Abstract
BackgroundPatients with vascular Ehlers-Danlos syndrome (EDS) occasionally suffer from arterial dissection. Eagle syndrome, which is caused by an elongated styloid process and also causes arterial dissection, is difficult to diagnose and could sometimes be overlooked. Little is known of the coexistence of these two diseases, and treatment strategy is not established. Here, we present a case of bilateral internal carotid artery (ICA) dissection due to Eagle syndrome in a patient with vascular EDS.Case presentationA 30-year-old man was admitted to our hospital because of sudden onset of mild sensory disturbance in his left limbs. He had a history of Ehlers-Danlos syndrome (EDS) and also had left cervical internal carotid artery (ICA) dissection 3 years before. Diffusion-weighted imaging showed acute cerebral infarcts in the right hemisphere. Cervical computed tomography angiography (CTA) revealed the right ICA narrowing at the cervical portion in addition to the previous left cervical ICA dissection. Cervical magnetic resonance imaging (MRI) revealed double-lumen and intramural hematoma at the narrowing portion of the right cervical ICA, which indicates arterial dissection. CT also revealed bilateral elongated styloid processes which are close to each side of cervical ICA. We diagnosed him as bilateral ICA dissection due to bilateral Eagle syndrome. Considering vascular complications due to vascular EDS, we performed closer follow-up with transoral carotid ultrasonography (TOCU). In 4 months, his right ICA dissection gradually improved without stroke recurrence or deterioration of dissection.ConclusionsSince patients with vascular EDS easily develop arterial dissection, Eagle syndrome may be overlooked. Clinicians should consider Eagle syndrome in the case of vascular EDS with extracranial ICA dissection and close follow-up should be prioritized in cases of Eagle syndrome with vascular EDS.
Highlights
Patients with vascular Ehlers-Danlos syndrome (EDS) occasionally suffer from arterial dissection
Clinicians should consider Eagle syndrome in the case of vascular EDS with extracranial internal carotid artery (ICA) dissection and close follow-up should be prioritized in cases of Eagle syndrome with vascular EDS
He had histories of sigmoid colon rupture and pneumothorax and underwent surgical therapies. He was admitted with transient amaurosis due to left ICA dissection, and a gene mutation analysis revealed the presence of vascular EDS with a missense mutation of COL3A1 cDNA
Summary
Since patients with vascular EDS develop arterial dissection, Eagle syndrome may be overlooked.
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