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

Constipation Clinical Trial

Official title:

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

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT05461924
• Other study ID #: NanjingCH-01
• Status: Recruiting
• Start date: July 15, 2022
• Est. completion date: August 20, 2023

Study information

• Verified date: November 2022
• Source: Nanjing Children's Hospital
• Contact: Weibing Tang, Dr
• Phone: 8613851683700
• Email: twbcn[@]163.com
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 50
• Est. completion date: August 20, 2023
• Est. primary completion date: August 20, 2023
• Accepts healthy volunteers: No
• Gender: All
• Age group: 3 Years and older

Eligibility

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

Related Conditions & MeSH terms

• Constipation
• Hirschsprung Disease
• Hirschsprung Disease, Long-Segment
• Incontinence
• Long Term Adverse Effects

Intervention

Other:
• questionnaire survey
questionnaire survey the long term outcomes of long-segment HSCR and compared with rectosigmoid HSCR

Locations

Country	Name	City	State
China	Children's Hospital of Nanjing Medical University	Nanjing	Jiangsu

Sponsors (1)

Lead Sponsor	Collaborator
Weibing Tang

Country where clinical trial is conducted

China, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Bowel function score	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.	through study completion, an average of 5 year
Secondary	Hirschspurng disease associated entrocolitis(HAEC)	The guidelines for the diagnosis and management of HAEC were defined by the American Pediatric Surgical Association in 2017	through study completion, an average of 5 year