Spontaneous Splenic Rupture in Vascular Ehlers-Danlos Syndrome.
Vascular Ehlers-Danlos Syndrome (VEDS) is a rare autosomal dominant collagen vascular disorder. Different from other Ehler-Danlos Syndrome subtypes, VEDS has poor prognosis due to severe fragility of connective tissues and association with life-threatening vascular and gastrointestinal complications. Spontaneous splenic rupture is a rare but hazardous complication related to this syndrome. To date, only 2 cases have been reported in the literature. Here we present another case of this uncommon complication, occurring in a 54-year-old woman in clinical follow-up for VEDS who presented with sudden onset of abdominal pain and hypotension.
Vascular Surgery Department, The Cleveland Clinic, Cleveland, OH, USA nayaracioffi@yahoo.com.
Nayara Cioffi
NC
Batagini
Vascular Medicine Department, The Cleveland Clinic, Cleveland, OH, USA.
Heather
H
Gornik
Vascular Surgery Department, The Cleveland Clinic, Cleveland, OH, USA.
Lee
L
Kirksey
D015746:Abdominal Pain / Q000209:etiology; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000175:diagnosis; D005260:Female; D006801:Humans; D007022:Hypotension / Q000209:etiology; D008875:Middle Aged; D012422:Rupture, Spontaneous; D013156:Splenectomy; D013161:Splenic Rupture / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
connective tissue disease; splenic rupture; vascular ehlers-danlos syndrome