The Association Between Calretinin and the Function of Ganglion Cells in Hirschsprung Disease

• Conditions: Hirschsprung Disease; Ganglion; Hirschsprung Disease, Long-Segment; Hypoganglionosis

• Registration Number: NCT04149093
• Lead Sponsor: St. Justine's Hospital

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.

Detailed Description

Not available

Study Timeline

• Study First Submitted: 2019-10-23
• Study First Submitted QC: 2019-11-01
• Study First Posted: 2019-11-04
• Study Start: 2020-05-01
• Status Verified: 2021-03
• Last Update Submitted: 2021-03-15
• Last Update Posted: 2021-03-17
• Primary Completion: 2021-09-01
• Study Completion: 2021-12-01

Recruitment & Eligibility

• Status: UNKNOWN
• Sex: All
• Target Recruitment: 50

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

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
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 Outcome Measures

Name	Time	Method
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)
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

Trial Locations

Locations (1)

CHU Sainte-Justine; 🇨🇦 Montréal, Quebec, Canada