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      Classification and diagnostic criteria of variants of Hirschsprung's disease.

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      Pediatric surgery international

      Springer Nature America, Inc

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          Abstract

          "Variants of Hirschsprung's disease" are conditions that clinically resemble Hirschsprung's disease (HD), despite the presence of ganglion cells in rectal suction biopsies. The diagnosis and management of these patients can be challenging. Specific histological, immunohistochemical and electron microscopic investigations are required to characterize this heterogeneous group of functional bowel disorders. Variants of HD include intestinal neuronal dysplasia, intestinal ganglioneuromatosis, isolated hypoganglionosis, immature ganglia, absence of the argyrophil plexus, internal anal sphincter achalasia and congenital smooth muscle cell disorders such as megacystis microcolon intestinal hypoperistalsis syndrome. This review article systematically classifies variants of HD based on current diagnostic criteria with an additional focus on pathogenesis, epidemiology, clinical presentation, management and outcome.

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          Author and article information

          Journal
          Pediatr. Surg. Int.
          Pediatric surgery international
          Springer Nature America, Inc
          1437-9813
          0179-0358
          Sep 2013
          : 29
          : 9
          Affiliations
          [1 ] National Children's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland.
          Article
          10.1007/s00383-013-3351-3
          23943250

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