Angiodysplasia
Gastric antral vascular ectasia (“watermelon stomach”)
Telangiectasia associated with multisystem disease (e.g. hereditary hemorrhagic telangiectasia, CREST syndrome, Turner syndrome)

Vascular tumors
Hemangiomas
Multiple hemangioma syndromes (e.g. intestinal hemangiomatosis, universal hemangiomatosis, blue rubber bleb nevus syndrome, Klippel–Trénaunay–Weber syndrome)
Malignant vascular tumors (e.g. angiosarcoma, hemangiopericytoma, Kaposi sarcoma)

Other vascular lesions
Dieulafoy lesion
Miscellaneous (e.g. multiple phlebectasia, pseudoxanthoma elasticum, Ehlers–Danlos syndrome)

CREST, syndrome of calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.

Management and course

Initial management consists of fluid resuscitation and replacement of blood loss. Acid suppression is of no benefit. Endoscopic therapy is used to treat the Dieulafoy lesion: injection therapy with epinephrine or polidocanol, bipolar electrocoagulation, thermal coagulation, laser photocoagulation, band ligation, and hemoclips. Up to 85% of patients achieve long-term hemostasis with this approach. Angiography with selective left gastric artery embolization has been used with limited success to treat gastric Dieulafoy lesions. If nonsurgical attempts to control bleeding fail, surgical vessel ligation, wedge resection, or proximal partial gastrectomy may be necessary. Colonic lesions unresponsive to endoscopic therapy should be managed surgically. Mortality averaged 25% in the past but diagnostic and therapeutic advances have dramatically improved the survival of patients with this condition.

Key practice points: Dieulafoy lesions

A Dieulafoy lesion is identified endoscopically as a pigmented protuberance, possibly an adherent clot with little surrounding edema or ulceration.
Endoscopic management includes clipping, banding, electrocautery, or injection therapy.

Angio-ectasia

Angio-ectasias consist of dilated, tortuous, thin-walled blood vessels lined by endothelium with little or no smooth muscle. Vascular ectasias that occur in association with lesions of the skin or other organs are termed telangiectasias.

Clinical presentation

Angio-ectasia clinically manifests with painless gastrointestinal hemorrhage. Most colonic lesions are located in the right colon and are associated with low-grade chronic bleeding or iron deficiency anemia but 10–15% of patients present with acute massive hemorrhage. Up to 60% of patients have multiple angio-ectasias within the same portion of the intestinal tract. Although the percentage of angio-ectatic lesions that bleed is unknown, autopsy series suggest that most do not produce clinically evident bleeding.

Diagnostic investigation

Endoscopy is the procedure of choice for diagnosing angio-ectasia. Upper gastrointestinal endoscopy, small bowel enteroscopy, capsule endoscopy, and colonoscopy are the primary methods for identifying angio-ectasia in the gastrointestinal tract. The lesions range in diameter from 0.2 to 1.0 cm and typically are discrete and bright red, composed of a dense reticular network of vessels. Angiography may identify colonic angio-ectasia overlooked on colonoscopy or angio-ectasia lesions in the small intestine not visualized by enteroscopy plus capsule endoscopy. The characteristic angiographic findings include a vascular tuft during the arterial phase of the study, rapid filling of the dilated vein, and slowly emptying veins. Because angio-ectasias bleed only intermittently, angiography demonstrates active bleeding in only 10–20% of patients. ^99mTc-labeled erythrocyte scintigraphy is more sensitive in detecting acute hemorrhage but it can identify only the general region of bleeding. Patients with acute lower tract bleeding should undergo emergency colonoscopy or erythrocyte scintigraphy as the initial imaging procedure. Positive erythrocyte scans should be followed by angiography or colonoscopy.

Management and course

Many angio-ectasias are asymptomatic and are incidentally noted by endoscopy for nonbleeding indications. About one-quarter of patients who bleed from angio-ectasias experience recurrent hemorrhage within 1 year, and one half rebleed over a 3-year period. Any therapeutic intervention for these patients must consider this natural history of angio-ectasia. Patients with mild, chronic blood loss who do not require transfusion are best managed conservatively with oral iron supplements. Several reports have suggested a benefit from estrogen alone or in combination with progesterone, especially for patients with renal failure or hereditary hemorrhagic telangiectasia. Other series have failed to demonstrate decreased transfusion requirements.

Although the efficacy of hormonal therapy for sporadic angio-ectasias remains in question, an empirical trial of oral contraceptives containing low-dose estrogen is often worthwhile for selected patients. Gynecomastia in males and recurrent menstruation in postmenopausal females may limit compliance. Hormonal therapy should be avoided for patients with histories of thromboembolism, atherosclerotic disease, or hormone-sensitive neoplasms.

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