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Hirschsprung’s Disease

  • Maria Grazia Faticato
  • Girolamo Mattioli
Chapter

Abstract

Hirschsprung’s disease (HSCR) is a congenital rare condition characterized by the absence of ganglion cells in the distal bowel, resulting in chronic functional obstruction. In most cases the aganglionosis involves the rectum or rectosigmoid, but it can extend for varying lengths. The incidence of Hirschsprung disease is approximately 1 in 5000 live births with male preponderance. The usual presentation of neonate affected by HSCR is intestinal obstruction during the first few days of life. A common severe complication presenting in many patients is enterocolitis. The gold standard for the diagnosis of HSCR is rectal suction biopsy.

The main goal in the treatment of patients with Hirschsprung’s disease is to resect the aganglionic segment and pull down the normoganglionic bowel.

Keywords

Hirschsprung’s disease Newborn Enterocolitis Pull-through 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Maria Grazia Faticato
    • 1
  • Girolamo Mattioli
    • 1
  1. 1.Paediatric Surgery Unit, Istituto Giannina GasliniDINOGMI University of GenoaGenoaItaly

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