Complications After Surgery for Anorectal Malformations: An ARM-Net consortium Registry Study

Objective: Establishing the incidence and types of complications following surgical intervention for ARM, primarily after reconstruction. Patient- and treatment-related risk factors were also determined. // Background: Postoperative complications of ARM surgery vary widely, with data predominantly derived from single-center retrospective studies with limited number of patients. Whether factors such as ARM type, associated congenital anomalies, prior enterostomy, or type of reconstructive surgery affect complication incidence remains unclear. // Methods: This multicenter cohort study was performed using the ARM-Net registry with prospectively collected data. Enterostomy-related and post-reconstructive complications in patients who underwent reconstructive surgery before the age of five years were recorded. Patients with more than 25 % missing data, unknown sex, ARM type, or reconstruction date, or without (information on) reconstruction or complications, were excluded. Multivariable analyses identified independent risk factors for the development of complications. // Results: A total of 2,043 patients were eligible for analysis. Complications after enterostomy formation and closure occurred in 25 % and 12 % of patients, respectively. Post-reconstructive complications occurred in 25 % of patients, with wound complications comprising half of the complications. In a multivariable analysis, recto-bladder neck fistula, any associated anomaly, and the LAARP procedure were identified as independent risk factors for post-reconstructive complications. In contrast, anoplasty and mini-PSARP reduce the risk of complications. // Conclusions: Post-reconstructive complications in ARM patients are common, and certain patient- and treatment-related characteristics affect postoperative outcomes. These results aid counselling and clinical decision-making, and may guide the operative planning of ARM types that are amenable to several different surgical approaches

Low maternal dietary intakes of iron, magnesium, and niacin are associated with spina bifida in the offspring.

Contains fulltext : 57481.pdf (publisher's version ) (Closed access)Evidence about the preventive effects of nutrients other than folate on the occurrence of spina bifida is scarce. Therefore, the aim of this work was to investigate the role of maternal nutritional intake and the risk of spina bifida in the offspring. In 106 cases and 181 controls, the mothers' nutrient intakes were obtained by an FFQ approximately 24 mo after conception of the index pregnancy. Energy-adjusted mean nutrient intakes were compared, and odds ratios (OR) and 95% CI were calculated. Although mean nutrient intakes were comparable to the Dutch food consumption survey data, fat, cholesterol, iron, and folate intakes were below the 1998 Dutch Recommended Daily Allowances. Case mothers had significantly lower intakes of plant proteins (7%), polysaccharides (4%), fiber (7%), iron (6%), magnesium (6%), and niacin (4%) than control mothers. Mono- and disaccharide intakes were significantly higher (6%) in the case mothers than in control mothers. The adjusted OR (95% CI) in the lowest quartiles for plant proteins was 5.4 (2.3-12.4), for fiber 3.1 (1.5-6.8), for iron 3.5 (1.4-8.3), for magnesium 1.9 (0.9-4.1), and for niacin 2.5 (1.2-5.2). Mono- and disaccharide and polysaccharide intakes in the highest quartile had ORs (95% CI) of 2.9 (1.4-6.3) and 0.5 (0.3-1.0), respectively. The nutritional intake of Dutch women from food groups containing iron and folate seems to be compromised. Low preconceptional intakes of plant proteins, iron, magnesium, and niacin are associated with a 2- to 5-fold increased risk of spina bifida

A Quality Assessment of the ARM-Net Registry Design and Data Collection.

BACKGROUND: Registries are important in rare disease research. The Anorectal Malformation Network (ARM-Net) registry is a well-established European patient registry collecting demographic, clinical, and functional outcome data. We assessed the quality of this registry through review of the structure, data elements, collected data, and user experience. MATERIAL AND METHODS: Design and data elements were assessed for completeness, consistency, usefulness, accuracy, validity, and comparability. An intra- and inter-user variability study was conducted through monitoring and re-registration of patients. User experience was assessed via a questionnaire on registration, design of registry, and satisfaction. RESULTS: We evaluated 119 data elements, of which 107 were utilized and comprised 42 string and 65 numeric elements. A minority (37.0%) of the 2278 included records had complete data, though this improved to 83.5% when follow-up elements were excluded. Intra-observer variability demonstrated 11.7% incongruence, while inter-observer variability was 14.7%. Users were predominantly pediatric surgeons and typically registered patients within 11-30 min. Users did not experience any significant difficulties with data entry and were generally satisfied with the registry, but preferred more longitudinal data and patient-reported outcomes. CONCLUSIONS: The ARM-Net registry presents one of the largest ARM cohorts. Although its collected data are valuable, they are susceptible to error and user variability. Continuous evaluations are required to maintain relevant and high-quality data and to achieve long-term sustainability. With the recommendations resulting from this study, we call for rare disease patient registries to take example and aim to continuously improve their data quality to enhance the small, but impactful, field of rare disease research. LEVEL OF EVIDENCE: V