What is a Dieulafoy Lesion?
A Dieulafoy lesion is a tortuous, abnormally dilated submucosal artery that penetrates through the gastrointestinal mucosa without an overlying ulcer, accounting for 1-2% of acute gastrointestinal bleeding cases and carrying a very high mortality risk if not promptly diagnosed and treated. 1, 2, 3
Anatomic Characteristics
- The lesion consists of a large-caliber submucosal artery (1-3mm diameter) that erodes through the mucosa over time, creating a small mucosal defect through which the vessel protrudes and becomes exposed to luminal stress and rupture 3, 4, 5
- Most commonly located in the stomach (particularly the posterior aspect near the gastroesophageal junction), but can occur throughout the entire GI tract including duodenum, small bowel, colon, rectum, and esophagus 1, 6, 4
- The absence of an underlying ulcer crater distinguishes it from peptic ulcer disease, making it particularly difficult to identify endoscopically 3, 4
Clinical Presentation
- Presents with sudden, massive gastrointestinal hemorrhage—typically hematemesis and melena in upper GI lesions, or hematochezia in lower GI lesions 6, 7, 8
- Bleeding can be intermittent, leading to diagnostic challenges as the lesion may not be actively bleeding during initial endoscopy 3, 6
- Median number of endoscopic examinations required for diagnosis is 2, reflecting the difficulty in detection 6
- Patients typically present with hemodynamic instability requiring aggressive resuscitation and transfusion 3, 7
Patient Demographics and Risk Factors
- Mean age of presentation is approximately 58-74 years, with male predominance (61-62% of cases) 6, 7
- Antiplatelet agent use (aspirin, clopidogrel) has a statistically significant relationship with early rebleeding (p=0.003), making this the most important modifiable risk factor 6
- Anticoagulation use is common (present in approximately 50% of cases) but does not independently predict early relapse 6, 7
- Alcohol use, smoking, and gender do not significantly affect outcomes 7
Management in Patients on Anticoagulants/Antiplatelets
Immediate Resuscitation (First Priority)
- Aggressive volume resuscitation with two large-bore IV catheters and normal saline infusion to stabilize vital signs must precede any diagnostic attempts 3, 9
- Transfuse packed red blood cells when hemoglobin <100 g/L in acute bleeding or when bleeding is severe 3
- Monitor pulse, blood pressure, and urine output continuously (target >30 mL/hour) 3, 9
Anticoagulation Management
- For warfarin: interrupt immediately at presentation for low-risk hemorrhage; for severe hemorrhage, reverse with prothrombin complex concentrate and vitamin K 1, 9
- For DOACs: withhold medication immediately; for life-threatening hemorrhage, consider specific reversal agents (idarucizumab for dabigatran, andexanet for anti-factor Xa inhibitors) 1, 9
- Correct coagulopathy if INR ≥1.5 with fresh frozen plasma and vitamin K 9
Antiplatelet Management
- Antiplatelet agents should be withheld in the acute setting despite evidence of poorer long-term cardiovascular outcomes, as these patients have significantly higher early rebleeding rates 1, 6
- No pharmacologic agent can reverse platelet dysfunction from antiplatelets; management relies on withholding the medication and allowing platelet function to recover over days 1
Diagnostic Approach
For Hemodynamically Stable Patients
- Upper endoscopy (esophagogastroduodenoscopy) should be performed as the first diagnostic study after resuscitation, successfully identifying the source in 95% of cases 1, 3
- Endoscopy within 24 hours of admission reduces resource utilization, decreases transfusion requirements, and shortens hospital stays 1
- If initial endoscopy is negative but clinical suspicion remains high, repeat endoscopy is warranted as the lesion may be intermittently bleeding 3, 6
For Hemodynamically Unstable Patients
- CT angiography should be considered as first-line imaging in unstable patients with suspected active bleeding, with sensitivity of 81% in high-risk patients (requiring ≥500 mL transfusion) 1, 3
- CTA can visualize Dieulafoy lesions and is most conspicuous on arterial phase imaging 1, 3
- Multiphase CT enterography can detect lesions, particularly those beyond the reach of standard endoscopy 3
Critical Diagnostic Pitfalls
- Positive oral contrast in the GI tract obscures active hemorrhage and interferes with subsequent endoscopy, angiography, or CT—never administer oral contrast in acute GI bleeding 1
- Sensitivity decreases to 50% in low-risk patients with slower bleeding rates, so negative CTA does not exclude the diagnosis 1
- Barium studies have no role and should never be performed in acute bleeding 1
Endoscopic Treatment
First-Line Therapy
- Endoscopic mechanical therapy with hemoclips is first-line treatment, particularly useful for actively bleeding large vessels with efficacy similar to band ligation 3
- Band ligation is equally effective as hemoclipping for mechanical hemostasis 3
- Primary hemostasis is achieved in 92-95% of cases with endoscopic therapy 3, 6, 7
Combination Therapy
- Epinephrine injection alone has higher rebleeding rates and should not be used as monotherapy 3
- Combination therapy (epinephrine injection plus thermal methods or mechanical therapy) may be more effective than injection alone for active arterial bleeding 3
- Local epinephrine injection followed by hemostatic clipping is a commonly used and effective combination 4
Post-Treatment Management
- High-dose proton pump inhibitor therapy is mandatory after successful endoscopic therapy 3
- Patients who are hemodynamically stable 4-6 hours after endoscopy can begin oral intake 3
- Close monitoring of vital signs with continuous observation is required 3
Rebleeding Risk and Long-Term Outcomes
- Early rebleeding occurs in approximately 19% of patients, with 12 cases during hospitalization and 2 cases after median 51 months 6
- Patients on antiplatelet therapy are significantly more likely to have early relapse (p=0.003), making this the single most important predictor 6
- Long-term prognosis is excellent, even in patients treated only with endoscopic methods, with no difference in outcomes based on age, gender, hemoglobin at presentation, presence of shock, or anticoagulation status 6
- Overall mortality is 17%, primarily associated with comorbidities rather than the Dieulafoy lesion itself, though death from exsanguination occurs in approximately 5% of cases 7
Alternative Interventions for Refractory Cases
- For lesions beyond the reach of standard endoscopy (small intestine), deep enteroscopy or intraoperative enteroscopy may be required 3
- Interventional radiology with angiographic embolization is reserved for endoscopic failures (required in <3% of cases) 6
- Surgical intervention is rarely needed (required in approximately 1-2% of cases) and should only be considered after all endoscopic and radiological options are exhausted 6
Special Considerations
- In patients with recurrent bleeding from Dieulafoy lesions, evaluate for underlying coagulopathies or review anticoagulant/antiplatelet medication necessity 3
- Colonic Dieulafoy lesions are remarkably rare but should be considered when no clear culprit is discovered during colonoscopy, particularly with concomitant antiplatelet or anticoagulant use 4, 8
- The lesion can present with variable severity from mild non-threatening bleeding to massive recurrent hemorrhage requiring ICU admission 8