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.

Formation During Lifetime of Arteriovenous Shunt in Developmental Venous Anomaly That Caused Intracerebral Hemorrhage.

BACKGROUND: Developmental venous anomaly (DVA) or venous angioma is a common anomaly of cerebral veins that is found incidentally in the majority of cases. There are few cases of arteriovenous shunting in DVA associated with a more malignant course of the disease. Whether these DVAs with shunts are of congenital pathology or lifetime formations is unclear. CASE DESCRIPTION: We report a case of lifetime arteriovenous shunt formation in DVA that caused intracerebral hemorrhage in a child. The patient underwent 2 sequential direct surgeries: an emergency evacuation of the intracerebral hematoma and a scheduled excision of the DVA with arteriovenous shunting. CONCLUSIONS: Arteriovenous shunting in DVA may develop during a lifetime and cause intracerebral hemorrhages. This case showed that localization of DVA with arteriovenous shunting in a noneloquent area enables its complete microsurgical excision with favorable functional outcomes.

Cerebellar Proliferative Angiopathy Associated with a Pontine Telangiectasia and a Developmental Venous Anomaly.

Various mixed associations between arteriovenous malformations, cavernous malformations, developmental venous anomalies, and capillary telangiectasias have been described, and a common pathophysiologic event has been suggested to be present, although it is yet to be elucidated. We depict herein the imaging features of a patient who presented with a spontaneous cerebellar hemorrhage, in whom radiologic studies demonstrated a pontine telangiectasia, a brainstem/cerebellar developmental venous anomaly, and a cerebellar proliferative angiopathy. This unique, not previously reported combination of lesions shows that the spectrum of mixed vascular malformations continues to expand. A pathophysiologic mechanism related to the angiogenesis seen in these malformations is also hypothesized.

Epidural Venous Angioma Presenting with Spinal Cord Compression in a 42-Year-Old Woman with Previous History of Ovarian Malignancy.

BACKGROUND: Venous angioma is an extremely rare vascular malformation of the epidural space. To the best of our knowledge, only 5 cases have been documented to date and none has been reported in the setting of a previous malignancy. CASE DESCRIPTION: We report the case of a 42-year-old woman with a previous history of ovarian cancer, treated by surgery plus chemotherapy; who presented with signs of spinal cord compression for 3 weeks. Magnetic resonance imaging showed an intensely enhancing epidural mass at the T2-T6 level causing major spinal cord compression, for which urgent surgery was indicated. During surgery, the tumor was extremely hemorrhagic and the hemostasis was hazardous. Blood loss was estimated at 1.5 L, causing hemodynamic instability and requiring intensive resuscitation with fluids and blood transfusions. Gross total resection was achieved and the pathologic examination confirmed the diagnosis of venous angioma. The patient recovered quickly postoperatively and was able to walk independently within 2 weeks of starting intensive rehabilitation. She was symptom free with no clinical or radiologic evidence of recurrence at 1 year follow-up. CONCLUSIONS: Venous angioma should be included in the differential diagnosis of spinal epidural masses even in case of previous malignancy. Subtle imaging features should alert clinicians to this rare yet potentially life-threatening condition. Surgery remains the cornerstone of the treatment and can result in remarkable recovery.

Brainstem cavernous malformations: Natural history versus surgical management.

While brainstem cavernous malformations were once considered inoperable, improvements in patient selection, surgical exposures, intraoperative MRI-guidance, MR tractography, and neurophysiologic monitoring have resulted in good outcomes in the majority of operated patients. In a consecutive series of 104 patients with brainstem cavernous malformations, only 14% of patients experienced cranial nerve or motor dysfunction that was worse at late follow-up, relative to their preoperative condition. Outcomes were predicted by several factors, including larger lesion size, lesions that crossed the midline, the presence of a developmental venous anomaly, older age, and greater time interval from lesion hemorrhage to surgery. The 14% of patients who experienced a persistent neurological deficit as a result of surgery, while substantial from any perspective, compares favorably with the risks of observation based on a recent meta-analysis. Curative resection is a safe and effective treatment for brainstem cavernous malformations that will prevent re-hemorrhage in symptomatic patients.

Trigeminal Neuralgia Caused by Venous Angioma: A Case Report and Review of the Literature.

BACKGROUND: Trigeminal neuralgia typically occurs in the middle-aged to elderly population, is believed to be related to abnormal conduction within the trigeminal nerve, and is possibly is attributable to changes in myelin induced by pulsatile mechanical trauma from an adjacent vessel. At the point just before it enters the brainstem, there is a short segment at which nerve axons are still ensheathed in central myelin (produced by oligodendrocytes), but after a few millimeters, there is a transition to peripheral myelin (produced by Schwann cells). The region of this transition is called the Obersteiner-Redlich zone. It is thought that the area of the nerve containing the central form of myelin is especially susceptible to pathologic changes from vascular contact that result in demyelination and altered conduction. When associated with a venous angioma at the root entry zone, trigeminal neuralgia usually presents at a younger age. METHODS: We report a 34-year-old man with a complaint of left hemifacial stabbing pain in maxillomandibular area that was triggered by chewing and speaking. On examination, no neurologic deficit was detected. The pain was not relieved significantly, even with the administration of carbamazepine. Magnetic resonance imaging demonstrated venous angioma in the left cerebello-pontine region. RESULTS: Microvascular decompression was performed uneventfully. The patient's pain was completely relieved without neurologic deficits. CONCLUSION: It seems that the trigeminal neuralgia caused by venous angioma may occur in the younger population. In most cases, the vessel that caused compression can be identified with magnetic resonance imaging without the need for intensive conventional angiography.

Spinal intradural cystic venous angioma originating from a nerve root in the cauda equina.

A spinal intradural extramedullary venous angioma is extremely rare and has not been previously reported. In this paper, the authors report on this entity with morphological and immunohistochemical evidence, and discuss the surgical strategy for its treatment. A 54-year-old woman presented to Nagoya University Hospital complaining of left-sided pain in the hip, thigh, and inguinal and perianal regions, with progressive worsening during the previous 2 weeks. Lumbar spine MRI showed an intradural extramedullary cyst at the level of T12-L1, which extended from the conus medullaris to the cauda equina. The cyst wall was not enhanced on T1-weighted MRI with Gd. Intraoperatively, a midline dural opening allowed the authors to easily visualize a dark-reddish cyst behind the spinal nerve rootlets in the cauda equina adjacent to the conus medullaris. The cyst was believed to originate from one of the spinal nerve rootlets in the cauda equina and a cluster of veins was identified on the cyst wall. The cyst was resected with the affected nerve rootlet. The surgery left no detectable neurological deficit. Based on the morphological and immunohistochemical evidence, the lesion was diagnosed as a venous angioma. No tumor recurrence was confirmed based on MRI at the time of the 2-year follow up. This is the first report of an intradural extramedullary cystic venous angioma that was successfully resected.

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.

Clinical features of Sturge-Weber syndrome without facial nevus: five novel cases.

Classic Sturge-Weber syndrome (SWS) is characterized by presence of flammeus nevus involving the first sensory branch of trigeminal nerve, ipsilateral leptomeningeal angiomatosis, and choroidal angioma. Sporadic cases of SWS without facial nevus (SWS type III) have been rarely reported. Here we report the clinical and neuroradiological findings of five patients with SWS type III and compare their findings with those described in the literature. This study confirmed that SWS type III should be considered in any child or young adult presenting with seizures or complicated migraine and intracranial unilateral calcification. The diagnosis must be confirmed with contrast-enhanced MRI images of the brain. Surgical therapy should be considered in patients with drug-resistant and persistent epileptic seizures.

Successful treatment of cervical dystonia induced by basal ganglion venous angioma with gamma knife thalamotomy.

Stereotatic radiosurgery is typically not the first line of treatment for cervical dystonia. We present a patient with a rare cervical dystonia induced by a venous angioma in the right basal ganglion. The patient was successfully treated with a gamma knife thalamotomy after failed treatments of botulinum toxin injections and peripheral denervation.

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)

Preliminary evidence of safety following administration of L-DOPA and buspirone in an incomplete monoplegic patient.

STUDY DESIGN: Case report. OBJECTIVES: To report a case where a monoplegic patient received L-DOPA and/or buspirone. These compounds, normally used in the treatment of Parkinson's disease and anxiety respectively, were recently shown to induce spinal locomotor network activity and reflex stepping-like movements in animal models of spinal cord injury (SCI). However, the safety of these drugs as potential treatments for Central Pattern Generator (CPG) activation in paralyzed individuals remain unclear. SETTING: St-Jean-Chrysostome, Quebec, Canada. METHOD: The acute effects induced by these compounds were qualitatively assessed by the patient, a 38-year-old man who underwent surgery 17 years ago to remove an intracavernous angioma located at the mid-thoracic level (T5-T6) of the spinal cord. RESULTS: Self-administration every 2 days of L-DOPA (200, 400 and 600 mg, p.o.) and buspirone (5, 10 and 15 mg, p.o.) either separately or combined led to no atypical side effects (that is, occasional sleepiness, nervousness, insomnia or mild headaches). No movement was induced ipsilaterally although some sensations referred to by the patient as an increased blood flow in the lower back and upper leg regions were reported shortly after administration of the combined treatment. CONCLUSION: The results show no significant side effects following acute administration of L-DOPA and/or buspirone. This constitutes the first report providing preliminary evidence of safety following administration of these drugs in incompletely paralyzed individuals. The sensations of increased blood flow ipsilaterally with the combined treatment may also suggest that the dose regimen was not optimal or sub-threshold for inducing detectable CPG-mediated leg movements.

Venous lesions as a cause of sylvian aqueductal obstruction: case report.

OBJECTIVE: The cases of two patients who presented with sylvian aqueductal obstruction caused by venous lesions (an ectatic vein and a developmental venous anomaly, which are uncommon causes of obstructive hydrocephalus) are reported. CLINICAL PRESENTATION: A 42-year-old man presented with chronic headache and behavior abnormalities. Magnetic resonance imaging revealed supratentorial ventricular dilation caused by an obstruction of the sylvian aqueduct by a developmental venous anomaly. An 18-year-old man complained of intermittent headaches. The magnetic resonance imaging scan disclosed aqueductal stenosis-type hydrocephalus secondary to an ectatic vein. INTERVENTION: Both patients underwent endoscopic third ventriculostomy and recovered well. During the procedures, the aqueductal obstruction by venous elements could be seen clearly. CONCLUSION: Venous anomalies may cause obstructive hydrocephalus and can be suspected in cases of chronic and intermittent headaches. Endoscopic third ventriculostomy is an effective treatment.

[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.

Treatment differences in cases with venous angioma.

Venous angiomas were found to be the most common cerebral vascular malformations, composing 63% of such lesions in two autopsy series. Annual bleeding risk associated with venous angiomas is about 0.22 % per year. Venous angiomas are generally silent lesions because of their dynamic features, and are low flow and low pressure vascular structures draining normal brain tissue. An angioma rarely causes symptoms such as bleeding, seizure, hemifacial spasm, trigeminal neuralgia, aqueduct compression, nonhemorrhagic infarction and thrombosis of the draining vein. Even if it should bleed, the lesion can be managed conservatively in asymptomatic or mildly symptomatic patients. In this paper we report two venous angioma cases. The first patient bled twice in a short period of time and the angioma was located at the posterior fossa next to the left lateral recess. The second patient recently suffered a cerebral stroke that was located in the vicinity of the right caudate nucleus and not associated with the venous angioma that was located next to the left caudate nucleus. This patient had been under warfarin sodium treatment for 14 years due to his previous coronary artery bypass surgery, but unknowingly there was a venous angioma located next to the caudate nucleus.

The association of venous developmental anomalies and cavernous malformations: pathophysiological, diagnostic, and surgical considerations.

Developmental venous anomalies (DVAs) are often associated with intracranial cavernous malformations (CMs). The frequency of this association and the observation of de novo CMs located near a known, preexisting DVA raise speculations as to the possible etiopathogenetic relationship between the two. In this article, the authors review the recent literature dealing with the potential etiopathogenetic, prognostic, and therapeutic implications of the association between DVAs and CMs.

Association of intra- and extradural developmental venous anomalies, so-called venous angioma and sinus pericranii.

INTRODUCTION: We report a case of cerebellar venous angioma and parietal sinus pericranii. DISCUSSION: Venous angioma is classified as a developmental venous anomaly (DVA) because it is not a neoplasm but a variant that develops during embryogenesis. Sinus pericranii should be classified as extradural-type DVA. Although there have been few reports of association between these conditions, both are suspected to have the same pathogenesis, i.e., transient venous hypertension in the late embryonic period influencing venous development.

Recurrent cryptic vascular malformation associated with a developmental venous anomaly.

Abnormal venous drainage patterns, such as developmental venous anomalies (DVAs), are frequent findings neighbouring cryptic vascular malformations (CVMs). Although the clinical relevance of DVAs remains controversial, increasing attention has been focused on the possible importance of venous outflow disturbance and venous hypertension in DVAs for the development of CVMs. We present the case of a 32-year-old man with dysphasic seizures symptomatic for recurrence and rebleeding of a CVM of the left angulare gyrus, which drained entirely into a large DVA. A cavernoma had been surgically removed 1 year before, while the associated DVA had been left in place. In the following second surgical procedure, the new malformation, which was histologically verified as arteriovenous angioma (AVM), was removed and the DVA was occluded for the length of the angioma. Postoperative course of the patient was unremarkable, the patient is seizure free and neurologically intact. This first report of such a sequence of events raises important questions regarding the association of various vascular malformations, regarding transitional and hybrid forms, as well as the role of anomalous venous drainage in their pathophysiology and probably in their development. Our case and selected cases in literature suggest that venous hypertension in a DVA might not be just a coincidential finding, but sometimes can induce or influence the development and recurrence of associated vascular lesions. The developmental interrelationship, the potential mechanisms for this association and the implications of similar cases reported in pertinent literature are discussed.

A case of venous angioma with arteriovenous shunts--case report.

A 35-year-old man presented with a sudden headache and disturbance of consciousness. On admission, his consciousness level was Japan Coma Scale 100. Computed tomography disclosed a subarachnoid hemorrhage (SAH) and right cerebellar hematoma. Angiography was performed and, at first, arteriovenous malformation of the posterior fossa was diagnosed. Then external decompression of the posterior fossa and ventricular drainage were performed, followed by barbiturate therapy. Repeat angiography revealed that the lesion was a venous angioma with arteriovenous shunts. On day 37, subtotal removal of the lesion was performed. Intraoperatively, acute brain swelling emerged and partial internal decompression of the right cerebellar hemisphere was performed. The postoperative course was comparatively good and the patient was discharged with very mild ataxia. The patient is now being followed up in our outpatient clinic.