Clinical Presentation & Protocol
Patient Usually Complains Of
Patient presents for evaluation of suspected Vascular Ehlers-Danlos Syndrome (vEDS). History significant for [spontaneous arterial rupture/dissection/organ perforation/recurrent pneumothorax]. Family history positive for [sudden death/vascular events]. Patient reports [easy bruising/acrogeria/thin, translucent skin]. No history of [joint hypermobility/severe scoliosis].
Clinical Examination Findings
General: Patient appears [chronically ill/well-developed]. Skin: Thin, translucent, visible subcutaneous venous pattern, particularly over chest and abdomen. Minimal subcutaneous fat. Scars: Atrophic, "cigarette-paper" appearance. Face: Characteristic features noted (thin vermilion of lips, micrognathia, narrow nose). Cardiovascular: [Normal S1/S2/murmurs absent/bruits present]. Extremities: Acrogeria noted; no significant joint hypermobility beyond digits.
Treatment Protocol
Management plan: 1. Strict blood pressure control (target <120/80 mmHg) using beta-blockers (e.g., Celiprolol). 2. Avoidance of elective surgery and invasive procedures unless life-threatening. 3. Avoidance of contact sports and heavy lifting. 4. Annual vascular surveillance via MRA/CTA of head, neck, chest, and abdomen. 5. Medic-Alert bracelet required. 6. Genetic counseling referral.