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

Study Overview
=================
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.

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.

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

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

Intervention / Treatment
-----------------
* Other: questionnaire survey


Participation Criteria
=================
Eligibility 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

Ages Eligible for Study
-----------------
Minimum Age: 3 Years

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| long-segment HSCR<br>Aganglionosis of non-rectosigmoid HSCR can extend to the descending colon, transverse colon, ascending colon, but not to the terminal ileum | Other: questionnaire survey<br>* questionnaire survey the long term outcomes of long-segment HSCR and compared with rectosigmoid HSCR<br>|
| rectosigmoid HSCR<br>Aganglionosis confined to the rectosigmoid | Other: questionnaire survey<br>* questionnaire survey the long term outcomes of long-segment HSCR and compared with rectosigmoid HSCR<br>|

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 | 

 Terms related to the study
=================
Keywords Provided by Centre Hospitalier Valida
-----------------
Hirschsprung disease associated enterocolitis, bowel function