title: A case of massive gastric necrosis in a young girl with Rett Syndrome.
abstract: 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.
authors.affiliation: Pediatric Surgery Unit, Università di Roma La Sapienza, Via del Casalaccio 14, 00046 Grottaferrata Rome, Italy. emanuele.baldassarre@uniroma1.it
authors.forename: Emanuele
authors.initials: E
authors.lastname: Baldassarre
authors.forename: Giorgio
authors.initials: G
authors.lastname: Capuano
authors.forename: Gabriele
authors.initials: G
authors.lastname: Valenti
authors.forename: Paolo
authors.initials: P
authors.lastname: Maggi
authors.forename: Andrea
authors.initials: A
authors.lastname: Conforti
authors.forename: Ilaria Prosperi
authors.initials: IP
authors.lastname: Porta
mesh_terms: D000293:Adolescent; D005260:Female; D005774:Gastrostomy / Q000379:methods; D006801:Humans; D007441:Intubation, Gastrointestinal / Q000379:methods; D009336:Necrosis; D015518:Rett Syndrome / Q000150:complications*; D013272:Stomach Diseases / Q000209:etiology* / Q000473:pathology / Q000601:surgery