Arteriovenous malformations of the lateral ventricle

Nine cases with arteriovenous malformations (AVM's) predominantly involving the lateral ventricle are presented. All the AVM's were small, but caused intraventricular hemorrhage in eight cases. Only two patients had an intracerebral hemorrhage large enough to warrant evacuation. Eight patients were under the age of 40 years at the onset of their disease. Computerized tomography demonstrated intraventricular hemorrhage in eight patients, and after intravenous administration of contrast medium a small area of enhancement with dilated subependymal draining veins was seen in seven. The lateral ventricles were of normal size in seven cases, and only two patients required a shunting procedure. Angiography demonstrated that the lesion was an AVM in eight patients, and did not visualize the lesion in the ninth. One patient suffered a recurrent intraventricular hemorrhage when the AVM was demonstrated, although repeated angiography had failed to disclose a vascular lesion at his first intraventricular hemorrhage 14 months before. All nine lesions were resected by microsurgical techniques, and the results were excellent in eight patients. Of four caudate lesions, three were resected through a frontal transcortical approach and the other was operated on through an anterior transcallosal approach; the results were excellent in three of these patients. Only one (Case 4) was left with neurological deficits; he had confusion and disorientation following a right frontal transcortical approach. Even in the dominant hemisphere, lesions in the head of the caudate nucleus could be safely resected by an anterior transcallosal approach. Two choroidal lesions located in the temporal horn and trigone on the dominant side were resected through a middle temporal gyrus approach, and three thalamic lesions through a posterior transcallosal approach, all with excellent results. In all cases the brain opening required was about the width of the retractor (maximum 2.0 cm, average 1.5 cm).     

A case of cavernous angioma at the septum pellucidum

A 35-year-old female presented with a cavernous angioma of the septum pellucidum manifesting as headache and nausea. Computed tomography and magnetic resonance imaging revealed a mass at the septum pellucidum with intraventricular hemorrhage and mild hydrocephalus. Digital subtraction angiography showed no abnormal finding and thallium-201 single photon emission computed tomography showed no uptake in the lesion. The preoperative diagnosis was intraventricular tumor, such as subependymoma. The lesion was completely resected through the transcallosal approach. The histological diagnosis was cavernous angioma. Cavernous angiomas are usually located in the cerebral parenchyma and rarely occur in the cerebral ventricles. In particular, cavernous angioma at the septum pellucidum is very rare. If magnetic resonance imaging does not clearly show the typical peripheral hypointense rim, neuroimaging diagnosis will be difficult. Surgical resection should be considered to confirm the histopathology, in particular if bleeding and hydrocephalus are present.     

Subependymoma in the lateral ventricle manifesting as intraventricular hemorrhage

A 32-year-old man presented with subependymoma in the lateral ventricle causing intraventricular hemorrhage and manifesting as severe headache and disturbance of consciousness. Computed tomography on admission showed a massive intraventricular hemorrhage and acute obstructive hydrocephalus. Cerebral angiography revealed no abnormal findings. Emergency external ventricular drainage was performed, and his neurological deficits gradually improved. Magnetic resonance imaging at 5 weeks after admission showed a tumor arising from the septum pellucidum or the floor of the right lateral ventricle, appearing as a mixed-intensity solid tumor, which was partially enhanced following gadolinium administration. The tumor had arisen from the septum pellucidum and was totally removed via an interhemispheric anterior transcallosal approach. Histological examination found typical subependymoma, with little vascularity. Intraventricular hemorrhage from cerebral neoplasms is usually due to highly vascular tumors. Since subependymomas are quite benign and show poor vascularity, intraventricular or subarachnoid hemorrhages are very rare, but do occasionally occur.     

Neurocytoma manifesting as intraventricular hemorrhage--case report

A 43-year-old man presented with a neurocytoma manifesting as severe headache and disturbance of consciousness. Computed tomography revealed intraventricular hemorrhage, and a small mass lesion with calcification on the wall of the left lateral ventricle. The lesion appeared as mixed intensity regions on both T(1)- and T(2)-weighted magnetic resonance imaging, and heterogeneous enhancement with gadolinium-diethylenetriaminepenta-acetic acid. Angiography showed the pooling sign near the calcification in the late venous phase. Neurologically, amnestic syndrome was demonstrated in the subacute phase. Gross total removal of the lesion was performed through a transcallosal approach. His transient memory disturbance resolved. The histological diagnosis was neurocytoma. Intraventricular hemorrhage is rare as the initial presentation of neurocytoma. Surgery should avoid fornix injury and the risk of permanent memory disturbance.     

A neurocytoma and an associated lenticulostriate artery aneurysm presenting with intraventricular hemorrhage: case report

OBJECTIVE AND IMPORTANCE: Hemorrhage associated with central neurocytoma has been described previously, but never in association with an aneurysm originating from a feeding artery. We present the first reported case of a central neurocytoma in a patient with intraventricular hemorrhage caused by rupture of an aneurysm on a lenticulostriate artery that supplied the tumor. CLINICAL PRESENTATION: A 35-year-old man who presented with an intraventricular hemorrhage underwent magnetic resonance imaging and cerebral angiography that disclosed a right lateral intraventricular mass and a 7-mm fusiform aneurysm from a lateral lenticulostriate branch of the right middle cerebral artery. INTERVENTION: The patient underwent a contralateral transcallosal exploration and resection of the tumor, with excision of the adjacent lenticulostriate artery aneurysm. Pathological review demonstrated that the tumor was a neurocytoma. The aneurysm was discrete from the tumor but occurred on a vessel that supplied the tumor. CONCLUSION: Previous reports have demonstrated that intraventricular neurocytoma may present with tumor hemorrhage. In this case, an aneurysm separate and distinct from the tumor was the bleeding culprit, and the aneurysm was on an artery that fed into the tumor. Any such aneurysm must be identified and treated appropriately for therapy to be complete.     

Choroid plexus arteriovenous malformation (AVM) in a newborn. Case report

We report here on an unusual case of AVM with intraventricular hemorrhage in a full-term newborn infant. Clinical and radiological findings are discussed in the light of the recent literature.     

Intraventricular meningiomas: a review of 16 cases with reference to the literature

BACKGROUND: Primary intraventricular meningiomas are notably rare with an incidence of 0.5 to 3% among all intracranial meningiomas. We present a series of 16 patients with histologically verified intraventricular meningiomas, the clinical and neuroradiological findings, and surgical results. METHODS: The charts of the patients including surgical records, discharge letters, histologic records, follow up records and imaging studies were analyzed retrospectively from 1978 to 2001. RESULTS: Collectively, 110 primary intraventricular tumors were surgically resected in our neurosurgical department from 1983 through 2001. There were 16 patients with primary intraventricular meningiomas. Thirteen (81.3%) of these tumors were located in the lateral ventricles, one (6.2%) in the third and two (12.5%) in the forth ventricle. Mean age was 47.1 years and there was no difference in incidence between the sexes. The most common presenting symptoms and signs were headache, mental change, and hemianopia. Other symptoms included gait ataxia, vertigo, hemiparesis, double vision, aphasia, and alexia/agraphia. On CT and MR studies most of the lateral ventricular meningiomas were located in the trigone (11 cases), two tumors originated at the level of foramen of Monro or in the frontal horn. The tumors were approached via a transcortical parieto-occipital (11 cases), transcallosal (3 cases), or median suboccipital (2 cases) route. Total removal was achieved in 15 out of 16 cases. There was no operative mortality. Clinical improvement was seen in symptoms like headaches, paresis, cerebellar signs and visual disturbances, whereas mental change showed less improvement. CONCLUSIONS: Although intraventricular meningiomas are quite rare, they represent an important differential diagnosis of intraventricular neoplasms. They can reach a substantial size by the time of diagnosis, yet total removal can be achieved in most cases.     

Oligodendroglioma of the lateral ventricle--report of 2 cases and a review

Oligodendrogliomas rarely grow primarily in the ventricles. When compared to more common hemispheric counterparts, such intraventricular oligodendrogliomas, or oligodendrogliomas of the midline group (Martin), present distinct clinical features, namely, (1) they occur in the younger age, (2) an interval between clinical onset and diagnosis or operation is shorter, and (3) initial symptoms are most often limited to those of increased intracranial pressure, although the patient may occasionally present mild organic dementia, callosal disconnection syndrome, and/or mild gait ataxia. Two cases of oligodendroglioma primarily involving the lateral ventricle are reported in female patients, aged 29 and 19, respectively, with sole complaints of an increased intracranial pressure. Magnetic resonance imaging proved to be most useful as a radiologic diagnostic procedure. The tumor was radically removed via a para-falcine transcallosal approach without causing any persistent neurological deficits. Merit and demerit of the approach are briefly discussed.     

Intraventricular meninigiomas: a report of 25 cases

In order to study the clinical characteristics of intraventricular meningiomas, we analyzed retrospectively 25 patients and reviewed the literature with regard to incidence, clinical manifestation, imaging features, preoperative diagnosis, surgical findings, and histopathological results. Intraventricular meningiomas are quite rare, but they represent an important differential diagnosis of intraventricular neoplasms. Computed tomography or magnetic resonance imaging enable a correct diagnosis of intraventricular meningiomas in most of the cases. The tumors often grow slowly to a substantial size before they become symptomatic. The operative route should be selected according to the tumors location. Out of the 24 lateral ventricular meningiomas in our series, 20 were resected via a posterior parieto-occipital transcortical approach, two were resected via a transcallosal approach, and another two tumors, located in the frontal horn and body of the lateral ventricle, were resected via a frontal approach. A median suboccipital craniotomy was performed for the fourth ventricular meningioma. The parieto-occipital route for lateral ventricular meningiomas is a safe surgical approach, which is not necessarily associated with postoperative visual deficits. Piecemeal removal of the tumor can be safely and easily performed and special attention should be paid to the choroidal vessels intraoperatively.     

Surgical approaches to intraventricular meningiomas of the trigone

A case of a large intraventricular meningioma of the trigone is described. The tumor was removed by the transcallosal route. The neuropsychological sequelae of this approach are discussed. The value of other surgical approaches for meningiomas of the trigone is reviewed. The transcallosal approach originally described by Kempe and Blaylock is highly recommended.     

Treatment of severe intraventricular hemorrhage by intraventricular infusion of urokinase

During the last three years, seven patients with severe intraventricular hemorrhage admitted to our clinic were treated with direct intraventricular infusion of urokinase. In each case, hemorrhage extended into the entire ventricular cavity and cast formation as well as an expansion of third and fourth ventricles were found. On the average, both the third and fourth ventricles became clear on the third day and the lateral ventricle on the ninth day after hemorrhage. Five of the seven patients showed good recovery or only moderate disability, and two died. Infection, convulsion, rebleeding, and peripheral or secondary hemorrhage due to the side effects of urokinase was not encountered during therapy.We conclude that this procedure can be applied effectively and safely in severe intraventricular hemorrhage.     

Isolated intraventricular hemorrhage due to a ruptured vertebral artery aneurysm

A rare case of ruptured aneurysm originating from the vertebral artery, and presenting an isolated clot in the fourth ventricle is reported. The patient underwent surgery via a right lateral suboccipital craniectomy on the third day. As the aneurysm was huge and partially thrombosed, trapping was performed and completed successfully. If isolated intraventricular hematoma was found without obvious parenchymal hemorrhage or subarachnoid clot, the diagnosis of vertebral artery aneurysm should be considered as a possibility in addition to posterior inferior cerebellar artery aneurysm. Complete vertebral angiography is indispensable for the recognition of this condition.     

Intrinsic third ventricular craniopharyngiomas: report on six cases and a review of the literature

BACKGROUND: Craniopharyngiomas constitute 2 to 4% of intracranial neoplasms. However, the purely intraventricular variety are rare. Their magnetic resonance imaging (MRI) characteristically shows an intact third ventricular floor, a patent suprasellar cistern, an intact pituitary stalk, and the absence of sellar abnormalities. METHODS: Between 1994 and June 2002, 6 patients with purely intraventricular craniopharyngioma were surgically managed. There were 4 cystic and 2 solid lesions. The surgical approaches utilized included a frontal, parasagittal, transcallosal approach with the third ventricle being accessed using either the transforaminal or subchoroidal approach (n = 3); pterional, transsylvian (n = 1), and bifrontal interhemispheric (n = 2) approaches in which the third ventricle was accessed via the lamina terminalis. A ventriculoperitoneal shunt was required for one of the 3 patients with hydrocephalus. RESULTS: Total excision was performed in 3 patients with cystic craniopharyngioma, while a small residual lesion was left adherent to the third ventricular floor in the others. There was one perioperative mortality because of septicemia. Two patients required thyroxine supplementation. Two patients developed transient and one other patient a sustained diabetes insipidus. The 2 patients with solid tumors received radiotherapy for the residual lesions. At follow up ranging from 8 to 36 months, neither tumor recurrence nor regrowth was observed in any of the patients. The symptoms of raised intracranial pressure, hypothalamic dysfunction or visual field defects had resolved. CONCLUSIONS: Intraventricular craniopharyngiomas occur in an older population and present mainly with raised intracranial pressure. Visual and endocrinologic imbalances are much less in these lesions compared to the suprasellar craniopharyngiomas. They mainly attach to the third ventricular floor. The surgical approaches to the third ventricle, along with radiotherapy and hormone supplementation, were successful in the management of these rare tumors.     

Endoscopic evacuation of intraventricular hematoma and third ventriculostomy

We described our experience of three cases treated with endoscopic evacuation of intraventricular hematoma and third ventriculostomy for a tight intraventricular hematoma associated with intracerebral hemorrhage. A steerable endoscope was introduced into the anterior horn of the lateral ventricle contralaterally to the intracerebral hemorrhage, through a 14 Fr. peel-away sheath. First, the hematoma in the lateral ventricle contralateral to the hemorrhage was evacuated by direct aspiration using a syringe connected to the operative channel of the endoscope, and evacuation of the hematoma was subsequently carried on the third ventricle, aqueduct and the fourth ventricle. After the evacuation of the intraventricular hematoma, third ventriculostomy was performed for acute obstructive hydrocephalus. Finally, the procedure was completed with septostomy and evacuation of the hematoma in the lateral ventricle ipsilateral to the hemorrhage. Sufficient evacuation of the hematoma was obtained in all cases and no major complications were encountered. We conclude that for patients with intraventricular hematoma associated with intracerebral hemorrhage endoscopic evacuation of intraventricular hematoma brings about sufficient removal of hematoma, reduction of hospitalization time and prevention of subsequent hydrocephalus.     

Tumours of the third ventricle

Summary Primary intraventricular tumors, including colloid cysts, choroid plexus papillomas, and ependymomas have their attachment within the IIIrd ventricle and stay within its confines. Secondary intraventricular tumours arise adjacent to the IIIrd ventricle, often in the suprasellar region, and include craniopharyngiomas, optic nerve gliomas, and meningiomas. Approaches to the ventricle include subfrontal, subtemporal, anterior transventricular, anterior and posterior transcallosal, and transtentorial routes. Memory problems have been noted with anterior dorsal methods, implicating damage to one or both anterior forniceal columns. Methods of opening the roof of the third ventricle must evaluate risk to the deep ventricular veins, the fornix, and the choroid plexus. The basal subfrontal method may be modified by opening the lamina terminalis or including a lateral approach from the pterional area. The application of these approaches in a series of 100 third ventricle tumours is analyzed.     

Pre-coronal, paramedian minicraniotomy: a minimal access approach for microsurgical, transcallosal, transforaminal removal of colloid cysts of the third ventricle

OBJECTIVE: Microsurgical excision of colloid cysts of the third ventricle is accomplished along the transcallosal or the transfrontal routes. In the transcallosal approach, venous tributaries of the superior sagittal sinus can often act as an impediment to entry into the interhemispheric fissure for accessing the corpus callosum. We propose a paramedian minicraniotomy anterior to the coronal suture for removing colloid cysts via the transcallosal approach as veins are relatively rare in this area. METHODS: A triangular minicraniotomy was designed with each side measuring 3 cm based on the midline in the pre-coronal area of the frontal bone on the right side. Nineteen cases of symptomatic colloid cysts of the third ventricle whose diagnoses were proven by CT and/or MRI were subjected to microsurgery in the period from June 2004 to May 2007. Following the minicraniotomy the cysts were removed utilizing the transcallosal transforaminal route. RESULTS: Venous tributaries crossing the interhemispheric fissure were seen in 2 patients and these could be avoided to access the corpus callosum. Complete excision could be achieved in all cases. All patients had a good outcome although one patient had transient left lower limb weakness. The mean operating time was 163 minutes, while the mean duration of stay in the intensive care unit and hospitalization were 1.35 days and 3.73 days, respectively. CONCLUSION: The pre-coronal, paramedian minicraniotomy is safe and effective for the total excision of colloid cysts of the third ventricle. As a minimal access approach, it needs only a short duration of postoperative hospitalized care.     

Subependymal giant cell astrocytoma associated with tuberous sclerosis exhibiting rapid regrowth after 17 years: a case report]

A 17-year-follow-up case of subependymal giant cell astrocytoma (SGCA) is reported. In 1979, when aged 28 years, the patient first presented obstructive hydrocephalus caused by a tumor in the right lateral ventricle close to the foramen of Monro. It was partially removed by a transcallosal approach. Pathological examinations showed gemistocytic astrocytoma or SGCA associated with tuberous sclerosis. A ventriculo-peritoneal shunt was carried out and 36Gy of radiation therapy was administered. Eight months later, the patient suffered from an intraventricular hemorrhage originating from SGCA, but he responded to conservative therapy. He was followed-up by CT scans over 17 years. In 1996, because of rapid regrowth of the tumor, total removal was performed by a transcortical approach via the right frontal horn. The pathological diagnosis was SGCA. The greater part of the recurrent tumor was composed of blood vessels. The tumor cells were grouped into two morphological types, large cells and spindle cells. We compared the tumor in 1996 with that in 1979, each revealing immunohistochemical stainability for glial fibrillary acidic protein (GFAP), neuron specific enolase (NSE) and S-100 protein (S-100). The large cells in 1979 were negative for GFAP, NSE and S-100, but were positive for NSE and S-100 in 1996. The spindle cells in 1979 were positive for GFAP, NSE and S-100, but were negative for GFAP in 1996. The pathological origin of SGCA remains a subject of controversy. These results suggest that the origin of SGCA could be variably differentiated cells like the germinal matrix cells.     

Treatment of severe intraventricular hemorrhage by intraventricular infusion of urokinase

Six patients with severe intraventricular hemorrhage were treated with direct intraventricular infusion of urokinase. In each case, hemorrhage extended into all ventricular chambers, and a cast formation and expansion of the third and fourth ventricles were found. Immediately after the therapy was started (within 7 days from onset of symptoms), reduction of intraventricular hematoma volume was observed on computerized tomography. On average, both the third and fourth ventricles became clear on the third day after hemorrhage; there was one exception, a case of ruptured aneurysm. Five of the six patients showed excellent or good outcome, although two developed delayed hydrocephalus. No infection or rebleeding was observed. The outcome in a retrospectively studied group of five patients not treated with urokinase is also reported. The authors conclude that this relatively easy method of treatment will greatly improve the prognosis of severe intraventricular hemorrhage.     

Intraventricular Malignant Triton Tumour

The authors present the clinical and pathological features of a malignant triton tumour (MTT) in the lateral ventricle with neurofibromatosis type 1. A 57 year-old man presented with disorientation and memory disturbance. A Computed tomographic scan and magnetic resonance imaging studies revealed an enhancing lesion in the left lateral ventricle. A parieto-occipital transcallosal approach was taken and resection of the lesion was performed. The operative findings suggested that the tumour arose from the perivascular nerves. The final pathological diagnosis was a MTT. This is the first case of an intraventricular MTT. Aggressive treatment including radical surgery combined with radiochemotherapy is recommended for a MTT of the central nervous system.     

Hypertensive primary intraventricular hemorrhage: a systematic review

Primary intraventricular hemorrhage (PIVH) is a special subtype of intraventricular hemorrhage (IVH) without a hemorrhagic parenchymal component. Different conditions may cause this uncommon hemorrhage including trauma, vascular anomalies, coagulation disorders, and others. Frequently, PIVH is associated with structural vascular anomalies such as aneurysms, arteriovenous malformations, and dural fistulas. Traditionally, hypertension has been considered a predisposing factor for PIVH. A wide variety of studies have been published describing patients with PIVH; however, studies describing exclusively patients with hypertensive PIVH are lacking in the literature. For this reason, the features of PIVH secondary to hypertension are not well described. The purpose of this study is to analyze and describe the characteristics of hypertensive PIVH. A PubMed and Scopus search adhering to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was performed to include studies reporting patients with hypertensive PIVH. The search yielded 19 articles reporting retrospective case series. The diagnosis of hypertensive PIVH should be established in patients meeting the following criteria: (a) elevation of blood pressure is observed at admission, (b) a cerebral angiography is negative for vascular anomalies, and (c) other causes of intracranial hemorrhage are ruled out. The prognosis is poorer in patients who present with low Glasgow Coma Score (GCS), old age, hydrocephalus, or more extensive intraventricular bleeding. The results of this study show that hypertension is the most common cause of PIVH, followed by hemorrhage caused by vascular anomalies. Hypertension may be a direct cause of PIVH, but also it may be a predisposing factor for bleeding in cases of an associated vascular anomaly.