Site | Incidence | Presentation patterns | Evaluation | Treatment |
Nasal telangiectasia | >90% | Nose bleeds are usually the first manifestation of HHT, frequently commencing in childhood. | History, inspection |
|
Mucocutaneous telangiectasia | 50 to 80% | Increase in size and number with age. Main concerns are cosmetic. May hemorrhage. | Inspection (oral, mucosa, conjunctivae, face, trunk, extremities, nail beds) |
|
Gastrointestinal telangiectasia | 11 to 40% | Onset generally over 30 years: Iron deficiency anemia, occasionally acute gastrointestinal hemorrhage. | Flexible endoscopy, endoscopy angiogram, capsule endoscopy |
|
Pulmonary AVMs | >50% | Usually silent. Cyanosis, clubbing, bruit, dyspnea, paradoxical embolism, cerebral abscess. | Chest radiography, blood gas measurement, helical CT, angiography, chest echocardiography |
|
Cerebral AVMs | 10 to 15% | Usually silent. Headache, epilepsy, ischemia, intracerebral hemorrhage. | CT, MRI, Doppler sonography, angiography |
|
Hepatic AVMs | 30 to 70% | Usually silent. Hepatic artery-hepatic vein AVMs: Hyperdynamic circulation. Portasystemic shunts: Ascites and encephalopathy. | Doppler sonography, CT, MRI |
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