# Diagnosis of vascular Ehlers Danlos syndrome and management of vascular complications: a vascular surgeons perspective

## TL;DR

This paper discusses the diagnosis and management of vascular complications in vascular Ehlers-Danlos syndrome from a vascular surgeon's viewpoint.

## Contribution

The paper provides a vascular surgeon's perspective on managing vascular complications in vascular Ehlers-Danlos syndrome.

## Key findings

- Vascular Ehlers-Danlos syndrome is a rare disorder with a high risk of vascular events from a young age.
- Current literature suggests a need for further studies on optimal treatment and follow-up for vascular Ehlers-Danlos syndrome.

## Abstract

The monogenic Ehlers – Danlos syndromes (EDS) constitute a clinically and genetically heterogenous group of connective tissue disorders with overlapping features of generalized joint hypermobility, skin hyperextensibility and tissue fragility. Vascular complications can be observed in several EDS types, but generalized tissue fragility resulting in significant increased risk on vascular events from a young age are a major clinical characteristic of vascular Ehlers – Danlos (vEDS, former Type IV). This is a rare, monogenic EDS type, with a suspected prevalence of 1:50 000. Even though progress regarding awareness and management of vEDS has been made, further studies are needed regarding optimal treatment and follow up. In this manuscript we present the perspective of a vascular surgeon regarding the current literature to management and treatment options for vascular complications in vEDS.