Dieulafoy’s Disease in Pregnancy: Pathophysiology, Clinical Presentation, and Management—A Case Report with Narrative Literature Review

TL;DR

A rare case of Dieulafoy’s disease during pregnancy presented with severe anemia instead of typical bleeding symptoms, highlighting the need for increased awareness of atypical presentations.

Contribution

Reports an atypical case of Dieulafoy’s disease in pregnancy presenting with severe anemia and no overt bleeding, expanding understanding of its clinical variability.

Findings

Dieulafoy’s disease in pregnancy often presents with acute bleeding, but can also manifest as severe anemia without gastrointestinal symptoms.

Upper gastrointestinal endoscopy is safe and effective for diagnosing and managing Dieulafoy’s disease during pregnancy.

Pregnancy-related physiological changes may mask typical symptoms, leading to delayed diagnosis.

Abstract

Background: Dieulafoy’s disease is a rare vascular anomaly of the gastrointestinal tract and an uncommon cause of acute upper gastrointestinal bleeding. Its occurrence during pregnancy is exceptionally rare, and the available literature is limited to isolated case reports that almost invariably describe acute and overt hemorrhagic presentations. As a result, atypical or clinically silent forms of the disease during pregnancy remain poorly characterized. Objective: To report an atypical case of Dieulafoy’s disease during pregnancy, presenting exclusively with severe progressive anemia in the absence of gastrointestinal symptoms and to contextualize this observation through a focused narrative review of the literature. Methods: An illustrative clinical case is presented, followed by a narrative review of the available literature on Dieulafoy’s disease in pregnancy. Particular attention was given to pregnancy-related physiological and hormonal adaptations, diagnostic challenges, therapeutic strategies, and reported maternal–fetal outcomes. All published cases identified in the literature were reviewed and summarized. Results: In the general population, Dieulafoy’s disease typically presents with sudden and overt gastrointestinal bleeding and is most commonly localized in the proximal stomach. In pregnancy, reported cases are rare and have almost exclusively involved acute hemorrhage occurring in the second or third trimester, frequently requiring urgent endoscopic intervention. Mechanical endoscopic hemostasis represents the treatment of choice and is generally associated with favorable maternal and fetal outcomes. In contrast, the illustrative case described herein demonstrates a clinically silent presentation, characterized by severe and progressive anemia without hematemesis, melena, or hematochezia, resulting in delayed diagnosis until upper gastrointestinal endoscopy identified multiple actively bleeding gastric Dieulafoy’s lesions. Conclusions: Dieulafoy’s disease should be considered in the differential diagnosis of severe, unexplained, or transfusion-dependent anemia during pregnancy, even in the absence of overt gastrointestinal bleeding. Pregnancy-related physiological adaptations may mask classic symptoms and complicate timely diagnosis. When clinically indicated, upper gastrointestinal endoscopy is safe and effective during pregnancy and remains central to both diagnosis and management. Increased awareness of atypical presentations may facilitate earlier recognition and improve maternal and fetal outcomes.