Fatal Hemorrhage during Nephrolithotomy in a Patient with Unknown Vascular Ehlers-Danlos Syndrome Type IV

Abstract

Ehlers-Danlos syndrome type IV (vascular EDS), is a life-threatening inherited connective tissue disorder resulting from mutations in the COL3A1 gene coding for type III procollagen. Vascular EDS causes severe fragility of connective tissues. We report a case of a 26-year-old male with bilateral staghorn renal calculi, the patient underwent a left nephrolithotomy, during the suspension of the renal artery incredibly, it was lacerated by the vessel loop, without any actraction. Subsequently, all hemostatic attempts, although gentle, resulted in important and catastrophic aortic and caval injures; the patient died due to an uncontrollable abdominal and thoracic hemorrhage. The early diagnosis of the vascular EDS is difficult if there are no known cases. Every surgical procedure in patients with Enlers-Danlos syndrome has a high risk of fatal vascular injuries.