Hereditary Hemorrhagic Telangiectasia (HHT)/Osler-Weber-Rendu Syndrome: A Review on Contemporary Knowledge, its Accompanying Clinical Manifestations, Diagnostics, and Oro-Dental Management Plan

Abstract

HHT/OWRS is a very rare, hereditary, genetic autosomal-dominant disorder characterized by local angiodysplasias on skin and mucosae, even more rarely reported in the oral and dental health-related literature. It affects blood vessel formation throughout the body and is often accompanied with recurrent and severe episodes of epistaxis and telangiectasia on the skin and mucous membranes, including oral mucosae; and henceforth, perhaps the first sign of OWRS/HHT that can be initially diagnosed is by us, dentists/odontologists, oral and cranio-maxillo-facial surgeons, and specialized oral, dental, head, face and neck health care providers. Such symptoms may be associated with an IgA deficiency and, rarely, with von Willebrand disease. Thus, we are in an optimal position to detect the OWRS/HHT symptoms early, even if symptoms of other conditions may or do mimic OWRS/HHT oral lesions. Herein, this chapter re-visits and reviews, for the interested clinician, surgeon, scientist, researcher, and Innovation in Osteogenesis Research reader, the current knowledge on a very rare yet complex disease. Hence, the aim herein is for it to serve as a reminder as well as a contemporary clinical and surgical guide aiding in detecting, diagnosing, understanding, and efficaciously managing (including Health Care Information Management) patients suffering from OWRS or HHT.