| Clinical manifestation | Evaluation | Prevention | Management |
| Arterial aneurysm, dissection, or rupture | - Use noninvasive imaging for surveillance (ultrasound, MRI, CT angiography).
| - Provide education about avoiding trauma and seeking medical attention for concerning symptoms.
- Avoid invasive arteriography if possible.
- Treat hypertension.
| - Use medical or surgical intervention as appropriate based on risk-benefit analysis.
|
| Intestinal rupture | | - Avoid routine colonoscopy; consider alternative methods of bowel cancer screening, including stool tests and genetic testing.
- Provide education about avoiding high-risk procedures and seeking medical attention for concerning symptoms.
| - Surgical management of rupture, usually with a colostomy and repair in approximately six months.
|
| Uterine rupture | | - Discuss maternal risks associated with pregnancy (approximately 50% have no significant complications and 5% are at risk for death).
- Provide obstetric care in a high-risk tertiary care setting or with an obstetrician who has experience in managing VEDS.
- Consider performing cesarean delivery at 36 to 38 weeks to minimize tissue injury associated with vaginal delivery. Individualized assessment should be used to determine the least stressful mode of delivery.
- Provide genetic counseling regarding recurrence risk.
| - Surgical intervention with delivery of the fetus and repair of the uterus or hysterectomy.
|
| Carotid cavernous sinus fistula | | - Repair by coiling or stenting with catheter intervention.
| |
