A case of massive gastric necrosis in a young girl with Rett Syndrome

Emanuele Baldassarre, Giorgio Capuano, Gabriele Valenti, Paolo Maggi, Andrea Conforti, Ilaria Prosperi Porta

Research output: Contribution to journalArticlepeer-review


This is the unusual case of a 17-year-old girl affected by Rett Syndrome (RS) who suffered acute abdominal distension and constipation for a week. Laparotomy showed massive gastric dilatation, with total necrosis and perforation. Total gastrectomy and Y-Roux esophagojejunostomy were performed. We believe the clinical status was caused by the mechanism of air swallowing, present in our patient and typical in RS. In fact, as reported, massive air bloat may result in a decrease of the intramural blood flow with consequential ischemia of the gastric wall. We stress the importance of early detection of the gastroenterological symptoms in these patients, with timely positioning of nasogastric tube and gastrostomy, to prevent serious complications potentially life-threatening as massive gastric necrosis.

Original languageEnglish
Pages (from-to)49-51
Number of pages3
JournalBrain and Development
Issue number1
Publication statusPublished - Jan 2006


  • Abdominal distention
  • Air swallowing
  • Gastrectomy
  • Gastric necrosis
  • Rett Syndrome

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Neurology


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