Hereditary hemorrhagic telangiectasia

This code represents Osler-Weber-Rendu disease, a genetic disorder characterized by malformations of blood vessels (telangiectasias and arteriovenous malformations) that can lead to recurrent bleeding, particularly epistaxis, gastrointestinal hemorrhage, and iron deficiency anemia. These vascular lesions can occur in various organs, including the skin, mucous membranes, lungs, liver, and brain.

Use this code for patients diagnosed with hereditary hemorrhagic telangiectasia (HHT) based on clinical criteria (e.g., recurrent spontaneous epistaxis, telangiectasias, visceral AVMs, family history) or genetic testing. This diagnosis is typically made by a specialist, such as a geneticist, gastroenterologist, or pulmonologist.

• Always code any associated manifestations or complications, such as epistaxis (784.0), gastrointestinal hemorrhage (578.9), or iron deficiency anemia (280.9).
• Be mindful of sequencing; if HHT is the primary reason for the encounter, it should be listed first.
• Ensure documentation clearly supports the diagnosis of HHT, including clinical findings and/or genetic confirmation.