Vascular Type EDS is considered the most serious form of EDS due to the possibility of arterial or organ rupture. If a patient presents with signs of chest, abdominal pain (etc.) it should be considered a TRAUMA SITUATION. Patient complaints should be immediately investigated using MRA, MRI or CT-Scan testing — not x-rays.

CAROTID-CAVERNOUS FISTULA: LIFE-THREATENING EMERGENCY

Emergency consideration should be given to any Vascular EDS patient who becomes aware of redness, pain and prominence of one or both eyes and the sound of pulsations in their head: this can be a manifestation of a life-threatening carotid-cavernous fistula.

In this emergency condition, high pressure blood from the internal carotid artery can pass directly into veins behind the eye, which shunts blood inappropriately into the tissue around the eyes and into the eye itself, thereby causing the presenting symptoms.

The greater risk is that the high pressure blood will leak out of the confines of the blood vessels and that could be life-threatening.

It is absolutely critical to seek immediate hospital-based medical attention, and to inform emergency medical staff of the patient’s Vascular EDS and the risk of a carotid-cavernous fistula.

Understanding Vascular Complications: A Primer of Essential Definitions (PDF)

By James H. Black III, MD, FACS and George Arnaoutakis, MD, with illustrations by Jennifer E. Fairman, CMI, FAMI

There is a silent animation by these authors that shows the types of aortic dissection and how they form. It is available in two formats; click on your choice of format, and the animation will either begin to play or you will be offered a choice to save it to your drive. In Windows Media Video .wmv format (5 MB) here; in Quicktime Movie .mov format (14 MB) here.

See also EDNF’s Clinical Reference Manual: Vascular Type (a full-color, 20-page PDF) with detailed information on diagnosis, treatment, surgery/emergency medicince, and an extensive glossary. There is also a quick reference PDF pamphlet available, our Vascular Type EDS Medical Resource Guides.