Hereditary Hemorrhagic Telangiectasia (HHT), also known as Osler-Weber-Rendu Disease, is a genetic disease that is characterized by the aberrant overgrowth and proliferation (dysplasia) of vascular tissue known as telangiectasias and arteriovenous malformations (AVMs).
HHT is a variable disease with clinical manifestations being directly tied to lesion location and burden. For most cases the presenting symptom is repeated and persistent nose bleeds.
Los Angeles plastic surgeon, Jason Hamilton, MD, discusses Hereditary Hemorrhagic Telangiectasia and the most common presenting symptom of nosebleeds.
Hereditary Hemorrhagic Telangiectasia (HHT) is a hereditary disorder characterized by abnormal clusters of fragile capillaries that bleed with minimal agitation. Telangiectasias, commonly involve the nasal mucosa causing recurrent nosebleeds that may be severe enough to require hospitalization, surgical cauterization as well as blood and/or iron transfusions.
Hereditary Hemorrhagic Telangiectasia (HHT) is a hereditary disorder characterized by abnormal clusters of weak bulging capillaries that bleed with little to no trauma or insult. They most commonly affect the nasal mucosa, but also involve the gut, skin, brain and lungs as well. Clinically patients most commonly report a history of recurrent nosebleeds (epistaxis) before being diagnosed with HHT and may suffer from lifelong recurrent epistaxis that may increase in severity as the patient ages.
Autoimmune disorders are characterized by antibodies that turn against the body’s own normal tissues. While typical antibodies fight off viruses, bacteria, and cancer cells, pathological autoantibodies directed against the bodies own tissues can result in complex autoimmune disorders. Wegener’s granulomatosis is an autoimmune disorder that attacks blood vessels throughout the body.
We have coined the term ‘Mega-perforation’ to refer to a septal perforation that approaches the limits of reparability. Typically perforations greater than 3.0-3.5cm and/or greater than 75% of the anterior septum size fall into this category.
The “watch and wait” approach to managing any septal perforations is typically the wrong approach because perforations increase in size as time passes.