• Keiichi UchidaEmail author
  • Mikihiro Inoue
  • Yuhki Koike
  • Kohei Matsushita
  • Yuka Nagano
  • Masato Kusunoki


Hirschsprung’s disease-associated enterocolitis is the most common complication of Hirschsprung’s disease. Clinical symptoms of Hirschsprung’s disease-associated enterocolitis vary from mild to severe, and it sometimes becomes a serious and life-threatening condition. Historically, surgical procedures for Hirschsprung’s disease have significantly progressed, but Hirschsprung’s disease-associated enterocolitis remains a frequent complication before and after surgery. The incidence of Hirschsprung’s disease-associated enterocolitis widely varies because of different diagnostic criteria. Historically, distal obstruction due to aganglionosis has been considered as the sole causative factor in the development of Hirschsprung’s disease-associated enterocolitis. Recent studies have shown that the pathogenesis of Hirschsprung’s disease-associated enterocolitis is complex and is associated with other factors, such as genetics, the gut mucosal barrier, mucosal immunity, the microbiome, and surgical factors. The development of management strategies for this condition can be applied before and after definitive pull-through surgery. These include prompt application of preventive strategies for Hirschsprung’s disease-associated enterocolitis, parenteral and enteral nutrition, and probiotics, which might contribute to the decreased mortality rate. In this chapter, we review the pathogenesis, diagnosis, therapeutic management, and prevention of Hirschsprung’s disease-associated enterocolitis.


Hirschsprung’s disease Enterocolitis 



We thank Edanz Group ( for editing a draft of this manuscript.


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Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  • Keiichi Uchida
    • 1
    Email author
  • Mikihiro Inoue
    • 1
  • Yuhki Koike
    • 1
  • Kohei Matsushita
    • 1
  • Yuka Nagano
    • 1
  • Masato Kusunoki
    • 1
  1. 1.Department of Gastrointestinal and Pediatric SurgeryMie University Graduate School of MedicineTsuJapan

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