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The incidental diagnosis of both developmental venous anomalies (DVAs) and cavernous malformations (CMs) in the central nervous system is increasing with improved imaging techniques. While classically silent diseases, these cerebrovascular pathologies can follow an aggressive course, particularly when present in the brainstem. In the last decade, substantial research has focussed on KRIT1-mediated tight junction gene expression and their role in CM development. However, our understanding of the physiologic conditions precipitating symptomatic CM development or CM haemorrhage with and without concomitant DVAs, remains lacking. The only established risk factor for CM haemorrhage is a previous history of haemorrhage, and literature currently reports trauma as the only precipitant for symptomatic events. While plausible, this occurs in a minority, with many patients experiencing occult events. This manuscript presents a hypothesis for symptomatic CM events by first discussing the anatomical pathways for intracranial venous outflow via the internal jugular veins (IJV) and vertebral venous plexus (VVP), then exploring the role of venous flow diversion away from the IJVs under physiologic stress during dynamic postural shift. The resultant increase in intracranial venous pressure can exacerbate normal and pre-existing structural DVA pathologies, with repeated exposure causing symptomatic or CM-inducing events. This pathophysiological model is considered in the context of the role of the autonomic nervous system (ANS) in postural intracranial venous outflow diversion, and how this may increase the risk of DVA or CM events. It is hoped that this hypothesis invokes further investigation into precipitants for DVA or CM events and their sequela and, also, furthers the current knowledge on pathophysiological development of DVAs and CMs.

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.

[No abstract available]

Developmental venous anomaly (DVA) is a common congenital venous malformation characterized by dilated medullary veins in caput medusa configuration and a draining vein. Despite the high incidence of DVAs, they are benign anatomic variations and rarely cause symptoms. Here, we report computed tomography and magnetic resonance imaging findings with perfusion images of acute infarction from underlying DVA in a 63-year-old female patient who presented with acute onset of neurologic symptoms and recovered without any neurologic deficit.

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 non‑pathologic 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 37‑year‑old man was diagnosed with symptomatic tri‑ventricular 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 follow‑up 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.

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 sexmatched 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

Background Klippel-Trenaunay-Weber syndrome (KTWS) is a rare syndrome characterized by the triad of cutaneous hemangiomas, venous varicosities, and osseous-soft tissue hypertrophy of the affected limb. Clinical manifestations, genetic testing, and radiologic imaging are the key steps in diagnosing this syndrome. Case Description An 18-month-old boy was brought for follow-up brain magnetic resonance imaging (MRI) with a history of right lower limb hypertrophy, cutaneous varicosities, and hemangiomas diagnosed at birth. A baseline MRI at 12 months revealed multiple hemorrhagic lesions within the cerebrum, the largest in the right temporal lobe, which was treated surgically at the age of 18 months because of its rapid growth. This is the youngest patient with KTWS treated surgically for intracranial hemangiomas. Conclusion KTWS is a rare disease with a wide range of manifestations. Multisystemic evaluation of this group of patients should be performed to identify cavernous hemangiomas at the early stage of life and adequately treat them in the future. Treatment of KTWS patients with cavernous hemangiomas should not be different from the treatment of patients with any other hemangiomas, and surgical intervention should be considered on a case-to-case bases.

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.

Developmental venous anomalies (DVAs), previously called venous angiomas, are the most frequently encountered cerebral vascular malformations. However, DVA is considered to be rather an extreme developmental anatomical variation of medullary veins than true malformation. DVAs are composed of dilated medullary veins converging centripetally into a large collecting venous system that drains into the superficial or deep venous system. Their etiology and mechanism are generally accepted that DVAs result from the focal arrest of the normal parenchymal vein development or occlusion of the medullary veins as a compensatory venous system. DVAs per se are benign and asymptomatic except for under certain unusual conditions. The pathomechanisms of symptomatic DVAs are divided into mechanical, flow-related causes, and idiopathic. However, in cases of DVAs associated with hemorrhage, cavernous malformations (CMs) are most often the cause rather than DVAs themselves. The coexistence of CM and DVA is common. There are some possibilities that DVA affects the formation and clinical course of CM because CM related to DVA is generally located within the drainage territory of DVA and is more aggressive than isolated CM in the literature. Brain parenchymal abnormalities surrounding DVA and cerebral varix have also been reported. These phenomena are considered to be the result of venous hypertension associated with DVAs. With the advance of diagnostic imagings, perfusion study supports this hypothesis demonstrating that some DVAs have venous congestion pattern. Although DVAs should be considered benign and clinically silent, they can have potential venous hypertension and can be vulnerable to hemodynamic changes.

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.

Background We aimed to evaluate the factors associated with de novo brain cavernoma formations after patients underwent gamma knife radiosurgery (GKRS) and confirmed whether developmental venous anomaly (DVA) presented with a cavernoma and whether the cavernoma was included in the GKRS target location. Methods From January 2003 to December 2008, 95 patients underwent radiosurgery for brain cavernoma at our institution. Of these, 15 with multiple cavernomas related to familial cavernoma or with a history of surgical treatment for cavernoma were excluded. A total of 80 patients (44 men and 36 women; average age, 39.4 years) with sporadic cavernoma were retrospectively analyzed by considering the patient characteristics, including sex, age, target volume, radiation dose, clinical symptoms, cavernoma location, radiosurgery complications, and morphology of DVA. Results The average target volume, mean radiation dose, and mean target percentage were 1019.2 mm3, 13.7 Gy, and 51.1%, respectively. Nineteen patients showed cavernomas associated with DVA; of these, de novo cavernoma formations were noticed in 4 patients at a median of 49.5 months after undergoing GKRS. All de novo cavernomas were related to the presence of DVA and were located near the brainstem or cerebral peduncle. De novo cavernomas occurred when DVAs were not included in the GKRS-target location. Conclusions All de novo cavernomas were located near the brainstem or cerebral peduncle, and they occurred in the presence of DVAs. The presence of DVA in the radiosurgery target location might be potentially an important factor associated with de novo cavernoma formation.

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.

Intracranial developmental venous anomalies are the most common vascular malformation. In the immense majority of cases, these anomalies are asymptomatic and discovered incidentally, and they are considered benign. Very exceptionally, however, they can cause neurological symptoms. In this article, we present three cases of patients with developmental venous anomalies that presented with different symptoms owing to complications derived from altered venous drainage. These anomalies were located in the left insula, right temporal lobe, and cerebellum. The exceptionality of the cases presented as well as of the images associated, which show the mechanism through which the symptoms developed, lies in the low incidence of symptomatic developmental venous anomalies reported in the literature.

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 anomalies (DVAs) are relatively common lesions, present in up to 3% of the population. The defining characteristic of these lesions is the confluence of radially oriented veins into a single dilated venous channel. DVAs are also known as cerebral venous angiomas, cerebral venous malformations, and cerebral venous medullary malformations. They are the most common type of cerebral vascular malformation found on autopsy studies, and they are often encountered as incidental findings on neuroimaging studies. DVAs are congenital lesions thought to arise from aberrations that occur during venous development, but continue to provide the normal venous drainage to the cerebral territory in which they reside. Although the natural history of DVAs is benign, they may be associated with cavernous malformations or other vascular abnormalities, which can lead to hemorrhage in the vicinity of the DVA. Surgical or endovascular obliteration of DVAs carries a significant risk of venous infarction; thus, conservative management is the treatment of choice for patients with these lesions.

Initially known as venous angioma, Developmental Venous Anomaly (DVA) is a persistent variant of fetal brain venous drainage [1, 2]. Unlike cerebellar DVA with or without brainstem extension, isolated pontine DVA is a rare condition [3, 4]; and as per our knowledge, its presentation with exclusive transpontine drainage has never been described before. The authors present the case of an isolated pontine DVA with a selective transpontine drainage discovered incidentally; the clinical presentation, the radiological findings, and the management are discussed.

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.

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.

BACKGROUND Developmental venous anomalies (DVAs) are rare vascular diseases becoming more frequently diagnosed. Most patients with DVAs have no clinical symptoms with the exception of a few patients with epilepsy, intracranial hemorrhage, or neuro-function deficit. There is still controversy with respect to treatment strategies for symptomatic DVAs. MATERIAL AND METHODS Forty-three cases of symptomatic DVAs from January 2006 to October 2015 were retrospectively reviewed and the imaging characteristics of DVAs by CT, MRI, and DSA and the treatment modalities for DVAs were studied. RESULTS Typical imaging characteristics of symptomatic DVAs were wedge or umbrella-shaped collections of dilated medullary veins converging in an enlarged subependymal or transcortical collecting vein, draining to the superficial or deep vein system. Based on location and draining vein features, symptomatic DVAs were tentatively classified into six different subtypes. Of the 43 cases, 19 were treated by surgical methods and 24 were treated conservatively. CONCLUSIONS We concluded that the rate of accompanying abnormalities in cases of symptomatic DVAs was high. Intracerebral hemorrhage was usually attributed to associated CMs or AVMs. The associated lesions and the branches responsible for bleeding could be resected while preserving the collecting vein as far as possible.

Developmental venous anomalies (DVAs) are usually asymptomatic. We report a case of DVA thrombosis with recurrent tiny frontal hematoma in a 24-year-old man. The contribution of T2-GRE and SWAN sequences are discussed. Follow-up attested complete recanalization after anticoagulation.

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 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 meta-analysis published by Horne et al. 2016. Curative resection is a safe and effective treatment for brainstem cavernous malformations that will prevent re-hemorrhage in symptomatic patients.

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.

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 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.

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.

Developmental venous anomaly (DVA) is a common lesion formerly known as venous angioma. DVAs drain normal brain parenchyma; however, parenchymal abnormalities surrounding DVAs have been reported. Unilateral putamen and caudate calcification in the drainage territory of DVAs has so far been reported in 7 cases, all with deep venous drainage. We present two additional cases of DVAs, one with superficial and the other one with deep venous drainage, associated with basal ganglia calcifications. We emphasize that DVAs should be in the differential diagnosis of unilateral basal ganglia calcifications.

We report a case of cerebellar venous angioma and parietal sinus pericranii. 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.

Cowden syndrome (CS) is a rare autosomal dominant genodermatosis, characterized by multiple hamartomas, particularly of the skin, associated with high frequencies of breast, thyroid, and genitourinary malignancies. Although Lhermitte-Duclos disease (LDD) or dysplastic gangliocytoma of the cerebellum, a slowly progressive unilateral tumor, is a major criterion of CS, its frequency in patients with CS is unknown. Other cerebral abnormalities, especially meningioma and vascular malformations, have also been described, albeit rarely, in these patients. The aim of the current study was to use cerebral magnetic resonance imaging (MRI) to evaluate LDD frequency and to investigate other brain abnormalities in CS patients recruited by dermatologists. A multicenter study was conducted in 8 hospital dermatology departments between January 2000 and December 2003. Twenty patients with CS were included; specific cerebral MRI abnormalities were found in 35% (7/20) of them. Cerebral MRI revealed LDD in 3 patients, a meningioma in 1, and numerous vascular malformations in 6 patients. Five patients had venous angiomas (3 associated with LDD) and 2 patients had cavernous angiomas (1 associated with LDD and a venous angioma). The discovery of asymptomatic LDD in 3 patients and a cavernous angioma in another prompted us to perform neurologic examinations regularly and MRI to estimate the size and the extension of the tumor, and to assess the need for surgery. CS similarities with Bannayan-Riley-Ruvalcaba (BRR) are discussed because some patients could also have the BRR phenotype (for example, genital lentigines, macrocephaly, multiple lipomas) and because BRR seems to have more central nervous system vascular anomalies. Because CS signs can involve numerous systems, all physicians who might encounter this disease should be aware of its neurologic manifestations. Our findings confirm the contribution of brain MRI to detecting asymptomatic LDD, vascular malformations, and meningiomas in patients with CS.

Although the majority of hemangiomas of infancy can be expected to follow a benign course, a significant subset may result in serious complications. Recently, hemangiomas of segmental morphology, or those which are large, plaque-like, and patterned in distribution, have been recognized as important markers for potential complications. PHACE syndrome represents the best known example of the variety of problems that can occur in this setting. The PHACE acronym, which stands for posterior fossa brain malformations, segmental cervicofacial hemangiomas, arterial anomalies, cardiac defects and coarctation of the aorta, and eye anomalies, is sometimes referred to as PHACE(S) when ventral developmental defects such as sternal clefting and supraumbilical raphe are present. This article reviews the specific manifestations of PHACE, reflects on pathogenesis, and discusses appropriate work-up and future directions for this complex and fascinating syndrome. We also discuss other complications associated with hemangiomas of segmental morphology, including ulceration, potential visceral involvement, and underlying anomalies related to the lumbosacral location.

The term "venous angioma" (VA) usually refers to a developmental venous anomaly (DVA). However, a group of vascular malformations called VAs shows no venous abnormalities on angiography. The clinical and histological features of histologically classified VAs were studied in eight patients who presented with hemorrhage or seizures to reevaluate these venous anomalies. Angiography showed no venous abnormalities in six patients. Histological study included immunostaining for smooth muscle actin and glial fibrillary acidic protein. Surgical specimens of 10 cases of cavernous angiomas, 10 cases of arteriovenous malformations, and two cases of capillary telangiectasias were studied to compare these types of VAs. Angiographically occult VAs were surgically removed safely, whereas removal of DVAs was complicated by brain swelling and hemorrhagic infarction of the brain. Histological examination found angiographically occult VAs contained malformed and compactly arranged vessels with partly degenerated walls, whereas DVAs had dilated thin-walled vessels that were diffusely distributed in the normal white matter. This study of our cases and a review of the reported cases of VAs suggests that two different clinical and pathological entities are commonly categorized as "VA," angiographically occult VAs and DVAs. These two entities should be carefully distinguished.

Developmental venous anomaly (DVA) is now considered common and benign disease within the field of cerebral vascular malformation. Though symptomatic DVA is uncommon, further management is necessary to alleviate the symptoms and signs induced by symptomatic DVA, such as parenchymal hemorrhage, venous infarction, brain edema, obstructive hydrocephalus, and nerve root compression. From the viewpoint of obstructive hydrocephalus, mostly resulted from obstruction of aqueduct of Sylvius. Herein, we reported a case with presentation of obstructive hydrocephalus caused by DVA induced fourth ventricle outlet obstruction.

Developmental venous anomalies (DVA) and cavernous malformations (CM) are a common form of mixed vascular malformation. The relationship between DVA, CM and hemorrhage is complicated. It is important to differentiate hemorrhagic CM and hemorrhagic DVA. A retrospective review of all patients with acute spontaneous intracerebral hemorrhages (ICH) between 1 May 2008 and 1 May 2013 was performed. ICH due to DVA or CM were identified and compared for demographic features, clinical symptoms, neurological deficits, and radiological findings. A total of 1706 patients with acute spontaneous ICH were admitted to our hospital during the study period. Among these, 10 (0.59%) were caused by DVA and 42 (2.47%) were caused by CM. No significant differences were found in age (p=0.252) or sex ratio (p=1.000) between the two groups. Compared with CM-induced ICH, DVA-induced ICH were characterized by cerebellar predominance (p=0.000) and less severe neurological deficits (p=0.008). Infratentorial hemorrhagic DVA are characterized by cerebellar predominance and benign clinical course. Infratentorial hemorrhagic CM are mainly located in the brainstem. DVA should be given suspected rather than CM when considering the etiology of a cerebellar hemorrhage, especially in young adults.

Hemifacial spasms (HFS) are usually caused by vascular compression on the extra-axial facial nerve. In this case, we concluded that an intra-axial brainstem cavernous angioma with a venous angioma diagnosed by MRI must have been responsible for HFS, because no other possible causes were found during intraoperative observations.

To determine the incidence of fenestrations from multislice spiral computed tomography angiography (CTA) series and to establish the possible association of fenestrations with aneurysms and other vascular diseases of the brain.Four hundred and eighty-six persons who had undergone brachiocephalic artery (BA) CTA for different clinical indications were selected for retrospective analysis. Postprocessor data processing was made to detect extra- and intracranial artery fenestrations. Saccular and fusiform cerebral aneurysms, arteriovenous malformations (AVMs), and venous angiomas were detected in addition to fenestrations. When aneurysms were concurrent with fenestrations, their locations were compared.Among the 486 patients, fenestrations were revealed in 50 (10.3%) cases, of which there were 46 (9.5%) intracranial artery fenestrations and 4 (0.8%) vertebral artery (VA) ones in the extracranial segments. Aneurysms of different locations were found in 163 (33.5%) patients. Venous angiomas of different locations were seen in 9 (1.9%) persons; AVMs were observed in 21 (4.3%) patients. Fifty-four multiple fenestrations were identified. The bulk of fenestrations were located in the area of the anterior communicating artery (ACA) in 28 (51.9%) patients. Aneurysms were found in 11 (22%) patients with fenestrations. Aneurysms were detected in 152 (34.9%) of 436 patients without fenestrations. The incidence of aneurysms was not statistically different in patients with and without fenestrations (p0.05); moreover, aneurysms were somewhat more common in patients without fenestrated vessels. Aneurysm located in the area of fenestration was in only one (9.1%) patient. In other cases aneurysms were located in the vessels having no fenestrations. Aneurysms were relatively frequently encountered in 5 (45.5%) patients with BA fenestration. There was no statistical difference in the incidence of AVMs in the fenestration and non-fenestration groups. Venous angiomas significantly more frequently occurred in patients with fenestrations (p0.05).CTA findings showed fenestrations 10.3% of patients. Fenestrations were located most frequently in the area of ACA and less frequently in the area of BA and middle cerebral artery. Those of other locations, concomitant and extracranial ones, were very rare. According to our data, there was no direct relationship of fenestrations to arterial aneurysms and AMVs. When fenestrations were in the area of BA, the incidence of aneurysms increased up to 45.5%. Venous angiomas occurred more commonly in patients with fenestrations.

A newborn girl with severe diffuse neonatal haemangiomatosis is described. She was treated with high dose systemic corticosteroids and high dose interferon-alpha-2a, but with fatal outcome. A review of the current literature is presented.

Developmental venous anomaly (DVA), also known as venous angioma, is becoming the most commonly encountered intracranial vascular malformation in central nervous system (CNS) imaging1,2. It is thought that DVA develops during development of the venous system which results in the preservation of primitive embryological medullary veins, draining into a single longer vein3. Thrombosis of the collector vein of a DVA is a rare complication leading to cerebral venous infarction (CVI)4 […] Thrombosed developmental venous anomaly Associated with cerebral venous infarct

A 67-year-old male presented with an atypical meningioma arising from the right tentorium associated with a venous angioma in the left cerebellar hemisphere. The venous angioma was detected incidentally during examinations for the brain tumor. The brain tumor was removed completely and the venous angioma followed conservatively. Venous angiomas associated with brain tumors should be followed conservatively, as the clinical significance and surgical indications are unclear.

Cerebral venous angioma is a congenital anomaly of the medullary vein, the vessel that drains into the transparenchymal venous stem. This lesion is also referred to as a developmental venous anomaly. A few reports in the literature have documented developmental venous anomaly—related epilepsy, neurologic deficits, and intracranial hemorrhage. A 3-year-old boy was referred to our hospital after he sustained an afebrile, tonic-clonic, focal seizure of 15 minutes' duration that affected his right arm, leg, and eyebrow. Cerebral digital subtraction angiography showed a bilateral, large periventricular developmental venous anomaly. This report describes the clinical and radiologic findings for this large venous angioma that caused seizures in a child.

As a vascular malformation, venous angioma in the spinal cord is extremely rare. To our knowledge, there are only five case reports in the literature, and it has not been previously reported in the pediatric age group.In this paper, we report on a 3-year-old patient who presented with progressive weakness in his left upper limb. Spinal magnetic resonance imaging (MRI) revealed an epidural cystic mass at the C6-T2 level. The lesion was diagnosed as venous angioma after total removal with laminectomy. Postoperatively, the patient remained symptom free, and no tumor recurrence was confirmed based on MRI at the time of the 18-month follow-up. The clinical, radiological, surgical, and pathological features of this abnormality are discussed, and all six reported cases were reviewed.Venous angiomas should be included in the differential diagnosis of spinal cystic lesions in children. A definitive diagnosis is difficult based on MRI alone. This rare lesion is amenable to surgery, and gross total removal (GTR) is usually achievable due to a well-demarcated dissection plane. A good clinical outcome after GTR can be expected.

A 75-year-old woman was admitted to our hospital because of sudden onset of paresis in her left arm and face. She had untreated hypertension and hyperlipidemia. When she came back home after playing with children in the park, she felt weakness in her left hand. On admission, physical examination revealed that her blood pressure was very high (200/102 mmHg). Only slight weakness in her left arm and left facial palsy were recognized neurologically. An electroencephalogram showed normal findings. Brain CT and MRI revealed a venous angioma near the right central sulcus. Gadolinium-DTPA enhanced MRI showed a group of small radiating veins (so called "the caput medusae sign") connected to the venous angioma. The remaining symptoms decreased with the normalization of blood pressure. It is suggested the intracranial motor tracts of the face and arm in the precentral gyrus are adjacent to the location of this venous angioma. The dilation of venous angioma due to high blood pressure was thought to cause the paresis of face and arm in this patient.

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.