Atypical Presentation of Forearm Compartment Syndrome in a Case of Vascular Type Ehlers-Danlos Syndrome

We describe a 30-year-old Chinese gentleman with vascular type Ehlers-Danlos Syndrome (“vEDS”) presents with spontaneous right forearm compartment syndrome due to pseudoaneurysms along the radial artery. There was no history of injury. Urgent CT Angiogram showed multiple pseudoaneurysms along radial and ulnar arteries. Emergency fasciotomy and reconstruction of radial artery with saphenous vein graft was performed. After subsequent skin grafting, his right forearm wound healed well eventually and his right upper limb function was preserved. Pathological examination of the diseased radial artery showed variable smooth muscle thickness, partial loss of muscle wall, aneurysmal dilatation, fibrinoid necrosis and inflammation. Genetic test showed a heterozygous missense variant c. 1852G>C in COL3A1 gene, which has not been reported and is likely pathogenic of vascular type of Ehlers-Danlos Syndrome. There was no complication or rupture of reconstructed radial artery up to 4 four years’ follow-up. We believe that autologous saphenous vein grafting is a safe, durable option to reconstruct a long segment of arterial defect in this group of patients.