Crohn’s disease in patients with Hirschsprung disease and Hirschsprung-associated enterocolitis: A review

Abstract

In the last decade, the incidence of inflammatory bowel disease in children has been increasing. Some studies have reported the development of Crohn’s disease in patients with Hirschsprung disease and Hirschsprung-associated enterocolitis. Suggestions have been made about the possible causes of the development of Crohn’s-like lesions after intestinal resection in early childhood. This study aimed to summarize information about Hirschsprung-associated Crohn’s disease and Crohn’s-like lesions after surgical treatment of Hirschsprung disease. The search was carried out in scientific databases of eLibrary, PubMed, ScienceDirect, and Google Scholar. The search time interval was 2000–2023. Fourteen articles dedicated to Hirschsprung-associated Crohn’s disease were identified. Common pathogenesis factors in inflammatory bowel diseases and Hirschsprung-associated enterocolitis were identified, such as microbiota disturbance and chronic inflammation. Predisposing factors for pathological immune response to dysbiosis and incidence of enterocolitis included enteric nervous system dysfunction, impaired neuroimmune regulatory mechanisms, general immunological disturbance, and “immaturity” and abnormality of the barrier function of the intestinal mucosa. The “classic form” of Crohn’s disease may not develop in patients with Hirschsprung disease and recurrent attacks of enterocolitis but Crohn’s-like lesions. Persistent functional or organic obstruction after surgical treatment of Hirschsprung disease enhances the negative effect of predisposing factors, leading to chronic inflammation of the intestinal mucosa. Delayed occurrence of Hirschsprung-associated enterocolitis is a predisposing factor for the development of chronic inflammation. When Hirschsprung-associated enterocolitis occurs repeatedly, endoscopic assessment should be performed for the exclusion of Crohn’s disease and chronic inflammatory changes in the anastomosis area for treatment selection.

Keywords

Crohn’s disease / Hirschsprung disease / Hirschsprung-associated enterocolitis / Hirschsprung-associated Crohn’s disease

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