OBJECTIVE: 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).
METHODS: 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).
CONCLUSIONS: 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.
METHODS: IV.

BACKGROUND: Fecal incontinence is a common problem for children with repaired anorectal malformations (ARM) and has significant implications for initiating school. While sex, anatomy, and medical comorbidities are known to influence continence outcomes, the impact of socioeconomic factors and neighborhood-level disadvantage are less well understood.
METHODS: We performed a single-center retrospective review of all school-aged (5-18 years) children with ARM at a longitudinal pediatric surgery clinic. Demographic, clinical, and socioeconomic variables were abstracted via chart review and geocoding was performed to obtain Area Deprivation Index (ADI) and Social Vulnerability Index (SVI) scores. Statistical analyses assessed for associations between the primary outcome of social continence (defined as no diaper usage and infrequent fecal accidents at age 5) and these variables.
RESULTS: 72 patients were included; of these, 45.8% were socially continent. On bivariate analysis, social continence was significantly associated with state ADI score as well as the SVI Housing characteristics score. These associations remained significant when adjusting for sex and medical comorbidities in separate multiple logistic regression models.
CONCLUSIONS: The relative disadvantage of the neighborhood in which a child with ARM lives may play a role in their ability to achieve continence by school age. Efforts are warranted to identify and develop targeted interventions to for this pediatric population.
METHODS: IV.

OBJECTIVE: To describe the long-term bowel function of anorectal malformation (ARM) patients and explore the potential influence factors.
METHODS: ARM patients with follow-up data > 10 years were included. Cases of cloaca, Currarino syndrome, and VACTERL syndrome were excluded. Rintala score and PedsQL 4.0 were used to assess bowel function score (BFS) and quality of life (QoL). Based on the results, patients were divided into satisfactory group with BFS ≥ 17 and unsatisfactory group with it < 17. Comparisons between the groups were made.
RESULTS: Among the 81 patients were 44 males and 37 females. Follow-up time was 138 (126,151) months. 16 (19.75%) patients had associated anomalies. 23 (28.40%) patients had reoperations, and fistula recurrence was the most common reason. BFS of the patients was 20 (18,20). QoL score was 100 (100,100), which correlated positively with BFS (r = 0.648, P < 0.001). The satisfactory and the unsatisfactory groups had 69 and 12 cases, and their BFS were 20 (20,20) and 11 (8,15) respectively, which had statistical difference (P < 0.001). Total QoL score and psycho-social health score of the unsatisfactory group were lower (P < 0.001). Only reoperations were statistically different between the groups (P < 0.001).
CONCLUSIONS: Long-term (> 10 years) bowel function of ARM patients is good in this study. Defecation problems have negative impacts on QoL and mainly affects their psycho-social health. Primary anorectoplasty is extremely important. Reoperations, which are most commonly seen in recto-urethral fistula recurrence, adversely affect the outcome.

BACKGROUND: This study describes the presentation and initial management of anorectal malformation (ARM); evaluating the frequency, causes and consequences of late diagnosis.
METHODS: A prospective, population cohort study was undertaken for newly diagnosed ARMs in the UK and Ireland from 01/10/2015 and 30/09/2016. Follow-up was completed at one year. Data are presented as n (%), appropriate statistical methods used. Factors associated with late diagnosis; defined as: detection of ARM either following discharge or more than 72 h after birth were assessed with univariable logistic regression.
RESULTS: Twenty six centres reported on 174 cases, 158 of which were classified according to the type of malformation and 154 had completed surgical data. Overall, perineal fistula was the most commonly detected anomaly 43/158 (27%); of the 41 of these children undergoing surgery, 15 (37%) had a stoma formed. 21/154 (14%, CI95{9-20}) patients undergoing surgery experienced post-operative complications. Thirty-nine (22%) were diagnosed late and 12 (7%) were detected >30 days after birth. Factors associated with late diagnosis included female sex (OR 2.06; 1.0-4.26), having a visible perineal opening (OR 2.63; 1.21-5.67) and anomalies leading to visible meconium on the perineum (OR 18.74; 2.47-141.73). 56/174 (32%) had a diagnosis of VACTERL association (vertebral, anorectal, cardiac, tracheal, oesophageal, renal and limb); however, not all infants were investigated for commonly associated anomalies. 51/140 (36%) had a cardiac anomaly detected on echocardiogram.
CONCLUSIONS: There is room for improvement within the care for infants born with ARM in the UK and Ireland. Upskilling those performing neonatal examination to allow timely diagnosis, instruction of universal screening for associated anomalies and further analysis of the factors leading to clinically unnecessary stoma formation are warranted.
METHODS: II (Prospective Cohort Study <80% follow-up).

BACKGROUND: Continence issues due to organic causes including previous colorectal surgery or neurological issues might benefit from Transanal irrigation (TAI) that proved to be highly effective but with a number of limitations including a relatively high discontinuation rates. Our study was aimed at evaluating the efficacy of an advanced protocol tailored to each patient to prevent dropout and increase satisfaction, independence, and quality of life.
METHODS: This was a prospective, interventional, multicenter, nonrandomized study involving children aged 4-18 years with bowel dysfunction unresponsive to conventional treatments who required TAI. TAI was performed in accordance to the best standards of care with a total irrigation volume that was determined based on low emission X-Ray barium enemas performed at the very beginning of the study. All patients underwent training and assessments of continence, patients\' perspectives and quality of life were performed at different timepoints from enrollment (T0) up to 6 months since TAI was introduced (T3).
RESULTS: A total of 78 patients were enrolled. Male to female ratio was 1.4:1. Mean age at enrollment was 106.1 ± 42.8 months. Discontinuation was reported by 3 patients (3.8 %). Continence, satisfaction and a number of other outcome measures increased from baseline (T0) to the last visit (T3). In particular, mean Rintala total score increased linearly from 7.8 to 14.8 during the study period (T0 to T3 timepoints). On a multivariate analysis, the only parameter that proved to be inversely associated with continence as well as with other outcome measures was the use of laxatives at enrollment and during the study.
CONCLUSIONS: This study has demonstrated the high efficacy of this innovative patient-tailored TAI protocol across all assessed scores. Of note, given the negative impact of laxatives, our findings suggest limiting their use in this patient population to further increase the efficacy of the procedure.

BACKGROUND: Cloacal exstrophy (CE) represents a rare sub-group of anorectal malformations. Traditionally managed with a permanent colostomy, colonic pull-through (PT) has emerged to allow cleanliness without a life-long stoma. We sought to understand outcomes of PT in a large multi-center CE population.
METHODS: We performed a retrospective study involving eleven pediatric hospitals. We gathered data on demographics, outcomes, and anatomical factors including colon length. Continuous variables were analyzed with Wilcoxon rank-sum tests and categorial variables with Fisher\'s exact tests.
RESULTS: There were 98 patients, of which the majority (n = 70, 71.4 %) never underwent PT. There were no differences in exstrophy type, demographics, or associated anomalies. Median age at PT was 1.3 years (IQR 0.3-3.7). Of the cohort that continue to use their PT, the majority (n = 16, 69.6 %) are not clean. In total, 7.1 % (n = 7) of the cohort is clean with a PT, and only one patient is continent. Clean patients have a longer colon length than those who are not clean or opt for re-do ostomy (64.0 cm [IQR 46.0-82.0] vs 26.5 cm [IQR 11.6-41.2], p = 0.005).
CONCLUSIONS: Overall, we demonstrate that most children born with CE will keep their stoma. Only a small percentage who elect to undergo colonic PT are clean for stool. Greater colon length correlates with success. This suggests that multiple factors, including colon length, are important when considering PT in a child with CE.
METHODS: III.

Limited data on the survival of anorectal malformation (ARM) patients from lower- and middle-income countries is available. This retrospective population-based study from the State of Johor, Malaysia, determines the incidence, mortality rate, and survival of ARM patients and factors associated with mortality. Kaplan-Meier survival analysis was used to estimate the survival of ARM patients at 1, 5, and 10 years. In addition, multivariate Cox regression analysis was used to analyze mortality-related factors. There were 175 ARM patients among 803,850 live births, giving an overall ARM incidence of 2.2 (95% confidence interval [CI], 1.9 to 2.5) per 10,000 live births. The male-to-female ratio was 1.5:1. There were 122 (69%) non-isolated ARM, of which 41 were Trisomy-21 and 34 had VACTERL association. Seventy-three (42%) had congenital heart disease (CHD), with 38 severe and 35 non-severe CHD. Overall, 33 (19%) patients died, with a median age of death of 5.7 months (interquartile range (IQR) 25 days to 11.2 months). The overall estimated 1-, 5-, and 10-year survival rate for ARM patients was 82% (95% CI, 76-89%), 77% (95% CI, 70-84%), and 77% (95% CI, 70-84%), respectively. Univariate analysis shows that non-isolated ARM, VACTERL association, and severe CHD were associated with mortality. However, only severe CHD is the independent factor associated with mortality, with a hazard ratio of 4.0 (95% CI, 1.9-8.4).  Conclusion: CHD is common among ARM patients, and one in five ARM patients had a severe cardiac defect, significantly affecting their survival. What is Known: • VACTERL association and congenital heart disease are common in patient with anorectal malformation. • Low birth weight and prematurity are associated with a lower rate of survival. What is New: • Congenital heart disease is common in ARM patients in a middle-income country. • Severe congenital heart disease plays a significant role in the survival of patients with an anorectal malformation in lower- and middle-income countries.

Congenital anorectal malformation (ARM) is a diverse group of anomalies affecting the development of the anal and rectal regions, with an estimated incidence of one in every 5000 live births. The colostomy is commonly performed as part of the staged management of children with ARM to prevent complications. However, the presence of acute watery diarrhea in children with ARM and colostomy poses significant management challenges due to the altered anatomy and physiology affecting stool regulation and absorption, exacerbated by various factors including infections, dietary issues, medication side effects, and underlying gastrointestinal complications.This case study explores the complexities involved in managing acute watery diarrhea in children with congenital ARM and colostomy. A comprehensive literature review was conducted to examine the existing evidence on the subject. The study highlights the multidisciplinary approach required, involving pediatricians, surgeons, and other specialists, to provide comprehensive care and support for these children. Effective management of acute watery diarrhea in children with congenital ARM and colostomy necessitates collaboration between pediatricians and surgeons. Pediatricians play a crucial role in assessing hydration status, monitoring electrolyte balance, and providing appropriate fluid and nutritional management. Surgeons address the surgical aspects of care and coordinate interventions with the management of acute diarrhea. The study underscores the importance of a multidisciplinary approach to deliver comprehensive care, optimize outcomes, and improve the quality of life for affected children. The management of acute watery diarrhea in children with congenital ARM and colostomy presents significant challenges due to the complex interplay of anatomical, physiological, and clinical factors. A multidisciplinary approach involving pediatricians, surgeons, and other specialists is vital for providing comprehensive care and support. This case study emphasizes the need for further research, guidelines, and collaborative efforts to enhance the management strategies for this vulnerable population.

OBJECTIVE: In girls born with an anorectal malformation (ARM), anatomical gynecological anomalies (GA) may be present and might need treatment. Therefore, the aim of this study was to provide an overview of GA in girls born with ARM in our cohort. Additionally, diagnostic timing and methods for GA were assessed.
METHODS: A retrospective mono-center study was performed from January 2000 to December 2022. All patients assigned female at birth were eligible for inclusion. GA were classified according to ESHRE/ESGE classification. Outcomes were the number of girls with GA with subsequent screening methods, factors associated with GA, and GA requiring treatment. Uni- and multivariable logistic regression analyses were performed to identify the association between baseline characteristics and the presence of GA.
RESULTS: In total, 128 girls were included, of whom 30 (24.1%) had additional GA, with vaginal anomalies being present most often (n = 17). Fifty-six patients (43.8%) underwent full screening, and this number improved over time (37.7% before 2018 vs 72.7% after 2018; P = .003). Thirteen of 30 patients (43.3%) required surgical treatment for their GA, without the occurrence of postoperative complications.
CONCLUSIONS: Additional GA were present in almost a quarter of the girls born with an ARM, with vaginal anomalies most often identified. Despite GA being most often found in patients with cloacal malformations, these anomalies were also identified in patients with other ARM types. Surgical treatment was required in almost half of the girls with GA. Therefore, this study emphasizes the importance of screening for GA in patients with an ARM, regardless of the ARM type.

OBJECTIVE: To explore nurses\' experiences with anal dilatations in babies with anorectal malformations.
BACKGROUND: Most babies with anorectal malformations require repeated anal dilatations, either before and/or after reconstructive surgery. Anal dilatation is usually performed without sedation or pain medication. Nurses participate in anal dilatations by assisting doctors doing anal dilatation, doing anal dilatation themselves, and instructing parents how to do anal dilatations. No previous studies have explored how nurses experience being involved in anal dilatations.
METHODS: Qualitative study design utilizing focus group interviews. The COREQ guidelines were applied.
METHODS: Nurses with either ≤2 or ≥10 years\' working experience participated in two different focus group interviews. The focus group interviews were transcribed and analyzed with content analysis.
RESULTS: Twelve nurses, two males, participated. Three main themes emerged from the focus group interviews. The first main theme, \"Anal dilatation causes distress\", describes the nurses\' worries about causing physical and/or psychological harm when doing anal dilatations. The second main theme, \"Need for guidelines and training\", contains nurses\' recommendations for more theoretical training in addition to written guidelines on anal dilatations. The third main theme, \"Collegial support is vital\", describes nurses\' needs and strategies for coping with difficult situations related to anal dilatations.
CONCLUSIONS: Anal dilatation causes distress in nurses, and collegial support is essential for coping. Guidelines and systematic training are recommended to improve current practice.
METHODS: VI.