title: Sudden death due to a dissecting intramural hematoma of the esophagus (DIHE) in a woman with severe neurofibromatosis-related scoliosis.
abstract: Dissecting intramural hematoma of the esophagus (DIHE) is a rare condition in which intramural hemorrhage can lead to submucosal dissection of the esophageal wall. DIHE is generally considered a benign disease, and the only mortality associated with DIHE has been due to operative intervention or to the presence of another underlying, life-threatening condition. We report, however, a case of sudden death due to the spontaneous rupture of a DIHE that occurred in a 32-year-old woman, affected by neurofibromatosis type 1. She was admitted to the local emergency room, presenting a 24-hour history of sudden onset, severe central chest and interscapular pain associated with dysphagia, odynophagia and vomiting. Her condition worsened and proved fatal within a 6-hour period. A complete autopsy was then conducted, showing a complete dissecting intramural hematoma with laceration of the third superior of the esophagus. We can hypothesize that abnormal variations of gastro-esophageal pressure during ingurgitation and during bolus movement could be predisposing factors in the pathogenesis of the dissection. On the other hand angular kyphoscoliosis deformity may have had play a role as precipitating factor while vomiting in the subject's medical history can then be interpreted as the likely activation phenomenon.
authors: Department of Forensic Pathology, University of Foggia, Ospedale Colonnello D'Avanzo, Via degli Aviatori 1, 71100 Foggia, Italy.
authors: Cristoforo
authors: C
authors: Pomara
authors: Stefania
authors: S
authors: Bello
authors: Stefano
authors: S
authors: D'Errico
authors: Michele
authors: M
authors: Greco
authors: Vittorio
authors: V
authors: Fineschi
mesh_terms: D000328:Adult; D003645:Death, Sudden / Q000209:etiology; D004935:Esophageal Diseases / Q000473:pathology*; D004947:Esophagus / Q000293:injuries* / Q000473:pathology; D005260:Female; D049429:Forensic Pathology; D006406:Hematoma / Q000473:pathology*; D006470:Hemorrhage / Q000150:complications / Q000209:etiology; D006491:Hemothorax / Q000473:pathology; D006801:Humans; D007738:Kyphosis / Q000209:etiology*; D009456:Neurofibromatosis 1 / Q000150:complications*; D012422:Rupture, Spontaneous / Q000150:complications; D012600:Scoliosis / Q000209:etiology*; D012769:Shock / Q000209:etiology