- Case Report
- Open access
- Published:
Persistent trigeminal artery in a patient with moyamoya disease:a case report and literature review
BMC Neurology volume 24, Article number: 54 (2024)
Abstract
Backgrounds
Persistent trigeminal artery (PTA) is a rare anastomosis between internal carotid artery (ICA) and basilar artery. In rare conditions, the PTA could be combined with others cerebrovascular anomalies, moyamoya disease (MMD) is one of them.
Case presentation
Here, we reported one rare case of MMD associated with PTA, the patient admitted to our department for severe dizziness and headache, imaging examination suggested MMD combined with right PTA, which arising from the ipsilateral cavernous portion of ICA. The patient received phased bilaterral revascularization with no any complication. In the subsequent follow-up, the patient’s symptoms and intracranial vascular condition gradually improved. Moreover, we conducted a literature review of coexistence of PTA and MMD, the results of a web of science regarding such condition, and a deep discussion providing brief insight into the status of co-occurrence of PTA and MMD, including its manifestation, treatment and outcome.
Conclusions
The coexistence of PTA and MMD was rarely reported, the pathogenesis of such condition remains unknown. We found that the features of the coexistence of PTA and MMD were diverse, revascularization might be a feasible for such patient.
Introduction
The persistent trigeminal artery (PTA) is the most common but rarely reported carotid-basilar anastomosis. Previous studies demonstrated that the incidence of PTA ranged from 0.1-0.6% [1], and the reports of PTA mainly focused on limited sample and case reports. The presence of PTA could combine with many other diseases, such as aneurysm [2,3,4], arteriovenous malformation [5, 6], and trigeminal neuralgia [7,8,9]. Moreover, moyamoya disease (MMD) is a rare disease characterized by occlusion and abnormal proliferation of the cerebral arteries and the formation of moyamoya vessels, which could ultimately lead to the occurrence of cerebral infarction or hemorrhage [10]. The revascularization, which could greatly reduce the incidence of hemorrhage and cerebral infarction, has been proved to be a safe and effective choice for patient with MMD after extensive clinical studies and practices. In very condition, the MMD could combine with PTA, no matter whether this artery is affected [2, 11]. To our knowledge, only 13 cases of MMD associated with PTA were reported in the past decades (including current case).
Case presentation
The case involved a 44-year-old woman who admitted to our department for further treatment. She experienced sudden, severe dizziness and headache three months ago without any identifiable cause. The head computed tomography scan performed at local hospital revealed infarction in the left temporal and basal ganglia, as well as severe stenosis of both internal carotid arteries (ICAs). She was initially treated symptomatically and gradually improved. Three months later, she scheduled for further treatment in our department. The computed tomography perfusion (CTP) imaging showed old infarction and partial liquefaction in the left frontal and temporal lobe. Additionally, the computed tomography angiography (CTA) and digital subtraction angiography (DSA) revealed a PTA arising from cavernous portion of the right ICA, and stenosis or occlusion in the bilateral anterior cerebral arteries (ACA), the M1 segment of bilateral middle cerebral arteries (MCAs), and the C5-C6 segment of bilateral ICAs, combining with many net-like vessels, but the anterior and posterior communicating arteries were patent (Fig. 1). Following thorough preoperative examinations, it was determined that bilateral revascularizations were needed. Considering the characteristics of the disease onset, the left revascularization was performed first. During the surgery, however, we found that all of the potential recipient vessels had a diameter smaller than 0.5 mm, while the superficial temporal artery had a diameter greater than 1.5 mm, a direct bypass would pose a high risk of postoperative hemorrhage. Therefore, an indirect bypass procedure was performed on the left side, the patient recovered well and was discharged within a week. During the follow-up visit three months later, imaging examination revealed severe stenosis of the arteries. However, there were improvements in the intracranial vascular conditions, and collateral vessels had formed on the left side. For similar reason mentioned above, an indirect bypass on the right side was performed during this hospitalization. The patient experienced a smooth recovery and symptom improvement. The most recent CTA, CTP tests and DSA of the head indicated significant improvement in both intracranial and extracranial collateral vessels. However, there was still relatively low cerebral blood flow perfusion in the left frontal and temporal lobe (Fig. 2).
Discussion
PTA, otic artery, hypoglossal artery, and proatlantic intersegmental artery are the four persistent embryologic carotid-basilar anastomoses, and PTA is the most common with a very low reported incidence [1]. It was first described in a specimen in 1841 [12] and demonstrated in a live subject in 1950 [13]. Based on its radiological and hemodynamic characteristics, Saltzman classified PTA into three types [14]. Type 1 is the most common, the PTA supplies the bilateral superior cerebellar arteries (SCAs) and the bilateral posterior cerebral arteries. In type 2, the PTA supplies bilateral SCAs. Type 3 represents a variant of PTA, which can be further subdivided into three subtypes: type 3a, where PTA directly drains into the ipsilateral SCA; type 3b, where PTA directly drains into the ipsilateral anterior inferior cerebellar artery (AICA); and type 3c, where PTA directly drains into the ipsilateral posterior inferior cerebellar artery (PICA). Salas classified PTA into lateral and medial types based on the anatomical relationship between PTA and the abducent nerve [15]. Weon further expanded the classification into five types [16]. Types 1 and 2 correspond to Saltzman’s classification, while in types 3 and 4, the PTA drains into the contralateral and ipsilateral posterior cerebral arteries, respectively. Type 5 can be further divided into subtypes based on its terminal arteries: type 5a (SCA), type 5b (AICA), and type 5c (PICA). (Fig. 3). It was reported that the type 1 was the most common [17].
The PTA draws attention for it’s usually reported to be associated with central nervous system disease, such as aneurysm [2,3,4], arteriovenous malformation [5, 6], MMD [2, 11], and trigeminal neuralgia [7,8,9]. It’s reported that the rough incidence of MMD with PTA is 0.0021% [11]. After carefully retrieval in web of science, we found that there were only 13 cases of patients with the coexistence of PTA with MMD (including current case). (Table 1) For the vascular features of MMD and PTA, the PTA could be the collateral vessel of MMD and protect the patients from complications, such as ischemia and hemorrhage (Fig. 4), but also prompt the stage of MMD [18]. Such patients mostly present with ischemia, with a small part with hemorrhage, and no age, gender, or lateralization patterns difference. Patients who underwent revascularization procedures generally achieved favorable outcomes. As the PTA coexistence with MMD is rarely reported, the incidence, clinical feature, and outcome of such condition remain further study.
In addition to PTA, the MMD could be combined with other anomalies, such as arteriovenous malformation [19,20,21], carotid artery absence [22,23,24], ICA hyperplasia [25] and so on. It is more likely that the increased blood to the arteriovenous malformation and increased blood turbulence lead to intimal hyperplasia and progressive occlusion [26]. The anastomotic vessels and their capillaries could open up and become distended in the presence of ischemia, these capillaries could only discharge the increased blood into normal veins which in tum become distended [19]. As the variant of PTA is usually poorly visualized, more attentions should be paid to such patient [25]. In patients with carotid artery abnormality, the presence of PTA could be vital collateral circulation [22, 23]. There is no currently direct etiological evidence show the relationships between MMD and these diseases, but it can be speculated that congenital factors may play an important role [22], as the timeframe in which the PTA regresses during embryonic development coincides with the period in which the vascular condition bears resemblance [27]. Uchino reported the higher incidence of PTA in patient also support this hypothesis [28], but the potential mechanism remains unknown [29]. As the imaging characteristics of MMD, the PTA could be the solely blood supply to the affected brain region [18]. Moreover, this artery could even change the haemodynamics of patients with MMD, and be used as a route for endovascular coiling for aneurysm [2]. Also, the PTA could play a protective role in cases of acquired occlusion of the ipsilateral ICA [30, 31], it could provide compensatory blood to the targeted area. In currtent case, the proximal basilar artery and bilateral vertebral arteries were very thin, the PTA could supply blood to the posterior circulation, especially in patient with MMD, whose cerebral arteries were badly affected. However, further studies are still needed to explore their connections.
Conclusions
The coexistence of PTA and MMD was rare reported, the pathogenesis of such condition remains yet to be explored. Based on the literature review, we found that the manifestation of such patient showed no classical characteristics. Revascularization might be a feasible choice for patient with PTA combined with MMD.
Data availability
Not applicable.
Abbreviations
- PTA:
-
Persistent trigeminal artery
- ICA:
-
Internal carotid artery
- MMD:
-
Moyamoya disease
- ACA:
-
Anterior cerebral artery
- MCA:
-
Middle cerebral artery
- CTP:
-
Computed tomography perfusion
- CTA:
-
Computed tomography angiography
- SCA:
-
Superior cerebellar arteries
- AICA:
-
Anterior inferior cerebellar artery
- PICA:
-
Posterior inferior cerebellar artery
- BA:
-
Basilar artery
- VA:
-
Vertebral artery
- PCA:
-
Posterior cerebral artery
- DSA:
-
Digital subtraction angiography
References
Alcala-Cerra G, Tubbs RS, Nino-Hernandez LM. Anatomical features and clinical relevance of a persistent trigeminal artery. Surg Neurol Int. 2012;3:111.
Hou K, Ji T, Guo Y, Xu K, Yu J. Coexistence of Persistent primitive trigeminal artery, Moyamoya Disease, and multiple intracranial aneurysms: a Case Report and Literature Review. WORLD NEUROSURG. 2019;124:313–8.
Sato H, Haraguchi K, Takahashi Y, Ohtaki S, Shimizu T, Matsuura N, Ogane K, Ito T. Flow-Diverter Stent for an unruptured aneurysm at the Junction of the Internal Carotid Artery and persistent primitive trigeminal artery: Case Report and Literature Review. WORLD NEUROSURG. 2019;132:329–32.
Shah KA, Katz JM. Ruptured persistent trigeminal artery-basilar artery Junction Aneurysm: Case Report and Review of Literature. WORLD NEUROSURG. 2020;133:159–62.
Choudhri O, Heit JJ, Feroze AH, Chang SD, Dodd RL, Steinberg GK. Persistent trigeminal artery supply to an intrinsic trigeminal nerve arteriovenous malformation: a rare cause of trigeminal neuralgia. J CLIN NEUROSCI. 2015;22(2):409–12.
Rodriguez-Hernandez LA, Martinez P, Baracaldo I, Alonso M, Lopez-Mena D, Montenegro RH. Persistent trigeminal artery associated with an occipital arteriovenous malformation: a case report and literature review. SURG RADIOL ANAT. 2022;44(9):1271–5.
Sun T, Huang Q, Li C, Wang W, He L, Liu J, Yang C. Microvascular decompression for trigeminal neuralgia caused by persistent trigeminal artery associated with craniosynostosis: a case report. J Med Case Rep. 2022;16(1):292.
de Bondt BJ, Stokroos R, Casselman J. Persistent trigeminal artery associated with trigeminal neuralgia: hypothesis of neurovascular compression. Neuroradiology. 2007;49(1):23–6.
Medhi G, Pendharkar H, TerBrugge K, Prasad C, Gupta AK. Trigeminal neuralgia caused by a new variant of persistent trigeminal artery associated with agenesis of internal carotid artery. INTERV NEURORADIOL. 2017;23(6):566–70.
Fujimura M, Tominaga T, Kuroda S, Takahashi JC, Endo H, Ogasawara K, Miyamoto S. 2021 Japanese guidelines for the management of Moyamoya Disease: guidelines from the Research Committee on Moyamoya Disease and Japan Stroke Society. Neurol Med Chir (Tokyo). 2022;62(4):165–70.
Kwak R, Kadoya S. Moyamoya disease associated with persistent primitive trigeminal artery. Report of two cases. J NEUROSURG. 1983;59(1):166–71.
The Anatomy of the Arteries of the Human. Body; with its applications to Pathology and operative surgery; in Lithographic drawings: with practical commentaries. Br Foreign Med Rev 1841, 11(21):210–1.
SUTTON D. Anomalous carotid-basilar anastomosis. Br J Radiol. 1950;23(274):617–9.
SALTZMAN GF. Patent primitive trigeminal artery studied by cerebral angiography. ACTA RADIOL. 1959;51(5):329–36.
Salas E, Ziyal IM, Sekhar LN, Wright DC. Persistent trigeminal artery: an anatomic study. NEUROSURGERY. 1998;43(3):557–61.
Weon YC, Choi SH, Hwang JC, Shin SH, Kwon WJ, Kang BS. Classification of persistent primitive trigeminal artery (PPTA): a reconsideration based on MRA. ACTA RADIOL. 2011;52(9):1043–51.
Liu YB, Feng PY, Zhang TZ, Sun J, Yang HX. A study on the persistent trigeminal artery and its classification based on magnetic resonance angiograph images. J Comput Assist Tomogr 2022.
Kinjo T, Mukawa J, Takara E, Nakasone S, Kuda H, Ishikawa Y. [Moyamoya disease associated with persistent primitive trigeminal artery–a case report and review of literature]. No Shinkei Geka. 1988;16(9):1107–12.
Lichtor T, Mullan S. Arteriovenous malformation in moyamoya syndrome. Report of three cases. J NEUROSURG. 1987;67(4):603–8.
Somasundaram S, Thamburaj K, Burathoki S, Gupta AK. Moyamoya disease with cerebral arteriovenous malformation presenting as primary subarachnoid hemorrhage. J NEUROIMAGING. 2007;17(3):251–4.
Noh JH, Yeon JY, Park JH, Shin HJ. Cerebral arteriovenous malformation associated with moyamoya disease. J Korean Neurosurg Soc. 2014;56(4):356–60.
Rossitti S, Raininko R. Absence of the common carotid artery in a patient with a persistent trigeminal artery variant. CLIN RADIOL. 2001;56(1):79–81.
Kim D, Baek JW, Heo YJ, Jeong HW. Congenital absence of the internal carotid artery with a persistent primitive trigeminal artery as a collateral pathway: a report of an anatomic variation. SURG RADIOL ANAT. 2018;40(7):811–3.
Jaeger HJ, Mehring UM, Gissler HM, Mathias KD. Congenital absence of the internal carotid artery and the basilar artery with persistent trigeminal artery associated with coarctation of the aorta. EUR RADIOL. 2000;10(11):1805–9.
Watanabe D, Endo H, Nakamura H. Persistent trigeminal artery variant associated with ipsilateral hypoplasia of the internal carotid artery. SURG RADIOL ANAT. 2022;44(6):947–50.
Hattori S, Kiguchi H, Ishii T, Nakajima T, Yatsuzuka H. Moyamoya disease with concurrent Von Recklinghausen’s disease and cerebral arteriovenous malformation. PATHOL RES PRACT. 1998;194(5):363–9.
Chen ST, Liu YH. Moyamoya disease associated with bilateral persistent primitive trigeminal arteries: report of a case. J FORMOS MED ASSOC. 1993;92(4):385–7.
Uchino A, Saito N, Takahashi M, Kurita H, Ishihara S. Cerebral arterial variations Associated with Moyamoya Disease diagnosed by MR Angiography. Neuroradiol J. 2014;27(6):697–701.
Suzuki S, Morioka T, Matsushima T, Ikezaki K, Hasuo K, Fukui M. Moyamoya disease associated with persistent primitive trigeminal artery variant in identical twins. Surg Neurol. 1996;45(3):236–40.
Xu Y, Kong Y, Xu Y, Wang P. The protective effect of persistent trigeminal artery in patients with ischemic stroke. BMC NEUROL. 2019;19(1):158.
Engelhardt J, El HG, Bojanowski MW. Persistent trigeminal artery as collateral circulation in ischemic stroke. WORLD NEUROSURG. 2021;148:67–9.
Handa J, Handa H. Progressive cerebral arterial occlusive disease: analysis of 27 cases. Neuroradiology. 1972;3(3):119–33.
Otsuki T, Fukawa O, Aihara H. [Case of persistent primitive trigeminal artery associated with moyamoya vessels]. Rinsho Hoshasen. 1982;27(13):1453–6.
Komiyama M, Kitano S, Sakamoto H, Shiomi M. An additional variant of the persistent primitive trigeminal artery: accessory meningeal artery–antero-superior cerebellar artery anastomosis associated with moyamoya disease. Acta Neurochir (Wien). 1998;140(10):1037–42.
Tan EC, Takagi T, Nagai H. Intracranial carotid artery occlusion with telangiectasia (moyamoya disease) associated with persistent primitive trigeminal artery–case report. Neurol Med Chir (Tokyo). 1991;31(12):800–3.
Acknowledgements
Not applicable.
Funding
This study was supported by the National Natural Science Foundation of China (No.81571202); the “five-five” project construction project of the Third Hospital of Sun Yat-sen University (No.2023WW504); Guangzhou Science and Technology Project (No.202201020578); Guangdong Basic and Applied Basic Research Foundation (No.2022A1515012433).
Author information
Authors and Affiliations
Contributions
Tao Sun: Data collection, conception, writing and modification, grammar and English improvements. Lixin Huang, Jun Sun and Zhimin Wu: imaging interpretation, data collection, graphics production and literatures review. Chuan Chen: manuscript revise and theoretical direction; Hui Wang: supervision, overall idea, design and funding.
Corresponding authors
Ethics declarations
Ethics approval and consent to participate
This study was approved by the ethics committee of the Third Affiliated Hospital of Sun Yat-Sen University and conducted ethically in accordance with the World Medical Association Declaration of Helsinki.
Consent for publication
Informed consent was obtained from the patient. All authors agree to submit this manuscript, and there is no debate about the authorship.
Competing interests
The authors declare no competing interests.
Additional information
Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
About this article
Cite this article
Sun, T., Huang, L., Sun, J. et al. Persistent trigeminal artery in a patient with moyamoya disease:a case report and literature review. BMC Neurol 24, 54 (2024). https://doi.org/10.1186/s12883-024-03545-y
Received:
Accepted:
Published:
DOI: https://doi.org/10.1186/s12883-024-03545-y