Haemothorax in vascular Ehlers-Danlos syndrome

Vascular Ehlers-Danlos syndrome (EDS IV) is a rare genetic disorder characterised by an alteration in the COL3A1 gene which encodes type III collagen. It is the most common type of collagen in vessels of medium size and certain organs such as the intestines and the uterus. The alteration of this type of collagen produces aneurisms and ruptures of vessels and organs. A high level of clinical suspicion is required for diagnosis. It is a complex...