Endovascular treatment of a ruptured aneurysm associated with unilateral moyamoya disease

We report a case presenting with subarachnoid hemorrhage due to a ruptured aneurysm associated with unilateral moyamoya disease. The patient was a 29-year-old woman exhibiting sudden onset headache. Computed tomography revealed subarachnoid hemorrhage around the brain stem. Cerebral angiography showed a saccular aneurysm at the junction of the left P1 portion of the posterior cerebral artery and its perforator. The right internal carotid artery was occluded at the terminal portion, and the right middle cerebral artery territory was perfused anterogradely via abnormal moyamoya vessels in the basal ganglia. The aneurysm was completely embolized with preservation of the perforator. We suggest that as compared to surgical clipping by craniotomy, endovascular therapy is safe and effective for treatment of intracranial aneurysms associated with moyamoya disease.     

Surgical management of a ruptured posterior choroidal intraventricular aneurysm associated with moyamoya disease using frameless stereotaxy: case report and review of the literature

OBJECTIVE AND IMPORTANCE: Prevention of rebleeding is the most important aspect of the management of hemorrhagic moyamoya disease, because rebleeding causes significant morbidity and mortality. CLINICAL PRESENTATION: A 26-year-old male patient with a history of moyamoya disease since the age of 3 years and multiple strokes was in a semicomatose state at presentation. He was found to have intraventricular and periventricular hemorrhages abutting the atrium of the right ventricle. His hospital course was complicated by a second hemorrhage. Both bleeding events were believed to be secondary to a ruptured right lateral posterior choroidal aneurysm. INTERVENTION: The aneurysm was excised and revealed histopathology consistent with a true saccular aneurysm. Frameless stereotactic guidance was used during surgery to minimize damage to collateral vessels and to shorten the surgical corridor. CONCLUSION: The management of hemorrhagic moyamoya disease should be modified based on the source of hemorrhage and its relation to a specifically located aneurysm. In the case of aneurysms arising from the choroidal artery, the general belief is that most of these represent pseudoaneurysms and have a tendency to regress spontaneously. Because of the rebleeding risk, we recommend early intervention in treating ruptured intracranial aneurysms using the least invasive surgical techniques.     

Intracerebral hemorrhage in a patient with moyamoya phenomenon caused by tuberculous arteritis: a case report]

A case of intracerebral hemorrhage which probably had been caused by a rupture of abnormal "moyamoya vessels" due to tuberculous arteritis was reported. A 42-year-old female had a history of tuberculous meningitis at the age of 2 years and suffered from a sudden onset of severe headache in January of 1998. CT scan disclosed a medium-sized intracerebral hematoma in the left frontal base and many calcifications in the basal cistern. Subsequent angiography demonstrated high-grade stenosis in the terminal portion of the right internal carotid artery and near-by "moyamoya vessels". No surgery was performed on the patient. A second angiography was carried out two months later and it newly disclosed ophthalmic artery-feeding "moyamoya vessels" in the place where the intracerebral hematoma had been located. This led us to conclude that a rupture of "moyamoya vessels" was the cause of the intracerebral hemorrhage and "moyamoya vessels" were not visible in the first angiograms because they had been compressed by the hematoma. Although cerebral infarction is common in tuberculous arteritis, cerebral hemorrhage is uncommon. The pathogenesis of cerebral hemorrhage due to tuberculous arteritis and its difference from that of hemorrhage caused by moyamoya disease is discussed.     

Aneurysm of the lenticulostriate artery--report of four cases.

Four rare cases of aneurysm of the lenticulostriate artery (LSA) are presented. LSA aneurysms were located at the origin in three patients and distally in one. Two cases were of multiple aneurysms, one was associated with hypertensive intracerebral hematoma (putaminal hemorrhage), and the other with moyamoya disease. Two patients were successfully treated by microsurgical procedures. The occurrence of LSA aneurysm suggests that aneurysm formation and growth are accentuated by hemodynamic alteration and stress.     

A case of rete mirabile with congenital dysplasia of bilateral internal carotid arteries

We report a case of dysplasia of the congenital bilateral internal carotid arteries with the rete mirabile. The rete mirabile is not usually seen in the course of human growth, but it is a common finding in other mammals. Accordingly, some investigators have thought that the rete mirabile is "developmental shift". Our case has dysplasia of the bilateral internal carotid arteries (one is aplasia and the other is hypoplasia), but the patient had suffered from no ischemic symptom because her brain had been sufficiently fed by each of the rete mirabile. Angiographically, the frequency of the rete mirabile formation is about 1/10,000. There were 20 cases reported until 1997 (including our case). There were 5 cases (27.8%) with ischemic symptoms in spite of internal carotid artery dysplasia, 2 cases (11.1%) with intracerebral hemorrhage, 6 cases (33.3%) with subarachnoid hemorrhage (there were only two cases with aneurysm) and 5 cases without symptoms. We have tried to class the rete mirabile by angiographical findings. One is the M type finding resembling moyamoya vessels in stages 3 & 4 of moyamoya disease, and the other is the N type finding resembling a nidus of arteriovenous malformation. M type occurred in younger patients more often than N type, so M type may be the previous stage of N type.     

Intracranial aneurysm associated with moyamoya disease in childhood

We report a 6-year-old girl with an intracranial aneurysm associated with moyamoya disease. The patient did not have a subarachnoid hemorrhage but had an ischemic attack. The aneurysm, located on the proximal portion of the lateral posterior choroidal artery, disappeared after external carotid-internal carotid anastomosis. The characteristics of the aneurysms associated with moyamoya disease are briefly reviewed. We emphasize that the treatment of choice in moyamoya disease is cerebral revascularization, because it reduces the increased blood flow through the moyamoya vessels, basilar artery and other uninvolved cerebral arteries, the sites where the aneurysms in this disease frequently develop, as the blood flow through the external carotid system is increased by such an operation.     

Distal anterior choroidal artery aneurysm in a patient with moyamoya disease: case report

OBJECTIVE AND IMPORTANCE: Distal anterior choroidal artery (AChA) aneurysms in moyamoya disease are rare, with few surgically verified reported cases. CLINICAL PRESENTATION: We report a rare case of distal AChA aneurysm associated with moyamoya disease in a 48-year-old man who presented with vomiting and severe headache. Computed tomographic scans revealed an intracerebral hematoma in the right temporoparietal lobe and a diffuse intraventricular hemorrhage. INTERVENTION: The hematoma was removed via computed tomography-guided stereotactic aspiration and ventricular drainage. Cerebral angiography showed a saccular aneurysm located at the distal branch of the right AChA. By means of magnetic resonance imaging, a small signal void lesion was detected in the periventricular area lateral to the trigone of the right lateral ventricle. The aneurysm was accurately accessed via a parietal cortical incision by use of magnetic resonance imaging-guided stereotactic localization. The aneurysm was successfully resected after undergoing trapping of the parent artery, and when the patient was discharged, he had no evidence of neurological deficit. The aneurysm was histologically verified to be a true aneurysm. CONCLUSION: Direct surgery should be considered in cases of ruptured distal AChA aneurysms located in the periventricular or intraventricular regions.     

Moyamoya disease associated with persistent primitive trigeminal artery--a case report and review of literature

A case of moyamoya disease associated with the persistent primitive trigeminal artery (PTA) is reported. 5 such cases reported in literature are reviewed. A 16-year-old female patient was admitted to our clinic, having complained of right hemiparesis for 8 years. CT scan revealed multiple low density areas in the left frontal, temporal and parietal lobes. In left carotid angiogram, the internal carotid artery was occluded at the C3 portion and basal moyamoya (stage IV) was found. In right carotid angiogram, the internal carotid artery was occluded at the C3 portion and basal moyamoya (stage V) was found. Furthermore the persistent primitive trigeminal artery was seen with marked moyamoya vessels from the posterior cerebral artery. In vertebral angiogram, the posterior cerebral artery was seen bilaterally with moyamoya vessels. In 133Xe-rCBF study, compared with the mean flow in each hemisphere, the flow decreased at the temporal lobe, but, in contrast, increased at the parieto-occipital lobes. Reviewing the literature, the following conclusions are suggested. 1. Persistent primitive trigeminal artery will promote the stage of moyamoya disease. 2. It will protect the brain from hemorrhage and/or ischemia in moyamoya disease.     

Moyamoya disease associated with aneurysm

Five cases of moyamoya disease associated with aneurysm are reported. In three cases, the aneurysms were located at the peripheral portion of the posterior choroidal artery, and in two at the basilar artery. Based on these cases, the symptoms and mechanisms of formation of aneurysms in moyamoya disease are discussed.     

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

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.     

A case of moyamoya disease associated with a peripheral artery aneurysm of the thalamus

The authors report a case of moyamoya disease associated with a peripheral artery aneurysm in the thalamus of a 54-year-old woman. Plain CT scans revealed the right thalamic hemorrhage with ventricular penetration. Cerebral angiography demonstrated the characteristic appearance of moyamoya disease with a peripheral artery aneurysm in the territory of the right medial posterior choroidal artery. This aneurysm ruptured three times during 8 days since onset and the thalamic hemorrhage became larger on each occasion. Although the clinical symptoms and signs and plain CT scans disclosed thalamic hemorrhage, it was difficult to explain the cause of the hemorrhage. The authors emphasize that cerebral angiography and enhanced CT scans are quite necessary to clarify the genesis of cerebrovascular disease.     

Familial cerebral aneurysms in three families, with two sibling pairs

The authors describe two pairs of siblings and a mother-son with cerebral aneurysms and the characteristics of familial intracranial aneurysms are briefly discussed. Family 1: A 54-year-old hypertensive woman (case 1) developed subarachnoid hemorrhage and a saccular aneurysm at the proximal portion of the left anterior cerebral artery was demonstrated on the angiogram. An azygos anterior cerebral artery was found as an associated anomaly. A 53-year-old hypertensive woman (case 2), a younger sister of case 1, suffered from subarachnoid hemorrhage and a saccular aneurysm at the distal portion of the right anterior cerebral artery was found on the angiogram. Vertebral angiogram showed bilateral fenestration of the extracranial vertebral arteries as a coincidental anomalies. Neck clipping for the aneurysms were successfully done in these two cases. Family 2: A 52-year-old hypertensive woman (case 3) suffered from subarachnoid hemorrhage and vertebral angiogram demonstrated a saccular aneurysm at the distal portion of the right posterior inferior cerebellar artery. The aneurysm was re-bled before surgical intervention and she died five days after admission. A 65-year-old hypertensive woman (case 4), an elder sister of case 3, was admitted with subarachnoid hemorrhage. Vertebral angiogram showed a saccular aneurysm on the proximal portion of the left posterior inferior cerebellar artery at the junction of the vertebral artery and a massive extravasation of the contrast medium from the aneurysm. The patient died three days after the onset before surgical intervention.(ABSTRACT TRUNCATED AT 250 WORDS)     

The minor leak preceding subarachnoid hemorrhage

Thirty-four of 87 consecutive patients with subarachnoid hemorrhage from a cerebral aneurysm had a premonitory minor leak. There were 12 men and 22 women, aged 25 to 73 years (mean 44.4 years). Twenty-two had a small and 12 had a large aneurysm located on the internal carotid artery (17 cases), anterior communicating artery (10 cases), middle cerebral artery (five cases), and pericallosal artery (two cases). Fifty-two percent of patients with a minor leak from an internal carotid artery aneurysm had ipsilateral, hemicranial, hemifacial, or periorbital pain. Half of the patients initially saw a physician, but in no case was the correct diagnosis made. Twenty-five patients had a major rupture within 24 hours to 4 weeks after findings suggesting a minor leak, with a mortality rate of 53%. Nine other patients were diagnosed by lumbar puncture or computerized tomography (CT) scanning after initial misdiagnosis and were operated on, without mortality, before a major rupture could occur. The CT scans were negative in 55% of patients with a minor leak, but lumbar puncture, when performed, was always positive. A minor leak prior to major aneurysmal rupture is a common occurrence and, if unrecognized, is associated with a high mortality. Computerized tomography scanning is unreliable in diagnosing this event, and lumbar puncture is the examination of choice once intracranial hypertension has been ruled out.     

Paradoxical association of moyamoya syndrome with large middle cerebral artery aneurysm and subarachnoid hemorrhage. Case report

A 69-year-old woman was admitted to our hospital because of fluctuating dysarthria during the past 2 months. Magnetic resonance imaging revealed old cerebral infarction of the left cerebral hemisphere with acute subarachnoid hemorrhage in the left sylvian fissure. Cerebral angiography showed a large saccular aneurysm, 14 mm in diameter, at the bifurcation of the left middle cerebral artery (MCA) in association with moyamoya vasculopathy with atherosclerosis, including steno-occlusive changes at the bilateral terminal internal carotid arteries and abnormal net-like vessels at the base of the brain. She underwent microsurgical neck clipping of the large aneurysm followed by superficial temporal artery-MCA anastomosis without complication. Intraoperative findings showed no evidence of aneurysm rupture, suggesting that the subarachnoid hemorrhage was due to the intrinsic pathology of moyamoya vasculopathy. The postoperative course was uneventful, and the patient was discharged without neurological deficit. Association of moyamoya syndrome with large MCA aneurysm is extremely rare, and formation of large aneurysm at the vascular territory of an occluded vessel is apparently unique.     

Cranial burr hole for revascularization in moyamoya disease

Currently, superficial temporal artery-middle cerebral artery (MCA) anastomosis, encephalomyosynangiosis (EMS), and encephalo-duro-arterio-synangiosis are used to treat moyamoya disease and are reported to effectively improve ischemic symptoms. All are methods of reversing the flow of blood from the external carotid artery system into the cortical branches of the MCA. As moyamoya disease advances, these operations alone will predictably not correct the deterioration in blood flow in the territory of the anterior cerebral artery. It was noted in a case of moyamoya disease with intraventricular hemorrhage that a burr hole, made in the frontal region for drainage purposes, induced marked neovascularization. Since then, similar frontal burr holes have been made in five juvenile cases of moyamoya disease; this procedure involved making a burr hole in both frontal bones and incising both the dura and the arachnoid membrane. In two cases a frontal burr hole in both frontal bones and incising both the dura and the arachnoid membrane. In two cases a frontal burr hole was placed simultaneously with EMS, and in the others the frontal burr hole was made following EMS. The clinical symptoms improved after the frontal burr hole was made, and dynamic computerized tomography revealed improved circulation in the frontal regions. Together with conventional surgical therapy for juvenile cases of moyamoya disease, this operation is considered beneficial both to the circulation in the frontal region and for the protection of frontal brain function.     

Moyamoya disease with intraventricular hemorrhage due to rupture of lateral posterior choroidal artery aneurysm: case report

Intracranial aneurysms associated with moyamoya disease are often reviewed. Aneurysms not around the circle of Willis but on the collateral vessels are rare. A 71-year-old woman presented with sudden onset of severe headache and vomiting. CT scan showed an intraventricular hemorrhage from bilateral lateral ventricle to fourth ventricle. Cerebral angiography showed the occlusion of the terminal portion of the bilateral internal carotid artery with moyamoya vessels and three aneurysms at the distal portion of the left lateral posterior choroidal artery. Clinical symptoms improved day by day, but 17 days later, due to rebleeding of the aneurysms the patient fell into coma. The day after rebleeding, endovascular embolization was performed using liquid particle, and the left lateral posterior choroidal artery and the aneurysm were occluded. Brain infarction with massive brain edema of the left cerebral hemisphere resulted in the patient's death. The management of the aneurysms in the basal ganglia and on the collateral vessels associated with moyamoya disease is controversial. We suggest positive intervention during the acute stage for the peripheral artery aneurysms taking endovascular embolization into consideration to prevent rupture or rebleeding.     

Distal anterior choroidal artery aneurysm following iatrogenic posterior cerebral artery occlusion

Aneurysms of the anterior choroidal artery are uncommon and account for only 2–5% of all intracranial aneurysms. Distal anterior choroidal artery aneurysms are rare and the pathogenesis of this aneurysm may be different from typical proximal ones. We describe an unusual case of ruptured de novo distal anterior choroidal artery aneurysm in a 55 year-old man who had previously undergone endovascular proximal occlusion for a fusiform aneurysm of the ipsilateral posterior cerebral artery (PCA). A comprehensive review of literature using Medline, PubMed and all related journals was also performed. Only 34 reported distal AchoA aneurysms were found: 16 associated with moyamoya disease, 10 idiopathic, two with atherosclerosis, two with arteriovenous malformation, two dissecting, one infectious and one due to trauma. It is probable that the increased hemodynamic stress within the AchoA contributed to the formation and rupture of the reported aneurysm. This late complication needs to be considered when large intracranial vessels are iatrogenically occluded in the management of complex intracranial aneurysms.     

Endovascular coil embolization of a ruptured distal anterior choroidal artery aneurysm associated with ipsilateral middle cerebral artery occlusion--case report

An 84-year-old woman with a history of hypertension and a brain infarction presented with a rare distal anterior choroidal artery (AChoA) aneurysm not associated with moyamoya disease manifesting as sudden onset of headache caused by intraventricular hemorrhage. Digital subtraction angiography revealed a peripheral aneurysm in the left AChoA located distal to the plexal point and steno-occlusive changes of the proximal left middle cerebral artery (MCA) and the left posterior cerebral artery (PCA). Collateral arterial channels to the left MCA and left PCA territories were observed along the left AChoA. No neuroimaging findings were compatible with moyamoya disease. Since the aneurysm did not shrink at 2 months after the onset, endovascular treatment was indicated. Under local anesthesia, a microcatheter was placed into the AChoA proximal to the aneurysm with the aid of a 0.008-inch microguidewire. After a provocation test, three detachable platinum coils were delivered into the aneurysm and the parent artery. Complete obliteration of the aneurysm was achieved without additional neurological sequelae. Successful treatment of a ruptured distal AChoA aneurysm associated with atherosclerotic changes of the intracranial arteries was achieved using a meticulous endovascular technique.     

Giant middle cerebral artery aneurysm with parent artery occlusion--case report.

A 54-year-old female was admitted with consciousness disturbance and right hemiparesis. Computed tomographic (CT) scans and angiograms revealed diffuse subarachnoid hemorrhage, a partially thrombosed, giant middle cerebral artery aneurysm (5 x 5 x 4 cm), and occlusion of the parent artery at the aneurysm site. Despite conservative treatment, a generalized convulsion occurred. Emergency CT scans revealed irregular enlargement of the left temporal high-density mass and severe mass effect due to cerebral infarction. Barbiturate coma therapy was administered, but she did not recover and died 9 days after admission. Only two cases of ruptured aneurysm with simultaneous occlusion of the major cerebral vessels have been reported, both with poor outcome. In this case, the mechanism of parent artery occlusion is unclear, but thrombus protrusion from the giant aneurysm into the parent artery may have been involved.     

Radiation-Induced Aneurysm and Moyamoya Vessels Presenting with Subarachnoid Haemorrhage

A rare case of a ruptured internal carotid artery (ICA) aneurysm, with ICA stenosis and moyamoya vessels, was diagnosed in a young female patient after radiotherapy for optic glioma. Retrospective analysis revealed that an extraordinarily high dose of radiation (110 Gy) had been delivered to the area around the ICA in two therapeutic sessions. Clipping and wrapping of the aneurysm were performed and the patient made a good recovery. A review of the literature suggested that rupture of radiation-induced aneurysms is a rare event, but may be fatal. Considerable care must therefore be taken when delivering high-dose radiation to cerebral arteries. We suggest that the incidence of radiation-induced moyamoya vessels is higher in younger patients.