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.

Intramural hematoma of the esophagus (IHE) is a rare but well-documented condition that is part of the spectrum of esophageal injuries which includes the more common Mallory-Weiss tear and Boerhaave's syndrome. Acute retrosternal or epigastric pain is a common clinical feature, which can be accompanied by dysphagia, odynophagia, or hematemesis. An early differentiation from Mallory-Weiss tear, Boerhaave syndrome, ruptured aortic aneurysm, aortic dissection, acute myocardial infarction, or pulmonary pathology can be difficult. Computed tomography (CT) is the imaging modality of choice and characteristically reveals a concentric or eccentric thickening of the esophageal wall with well-defined borders and variable degree of obliteration of the lumen. Measurement of the attenuation values within the lesion will reveal blood density which varies according to the age of the hematoma. CT should be considered the preferred diagnostic technique, thereby facilitating proper clinical management. Early diagnosis is crucial as most patients may be treated conservatively with good outcome.