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Aim: There is a knowledge gap regarding which patients with anorectal malformations (ARMs) are at highest risk of anorectal prolapse (AP), and which risk factors predispose to AP in ARM. The aims of the study were to define the frequency of AP after ARM reconstruction, and explore risk factors., Method: Data from the ARM-Net registry inserted between 2007 and 2023 were used. Inclusion criteria were the reconstruction performed, no stoma at 1-year follow-up and all data available at 1-year follow-up. The statistics used were univariable and multivariable logistic regression models., Results: After exclusions the incidence of AP was 163 in 1117 patients (14.6%) in data inserted by 31 centres from 12 countries. The AP incidence was unevenly distributed between the centres (interquartile range 6.3%-21.7%). AP was more frequent in boys than girls (20.9% vs. 8.1%; P < 0.001). In both sexes the incidence of AP was higher in complex ARM subtypes (P < 0.001). AP was most frequent after laparotomy- and laparoscopic-assisted reconstructions (50.0% and 37.5%, respectively). Spinal and sacral anomalies constituted risk factors for AP in univariable analyses, while tethered cord did not. Adjusted risk factors for AP were severity of ARM subtype (40% in long-channel cloaca and bladder neck fistula, OR 3.1, 95% CI 1.0-10.2), laparotomy-assisted posterior sagittal anorectoplasty (50%, OR 3.7, 95% CI 1.6-8.4) and larger neo-anus at 1-year follow-up (Hegar 13.6 vs. 13.1; OR 1.2, 95% CI 1.1-1.4). Constipation was not a risk factor for AP., Conclusion: Anorectal prolapse is a frequent postoperative sequela. Adjusted analyses indicate that severity of ARM, abdominal open access during reconstruction and larger size of anus are risk factors., (© 2025 The Author(s). Colorectal Disease published by John Wiley & Sons Ltd on behalf of Association of Coloproctology of Great Britain and Ireland.)

Anorectal malformations (ARM) are rare congenital anomalies with an overall prevalence of 3.32 per 10,000 pregnancies. ARM describe a spectrum of anomalies of the anus and rectum ranging from a minimally displaced anal canal to a complete fusion of the anorectum, vagina and urethra with hypoplastic sphincter and pelvic floor muscle. Aberrant septation of the hindgut with anomalous cloacal membrane during weeks 6 to 9 of gestation form the developmental basis for a spectrum of anomalies defined as ARM. Although underlying specific syndromes and occasional familiar occurrence suggest genetic aetiology, most ARM are non-syndromic and their causal genetic mechanisms and non-genetic insults remain unclear. ARM is a clinical diagnosis, generally made early after birth via careful inspection of the perineum. Prenatal detection remains rare, and modern technical developments have added little to prenatal diagnostics. ARM is corrected surgically. Since its introduction in 1982, posterior sagittal anorectoplasty is the most common surgery for ARM reconstruction. Subsequent surgical adaptations focus on minimizing iatrogenic operative injury by limiting surgical invasiveness. They include laparoscopic procedures and shortening of incisions with confined dissection in open surgery. Although outcomes in patients with ARM have evolved throughout the past decades, there is urgent need for further improvements both in functional outcomes and quality of life. The importance of psychosocial experiences of affected patients is increasingly recognized. Continued research is necessary to improve prenatal detection, to elucidate genetic and epigenetic alterations and to refine optimal surgical procedures., Competing Interests: Competing interests: The authors declare no competing interests., (© 2024. Springer Nature Limited.)

Background: Genital malformations are frequently diagnosed in patients with VACTERL, but are currently not included in the acronym. This study aimed to analyze the frequency of genital anomalies in patients with esophageal atresia (EA) and/or anorectal malformation (ARM), with a subgroup analysis of children fulfilling the VACTERL criteria., Method: This was a cross-sectional retrospective analysis of two prospectively collected registries of patients operated on for ARM and EA between 2012 and 2022 at a specialized national center. Children were screened routinely for malformations according to the VACTERL acronym., Results: A total of 174 children were included in the study. VACTERL was diagnosed in 60 children (34%), while 114 children (66%) were defined as non-VACTERL. Genital malformations were diagnosed in 38% (23/60) of the children with VACTERL, and in 11% (13/114) of the children without VACTERL (p < 0.001). The presence of genital malformations correlated linearly with the number of diagnosed component features (CFs). In boys with VACTERL, the most common genital malformation was undescended testes present in 10/27 (21%) compared to 1/71 (1%) in non-VACTERL boys (p < 0.001). Müllerian duct anomalies were found in 26% of girls with VACTERL vs. 7% in non-VACTERL girls (p < 0.05)., Conclusion: There was a higher frequency of genital malformations in patients with VACTERL emphasizing the importance of genital assessment for these patients. We propose VACTERL-G as an extension of the current acronym aiming to reduce the risk of long-term morbidity due to delayed diagnosis of reproductive anomalies., Competing Interests: Conflicts of interest None., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Vesico-ureteral reflux (VUR) is a common associated urological anomaly in anorectal malformation (ARM)-patients. High-grade VUR requires antibiotic prophylaxis to prevent urinary tract infections (UTI's), renal scarring and -failure. The exact prevalence of high-grade VUR in ARM patients is unknown. Hence, the aim of this study was determining the incidence of high-grade VUR in ARM-patients, and its associated risk factors., Methods: A multicenter retrospective cohort study was performed using the ARM-Net registry, including data from 34 centers. Patient characteristics, screening for and presence of renal anomalies and VUR, sacral and spinal anomalies, and sacral ratio were registered. Phenotypes of ARM were grouped according to their complexity in complex and less complex. Multivariable analyses were performed to detect independent risk factors for high-grade (grade III-V) VUR., Results: This study included 2502 patients (50 % female). Renal screening was performed in 2250 patients (90 %), of whom 648 (29 %) had a renal anomaly documented. VUR-screening was performed in 789 patients (32 %), establishing high-grade VUR in 150 (19 %). In patients with a normal renal screening, high-grade VUR was still present in 10 % of patients. Independent risk factors for presence of high-grade VUR were a complex ARM (OR 2.6, 95 %CI 1.6-4.3), and any renal anomaly (OR 3.3, 95 %CI 2.1-5.3)., Conclusions: Although renal screening is performed in the vast majority of patients, only 32 % underwent VUR-screening. Complex ARM and any renal anomaly were independent risk factors for high-grade VUR. Remarkably, 10 % had high-grade VUR despite normal renal screening. Therefore, VUR-screening seems indicated in all ARM patients regardless of renal screening results, to prevent sequelae such as UTI's, renal scarring and ultimately renal failure., Type of Study: Observational Cohort-Study., Level of Evidence: III., Competing Interests: Conflict of interest None., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Introduction:  To investigate the current experience of the ARM-Net Consortium in the management of epididymo-orchitis (EO) in patients with anorectal malformations (ARMs), and to identify specific risk factors and the need for urological care involvement., Materials and Methods:  We retrospectively collected data of EO in patients with ARM between 2015 and 2019. Data on urological aspects, ARM type, surgical approach, associated anomalies, diagnosis, and treatment of EO were collected and analyzed., Results:  Twenty-nine patients were reported by 12 centers. Twenty-six patients with EO (90%) had ARM with a rectourinary fistula. Median age at first EO was 2 years (range: 15 days-27 years). Twenty patients (69%) experienced multiple EO, and 60% of recurrences were ipsilateral. Associated urological anomalies included vesicoureteral reflux (48%), urethral anomalies (41%), neurogenic bladder (41%), and ectopic vas (10%). A positive urine culture during EO was present in 69%. EO was treated with antibiotics (90%), limiting surgical exploration to 14%. Prevention of recurrences included surgery (bulking agents 15%, vasectomy 15%, and orchiectomy 5%) and antibiotic prophylaxis (20%)., Conclusion:  Urologists may encounter patients with EO in ARM patients, frequently with positive urine culture. An appropriate urologic work-up for most ARM patients is necessary to identify and treat underlying risk factors. A practical scheme for the work-up is suggested for a close collaboration between pediatric surgeons and urologists., Competing Interests: None declared., (Thieme. All rights reserved.)

Background Abdominal scarring in patients with anorectal malformations (ARM) is a permanent visible sign of a chronic congenital condition. The study's aims were to assess the physical and psychosocial significance of abdominal scarring in ARM and to propose a scar treatment approach. Methods A patient- and observer reported cross-sectional study of ARM patients with previous colostomies surgically treated 1997-2015 with minimum 4 years' follow-up after stoma closure. A maximum of 3 patient-selected scars per patient were evaluated by a) the Patient and Observer Scar Assessment Scale (POSAS; 11= no symptoms, 110=worst symptoms), b) pictorial scar treatment assessment by a plastic surgeon, c) questionnaire evaluation of the scarring's psychosocial aspects, bowel- and urinary function and quality of life. Descriptive statistics were presented in median (range) and percent (%), and Pearson's r was used to evaluate linear correlations. Ethical approval and written consents were obtained. Results Twenty-seven patients (48% females) aged 12 (5-24) years old were included. Two (1-3) scars were evaluated per patient with a median POSAS score of 44 (15-78). Six patients (22%) reported scar pain, five (19%) scar pruritus and nine (33%) affected behavior, e.g. always wearing full-covered clothing in public places. Higher (worse) POSAS score and increasing age correlated (r = 0.40, p = 0.04). According to the plastic surgeon's assessment, 21 patients (78%) were suitable candidates for surgical scar treatment, among whom eight patients (30%) requested scar treatment. Conclusions Postoperative abdominal scarring should require attention in clinical ARM follow-up programs including potential corrective plastic surgery for selected patients., Competing Interests: Declaration of interests None., (Copyright © 2021. Published by Elsevier Inc.)

Background: Outcome of patients operated for anorectal malformation (ARM) type rectovestibular fistula (RVF) is generally considered to be good. However, large multi-center studies are scarce, mostly describing pooled outcome of different ARM-types, in adult patients. Therefore, counseling parents concerning the bowel function at early age is challenging. Aim of this study was to evaluate bowel function of RVF-patients at preschool/early childhood age and determine risk factors for poor functional outcome., Methods: A multi-center cohort study was performed. Patient characteristics, associated anomalies, sacral ratio, surgical procedures, post-reconstructive complications, one-year constipation, and Bowel Function Score (BFS) at 4-7 years of follow-up were registered. Groups with below normal (BFS < 17; subgroups 'poor' ≤ 11, and 'fair' 11 < BFS < 17) and good outcome (BFS ≥ 17) were formed. Univariable analyses were performed to detect risk factors for outcome., Results: The study included 111 RVF-patients. Median BFS was 16 (range 6-20). The 'below normal' group consisted of 61 patients (55.0%). Overall, we reported soiling, fecal accidents, and constipation in 64.9%, 35.1% and 70.3%, respectively. Bowel management was performed in 23.4% of patients. Risk factors for poor outcome were tethered cord and low sacral ratio, while sacral anomalies, low sacral ratio, prior enterostomy, post-reconstructive complications, and one-year constipation were for being on bowel management., Conclusions: Although median BFS at 4-7 year follow-up is nearly normal, the majority of patients suffers from some degree of soiling and constipation, and almost 25% needs bowel management. Several factors were associated with poor bowel function outcome and bowel management., Level of Evidence: Level III., Competing Interests: Declarations of Competing Interest None., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Surgical safety during posterior sagittal anorectal plasty (PSARP) for anorectal malformations (ARM) depends on accurate pre-operative fistula localization. This study aimed to evaluate accuracy of pre-operative fistula diagnostics., Methods: Ethical approval was obtained. Diagnostic accuracy of pre-PSARP symptoms (stool in urine, urine in passive ostomy, urinary tract infection) and examination modalities (voiding cystourethrogram (VCUG), high-pressure colostogram, cystoscopy and ostomy endoscopy) were compared to final intra-operative ARM-type classification in all male neonates born with ARM without a perineal fistula treated at a tertiary pediatric surgery center during 2001-2020., Results: The 38 included neonates underwent reconstruction surgery through PSARP with diverted ostomy. Thirty-one (82%) had a recto-urinary tract fistula and seven (18%) no fistula. Ostomy endoscopy yielded the highest diagnostic accuracy for fistula presence (22 correctly classified/24 examined cases; 92%), and pre-operative symptoms the lowest (21/38; 55%). For pre-operative fistula level determination, cystoscopy yielded the highest diagnostic accuracy (14/20; 70%), followed by colostogram (23/35; 66%), and VCUG (21/36; 58%). No modality proved to be statistically superior to any other., Conclusions: Ostomy endoscopy has the highest diagnostic accuracy for fistula presence, and cystoscopy and high-pressure colostogram for fistula level determination. Correct pre-operative ARM-typing reached a maximum of 60-70%.

Background: The impact of perioperative care interventions on postreconstructive complications and short-term colorectal outcome in patients with anorectal malformation (ARM) type rectovestibular fistula is unknown., Methods: An ARM-Net consortium multicenter retrospective cohort study was performed including 165 patients with a rectovestibular fistula. Patient characteristics, perioperative care interventions, timing of reconstruction, postreconstructive complications and the colorectal outcome at one year of follow-up were registered., Results: Overall complications were seen in 26.8% of the patients, of which 41% were regarded major. Differences in presence of enterostomy, timing of reconstruction, mechanical bowel preparation, antibiotic prophylaxis and postoperative feeding regimen had no impact on the occurrence of overall complications. However, mechanical bowel preparation, antibiotic prophylaxis ≥48 h and postoperative nil by mouth showed a significant reduction in major complications. The lowest rate of major complications was found in the group having these three interventions combined (5.9%). Multivariate analyses did not show independent significant results of any of the perioperative care interventions owing to center-specific combinations. At one year follow-up, half of the patients experienced constipation and this was significantly higher among those with preoperative mechanical bowel preparation., Conclusions: Differences in perioperative care interventions do not seem to impact the incidence of overall complications in a large cohort of European rectovestibular fistula-patients. Mechanical bowel preparation, antibiotic prophylaxis ≥48 h, and postoperative nil by mouth showed the least major complications. Independency could not be established owing to center-specific combinations of interventions., Type of Study: Treatment study., Level of Evidence: III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Aim of the Study: To assess the frequency of and identify contributing factors to wound dehiscence after posterior sagittal anorectoplasty (PSARP) in children born with anorectal malformations (ARM)., Methods: Ethical approval was obtained (DNR 2017/191). Charts of all children with anorectal malformations (ARM) reconstructed with PSARP, limited PSARP, or PSARVUP at a tertiary centre of paediatric surgery between 2001 and 2016 were reviewed. Wound dehiscence within 30 days postoperatively was analysed regarding gender, prematurity, birth weight, type of ARM, other congenital malformations, single- or multistaged reconstruction, age and weight at reconstruction, postoperative antibiotics, and fasting. Multiple regression analysis was performed for risk factors in single-stage PSARP or limited PSARP, presented as odds ratio (OR) with 95% confidence interval (CI)., Main Results: Ninety patients were included, of which 53 (59%) were males. Single-staged PSARP was performed in 40 (44%) patients and 50 (56%) had a multistaged reconstruction with a colostomy. Wound dehiscence was significantly more common among patients without a colostomy; 17 (43%) vs. 11 (22%) (p=0.043). In patients with single-stage PSARP, no single factor was identified to increase the risk for wound dehiscence: cardiac malformations (OR 3.73) (95% CI 0.78-17.88), low weight at surgery (OR 1.56) (95% CI 0.36-6.99), antibiotics < 1 day (OR 1.6) (95% CI 0.43-5.94), or short fasting 0-3 days (OR 4.44) (95% CI 0.47-42.18)., Conclusions: A divided colostomy protected against wound dehiscence after PSARP. No risk factor for wound dehiscence after single-staged PSARP was identified. Further studies are needed to establish contributing factors to uncomplicated wound healing after PSARP.

Aim: The aim was to describe the frequency of lower urinary tract symptoms (LUTS) in children with anorectal malformations with rectoperineal fistulas (ARM-P), as compared with healthy controls based on gender., Method: LUTS were defined using the 2014 definitions of the International Children's Continence Society. Data were collected at 2 tertiary pediatric surgery centers in 2 countries from all children aged 4-12years who had undergone an operation for ARM-P., Results: A total of 24 girls and 33 boys, with a median age of 8 (4-12)years, were eligible and compared with 165 controls. Of the patient group, 4 (17%) girls had 8 urinary tract anomalies (UTA), and 8 (24%) boys had 13 UTA. There were no gender differences in LUTS among the patients. The frequency of urinary tract infections was higher among the patients (5/24 girls and 7/55 boys) than the controls (1/55 and 4/110) (p=0.009). More patients (5/24 girls and 5/33 boys) than controls (1/55 and 2/110) used daily urinary medications (p=0.009 and p=0.007, respectively). Patients with UTA reported urinary infections more frequently (3/4 girls and 4/8 boys) than those without UTA (2/20 girls and 0/25 boys) (p=0.018 and p=0.002, respectively)., Conclusion: Children with ARM-P had more LUTS than controls, and patients with concomitant UTA had more LUTS than patients without UTA. Therefore, children with ARM-P are suggested to have routine follow-up for both UTA and LUTS., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background Advice on the mode of delivery to females born with anorectal malformation (ARM) is needed. The primary aim was to evaluate the anatomy of the pelvic floor muscles in females with ARM operated with posterior sagittal anorectal plasty (PSARP). The second aim was to correlate the extent of muscle defects to the bowel symptoms. Methods This interventional study with perineal 4D/3D ultrasonography describes the smooth muscles in the intestinal wall (neo-IAS), external sphincter, levators and anal canal using a muscle score (0–6 worst). The bowel symptoms were prospectively registered with Krickenbeck criteria score (0–7 worst). Results Forty females with different subtypes of ARM, median age 13 (4–21), were followed up regarding bowel symptoms. Seventeen were examined with ultrasonography. Bowel symptoms were similar for those examined with ultrasonography and those not, median score 5 and 3 (1–7) respectively, (p = 0.223, Fisher’s exact test). All the females had at least one muscular defect. There was no significant correlation between muscle defects and bowel symptoms (p = 0.094, Spearman’s correlation). Conclusion Females with ARM have considerable defects in the pelvic floor without any significant correlation to bowel symptoms. All women with ARM would benefit from individualized predelivery evaluations and caesarian section should be considered. [ABSTRACT FROM AUTHOR]