1.
J Emerg Med
; 14(1): 25-31, 1996.
Article
in English
| MEDLINE
| ID: mdl-8655934
ABSTRACT
The Ehlers-Danlos syndrome (EDS) is a group of hereditary connective tissue disorders that are characterized by abnormalities of the skin, joints, and a diversity of other phenotypic manifestations. An awareness of the disease is vital for optimal outcome in this rare group of patients who may present with a variety of life-threatening illnesses. Ehlers-Danlos type IV has been associated with vascular catastrophes, perforated viscous, ruptured uterus, and pneumothorax (1-4). We present a case of aneurysmal formation and spontaneous rupture of the great vessels in a 15-year-old male with EDS type IV, who remained undiagnosed until the time of autopsy.