HHT (also known as Osler-Weber-Rendu disease) is an autosomal dominant disorder characterized by vascular abnormalities, as recently reviewed. There are two types of vascular lesions in HHT. The first type is the mucocutaneous telangiectasia. Telangiectasias are small focal dilations of capillaries and postcapillary venules within the mucosa resulting in direct connections between arterioles and venules. When these lesions are found within the gastrointestinal tract they may be termed angiodysplasia. The second type of lesion is the visceral AVM. AVMs are direct connections between arteries and veins that lack intervening capillaries and are larger than telangiectasias or angiodysplasia. In patients with HHT, telangiectasias and/or AVMs may be found in the lungs, brain, skin, nose, liver and pancreas, as well as throughout the gastrointestinal tract.
Telangiectasias occur in the gastrointestinal tract in about 60% of patients with HHT; AVMs are less common. GIH occurs in 10% to 45% of patients with HHT, commonly originating from telangiectasias and presenting as iron deficiency anemia. Less commonly, HHT patients will have bleeding from AVMs, presenting with sudden, massive hemorrhage . In a retrospective case series of 28 patients with HHT and GIH, 40% of patients presented with an upper GIH, 10% with a lower GIH and in 50% the bleeding site was indeterminate. EGD was performed on 17 of these patients, and while 70% had typical lesions in the stomach, no duodenal lesions were described . Duodenal telangiectasias in HHT have been reported by other authors. Cheapest online shopping – find at best fully-licensed pharmacy.