"It's Not a Tumor": A Rare Case of Symptomatic Cerebellar Developmental Venous Anomaly.

Cerebral developmental venous anomalies (DVAs) are benign anatomical variants of the venous system and are commonly described as an incidental finding without clinical significance. Neurologic symptoms or abnormal examination findings are rare and usually attributed to hemorrhagic complications related to coexisting cavernous malformations. There have been limited case reports of symptomatic, uncomplicated DVAs described in the literature. The following case describes a previously healthy child who presented to the emergency department with an acute onset of altered mental status, headache, and focal neurologic examination abnormalities. Magnetic resonance imaging revealed a prominent cerebellar DVA. There was no evidence of a cavernous angioma, hemorrhage, or acute parenchymal injury. This case report illustrates a clinically symptomatic, uncomplicated posterior fossa DVA. It provides additional evidence regarding the potential for a cerebral venous malformation in causing focal neurologic deficits.

Developmental venous anomaly as a rare cause of obstructive hydrocephalus.

Developmental venous anomalies (DVA), previously known as cerebral venous malformations or venous angiomas, are common benign entities often incidentally discovered at MRI examinations. They are non­pathologic variants of normal deep parenchymal veins that are usually asymptomatic, but they can rarely cause some complications. In this paper we described a rare case of obstructive hydrocephalus caused by a DVA located within the cerebral aqueduct and we also reviewed the previous literature on this topic. A 37­year­old man was diagnosed with symptomatic tri­ventricular hydrocephalus that during the last year caused episodes of ictal headaches accompanied by vertigo, diaphoresis and lipothimic events. The MRI and the angiography showed the presence of a large collector vein coursing within the aqueduct, which was part of a complex DVA in the posterior fossa, responsible for the aqueductal stenosis and obstructive hydrocephalus. The patient was submitted to endoscopic third ventriculostomy (ETV) and then he had a complete resolution of symptoms. A literature review was performed through Medline Pubmed on papers published in English from 1937 to 2015 with the following key words: DVA, obstructive hydrocephalus, acqueductal stenosis, venous angioma, venous malformation, medullary malformation. Including our case, the literature research identified 16 reports in the Medline database for 18 patients presenting with obstructive hydrocephalus caused by a DVA including our case. In approximately 60% (10/17) of cases, the stenosis causing obstructive hydrocephalus was present in the cerebral aqueduct. The floor of the IV ventricle and the third ventricle were the second most common locations and DVAs were identified in 20% of cases (3/17) for each of these regions. In the remaining case the venous malformation was found in the Foramen of Monro. Clinical presentation before diagnosis lasted on average 23 months ranging from 1 month to 7 years. The mean age at diagnosis was 27 (range 3 days- 58 years). The most common presenting symptom was persistent headache, representing a major complaint in 80% of cases (12/15). In 78% (14/18) of cases MRI was crucial in the diagnosis. ETV was performed in 47% (8/17) of patients, whereas no treatment or clinical follow­up was required in 18% (3/17) and VP Shunt in 12% (2/17) of cases. Instead, VA shunt, transcallosal approach and acqueductal stenting were performed in one patient each, corresponding singularly to 6% (1/17) of all cases. Despite the fact that DVAs are asymptomatic, they may rarely cause obstructive hydrocephalus because of impairment in the CSF flow. They should be considered in the differential diagnosis of any patient presenting with obstructive hydrocephalus. ETV has been demonstrated as an effective treatment option in the management of obstructive hydrocephalus due to a DVA.

Clinical manifestations and imaging findings of thrombosis of developmental venous anomalies.

AIM: To determine clinical manifestations, imaging findings and outcome of patients with thrombosed developmental venous anomalies (DVAs). MATERIALS AND METHODS: The radiology database was searched retrospectively for thrombosed DVAs between 01/01/2000 to 07/01/2016. Demographic variables, associated risk factors, clinical manifestations, imaging findings, treatments, and follow-up were recorded. RESULTS: Six patients were found (four female and two male; age range 16-45 years with mean age, 21.3 years). The most common clinical presentation was headaches followed by neurological deficits and seizures. Venous infarction, parenchymal haemorrhage, venous congestive oedema were noted as the radiological findings. Clinical outcome was favourable in all patients with complete recovery or persistence of mild neurological symptoms. CONCLUSION: Thrombosed DVAs may occur under rare circumstances, which lead to variable symptoms. Familiarity with this entity and early recognition of associated findings including venous infarction, parenchymal haemorrhage, and venous congestive oedema would help early diagnosis and prompt treatment.

Enfermedad quística subadventicial en vena femoral común / Subadventitial cystic disease of common femoral vein

No disponible

Cluster headache - a symptom of different problems or a primary form? A case report.

Headache with severe, strictly one-sided unilateral attacks of pain in orbital, supraorbital, temporal localisation lasting 15-180 minutes occurring from once every two days to 8 times daily, typically with one or more autonomic symptoms, is recognized as cluster headache (CH). Headache with normal neurological examination and abnormal neuroimaging studies, mimicking cluster headache, is reported by several authors. We present an elderly woman with a cluster-like headache probably associated with other comorbidities. We differentiate between primary, but 'atypical' CH and symptomatic cluster headache due to frontal sinusitis, pontine venous angioma or vascular compression of the trigeminal nerve root. This headache is not so rare in the general population and its secondary causes must be ruled out before the diagnosis of a primary headache as cluster headache is made.

Trigeminal neuralgia caused by venous angioma: case report.

A 34-year-old female presented with trigeminal neuralgia caused by a venous malformation in the right cerebello-pontine region. Computed tomography and magnetic resonance imaging demonstrated the abnormal draining veins from the venous malformation. The dilated vessels extended around the trigeminal nerve and compressed the root entry zone. Microvascular decompression (MVD) was performed, and her trigeminal neuralgia was completely relieved without neurological deficits. The offending vessel in most cases of trigeminal neuralgia is an arterial branch. Veins may also be associated with trigeminal neuralgia. The present rare case shows that MVD may be useful for the treatment of trigeminal neuralgia associated with venous malformation. Good outcome depends on decompression of the root entry zone without injury to the vessel. Surgical injury in this region can cause severe neurological deficits. Several treatment options should be prepared for the surgery, such as MVD or rhizotomy.

Non-invasive imaging of intracranial pediatric vascular lesions.

OBJECTIVES: The goal of this review is to discuss the different non-invasive imaging techniques as well as the age-specific pediatric vascular pathologies and their imaging features. MATERIAL AND METHODS: Ultrasound, computed tomography, and magnetic resonance imaging features of pediatric ischemic stroke, intracranial hemorrhage, aneurysms, cavernomas, developmental venous anomalies, and arteriovenous malformations are presented. In addition, multiple non-invasive angiographic techniques (CT and MR angiography) and functional MRI sequences (diffusion, perfusion, and susceptibility-weighted imaging) are discussed. CONCLUSION: Neurovascular imaging plays a central role in the early, sensitive, and specific diagnosis of pediatric intracranial vascular disorders. A detailed knowledge of the quality and exact angioarchitecture of pediatric vascular pathologies as well as their impact on the cerebral hemo-dynamics is essential to guide and monitor treatment options and to predict functional outcome.

Cerebral developmental venous anomalies: current concepts.

Cerebral developmental venous anomalies are the most frequently encountered cerebral vascular malformation, and as such, are frequently reported as fortuitous findings in computed tomography (CT) and magnetic resonance imaging (MRI) studies. Developmental venous anomalies (DVAs) are generally considered extreme anatomical variations of the cerebral vasculature, and follow a benign clinical course in the vast majority of cases. Here we review current concepts on DVAs with the aim of helping clinicians understand this complex entity. Morphological characteristics that are necessary to conceptualize DVAs are discussed in depth. Images modalities used in diagnosing DVAs are reviewed, including new MRI or CT techniques. Clinical presentation, association with other vascular malformations and cerebral parenchymal abnormalities, and possible physiopathological processes leading to associated imaging or clinical findings are discussed. Atypical forms of DVAs are also reviewed and their clinical significance discussed. Finally, recommendations as to how to manage asymptomatic or symptomatic patients with a DVA are advanced.

Hemorragia intracerebral secundaria a trombosis espontánea de una anomalíavenosa del desarrollo: 2 casos y revisión de la literatura / Two cases of brain haemorrhage secondary to developmental venous anomaly trombosis. Bibliographic

Presentamos dos casos de hemorragia cerebral secundaria a la trombosis de una anomalía venosa del desarrollo (AVD) recientemente tratados en nuestro servicio. La primera paciente era una mujer de 28 años que tomaba anticonceptivos orales desde hacía un mes, que ingresó tras deterioro agudo del nivel de conciencia precedido de hemiparesia, cefalea y vómitos de 24 horas de evolución. La tomografía computarizada (TC) realizada al ingreso demostró una imagen hipodensa con áreas de hiperdensidad en su interior y que ejercía efecto masa con desviación de la línea media. La lesión era sugestiva de un infarto hemorrágico que ocupaba casi la totalidad del lóbulo frontal derecho. Tanto en la TC, la resonancia magnética (RM) y la angioRM destacaba la existencia de una estructura tubular en el interior del hematoma compatible con un vaso con un trombo en su interior. La paciente fue sometida a una craneotomía urgente con evacuación parcial del hematoma. La arteriografía confirmó la existencia de un drenaje venoso anómalo (DVA) con un defecto de replección en su interior en el que desembocaba un penacho de venas con el patrón típico de "Cabeza de Medusa" por lo que, ante la sospecha de trombosis venosa, se procedió a la anticoagulación sistémica de la paciente. A los 4 días de la intervención se procede a la retirada progresiva de la sedación y de la intubación presentando la paciente un GCS de 15 con leve hemiparesia izquierda 4/5. El segundo caso fue un varón de 38 años que ingresó por un cuadro de crisis comicial tónico-clónica en hemicuerpo izquierdo seguida por una sensación de disestesia en la misma localización. La TC inicial mostraba una imagen de hemorragia intracraneal en la cual, al inyectar contraste, se apreciaba una imagen de vaso anómalo dilatado que, en el estudio angiográfico, resultó corresponder a un anomalía del desarrollo venoso trombosada. Se adoptó una actitud expectante con seguimiento angiográfico periódico de la lesión. El paciente fue dado de alta sin déficits neurológicos (AU)

We report two cases of intracraneal brain haemorrhage secondary to developmental venous anomaly trombosis recently treated at our Department. First patient was a 28-year old woman on oral contraceptive treatment for a month who was referred to our Department with sudden-onset conscious level deterioration after presenting 24 hours previously with headache, vomits and hemiparesis. Computed Tomography revealed a predominant hypodense area containing hyperdense foci causing mild mass effect and midline-shift in keeping with a haemorrhagic infarction occupying almost completely the right frontal lobe. On CT, magnetic resonance (MR) and magnetic resonance angiography (MRA) there was a prominent tubular structure adjacent to the hematoma in keeping with a partly thrombosed vessel. Urgent craniotomy and partial hematoma evacuation was performed. Digital subtraction angiography confirmed the presence of a filling defect within the draining vein of a typical caputmedusae pattern developmental venous anomaly (DVA). Systemic anticoagulation was started and four days after surgery sedation was reversed and the patient awoke with normal conscious level although mild (4/5) hemiparesis persisted. Second patient was a 38-year old male evaluated in the Emergency Department due to tonic-clonic seizures in the left side followed by altered sensation in the same distribution. Initial CT revealed an intracranial bleed. After contrast administration there was an anomalous vessel in the same location that was confirmed angiographically represented a partly thrombosed DVA. Conservative management was favoured and the patient was discharged from hospital without clinical neurological deficits (AU)

Mental retardation, facial anomalies, brachydactyly, cerebral angiomas, femoral nucleus necrosis: a new entity or Hall-Riggs syndrome?

We report on a 4-year-old boy with mental retardation, facial and skeletal anomalies, cerebral angiomas, femoral nucleus necrosis, mild biochemical abnormalities. This complex of features resembles the Hall-Riggs syndrome but could represent a novel syndrome.

Diffuse hemangiomatosis with predominant central nervous system involvement.

Diffuse neonatal hemangiomatosis presents with multiple, progressive, rapidly growing cutaneous hemangiomas associated with widespread visceral hemangiomas in the liver, lungs, gastrointestinal tract, brain, and meninges. Described here is a case of diffuse neonatal hemangiomatosis with predominant central nervous system involvement. The early appearance of central nervous system lesions on imaging can overlap with that of cavernous malformations, confounding diagnosis; however, rapid growth, response to steroids, cystic appearance with sedimentation levels of the mature lesions, and involvement of other visceral organs can help confirm the diagnosis.

Brain parenchymal signal abnormalities associated with developmental venous anomalies: detailed MR imaging assessment.

BACKGROUND AND PURPOSE: The occurrence of brain parenchymal signal-intensity changes within the drainage territory of developmental venous anomalies (DVAs) in the absence of cavernous malformations (CMs) has been incompletely assessed. This study was performed to evaluate the prevalence of brain parenchymal signal-intensity abnormalities subjacent to DVA, correlating with DVA morphology and location. MATERIALS AND METHODS: One hundred sixty-four patients with brain MR imaging with contrast studies performed from July 2005 through June 2006 formed the study group. The examinations were reviewed and data were collected regarding the following: location, depth, size of draining vein, associated increased signal intensity on fluid-attenuated inversion recovery and T2-weighted images, associated CMs, and associated signal intensity on gradient recalled-echo sequences. RESULTS: Of the 175 DVAs identified, 28 had associated signal-intensity abnormalities in the drainage territory. Seven of 28 DVAs with signal-intensity abnormalities were excluded because of significant adjacent white matter signal-intensity changes related to other pathology overlapping the drainage territory. Of the remaining DVAs imaged in this study, 21/168 (12.5%) had subjacent signal-intensity abnormalities. An adjusted prevalence rate of 9/115 (7.8%) was obtained by excluding patients with white matter disease more than minimal in degree. Periventricular location and older age were associated with DVA signal-intensity abnormality. CONCLUSION: Signal-intensity abnormalities detectable by standard clinical MR images were identified in association with 12.5% of consecutively identified DVAs. Excluding patients with significant underlying white matter disease, we adjusted the prevalence to 7.8%. The etiology of the signal-intensity changes is unclear but may be related to edema, gliosis, or leukoaraiosis secondary to altered hemodynamics in the drainage area.

[Case of venous angioma with chronic encapsulated hematoma].

We would like to report a rare case of a venous angioma with a chronic encapsulated hematoma, in a 31-year-old woman who was admitted to our hospital in November, 2005. Her neurological examination was noted to be normal. A CT was performed which showed an isodense mass in the left cerebellar hemisphere. This finding was confirmed on MR imaging, which showed a hyperintense mass on T1-weighted imaging, and a hypo to isointense area on T2-weighted images. T1-weighted images with Gd-DTPA enhancement revealed a dilated vein and, as a result, an angiogram was obtained. The venous phase demonstrated medullary veins converging on a central vein, which is usually known as a caput medusae. Follow up T1-weighted images with Gd-DTPA enhancement showed peripheral ring enhancement and expansion of the capsulated hematoma. Total removal of the hematoma and its capsule was performed in July, 2006. The hematoma was seen both inside and outside the tough capsule. A histological examination revealed that the capsule consisted of an outer collagenous layer and an inner granulated layer with deposits of hemosiderin. The hemorrhage rate of a venous angioma has been reported as between 0.22-0.61% per year. We thought that the initial small hemorrhage from the venous angioma resulted in the development of an encapsulated hematoma, and the total hematoma had expanded not only inside due to the hemorrhage of the capsule, but also outside because of the venous angioma.

Asociación de angiomas cavernosos con venosos / Association of cavernous and venous angiomas

Introducción. Los angiomas cavernosos y venosos deforma aislada son malformaciones vasculocerebrales frecuentes.Su asociación se identifica cada vez más debido ala mayor utilización de la resonancia magnética (RM) en losestudios cerebrales. Objetivo. Analizar las características de la asociaciónentre angioma cavernoso y venoso y la relación con su localizacióny tamaño, el sexo del paciente y el uso de contrasteintravenoso para el diagnóstico por RM. Métodos. Retrospectivamente se han revisado los estudiosde RM cerebral de 37 pacientes con angiomas cavernosos.Las relaciones estadísticas se establecieron con tablas decontingencia y el estadístico de la χ2 de Pearson y la t deStudent para muestras independientes. Resultados. Se identificaron 52 angiomas cavernososen 37 pacientes. No se han encontrado diferencias estadísticamentesignificativas que relacionen el tamaño del angiomacavernoso, su localización y el sexo del paciente con laexistencia de un angioma venoso asociado. Sólo la administraciónde contraste ha mostrado una relación estadísticamentesignificativa para su diagnóstico. Conclusiones. La asociación de angioma cavernoso y venosoes frecuente (30 %). Es necesario administrar contrastepara detectar los angiomas venosos. Esta asociación tiene implicacionespronósticas por el riesgo de sangrado y quirúrgicas,ya que la resección de la anomalía venosa no se aconsejapor la posibilidad de desencadenar infartos venosos

Introduction. Isolated cavernous and venous angiomasare common vascular cerebral malformations. Due to theroutine use of magnetic resonance imaging (MRI) in cerebral studies, their association has been identified more frequently. Objective. To analyze the characteristics of the association between both lesions (cavernous and venous angiomas), and the relationships with localization, size, patient gender and the use of intravenous contrast material in the diagnosis with MR.Methods. A retrospective analysis of the cerebral MRIstudies of 37 patients with cavernous angiomas was made.Statistical relationships were established with contingency tables and statistical methods of Pearson χ2 and of Student’s test for independent samples. Results. A total of 52 cavernous angiomas were identified in 37 patients. No significant statistical differenceswere found that related the cavernous angioma size, localization or patient gender with the existence ofan associated venous angioma. Only the administrationof contrast material has shown a statistically significantrelationship for the diagnosis. Conclusions. The association of cavernous and venous angioma is frequent (30 %). Contrast material must be administeredto detect venous angiomas. This association hasprognostic implications, due to bleeding risk and surgerysince resection of the venous anomaly is not recommendeddue to the possible venous infarction