Isolated hypoganglionosis: Systematic review of a rare intestinal innervation defect

  • Dingemann J
  • Puri P
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Isolated hypoganglionosis (IH) is rare and resembles Hirschsprung's disease. The diagnosis by suction biopsy is difficult. A full-thickness biopsy is essential. Histological features of IH include sparse, small myenteric ganglia, low acetylcholinesterase activity in the lamina propria, and hypertrophy of muscularis mucosae and circular muscle. This review investigates the epidemiology, symptoms, diagnosis, and outcome of patients with IH.

Author-supplied keywords

  • Full-thickness biopsy
  • Hirschsprung's related disease
  • Intestinal hypoganglionosis
  • Isolated hypoganglionosis
  • Neuronal intestinal malformation

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