Summary
A Dieulafoy lesion is a vascular anomaly characterized by an abnormally large, tortuous submucosal artery that erodes the overlying GI mucosa, leading to severe and sometimes recurrent bleeding without a primary ulcer, inflammation, or other vascular abnormality. It typically manifests in the fifth decade of life and more commonly occurs in men than women. Dieulafoy lesions account for a small percentage of acute GI bleeds, and the exact cause is unknown. The most common location for the lesion is the stomach. Clinical features include melena, hematemesis, and signs of hemodynamic instability. The diagnosis is primarily made via esophagogastroduodenoscopy (EGD), which may need to be repeated. If EGD is inconclusive, CT angiography or conventional angiography may be performed. Management begins with hemodynamic stabilization, followed by first-line endoscopic hemostasis (e.g., band ligation, clips, thermal coagulation). For refractory or recurrent bleeding, options include angiographic embolization or surgery.
Definitions
A Dieulafoy lesion is a vascular anomaly characterized by a tortuous, abnormally large submucosal artery that erodes the overlying mucosa, leading to severe GI bleeding without an ulcer, inflammation, or other vascular abnormality. [1][2]
Epidemiology
-
Frequency
- Accounts for ∼ 1–2% of acute GI bleeds [2]
- Accounts for up to 6.5% of acute nonvariceal upper GI bleeds [1]
- Age: can occur at any age but most commonly between 40 and 49 years of age [2][3]
- Sex: ♂ > ♀ (∼ 2:1) [2][3]
- Location
Epidemiological data refers to the US, unless otherwise specified.
Etiology
The exact cause is unknown; it is unclear if the lesion is congenital or acquired. [1][2][3]
Risk factors [1]
- Cardiovascular comorbidities
- Hypertension
- Chronic renal failure
- Diabetes mellitus
- Medications
- NSAIDs
- Anticoagulants and antiplatelet agents [3]
- Physiological stress due to medical conditions [2]
Clinical features
Patients are often asymptomatic until the onset of bleeding. [2]
- Acute, severe GI bleeding without pain [2][3]
- May be recurrent
- Common manifestations include: [2][3]
- Melena (∼ 44%)
- Hematemesis (∼ 30%)
- Both melena and hematemesis (∼ 18%)
- Hematochezia (∼ 6%)
- Signs of hemodynamic instability (e.g., tachycardia, hypotension) are common. [2][4]
- Rare: iron-deficiency anemia without overt bleeding [2]
Diagnosis
EGD [4]
- Preferred initial study
- Supportive findings [3]
- Repeat endoscopy may be necessary if initial testing is nondiagnostic. [2][4]
Additional testing
Angiography [2][3]
- May be helpful if EGD is nondiagnostic
- Options
- Findings
- Abnormally enlarged, tortuous submucosal artery
- May show active contrast extravasation
Further endoscopic testing [4]
- May be used to evaluate for a suspected distal source of bleeding
- Options
- Colonoscopy: performed after a negative EGD in patients with suspected lower GI bleeding
- Deep enteroscopy (e.g., double-balloon enteroscopy): to identify lesions in the jejunum or ileum
- Capsule endoscopy: to identify lesions in the small bowel
Differential diagnoses
- Mallory-Weiss tear [2]
- Peptic ulcer disease [3]
- Angiodysplasia [2]
- Gastrointestinal tumors (e.g., GIST) [4]
- Aneurysms [2]
- See also “Common causes of GI bleeding.”
The differential diagnoses listed here are not exhaustive.
Management
See “Initial management of overt GI bleeding” for immediate measures for acute GI bleeding, including hemodynamic stabilization for unstable patients (e.g., IV fluid resuscitation, blood transfusion). [3][4]
Endoscopic hemostasis [1]
- First-line treatment
- Techniques
- Mechanical therapy (preferred) [5]
- Endoscopic band ligation
- Clip placement
- Thermal coagulation [4][5]
- Heater probe coagulation
- Bipolar/multipolar electrocoagulation
- Argon plasma coagulation
- Sclerosant injection therapy (e.g., ethanol) [5]
- Epinephrine injection: may be used in combination with other modalities [4]
- Mechanical therapy (preferred) [5]
Alternative treatment options [1]
- Consider if endoscopic management is insufficient and in patients with recurrent bleeding.
- Options
- Catheter angiography with selective angioembolization
- Surgical therapy (e.g., wedge resection) is reserved as a last resort for bleeding that is refractory to endoscopic and angiographic treatment. [3]
Complications
- Hemorrhagic shock [1]
- Recurrent bleeding [4]
We list the most important complications. The selection is not exhaustive.