A Case of Hereditary Hemorrhagic Telangiectasia: Surgical Treatment with Modified Septodermoplasty / 대한이비인후과학회지

ABSTRACT

Hereditary hemorrhagic telangiectasia (HTT) or Rendu-Osler-Weber disease, identified nearly a century ago, is a familiar syndrome inherited by an autosomal dominant mode. It is characterized by recurrent epistaxis with multiple telangiectatic lesions on the mucosa and skin, basically capable of involving blood vessels in any part of the body. HTT is an important disease for otolaryngologists to familiarize with, because 90 percent of patients who experience epistaxis are referred to the department of otorhinolaryngology for its evaluation and treatment. Its occurence is not unusual in the western countries, but relatively rare in many Asian countries. We report a recent case of a 64-year-old male patient who had been suffering from GI bleeding, and experiencing recurrent epistaxsis for more than 30 years. His epistaxis was successfully treated with modified septodermoplasty.