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Ehlers-Danlos syndrome type IV or vascular type is a rare connective-tissue disorder caused by impaired collagen III synthesis. It occurs due to the mutation in the COL3A1 gene, which is inherited or caused sporadically by mutation de novo. Due to collagen III deficit the vascular tissue and tissue of visceral organs is fragile, which leads to numerous life-threatening complications. The most common amongst these are vascular events with dangerous haemorrhages, followed by spontaneous gastrointestinal perforations, ruptures of visceral organs and uterus in pregnancy. The aforementioned complications are in 70% of cases the first manifestation of the disease which is already expressed in young patients. There is no specific therapy, therefore treatment is symptomatic and focused on preventive measures and genetic counselling. Surgical intervention is warranted only in urgent states; non-urgent operations and invasive diagnostic investigations are not advised. Early discovery and monitoring of vascular lesions is important. If lesions imperil patient’s life, we can eliminate them with elective operations before the onset of complications. In the article, we present a case of a 29-year-old patient with known vascular type of Ehlers-Danlos syndrome, whose life was endangered due to an aneurysm rupture of the lienal artery. The origin of the intraabdominal haemorrhage was revealed with CT angiography. The haemorrhage was stopped with a ligature of the lienal artery without splenectomy and the postoperative course passed without complications.