ReviewOrphanet journal of rare diseases2020
ERNICA guidelines for the management of rectosigmoid Hirschsprung's disease.
Review in Orphanet journal of rare diseases, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 98 papers, 9 of them syntheses that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
98 citing papers in PubMed, 9 syntheses or guidelines pooled it, 173 citations in OpenAlex.
- Does laparoscopic-assisted transanal endorectal pull-through improve outcomes compared with transanal endorectal pull-through in Hirschsprung disease? A systematic review and meta-analysis.Pediatric surgery international · 2026Pooled it
- Updated European Reference Network for rare Inherited and Congenital Digestive and Gastrointestinal Anomalies guidelines for the management of rectosigmoid Hirschsprung's disease 2025.Journal of pediatric gastroenterology and nutrition · 2026Guideline
- Nutritional management of neonates who undergo major surgery for gastrointestinal disorders: a joint position paper of the Italian Society of Neonatology (SIN), the Italian Society of Pediatric Surgery (SICP), and the Italian Society of Pediatric Nutrition (SINUPE).Italian journal of pediatrics · 2026Guideline
- Bibliometric analysis of clinical treatment in Hirschsprung disease: trends and emerging hotspots.Frontiers in pediatrics · 2026Pooled it
- Postoperative outcomes of robotic-assisted vs. laparoscopic pull-through surgery in pediatric Hirschsprung's disease: a systematic review and meta-analysis.Pediatric surgery international · 2025Pooled it
- Volume-outcome relationship in corrective surgery for Hirschsprung's disease: a systematic literature review of direct evidence and an overview of indirect evidence.Pediatric surgery international · 2025Pooled it
- Pooled it
- Systematic appraisal of the guidelines for the diagnosis and treatment of Hirschsprung's disease.Pediatric surgery international · 2022Pooled it
- Prevalence of Hirschsprung-associated enterocolitis in patients with Hirschsprung disease.Pediatric surgery international · 2022Pooled it
- Clinical characteristics, pathological comorbidities, BMI-for-age Z-score, and machine learning-based exploration of postoperative enterocolitis in iIntestinal neuronal dysplasia: a 20-year retrospective study.Pediatric surgery international · 2026Article
- NOTCH-mediated glial CXCL9:SPP1 polarization by GSK3β exacerbates postnatal enteric nervous system dysfunction in Hirschsprung disease.Journal of gastroenterology · 2026Article
- Subtypes of Enteric Neurons in Hirschsprung Disease: A Comparison of Fully Ganglionic and Transitional Zone.Neurogastroenterology and motility · 2026Article
- RNA methylation and the potential role of folate supplementation in preventing Hirschsprung disease.European journal of nutrition · 2026Article
- Intestinal Neuronal Dysplasia Type B as Rare Organic Cause of Refractory Constipation in Adults: A Case Report and Literature Review.Clinics and practice · 2026Article
- Total colonic aganglionosis: a bibliometric analysis of trends and themes (1978-2024).Orphanet journal of rare diseases · 2026Article
- Fostering continuous quality improvement in a European rare disease network: Where are we now?Orphanet journal of rare diseases · 2026Review
- European Reference Networks - a flagship activity of the EU in the field of rare and complex diseases: from 2017 to 2025.Orphanet journal of rare diseases · 2026Review
- A pelvic floor muscle rehabilitation programme for pediatric patients undergoing colorectal surgery with sphincters involvement provides excellent results and more optimistic prognostic expectations.Pediatric surgery international · 2026Article
- Contrast enema in Hirschsprung disease: radiological signs and clinical symptoms as predictors in a logistic regression model.Pediatric radiology · 2026Article
- Surgical Complications in Hirschsprung Disease and the Impact of Botulinum Toxin Injection on Hirschsprung-Associated Enterocolitis.Journal of clinical medicine · 2026Article
38 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
20 authors at 14 institutions in 9 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundHirschsprung's disease (HSCR) is a serious congenital bowel disorder with a prevalence of 1/5000. Currently, there is a lack of systematically developed guidelines to assist clinical decision-making regarding diagnostics and management.
aimsThis guideline aims to cover the diagnostics and management of rectosigmoid HSCR up to adulthood. It aims to describe the preferred approach of ERNICA, the European Reference Network for rare inherited and congenital digestive disorders.
methodsRecommendations within key topics covering the care pathway for rectosigmoid HSCR were developed by an international workgroup of experts from 8 European countries within ERNICA European Reference Network from the disciplines of surgery, medicine, histopathology, microbiology, genetics, and patient organization representatives. Recommendation statements were based on a comprehensive review of the available literature and expert consensus. AGREE II and GRADE approaches were used during development. Evidence levels and levels of agreement are noted.
resultsThirty-three statements within 9 key areas were generated. Most recommendations were based on expert opinion.
conclusionIn rare or low-prevalence diseases such as HSCR, there remains limited availability of high-quality clinical evidence. Consensus-based guidelines for care are presented.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.