Diagnosis and management of gastrointestinal bleeding in patients with hereditary hemorrhagic telangiectasia

Abstract

Some HHT patients with GI bleeding improve on drug therapies, but others fail. Transfusion-dependent GI bleeding is difficult to manage, and optimal management may include both medical and endoscopic treatments.

Hereditary haemorrhagic telangiectasia: a cause of preventable morbidity and mortality

“…Humidification of the air and lubrication of the lining of the nose help keep the mucous membrane of the nose moist and can reduce nosebleeds [10]. The hormonal and other pharmacological treatments that are used for GI bleeding related to HHT may also be effective in epistaxis [12]. The tendency to haemorrhage in HHT patients may also be modified by variations in fibrinolysis; reducing excessive fibrinolysis with aminocaproic acid has had some beneficial effects in some but not all patients tested [6].…”

“…Also, epistaxis can lead to swallowing of blood, which can mimic GI bleeding, further complicating recognition of the source of blood loss [12]. Telangiectases can be distributed throughout the GI tract, but are most common in the stomach, duodenum and jejunum [12]. They are usually similar in size and appearance to those on the nasal and oral mucosa, but larger AVMs and angiodysplasias may also be present [1,2].…”

“…GI bleeding occurs from telangiectases in 25-33% of patients with HHT, and can cause severe anaemia and high blood transfusion requirements, especially in the 6th and 7th decades of life [12,19]. Under-recognised epistaxis is a more common cause of iron deficiency anaemia than GI bleeding [2].…”

“…GI bleeding in HHT can be hard to identify, as many HHT patients already take supplemental iron, which can result in chronically dark stools, making identification of melaena difficult. Also, epistaxis can lead to swallowing of blood, which can mimic GI bleeding, further complicating recognition of the source of blood loss [12]. Telangiectases can be distributed throughout the GI tract, but are most common in the stomach, duodenum and jejunum [12].…”

Subtotal Splenectomy and Central Splenorenal Shunt for Treatment of Bleeding from Roux En Y Jejunal Loop Varices Secondary to Portal Hypertension

“…The prevalence of gastrointestinal bleeding (GI) bleeding from ectopic varices is low, ranging from 1 to 5% [4,5]. Enterostomies are the most frequently affected site of nongastroesophageal variceal bleeding [6][7][8][9][10].…”