Yoshifumi Horita, Takeshi Mikami, Kiyohiro Houkin, Nobuhiro Mikuni
  1. Department of Neurosurgery, Sapporo Medical University, Sapporo, Japan
  2. Department of Neurosurgery, Hokkaido University, Graduate School of Medicine, Sapporo, Japan

Correspondence Address:
Takeshi Mikami
Department of Neurosurgery, Sapporo Medical University, Sapporo, Japan


Copyright: © 2015 Horita Y. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

How to cite this article: Horita Y, Mikami T, Houkin K, Mikuni N. Cerebral aneurysms associated with segmental dilative arteriopathy of the circle of Willis. Surg Neurol Int 25-Jun-2015;6:

How to cite this URL: Horita Y, Mikami T, Houkin K, Mikuni N. Cerebral aneurysms associated with segmental dilative arteriopathy of the circle of Willis. Surg Neurol Int 25-Jun-2015;6:. Available from:

Date of Submission

Date of Acceptance

Date of Web Publication


Background:Dilative arteriopathy is a form of dolichoectasia. It is sometimes observed in the posterior circulation, and it may be associated with various type of stroke. Herein, we report two unusual cases of saccular aneurysms associated with a segmental dilative arteriopathy located in the anterior circulation.

Case Descriptions:The first case is a 39-year-old woman with irregular tortuosity and coiling of the left internal cerebral artery along with saccular aneurysms in this artery. The second case is a 45-year-old woman presenting with a ruptured saccular aneurysm in the coiling of the anterior cerebral artery. In both cases, the aneurysm was clipped successfully, and the patients recovered uneventfully with no neurological deficits.

Conclusion:Dilative arteriopathy of the circle of Willis is an unusual anomaly and is characterized by tortuous and elongated arteries, which are sometimes observed in patients with a congenital anomaly. This report describes two cases of saccular aneurysm associated with dilative arteriopathy of the circle of Willis with no medical history, which to the best of our knowledge has not previously been described.

Keywords: Anterior cerebral artery, arteriopathy, dolichoectasia, subarachnoid hemorrhage


Dolichoectasia is a broad concept that may include many diseases. Intracranial dolichoectasia is characterized by enlargement, tortuosity, and elongation of the major arteries of the brain and is typically observed in the posterior circulation.[ 6 ] Various anomalies have been diagnosed as dolichoectasia, including fusiform aneurysms, coiling, and multiple vascular enlargements. In any case, dilation is the most striking feature; therefore this condition is now referred to as dilative arteriopathy. Although dilative arteriopathy is frequently asymptomatic in children, it may be associated with stroke, subarachnoid hemorrhage, cranial nerve palsy, compression of the midbrain or obstructive hydrocephalus in adults.[ 14 15 18 ] In this report, we describe two cases of saccular aneurysm associated with segmental dilative arteriopathy of the internal cerebral artery (ICA) or anterior cerebral artery (ACA), one of which was a ruptured aneurysm.


Case 1

A 39-year-old woman with no remarkable medical or familial history underwent magnetic resonance (MR) imaging for headache. MR angiography and three-dimensional (3D) computed tomographic (CT) angiography revealed irregular tortuosity of the ICA from the ophthalmic artery to the terminal portion (C1, C2) on the left side and saccular aneurysms in the left C1. Cerebral angiography revealed that the ICA coiled twice between the ophthalmic artery and the anterior choroidal artery and contained four saccular aneurysms distal to the posterior communicating artery [ Figure 1a ]. All other intracranial vessels appeared completely normal.

Figure 1

(a) Three-dimensional digital subtraction angiography (DSA) showing the elongated and tortuous left internal cerebral artery (ICA) with saccular aneurysms (white arrow). (b) Postoperative DSA showing successful clipping of these aneurysms. Intraoperative photographs (c and d) and illustrations. (e and f) Enlarged and tortuous left ICA with saccular aneurysms (arrow), and successful clipping of these aneurysms


The patient was taken for microsurgical exploration. The left ICA was dilated and coiled. During temporary occlusion of the ICA, the artery collapsed completely; no arterial dissection or atherosclerotic changes were observed [Figure 1c and e ]. Accordingly, the aneurysm was successfully clipped at the neck [Figure 1d and f ]. Postoperative angiogram revealed obliteration of the aneurysmal neck and the absence of residual opacification [ Figure 1b ]. The patient recovered uneventfully and was discharged with no neurological deficits. Aneurysm has not recurred at the same site in the 5 years since the surgery.

Case 2

A 45-year-old woman with no remarkable medical or familial history suddenly developed a severe headache and nausea and was immediately transported to our hospital by ambulance. At the time of admission, she experienced mild consciousness disturbance but no other neurological deficits. She had no history of head trauma or connective tissue disorder. CT revealed diffuse subarachnoid hemorrhage, and 3D CT angiography and cerebral angiography revealed irregular tortuosity of the horizontal portion of the ACA (A1) on the left side and a saccular aneurysm in the left A1 segment [Figure 2a , c and d ]. All other intracranial vessels appeared completely normal.

Figure 2

(a) Digital subtraction angiography (DSA) showing the elongated and tortuous left anterior cerebral artery (ACA) with saccular aneurysm (white arrow). (b) Postoperative DSA showing successful clipping of the aneurysm. Preoperative three-dimensional computed tomographic angiography (c and d), intraoperative photographs (e and f) and illustrations. (g and h) Enlarged and tortuous left ACA with saccular aneurysm (arrow), and successful clipping of the aneurysm


On day 1, the patient was taken to the operating room for microsurgical exploration, and a left frontotemporal craniotomy was performed. The dome of the aneurysm, including the rupture point, was buried in the frontal lobe. The left A1 was dilated, elongated, and tortuous [Figure 2e , f , and g ]. The neck of the aneurysm was apparent, however, and the aneurysm was successfully clipped at the neck [Figure 2f and h ]. Postoperative angiogram revealed complete clipping of the aneurysm [ Figure 2b ]. The patient recovered uneventfully and was discharged 22 days after surgery in an ambulatory condition with no neurological deficits. Aneurysm at the same site has not recurred in the 7 years since the surgery.


Several diseases have been implicated as potential causes of dilative arteriopathy, including both congenital and acquired conditions.[ 2 6 ] From a pathological perspective, arterial dilation is caused by aberrant vascular remodeling,[ 6 ] which is induced by the disruption of the internal elastic lamina. Breakdown and remodeling of the internal elastic lamina are controlled by early growth response protein 1 and matrix metalloprotease (MMP).[ 16 ] Overexpression of MMPs damages the fibronectin mesh that smooth muscle cells used to travel within the vessel wall,[ 10 ] and arterial dilation arises as a result. Morphologically, dilation of the arteries can develop according to two patterns. One is a nonsegmental pattern in which dilation occurs over a comparatively wide area.[ 8 ] The other is a segmental pattern expressing geotropism, which might be related to focal dysfunction. Nonsegmental dilative arteriopathy represents the fragility of the entire arterial system when exposed to a specific trigger. This condition is found in patients with atherosclerosis, alpha-glucosidase deficiency, Marfan's syndrome, Ehlers–Danlos syndrome, Loyes–Dietz syndrome, AIDS, Fabry's disease, and sickle cell disease.[ 4 6 9 11 ] Although several genetic disorders are included in this category, our two cases were negative for these genetic diseases. Therefore, further genetic investigation was conducted. Segmental dilative arteriopathy, in contrast, is related to the segmental identity of the vessel and its associated segmental vulnerability. This condition is sometimes found in patients with PHACES syndrome or moyamoya disease.[ 2 17 ] Cerebral vasculopathy in PHACES syndrome chiefly comprises arterial anomalies, with the arteries of the circle of Willis most commonly involved.[ 7 ] Segmental dilative arteriopathy can also derive from embryological factors: Focal defects and destruction of the internal elastic lamina have been implicated in its pathogenesis.[ 6 13 ] Lasjaunias advocated for the identification of seven segments within the ICA,[ 8 ] namely, (1) cervical, (2) ascending petrous, (3) horizontal petrous, (4) ascending cavernous, (5) horizontal cavernous, (6) clinoid, and (7) terminal. Segmental arteriopathy is induced primarily through congenital dysgenesis of one ICA segment. Based on this theory, the carotid rete mirabile might be also subject to segmental arteriopathy. Anomaly of the terminal portion of the ICA or the ACA is extremely rare.[ 3 5 ] In one of our two cases, vascular abnormality was limited to the region between the anterior choroidal artery and the posterior communicating artery. In the other case, vascular abnormality was limited to segment A1. These cases provide support for the segmental arteriopathy hypothesis.

Although associated saccular aneurysms are typically induced by hemodynamic stress due to dilative arteriopathy, most aneurysms associated with dolichoectasia are dissecting aneurysms or fusiform aneurysms.[ 12 ] As for the causes of our cases, hemodynamic stress resulting from the unique flow associated with vulnerable vessels might be involved in addition to congenital factors, given the relatively broad-based lesions from irregular segments. Careful long-term observation would be required to confirm this. The aneurysms in our cases were saccular aneurysms that were confirmed perioperatively and successfully clipped. Aneurysm clipping will certainly not be possible in all cases, however, for such cases, flow alteration treatment using a bypass graft should also be available as an alternate strategy.[ 1 ] With either treatment method, careful long-term observation is required, especially as a better understanding of these arteriopathies may improve our ability to predict stroke in affected patients.


1. Anson JA, Lawton MT, Spetzler RF. Characteristics and surgical treatment of dolichoectatic and fusiform aneurysms. J Neurosurg. 1996. 84: 185-93

2. Baccin CE, Krings T, Alvarez H, Ozanne A, Lasjaunias PL. A report of two cases with dolichosegmental intracranial arteries as a new feature of PHACES syndrome. Childs Nerv Syst. 2007. 23: 559-67

3. Beringer W, Alenghat J. Pericallosal artery ectasia with associated stenosis. AJNR Am J Neuroradiol. 2004. 25: 1197-8

4. Dhouib A, Beghetti M, Didier D. Imaging findings in a child with Loeys-Dietz syndrome. Circulation. 2012. 126: 507-8

5. Doran SE, Deveikis JP, Chandler WF. Dolichoectasia of the anterior cerebral arteries in an adolescent. AJNR Am J Neuroradiol. 1995. 16: 1548-50

6. Gutierrez J, Sacco RL, Wright CB. Dolichoectasia-an evolving arterial disease. Nat Rev Neurol. 2011. 7: 41-50

7. Heyer GL, Dowling MM, Licht DJ, Tay SK, Morel K, Garzon MC. The cerebral vasculopathy of PHACES syndrome. Stroke. 2008. 39: 308-16

8. Lasjaunias P, Santoyo-Vazquez A. Segmental agenesis of the internal carotid artery: Angiographic aspects with embryological discussion. Anat Clin. 1984. 6: 133-41

9. Makos MM, McComb RD, Hart MN, Bennett DR. Alpha-glucosidase deficiency and basilar artery aneurysm: Report of a sibship. Ann Neurol. 1987. 22: 629-33

10. Mercurius KO, Morla AO. Inhibition of vascular smooth muscle cell growth by inhibition of fibronectin matrix assembly. Circ Res. 1998. 82: 548-56

11. Mitsias P, Levine SR. Cerebrovascular complications of Fabry's disease. Ann Neurol. 1996. 40: 8-17

12. Mizutani T, Kojima H. Clinicopathological features of non-atherosclerotic cerebral arterial trunk aneurysms. Neuropathology. 2000. 20: 91-7

13. Nakatomi H, Segawa H, Kurata A, Shiokawa Y, Nagata K, Kamiyama H. Clinicopathological study of intracranial fusiform and dolichoectatic aneurysms: Insight on the mechanism of growth. Stroke. 2000. 31: 896-900

14. Pessin MS, Chimowitz MI, Levine SR, Kwan ES, Adelman LS, Earnest MP. Stroke in patients with fusiform vertebrobasilar aneurysms. Neurology. 1989. 39: 16-21

15. Smoker WR, Corbett JJ, Gentry LR, Keyes WD, Price MJ, McKusker S. High-resolution computed tomography of the basilar artery: 2. Vertebrobasilar dolichoectasia: Clinical-pathologic correlation and review. AJNR Am J Neuroradiol. 1986. 7: 61-72

16. Tronc F, Mallat Z, Lehoux S, Wassef M, Esposito B, Tedgui A. Role of matrix metalloproteinases in blood flow-induced arterial enlargement: Interaction with NO. Arterioscler Thromb Vasc Biol. 2000. 20: E120-6

17. Yamada K, Hayakawa T, Ushio Y, Mitomo M. Cerebral arterial dolichoectasia associated with moyamoya vessels. Surg Neurol. 1985. 23: 19-24

18. Yu YL, Moseley IF, Pullicino P, McDonald WI. The clinical picture of ectasia of the intracerebral arteries. J Neurol Neurosurg Psychiatry. 1982. 45: 29-36

Leave a Reply

Your email address will not be published. Required fields are marked *