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Purpose: To analyze and compare the outcomes in patients with anorectal malformation with rectoprostatic and rectourethral fistula between laparoscopic-assisted anorectoplasty (LAARP) versus posterior sagittal anorectoplasty (PSARP).
Method: We performed a retrospective review on all males with anorectal malformation (ARM) with recto-prostatic (ARM-RP) or recto-bulbar urethral fistula (ARM-RB) treated in five tertiary paediatric surgical centres in the past 25 years. Defecative function was assessed using the Krickenbeck classification and Kelly's score. Functional outcomes between patients with LAARP and PSARP were compared.
Results: There were a total of 136 males with ARM-RP and ARM-RB for analysis, among which 73 (53.7%) had ARM-RP and 63 (46.3%) had ARM-RB. The median age of the patients was 9.4 years (range 0.8-24.7 years) and the median age at operation was 0.4 years (0 day-3.1 years). 57 (41.9%) and 79 patients (58.1%) underwent PSARP and LAARP respectively. 34 patients (25%) had VACTERL association. 111 (81.6%) and 103 patients (75.7%) had sacral and spinal cord anomalies respectively. 19 patients (13.9%) eventually required Malone's Antegrade Continence Enema (MACE). For the comparison between PSARP and LAARP, no difference in Kelly scores (4.58 ± 1.63 versus 4.67 ± 1.36) was identified (p = 0.79). Logistic regression for voluntary bowel movement showed that VACTER association (p = 0.02) and fistula location (p = 0.01) were significant prognostic factors, whereas the operation approach (PSARP or LAARP) was not (p = 0.65).
Conclusion: VACTERL association and fistula location were significant prognostic factors for voluntary bowel movement, and there appeared to be no significant difference in functional outcome between PSARP and LAARP.
Level Of Evidence: IV.
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http://dx.doi.org/10.1016/j.jpedsurg.2024.07.037 | DOI Listing |
Dis Colon Rectum
September 2025
Division of Colon and Rectal Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School.
Background: Individuals born with anorectal and pelvic malformations require lifelong management. Although initially cared for by pediatric providers, these conditions continue to impact patients' health and quality of life into adulthood.
Objective: To assess the prevalence of psychiatric disorders and substance use among adults with congenital colorectal and pelvic malformations, and to explore their distribution across demographic and clinical variables.
J Pediatr Urol
August 2025
Department of Pediatric Surgery, Rigshospitalet, Clinical Medicine, Copenhagen University, Copenhagen, Denmark; Department of Women's and Children's Health, Center for Molecular Medicine, Karolinska Institutet, Stockholm, Sweden. Electronic address:
The European Reference Networks (ERNs) initiative was started by the European Commission. The initiative was launched in 2017 in response to the 2011 EU Directive on Cross-Border Healthcare (Directive 2011/24/EU), which emphasized the need to improve healthcare for patients with rare diseases and complex conditions across the European Union. Currently, 24 ERNs are available for rare diseases.
View Article and Find Full Text PDFJ Pediatr Surg
September 2025
Department of Gastrointestinal and Pediatric Surgery, Oslo University Hospital, Oslo, Norway; Institute of Clinical Medicine, University of Oslo, Norway.
Aim: To identify reported parameters and study characteristics in the literature on antegrade continence enema (ACE) in patients with anorectal malformations (ARM) and Hirschsprung disease (HD).
Methods: Following PRISMA guidelines, we performed a search using the terms "anorectal malformation" or "Hirschsprung disease", combined with "appendicostomy," "antegrade continence enema," "ACE", "Malone", or "cecostomy" in the databases Ovid MEDLINE, Cochrane Central, Embase, and CINAHL. Two reviewers independently screened and extracted data.
Cureus
August 2025
Pediatric Surgery, Manchester University NHS Foundation Trust, Manchester, GBR.
Despite advancements in surgical techniques and improved survival rates for anorectal malformation (ARM) patients, the lack of a standardized way for assessing bowel function and quality of life remains a challenge. Variability in scoring outcomes exists between systems due to inconsistencies in criteria and precise definitions, such as soiling, constipation, and continence. While older tools lack proper validation, newer scales, including the Rintala and Baylor Continence Scale (BCS), offer more structured assessment.
View Article and Find Full Text PDFUrol Case Rep
September 2025
Dept of Paediatric Surgery, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India.
Anorectal malformations (ARM)being the common congenital anomalies occurring in 1 in 4000-5000 newborns. Associated recto urethral fistula being the most common in males and recto vestibular fistula being the common in females. Hence ARMs require staged surgical procedures.
View Article and Find Full Text PDF