EDS

Spontaneous ruptured dissection of the right common iliac artery in a patient with classic Ehlers-Danlos syndrome phenotype.

This case study explores a rare instance of a spontaneous ruptured dissection of the right common iliac artery in a 39-year-old man with a classic Ehlers-Danlos syndrome (EDS) phenotype. Typically, such vascular manifestations are more associated with vascular EDS, not classic EDS. Upon hospital admission, the patient was in hypovolemic shock, and a CT angiogram revealed the dissection with rupture. An endovascular repair attempt transitioned into an open repair surgery due to the rupture. Genetic analysis identified a COL1A1 genetic mutation, characterizing a rare variant of classic EDS. This case underscores the importance of recognizing potential serious vascular complications in classic EDS variants, influencing diagnostic and management strategies.