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.
URGENT INFORMATION on Vascular EDS Emergencies
ARTERIAL RUPTURE IS THE MOST COMMON CAUSE OF SUDDEN DEATH.
Arterial or intestinal rupture commonly presents as acute abdominal or flank pain that can be diffuse or localized.
Spontaneous arterial rupture is most likely to occur in a person’s twenties or thirties, but can occur at any point in life.
Cerebral arterial rupture may present with altered mental status and be mistaken for drug overdose.
Mid-size arteries are commonly involved.
Arterial, intestinal or uterine fragility or rupture usually arise in EDS Vascular Type, but should be investigated for any EDS type.
CAROTID-CAVERNOUS FISTULA: LIFE-THREATENING EMERGENCY
Emergency consideration should be given to any Vascular EDS patient who becomes aware of redness and pain in 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
By James H. Black III, MD, FACS and George Arnaoutakis, MD, with illustrations by Jennifer E. Fairman, CMI, FAMI
Below is a silent animation by these authors that shows the types of aortic dissection and how they form.
See also EDNF’s Clinical Reference Manual: Vascular Type (a full-color, 20-page PDF) with detailed information on diagnosis, treatment, surgery/emergency medicine and an extensive glossary. There is also a quick reference PDF pamphlet available, our Vascular Type EDS Medical Resource Guide.