ABSTRACT

Rendu-Osler-Weber disease (hereditary hemorrhagic telangiectasia) is an autosomal dominant familial angiogenesis disorder which presents with the clinical consequences of vascular malformations in mucocutaneous tissues, visceral organs and central nervous system. The disorder is characterized with multiple dermal, mucosal, visceral telengiectasies and accompanying bleeding. Bleeding mostly presents with epistaxis but gastroenteric, urinary or airway bleeding may also occur. Arteriovenous malformations which cause capillary bypass are quite frequent (gastrointestinal, pulmonary, neurologic). Classical triad is telengiectasy, recurrent epistaxis and family history. Patients may present in many different forms such as recurrent epistaxis, cerebrovascular events, gastrointestinal bleeding, pericardial effusion or tamponade, myocardial ischemia, haemoptysis, haemothorax, paradoxical emboli, and syncope. We report a case of uncommon clinical manifestation and management of Rendu-Osler-Weber patient who was admitted to emergency department.