Dieulafoy Lesion

Epidemiology

• Affects predominantly males (2:1 ratio), often elderly
• Up to 90% of cases have significant comorbidities (cardiovascular disease, chronic kidney disease, diabetes, alcohol use)

Pathophysiology

• Histologically: A large, tortuous artery (1–3 mm) persists into the submucosa and ruptures through a 2–5 mm mucosal defect
• No inflammation or ulceration is typically present
• Not aneurysmal in the classical sense, but may rupture due to mucosal ischaemia or mechanical erosion.

Common Sites

• 70–80% in the stomach, particularly the proximal lesser curvature within 6cm of the gastroesophageal junction
• Common alternate sites: duodenum; colon and rectum; jejunum and ileum; oesophagus
• Rarely: bronchus, anal canal

Clinical Presentation

• Sudden, often massive, gastrointestinal bleeding
• Haematemesis; Melaena; Hematochezia (if lower GI site)
• Typically no prior history of GI disease

Diagnosis

Endoscopy (first-line)

• Diagnostic in 49–92% of cases; May require multiple procedures
• Endoscopic criteria:
  1. Active arterial spurting or micropulsatile bleeding from a pinpoint mucosal defect
  2. Visible protruding vessel with or without bleeding
  3. Fresh adherent clot without ulcer base

Other modalities:

• Angiography (if endoscopy fails)
• CT angiography
• Endoscopic ultrasound (EUS)
• Capsule endoscopy or intraoperative enteroscopy (for small bowel lesions)

Management

Endoscopic Therapy (first-line)

• Haemostasis success >90%
  • Injection (e.g., epinephrine, sclerotherapy)
  • Thermal coagulation (heat probe, argon plasma)
  • Mechanical (hemoclip, band ligation)
  • Combination therapies are superior to monotherapy

Angiographic Intervention: Lesions inaccessible to endoscopy or recurrent bleeding

Surgical Resection: Reserved for refractory cases or when precise localization allows targeted resection

Prognosis

Mortality has dropped from 80% (pre-endoscopy era) to 8.6% with modern diagnosis and treatment

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History of the Dieulafoy Lesion

1876 – Théophile Jean Gallard (1828-1887) presents two cases of sudden fatal gastric haemorrhage due to “de petits anévrysmes miliaires” of the stomach at the Association Française pour l’Avancement des Sciences in Clermont-Ferrand.

1877 – Sir Richard Douglas Powell (1842-1925) reported on a small aneurysm of the coronary artery in an ulcer of the lower curvature of the stomach in a 44-year-old man who died suddenly after massive gastric haemorrhage. At autopsy, the stomach contained dark blood, and within a shallow ulcer on the lesser curvature was

a small aneurysm, the size of a pea, presenting at its summit an opening through which the haemorrhage had proceeded… communicating with a subjacent branch of the coronary artery of the stomach.

Powell 1877

1884 – Gallard publishes a third case and reviews all three in Anéurismes miliaires de l’estomac, concluding:

1898 – Georges Dieulafoy (1839–1911) coins the term exulceratio simplex and provides the first comprehensive clinicopathological description. He described cases of sudden, fatal haematemesis due to minute ulcers eroding large submucosal vessels at the gastric cardia and fundus. He emphasised that these lesions were distinct from ordinary peptic ulcers and could bleed massively without warning.

Histological specimen from Exulceratio simplex: Leçons 1–3, Clinique médicale de l’Hôtel-Dieu de Paris. 1898. The figure legend captures the critical point: the mucosal defect is tiny, but it overlies a disproportionately large submucosal arteriole, rupture of which produces “hémorragies mortelles.”

Surgical excision became the mainstay treatment following Dieulafoy’s recommendations. With advancements in endoscopic haemostasis through the late 20th century, mortality from approximately 80% dropped to under 10%.

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Associated Persons

• Sir Richard Douglas Powell (1842-1925)
• Théophile Jean Gallard (1828-1887)
• Georges Dieulafoy (1839–1911)

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Alternative names

• Cirsoid aneurysm
• Submucosal arterial malformation
• Caliber-persistent artery
• Exulceratio simplex (Dieulafoy, 1898)

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References

Historical references

• Powell RD. Small aneurysm of the coronary artery in an ulcer of the lower curvature of the stomach. Transactions of the Pathological Society of London 1878; 29: 133-135
• Gallard T. Anéurismes miliaires de l’estomac, donnant lieu à une hématémèses mortelle. Bulletin et mémoires de la Société médicale des hôpitaux de Paris, 1884; 1: 84-91.
• Dieulafoy G. Exulceratio simplex. L’intervention chirurgicale dans les hématémèses foudroyantes consécutives à l’exulcération simple de l’estomac. Bulletin de l’Académie nationale de médecine 1898; 39: 49-84.
• Dieulafoy G. Exulceratio simplex. Clinique médicale de l’Hôtel-Dieu de Paris, 1898: II

Eponymous term review

• Lee YT, Walmsley RS, Leong RW, Sung JJ. Dieulafoy’s lesion. Gastrointest Endosc. 2003 Aug;58(2):236-43.
• Baxter M, Aly EH. Dieulafoy’s lesion: current trends in diagnosis and management. Ann R Coll Surg Engl. 2010 Oct;92(7):548-54.
• Karamanou M, Fiska A, Demetriou T, Androutsos G. Georges-Paul Dieulafoy (1839-1911) and the first description of “exulceratio simplex”. Ann Gastroenterol. 2011;24(3):188-191.
• Senger JL, Kanthan R. The Evolution of Dieulafoy’s Lesion Since 1897: Then and Now-A Journey through the Lens of a Pediatric Lesion with Literature Review. Gastroenterol Res Pract. 2012;2012:432517.
• Kolli S, Dang-Ho KP, Mori A, Gurram K. The Baader-Meinhof Phenomenon of Dieulafoy’s Lesion. Cureus. 2019 May 3;11(5):e4595.
• Talia FM, Fatima A. Dieulafoys Lesion Causing Gastrointestinal Bleeding. National Library of Medicine, StatPearls, 2023.