Both influenza A infection (IAV) and type I hypersensitivity mechanisms have been independently linked to acute appendicitis. Particularly the expression of type I hypersensitivity associated with cytokine interleukin (IL) 13 has been shown to be of significance in both, appendicitis and influenza infection. The aim of the current study was to analyze possible associations of respective gene expressions at the level of mathematical correlations. We analyzed messenger RNA (mRNA) gene expressions of IAV-associated markers, hypersensitivity type I-related cytokines and inflammatory markers IL-17A and c-reactive protein (CRP) in peripheral blood mononuclear cells from 29 children aged 7–17 years, who were operated for histologically confirmed appendicitis at Charité–Universitätsmedizin Berlin between April and August 2019. Statistical relationships between gene expressions were investigated using Spearman’s correlation analysis, with a correlation coefficient r ≥ 0.5 or r ≤ − 0.5 representing high, and r ≥ 0.7 or r ≤ − 0.7 very high correlations. Statistical significance was assumed at P < 0.01. A total of 29 patients were involved in this study. Particularly the expression of IL-13 showed highly significant negative correlations with that of IAV-specific antiviral response genes, ranging from – 0.89 (P < 0.0001) to – 0.49 (P = 0.006). A strong inflammatory background was demonstrated by very high correlations of IL-13 with CRP (r = 0.91, P < 0.0001) and IL-17A (r = 0.82, P < 0.0001). These findings suggest a connection between IAV infection and acute appendicitis in children, implicating sequential immune responses, including hypersensitivity type I mechanisms, in appendicitis pathophysiology.

OBJECTIVE:To investigate surgical methods of repair's impact on reoperation for recurrence in adolescents undergoing groin hernia surgery. BACKGROUND:Despite established international guidelines for groin hernia repairs in adults, no current consensus exists regarding the preferred surgical method for adolescents. Proponents claim mesh is superior in preventing recurrence, while critics highlight potential risks for growing individuals. Investigating repair methods is an important step in improving patient outcomes in this population. METHODS:This is a nationwide, population-based observational cohort study with prospectively collected data from the Swedish Hernia Register of adolescents undergoing groin hernia repair between 2000-2022 with follow-up until 2024. Patients aged 15-18 year were included. Primary outcome was reoperation for recurrence. Secondary objective was other risk-factors associated with reoperation for recurrence. RESULTS:Totally 1,192 adolescent repairs were analysed, predominantly involving indirect hernias (90.5%) and male repairs (84.6%). Overall reoperation rate for recurrence were 4.6%, higher in open anterior mesh repairs (3.3%), open tissue suture repairs (6.5%), and open combined anterior/posterior mesh repairs (10.3%) compared to endo-laparoscopic mesh repairs (1.5%). Multivariable analysis revealed a significantly increased hazard ratio of 3.68 (95% CI 1.13-12.02) for open tissue suture repair and 5.78 (95% CI 1.35-24.74) for open combined anterior/posterior mesh repair compared to endo-laparoscopic mesh repairs. No patient or hernia-related variables were identified as significant risk-factors for reoperation for recurrence. CONCLUSION:The overall reoperation rate for recurrence was generally favorable across all techniques, with endo-laparoscopic mesh repairs associated with a lower risk, which may offer an advantage in preventing recurrences in adolescents undergoing groin hernia surgery.

Objective: To investigate surgical methods of repair’s impact on reoperation for recurrence in adolescents undergoing groin hernia surgery. Background: Despite established international guidelines for groin hernia repairs in adults, no current consensus exists regarding the preferred surgical method for adolescents. Proponents claim mesh is superior in preventing recurrence, while critics highlight potential risks for growing individuals. Investigating repair methods is an important step in improving patient outcomes in this population. Methods: This is a nationwide, population-based observational cohort study with prospectively collected data from the Swedish Hernia Register of adolescents undergoing groin hernia repair between 2000-2022 with follow-up until 2024. Patients aged 15-18 year were included. Primary outcome was reoperation for recurrence. Secondary objective was other risk-factors associated with reoperation for recurrence. Results: Totally 1,192 adolescent repairs were analysed, predominantly involving indirect hernias (90.5%) and male repairs (84.6%). Overall reoperation rate for recurrence were 4.6%, higher in open anterior mesh repairs (3.3%), open tissue suture repairs (6.5%), and open combined anterior/posterior mesh repairs (10.3%) compared to endo-laparoscopic mesh repairs (1.5%). Multivariable analysis revealed a significantly increased hazard ratio of 3.68 (95% CI 1.13-12.02) for open tissue suture repair and 5.78 (95% CI 1.35-24.74) for open combined anterior/posterior mesh repair compared to endo-laparoscopic mesh repairs. No patient or hernia-related variables were identified as significant risk-factors for reoperation for recurrence. Conclusion: The overall reoperation rate for recurrence was generally favorable across all techniques, with endo-laparoscopic mesh repairs associated with a lower risk, which may offer an advantage in preventing recurrences in adolescents undergoing groin hernia surgery.

Abstract Introduction Persistent bowel dysfunction in Hirschsprung disease (HSCR) may impair health-related quality of life (HRQoL), yet data on the effects of disease burden on generic and disease-specific HRQoL are limited. This study evaluated generic and gastrointestinal HRQoL in children and adolescents with HSCR and explored risk factors for reduced HRQoL. Methods This national cross-sectional study recruited children and adolescents aged 8–18 years surgically treated for HSCR at pediatric surgery departments in Sweden between 2004 and 2018. Patients and parents completed the Pediatric Quality of Life Inventory Generic Core Scales version 4.0 (PedsQLv4.0), the PedsQL Gastrointestinal Symptoms Module (PedsQL GI), and the Rintala Bowel Function Score (BFS). Demographic and clinical data were obtained from medical records. Results Seventy patients were included; 31 were aged 8–12 years and 39 were aged 13–18 years (median age 13 years, IQR 11–16), and 16 had associated malformations or syndromes. Children with normal bowel function (BFS ≥17) reported higher PedsQLv4.0 and PedsQL GI scores than those with impaired bowel function in both child- and parent-reported outcomes (PedsQLv4.0: 93 versus 85, P = 0.010; 90 versus 80, P = 0.014; PedsQL GI: 90 versus 83, P = 0.002; 89 versus 79, P = 0.004). Parent- and child-reported scores showed strong correlations for PedsQLv4.0 (ρ = 0.79) and PedsQL GI (ρ = 0.86). Discussion Despite generally high HRQoL in patients with HSCR, impaired bowel function was associated with reduced generic and gastrointestinal HRQoL, highlighting the importance of long-term bowel function management and psychosocial support.

Objectives: To revise the 2018 European Reference Network for rare Inherited and Congenital Digestive and Gastrointestinal Anomalies (ERNICA) clinical guideline for the management of rectosigmoid Hirschsprung's disease (HSCR) based on new evidence and evolving clinical priorities, ensuring continued relevance, trustworthiness, and consistency in care. Methods: The update followed a structured five-step process: identifying new evidence (2018-2024), prioritizing recommendations using the Update Priority Tool, and revising selected topics using the Grading of Recommendations Assessment, Development and Evaluation-Evidence to Decision (GRADE EtD) framework. A multidisciplinary panel of pediatric surgeons, patient representatives, and methodologists from 12 countries oversaw the process. Revised recommendations were developed through evidence review and consensus meetings. Results: Ten prioritized clinical topics were revised. Key updates include refined recommendations on histopathological staining in diagnosis, preoperative screening for renal anomalies and malnutrition, postoperative anastomotic calibration, individualized dietary guidance, sexual and fertility health during follow-up, transition to adult care, and the role of botulinum toxin in managing Hirschsprung's-associated enterocolitis (HAEC). Routine preventive anal dilatations were no longer recommended. The guideline highlights the importance of structured, multidisciplinary, and patient-centered care throughout the lifespan and identifies several research gaps, including nutrition, transition of care, fertility, and HAEC definitions. Conclusion: This updated guideline reflects current evidence and expert consensus, supporting equitable, patient-centered HSCR care. Implementation will be supported via the European Pediatric Surgical Audit (EPSA), and future updates will be informed by registry data and ongoing research.

Background: It is unclear whether functional outcomes improve or deteriorate with age following surgery for Hirschsprung's disease. The aim of this cross-sectional study was to determine the long-term functional outcomes and quality of life (QoL) in patients with Hirschsprung's disease. Methods: Patients with pathologically proven Hirschsprung's disease older than 7 years were included. Patients with a permanent stoma or intellectual disability were excluded. Functional outcomes were assessed according to the Rome IV criteria using the Defaecation and Faecal Continence questionnaire. QoL was assessed by means of the Child Health Questionnaire Child Form 87 or World Health Organization Quality of Life questionnaire 100. Reference data from healthy controls were available for comparison. Results: Of 619 patients invited, 346 (55 & centerdot;9 per cent) responded, with a median age of 18 (range 8-45) years. The prevalence of constipation was comparable in paediatric and adult patients (both 22 & centerdot;0 per cent), and in patients and controls. Compared with controls, adults with Hirschsprung's disease significantly more often experienced straining (50 & centerdot;3 versus 36 & centerdot;1 per cent; P = 0 & centerdot;011) and incomplete evacuation (47 & centerdot;4 versus 27 & centerdot;2 per cent; P < 0 & centerdot;001). The prevalence of faecal incontinence, most commonly soiling, was lower in adults than children with Hirschsprung's disease (16 & centerdot;8 versus 37 & centerdot;6 per cent; P < 0 & centerdot;001), but remained higher than in controls (16 & centerdot;8 versus 6 & centerdot;1 per cent; P = 0 & centerdot;003). Patients with poor functional outcomes scored significantly lower in several QoL domains. Conclusion: This study has shown that functional outcomes are better in adults than children, but symptoms of constipation and soiling persist in a substantial group of adults with Hirschsprung's disease. The persistence of defaecation problems is an indication that continuous care is necessary in this specific group of patients.

BACKGROUND:There has been an increasing number of single-centre studies describing the long-term outcomes of patients with Hirschsprung disease. This study aimed to systematically review the literature on long-term bowel, urological, and sexual functional outcomes, fertility and quality of life in adults with Hirschsprung disease. METHODS:A PROSPERO-registered systematic review of the English literature was conducted for studies published up to July 2025 that reported functional outcomes beyond childhood (≥16 years) for patients who had undergone surgery for Hirschsprung disease. Centres were contacted individually for secondary analyses of patient-level data on bowel function score, Gastrointestinal Quality of Life Index, and Short Form 36 questionnaire. Data were analysed and compared with those from healthy controls in the studies retrieved and from a reference healthy population. Hirschsprung disease clinical and surgical variables were correlated with these outcomes of interest in a patient-level analysis. RESULTS:Fifty-three manuscripts fulfilled the inclusion criteria of 4277 papers retrieved. Patients with Hirschsprung disease had a greater likelihood of constipation (odds ratio 9.27, 95% confidence interval (c.i.) 4.78 to 18.06) and soiling (odds ratio 2.76, 1.96 to 3.89) compared with healthy controls. They scored lower on the Gastrointestinal Quality of Life Index (mean difference -5.21, 95% c.i. -9.53 to -0.89; P = 0.020). There were no significant differences in Short Form 36 domain scores except for physical functioning (mean difference -6.30, -8.74 to -3.87; P < 0.001). At a patient level, longer-segment disease (P < 0.001) and redo pull-through surgery (P = 0.002) were associated with a poorer bowel function score. Short form 36 scores were lower in women across six of eight domains; similarly, Gastrointestinal Quality of Life Index scores were lower in women (P < 0.001) and in patients with longer-segment disease (P < 0.001). CONCLUSION:Among patients with Hirschsprung disease, women, those with longer-segment disease, and patients who underwent redo surgery may be at risk of poorer quality of life.

To describe characteristics of offspring born to mothers having an anorectal malformation (ARM). This was a national population-based cohort study. The exposed group was the offspring of females with an ARM diagnosis (ICD-codes) in the Swedish National Patient Register 1964–2008. The unexposed group was the offspring of mothers without ARM. Five age-matched mothers without ARM were randomly identified for each mother with ARM by Statistics Sweden. Females with chromosomal aberrations were excluded prior to analysis. Outcomes of the mothers were retrieved from the National Patient Register (1964–2021) and Medical Birth Register (1973–2021). Outcomes of the offspring were based on data from Medical Birth Register. Using descriptive statistics, the children of mothers with an ARM were compared to children of mothers not having an ARM. The ethical review authorities approved the study. The mothers included 464 females with ARM and 2313 females without ARM. Of the 464 females with ARM, 132 (28.4 Little is known regarding characteristics of the offspring to mothers with an anorectal malformation (ARM). The first-born of mothers with ARM are more commonly SGA than the first-born of mothers without ARM. A higher prevalence of ARM in the neonates to mothers with ARM suggests an autosomal inheritance pattern of ARM in certain cases.

The Swedish National Board of Health and Welfare centralized the surgical care of bladder exstrophy epispadias complex (BEEC) from four centers to one on 1 July 2018. The aim of this study was to assess short-term postoperative outcomes in BEEC patients following centralization. This retrospective observational study included all patients who underwent primary surgery for BEEC in Sweden from 1 July 2013 to 30 June 2023. Patients treated in the 5 year-period prior to centralization were compared with those treated during the 5 year-period after centralization. Main outcomes were unplanned readmissions, surgical procedures, and complications graded according to Clavien Madadi >3 within 90 postoperative days. Nineteen and 21 patients underwent primary surgery due to BEEC before and after centralization, respectively. Median age at first contact with a paediatric urologist and age at primary surgery was similar before and after centralization. Osteotomies were more common prior to centralization (92% vs 36%, p=0.01). Median length of hospital stay, epispadias patients excluded, was shorter after centralization (16 vs 26 days, p<0.001). Unplanned readmissions were required in 19 % (p=0.92) of patients both prior and after centralization and unplanned surgical procedures in 5% vs 10% (p=0.92) respectively. One complication grade IIIa occurred before centralization compared to two grade IIIa complications post centralization (p=0.92). Centralization of care for BEEC did not delay time to first visit with paediatric urologist or time to primary surgery. Unplanned readmission or surgical procedures did not increase after centralization. Performing osteotomies was less common post centralization, probably explaining shorter hospital stays.

AIM:There is very limited data regarding the impact of centralization of care of patients with congenital diaphragmatic hernia (CDH). The National Board of Health and Welfare in Sweden decided to centralize the care of CDH from July 2018. The aim of this study was to assess the short-term impact of centralization in patient outcomes. METHODS:This was a retrospective observational study including all centers in Sweden managing CDH before and after centralization. Outcomes for patients born with CDH who were managed from 1st of July 2013 to 30th of June 2018 (before centralization) were compared with patients managed 1st of July 2018 to 30th of June 2023 (after centralization). Fisher's exact test and non-parametric tests were used to compare differences between the two time periods and a p<0.05 was considered statistically significant. The study was approved by the Swedish Ethical Review Authority (2023-02650-01) MAIN RESULTS: A total of 243 patients with CDH were managed nationally during the study period, 111 before and 132 after centralization. The rates of prenatal diagnosis, side of the defect, need of patch, or mortality did not differ significantly between the study periods. The need for ECMO decreased significantly after centralization 18% vs. 7%, p<0.01). CONCLUSIONS:After centralization, we observed a decreased rate of need for ECMO support. Although the time from centralization push through is short, it underscores the importance of an ongoing evaluation, optimization and strategic planning in order to find, describe and maximize the possible benefits of a centralization of CDH in our settings. TYPE OF STUDY:Retrospective cohort study, nationwide LEVEL OF EVIDENCE: II.

Background Support for the treatment of uncomplicated appendicitis with non-operative management rather than surgery has been increasing in the literature. We aimed to investigate whether treatment of uncomplicated appendicitis with antibiotics in children is inferior to appendicectomy by comparing failure rates for the two treatments. Methods In this pragmatic, multicentre, parallel-group, unmasked, randomised, non-inferiority trial, children aged 5-16 years with suspected non-perforated appendicitis (based on clinical diagnosis with or without radiological diagnosis) were recruited from 11 children's hospitals in Canada, the USA, Finland, Sweden, and Singapore. Patients were randomly assigned (1:1) to the antibiotic or the appendicectomy group with an online stratified randomisation tool, with stratification by sex, institution, and duration of symptoms (>= 48 h vs <48 h). The primary outcome was treatment failure within 1 year of random assignment. In the antibiotic group, failure was defined as removal of the appendix, and in the appendicectomy group, failure was defined as a normal appendix based on pathology. In both groups, failure was also defined as additional procedures related to appendicitis requiring general anaesthesia. Interim analysis was done to determine whether inferiority was to be declared at the halfway point. We used a non-inferiority design with a margin of 20%. All outcomes were assessed in participants with 12-month follow-up data. The trial was registered at ClinicalTrials.gov (NCT02687464). Findings Between Jan 20, 2016, and Dec 3, 2021, 936 patients were enrolled and randomly assigned to appendicectomy (n=459) or antibiotics (n=477). At 12-month follow-up, primary outcome data were available for 846 (90%) patients. Treatment failure occurred in 153 (34%) of 452 patients in the antibiotic group, compared with 28 (7%) of 394 in the appendicectomy group (difference 267%, 90% CI 224-309). All but one patient meeting the definition for treatment failure with appendicectomy were those with negative appendicectomies. Of those who underwent appendicectomy in the antibiotic group, 13 (8%) had normal pathology. There were no deaths or serious adverse events in either group. The relative risk of having a mild-to-moderate adverse event in the antibiotic group compared with the appendicectomy group was 43 (95% CI 21-87; p<00001). Interpretation Based on cumulative failure rates and a 20% non-inferiority margin, antibiotic management of non-perforated appendicitis was inferior to appendicectomy.

BackgroundThe European Reference Network for rare Inherited Congenital Anomalies (ERNICA) is a clinical network dedicated to improving the quality of care for patients with rare and complex digestive and gastrointestinal diseases, many of whom require surgery in early life. The network brings together clinicians, researchers and patient representatives from 22 countries in Europe. By pooling expertise, ERNICA is able to facilitate improvement initiatives that may not otherwise be possible. However, describing the desired quality of care and transferring it to local practice remains a challenge, complicated by our low-prevalence patient population, multidisciplinary clinical involvement and heterogeneous European context. In an attempt to mitigate these challenges, and foster a system of continuous quality improvement, we present the “ERNICA quality cycle”.Main bodyThe ERNICA quality cycle is comprised of five steps: (1) Describing the desired quality of care (2) Promoting guideline implementation (3) Measuring quality of care (4) Evaluating clinical practice (5) Conducting research. It offers a structured, continuous and collaborative approach to the improvement of care for patients with rare and complex digestive and gastrointestinal diseases. Evaluating the approach, through qualitative process evaluation, will be critical to capturing learning points.ConclusionsThe ERNICA quality cycle holds tremendous potential for improving the quality of care for patients with rare and complex conditions, both within ERNICA and for other European Reference Networks.

Introduction: Composite long-term outcomes of cloacal malformations remain unclear. We aimed to evaluate bowel and bladder control, sexual function, reproductive health, and quality of life in adulthood. Methods: A Nordic multi-center cross-sectional observational study evaluating the outcome of adult patients with cloacal malformations was performed. Patient characteristics were retrieved from case records. Established questionnaires were sent to the patients to evaluate bowel- bladder- and sexual function and quality of life. Results: Thirty-four of 48 (70%) eligible patients with median age 28 years (18-45) responded. Eight (24%) patients had a common channel >3 cm. Imaging-confirmed spinal and sacral abnormalities were present in 4 and 17 patients respectively. 85% (n = 29/34, four patients with permanent urinary diversions after bladder neck closure excluded) reported no urinary leakage without physical activity or urge to urinate. Three patients had a permanent enterostomy. 42 % (n = 11/26) reported acceptable bowel function according to Bowel Function Score (with eventual ongoing bowel management). The median Profile of Female Sexual Function Score was below normative values. Quality of life was comparable to the reference population, but thirty percent scored within the distress domains. Eight patients (24%) had been pregnant (15 pregnancies), resulting in seven live births, but the need of assisted reproduction techniques (40%, n = 6/15) and miscarriage (40%, n = 6/15) was common. Conclusions: Adequate spontaneous bowel control was rare, while most patients were dry for urine without additional procedures. Cloacal malformation also have a negative impact on sexual function health related quality of life and reproductive health. Long-term follow-up is crucial, not only regarding bowel and bladder function, but also for sexual and reproductive function, which may be important negative factors for health-related quality of life. Level of Evidence: Level IV. (c) 2024 The Author(s). Published by Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

AimThis study aimed to develop a universally applicable core set of quality indicators for Hirschsprung's disease care through a consensus-driven process, to standardise and improve care quality across Europe. MethodsA modified Delphi method was used to achieve consensus among healthcare professionals (HPs) and patient representatives (PRs) across Europe. Participants completed three rounds of anonymous surveys, rating quality indicators for Hirschsprung's disease care. A systematic literature review informed the initial item list. Results were analysed using predefined criteria, and a final consensus meeting established the core set of indicators. ResultsAn international panel of 8 PRs and 96 multidisciplinary health care professionals representing 59 European hospitals completed all questionnaires, eventually including 12 baseline characteristics and 39 indicators. Six of the top 10 indicators were commonly prioritised by both groups. The remaining items were refined through debate and finalised during a consensus meeting. ConclusionThis study established a core set of 12 baseline characteristics and 14 quality indicators for evaluating Hirschsprung's disease care. These indicators will support benchmarking and continuous quality improvement within the European Paediatric Surgical Audit framework, ultimately enhancing outcomes and care for children with Hirschsprung's disease.

The emergence of large language models (LLMs) has led to notable advancements across multiple sectors, including medicine. Yet, their effect in pediatric surgery remains largely unexplored. This study aims to assess the ability of the artificial intelligence (AI) models ChatGPT-4 and Microsoft Copilot to propose diagnostic procedures, primary and differential diagnoses, as well as answer clinical questions using complex clinical case vignettes of classic pediatric surgical diseases.We conducted the study in April 2024. We evaluated the performance of LLMs using 13 complex clinical case vignettes of pediatric surgical diseases and compared responses to a human cohort of experienced pediatric surgeons. Additionally, pediatric surgeons rated the diagnostic recommendations of LLMs for completeness and accuracy. To determine differences in performance, we performed statistical analyses.ChatGPT-4 achieved a higher test score (52.1%) compared to Copilot (47.9%) but less than pediatric surgeons (68.8%). Overall differences in performance between ChatGPT-4, Copilot, and pediatric surgeons were found to be statistically significant (p < 0.01). ChatGPT-4 demonstrated superior performance in generating differential diagnoses compared to Copilot (p < 0.05). No statistically significant differences were found between the AI models regarding suggestions for diagnostics and primary diagnosis. Overall, the recommendations of LLMs were rated as average by pediatric surgeons.This study reveals significant limitations in the performance of AI models in pediatric surgery. Although LLMs exhibit potential across various areas, their reliability and accuracy in handling clinical decision-making tasks is limited. Further research is needed to improve AI capabilities and establish its usefulness in the clinical setting.

There are limited data to show how sex impacts the early clinical course of patients with Hirschsprung disease (HSCR). This study aimed to explore potential sex related disparities in the preoperative, surgical, and early postoperative course of HSCR patients. This retrospective study analyzed data of HSCR patients who underwent pull-through surgery at pediatric surgery centers in Sweden from July 1st, 2013, to June 30th, 2023. Male and female patients were compared regarding diagnostics, surgical treatment, unplanned procedures under general anesthesia or readmissions within 90 days after pull-through, and complications (Clavien-Madadi grade ≥ 3) up to 30 days after pull-through. A total of 197 patients were included from four treating centers (158 males, 39 females). Females had a higher prevalence of familial disease (28.2

OBJECTIVE:Hirschsprung is a congenital disorder affecting the gastrointestinal tract. However, pelvic colorectal surgery in infancy has been hypothesised to impact gynaecological outcomes in later life. Describe sexual function and fertility outcomes in women with Hirschsprung disease compared to population controls. Assess factors associated with poor outcomes (sexual dysfunction and subfertility). DESIGN:International multicentre cross-sectional cohort study with comparison to controls from the general population. SETTING:Status post-discharge from paediatric services. POPULATION:Female patients aged > 20 years. METHODS:Validated questionnaire-based survey with linkage to patient medical records. Comparison with controls using univariate analyses. MAIN OUTCOME MEASURES:Sexual dysfunction (Female Sexual Function Index; FSFI ≤ 26), Subfertility at 1 and 2 years. RESULTS:Sexual dysfunction as per the FSFI was more common in patients and associated with poor functional outcomes; sexual abstinence seemed to associate even more so with poor bowel outcomes. Subfertility was higher in patients compared to controls (1 year: 21/45 (47%) vs. 38/178 (21%), p = 0.0008; 2 years: 12/45 (27%) vs. 17/178 (10%), p = 0.004). There was an increased proportion of patients who had accessed fertility services (20/45 (44%) vs. 43/178 (24%); p = 0.009), the proportion of successful pregnancies in patients attempting to conceive with IVF (11/17 (65%) vs. 27/43 (63%); p = 1.0) was similar. CONCLUSIONS:These novel data suggest that women with Hirschsprung disease who have undergone reconstructive surgery may be at risk for adverse sexual function and fertility outcomes.

One in 4000 Swedish children is born with abdominal wall defect (AWD). Little is known about their neurodevelopmental trajectory and long-term morbidity. The aim was to determine the risk of neurodevelopmental disorders in children born with omphalocele or gastroschisis. This was a population-based national cohort study including children born with omphalocele or gastroschisis in Sweden 1997–2016. Individuals with chromosomal abnormality were excluded. Ten age and sex-matched unexposed individuals were randomly selected for every AWD case. Main outcomes were autism spectrum disorder (ASD) and attention-deficit/hyperactivity disorder (ADHD). Data were collected from the national health registers. During the study period, 496 children were born with AWD and included in the exposed cohorts. The unexposed cohorts consisted of 4943 children. Neurodevelopmental diagnoses were found in 11 (6.1

BACKGROUND:The Swedish National Board of Health and Welfare centralized the surgical care of patients with anorectal malformations from four to two centres in 2018. This retrospective review compares short-term complications after anorectal reconstruction before and after centralization. METHODS:Hospital records of all infants in Sweden who underwent reconstruction of an anorectal malformation between 1 July 2013 and 30 June 2023 were reviewed and divided in two 5-year periods: before and after centralization. The main outcomes were unplanned readmissions and surgical procedures requiring general anaesthesia up to 90 days after reconstruction, as well as early complications classified according to the Clavien-Madadi system up to 30 days after the procedure. RESULTS:Before centralization, 173 infants underwent anorectal reconstruction, compared with 176 infants after centralization. Patient groups were comparable with respect to associated malformations and type of anorectal malformation. Before centralization, 80 infants (46.2%) had a colostomy before the anorectal reconstruction, compared with 89 infants (50.6%) after centralization (P = 0.454). Anorectal reconstruction was performed at a median age of 61 and 47 days of age before and after centralization, respectively (P = 0.794). Unplanned readmissions up to 90 days after anorectal reconstruction were needed in 12 infants (6.9%) before centralization, compared with 22 infants (12.5%) after centralization (P = 0.104). Unplanned surgical procedures under general anaesthesia were required in 20 (11.6%) and 22 (12.5%) infants before and after centralization, respectively (P = 0.870). Complications (Clavien-Madadi grade III-V) within 30 days after anorectal reconstruction were seen in 16 (9.2%) and 12 (6.8%) infants before and after centralization, respectively (P = 0.436). CONCLUSION:Centralization of the surgical care of patients with anorectal malformations in Sweden did not seem to have an impact on short-term complications.

The aim was to investigate sexual function and fertility in female adults operated on for anorectal malformations (ARM). This was a cross-sectional questionnaire-based study including female adult patients treated for ARM at our institution between 1994 and 2003. Sexual function in females was assessed using the Profile of Sexual Function (PFSF). Additional questions regarding fertility were answered by the participants. Patient characteristics were retrospectively retrieved from the medical records and descriptive statistics were used for analysis. Sexual function outcomes were compared to a control group from a previously published group of females. Composite outcome analysis was performed using previously published data to determine the potential impact of bowel function and health-related quality of life on sexual function. The ethics review authorities approved the study. A total of 14 of 30 (46.7