Bilateral carotid artery dissection due to Eagle syndrome in a patient with vascular Ehlers-Danlos syndrome: a case report

Abstract

Background Patients 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 presentation A 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. Conclusions Since 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.

Bilateral carotid dissection due to Eagle syndrome in a young female

“…Eagle syndrome may cause carotid artery disease, including vessel stenosis, artery dissection, or pseudoaneurysm, and may be complicated by cerebrovascular events [ 3 ]. Bilateral carotid dissection from ES is rare, and only a few cases are reported in the available literature ( Supplementary Table 1 ) [ [3] , [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] ]. As previously reported, there exists a strong male predominance on carotid dissection cases secondary to ES [ 3 ].…”

“…Previous case series have documented high rates of recurrent infarcts in patients with ICA dissection secondary to ES [ 9 ]. We identified 12 cases of bilateral carotid dissection secondary to ES [ [3] , [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] ]. Of the cases identified, 7 documented an acute ischemic strokes on admission [ 4 , 5 , [7] , [8] , [9] , [10] , 12 ].…”

“…We identified 12 cases of bilateral carotid dissection secondary to ES [ [3] , [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] ]. Of the cases identified, 7 documented an acute ischemic strokes on admission [ 4 , 5 , [7] , [8] , [9] , [10] , 12 ]. Nine cases were treated medically or with thrombectomy on arrival, and five of them ended up having new or recurrent cerebrovascular events [ [3] , [4] , [5] , 7 , 8 , [10] , [11] , [12] ].…”

“…Of the cases identified, 7 documented an acute ischemic strokes on admission [ 4 , 5 , [7] , [8] , [9] , [10] , 12 ]. Nine cases were treated medically or with thrombectomy on arrival, and five of them ended up having new or recurrent cerebrovascular events [ [3] , [4] , [5] , 7 , 8 , [10] , [11] , [12] ]. Only two cases were identified where acute carotid artery stenting was performed, including our case [ 9 ].…”

Recurrence of internal carotid artery dissection associated with elongated styloid process: A case report

“…[ 2 , 3 , 11 , 14 ] ICA dissection due to the elongated styloid process was determined to have higher recurrence risk than that of idiopathic ICA dissection. [ 7 , 18 ] Conversely, the possibility of elongated styloid process when ischemic event recurs should be considered. Moreover, some cases of ischemic event recurrence have led us to identify the underlying etiology of elongated styloid process.…”