Unusual clinical course, Challenging differential diagnosis, Management of emergency care, Rare disease
Renato Farina, Pietro Valerio Foti, Isabella Pennisi, Andrea Conti, Gaetano A. Meli, Tiziana Vasile, Cecilia Gozzo, Eliana Tallamona, Corrado Inì, Stefano Palmucci, Massimo Venturini, Antonio Basile
Radiodiagnostic and Radiotherapy Unit, Department of Medical and Surgical Sciences and Advanced Technologies “GF Ingrassia”, University of Catania, Catania, Italy
Am J Case Rep 2021; 22:e932035
Available online: 2021-07-13
Eagle syndrome is a vascular compression syndrome that is caused by a very elongated styloid process and/or calcification of the stylohyoid ligament compressing the vascular or nerve structures of the neck, resulting in vascular congestion, thrombosis, or neurological symptoms (eg, dysphagia, neck pain, ear pain). Stylo-jugular venous compression syndrome is a subtype of Eagle syndrome and is caused by compression of the internal jugular vein. Treatment varies according to the symptoms and the severity of the compression, and it can be pharmacological or surgical, with vascular stenting and/or removal of the styloid process. We describe a rare case of left cerebral venous sinus thrombosis and ipsilateral internal jugular vein stenosis sustained by excessive length of the left styloid process.
CASE REPORT: A 36-year-old woman presented with recurrent episodes of drug-resistant headache and recent memory disturbances. She underwent cerebral and neck multidetector computed tomography-angiography and Doppler ultrasound of the epiaortic vessels that respectively revealed thrombosis of the left cerebral venous sinus and left internal jugular vein stenosis due to a very long styloid process. The patient was treated with anticoagulant drugs and experienced a gradual remission of symptoms.
CONCLUSIONS: Compression of the jugular vein by the styloid process is a rare entity, and it often goes undiagnosed when it is asymptomatic. Doppler ultrasound is a sensitive method for identifying jugular vein stenosis and can provide an estimated degree of stenosis, which is useful for treatment planning. Doppler ultrasound should be combined with multidetector computed tomography-angiography to rule out compression of other vascular structures and other causes of compression. Failure to treat these patients could have serious health consequences for them.
Keywords: Cerebral Veins, Eagle Syndrome, Jugular Veins, multidetector computed tomography, Ultrasonography, Ultrasonography, Doppler