The Long Term Outcomes After Pull-through of Long Segment Hirschsprung Disease
Study Details
Study Description
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.
| Condition or Disease | Intervention/Treatment | Phase |
|---|---|---|
|
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 Design
Arms and Interventions
| Arm | 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
|
Outcome Measures
Primary Outcome Measures
- Bowel function score [through study completion, an average of 5 year]
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.
Secondary Outcome Measures
- Hirschspurng disease associated entrocolitis(HAEC) [through study completion, an average of 5 year]
The guidelines for the diagnosis and management of HAEC were defined by the American Pediatric Surgical Association in 2017
Eligibility Criteria
Criteria
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
Contacts and Locations
Locations
| Site | City | State | Country | Postal Code | |
|---|---|---|---|---|---|
| 1 | Children's Hospital of Nanjing Medical University | Nanjing | Jiangsu | China | 210008 |
Sponsors and Collaborators
- Weibing Tang
Investigators
- Principal Investigator: Changgui Lu, Dr, Department of pediatric surgery of Children's hospital of Nanjing medical unverisity
Study Documents (Full-Text)
None provided.More Information
Publications
None provided.- NanjingCH-01