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Bowel dysfunction has been proven as the most common complication after pull-through(PT) of Hirschsprung disease(HD) ，which may persist to adulthood and lead to social problems.The reason of bowel dysfunction is complicated and the risk factors were not defined.
Hirschsprung disease (HD) is a distinctive congenital disease with the absence of ganglion cells in the distal intestine leads to distal bowel obstruction and defecation disorders. HD is a rare disease, with a reported incidence of 1:5,000, requiring proper surgical treatment for the maintenance of normal or near-normal bowel movement.To date, there is insufficient evidence to recommend a preferred or superior method for the surgical repair, and a pull-through with or without laparoscopy was the most common procedure for correction of HD,which may have faster recovery and less rate of complication compared to other surgical approaches.The short or long-term outcomes of HD have been deeply assessed in the past few years,and bowel dysfunction,consist of soling, incontinence and constipation, had been proven as the most common complication,which may be a ongoing issue and lead to social problems,which should not be ignored. Otherwise,there were many reports proved that poor bowel function in preschool and early childhood may lead to social problems and depression in adolescence or adult.Many factors may lead to bowel dysfunction in HD, including length of aganglionic segement,anastomotic leakage or redo-PT,unsuitable timing of surgery,etc.Thus,the present study was designed to seek the risk factors for bowel dysfunction of HD,which is meaningful to maintain or improve the bowel function in preschool and early childhood.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Dysfunction group | Bowel function score(BFS,total 20 points) was approved by Rintala in 1995 and patients with a score < 17 were considered to have bowel dysfunction. |
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| near-normal group | Bowel function score(BFS,total 20 points) was approved by Rintala in 1995 and patients with a score > 17 were considered to have normal bowel habits. |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| bowel function score(BFS) questionnaire survey | Other | A cross-sectional bowel function score(BFS) questionnaire survey was conducted in patients with HD who underwent PTs between January 2014 and December 2019 at Children's Hospital of Nanjing Medical University.Patients consented to the survey were asked to complete the questionnaire,and were divided into two groups: dysfunction group(BFS<17 ); and normal group (BFS>=17 ). The operative age, birth weight, premature delivery status, operative weight, aganglionic segment, single or stage PT, surgical approach(laparotomy or laparoscopy), post-operative Hirschsprung-associated enterocolitis (HAEC),and immediate complications were recorded and compared between two groups。Both univariate and multivariate regression analysis were used to seek the risk factors of bowel dysfunction after PTs in HD. . |
| Measure | Description | Time Frame |
|---|---|---|
| bowel function score | Bowel function score(BFS,total 20 points) was approved by Rintala in 1995 and patients with a score > 17 were considered to have normal bowel habits. 7 items were in BFS,including ability to hold back defecation,feels/reports the urge to defecate,frequency of defecation,soiling,accidents,constipation. | through study completion, an average of 5 year |
| Clavien-Dindo classifification of surgical complications | Clavien-Dindo is an objective grading system, which is used for grading postoperative complications in a reproduceable manner.It consists of five grades ranging from any deviation from a normal postoperative course (Grade I) to death (Grade V) | through study completion, an average of 5 year |
| Hirschsprung disease associated enterocolitis(HAEC) | The guidelines for the diagnosis and management of HAEC were defined by the American Pediatric Surgical Association in 2017. There are three types of HAEC defined by severity: possible HAEC [grade I]; definite HAEC [gradeâ…¡]; and severe HAEC [grade â…¢]. | through study completion, an average of 5 year |
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Inclusion Criteria:
Exclusion Criteria:
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Patients with HD who underwent PTs between January 2014 and December 2019 at Children's Hospital of Nanjing Medical University.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Changgui Lu, Dr | Contact | 13770848448 | luchanggui1984@163.com |
| Name | Affiliation | Role |
|---|---|---|
| Weibing Tang, Dr | Children's Hospital of Nanjing Medical University | Study Director |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Children's Hospital of Nanjing Medical University | Recruiting | Nanjing | Jiangsu | 210008 | China |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 40634926 | Derived | Pan S, Li W, Shi C, Tang W, Lu C. Features of defecation dysfunction among patients with Hirschsprung disease in early childhood. BMC Gastroenterol. 2025 Jul 9;25(1):510. doi: 10.1186/s12876-025-04106-4. |
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All data generated or analyzed during this study are available from the corresponding authors upon reasonable request.
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| ID | Term |
|---|---|
| D007410 | Intestinal Diseases |
| D006627 | Hirschsprung Disease |
| D003248 | Constipation |
| ID | Term |
|---|---|
| D005767 | Gastrointestinal Diseases |
| D004066 | Digestive System Diseases |
| D004065 | Digestive System Abnormalities |
| D008531 | Megacolon |
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| D003108 | Colonic Diseases |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D012817 | Signs and Symptoms, Digestive |
| D012816 | Signs and Symptoms |
| D013568 | Pathological Conditions, Signs and Symptoms |