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

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

UNKNOWN

Total Enrollment

50 participants

Study Classification

OBSERVATIONAL

Study Start Date

2022-07-15

Study Completion Date

2023-08-20

Brief Summary

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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.

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Detailed Description

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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.

Conditions

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

Study Design

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Observational Model Type

CASE_CONTROL

Study Time Perspective

RETROSPECTIVE

Study Groups

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long-segment HSCR

Aganglionosis of non-rectosigmoid HSCR can extend to the descending colon, transverse colon, ascending colon, but not to the terminal ileum

questionnaire survey

Intervention Type OTHER

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

rectosigmoid HSCR

Aganglionosis confined to the rectosigmoid

questionnaire survey

Intervention Type OTHER

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

Interventions

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questionnaire survey

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

Intervention Type OTHER

Eligibility Criteria

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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

Minimum Eligible Age

3 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No