Ganglion, Hirschsprung Disease, Hirschsprung Disease, Long-Segment, Hypoganglionosis
Conditions
Brief summary
This study aims to compare the outcomes of patients with long segment Hirschsprung disease or total colonic aganglionosis who had negative calretinin staining and positive ganglion cells on the proximal resection margins to those who had both positive findings.
Interventions
DIAGNOSTIC_TESTCalretinin staining
A paraffin-embedded hematoxylinphloxin-safran sections will be performed on tissue specimens of proximal resection margins to evaluate the presence or absence of ganglion cells. Immunohistochemistry (IHC) will then be done on paraffin-embedded sections for calretinin staining.
Sponsors
St. Justine's Hospital
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
0 Years to 18 Months
Healthy volunteers
No
Inclusion criteria
* Confirmed histopathological diagnosis of Hirschsprung disease from January 1, 1990 to August 31, 2019 * Long-segment Hirschsprung disease (type A and B) * Total colonic aganglionosis (TCA) * Surgery at CHUSJ * Tissue blocks of proximal resection margin available for pathological analysis * Minimum 1 month of postoperative follow-up
Exclusion criteria
* Short-segment Hirschsprung disease (conventional form, rectosigmoid junction) * Total colonic with small bowel aganglionosis (TCSA) * Surgery done at another institution * Early deaths * Lost to follow-up * No documented post-operative outcome
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Long-term outcomes (Quality of life questionnaire) | 1 year | To determine if calretinin has a role in the function of ganglion cells in long segment Hirschsprung disease and total colonic aganglionosis. HAQL (Hirschsprung's disease and Anorectal malformations Quality of Life) questionnaire. For each item the response is scored from 0 to 3 and then, linearly transformed to a 0 (minimum value) to 100 (maximum value) scale. Higher score suggests a better quality of life. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Surgical complications | 1 year | * Number of reoperations * Number of rebiopsies * Number of readmissions * Number of bowel obstructions * Number of bowel perforation * Number of anastomotic leaks * Number of anastomotic strictures * Number of fistula * Number of anal stenosis * Number of Hirschsprung-associated enterocolitis |
| Functional outcomes | 1 year | * Presence of constipation (yes/no) * Presence of diarrhea (yes/no) * Presence of incontinence and soiling (yes/no) * Presence of abdominal distension (yes/no) * Presence of dependance on enemas/medications (yes/no) |
Countries
Canada
Outcome results
None listed