Clinical trial NCT05461924: The Long Term Outcomes After Pull-through of Long Segment Hirschsprung Disease
Official title: The Long Term Outcomes After Pull-through of Long Segment Hirschsprung Disease
Study type: OBSERVATIONAL
Overall status: UNKNOWN
Conditions: Constipation
Incontinence
Hirschsprung Disease, Long-Segment
Long Term Adverse Effects
Keywords: Hirschsprung disease associated enterocolitis
bowel function
Lead sponsor: Nanjing Children's Hospital
Sponsor class: OTHER
First posted: 2022-07-18
Enrollment: 50
Sex: ALL
Minimum age (years): 3
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: CASE_CONTROL
Time perspective: RETROSPECTIVE
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.

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.

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

Primary outcomes:
measure: Bowel function score
timeFrame: through study completion, an average of 5 year
Secondary outcomes:
measure: Hirschspurng disease associated entrocolitis(HAEC)
timeFrame: through study completion, an average of 5 year
Other outcomes:
Locations:
city: Nanjing
country: China
facility: Children's Hospital of Nanjing Medical University
state: Jiangsu
Study officials:
affiliation: Department of pediatric surgery of Children's hospital of Nanjing medical unverisity
name: Changgui Lu, Dr
role: PRINCIPAL_INVESTIGATOR
References: