PAEF is a rare but often life-threatening cause of massive gastrointestinal bleeding. PAEFs have a mortality rate of nearly 100% in the absence of surgical intervention, and in most case, the diagnosis is not established preoperatively. This report presents one such case wherein the cause of death was PAEF-induced massive gastrointestinal bleeding that occurred after discharge of the patient from the hospital. Few previous reports on PAEF provide both the clinical course, including gastrointestinal endoscopic findings, and detailed autopsy findings.
A research team was led by Dr. Ihama address this case and was published on August 7, 2008 in the World Journal of Gastroenterology.
They found that a considerable number of sudden and unexpected deaths have occurred due to PAEFs in cases wherein the diagnosis had not been established. The incidence of PAEF is probably higher than estimated. Moreover, PAEF is a fatal but not an untreatable gastrointestinal disease. However, a PAEF patient cannot be saved without early diagnosis because only an appropriate and urgent surgical invention can rescue the patient. The classical triad of symptoms, i.e., gastrointestinal bleeding, abdominal pain, and a pulsating abdominal mass is probably overemphasized because it occurs in less than 25% of PAEF cases. On the contrary, PAEFs usually present with a herald bleed prior to exsanguination, which is usually minor and self-limiting. If PAEF is diagnosed simultaneously with the herald bleeding, surgical therapy may salvage many patients of PAEF.
Endoscopy is generally the most preferred primary procedure and provides valuable information in cases of gastrointestinal bleeding. In PAEF patients, absence of identifiable bleeding lesions on initial gastrointestinal endoscopy is regarded by clinicians as a strong indicator for laparotomy. However, gastrointestinal endoscopy may be a double-edged sword when PAEFs coexist with multiple bleeding sites. Furthermore, even detection of coexisting bleeding sites by upper endoscopy or colonoscopy, even by capsule endoscopy, may be misleading. Ultimately, the key to early diagnosis of PAEF is endoscopist's heightened index of suspicion; therefore, endoscopists need to recognize PAEF as a potential cause of gastrointestinal bleeding. This article is a valuable step to publicize PAEF.