Clin Neuroradiol DOI 10.1007/s00062-014-0314-6
Correspondence
A Case of Giant Thrombosed Persistent Primitive Trigeminal Artery Aneurysm Presenting with Trigeminal Neuralgia and Successfully Treated by a Covered Stent: Case Report and Review of Literature W.-H. Chen · T.-H. Tsai · S.-C. Shen · C.-C. Shen · Y.-S. Tsuei Received: 21 January 2014 / Accepted: 10 May 2014 © Springer-Verlag Berlin Heidelberg 2014
Introduction
Case Presentation
The persistent primitive trigeminal artery (PPTA) is rare with an incidence ranging from 0.1 to 0.6 % and accounts for approximately 80–85 % of persistent carotid-basilar anastomosis [1]. Studies cited in the literature indicate that aneurysms may be associated with nearly 14 % of all PPTA cases, and may occur in a PPTA itself in 2 % of cases [2]. In addition, the prevalence of a PPTA in patients presenting with trigeminal neuralgia was reported as 2.2 % [3]. Our patient had both rare conditions, aneurysm formation at origin of PPTA and clinical presentation with trigeminal neuralgia. To our knowledge, this is the first paper to illustrate successful treatment of a PPTA aneurysm by endovascular obliteration with a covered stent.
A 69-year-old woman with hypertension presented to the emergency room with severe pulsatile pain around the left orbital area and diplopia for 2 days. Neurological examination revealed paresthesia over the upper two-thirds of left face innervated by the ophthalmic and maxillary divisions of trigeminal nerve and diplopia secondary to left abducens nerve palsy. Brain noncontrast computed tomography (CT) showed a 2.5 × 2.5 × 2.0 cm hyperdense mass over left cavernous sinus suggestive of thrombus formation (Fig. 1a). CT angiography (CTA) showed a giant, partially thrombosed aneurysm arising from the intracavernous internal carotid artery (ICA) (Fig. 1b). Digital subtraction angiography (DSA) showed a large persistent primitive trigeminal artery (PPTA) connecting the ICA to the distal part of the hypoplastic middle basilar artery (BA), with a 1.2 × 1.2 × 1.0 cm aneurysm sac at the origin of PPTA and hypoplasia in the left vertebral artery (VA). (Fig. 1c–f) Steroid therapy was prescribed initially and endovascular surgery was arranged. Aspirin (400 mg) and clopidogrel (300 mg) were administered before the treatment. All procedures were performed under general anesthesia and full heparinization. The covered stent, Jostent stent graft (previously JoMed, Helsingborg, Sweden; now Abbott Vascular, Redwood City, California, USA), was successfully deployed at left ICA to cover the orifice of persistent primitive trigeminal artery (Fig. 2). The postoperative course was uneventful. Trigeminal neuralgia improved immediately after treatment but diplopia persisted. One-year follow-up of left ICA DSA showed ICA patency was preserved and there was no recurrent aneurysm. In addition, the right VA DSA showed a small remnant of PPTA supplied by the vertebrobasilar artery (Fig. 3). The clinical situation at one-year follow-up showed complete resolution of trigeminal neural-
Y.-S. Tsuei () No. 160, Sec. 3, Chung-Kang Rd., Taichung, Taiwan e-mail: [email protected] W.-H. Chen, MD Department of Neuroradiology, Taichung Veterans General Hospital, Taichung, Taiwan T.-H. Tsai, MD · S.-C. Shen, MD · C.-C. Shen, MD · Y.-S. Tsuei Department of Neurosurgery, Triservice General Hospital, National Defense Medical Center, Taipei, Taiwan S.-C. Shen, MD · C.-C. Shen, MD · Y.-S. Tsuei Department of Neurosurgery, Taichung Veterans General Hospital, Taichung, Taiwan
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Fig. 1 Computed tomography (CT) and digital subtraction angiography (DSA). (a) Non-contrast CT scan showed a hyperdense mass about 2.5 cm in long axis at left parasellar region suggestive of partial thrombosed aneurysm. (b) CT angiography with oblique sagittal reconstruction showed an aneurysm with partial thrombosis arising from left cavernous portion of the internal carotid artery (ICA). Left ICA angiogram, frontal view (c), and lateral view (d) showed persistent primitive trigeminal artery (PPTA) arising from cavernous portion ICA with connection to basilar artery. An aneurysm was noted at junction of ICA and PPTA. Right vertebral angiogram, frontal view (e) and lateral view (f) showed reverse contrast opacification of PPTA and aneurysm
gia; there was no diplopia while looking straight ahead, but she still had partial abducens nerve palsy. Discussion The PPTA or its variants are usually incidental findings without any clinical importance. The PPTA could involve
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the cranial nerves III, IV, V, and VI, depending on its anatomic relationship. In 1993, Ohshiro et al. [4] reviewed 25 autopsy cases and classified PPTA course into two types: medial type, with the artery running through the dorsum sellae and perforating the dura mater near the clivus, and lateral type, with the artery running underneath the abducens nerve and continuing caudally between the trigeminal and abducens nerves to join the distal BA. These relationships
A Case of Giant Thrombosed Persistent Primitive Trigeminal Artery Aneurysm Presenting …
3
Fig. 2 Left internal carotid artery (ICA) angiogram before (a) and after (b) stenting. Aneurysm was completely sealed after stent deployment
Fig. 3 Follow-up angiograms at one year after stenting. Lateral view of left internal carotid artery (ICA) angiogram (a), rotational angiogram of left ICA with volume rendering reconstruction (b), right vertebral angiogram, frontal (c), and lateral (d) view showed successful obliteration of aneurysm and a remnant of distal persistent primitive trigeminal artery stump with perforating branches supplied from BA
may explain the reported cranial nerve syndrome related to PPTA persistence. The anatomical and angiographic studies suggest that trigeminal neuralgia and abducens nerve plasy might have resulted from the anomalous lateral type PPTA in our case. In addition to the formation of aneurysm associated with PPTA, other associated cerebral vascular anomalies, such as arteriovenous malformations [5], carotid-cavernous fistu-
las [6], and vertebrobasilar embolic ischemia [7] have also been reported. However, aneurysm formation was the most frequently associated anomaly in patients with PPTA. Kai Y et al. [8] reviewed 261 patients with PPTA reported from 1950 to 2008 and found 40 (15.3 %) presented with PPTAassociated aneurysm. In 18 of 40 cases, the location of the aneurysm was at the junction of cavernous ICA and PPTA, as in our case. The managements of these 18 aneurysms
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included surgical clipping in one case, surgical occlusion of ICA in 4, ICA occlusion with balloon in 3, endovascular embolization with coils in 5, and observation in 5. Surgical treatment of PTA aneurysms is often complicated by cranial nerve palsies as it involves dissection in the cavernous sinus or sacrifice of ICA accompanied with EC-IC bypass [9]. Endovascular treatment is a relatively less invasive and safer management, and includes balloon-assisted and stentassisted coiling techniques for wide-necked aneurysms [8]. To our knowledge, PPTA aneurysm treated with a covered stent has not been previously reported. This is the first study to report successful treatment of PPTA aneurysm using a covered stent. The advantages of using a covered stent for this patient was that effective relief of trigeminal neuralgia could be achieved and focal mass effect due to coil compression could be avoided. The disadvantage of a covered stent is the increased potential risk of intraarterial thrombosis or restenosis of the parent artery and its application is limited to cases with favourable straight course of internal carotid artery. The use of another flow diverter, such as pipeline stent (PED; ev3 Endovascular, Plymouth, MN, USA), might be a good choice but this method has not been approved in Taiwan, so a coronary covered stent was used instead. Another concern with the surgical approach is whether PPTA should be preserved as the perforating arteries originating from the PPTA may be important in supplying the hindbrain, and the blood supply of distal BA may be mainly from the PPTA. Eri O’uchi and Toshihiro O’uchi [10] reported a series of patients with PPTA with no BA hypoplasia in 26 % of cases, moderate hypoplasia in 47 %, and severe hypoplasia in 28 %. In our case, although there was severe hypoplasia in the left vertebral artery and moderate hypoplasia in the middle portion of BA, distal BA was still supplied sufficiently by flow from the right VA, and this result was only confirmed by DSA. The limitation of this
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W.-H. Chen et al.
intervention is that there may be a possible risk of ischemia without an adequate balloon occlusion test. Conflict of Interest The authors declare that there are no actual or potential conflicts of interest in relation to this article.
References 1. Meckel S, Spittau B, McAuliffe W. The persistent trigeminal artery: development, imaging anatomy, variants, and associated vascular pathologies. Neuroradiology. 2013;55(1):5–16. 2. Vasović L, Jovanović I, Ugrenović S, Vlajković S, Jovanović P, Stojanović V. Trigeminal artery: a review of normal and pathological features. Childs Nerv Syst. 2012;28(1):33–46. 3. de Bondt BJ, Stokroos R, Casselman J. Persistent trigeminal artery associated with trigeminal neuralgia: hypothesis of neurovascular compression. Neuroradiology. 2007;49(1):23–26. 4. Ohshiro S, Inoue T, Hamada Y, Matsuno H. Branches of the persistent primitive trigeminal artery—an autopsy case. Neurosurgery. 1993;32(1):144–8. 5. Kono K, Matsuda Y, Terada T. Resolution of trigeminal neuralgia following minimal coil embolization of a primitive trigeminal artery associated with a cerebellar arteriovenous malformation. Acta Neurochir (Wien). 2013;155(9):1699–701. 6. Guglielmi G, Viñuela F, Dion J, Duckwiler G, Cantore G, Delfini R. Persistent primitive trigeminal artery-cavernous sinus fistulas: report of two cases. Neurosurgery. 1990;27(5):805–8. 7. Kwon JY, Lee EJ, Kim JS. Brainstem infarction secondary to persistent trigeminal artery occlusion: successful treatment with intravenous rt-PA. Eur Neurol. 2010;64(5):311. 8. Kai Y, Ohmori Y, Watanabe M, Morioka M, Hirano T, Kawano T, Sakurama T, Miura A, Kuratsu J. Coil embolization of an aneurysm located at the trunk of the persistent primitive trigeminal artery. Neurol Med Chir (Tokyo). 2011;51(5):361–4. 9. Morrison G, Hegarty WM, Brausch CC, Castele TJ, White RJ. Direct surgical obliteration of a persistent trigeminal artery aneurysm. Case report. J Neurosurg. 1974;40(2):249–51. 10. O’uchi E, O’uchi T. Persistent primitive trigeminal arteries (PTA) and its variant (PTAV): analysis of 103 cases detected in 16,415 cases of MRA over 3 years. Neuroradiology. 2010;52:1111–9.
Correspondence
A Case of Giant Thrombosed Persistent Primitive Trigeminal Artery Aneurysm Presenting with Trigeminal Neuralgia and Successfully Treated by a Covered Stent: Case Report and Review of Literature W.-H. Chen · T.-H. Tsai · S.-C. Shen · C.-C. Shen · Y.-S. Tsuei Received: 21 January 2014 / Accepted: 10 May 2014 © Springer-Verlag Berlin Heidelberg 2014
Introduction
Case Presentation
The persistent primitive trigeminal artery (PPTA) is rare with an incidence ranging from 0.1 to 0.6 % and accounts for approximately 80–85 % of persistent carotid-basilar anastomosis [1]. Studies cited in the literature indicate that aneurysms may be associated with nearly 14 % of all PPTA cases, and may occur in a PPTA itself in 2 % of cases [2]. In addition, the prevalence of a PPTA in patients presenting with trigeminal neuralgia was reported as 2.2 % [3]. Our patient had both rare conditions, aneurysm formation at origin of PPTA and clinical presentation with trigeminal neuralgia. To our knowledge, this is the first paper to illustrate successful treatment of a PPTA aneurysm by endovascular obliteration with a covered stent.
A 69-year-old woman with hypertension presented to the emergency room with severe pulsatile pain around the left orbital area and diplopia for 2 days. Neurological examination revealed paresthesia over the upper two-thirds of left face innervated by the ophthalmic and maxillary divisions of trigeminal nerve and diplopia secondary to left abducens nerve palsy. Brain noncontrast computed tomography (CT) showed a 2.5 × 2.5 × 2.0 cm hyperdense mass over left cavernous sinus suggestive of thrombus formation (Fig. 1a). CT angiography (CTA) showed a giant, partially thrombosed aneurysm arising from the intracavernous internal carotid artery (ICA) (Fig. 1b). Digital subtraction angiography (DSA) showed a large persistent primitive trigeminal artery (PPTA) connecting the ICA to the distal part of the hypoplastic middle basilar artery (BA), with a 1.2 × 1.2 × 1.0 cm aneurysm sac at the origin of PPTA and hypoplasia in the left vertebral artery (VA). (Fig. 1c–f) Steroid therapy was prescribed initially and endovascular surgery was arranged. Aspirin (400 mg) and clopidogrel (300 mg) were administered before the treatment. All procedures were performed under general anesthesia and full heparinization. The covered stent, Jostent stent graft (previously JoMed, Helsingborg, Sweden; now Abbott Vascular, Redwood City, California, USA), was successfully deployed at left ICA to cover the orifice of persistent primitive trigeminal artery (Fig. 2). The postoperative course was uneventful. Trigeminal neuralgia improved immediately after treatment but diplopia persisted. One-year follow-up of left ICA DSA showed ICA patency was preserved and there was no recurrent aneurysm. In addition, the right VA DSA showed a small remnant of PPTA supplied by the vertebrobasilar artery (Fig. 3). The clinical situation at one-year follow-up showed complete resolution of trigeminal neural-
Y.-S. Tsuei () No. 160, Sec. 3, Chung-Kang Rd., Taichung, Taiwan e-mail: [email protected] W.-H. Chen, MD Department of Neuroradiology, Taichung Veterans General Hospital, Taichung, Taiwan T.-H. Tsai, MD · S.-C. Shen, MD · C.-C. Shen, MD · Y.-S. Tsuei Department of Neurosurgery, Triservice General Hospital, National Defense Medical Center, Taipei, Taiwan S.-C. Shen, MD · C.-C. Shen, MD · Y.-S. Tsuei Department of Neurosurgery, Taichung Veterans General Hospital, Taichung, Taiwan
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W.-H. Chen et al.
Fig. 1 Computed tomography (CT) and digital subtraction angiography (DSA). (a) Non-contrast CT scan showed a hyperdense mass about 2.5 cm in long axis at left parasellar region suggestive of partial thrombosed aneurysm. (b) CT angiography with oblique sagittal reconstruction showed an aneurysm with partial thrombosis arising from left cavernous portion of the internal carotid artery (ICA). Left ICA angiogram, frontal view (c), and lateral view (d) showed persistent primitive trigeminal artery (PPTA) arising from cavernous portion ICA with connection to basilar artery. An aneurysm was noted at junction of ICA and PPTA. Right vertebral angiogram, frontal view (e) and lateral view (f) showed reverse contrast opacification of PPTA and aneurysm
gia; there was no diplopia while looking straight ahead, but she still had partial abducens nerve palsy. Discussion The PPTA or its variants are usually incidental findings without any clinical importance. The PPTA could involve
13
the cranial nerves III, IV, V, and VI, depending on its anatomic relationship. In 1993, Ohshiro et al. [4] reviewed 25 autopsy cases and classified PPTA course into two types: medial type, with the artery running through the dorsum sellae and perforating the dura mater near the clivus, and lateral type, with the artery running underneath the abducens nerve and continuing caudally between the trigeminal and abducens nerves to join the distal BA. These relationships
A Case of Giant Thrombosed Persistent Primitive Trigeminal Artery Aneurysm Presenting …
3
Fig. 2 Left internal carotid artery (ICA) angiogram before (a) and after (b) stenting. Aneurysm was completely sealed after stent deployment
Fig. 3 Follow-up angiograms at one year after stenting. Lateral view of left internal carotid artery (ICA) angiogram (a), rotational angiogram of left ICA with volume rendering reconstruction (b), right vertebral angiogram, frontal (c), and lateral (d) view showed successful obliteration of aneurysm and a remnant of distal persistent primitive trigeminal artery stump with perforating branches supplied from BA
may explain the reported cranial nerve syndrome related to PPTA persistence. The anatomical and angiographic studies suggest that trigeminal neuralgia and abducens nerve plasy might have resulted from the anomalous lateral type PPTA in our case. In addition to the formation of aneurysm associated with PPTA, other associated cerebral vascular anomalies, such as arteriovenous malformations [5], carotid-cavernous fistu-
las [6], and vertebrobasilar embolic ischemia [7] have also been reported. However, aneurysm formation was the most frequently associated anomaly in patients with PPTA. Kai Y et al. [8] reviewed 261 patients with PPTA reported from 1950 to 2008 and found 40 (15.3 %) presented with PPTAassociated aneurysm. In 18 of 40 cases, the location of the aneurysm was at the junction of cavernous ICA and PPTA, as in our case. The managements of these 18 aneurysms
13
4
included surgical clipping in one case, surgical occlusion of ICA in 4, ICA occlusion with balloon in 3, endovascular embolization with coils in 5, and observation in 5. Surgical treatment of PTA aneurysms is often complicated by cranial nerve palsies as it involves dissection in the cavernous sinus or sacrifice of ICA accompanied with EC-IC bypass [9]. Endovascular treatment is a relatively less invasive and safer management, and includes balloon-assisted and stentassisted coiling techniques for wide-necked aneurysms [8]. To our knowledge, PPTA aneurysm treated with a covered stent has not been previously reported. This is the first study to report successful treatment of PPTA aneurysm using a covered stent. The advantages of using a covered stent for this patient was that effective relief of trigeminal neuralgia could be achieved and focal mass effect due to coil compression could be avoided. The disadvantage of a covered stent is the increased potential risk of intraarterial thrombosis or restenosis of the parent artery and its application is limited to cases with favourable straight course of internal carotid artery. The use of another flow diverter, such as pipeline stent (PED; ev3 Endovascular, Plymouth, MN, USA), might be a good choice but this method has not been approved in Taiwan, so a coronary covered stent was used instead. Another concern with the surgical approach is whether PPTA should be preserved as the perforating arteries originating from the PPTA may be important in supplying the hindbrain, and the blood supply of distal BA may be mainly from the PPTA. Eri O’uchi and Toshihiro O’uchi [10] reported a series of patients with PPTA with no BA hypoplasia in 26 % of cases, moderate hypoplasia in 47 %, and severe hypoplasia in 28 %. In our case, although there was severe hypoplasia in the left vertebral artery and moderate hypoplasia in the middle portion of BA, distal BA was still supplied sufficiently by flow from the right VA, and this result was only confirmed by DSA. The limitation of this
13
W.-H. Chen et al.
intervention is that there may be a possible risk of ischemia without an adequate balloon occlusion test. Conflict of Interest The authors declare that there are no actual or potential conflicts of interest in relation to this article.
References 1. Meckel S, Spittau B, McAuliffe W. The persistent trigeminal artery: development, imaging anatomy, variants, and associated vascular pathologies. Neuroradiology. 2013;55(1):5–16. 2. Vasović L, Jovanović I, Ugrenović S, Vlajković S, Jovanović P, Stojanović V. Trigeminal artery: a review of normal and pathological features. Childs Nerv Syst. 2012;28(1):33–46. 3. de Bondt BJ, Stokroos R, Casselman J. Persistent trigeminal artery associated with trigeminal neuralgia: hypothesis of neurovascular compression. Neuroradiology. 2007;49(1):23–26. 4. Ohshiro S, Inoue T, Hamada Y, Matsuno H. Branches of the persistent primitive trigeminal artery—an autopsy case. Neurosurgery. 1993;32(1):144–8. 5. Kono K, Matsuda Y, Terada T. Resolution of trigeminal neuralgia following minimal coil embolization of a primitive trigeminal artery associated with a cerebellar arteriovenous malformation. Acta Neurochir (Wien). 2013;155(9):1699–701. 6. Guglielmi G, Viñuela F, Dion J, Duckwiler G, Cantore G, Delfini R. Persistent primitive trigeminal artery-cavernous sinus fistulas: report of two cases. Neurosurgery. 1990;27(5):805–8. 7. Kwon JY, Lee EJ, Kim JS. Brainstem infarction secondary to persistent trigeminal artery occlusion: successful treatment with intravenous rt-PA. Eur Neurol. 2010;64(5):311. 8. Kai Y, Ohmori Y, Watanabe M, Morioka M, Hirano T, Kawano T, Sakurama T, Miura A, Kuratsu J. Coil embolization of an aneurysm located at the trunk of the persistent primitive trigeminal artery. Neurol Med Chir (Tokyo). 2011;51(5):361–4. 9. Morrison G, Hegarty WM, Brausch CC, Castele TJ, White RJ. Direct surgical obliteration of a persistent trigeminal artery aneurysm. Case report. J Neurosurg. 1974;40(2):249–51. 10. O’uchi E, O’uchi T. Persistent primitive trigeminal arteries (PTA) and its variant (PTAV): analysis of 103 cases detected in 16,415 cases of MRA over 3 years. Neuroradiology. 2010;52:1111–9.
