Vascular Ehlers-Danlos syndrome

This code identifies Vascular Ehlers-Danlos Syndrome (vEDS), a rare, inherited connective tissue disorder characterized by fragile blood vessels, organs, and skin. It results from mutations in the COL3A1 gene, leading to defective type III collagen. Patients are at high risk for arterial, intestinal, and uterine rupture.

Use this code for patients with a confirmed diagnosis of vEDS, typically established through genetic testing or clinical criteria. This code is appropriate for documenting the underlying genetic condition contributing to various vascular, gastrointestinal, or obstetric complications. Documentation should clearly state the diagnosis of Vascular Ehlers-Danlos syndrome.

• Do not confuse with other types of Ehlers-Danlos syndrome; ensure documentation specifies "vascular type."
• Sequence as a primary diagnosis when vEDS is the reason for the encounter, or as a secondary diagnosis when managing complications related to vEDS.
• Code any associated complications (e.g., arterial dissection, organ rupture) in addition to this code.