The Long Term Outcomes After Pull-through of Long Segment Hirschsprung Disease

Recruiting

• Conditions: Constipation; Hirschsprung Disease, Long-Segment; Incontinence; Long Term Adverse Effects
• Interventions: Other: questionnaire survey

• Registration Number: NCT05461924
• Lead Sponsor: Weibing Tang

Brief Summary

Hirschsprung disease (HSCR) is characterized by the absence of ganglion cells (aganglionosis) in the distal bowel extending proximally for varying distances that results in persistent spasm in the affected bowel and functional intestinal obstruction. Patients can be classified as rectosigmoid HSCR when aganglionosis confined to the rectosigmoid and long-segment or total colonic HSCR when aganglionosis extends beyond the upper sigmoid. Aganglionosis of long-segment HSCR can extend to the descending colon, transverse colon, ascending colon, but not to the terminal ileum. To date, there is insufficient evidence to recommend a preferred or superior method for the surgical repair for long-segment HSCR. In general, a pull-through with standard of care for the intestine and mesentery, which avoids excessive resection of the colon and coloanal reconstruction, is performed for long-segment HSCR.There are reports that a significant percentage of long-segment HSCR patients continue to have difficulty with soiling and incontinence,however there were also reports long-segment HSCR patients have the same continece as rectosigmoid HSCR.The outcome of long-segment HSCR should be thoroughly evaluated. The present study was designed to evaluate the long-term outcomes of long-segment HSCR.

Detailed Description

Hirschsprung disease (HSCR) is characterized by the absence of ganglion cells (aganglionosis) in the distal bowel extending proximally for varying distances that results in persistent spasm in the affected bowel and functional intestinal obstruction. Patients can be classified as rectosigmoid HSCR when aganglionosis confined to the rectosigmoid and long-segment or total colonic HSCR when aganglionosis extends beyond the upper sigmoid. Aganglionosis of long-segment HSCR can extend to the descending colon, transverse colon, ascending colon, but not to the terminal ileum. To date, there is insufficient evidence to recommend a preferred or superior method for the surgical repair for long-segment HSCR. In general, a pull-through with standard of care for the intestine and mesentery, which avoids excessive resection of the colon and coloanal reconstruction, is performed for long-segment HSCR.There are reports that a significant percentage of long-segment HSCR patients continue to have difficulty with soiling and incontinence,however there were also reports long-segment HSCR patients have the same continece as rectosigmoid HSCR.The outcome of long-segment HSCR should be thoroughly evaluated. The present study was designed to evaluate the long-term outcomes of long-segment HSCR.

The long-term outcome would be indicated by bowel function SCORE.

Study Timeline

• Study First Submitted: 2022-07-12
• Study Start: 2022-07-15
• Study First Submitted QC: 2022-07-15
• Study First Posted: 2022-07-18
• Status Verified: 2022-11
• Last Update Submitted: 2022-11-30
• Last Update Posted: 2022-12-01
• Primary Completion: 2023-08-20
• Study Completion: 2023-08-20

Recruitment & Eligibility

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

Inclusion Criteria

Clinical diagnosis of Hirschsprung disease Must be performed with the operation of pull-through Must be followed up more than 3 years

Exclusion Criteria

Clinical diagnosis of Down syndrome Clinical diagnosis of tolal colonic Hirschsprung disease Clinical diagnosis of degestive malformation except Hirschsprung disease

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
long-segment HSCR	questionnaire survey	Aganglionosis of non-rectosigmoid HSCR can extend to the descending colon, transverse colon, ascending colon, but not to the terminal ileum
rectosigmoid HSCR	questionnaire survey	Aganglionosis confined to the rectosigmoid

Primary Outcome Measures

Name	Time	Method
Bowel function score	through study completion, an average of 5 year	Bowel function was evaluated by the BFS (20 points), which was approved by Rintala in 1995 ; patients with a score \> 18 were considered to have normal bowel habits.

Secondary Outcome Measures

Name	Time	Method
Hirschspurng disease associated entrocolitis(HAEC)	through study completion, an average of 5 year	The guidelines for the diagnosis and management of HAEC were defined by the American Pediatric Surgical Association in 2017

Trial Locations

Locations (1)

Children's Hospital of Nanjing Medical University; 🇨🇳 Nanjing, Jiangsu, China