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caliber-persistent artery

Monday 12 March 2012

Caliber-Persistent Artery (Dieulafoy Lesion)

The entity variously termed caliber-persistent artery, cirsoidaneurysm, or Dieulafoy lesion tends to affect middle-aged
and elderly men.

Median patient age is 52 to 54 years,with patients ranging in age from 16 to 91.

The disorder presents as recurrent, massive, and sometimes fatal hematemesis.

Massive hemorrhage and rupture occur whena large submucosal artery impinges on the mucosa while pursuing its tortuous course through the submucosa.

The lesion is thought to represent a congenital anomaly related to defective arterial involution or elongation and even curling of a deep elongated submucosal vessel.

Because the lesions are flat, they are hard to detect.

However, the angiographic appearance is characteristic.

The lesion is often not visible endoscopically, although one may see an area of bleeding.

When endoscopically identifiable, the lesion presents as a volcanic crater with a central whitish discoloration projecting from an otherwise normal gastricmucosa.

An abnormally large submucosal vessel protrudesthrough a minute mucosal defect.

Ulceration is usually not present in the region surrounding the place where the vessel breaks through the mucosa.

In most cases, the bleeding site lies within the first 6 cm of the gastro-esophageal junction, usually on the lesser curvature.

Histologically, an abnormally large oversized tortuous muscular artery measuring about 1.5 mm in diameter runs through the submucosa and approaches the mucosa, often with superficial erosion.

Veins often accompany the lesion.

The arterial wall may show medial hypertrophy and adventitial fibrosis, but the lesion typically lacks inflammation, aneurysm formation, atherosclerosis, or dystrophic calcification.

An elastic tissue stain demonstrates the normal architecture of an arterial wall with only slight intimal hyperplasia and reduplication of the internal elastic lamina.

If the wall of the artery is eroded at the base of a mucosal ulcer, hemorrhage ensues.

The ulcer is often minute even though the hemorrhage is massive.

The overlying ulcer usually lacks the intense inflammation typical of peptic ulcer disease and is superficial with no involvement of the muscularis propria or associated mural fibrosis.

The part of the artery at the base of the ulcer usually shows focal necrosis and rupture.

A thrombus may be attached to a protruding open artery.

The artery forces the mucosa upward and a wide submucosal area characteristically exists between these arteries and the true muscularis propria.

See also

- gastric vascular anomalies