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Is Eagle Jugular Syndrome an Underestimated Potentially Life-Threatening Disease?

Published online by Cambridge University Press:  04 March 2020

Enrico Nastro Siniscalchi
Affiliation:
Division of Maxillofacial Surgery, BIOMORF Department, University of Messina, Messina, Italy
Giovanni Raffa*
Affiliation:
Division of Neurosurgery, BIOMORF Department, University of Messina, Messina, Italy
Antonino Germanò
Affiliation:
Division of Neurosurgery, BIOMORF Department, University of Messina, Messina, Italy
Francesco Saverio De Ponte
Affiliation:
Division of Maxillofacial Surgery, BIOMORF Department, University of Messina, Messina, Italy
*
Correspondence to: Giovanni Raffa, Division of Neurosurgery, BIOMORF Department, University of Messina, Via Consolare Valeria 1, 98125, Messina, Italy. Email: giovanni.raffa@unime.it
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Abstract

Type
Letter to the Editor
Copyright
Copyright © 2020 The Canadian Journal of Neurological Sciences Inc.

We have read with interest the article titled “Eagle Syndrome as a Cause of Cerebral Venous Sinus Thrombosis” by Fu-Liang Zhang et al. published in the May 2019 issue of The Canadian Journal of Neurological Sciences.Reference Zhang, Zhou, Guo and Yang1

The authors describe a case of Eagle syndrome in a 15-year-old teenager with a 2-month history of headache that was caused by left transverse-sigmoid sinus thrombosis due to compression of the left jugular vein by an elongated styloid process (SP). This is quite unusual, since the classic Eagle syndrome is primarily caused by compression of the internal carotid artery, resulting in a wide spectrum of clinical and neuroradiological findings from cervicofacial pain to cerebral ischemia.Reference Chung, Chao, Hsu, Lin and Hu2,Reference Galletta, Granata and Longo3

The paper by Fu-Liang Zhang deserves merit since it describes an unusual effect of the presence of elongated SPs, i.e., compression of the jugular vein, leading to some interesting considerations regarding what has been defined as “Eagle jugular syndrome.”Reference Galletta, Siniscalchi, Ciccio, Velo and Granata4 It is well known that prolonged venous compression can lead to the development of deep venous thrombosis (DVT). DVT is much more frequent in the lower limbs, but it may occur in any section of the venous system, including arms, abdominal, and also the jugular veins.Reference Illig and Doyle5 For instance, thrombosis of the subclavian vein at the costoclavicular junctions, also known as Paget-Schroetter syndrome, is caused by intermittent venous compression due to muscular stretch in the thoracic outlet and may potentially result in pulmonary embolism initially classified as of unknown origin.Reference Martinelli6

Similarly, it has already been demonstrated that compression of the jugular vein caused by an elongated SP may result in jugular thrombosis, thus configuring the case of “Eagle jugular syndrome.” In such a scenario, clinical manifestations may vary from migraine to cerebral venous thrombosis.Reference Galletta, Siniscalchi, Ciccio, Velo and Granata4,Reference Porfidia, Porceddu and Feliciani7

In light of such evidence, it is reasonable to hypothesize that jugular vein thrombosis may also lead to pulmonary embolism. It has been reported that in approximately 20% of cases of pulmonary embolism, the source of emboli cannot be identified, representing the so-called “isolated pulmonary embolism,” which is frequently caused by unusual forms of venous thrombosis and thrombophilia.Reference Siniscalchi8 It could be speculated that jugular vein thrombosis in Eagle syndrome may be responsible for some of these cases of pulmonary embolism, given that this eventuality has been poorly investigated.

Our suggestion is to include also the investigation of jugular vein compression due to elongated SPs in cases of pulmonary embolism of unknown origin. As previously reported, we strongly support the necessity of a dynamic evaluation of compression of neck structures due to elongated SPs in Eagle syndrome.Reference Zamboni, Scerrati and Menegatti9 The diagnostic accuracy of computed tomography angiography (CTA)/magnetic resonance angiography (MRA) can be increased through a “dynamic” evaluation of the spatial relationship between SPs and neck vascular structures simply performing acquisitions using different orientations of the head. This could better identify the compression that occurs only with a specific head orientation (e.g. rotation) but not in a “neutral” head position.

In conclusion, we think that, amongst others, Eagle syndrome may result in compression and thrombosis of the jugular vein, thus causing life-threatening clinical manifestations, including cerebral venous thrombosis, as reported by Fu-Liang Zhang et al.,Reference Zhang, Zhou, Guo and Yang1 along with isolated, recurrent, pulmonary embolism events, the origin of which may be misdiagnosed. The dynamic exploration of the spatial relationship between elongated SPs and jugular veins though CTA and/or MRA may lead to a prompt diagnosis of this eventuality and to an effective treatment of these patients. Further investigations are needed to confirm or deny our hypothesis.

Disclosures

The authors have no conflicts of interest to declare.

Statement of Authorship

ENS conception of the work; drafting the work and revising it critically for important intellectual content; final approval of the version to be published; agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. GR conception of the work; drafting the work and revising it critically for important intellectual content; final approval of the version to be published; agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. AG conception of the work; final approval of the version to be published; agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. FSDP conception of the work; final approval of the version to be published; agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Footnotes

*

The authors equally contributed as first authors.

References

Zhang, F-L, Zhou, H-W, Guo, Z-N, Yang, Y. Eagle syndrome as a cause of cerebral venous sinus thrombosis. Can J Neurol Sci. 2019;46(3):344–5.CrossRefGoogle ScholarPubMed
Chung, C-P, Chao, AC, Hsu, H-Y, Lin, S-J, Hu, H-H. Decreased jugular venous distensibility in migraine. Ultrasound Med Biol. 2010;36:11–6.CrossRefGoogle ScholarPubMed
Galletta, K, Granata, F, Longo, M, et al.An unusual internal carotid artery compression as a possible cause of Eagle syndrome – a novel hypothesis and an innovative surgical technique. Surg Neurol Int. 2019;10:174.CrossRefGoogle Scholar
Galletta, K, Siniscalchi, EN, Ciccio, M, Velo, M, Granata, F. Eagle syndrome: a wide spectrum of clinical and neuroradiological findings from cervico-facial pain to cerebral ischemia. J Craniofac Sur. 2019;30:e4248.CrossRefGoogle ScholarPubMed
Illig, KA, Doyle, AJ. A comprehensive review of Paget-Schroetter syndrome. J Vasc Sur. 2010;51:1538–47.CrossRefGoogle ScholarPubMed
Martinelli, I. Unusual forms of venous thrombosis and thrombophilia. Pathophysiol Haemost Thromb. 2002;32:343–5.CrossRefGoogle ScholarPubMed
Porfidia, A, Porceddu, E, Feliciani, D, et al.Differences in clinical presentation, rate of pulmonary embolism, and risk factors among patients with deep vein thrombosis in unusual sites. Clinic Appl Thromb Hemost. 2019;25:1076029619872550.Google ScholarPubMed
Siniscalchi, EN. Dynamic imaging in suspected Eagle syndrome. Eur Arch OtorhinoLaryngol. 2020;277:307.CrossRefGoogle ScholarPubMed
Zamboni, P, Scerrati, A, Menegatti, E, et al.The eagle jugular syndrome. BMC Neurol. 2019;19:333.CrossRefGoogle ScholarPubMed
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