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

NCT05461924 | Unknown

• 1 other identifier: NanjingCH-01
• Study Type: observational
• Target: 50
• Locations: 1 country
• Sites: 1

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.

Conditions

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

Keywords

Hirschsprung disease associated enterocolitis; bowel function

Outcome Measures

Primary Outcomes (1)

• 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 Outcomes (1)

• Hirschspurng disease associated entrocolitis(HAEC)

  through study completion, an average of 5 year

Study Arms (2)

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

rectosigmoid HSCR

Aganglionosis confined to the rectosigmoid

Other: questionnaire survey

Interventions

questionnaire survey OTHER

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

long-segment HSCR; rectosigmoid HSCR

Eligibility Criteria

• Age: 3 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• 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.

You may qualify if:

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

You may not qualify if:

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

Sponsors & Collaborators

• Weibing Tang: lead

Study Sites (1)

Children's Hospital of Nanjing Medical University

Nanjing, Jiangsu, 210008, China

RECRUITING

MeSH Terms

Conditions

Constipation; Hirschsprung Disease; Long Term Adverse Effects

Interventions

Surveys and Questionnaires

Condition Hierarchy (Ancestors)

Signs and Symptoms, Digestive; Signs and Symptoms; Pathological Conditions, Signs and Symptoms; Digestive System Abnormalities; Digestive System Diseases; Megacolon; Colonic Diseases; Intestinal Diseases; Gastrointestinal Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Pathologic Processes

Intervention Hierarchy (Ancestors)

Data Collection; Epidemiologic Methods; Investigative Techniques; Health Care Evaluation Mechanisms; Quality of Health Care; Health Care Quality, Access, and Evaluation; Public Health; Environment and Public Health

Study Officials

• Changgui Lu, Dr

  Department of pediatric surgery of Children's hospital of Nanjing medical unverisity

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Weibing Tang, Dr

CONTACT

8613851683700; twbcn@163.com

Changgui Lu, Dr

CONTACT

8613770848448; luchanggui1984@163.com

Study Design

• Study Type: observational
• Observational Model: CASE CONTROL
• Time Perspective: RETROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR INVESTIGATOR
• PI Title: Doctor

Study Record Dates

• First Submitted: July 12, 2022
• First Posted: July 18, 2022
• Study Start: July 15, 2022
• Primary Completion: August 20, 2023
• Study Completion: August 20, 2023
• Last Updated: December 1, 2022

Record last verified: 2022-11

Data Sharing

• IPD Sharing: Will not share

Locations

CN (1)