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.     

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.     

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.     

Peripheral aneurysms of the lateral posterior choroidal artery: clinical presentation and endovascular treatment: report of two cases

OBJECTIVE AND IMPORTANCE: To describe the clinical presentation and endovascular management of peripheral aneurysms of the lateral posterior choroidal artery. Aneurysms in this location are exceptionally rare and optimal treatment may be difficult. CLINICAL PRESENTATION: Two patients with peripheral aneurysms of the distal portion of the lateral posterior choroidal artery presented with headaches from extensive intraventricular hemorrhage. INTERVENTION: Endovascular surgical therapy by use of superselective n-butylcyanoacrylate embolization of the aneurysm and adjacent distal parent artery was successful in both patients. CONCLUSION: Patients with peripheral aneurysms of the lateral posterior choroidal artery usually present with intraventricular hemorrhage. They may be difficult to treat by open surgical techniques owing to their intraventricular location and the frequent inability to preserve the parent artery by aneurysm clipping. Instead, it is typical that either proximal parent artery occlusion or aneurysm trapping must be used. An equivalent endovascular surgical technique may be an attractive alternative method of management.     

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.     

Two adult cases of moyamoya disease with intraventricular hemorrhage--angiographic evaluation of periventricular vascular anatomy

In order to show the vascular anatomy in periventricular region, two adult cases of moyamoya disease with intracerebral hemorrhage extending into lateral ventricle were presented. And we discussed the relation between the vascular anatomy and the form of hemorrhage in moyamoya disease. The first case was a 46-year-old woman admitted for nausea and vomiting following headache for four days. CT scan revealed a high density area in the upper portion of body of right lateral ventricle showing intracerebral hemorrhage which extended into the lateral ventricle. On angiogram, typical moyamoya disease was noted. The ventriculofugal arteries from the posterior lateral choroidal artery was detected which indicated developed ventriculofugal perfusion. The second case was a 52-year-old man who was hospitalized sudden onset of headache, vomiting and consciousness disturbance. CT scan revealed a high density area suggesting of hemorrhage in the head of right caudate nucleus. It perforated into the right anterior horn of lateral ventricle with spreading over the other ventricles. We observed similar findings on the angiograms to the first case. In this case, however, the lateral striate arteries were involved instead of the posterior lateral choroidal artery. It should be pointed out that, in both cases, the area of hemorrhage in CT scan coincided with the area of developed ventriculofugal perfusion in angiograms.     

A ruptured choroidal artery aneurysm of the anterior inferior cerebellar artery obliterated via the endovascular approach: case report

Intraventricular aneurysms associated with fourth ventricular hemorrhage are rare. A case of a ruptured aneurysm in a choroidal branch of the right anterior inferior cerebellar artery (AICA) is reported here. A 56-year-old man presented with sudden onset of vertigo and nausea. CT scan showed an intraventricular hemorrhage within the fourth ventricle. Cerebellar angiography showed an aneurysm at the choroidal artery branching from the right AICA. The patient rejected both general anesthesia and craniotomy, so endovascular embolization under local anesthesia was performed using Guglielmi detachable coils (GDCs) and a fibered platinum coil. The distal portion of the right AICA and the aneurysm were obliterated. His postoperative course was fairly satisfactory. He suffered from a minimal gait disturbance caused by truncal ataxia for several days after the operation. He was discharged from hospital without neurological deficit. There have been only a few articles about choroidal artery aneurysms. As treatment, direct surgery has been recommended in past cases, but endovascular embolization of the parent artery was successfully performed in this case. Not only direct surgery but also endovascular surgery may be regarded as the treatments of choice for intraventricular aneurysms, depending on the size of the parent artery.     

Moyamoya Disease of Adult Onset Brain Stem Haemorrhage Associated with Bilateral Occlusion of the Vertebral Arteries – Case Report

Summary An unusual and first case of moyamoya disease of adult onset brain stem haemorrhage associated with occlusion of both vertebral arteries is reported. A 30-year-old man suddenly suffered from dyspnea, dysphagia, and left-sided hemisensory disturbance. Computed tomography and magnetic resonance imaging revealed a fresh haematoma in the left medulla oblongata and various-sized old infarcts in both parietal lobes. Cerebral angiograms disclosed occlusion of the bilateral internal carotid arteries on both sides at their intracranial portion, accompanied with the developed basal moyamoya vessels. The right vertebral artery occluded at its V2-V3 segment, in which the posterior inferior cerebellar artery was opacified via the posterior spinal artery, and the basilar artery was filled from the anterior spinal artery. The left vertebral artery was also occluded at the craniovertebral junction (V4) with collateral flow. Only one case of moyamoya disease associated with bilateral occlusion of the vertebral artery has been reported previously, and a haemorrhage into the medulla oblongata in moyamoya disease has never been described.     

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.     

Ruptured intracranial aneurysms associated with moyamoya disease: three case reports

Three cases of ruptured intracranial aneurysm associated with moyamoya disease are presented. Endovascular treatments were performed successfully in two patients with major artery aneurysms. One patient with a collateral aneurysm was managed conservatively and follow-up angiography 1 year later demonstrated spontaneous disappearance of the aneurysm. Our experience suggests that although aneurysms associated with moyamoya disease show differences in evolution and location, endovascular treatment of major artery aneurysms is safe and effective, and peripheral aneurysms which cannot be directly accessed for surgery or endovascular embolization may be treated conservatively.     

NBCA embolization of a ruptured intraventricular distal anterior choroidal artery aneurysm in a patient with moyamoya disease

Occasionally an aneurysm is the cause of hemorrhage in patients with moyamoya disease (MMD). We present a case of a ruptured intraventricular distal anterior choroidal artery (AChA) aneurysm treated with n-butyl cyanoacrylic acid (nBCA) (Trufill nBCA Liquid Embolic, Codman Neurovascular, Raynham, Massachusetts, USA) embolization in a patient with MMD. There were no procedural complications and at 6 month follow-up she remained neurologically normal. Six month follow-up cerebral angiography showed no residual aneurysm. The endovascular route is an attractive option for many aneurysms associated with MMD as the lesions can be treated without disturbing the moyamoya collaterals. nBCA, delivered through a flow-guided microcatheter, is a good embolic agent choice when the lesion is distal on a small vessel and when distal parent artery occlusion can be tolerated. Intraventricular AChA aneurysms are well suited for this treatment strategy.     

Intracerebral hemorrhage after prophylactic revascularization in a patient with adult moyamoya disease

BACKGROUND: The effect of revascularization surgery for a patient with moyamoya disease remains controversial. CASE DESCRIPTION: A 60-year-old man presented with bleeding from asymptomatic moyamoya vessels 10 years after prophylactic revascularization surgery. Cerebral angiography 10 years after the surgery demonstrated that the bilateral direct anastomoses remained effective and a small aneurysm persisted in the anterior choroidal artery. The territories of perfusion through the anastomoses and the size of the aneurysm remained unchanged compared with the angiograms performed 10 years ago. CONCLUSIONS: Direct revascularization surgery may not always resolve microaneurysms in the moyamoya vessels and prevent rebleeding in patients with hemorrhagic moyamoya disease or bleeding in the nonaffected side.     

"Kissing" aneurysms of the internal carotid artery treated by coil embolization

A 62-year-old female complaining of sudden severe headache was referred to our stroke center in consultation. Computed tomography showed diffuse spread of thick subarachnoid hemorrhage to the basal cistern and both sylvian fissures, and digital subtraction angiography showed kissing aneurysms arising in the left lateral and occipital directions in the C(1) portion of the internal carotid artery (ICA). The anterior choroidal artery was situated between the two aneurysms, and another small branch originating from the dome of the distal aneurysm was confirmed as a duplicated middle cerebral artery (MCA). Endovascular treatment was successfully performed to spare the two vessels involved. This case of kissing aneurysms and ICA-duplicated MCA is very rare, and presents difficulties for both surgical and endovascular treatments.     

Hemangiopericytoma arising in the body of the lateral ventricle

A 65-year-old woman presented with a very rare hemangiopericytoma in the body of the lateral ventricle. Magnetic resonance imaging demonstrated a homogeneously enhancing mass lesion occupying the bilateral medial portions of the body of the lateral ventricle. Cerebral angiography disclosed a vascular-rich tumor, fed mainly by the left lateral posterior choroidal artery. After devascularization of the feeding vessel by endovascular coiling, the patient underwent complete surgical excision of the tumor via an anterior transcallosal approach, followed by radiation therapy, and has thus far been disease-free for 5 years. The present patient represents the first reported case of hemangiopericytoma arising in the body of the lateral ventricle. In this location of hemangiopericytoma, preoperative embolization could provide a promising option in terms of reducing the intraoperative blood loss and achieving total tumor extirpation with minimum damage to the surrounding structures. Since preoperative identification of hemangiopericytomas confers therapeutic advantages, it is important to be aware that they can occur at unusual ventricular sites.     

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.     

Unilateral development of moyamoya vessels after contralateral cervical internal carotid artery occlusion. Case report]

A 41-year-old female suffered transient ischemic attack. Cerebral angiography revealed occlusion of the left internal carotid artery at the cervical portion and collateral pathways consisting of transpial anastomosis and parenchymal anastomosis from the posterior circulation. Five years later, the second angiography was carried out. Left carotid angiography revealed appearance of transdural anastomosis from the middle meningeal artery and the anterior ethmoidal artery. Right carotid angiography revealed severe stenosis at the carotid fork with moyamoya vessels. Bilateral encephalomyo-arterio-synangiosis was performed for revascularization. Postoperative bilateral carotid angiograms revealed good neovascularization on both sides. In typical moyamoya disease, occlusive change of the carotid fork with moyamoya vessels appeared symmetrically and simultaneously on both sides. Although this case is not a typical moyamoya disease, its pathogenesis is quite similar to that of moyamoya disease.     

Intracranial ruptured aneurysm accompanying moyamoya phenomenon

Summary Three cases of moyamoya phenomenon associated with an aneurysm in the periphery of a lateral ventricle presenting with intracranial haemorrhage are reported.In Case 1 the aneurysm was located in the right basal ganglia, and the patient improved under conservative management. The aneurysm had increased in size on the second angiogram, and disappeared on the third angiogram obtained nine months after the attack. In Case 2 the aneurysm was located in the peripheral portion of the left posterior cerebral artery, and was surgically excised. Pathological examination on surgical material revealed collagenous tissue and laminae only in part of the aneurysmal wall. In Case 3 the aneurysm was located in the peripheral portion of the right anterior choroidal artery. This patient died, and the autopsy revealed an angiomatous lesion in the choroid plexus of the right lateral ventricle, but the aneurysm itself could not be identified.These findings indicate that an aneurysm has formed in a portion of a weakened vascular group represented by an abnormally dilated collateral pathway, and that the most likely reason for this would be haemodynamic stress.     

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.     

A case of posterior cerebral artery aneurysm associated with idiopathic bilateral internal carotid artery occlusion: case report

BACKGROUND: Aneurysms of the posterior circulation are challenging lesions to neurosurgeons, despite improvements in microsurgical techniques and advances in skull base approaches. We present a rare case of a posterior cerebral artery (PCA)-posterior communicating artery (PcomA) junction aneurysm associated with bilateral internal carotid artery (ICA) occlusion successfully treated with an endovascular procedure. CASE DESCRIPTION: A 57-year-old female presented with sudden onset of severe headache and loss of consciousness. CT scan showed diffuse subarachnoid hemorrhage and acute hydrocephalus. The patient developed severe neurogenic pulmonary edema and shock. Although her neurogenic pulmonary edema did not resolve, she recovered from shock. However, her general condition was so critical and her vital signs so unstable, that direct surgery under general anesthesia was considered too risky. A cerebral angiogram showed complete occlusion of both internal carotid arteries without any Moyamoya vessels. A saccular aneurysm located at the right PCA-PcomA junction was seen. To obliterate the aneurysm and prevent rerupture, the patient underwent coil embolization via an endovascular approach under sedation with local anesthesia. The balloon remodeling technique was useful to prevent occlusion of parent arteries. Finally, four interlocking detachable coils (IDC) with a total length of 44 cm were used to completely obliterate the aneurysm using the balloon remodeling technique. The patient made a full recovery after treatment and the aneurysm remained obliterated 2 years after coil embolization. CONCLUSIONS: We emphasize the advantages of the endovascular approach for the patient in critical condition. We believe that this is the first report of a PCA-Pcom junction aneurysm associated with bilateral ICA occlusion without moyamoya disease.     

Moyamoya disease associated with bilateral occlusion of the vertebral artery

An unusual case of moyamoya disease associated with bilateral occlusion of the vertebral artery is reported. This case shows several embryologic collateral channels, which are typical of moyamoya disease, around the brainstem, that is, a transdural anastomosis between the superior cerebellar artery and the posterior meningeal artery, a leptomeningeal anastomosis between the superior cerebellar artery and the posterior inferior cerebellar artery, and moyamoyalike collateral vessels via primitive lateral anastomotic channels of Padget. Bilateral encephalomyosyn-angiosis (temporal muscle graft) were performed. Post-operatively, no ischemic attacks have developed for 6 years.