ICD-10 Code I78.0 – Hereditary hemorrhagic telangiectasia (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code I78.0 – Hereditary hemorrhagic telangiectasia
What it is
I78.0 identifies hereditary hemorrhagic telangiectasia, an inherited vascular disorder also called Osler-Weber-Rendu syndrome. It causes abnormal blood vessels that can bleed easily and form direct artery-to-vein connections.
Clinical signs
Typical findings include recurrent nosebleeds, visible telangiectasias on the skin or mucosa, and a family history of the condition. Clinical features vary; refer to documentation for confirmed organ involvement such as pulmonary, cerebral, or gastrointestinal vascular lesions.
When to use this code
Use I78.0 when the provider documents hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu syndrome. Code it for confirmed diagnosis, even if the current encounter is for screening, evaluation, or management of bleeding related to the disorder.
Assign this code when the record links abnormal vascular lesions or recurrent hemorrhage to HHT. If the note only mentions unexplained epistaxis or a vascular malformation without the inherited syndrome, check documentation before coding.
Do not use for
Do not use I78.0 for isolated nosebleeds, simple telangiectasia, or noninherited vascular malformations without HHT. Check documentation if the diagnosis is only suspected or not clearly stated.
Coding tip
Look for the exact syndrome name or a documented family history plus characteristic vascular findings before assigning I78.0.