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 population-matched controls 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 Mann-Whitney and Fisher’s Exact tests. Multivariable logistic regression using outcomes of sexual dysfunction and subfertility. 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 moreso 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), however the proportion of successful pregnancies in patients attempting to conceive with IVF (11/17 (65%) vs 27/43 (63%); p=1.0) were similar. Conclusions: This novel data suggests that women with Hirschsprung disease who have undergone reconstructive surgery may be at risk for adverse sexual functional and fertility outcomes.

BACKGROUND Preoperative evaluation of Image Defined Risk Factors (IDRFs) in neuroblastoma (NB) is crucial for determining suitability for upfront resection or tumor biopsy. IDRFs are linked with a higher potential morbidity at operation and lessen the chance of complete tumor resection. The IDRFs do not all carry the same weight in predicting tumor complexity and surgical risk. In this study we aimed to assess and categorize the degrees of surgical complexity (Surgical Complexity Index, SCI) in NB resection.  PROCEDURE A panel of 15 surgeons was involved in an electronic Delphi consensus survey to identify and score a set of shared items predictive and/or indicative of surgical complexity, including the number of preoperative IDRFs. Risk categories included - (a) Standard risk; (b) Moderate risk; (c) High risk; (d) Very high risk. A shared agreement included the achievement of at least 75% consensus focused on a single category or, alternatively, on the sum between the prevailing category and an immediately closest one. RESULTS After 3 Delphi rounds, agreement was established on 25/27 items (92.6%). A severity score was established for each item ranging from 0 to 3 with an overall SCI range varying from a minimum score of zero to a maximum score of 29 points for any given patient.  CONCLUSIONS A consensus on a SCI to stratify the risks related to tumor resection was established by the panel experts. This index will now be deployed to critically assign a better severity score to IDRFs involved in NB surgery.