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

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.

Study Overview

• Status: Recruiting
• Conditions: Constipation; Incontinence; Long Term Adverse Effects; Hirschsprung Disease, Long-Segment
• Intervention / Treatment: Other: questionnaire survey

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 Type: Observational
• Enrollment (Anticipated): 50

Contacts and Locations

• Study Contact: Name: Weibing Tang, Dr; Phone Number: 025-83117354 8613851683700; Email: twbcn@163.com
• Study Contact Backup: Name: Changgui Lu, Dr; Phone Number: 025-83117354 8613770848448; Email: luchanggui1984@163.com

Study Locations

• China
  • Jiangsu
    • Nanjing, Jiangsu, China, 210008
      • Recruiting
      • Children's Hospital of Nanjing Medical University
      • Contact: Lu Changgui, Dr; Phone Number: 8613770848448; Email: luchanggui1984@163.com

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 3 years and older (Child, Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

patients with with rectosigmoid and long-segment Hirschsprung disease after operation.

The follow-up age must more than 3 years old.

Description

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 Plan

How is the study designed?

Design Details

• Observational Models: Case-Control
• Time Perspectives: Retrospective

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
long-segment HSCR; Aganglionosis of non-rectosigmoid HSCR can extend to the descending colon, transverse colon, ascending colon, but not to the terminal ileum	Other: questionnaire survey; questionnaire survey the long term outcomes of long-segment HSCR and compared with rectosigmoid HSCR
rectosigmoid HSCR; Aganglionosis confined to the rectosigmoid	Other: questionnaire survey; questionnaire survey the long term outcomes of long-segment HSCR and compared with rectosigmoid HSCR

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
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 Outcome Measures

Outcome Measure	Measure Description	Time Frame
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

Collaborators and Investigators

• Sponsor: Weibing Tang
• Investigators: Principal Investigator: Changgui Lu, Dr, Department of pediatric surgery of Children's hospital of Nanjing medical unverisity

Study record dates

Study Major Dates

• Study Start (Actual): July 15, 2022
• Primary Completion (Anticipated): August 20, 2023
• Study Completion (Anticipated): August 20, 2023

Study Registration Dates

• First Submitted: July 12, 2022
• First Submitted That Met QC Criteria: July 15, 2022
• First Posted (Actual): July 18, 2022

Study Record Updates

• Last Update Posted (Actual): December 1, 2022
• Last Update Submitted That Met QC Criteria: November 30, 2022
• Last Verified: November 1, 2022

More Information

Terms related to this study

• Keywords: bowel function; Hirschsprung disease associated enterocolitis
• Additional Relevant MeSH Terms: Digestive System Diseases; Pathologic Processes; Congenital Abnormalities; Signs and Symptoms, Digestive; Gastrointestinal Diseases; Colonic Diseases; Intestinal Diseases; Digestive System Abnormalities; Megacolon; Constipation; Hirschsprung Disease; Long Term Adverse Effects
• Other Study ID Numbers: NanjingCH-01

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: No

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No