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


We report a 4-year delay in diagnosing a combined carotid arterial and jugular venous styloid compression. The symptoms, which included dull neck pain, dizziness, intermittent diplopia, tinnitus, severe incapacitating right side headache and eye bloating, were challenging and wrongly attributed initially to various facial neuralgias. The patient presented during COVID-19 pandemic and was labelled as ‘carotidynia’ first and later as a transient perivascular inflammation of carotid artery syndrome. Combined targeted duplex ultrasonography and CT angiography with 3D reconstruction revealed a long styloid process and its tendinous-ligamentous attachments, injuring the internal carotid artery. Moreover, there was substantial internal jugular vein compression on a long C1 transverse process with a nutcracker syndrome. Release of the tendinous portion of the long styloid process and repair of the carotid artery pseudoaneurysm ended the patient’s complaints and allowed him to have a better quality of life.


What a great article! Thanks for always finding all these great informational articles and sharing it with us!!!


@Ddmarie thanks I try to get the most recent ones. All the ones I posted today are from 2022.


What is also interesting in this particular study is, they DID not remove the styloid process. They lifted it and cut the ligaments to release it and make a room for the ICA & Jugular Vein to expand. I wonder if there is risk in the future that the Angle of the Styloid might change again to point downward towards the Jugular or Carotid branches since Styloid was not removed. I guess time will tell.

An open cervical neck approach was adopted for our patient. Following a cervical neck incision along the sternocleidomastoid muscle’s anterior border, the styloid process’s distal end was lifted, and its proximity to the hypoglossal, glossopharyngeal, vagus and chorda tympani of the facial nerve was observed. The ligamentous and/or tendinous structure strapped to the proximal ICA was cut. The styloid process’s pointing and angle had sprung away to enlarge the space for both the artery and the vein (figure 2). Once the styloid process recoiled out of the surgical field, the whole space was freed to perform an end-to-end ICA anastomosis (figure 3). The IJV expanded, and its physiological response was restored with the visualisation of the ‘A’ wave and oscillation.


Good article. First time I ever saw the phrase “eye bloating” used. I like it!

I also find it interesting that the patients symptoms got better without angioplasty given the age of the patient and presumably long duration of stenosis of the IJV.

The one thing all these studies have in common is that they all say not enough is known about this condition…


Thanks again @KoolDude, very interesting article!

@Buzz You might want to take a look at above Study. It contains nice images of the different configurations of Styloid/Stylohyoid ligament and how it interacts with cranial nerves and blood vessels.

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Figure 5

Schematic diagram depicting primary styloid-carotid-jugular syndrome: (A) Venous nutcracker internal jugular syndrome is due to compression of the internal jugular vein (IJV) between the elongated transverse process of the right C1 and proper styloid process and its ossified mineralised ligamentous-tendinous attachments. (B) The long right styloid process strapping the two loops of the right ICA with the formation of a pseudoaneurysm of mid-ICA. The mineralised ossified styloid ligament is strapping the looped ICA. Notice the proximity of the ligamentous-tendinous part of the right styloid process to the pseudoaneurysm. (C) Combined carotid arterial and jugular venous styloid compression due to the tendinous-ligamentous of the long styloid process injuring the ICA causing a pseudoaneurysm, with IJV compression due to a long C1 transverse process that caused a nutcracker with the styloid process on IJV. The elongated styloid process instigates continuous prickliness of cranial nerves, most commonly the glossopharyngeal nerve, hypoglossal nerve, trigeminal, facial and vagus cranial nerves. (Authors declare that these figures are original and drawn by them.)


@KoolDude Fascinating study, thank you.

All of this is just making me more excited for the surgery. I can’t being to imagine how different I’m going to feel, and what symptoms will reduce. I can only hope that there isn’t too much permanent damage.