Dieulafoy Lesion

name: Dieulafoy Lesion
creation_date: '2026-01-14T23:44:30Z'
updated_date: '2026-02-16T20:19:38Z'
category: Acquired
description: >
  Dieulafoy lesion is a rare vascular anomaly characterized by a caliber-persistent submucosal
  artery that erodes the overlying mucosa without forming a true ulcer, leading to sudden,
  potentially life-threatening gastrointestinal bleeding anywhere along the GI tract.
disease_term:
  preferred_term: Dieulafoy lesion
  term:
    id: MONDO:0001427
    label: Dieulafoy lesion
parents:
- Gastrointestinal Disease
pathophysiology:
- name: Caliber-Persistent Submucosal Artery
  description: >
    An abnormally large, tortuous submucosal artery fails to taper and can erode through
    intact mucosa, predisposing to arterial bleeding without prior ulceration.
  evidence:
  - reference: PMID:35243119
    supports: SUPPORT
    snippet: "Dieulafoy's lesion is an abnormally large, tortuous, submucosal vessel that erodes the overlying mucosa, without primary ulceration or erosion."
    explanation: Review defines Dieulafoy lesion as a caliber-persistent submucosal artery that breaches mucosa without ulceration, causing bleeding.
  - reference: PMID:37425531
    supports: SUPPORT
    snippet: "A duodenal Dieulafoy lesion is characterized by the presence of a larger-caliber artery that protrudes through the GI mucosa and can lead to massive hemorrhage."
    explanation: Case review reiterates the defining pathology of a large-caliber artery protruding through mucosa leading to hemorrhage.
- name: Exposed Aberrant Vessel with Intermittent Arterial Spurting
  description: >
    The exposed artery protrudes through a tiny mucosal defect, producing intermittent,
    brisk arterial hemorrhage that can be difficult to localize endoscopically.
  evidence:
  - reference: PMID:37065413
    supports: SUPPORT
    snippet: "A Dieulafoy lesion is an aberrant vessel that does not reduce in caliber when it extends from the submucosa to the mucosa. Damage to this artery can result in severe, intermittent arterial bleeding from tiny, difficult-to-visualize vessel stumps."
    explanation: Case review highlights the persistent caliber artery and intermittent, severe arterial bleeding typical of Dieulafoy lesions.
phenotypes:
- name: Gastrointestinal Hemorrhage
  category: Gastrointestinal
  frequency: VERY_FREQUENT
  diagnostic: true
  phenotype_term:
    preferred_term: Gastrointestinal hemorrhage
    term:
      id: HP:0002239
      label: Gastrointestinal hemorrhage
  evidence:
  - reference: PMID:39839160
    supports: SUPPORT
    snippet: "Twenty-five patients (92.6%) presented with hematemesis and melena as chief complaints."
    explanation: Systematic review shows most patients present with overt gastrointestinal bleeding.
  - reference: PMID:35746982
    supports: PARTIAL
    snippet: "a 71-year-old female patient who presented with a bright red bleed per rectum"
    explanation: Colonic Dieulafoy case highlights overt lower GI bleeding presentation.
  - reference: PMID:37620810
    supports: PARTIAL
    snippet: "hematochezia of ≥ 2 episodes constituted the independent factor associated with ≥ 2 double-balloon endoscopy diagnoses"
    explanation: Small-bowel Dieulafoy series shows recurrent hematochezia is common and predicts repeat diagnostic endoscopy.
- name: Hypotension from Acute Blood Loss
  category: Cardiovascular
  frequency: FREQUENT
  phenotype_term:
    preferred_term: Hypotension
    term:
      id: HP:0002615
      label: Hypotension
  evidence:
  - reference: PMID:37065413
    supports: SUPPORT
    snippet: "These catastrophic bleeding episodes frequently result in hemodynamic instability and the need for transfusion of multiple blood products."
    explanation: Case report notes hemodynamic instability, consistent with hypotension during bleeding episodes.
biochemical:
- name: Hemoglobin
  presence: Decreased
  context: Acute blood loss anemia during bleeding episodes
  evidence:
  - reference: PMID:36636361
    supports: PARTIAL
    snippet: "Initial laboratory investigation revealed severe anemia, requiring packed red blood cell transfusion."
    explanation: Cecal Dieulafoy case report documents severe anemia from hemorrhage requiring transfusion, illustrating hemoglobin drop during bleeding.
environmental:
- name: Hypertension
  notes: Common comorbidity associated with Dieulafoy lesion presentations.
  evidence:
  - reference: PMID:35243119
    supports: PARTIAL
    snippet: "Major underlying disorders were hypertension 29%, diabetes mellitus 21%, and chronic kidney disease 16%."
    explanation: Review documents hypertension as the most frequent comorbidity in rectal Dieulafoy lesion cases.
- name: Chronic Kidney Disease
  notes: Comorbidity observed in a subset of patients with Dieulafoy lesions.
  evidence:
  - reference: PMID:35243119
    supports: PARTIAL
    snippet: "Major underlying disorders were hypertension 29%, diabetes mellitus 21%, and chronic kidney disease 16%."
    explanation: Review reports chronic kidney disease among common underlying conditions in patients with rectal Dieulafoy lesions.
- name: Diabetes Mellitus
  notes: Common comorbidity observed among Dieulafoy lesion patients.
  evidence:
  - reference: PMID:35243119
    supports: PARTIAL
    snippet: "Major underlying disorders were hypertension 29%, diabetes mellitus 21%, and chronic kidney disease 16%."
    explanation: Comorbidity profile shows diabetes in over one-fifth of rectal Dieulafoy cases.
treatments:
- name: Endoscopic Mechanical Hemostasis
  description: >
    Hemostatic clipping or band ligation applied directly to the exposed vessel to achieve
    primary hemostasis and prevent rebleeding.
  treatment_term:
    preferred_term: supportive care
    term:
      id: MAXO:0000950
      label: supportive care
  evidence:
  - reference: PMID:35243119
    supports: SUPPORT
    snippet: "In regard to treatment, endoscopic therapy was applied in 80% ... The endoscopic treatment was a feasible choice for rectal disease, with a primary hemostasis rate of 88%."
    explanation: Systematic review shows endoscopic mechanical methods are effective first-line therapy with high primary hemostasis rates.
  - reference: PMID:37425531
    supports: PARTIAL
    snippet: "The treatment of duodenal DL includes thermal electrocoagulation, local epinephrine injection, sclerotherapy, banding, and hemoclipping."
    explanation: Duodenal Dieulafoy case review lists banding and hemoclipping among standard endoscopic hemostasis options.
- name: Angiographic Embolization
  description: >
    Transcatheter arterial embolization used as a salvage option when endoscopic methods are
    not feasible or fail to control bleeding.
  treatment_term:
    preferred_term: supportive care
    term:
      id: MAXO:0000950
      label: supportive care
  evidence:
  - reference: PMID:35243119
    supports: SUPPORT
    snippet: "angiographic embolization in 4%"
    explanation: Review notes angiographic embolization employed in a subset of cases as an alternative to endoscopy or surgery.
- name: Surgical Ligation or Wedge Resection
  description: >
    Surgical oversewing or resection of the bleeding segment reserved for refractory cases
    when endoscopic and radiologic approaches fail.
  treatment_term:
    preferred_term: surgical procedure
    term:
      id: MAXO:0000004
      label: surgical procedure
  evidence:
  - reference: PMID:38113567
    supports: SUPPORT
    snippet: "Endoscopic methods ... are preferred for treatment, boasting success rates over 90 %. Surgical measures become a last resort for uncontrolled bleeding."
    explanation: Case series emphasizes surgery as rescue therapy after failed endoscopic management of Dieulafoy lesions.
  - reference: PMID:39298100
    supports: NO_EVIDENCE
    snippet: "Fatal exulceratio simplex (dieulafoy lesion)"
    explanation: Autopsy case underscores that uncontrolled Dieulafoy bleeding can be fatal, highlighting need for definitive rescue measures when endoscopy fails.
differential_diagnoses:
- name: Peptic Ulcer Disease
  description: Ulcerative mucosal defects of stomach or duodenum that commonly cause overt GI bleeding and can mimic Dieulafoy lesions endoscopically.
  disease_term:
    preferred_term: peptic ulcer disease
    term:
      id: MONDO:0004247
      label: peptic ulcer disease
  distinguishing_features:
  - Ulcer crater with fibrin base and surrounding inflammation versus normal mucosa overlying a pinpoint vessel in Dieulafoy lesions
  - Often associated with NSAID use or H. pylori; Dieulafoy lacks primary ulceration
- name: Angiodysplasia of Colon
  description: Dilated submucosal vessels typically in the colon that cause painless lower GI bleeding and can resemble vascular Dieulafoy bleeding sites.
  disease_term:
    preferred_term: angiodysplasia
    term:
      id: MONDO:0002322
      label: angiodysplasia
  distinguishing_features:
  - Typically multiple flat vascular ectasias; Dieulafoy is solitary with a protruding caliber-persistent artery
  - Bleeding is often low-grade and occult; Dieulafoy bleeding is brisk arterial
- name: Gastric Antral Vascular Ectasia (GAVE)
  description: Watermelon stomach with dilated antral vessels producing chronic or acute upper GI bleeding that must be distinguished from Dieulafoy lesions.
  disease_term:
    preferred_term: gastric antral vascular ectasia
    term:
      id: MONDO:0006767
      label: gastric antral vascular ectasia
  distinguishing_features:
  - Endoscopic “watermelon” stripes and diffuse antral ectasia versus focal point bleeding in Dieulafoy
  - Often associated with portal hypertension or systemic sclerosis; Dieulafoy lesions occur without diffuse mucosal changes
- name: Esophageal Varices
  description: Dilated submucosal veins in the esophagus due to portal hypertension that can cause massive upper GI bleeding.
  disease_term:
    preferred_term: esophageal varices
    term:
      id: MONDO:0001221
      label: esophageal varices
  distinguishing_features:
  - Multiple serpiginous venous columns with red wale signs versus solitary arterial stump in Dieulafoy
  - Strong association with portal hypertension and cirrhosis; Dieulafoy may occur without liver disease
- name: Gastric Carcinoma
  description: Malignant epithelial tumor of the stomach that can ulcerate and bleed, mimicking Dieulafoy-related hemorrhage.
  disease_term:
    preferred_term: gastric carcinoma
    term:
      id: MONDO:0004950
      label: gastric carcinoma
  distinguishing_features:
  - Mass or irregular ulcerated lesion with friable tissue versus normal-appearing mucosa in Dieulafoy
  - Constitutional symptoms and imaging evidence of mass help differentiate from focal vascular lesion
- name: Meckel Diverticulum
  description: Congenital ileal diverticulum that may harbor ectopic gastric mucosa causing brisk lower GI bleeding, particularly in younger patients.
  disease_term:
    preferred_term: Meckel diverticulum
    term:
      id: MONDO:0007955
      label: Meckel diverticulum
  distinguishing_features:
  - Technetium-99m pertechnetate uptake (Meckel scan) localizes ectopic mucosa; Dieulafoy requires endoscopic visualization
  - Bleeding often in children/young adults; Dieulafoy more often in older adults with comorbidities