Anorectal Malformations
Conditions
Keywords
Anorectal Malformation, Perineal Fistula, Rectovestibular Fistula
Brief summary
This is a national, multicenter, retro-prospective longitudinal cohort study evaluating mid- and long-term outcomes of different surgical techniques used for the correction of anorectal malformations (ARM) with perineal or vestibular fistula. Patients undergoing surgical correction between 2020 and 2027 will be included. Functional outcomes, particularly fecal continence assessed using the Krickenbeck score, will be evaluated at 3 and 6 years post-surgery or until continence is achieved.
Detailed description
This is a national, multicenter ambispective (retro-prospective) longitudinal cohort study. The study includes patients who underwent surgical correction between 2020 and 2027. Data collected prior to study activation (2020-study start date) will be analyzed retrospectively from medical records, while patients enrolled after study activation will be followed prospectively with annual assessments until achievement of fecal continence or up to 6 years of age, whichever occurs first. Anorectal malformations (ARM) are rare congenital anomalies requiring complex reconstructive surgery. Posterior sagittal anorectoplasty (PSARP) remains the gold standard; however, alternative techniques such as anterior sagittal anorectoplasty (ASARP) and transanal proctoplasty (TAP) are increasingly adopted. This study aims to compare surgical techniques in terms of: Fecal continence (Krickenbeck score) Postoperative complications (Clavien-Madadi classification) Quality of life (HAQL questionnaire) Constipation and bowel management requirements Time to continence achievement Patients will be followed annually until 6 years of age or until continence is reached, whichever occurs first.
Interventions
None listed
Sponsors
Meyer Children's Hospital IRCCS
Study design
Observational model
COHORT
Time perspective
OTHER
Eligibility
Sex/Gender
ALL
Age
0 Days to 18 Years
Healthy volunteers
No
Inclusion criteria
* Pediatric patients (\<18 years) * Diagnosis of anorectal malformation with perineal or vestibular fistula * Surgical correction with PSARP (or variants), ASARP, or TAP * Signed informed consent from parents/legal guardians
Exclusion criteria
* Age \>18 years * Other types of anorectal malformations * Surgical technique other than those specified
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Fecal Continence at 3 YEARS | 3 years post-surgery | Measured by proportion of children achieving fecal continence according to Krickenbeck score. |
| Fecal Continence at 6 Years | 6 years post-surgery | Measured by proportion of children achieving fecal continence according to Krickenbeck score. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Postoperative Complications Within 30 Days | Within 30 days after surgical correction | Presence and severity of postoperative complications occurring within 30 days after surgery, classified according to the Clavien-Madadi pediatric surgical complication grading system. |
| ectal Mucosal Prolapse | From surgery until 6 years of age or achievement of fecal continence (up to 6 years) | Occurrence of rectal mucosal prolapse during follow-up, including frequency and need for medical or surgical treatment. |
| Quality of Life (HAQL Questionnaire) | Assessed annually until 6 years of age or achievement of fecal continence | The HAQL questionnaire evaluates disease-specific quality of life in patients with Hirschsprung's disease and anorectal malformations. The questionnaire generates domain-specific scores and a total score ranging from 0 to 100, where higher scores indicate better quality of life. Validated Italian age-specific versions of the HAQL questionnaire will be used when applicable. |
| Constipation Severity and Bowel Management Requirements | Assessed annually until 6 years of age or achievement of fecal continence | Presence and severity of constipation, including need for dietary measures, laxatives, enemas, or structured bowel management programs, evaluated according to the Krickenbeck criteria. |
| time to Achievement of Fecal Continence | From date of surgery until continence is achieved or up to 6 years of age | Time from surgical correction to achievement of fecal continence, defined according to the Krickenbeck score (voluntary bowel movements, ability to verbalize urge, and absence of significant soiling). |
Countries
Italy
Contacts
CONTACTRiccardo Coletta
CONTACTLucia Paolini
PRINCIPAL_INVESTIGATORRiccardo Coletta
Meyer IRCSS
Outcome results
None listed