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Table 1 Clinical diagnosis of vascular Ehlers–Danlos syndrome is based on the Villefranche criteria [5]

From: Ehlers–Danlos syndrome type IV: a case report of a rare cause of spontaneous sigmoid perforation and enteroatmospheric fistulae in a child

Major criteria

Minor criteria

Arterial, digestive or uterine fragility or rupture

Positive family history or sudden death in a close relative

Thin, translucent skin

Acrogeria

Extensive bruising

Hypermobility of small joints

Characteristic facial appearance

Tendon and muscle rupture

 

Talipes equinovarus

 

Early-onset varicose veins

 

Spontaneous pneumothorax or haemothorax