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

Weibing Tang

Status: Enrolling

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

Treatments: Other: questionnaire survey

Study type: Observational

Funder types: Other

Identifiers

NCT05461924

NanjingCH-01

Details and patient eligibility

About

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.

Full 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.

Enrollment: 50 estimated patients

Sex: All

Ages: 3+ years old

Volunteers: No Healthy Volunteers

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

Trial design

50 participants in 2 patient groups

long-segment HSCR

Description:

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

Treatment:

Other: questionnaire survey

rectosigmoid HSCR

Description:

Aganglionosis confined to the rectosigmoid

Treatment:

Other: questionnaire survey