Disease
Conditions
Keywords
Hirschsprung disease, Scintigraphic defecography
Brief summary
This study aims to investigate long term outcomes of surgical intervention for Hirschsprung's disease and to explore the relation between scintigraphic defecography, type of surgery performed and symptoms as evaluated by a disease specific questionnaire.
Detailed description
Hirschsprung's disease (HD) is a congenital deficiency with incomplete development of nerves in the gastrointestinal tract leading to absence of peristalsis in the affected segment of bowel. Most patients require surgical intervention with removal of the affected segment of bowel. Physical symptoms affecting quality of life are not uncommon in patients born with HD in spite of surgical intervention. This renders a need for a tool for evaluating the functional outcome in these patients. Scintigraphic defecography examines the defecation in terms of the ability to empty the bowel and examines the relocation of faeces within the bowel in relation to defecation. This study aims to explore the relation between scintigraphic defecography, type of surgery performed and symptoms as evaluated by a disease specific questionnaire (Hirschsprung and Anorectal Malformations Quality of Life Questionnaire).
Interventions
None listed
Sponsors
University of Southern Denmark
Study design
Observational model
COHORT
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
15 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Patients operated with any type of surgery for Hirschsprung's disease between 1985-2014 * Age \> 15 years
Exclusion criteria
* Unable to understand given instructions in relation to the defecography (ex. retarded) * Pregnancy * Missing consent
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Evacuation fraction (%) of radioactive material between first, second and third round of scintigraphic imaging | Day 1 | The fraction of evacuated radioactive material by defecation between rounds of scintigraphic imaging |
| Presence of retrograde movement of radioactive material (yes/no) | Day 1 | Presence of retrograde movement of radioactive material within the bowel on scintigraphic images between rounds of scintigraphic imaging |
| Hirschsprung and Anorectal Malformations Quality of life Questionnaire sub scales and total score | Day 1 | Disease specific questionnaire including sub scales for laxative and constipating diet, presence of diarrhoea and constipation, faecal and urinary incontinence, social and emotional functioning, body image, physical symptoms and sexual functioning. Each item is phrased as a multiple-choice question with four response options; never, once in a while, often and very often. Responses are linearly transformed on a 0-100 scale with a higher score indicating better QOL. |
| Type of surgical intervention performed in each individual (for example pull-through surgery) | Day 1 | Type of surgical intervention performed to treat Hirschsprung's disease |
Countries
Denmark
Outcome results
None listed