The Future Consideration in Allied Disorders of Hirschsprung’s Disease

  • Shigeru OnoEmail author


The allied disorders of Hirschsprung’s disease (ADHDs) are characterized by functional intestinal obstruction that clinically resembles Hirschsprung’s disease despite the presence of ganglion cells in the rectum [1]. They can be divided into conditions with abnormal ganglia, such as immaturity of ganglia (IG), hypoganglionosis (HG), and intestinal neuronal dysplasia (IND), and conditions with normal ganglia, such as megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), segmental dilatation (SD), internal anal sphincter achalasia (IASA), and chronic idiopathic intestinal pseudo-obstruction (CIIP) [2]. This classification of ADHDs may be reasonable and has been widely accepted in Japan. However, Puri et al. proposed a slightly different classification of these disorders, called variants of Hirschsprung’s disease in 2012 [3]. Variations in pathological characteristics and morbidity affect the clinical course and prognosis. All studies and discussions about ADHDs should be based on the same classification system with incorporation of pathological findings. International collaboration in data collection and classification of ADHDs is required to understand the clinical characteristics, reduce mortality, and improve clinical outcomes.


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© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  1. 1.Pediatric SurgeryJichi Medical UniversityTochigiJapan

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