Styloidogenic-cervical spondylotic internal jugular venous compression, a vascular disease related to several clinical neurological manifestations: diagnosis and treatment—a comprehensive literature review

Abstract

Internal jugular vein (IJV) stenosis is associated with several central nervous system disorders such as Ménière or Alzheimer's disease. The extrinsic compression between the styloid process and the C1 transverse process, is an emerging biomarker related to several clinical manifestations. However, nowadays a limited number of cases are reported, and few information are available about treatment, outcome and complications. Our aim is to collect and identify clinical-radiological characteristics, diagnosis and treatment of the styloidogenic internal jugular venous compression. We performed a comprehensive literature review. Studies reporting patients suffering from extracranial jugular stenosis were searched. For every patient we collected: demography, clinical and radiological characteristics and outcome, type of treatment, complications. Thirteen articles reporting 149 patients were included. Clinical presentation was nonspecific. Most frequent symptoms were headache (46.3%), tinnitus (43.6%), insomnia (39.6%). The stenosis was monolateral in 51 patients (45.9%) and bilateral in 60 (54.1%). Anticoagulants were the most common prescribed drug (57.4%). Endovascular treatment was performed in 50 patients (33.6%), surgery in 55 (36.9%), combined in 28 (18.8%). Improvement of general conditions was reported in 58/80 patients (72.5%).Complications were reported in 23% of cases. Jugular stenosis is a complex and often underestimated disease. Conservative medical treatment usually fails while surgical, endovascular or a combined treatment improves general conditions in more than 70% of patients.

Stylohyoid Eagle syndrome and EXTracranial INternal Carotid arTery pseudoaneurysms (EXTINCT) with internal jugular vein nutcracker syndrome: a challenging clinical scenario

“…In addition, the anomalous stylohyoid complex anatomy can provoke ICA-IJV injury, such as dissection or thrombosis, with neck rotation, stretching, talking, laughing and swallowing. 6–10 Intraoral palpation can identify an elongated styloid process by placing the index finger in the tonsillar fossa and applying gentle pressure. These manoeuvres can reproduce the pain, or the patient can feel it referred to the ear, face or head.…”

“…Failure of conservative treatment commands a surgical or endovascular approach and must be tailored to each case individually to achieve favourable results and reduce possible complications. 6 …”

“…The carotid artery and jugular vein compression by anomalous cervical structures is scarce, and the development of neurovascular complications related to mechanical impingement is an exception 4 6. In 1937, ‘Eagle syndrome’ or ‘stylocarotid artery syndrome’ was reported 2.…”

“…In his first report, Dr Watt Eagle postulated that Eagle syndrome occurs following surgical trauma post-tonsillectomy and local chronic inflammation results in osteitis, periostitis or tendonitis of the styloid process and stylohyoid ligaments. This, in turn, results in reactive, ossified hyperplasia of the ligament 1 6–8. Reactive hyperplasia and reactive metaplasia result in the overgrowth of the styloid process with subsequent ossification.…”

“…There are two distinct types of primary Eagle syndrome: primary stylo-carotid and stylo-jugular syndrome. The former refers to stenosis or internal carotid artery (ICA) dissection, and the latter is due to impingement on the internal jugular vein (IJV) with venous reflux obstruction 1–6. The secondary or classic syndrome mainly occurs post-tonsillectomy with the irritation of the glossopharyngeal nerve by an inflamed styloid process 4…”