Hirschsprung disease research shifts toward guideline-driven care and HAEC preventionHirschsprung Disease Research Grows as Guidelines Shape Care

BackgroundHirschsprung disease (HSCR) is a congenital gastrointestinal malformation defined by the absence of enteric ganglion cells in the distal bowel. Although surgical resection remains the primary treatment, the field has witnessed rapid evolution toward precision minimally invasive strategies, including laparoscopic/robotic-assisted procedures and standardized perioperative protocols. The expanding literature volume necessitates a systematic synthesis of the research landscape.MethodsWe retrieved HSCR clinical treatment publications from Web of Science Core Collection and Scopus (2005–2025). After deduplication, 3,816 publications (3,201 articles, 615 reviews) were analyzed using CiteSpace 6.4.R1 and VOSviewer 1.6.20, covering annual trends, collaboration networks, journal distribution, reference bursts, and keyword bursts.ResultsAnnual output rose from 103 (2005) to 313 (2025), exceeding 200 since 2019. The United States (27.2%), China (13.0%), and the United Kingdom (8.2%) led contributions. Nationwide Children's Hospital and Huazhong University of Science and Technology were top institutional producers, while Journal of Pediatric Surgery was the most productive journal. Reference burst analysis identified ERNICA rectosigmoid HSCR guidelines (strength = 25.65) and HAEC management guidelines (strength = 23.55) as landmark works. Keyword bursts revealed three evolutionary phases: surgical technique optimization (2005–2012, dominated by “surgical technique,” strength = 18.96); pathophysiological and experimental therapies (2011–2018, with “stem cell” and “transplantation”); and guideline-driven standardized management (2019–present, with active bursts in “guidelines,” strength = 8.53; “colorectal surgery,” strength = 10.68; and “prematurity,” strength = 7.89). Persistent bursts in colorectal surgery and guideline-related terms indicate sustained attention, while stem cell-based regenerative therapies remain at an early experimental stage and represent an emerging frontier, although clinical translation has not yet been demonstrated.ConclusionsHSCR clinical treatment research has grown substantially over two decades, transitioning from surgical technique optimization through experimental therapies to standardized multidisciplinary management. Current hotspots include HAEC prevention, long-term functional follow-up, and clinical guideline implementation—all core components of precision minimally invasive frameworks. Stem cell-based regenerative therapies (still at a preclinical stage) and precision surgery for long-segment HSCR are future directions warranting further investigation. These findings provide an evidence-based reference for navigating the evolving landscape and prioritizing investigations.