Hereditary Hemorrhagic Telangiectasia (HHT)

Formerly Osler-Weber-Rendu

Your Path

  • Hereditary Hemorrhagic Telangiectasia (HHT)

Etiology

  • Rare congenital d/o
  • AVMs within lungs, nose, mouth, GI tract→ duodenum, stomach, colon
  • Right to left shunt that occurs within AVMs can be path for paradoxical emboli (stroke, CNS abscess)

Symptoms

  • Nose bleeds
  • SOB
  • Onset typically in teens
  • Cryptic GI bleeds/iron defic anemia
  • Cryptic stroke or CNS abscess

Physical Exam Findings

  • Recurrent nose bleed
  • Telangiectasis on nasal mucosa, lips, tongue