title: Spontaneous Dissection of the Renal Artery in Vascular Ehlers-Danlos Syndrome.
abstract: Ehlers-Danlos syndrome (EDS) is a rare heterogeneous group of connective tissue disorders. The vascular type (vEDS) is an autosomal dominant disorder caused by heterozygous mutations in the COL3A1 gene predisposing to premature arterial, intestinal, or uterine rupture. We report a case of a 38-year-old woman with a recent diagnosis of vEDS admitted in the Emergency Department with a suspicion of a pyelonephritis that evolved to a cardiopulmonary arrest. A fatal retroperitoneal hematoma related with a haemorrhagic dissection of the right renal artery was found after emergency surgery. This case highlights the need to be aware of the particular characteristics of vEDS, such as a severe vascular complication that can lead to a fatal outcome.
authors.affiliation: Serviço de Anestesiologia, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, Portugal.
authors.forename: Filipa
authors.initials: F
authors.lastname: Pereira
authors.affiliation: Unidade de Cuidados Intensivos Polivalente, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, Portugal.
authors.forename: Teresa
authors.initials: T
authors.lastname: Cardoso
authors.affiliation: Serviço de Anestesiologia, Centro Hospitalar do Porto, Largo Professor Abel Salazar, 4099-001 Porto, Portugal.
authors.forename: Paula
authors.initials: P
authors.lastname: Sá