Remote vascular catastrophes after neurovascular interventional therapy for type 4 Ehlers-Danlos Syndrome.
Type 4 Ehlers-Danlos Syndrome (EDS 4) is the most malignant form of Ehlers-Danlos Syndrome, often accompanied by neurovasacular complications secondary to vessel dissection or aneurysms. The fragile nature of connective tissue in these patients makes exovascular and endovascular treatment hazardous. We have treated four patients with EDS 4 over the last 8 years by using neuroendovascular procedures. Two of these individuals suffered remote vascular injuries around the time of their procedures and ultimately died. The circumstances surrounding their deaths will make up the body of this report.
Horowitz
M B
MB
Department of Neurosurgery, University of Pittsburgh Medical Center, PA 15213-2582, USA.
Purdy
P D
PD
Valentine
R J
RJ
Morrill
K
K
D000293:Adolescent; D000328:Adult; D000784:Aortic Dissection / Q000000981:diagnostic imaging; D020212:Carotid Artery Injuries / Q000000981:diagnostic imaging; D020216:Carotid-Cavernous Sinus Fistula / Q000000981:diagnostic imaging / Q000628:therapy*; D004535:Ehlers-Danlos Syndrome / Q000000981:diagnostic imaging / Q000628:therapy*; D004621:Embolization, Therapeutic / Q000295:instrumentation*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007083:Iliac Artery / Q000293:injuries / Q000601:surgery; D011859:Radiography; D012086:Reoperation; D012422:Rupture, Spontaneous; D013157:Splenic Artery / Q000293:injuries / Q000601:surgery