Spontaneous middle cerebral artery occlusion leading to moyamoya phenomenon and aneurysm formation on collateral arteries

doi:10.1016/S0090-3019(00)00339-6

Surgical Neurology

Volume 55, Issue 1, January 2001, Pages 58-62

https://doi.org/10.1016/S0090-3019(00)00339-6 Get rights and content

Abstract

Background

Spontaneous middle cerebral artery occlusion associated with moyamoya phenomenon is distinct from moyamoya disease. The hemodynamic stress on the collateral channel occasionally leads to aneurysm formation, which may manifest as hemorrhage. The etiology of this disease has not been fully understood.

CASE DESCRIPTION

A 63-year-old woman presented with left putaminal hemorrhage. The cerebral angiogram revealed a significant stenosis in the proximal segment of the left middle cerebral artery. Collateral arteries originating from the horizontal segment of the ipsilateral anterior cerebral artery and the ambient segment of the ipsilateral posterior cerebral artery supplied the middle cerebral artery distal to the stenosis. Both of the collateral channels had associated aneurysms that were surgically obliterated. The aneurysm on the collateral artery from the posterior cerebral artery was responsible for the putaminal hemorrhage.

CONCLUSIONS

Spontaneous middle cerebral artery occlusion may lead to focal moyamoya phenomenon and aneurysmal intracerebral, intraventricular, or subarachnoid hemorrhage. The presence of a co-existing anomalous collateral artery in the present case suggests a congenital etiology of the focal middle cerebral artery occlusion.

Section snippets

Case report

A 63-year-old woman presented with a sudden onset of aphasia and right hemiparesis. Her blood pressure recordings revealed that she was normotensive (140/70 mmHg). Her computed tomography (CT) scan showed approximately 40 mL of left putaminal hematoma and a small amount of subarachnoid clot in the left Sylvian fissure (Figure 1). There was no intraventricular hemorrhage. Her left carotid angiogram demonstrated a severe focal stenosis in the proximal segment (M1) of the MCA. The circulation to

Discussion

In 1981 Fukawa et al described a clinical entity of spontaneous MCA occlusion associated with moyamoya phenomenon [1]. Patients with this disease usually present with either cerebral hemorrhage or ischemia in their 5th or 6th decade, but this disease can also be found in childhood [12]. This category of cerebrovascular occlusive disease is distinct from moyamoya disease as the carotid angiogram shows occlusion or stenosis restricted to the M1 segment of the MCA associated with localized

References (13)

M. Matsuda et al.
Ruptured cerebral aneurysms associated with arterial occlusion
Surg Neurol
(1983)
J.W. Walsh et al.
Subarachnoid hemorrhage in an adolescent with spontaneous occlusion of the left middle cerebral artery and reconstruction by collateral vessels (moyamoya phenomenon)
Surg Neurol
(1984)
Fukawa O, Aihara H, Ishii K, Kikuchi J, Mashiyama S, Katayama M, Kogure T. Middle cerebral artery occlusion with...
O. Fukawa et al.
Middle cerebral artery occlusion with moyamoya phenomenon. Second reportpathological study of an autopsy case
Neurol Surg (Japan)
(1982)
J.C. Grabel et al.
Moyamoya-like disease associated with a lenticulostriate region aneurysm
J Neurosurg
(1989)
T. Kageji et al.
Spontaneous middle cerebral artery occlusion with moyamoya-like vessels associated with contralateral middle cerebral artery aneurysm; a case report
Neurol Surg (Japan)
(1992)

There are more references available in the full text version of this article.

Cited by (31)

Characterization of Moyamoya and Middle Cerebral Artery Diseases by Carotid Canal Diameter and RNF213 p.R4810K Genotype
2022, Journal of Stroke and Cerebrovascular Diseases
It is sometimes difficult to differentiate middle cerebral artery disease from moyamoya disease because the two can present similarly yet have different treatment strategies. We investigated whether the presence of a narrow carotid canal and the RNF213 mutation can help differentiate between the two phenotypes.
We analyzed 78 patients with moyamoya disease, 27 patients with middle cerebral artery disease, and 79 controls from 2 facilities. The carotid canal diameter was measured using computed tomography. The p.R4810K mutation was genotyped by TaqMan assay. A receiver operating characteristics analysis was performed to assess the significance of the carotid canal diameter for the accurate diagnosis of moyamoya disease.
The carotid canal diameter was significantly narrower in patients with moyamoya disease than in controls. The optimal cutoff values were 5.0 mm for adult males and 4.5 mm for adult females and children (sensitivity: 0.82; specificity: 0.92). Among the patients with middle cerebral artery disease, 18.5% and 25.0% of the affected hemispheres had the p.R4810K mutation and narrow canal (i.e., below the cutoff), respectively, whereas only 3.1% of those had both. Contrastingly, 68.8% of the affected hemispheres in patients with moyamoya disease had both these characteristics. Among the patients with moyamoya disease, those with the p.R4810K mutation tended to have narrower carotid canals.
Although the presence of a narrow carotid canal or the p.R4810K mutation alone could not be used to distinguish those with moyamoya disease from those with middle cerebral artery disease, the combination of these factors could better characterize the two phenotypes.
Moyamoya Vasculopathy: Cause, Clinical Manifestations, Neuroradiologic Features, and Surgical Management
2022, World Neurosurgery
Citation Excerpt :
The development of moyamoya collaterals may serve as an endogenous bypass procedure and have a protective role from hypoperfusion. Data from the literature have shown that intracranial hemorrhage can be related to the direct rupture of these tiny vessels or to aneurysm bleeding formed on their wall as described in patients with MMD.72,73 Tanaka et al.74 showed that in MMS, secondary to intracranial atherosclerosis, moyamoya vessels are more developed in the presence of multiple steno-occlusive lesions involving the ACA and MCA and in poorly developed leptomeningeal collateral vessels from the ACA.
In moyamoya disease, the progressive occlusion of the distal portion of the internal carotid artery and its major branches is typically responsible for the formation of an extensive network of collateral vessels at the base of the brain. When moyamoya collateral network develops in association with various systemic or acquired diseases, the term moyamoya syndrome is used to denote this phenomenon.
Sudden changes in the supraclinoid internal carotid artery and middle cerebral artery can be recognized with noninvasive neurovascular imaging techniques, which also allow a differential diagnosis with similar diseases such as degenerative steno-occlusive disease, cerebral vasculitis, and twig-like middle cerebral artery. Once the diagnosis is established, the definitive treatment for moyamoya disease is surgical revascularization, with the goal of increasing cerebral blood flow and preventing recurrent stroke.
We provide a comprehensive review of the clinical and radiologic features in moyamoya vasculopathy along with its surgical management.
Preventive Effect of Bypass Surgery on Rebleeding in Patients with Hemorrhagic Twiglike Middle Cerebral Artery
2021, World Neurosurgery
Aplastic or twiglike middle cerebral artery (Ap/T-MCA) is a rare cerebrovascular anomaly correlated to stroke. The most common characteristic of the disease is intracerebral hemorrhage. However, treatment measures that can prevent rebleeding have not yet been established.
We sought to validate whether extracranial-intracranial (EC-IC) bypass is effective in preventing recurrent intracerebral hemorrhage in patients with Ap/T-MCA.
Ap/T-MCA was defined on the basis of the following criteria: 1) M1 occlusion with plexiform vessels, 2) unilateral MCA involvement without progression to the contralateral side, 3) no significant stenosis in the M2 segment, and 4) no stenoocclusive lesions in other intracranial major arteries. The clinical features, surgical procedures, radiologic changes, and treatment outcomes of patients with Ap/T-MCA who presented with ipsilateral intracerebral hemorrhage at our institution from April 2004 to December 2019 were retrospectively reviewed.
Seven patients fulfilled the criteria for hemorrhagic Ap/T-MCA. Female preponderance was observed, and the mean age at the time of onset was 38 (range: 28–47) years. Three patients presented with pure intraventricular hemorrhage and 4 with intraparenchymal hemorrhage. Of the 4 patients, 2 had intraventricular extension. All patients underwent EC-IC bypass and did not present with stroke during the follow-up period (median: 36 [range: 5–140] months). Follow-up magnetic resonance imaging revealed regression of the twiglike vessels or periventricular choroidal anastomosis within 1 year after surgery in all patients.
EC-IC bypass may be a treatment option that can prevent rebleeding in patients with hemorrhagic Ap/T-MCA.
Clinical Manifestations of Isolated Chronic Middle Cerebral Artery Occlusion in Relation to Angiographic Features
2017, World Neurosurgery
Isolated chronic middle cerebral artery occlusion (ChMCAO) is not a rare condition and is known to cause hemodynamic stroke. The purpose of this study was to evaluate differences in clinical manifestations and prognosis of isolated ChMCAO in relation to angiographic features.
This retrospective study enrolled 56 patients with isolated ChMCAO. In accordance with degree of anterograde collateral flow (AF) on angiography, patients were categorized into poor and good AF groups. The 2 groups were assessed and compared for the presence and recurrence of neurologic symptoms.
Of 56 patients, 33 were in the poor AF group and 23 were in the good AF group. The prevalence of ischemic symptoms was significantly higher in the poor AF group compared with the good AF group (P < 0.05). During an average follow-up period of 33.8 months, recurrent ipsilateral symptoms occurred in 6 of 45 patients. The hazard ratio conferred by poor AF was 5.36 (95% confidence interval, 1.08–26.57) for recurrent symptoms.
AF through the basal collateral network may be related to clinical manifestations of ChMCAO. Good AF in isolated ChMCAO may play an important role in preventing recurrence of an ischemic event.
Microsurgical Repair of Ruptured Aneurysms Associated with Moyamoya-Pattern Collateral Vessels of the Middle Cerebral Artery: A Report of Two Cases
2017, World Neurosurgery
Citation Excerpt :
Surgery has the added benefit of allowing evacuation of a cerebral hematoma or decompressive craniectomy, as was done in our cases. Although the limited number of reported cases makes it difficult to definitely recommend a treatment strategy, microsurgical approach appears to be safe and effective and should be considered, given the high mortality associated with aneurysm rebleed if left untreated.4,9-11,31,46-48 Alternative interventions to microsurgery also should be considered, depending on the location and accessibility of the aneurysm.
Patients with Moyamoya or other intracranial steno-occlusive disease are at risk for developing aneurysms associated with flow through collateral vessels. Because these lesions are rare, the optimal management remains unclear. Here, we describe 2 cases of microsurgical repair of ruptured collateral vessel aneurysms associated with middle cerebral artery (MCA) occlusion.
The first patient was a 61-year-old man who presented with right frontal and intraventricular hemorrhage. Angiography revealed chronic right M1 occlusion and a 3-mm spherical lenticulostriate aneurysm. The frontal lobe hematoma was evacuated to reveal the aneurysm, which was safely cauterized and resected by coagulating and dividing the lenticulostriate parent vessel. The procedure was carried out with neuronavigation guidance and intraoperative neuromonitoring. The patient was discharged with no neurologic deficits. The second patient was a 53-year-old woman who presented with subarachnoid and intracerebral hemorrhage. Computed tomography angiogram showed a 2-mm saccular MCA aneurysm. Emergency left decompressive hemicraniectomy and hematoma evacuation were performed. The aneurysm, arising from a small collateral type vessel, was safely clipped without complications. Postoperative angiography revealed absence of the superior MCA trunk with a dense network of collateral vessels at the site of the clipped aneurysm. The patient recovered well and was ambulating independently 6 months postoperatively. No rebleeding occurred in the 2 patients.
Our experience suggests that patients with MCA occlusion can harbor associated aneurysms related to flow through collateral vessels and can present with hemorrhage. Microsurgical repair of these aneurysms can be performed safely to prevent rebleeding.
Ruptured Basilar Tip Aneurysm in a Patient with Bilateral Internal Carotid Artery Occlusion Successfully Treated with Bilateral Superficial Temporal Artery-Middle Cerebral Artery Anastomoses: Case Report
2016, World Neurosurgery
Citation Excerpt :
Hemodynamic stress can lead to the formation of cerebral aneurysms.1-5 Stenosis or occlusion of intracranial or extracranial arteries can result in the development of a collateral blood network, which may increase the hemodynamic stress and subsequent aneurysm formation.5,6 We report a case of a ruptured basilar tip aneurysm in an adult with bilateral internal carotid artery (ICA) occlusion.
Some ruptured intracranial aneurysms concomitant with major artery occlusion may not be amenable for standard clipping or coiling due to the specific hemodynamic conditions. The long-term effect of flow reduction therapy for such aneurysms is largely unknown.
A 45-year-old woman presented with subarachnoid hemorrhage due to a ruptured basilar tip aneurysm. Angiography revealed that the bilateral internal carotid arteries were hypoplastic and that the anterior circulation was fed by the collateral flow through the bilateral posterior communicating arteries and the posterior choroidal arteries. Endovascular treatment failed because it was extremely difficult to secure the catheter at the neck of the aneurysm and insert the coil safely because it was a small aneurysm with a wide neck. In addition, direct clipping was risky due to the location and projection of the aneurysm. We therefore adopted the 2-stage bilateral superficial temporal artery–middle cerebral artery bypass to alleviate the vascular demand on the anterior circulation, with the aim of reducing the hemodynamic stress to the basilar bifurcation. The patient had no rebleeding for 4 years after surgery, with gradual shrinkage of the aneurysm.
All the possible treatment options should be carefully assessed because the long-term effect of the flow alternation method to prevent rebleeding has not been proved. However, our case suggests that the strategy of reducing the hemodynamic stress at the parent artery may be effective even in selected cases of ruptured aneurysms for which standard clipping or coiling is not feasible.

View all citing articles on Scopus

View full text

VascularSpontaneous middle cerebral artery occlusion leading to moyamoya phenomenon and aneurysm formation on collateral arteries

Abstract

Section snippets

Case report

Discussion

Surg Neurol

Surg Neurol

Middle cerebral artery occlusion with moyamoya phenomenon. Second reportpathological study of an autopsy case

Neurol Surg (Japan)

Moyamoya-like disease associated with a lenticulostriate region aneurysm

J Neurosurg

Spontaneous middle cerebral artery occlusion with moyamoya-like vessels associated with contralateral middle cerebral artery aneurysm; a case report

Neurol Surg (Japan)

Vascular
Spontaneous middle cerebral artery occlusion leading to moyamoya phenomenon and aneurysm formation on collateral arteries