OBSOLETE: Cerebrofacial arteriovenous metameric syndrome type 1

Cerebrofacial arteriovenous metameric syndrome type 1 (CAMS 1), also known as Bonnet-Dechaume-Blanc syndrome or Wyburn-Mason syndrome, is an extremely rare vascular condition that affects blood vessels in the face and brain. In this condition, abnormal connections form between arteries and veins (called arteriovenous malformations or AVMs) in a specific pattern along the midline of the face and the front part of the brain. These malformations follow a developmental pattern related to how blood vessels form in the embryo, specifically involving the first metameric segment (the nose, forehead, and frontal brain region). The term 'OBSOLETE' in the disease name indicates that this classification has been updated or merged into broader categories in some medical databases, but the condition itself is still recognized clinically. Patients may experience a range of symptoms depending on the size and location of the vascular malformations. These can include visible red or purple marks on the face, nosebleeds, headaches, seizures, and vision problems if the malformations involve the eye or optic pathways. In some cases, the brain AVMs can bleed, which is a serious medical emergency. The condition is present from birth, though it may not be fully recognized until symptoms develop later. There is currently no cure for CAMS 1. Treatment focuses on managing symptoms and reducing the risk of complications from the arteriovenous malformations. Options may include embolization (blocking abnormal blood vessels), surgery, or stereotactic radiosurgery, depending on the location and severity of the malformations. A multidisciplinary team of specialists is typically needed to manage this complex condition.

Bring these to your next appointment

• Q1.What is the exact location and size of my arteriovenous malformations?,What is the risk of bleeding from my brain AVM, and how can we reduce that risk?,What treatment options are available for my specific situation, and what are the risks and benefits of each?,How often should I have imaging follow-up to monitor the malformations?,Are there any activities or medications I should avoid?,Should I see an ophthalmologist regularly to check for eye involvement?,Are there any clinical trials or research studies I could participate in?

Common questions about OBSOLETE: Cerebrofacial arteriovenous metameric syndrome type 1