Clinical Outcome of Nonfistulous Cerebral Varices: the Analysis of 39 Lesions

OBJECTIVE: Cerebral varices (CVs) without an arteriovenous shunt, so called nonfistulous CVs, are very rare, and their etiology and natural course are not well understood. The aim of this study is to evaluate the clinical outcomes of nonfistulous CVs by the analysis of 39 cases. METHODS: From 2000 to 2015, 22 patients with 39 nonfistulous CVs (≥5 mm) were found by searching the medical and radiologic records of our institute. Clinical data and radiological data including numbers, sizes and locations of CVs and associated anomalies were retrospectively collected and analyzed. Previously reported cases in literature were reviewed as well. RESULTS: The mean age of the patients was 21 years (range, 0–78 years). On average, 1.8±1.2 CVs were found per patient. CVs were categorized as either fusiform or saccular depending on their shapes. Two patients had saccular type CVs, seventeen patients had fusiform types, and three patients had both fusiform and saccular CVs. Eight patients had associated compromise of the vein of Galen and the straight sinus. Four of those patients had sinus pericranii, as well. Five patients had CVs that were distal draining veins of large developmental venous anomalies. One patient had associated migration anomaly, and two patients had Sturge- Weber syndrome. Six patients with an isolated cerebral varix were observed. Of the 39 CVs in 22 patients, 20 lesions in 14 patients were followed up in outpatient clinics with imaging studies. The average follow-up duration was 6.6 years. During this period, no neurological events occurred, and all the lesions were managed conservatively. CONCLUSION: Nonfistulous CVs seemed to be asymptomatic in most cases and remained clinically silent. Hence, we suggest conservative management.

Clinical Outcome of Nonfistulous Cerebral Varices: the Analysis of 39 Lesions

OBJECTIVE: Cerebral varices (CVs) without an arteriovenous shunt, so called nonfistulous CVs, are very rare, and their etiology and natural course are not well understood. The aim of this study is to evaluate the clinical outcomes of nonfistulous CVs by the analysis of 39 cases.METHODS: From 2000 to 2015, 22 patients with 39 nonfistulous CVs (≥5 mm) were found by searching the medical and radiologic records of our institute. Clinical data and radiological data including numbers, sizes and locations of CVs and associated anomalies were retrospectively collected and analyzed. Previously reported cases in literature were reviewed as well.RESULTS: The mean age of the patients was 21 years (range, 0–78 years). On average, 1.8±1.2 CVs were found per patient. CVs were categorized as either fusiform or saccular depending on their shapes. Two patients had saccular type CVs, seventeen patients had fusiform types, and three patients had both fusiform and saccular CVs. Eight patients had associated compromise of the vein of Galen and the straight sinus. Four of those patients had sinus pericranii, as well. Five patients had CVs that were distal draining veins of large developmental venous anomalies. One patient had associated migration anomaly, and two patients had Sturge- Weber syndrome. Six patients with an isolated cerebral varix were observed. Of the 39 CVs in 22 patients, 20 lesions in 14 patients were followed up in outpatient clinics with imaging studies. The average follow-up duration was 6.6 years. During this period, no neurological events occurred, and all the lesions were managed conservatively.CONCLUSION: Nonfistulous CVs seemed to be asymptomatic in most cases and remained clinically silent. Hence, we suggest conservative management.

Venous angioma may be associated with epilepsy in children / 소아과

PURPOSE: Venous angioma (VA) is the most common congenital abnormality of the intracranial vasculature. This study aimed to investigate the relationship between VA and epilepsy and to identify the characteristics of children with VA and epilepsy. METHODS: The records of all patients aged less than 18 years who underwent brain magnetic resonance imaging (MRI) at Pusan National University Hospital were retrospectively reviewed. Patients with isolated VA and patients with normal MRI were compared in terms of the prevalence of epilepsy. RESULTS: In total, 2,385 pediatric patients who underwent brain MRI were enrolled. Isolated VA was identified in 26 patients (VA group). Among the patients with normal MRI findings, 225 age- and sex-matched patients to the VA-group were assigned to the control group. Nine patients in the VA group (9 of 26, 34.6%) and 27 patients in the control group (26 of 225, 11.5%; P<0.001) had epilepsy. In the VA group, 20 patients (76.9%) had the VA in the cerebral hemispheres, and 6 patients (23.1%) had the VA in the brainstem and cerebellum. The latter showed a higher prevalence of epilepsy (5 of 6, 83.3%) than the former (4 of 20, 20.0%; P=0.004). Among the nine patients who had epilepsy with VA, patients whose VA involved the brainstem and cerebellum showed a significantly higher frequency of abnormal Electroencephalographic findings than patients whose VA involved the cerebral hemispheres (P=0.016). CONCLUSION: VA, especially in the brainstem and cerebellum, might be associated with epilepsy.

Presentation of epilepsy in a patient with Wilson's disease and developmental venous anomaly [venous angioma] in the brain

Intracranial developmental venous anomalies [DVAs], also called venous angiomas, and Wilson's disease are both considered rare disorders with varying degrees of neurologic and systemic manifestations; yet the coexistence of the two disorders is considered extremely rare, bearing in mind the low prevalence of each disorder. Epilepsy is a recognised presentation in these disorders and will be the focus of discussion in our report of a 21-year-old male patient who, based on a clinical examination and laboratory and neuroimaging results, was diagnosed with both Wilson's disease and DVA. He presented initially at Sultan Qaboos University Hospital, Oman with tremors and writing difficulties in the right hand followed by the development of epilepsy, and was treated medically by de-coppering and antiepileptic medications. We also present a brief literature review of both disorders, their association with epilepsy, and treatment options. Family screening for patients with Wilson's disease is pivotal in preventing unfavourable outcomes

Cerebral Venous Angioma Complicated by Non-hemorrhagic Venous Infarction

Venous angioma is normally asymptomatic, but it can present with clinical presentations such as seizures, headache, and focal neurological deficits. Brain hemorrhage is known to be the most common complication, with nonhemorrhagic brain infarction due to venous angioma being very rare. We report the first case of supratentorial venous angioma complicated by nonhemorrhagic venous infarction. This case occurred in a 49-year-old female, and was confirmed by magnetic resonance (MR) imaging including contrast-enhanced MR venography and perfusion imaging.

Congenital orbital lymphangioma in a 20-years old girl a case report and review of literature

We report a case of a 20-year-old girl who presented to the out-patients' department with congenital, progressive unilateral proptosis and reduced vision. Ultrasound, computed tomography [CT] scan and magnetic resonance imaging [MRI] were performed. Diagnosis of orbital lymphangioma was made on imaging. Authors highlight the crucial role of imaging in diagnosis and to plan therapeutic approach. This case is reported because of its extreme rarity and unusual presentation

Cerebellar Venous Angioma Confused with Peripheral Vestibulopathy / 대한이비인후과학회지

Venous angioma is a congenital disease of vascular malformation, which is a mass like lesion consisted of dilated medullary veins. It is generally a silent lesion since it is found in normal brain tissues and tends to have low blood flow and pressure. The venous angioma could be diagnosed with magnetic resonance imaging and angiography, but most of them are incidentally diagnosed, because it is usually asymptomatic. The patients with venous angioma have nonspecific symptoms, such as headache, nausea and vomiting, seizures, progressive neurological deficit and hemorrhage. In particular, the venous angioma in cerebellum could cause hearing disturbance, tinnitus and dizziness. We report a case of venous angioma in cerebellum that had been mistaken for peripheral vestibulopathy in a patient complaining of dizziness

Midbrain venous angioma with obstructive hydrocephalus.

A rare case of a mid brain venous angioma with obstructive hydrocephalus is described. A dilated draining vein from the lesion in the aqueduct as the cause of the hydrocephalus is highlighted, and interesting features of the pathology of venous angiomas and associated cavernous hemangioma are described. The management of this interesting condition is discussed.

Patients with migraine headache role of MR imaging of the brain

To prospectively evaluate the role of MR imaging in cases of migraine headache and determine if there are associated brain parenchymal changes and to compare the incidence of these lesions in our study compared to the previous reports concerning the type of migraine, age and sex of the patients. Thirty-two patients with clinical diagnosis of migraine headache, with an average age of 29 years, were evaluated by MR imaging. Each patient was examined in the axial plane with long TR/short and long TE images. Eighteen patients also had additional T 1-W short TR/short TE MR scans, enhancement with gadopentate dimeglumine was used in four patients. Foci of high signal intensities in the deep white matter and periventricular regions were detected on T2 W MR images in four cases and a venous angioma in only two cases. Our study demonstrated that parenchymal brain abnormalities may be found in cases of migraine headache in only 12% of the included cases, while the previously reported studies have indicated that the parenchymal brain abnormalities may be found in up to 40% of patients with migraine headache, also the white matter changes were found in complicated cases of migraine headache and as the age of the patient advances compared to the other types of migraine and those less than 40 years and these white matter changes are non specific

Nuestra experiencia en el manejo de los angiomas cavernosos del sistema nervioso central / Our experience in the management of cavernomas

El objetivo del presente trabajo es analizar nuestra experiencia en el manejo de los cavernomas del sistema nervioso central (SNC). Se analizaron, retrospectivamente, aspectos epidemiológicos, clínicos, diagnósticos y terapéuticos de 22 pacientes admitidos al Servicio de Neurocirugía del Sanatorio Allende entre enero de 1985 y diciembre de 1999 con diagnóstico de cavernoma del SNC. La muestra está compuesta por nueve pacientes menores de 15 años y 13 adultos, 13 varones y 9 mujeres. Se incluyen 3 pacientes de sexo masculino familiares directos (cavernomatosis familiar). Doce pacientes consultaron por convulsiones (3 de ellos con epilepsia refractaria), ocho por déficit neurológico, uno por cefalea y otro asintomático miembro de la familia portadora de cavernomas múltiples. En todos los casos el diagnóstico se realizó con RNM. De 18 pacientes a los que se les realizó TAC, en seis casos el estudio reveló anomalias, en cinco se observaron calcificaciones, en otros cinco se observaron signos de sangrado agudo y en uno se demostró una lesión hipodensa en el sitio donde posteriormente se confirmó el cavernoma. En cuanto a la localización 13 casos fueron supratentoriales (5 temporales, 4 parietales, 2 frontales, 1 occipital y 1 de ganglios basales) y 2 medulares cervicales. Doce pacientes fueron operados, 7 por convulsiones (3 con epilepsia refractaria) y los cinco restantes por déficit neurológico; 6 recibieron sólo drogas antiepilépticas y en 4 casos la observación fue la única medida. En el postoperatorio inmediato un paciente falleció por hematomas cerebrales múltiples diagnosticándose histopatológicamente amiloidosis cerebral primaria, uno presentó hipo incoercible, uno hemianopsia homónima y otro presentó una fístula del LCR tratada médicamente. Los pacientes con epilepsia refractaria lograron controlar las crisis y uno se encuentra actualmente sin medicación alguna. La media de seguimiento clínico fue de 72 meses y el rango 1 a 180 meses

MR Angiography in the Diagnosis of Cerebral Venous Angiomas: 3D TOF Versus Phase Contrast

PURPOSE: To seek adequate sequence of MR angiography (MRA) on developmental venous anomaly in the brain. MATERIAL AND METHODS: We collected ten cases who demonstrated developmental venous anomaly on MR image. Eight patients among them, both 3D time-of-flight (TOF) with or without Gadolinium-DTPA enhancement and phase contrast(PC) were performed: TOF without enhancement in five, TO F with enhancement in six, and PC with 10 cm/sec in five, PC with 30 cm/sec in three, and PC with 5 cm/sec velocity encoding (VNEC) in two cases. RESULTS: On enhanced 3D TOF, both dilated collecting veins and medullary veins were well depicted in five of six cases, however, the signal intensity of the collecting and medullary veins are less than arteries. Dilated hyperintense collecting veins were well demonstrated on 3D PC with below the 10cm/sec VNEC in all seven cases, but the medullary veins were poorly defined. Unenhanced 3D TOF IVIRA could not reveal any dilated collecting and medullary veins in all five cases. CONCLUSION: Enhanced 3D TO F could demonstrate the collecting and medullary veins of developmental venous anomaly, however, 3D PC with below the 10 cm/sec VN EC could showthe dilated collecting and larger draining veins. We considered that combined enhanced 3D TOF MRA and PC with VENC 10cm/sec can substitute for conventional anglogram in the diagnosis of developmental venous anomaly.

Gd-DTPA Eenhanced IVIRI of the Cerebral Venous Angiomas: Cornparision with Cerebral Angiography

PURPOSE: The purpose of this study is to evaluate the gadolinium-enhanced MR imaging findings of cerebral venous angiomas, and to compare those findings with those of cerebral angiography. MATERIALS AND METHODS: We reviewed the findings of gadolinium-enhanced MR images of 11 cases with angiographically proved cerebral venous angiomas. We obtained Tl-and T2-weighted images with gadoliniumenhanced Tl-weighted images at a 0.5 T MR imager with a spin-echo technique. We analyzed the location and signal intensity of the lesion, pattern of contrast enhancement, the distribution of draining veins and medullary veins before and after contrast enhancement, and then compared with the findings of cerebral angiography. RESULTS: The characteristic central tubular signal void structure showed low signal intensity on Tl-weighted images and high signal intensity on T2-weighted images in all cases. After gadolinium enhancement, we could find a characteristic enhancement of all medullary and central veins in 7 cases. CONCLUSION: Gadolinium-enhanced MR image was useful in the diagnosis of venous angiomas especially in the detection of medullary veins. The invasive cerebral angiography could be replaced by this technique in this disease.

Intracranial Venous Angioma with Arteriovenous Malformation: Case Report

Intracerebral venous angiomas with arterial components have been reported rarely. A case of venous angioma with arterial component is presented. The patient was a 29-year-old-female who presented with abrupt headache followed by left hemiparesis. Computerized tomography and magnetic resonance imaging revealed intracerebral hematoma in right temporoparietal lobe and basal ganglia with surrounding signal voids. The cerebral angiography showed the characteristic umbrella appearance and single large draining vein of venous angioma in right temporoparietal lobe and fine arterial branches supplying the vascular malformation. At the operation, multiple arteriovenous fistulas without nidus were observed and delicate angiomatous network with red veins were found around the hematoma cavity, Histologic examination of the angiomatous network proved it to be an arteriovenous malformation. It was a variant of mixed angioma, venous angioma combined with arteriovenous malformation. Clinical, angiographic, and pathological features of intracerebral venous angiomas with arterial components were reviewed from literatures.