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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.
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| PSARP group | Patients undergoing posterior sagittal anorectoplasty (PSARP) or its variants (miniPSARP) | ||
| TAP group | Patients undergoing transanal proctoplasty (TAP). |
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| Measure | Description | Time Frame |
|---|---|---|
| Fecal Continence at 3 YEARS | Measured by proportion of children achieving fecal continence according to Krickenbeck score. | 3 years post-surgery |
| Fecal Continence at 6 Years | Measured by proportion of children achieving fecal continence according to Krickenbeck score. | 6 years post-surgery |
| Measure | Description | Time Frame |
|---|---|---|
| Postoperative Complications Within 30 Days | Presence and severity of postoperative complications occurring within 30 days after surgery, classified according to the Clavien-Madadi pediatric surgical complication grading system. | Within 30 days after surgical correction |
| ectal Mucosal Prolapse |
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Inclusion Criteria:
Exclusion Criteria:
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Pediatric patients (<18 years) diagnosed with anorectal malformation with perineal or vestibular fistula undergoing surgical correction between 2020 and 2027.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Riccardo Coletta | Contact | 390555662499 | riccardo.coletta@meyer.it | |
| Lucia Paolini | Contact | 390555662499 | lucia.paolini@meyer.it |
| Name | Affiliation | Role |
|---|---|---|
| Riccardo Coletta | Meyer IRCSS | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Aou Meyer IRCSS | Recruiting | Florence | Florence | 50139 | Italy |
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Occurrence of rectal mucosal prolapse during follow-up, including frequency and need for medical or surgical treatment. |
| From surgery until 6 years of age or achievement of fecal continence (up to 6 years) |
| Quality of Life (HAQL Questionnaire) | 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. | Assessed annually until 6 years of age or achievement of fecal continence |
| Constipation Severity and Bowel Management Requirements | Presence and severity of constipation, including need for dietary measures, laxatives, enemas, or structured bowel management programs, evaluated according to the Krickenbeck criteria. | Assessed annually until 6 years of age or achievement of fecal continence |
| time to Achievement of Fecal Continence | 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). | From date of surgery until continence is achieved or up to 6 years of age |
| ASST Papa Giovanni XXIII | Not yet recruiting | Bergamo | Italy |
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| Ospedale dei Bambini | Not yet recruiting | Brescia | Italy |
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| UOC Chirurgia Pediatrica ARNAS Garibaldi, Catania | Not yet recruiting | Catania | Italy |
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| Chirurgia pediatrica P.O. "V.Fazzi" | Not yet recruiting | Lecce | Italy |
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| Policlinico di Modena | Not yet recruiting | Modena | Italy |
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| Ospedale Maggiore della Carità di Novara | Not yet recruiting | Novara | Italy |
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| Ospedale universitario di Padova | Not yet recruiting | Padova | Italy |
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| Policlinico Universitario Paolo Giaccone di Palermo | Not yet recruiting | Palermo | Italy |
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| Fondazione IRCCS Policlinico San Matteo | Not yet recruiting | Pavia | Italy |
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| PO: Santo Spirito | Not yet recruiting | Pescara | Italy |
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| A.O.R. "S. Carlo" - Potenza | Not yet recruiting | Potenza | Italy |
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| AUSL della Romagna - Ospedale Infermi Rimini | Not yet recruiting | Rimini | Italy |
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| Azienda Ospedaliera S.Camillo-Forlanini | Not yet recruiting | Roma | Italy |
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| Fondazione Policlinico Universitario Agostino Gemelli IRCCS | Not yet recruiting | Roma | Italy |
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| Ospedale Pediatrico Bambino Gesù | Not yet recruiting | Roma | Italy |
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| Aou Senese | Not yet recruiting | Siena | Italy |
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| Ospedale infantile Regina Margherita di Torino | Not yet recruiting | Torino | Italy |
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| S. Antonio Abate - Trapani | Not yet recruiting | Trapani | Italy |
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| Ospedale Filippo Del Ponte - Varese | Not yet recruiting | Varese | Italy |
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| Azienda Ospedaliera Universitaria Verona | Not yet recruiting | Verona | Italy |
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| ID | Term |
|---|---|
| D000071056 | Anorectal Malformations |
| ID | Term |
|---|---|
| D004065 | Digestive System Abnormalities |
| D004066 | Digestive System Diseases |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
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