J Korean Neurosurg Soc.  2010 Dec;48(6):528-531. 10.3340/jkns.2010.48.6.528.

Fusiform Aneurysm Presenting with Cervical Radiculopathy in Ehlers-Danlos Syndrome

• Kim HS
• Choi CH
• Lee TH
• Kim SP

• KMID: 2018400
• DOI: http://doi.org/10.3340/jkns.2010.48.6.528

Abstract

Ehlers-Danlos syndrome (EDS) type IV is characterized by its clinical manifestations, which are easy bruising, thin skin with visible veins, and rupture of arteries, uterus, or intestines. Arterial complications are the leading cause of death in vascular EDS because they are unpredictable and surgical repair is difficult due to tissue fragility. The authors report a case presented with cervical radiculopathy due to a segmental fusiform aneurysm of the cervical vertebral artery. Transfemoral cerebral angiography (TFCA) was done to verify the aneurysmal dilatation. However, during TFCA, bleeding at the puncture site was not controlled, skin and underlying muscle was disrupted and profound bleeding occurred during manual compression after femoral catheter removal. Accordingly, surgical repair of the injured femoral artery was performed. At this time it was possible to diagnose it as an EDS with fusiform aneurysm on cervical vertebral artery. Particularly, cervical fusiform aneurysm is rare condition, and therefore, connective tissue disorder must be considered in such cases. If connective tissue disorder is suspected, the authors suggest that a noninvasive imaging modality, such as, high quality computed tomography angiography, be used to evaluate the vascular lesion to avoid potential arterial complications.

Keyword

Ehlers-Danlos syndrome; Cervical radiculopathy; Fusiform aneurysm; Vascular reconstruction

MeSH Terms

Aneurysm
Angiography
Arteries
Catheters
Cause of Death
Cerebral Angiography
Connective Tissue
Dilatation
Ehlers-Danlos Syndrome
Femoral Artery
Hemorrhage
Intestines
Muscles
Punctures
Radiculopathy
Rupture
Skin
Uterus
Veins
Vertebral Artery