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Open Medicine

formerly Central European Journal of Medicine

Editor-in-Chief: Darzynkiewicz, Zbigniew


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Volume 11, Issue 1

Issues

Volume 10 (2015)

A rare case of persistent hypoglossal artery associated with contralateral proximal subclavian stenosis

Antonio Romeo
  • Corresponding author
  • Department of Medicine and Health Sciences, University of Molise, Campobasso, via A. Mancini, 80127 Naples (Italy)
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/ Giuseppina Napolitano / Giuseppe Leone
  • Unit of Neuroradiology, Department of Advanced Biomedical Sciences, Federico II University of Naples, Italy
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/ Alessandra Aiello
  • Unit of Neuroradiology, Department of Advanced Biomedical Sciences, Federico II University of Naples, Italy
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/ Antonietta La porta / Enrico Tedeschi
  • Unit of Neuroradiology, Department of Advanced Biomedical Sciences, Federico II University of Naples, Italy
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/ Francesco Briganti
  • Unit of Neuroradiology, Department of Advanced Biomedical Sciences, Federico II University of Naples, Italy
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/ Ferdinando Caranci
  • Unit of Neuroradiology, Department of Advanced Biomedical Sciences, Federico II University of Naples, Italy
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Published Online: 2016-08-12 | DOI: https://doi.org/10.1515/med-2016-0050

Abstract

The persistent hypoglossal artery is rare vascular anomalies. We report the case of a 50-year old man with right hypoglossal artery, ipsilateral hypoplasic internal carotid artery, associated with left proximal subclavian stenosis with subclavian steal syndrome. Power-Doppler-Ultra-Sonography spectral images obtained after the patient exercised the left arm showed mid-systolic deceleration with retrograde late-systolic velocities. A Computed Tomography Angiography demonstrated a proximal stenosis of the left SA, a mild right ICA hypoplasia and an anomalous artery arising from right ICA at C2–C3 level, entering the cranium via the hypoglossal canal and joining the basilar artery. Usually the presence of PHA may be completely asymptomatic, and detected as an incidental finding by CTA or MRA, but in our case its diagnosis is extremely important because it is often the only vessel supplying blood to the basilar trunk and posterior circulation.

Keywords: Arteries; Abnormalities; Persistent primitive hypoglossal artery; CT angiography

1 Introduction

Persistent carotid-basilar artery anastomoses are rare vascular anomalies, including the trigeminal, otic, and hypoglossal arteries [1]. The persistent hypoglossal artery (PHA) is the second most common carotid-vertebrobasilar artery anastomosis after trigeminal artery, with a prevalence of 0.02%–0.10% [2]. We report on the case of a 50-year old man with right hypoglossal artery, ipsilateral hypoplasic internal carotid artery (ICA), associated with left proximal subclavian stenosis with subclavian steal syndrome (SSS).

2 Case report

A 50 year-old man was admitted to our Institution reporting dizziness, several episodes of syncope, dysarthria, severe memory problems and blood pressure differential between the arms to investigate a SSS.

The study was conducted at the Department of Radiology at the University Federico II of Naples, according to the principles of the Declaration of Helsinki and approved by the Ethics Committee of the University of Molise. Written informed consent was obtained from subject.

Color-Doppler-Ultra-Sonography (CD-US) spectral images which was obtained after the patient exercised the left arm (by opening and closing the hand for two minutes) showed mid-systolic deceleration with retrograde late-systolic velocities (Figure 1); this CD-US spectrum may resemble the profile image of a rabbit (the “bunny rabbit” sign) [3]. The left subclavian artery (SA) stole blood from the left VA to supply the ischemic arm; the right VA wasn’t demonstrated.

Color-Doppler-Ultra-Sonography spectral images obtained after the patient exercised the left arm (by opening and closing the hand for two minutes): mid-systolic deceleration with retrograde late-systolic velocities.
Figure 1

Color-Doppler-Ultra-Sonography spectral images obtained after the patient exercised the left arm (by opening and closing the hand for two minutes): mid-systolic deceleration with retrograde late-systolic velocities.

A Computed Tomography Angiography (CTA) with multiplanar (MPR) and Volume-Rendering (VR) reconstructions demonstrated a proximal stenosis of the left SA (Figure 2), a mild right ICA hypoplasia and an anomalous artery arising from right ICA at C2–C3 level, entering the cranium via the hypoglossal canal and joining the basilar artery (BA) (Figure 3, 4). The right hypoglossal canal was larger than the contralateral. There were no findings suggesting bony destruction of the hypoglossal canal.

Computed Tomography Angiography (MPR reconstruction): proximal stenosis of the left subclavian artery (arrow).
Figure 2

Computed Tomography Angiography (MPR reconstruction): proximal stenosis of the left subclavian artery (arrow).

Computed Tomography Angiography (MPR reconstruction): anomalous artery arising from the right internal carotid artery at C2–C3 level, entering the cranium via the hypoglossal canal.
Figure 3

Computed Tomography Angiography (MPR reconstruction): anomalous artery arising from the right internal carotid artery at C2–C3 level, entering the cranium via the hypoglossal canal.

Computed Tomography Angiography (VR reconstruction): the anomalous artery arising from the right internal carotid artery at C2–C3 level joins the basilar artery.
Figure 4

Computed Tomography Angiography (VR reconstruction): the anomalous artery arising from the right internal carotid artery at C2–C3 level joins the basilar artery.

The ipsilateral (right) vertebral artery was completely absent, while the contralateral left vertebral artery appeared regular. Due to the left SSS, the basilar artery was only refurnished by the right PHA.

3 Discussion

PHA originates from the internal carotid artery at the level of C1-C3 vertebral bodies, courses through the hypoglossal canal, and anastomoses with the basilar artery. Definitive diagnosis is based on the recognition of an anomalous artery in the enlarged hypoglossal canal [1, 2].

According to Padget [4], during the early stage of embryological development the carotid system and two parallel longitudinal neural arteries supply the forebrain and the hindbrain, respectively. Four primitive anastomoses (named from the cranial nerves with which they course) occur between the carotid and vertebrobasilar systems: the trigeminal artery, the otic artery, the hypoglossal artery and the proatlantal artery. Normally, these anastomoses remain functional for approximately 7–10 days during the early stage of fetal development and then obliterate at the rate at which the posterior communicating arteries and vertebral arteries develop. Failure of this obliteration results in the persistence of embryonic arteries, which leads to hypoplasia of the vertebrobasilar system [5].

Brismer [6] defined three essential diagnostic criteria in describing the PHA: the origin from ICA as an extracranial branch, the passage through the hypoglossal canal, the joining through the caudal part of the basilar artery. However, unusual and extremely rare variants of PHA have been reported, such as those arising from the external carotid artery (ECA) [7] or ending in the posterior inferior cerebellar artery (PICA) without an interposed segment of the basilar artery [8].

Uchino et al. [9] in a recent review proposed a new classification, naming “type 1” the usual PHA arising from the cervical ICA, “type 2” the PHA arising from the ECA, and “PHA variant” when the postero-inferior cerebellar artery (PICA) arises from the carotid system without connection to the VA.

The PHA is usually an incidental finding but its diagnosis is extremely important because it is often the only vessel supplying blood to the basilar trunk and posterior circulation; moreover, it could be associated to glossopharyngeal neuralgia [10], hypoglossal palsy [11], intracranial aneurysms [12]. In particular, a relationship between PHA and intracranial aneurysms has been reported, up to 26-33% in different series [9, 12].

In our case, all Brismer [6] diagnostic criteria were adhered to: the right VA was absent and the right ICA was hypoplastic. As demonstrated in large retrospective series [9], these findings suggest that the absence of the VA ipsilateral to PHA may result in the persistence of PHA. Left VA, as demonstrated by CD-US, showed retrograde flow due to proximal subclavian stenosis.

The left VA was functionally absent because of its retrograde flow and the vertebrobasilar system was only refurnished by the PHA. To the best of our knowledge, a similar anatomic and hemodynamic situation in a “type I” PHA (according to Uchino et al. classification [9]) has never been reported. Moreover, the anatomic and functional variant described in our patient is clinically important because both the anterior and posterior cerebral circulation are dependent on the arterial supply of the ICA, and the risk of bilateral occipital ischemia caused by embolism from the ICA to posterior cerebral arteries (PCA) through PHA is increased [13].

This suggests that it is important to remember that surgical and endovascular procedures in the ICA must be performed carefully in patients with PHA. This is because ICA cross-clamping during thrombo-endoarterectomy or its temporary occlusion during endovascular occlusion test [13-18] may significantly lower cerebral and brainstem perfusion.

4 Conclusion

The presence of PHA may be completely asymptomatic, and may be detected as an incidental finding by CTA or MRA. The recognition of PHA is essential to determine the therapeutic management of patients with atherosclerosis of the supraaortic vessels and vertebrobasilar ischemia.

References

  • [1]

    Dimmick SJ, Faulder KC. Normal variants of the cerebral circulation at multidetector CT angiography. Radiographics. 2009; 29:1027-1043 Google Scholar

  • [2]

    Hahnel S, Hartmann M, Jansen O, Sartor K. Persistent hypoglossal artery: MRI, MRA and digital subtraction angiometry. Neuroradiology 2001; 43: 767-769 Google Scholar

  • [3]

    Kliewer MA, Hertzberg BS, Kim DH, et al. Vertebral artery Doppler waveform changes indicating subclavian steal physiology. AJR Am J Roentgenol 2000; 174(3):815-819 Google Scholar

  • [4]

    Padget DH. The development of the cranial arteries in the human embryo. Cont Embryol 1948;32:205-261 Google Scholar

  • [5]

    Caranci F, Napoli M, Cirillo M et al. Basilar artery hypoplasia. Neuroradiol J. 2012 20;25:739-743 Google Scholar

  • [6]

    Brismer J. Persistent hypoglossal artery, diagnostic criteria. Acta Radiol 1976;17:160-166 Google Scholar

  • [7]

    Sabouri S, Ebrahimzadeh SA, Rahimian N. Unusual Variant of Persistent Primitive Hypoglossal Artery Diagnosed by CT Angiography: A Case Report and Literature Review. Clin Neuroradiol 2014; 24:59-63 Google Scholar

  • [8]

    Andoh K, Tanohata K Moriya N, et al. Posterior inferior cerebellar artery arising from the extracranial segment of the internal carotid artery via the hypoglossal canal without an interposed segment of the basilar artery: a persistent primitive hypoglossal artery variant. Clin Imaging. 2001; 25:86-89 Google Scholar

  • [9]

    Uchino A, Saito N, Okada Y et al. Persistent hypoglossal artery and its variants diagnosed by CT and MR angiography. Neuroradiology 2013; 55:17-23 Google Scholar

  • [10]

    Kempe LG, Smith DR. Trigeminal neuralgia, facialspasm, intermedius and glossopharyngeal neuralgia with persistent carotid basilar anastomosis. J Neurosurg 1969; 31:445-451 Google Scholar

  • [11]

    Meila D, Wetter A, Brassel F, Nacimiento W. Intermittent hypoglossal nerve palsy caused by a calcified persistent hypoglossal artery: an uncommon neurovascular compression syndrome. J Neurol Sci. 2012; 323:248-249 Google Scholar

  • [12]

    Teo M, Bhattacharya J, Suttner N. Persistent hypoglossal artery-an increased risk for intracranial aneurysms? Br J Neurosurg 2012 26:891-892 Google Scholar

  • [13]

    Conforto AB, De Souza M, Puglia P JR. Bilateral occipital infarcts associated with carotid atherosclerosis and a persistent hypoglossal artery. Clin Neurol Neurosurg 2007;109:364-367 Google Scholar

  • [14]

    Briganti F, Tedeschi E, Leone G, Marseglia M, Cicala D, Giamundo M, Napoli M, Caranci F. Endovascular treatment of vertebro-vertebral arteriovenous fistulae: a report of three cases and literature review. Neuroradiol J 2013;26:339-346Google Scholar

  • [15]

    De Bernardi IC, Floridi C, Muollo A, Giacchero R, Dionigi GL, Reginelli A, et al. Vascular and interventional radiology radiofrequency ablation of benign thyroid nodules and recurrent thyroid cancers: literature review. Radiol Med. 2014 Jul;119(7):512-520Google Scholar

  • [16]

    Amato, B., Bianco, T., Compagna, R., Serra, R., De Franciscis, S.: Surgical resection of carotid body paragangliomas: 10 years of experience. American Journal of Surgery, 2014, 207 (2), pp. 293-298 CrossrefGoogle Scholar

  • [17]

    Compagna, R., Vigliotti, G., Rispoli, C., Sivero, L., Amato, B.: Carotid screening with Duplex ultrasound in elderly asymptomatic patients candidate to general surgery. Chirurgia (Turin), 2013, 26 (4), pp. 291-294Google Scholar

  • [18]

    Amato, B., Markabaoui, A.K., Piscitelli, V., Masone, S., Persico, G.: Carotid endarterectomy under local anesthesia in elderly: Is it worthwhile? Acta Biomedica de l’Ateneo Parmense, 2005, 76 (SUPPL. 1), pp. 64-68 Google Scholar

About the article

Received: 2016-05-14

Accepted: 2016-05-23

Published Online: 2016-08-12

Published in Print: 2016-01-01


Conflict of interest

Authors state no conflict of interest.


Citation Information: Open Medicine, Volume 11, Issue 1, Pages 252–255, ISSN (Online) 2391-5463, DOI: https://doi.org/10.1515/med-2016-0050.

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© 2016 Antonio Romeo et al.. This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License. BY-NC-ND 3.0

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