The Evolution of Dieulafoy's Lesion Since 1897: Then and Now-A Journey through the Lens of a Pediatric Lesion with Literature Review

Abstract

Background. In 1897, Dieulafoy was the first to characterize a gaping arteriole within the gastric mucosa causing massive hematemesis, designating it as "exulceratio simplex." A hundred years later, this vascular abnormality, now commonly referred to as a "Dieulafoy lesion," has been identified through the entire gastrointestinal tract and the bronchus. Objectives and Methods. As the original findings have been subjected to revisions and modifications by modern authors, Dieulafoy's original paper was reviewed and analyzed. The evolution of the current usage of "Dieulafoy's lesion" in the literature has been summarized with comparisons to the original report. Additionally, an index case of a 10-year-old female with a gastric "exulceratio simplex" is reported with a review of previously reported paediatric Dieulafoy lesions. Conclusions. The term "Dieulafoy lesion" in modern literature no longer adheres to the initial conclusions with regards to its origin, demographics, and presenting symptoms. Dieulafoy lesions remain a rare cause of gastrointestinal bleeding that can cause life-threatening haemorrhages in children.

Figure 1

Intraoperative specimen of stomach at partial gastrectomy showing the rich vascular abnormality seen within the posterior gastric wall (arrow).

Figure 2

Histopathological findings. (a) Whole-mount scan of hematoxylin and eosin stained slide showing the exulceratio simplex (∗) in the submucosa (SM) (below the mucosa (M)) as described by Dieulafoy. Additionally, (#) shows the presence of a complex rich anastomosis of varying sized vessels in the submucosa with an intraluminal haemorrhagic thrombus (cirsoid aneurysm of gastric vessels). (b) Low magnification (objective lens ×2) of hematoxylin and eosin-stained slides shows the presence of blood within the large tortuous, caliber-persistent artery () in the submucosa (SM) with evidence of rupture and haemorrhage (#) through the overlying mucosa (M). A normal-sized submucosal arteriole (∗) is seen at the same level. (c) High magnification (objective lens ×10) of hematoxylin and eosin-stained slides shows the presence of the large caliber-persistent artery () in the submucosa (SM)—Dieulafoy's lesion—with communications (#) into the overlying noninflamed mucosa (M). (d) High magnification (objective lens ×10) of elastin-stained slides shows the presence of elastin in the wall (#) of the exulceratio simplex confirming its arterial origin.