Developmental Venous Anomalies.

Developmental venous anomalies (DVAs) are the most common vascular malformation detected on intracranial cross-sectional imaging. They are generally benign lesions thought to drain normal parenchyma. Spontaneous hemorrhages attributed to DVAs are rare and should be ascribed to associated cerebral cavernous malformations, flow-related shunts, or venous outflow obstruction. Contrast-enhanced MRI, susceptibility-weighted imaging, and high-field MRI are ideal tools for visualizing vessel connectivity and associated lesions. DVAs are not generally considered targets for treatment. Preservation of DVAs is an established practice in the microsurgical or radiosurgical treatment of associated lesions.

Five-Year Serial Brain MRI Analysis of Military Members Exposed to Chronic Sub-Concussive Overpressures.

BACKGROUND: The Canadian Special Operations Forces Command conducts explosives operations and training which exposes members to explosive charges at close proximity. This 5-year longitudinal trial was conducted in follow-up to our initial trial which examined military breachers with MRI and EEG pre and post blast exposure. PURPOSE: To examine brain MRI findings in military personnel exposed to multiple repeated blast exposures. STUDY TYPE: Five-year longitudinal prospective trial. POPULATION: Ninety-two males aged 23-42 with an average of 9.4 years of blast exposure. FIELD STRENGTH/SEQUENCE: 3 T brain MRI/T1-weighted 3D with reconstruction in three planes, T2-weighted, T2-weighted fluid attenuated inversion recovery (FLAIR) 3D with reconstruction in three planes, T2-weighted gradient spin echo (GRE), saturation weighted images, DWI and ADC maps, diffusion tensor imaging. ASSESSMENT: All MRI scans were interpreted by the two neuroradiologists and one neuroradiology Fellow in a blinded fashion using a customized neuroradiology reporting form. STATISTICAL TESTS: Matching parametric statistics represented the number of participants whose brain parameters improved or deteriorated over time. Odds ratio (OR) and 95% confidence intervals (CI) were computed using log regression modeling to determine volume loss, white matter lesions, hemosiderosis, gliosis, cystic changes and enlarged Virchow Robin (VR) spaces. A Kappa (κ) statistic with a 95% CI was calculated to determine rater variability between readers. RESULTS: A significant deterioration was observed in volume loss (OR = 1.083, 95% CI 0.678-1.731, permutation test), white matter changes (OR: 0.754, 95% CI 0.442-1.284, permutation test), and enlargement of VR spaces (OR: 0.775, 95% CI 0.513-1.171). Interrater reliability was low: κ = 0.283, 0.156, and 0.557 for volume loss, white matter changes, and enlargement of VR spaces, respectively. DATA CONCLUSION: There were significant changes in brain volume, white matter lesions, and enlargement of VR spaces. EVIDENCE LEVEL: 2 TECHNICAL EFFICACY: Stage 2.

Management of Symptomatic Hemorrhage From a Developmental Venous Anomaly.

Developmental venous anomalies (DVAs) are clinically benign, low-flow vascular malformations that classically hemorrhage only when associated with a cerebral cavernous malformation. It is very rare for an isolated DVA to hemorrhage. Resection of the DVA is generally contraindicated because of the high risk of venous infarct. We present the case of a large symptomatic hemorrhage stemming from an isolated DVA. The hematoma was evacuated and the hemorrhagic portion of the DVA was resected. This case demonstrates that in rare circumstances, careful resection can successfully treat hemorrhagic DVAs.

Aqueductal developmental venous anomaly causing obstructive hydrocephalus: A case report and review of the literature.

A developmental venous anomaly (DVA) is a venous drainage of the associated parenchyma that is normally asymptomatic. However, a DVA located adjacent to the aqueduct can cause obstructive hydrocephalus by blocking the flow of cerebrospinal fluid. We describe a rare case of obstructive hydrocephalus due to aqueductal stenosis secondary to a DVA. A 43-year-old man presented with sudden bilateral temporal pain during weight training. Using a 3-Tesla scanner, cranial magnetic resonance imaging (MRI) was performed, and hydrocephalus was found with mild enlargement of the lateral and third ventricles. Susceptibility-weighted imaging and postcontrast MRI revealed that the DVA from the bilateral thalami narrowed the orifice of the aqueduct on its drainage route towards the vein of Galen. We assumed that force exerted during weight training may have caused dilation of the anomalous veins, leading to his symptom. A review of the relevant English-language literature yielded only 19 cases of aqueductal stenosis due to DVA. In comparison to these cases, the duration of symptom in our case was extremely short because the patient had a history of ventriculomegaly detected on plain computed tomography and was diagnosed quickly based on the characteristic finding of DVA: the caput medusae appearance.

Eagle jugular syndrome accompanied by de novo brainstem cavernous malformation: a case-based systematic review.

BACKGROUND: Eagle jugular syndrome (EJS), recently identified as a cause of cerebrovascular disease (CVD) due to venous obstruction by an elongated styloid process (SP), is reported here alongside a case of concurrent de novo cerebral cavernous malformation (CCM). This study aims to explore the potential causal relationship between EJS and de novo CCM through a comprehensive literature review. METHOD: Systematic literature reviews, spanning from 1995 to 2023, focused on EJS cases with definitive signs and symptoms and de novo CCM cases with detailed clinical characteristics. Data on the pathophysiology and clinical manifestations of EJS, as well as potential risk factors preceding de novo CCM, were collected to assess the relationship between the two conditions. RESULT: Among 14 patients from 11 articles on EJS, the most common presentation was increased intracranial hypertension (IIH), observed in 10 patients (71.4%), followed by dural sinus thrombosis in four patients (28.6%). In contrast, 30 patients from 28 articles were identified with de novo CCM, involving 37 lesions. In these cases, 13 patients developed CCM subsequent to developmental venous anomalies (43%), seven following dural arteriovenous fistula (dAVF) (23%), and two after sinus thrombosis (6%). In a specific case of de novo brainstem CCM, the development of an enlarged condylar emissary vein, indicative of venous congestion due to IJV compression by the elongated SP, was noted before the emergence of CCM. CONCLUSION: This study underscores that venous congestion, a primary result of symptomatic EJS, might lead to the development of de novo CCM. Thus, EJS could potentially be an indicator of CCM development. Further epidemiological and pathophysiological investigations focusing on venous circulation are necessary to clarify the causal relationship between EJS and CCM.

Microvascular decompression for developmental venous anomaly causing hemifacial spasm: illustrative case.

BACKGROUND: Developmental venous anomaly (DVA) is a rare cause of hemifacial spasm (HFS). The treatment of HFS caused by a DVA varies in the literature and includes medication management, botulinum toxin injections, and microvascular decompression (MVD). OBSERVATIONS: A 64-year-old woman presented with right-sided HFS. Preoperative magnetic resonance imaging showed a DVA in the right inferior pons, with an enlarged segment compressing the facial nerve at its root detachment point prior to drainage into the superior petrosal sinus. MVD was performed, and the facial nerve was decompressed without sacrifice of the vein. Immediately following the procedure, the patient had significantly reduced spasms. The patient became spasm-free 3 months after MVD and maintained spasm freedom for 3 months. Six months after MVD, the patient had a partial return of spasms. At 8 months, the patient continued to have reduced and intermittent spasms in the right orbicularis oculi muscle. LESSONS: MVD for HFS caused by a DVA is a safe procedure and can be effective at reducing spasm frequency and severity.

Cavernous malformations of the central nervous system: An international consensus statement.

Introduction: Cavernous malformations (CM) of the central nervous system constitute rare vascular lesions. They are usually asymptomatic, which has allowed their management to become quite debatable. Even when they become symptomatic their optimal mode and timing of treatment remains controversial. Research question: A consensus may navigate neurosurgeons through the decision-making process of selecting the optimal treatment for asymptomatic and symptomatic CMs. Material and methods: A 17-item questionnaire was developed to address controversial issues in relation to aspects of the treatment, surgical planning, optimal surgical strategy for specific age groups, the role of stereotactic radiosurgery, as well as a follow-up pattern. Consequently, a three-stage Delphi process was ran through 19 invited experts with the goal of reaching a consensus. The agreement rate for reaching a consensus was set at 70%. Results: A consensus for surgical intervention was reached on the importance of the patient's age, symptomatology, and hemorrhagic recurrence; and the CM's location and size. The employment of advanced MRI techniques is considered of value for surgical planning. Observation for asymptomatic eloquent or deep-seated CMs represents the commonest practice among our panel. Surgical resection is considered when a deep-seated CM becomes symptomatic or after a second bleeding episode. Asymptomatic, image-proven hemorrhages constituted no indication for surgical resection for our panelists. Consensus was also reached on not resecting any developmental venous anomalies, and on resecting the associated hemosiderin rim only in epilepsy cases. Discussion and conclusion: Our Delphi consensus provides an expert common practice for specific controversial issues of CM patient management.

Conjoined Pathways: Unraveling the Coexistence of Developmental Venous Anomaly With Arteriovenous Malformation.

Developmental venous anomalies (DVAs) are intracranial vascular malformations typically characterized by their benign nature, often obviating the need for radiological follow-up. These anomalies arise from variations in the standard drainage pattern. While previously deemed congenital, there has been ongoing debate about a developmental component contributing to their etiology. They frequently coexist with other cerebral venous malformations (CVM); however, their association with arteriovenous malformations (AVM) is exceedingly rare. Such mixed malformations pose a therapeutic challenge, necessitating meticulous consideration for appropriate treatment. We present a noteworthy case involving a patient with arteriovenous malformation along with dual developmental venous anomalies, one of which served as the draining vein for the AVM.

Quiz case: a clinical reasoning challenge in the emergency stroke setting.

A right-handed woman in her 80s was admitted to the emergency department 1 h after sudden-onset global aphasia and right-sided hemiparesis. Medical history included arterial hypertension, dyslipidemia, hyperuricemia, aortic stenosis, osteoporosis, and recent pulmonary embolism. Medication consisted of apixaban, bisoprolol, hydrochlorothiazide, allopurinol, fenofibrate, and vitamin D. Vital parameters (blood pressure, heart rate, body temperature, oxygen saturation) and glycemia were all within normal range. Electrocardiogram showed a first-degree atrioventricular block. The patient was promptly transported to the emergency computed tomography (CT) scanner. Non-contrast brain CT revealed a unilateral hyperdensity in the left caudate and lentiform nuclei. No other intracranial lesion was visualized. Laboratory studies were not yet available at the time of imaging. What is your diagnosis?

I saw Medusa's head sign and turned to stone.

Developmental venous anomalies (DVAs) are characterized by many radially oriented medullary veins surrounding a central draining vessel. When the imaging plane is perpendicular to the central vessel, these medullary veins resemble Medusa's head of snakes. Medusa's head sign, or caput medusae, can be appreciated on contrast enhanced CT scans and MRIs of the brain and is highly indicative of a DVA.

Developmental venous anomaly associated with dural arteriovenous fistula: Etiopathogenesis and hemorrhagic risk.

Introduction: Developmental venous anomalies (DVAs) have traditionally been defined as non-pathological congenital lesions. Compared to isolated DVAs, the association of DVAs with arteriovenous shunts seems to have a more adverse clinical connotation. In this review, we describe the association between DVA and dAVF and discuss the hemorrhagic risk. We also advance a hypothesis about the potential de novo formation of a DVA and challenge the dogma about their "developmental" or "congenital" nature. Methods: A systematic review of the literature on the association of DVA and dAVF was performed in accordance with the PRISMA-P (Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols) guidelines. Results: A number of 678 papers was initially identified, but only 9 studies were included in the final qualitative analysis. Most of the patients presented with bleeding (56%), with a median GCS of 14 (range 10-15). In 56% of the cases the DVA had a supratentorial location. Supratentorial DVAs mostly drained in the superior sagittal sinus (80%), while all of infratentorial/combined DVAs drained in deep ependymal veins of the 4th ventricle. All the supratentorial dAVFs drained into the superior sagittal sinus, while the infratentorial/combined dAVFs mostly drained in the jugular bulb, Vein of Rosenthal, or transverse-sigmoid sinuses (75%). Most of the dAVFs were classified as Cognard type IIa + b (67%), while in a smaller number of cases type I (22%) and type V (11%). The dAVF was the target of treatment in each case and most patients underwent endovascular treatment (78%). The dAVF was completely occluded in 78% of cases and no periprocedural complications were reported. Conclusion: The clinical presentation, radiological findings, and treatment outcomes of DVAs and associated dAVFs have been discussed. Despite the general opinion that DVAs are benign congenital lesions, increasing epidemiological and radiological evidence supports a potential acquired origin, and the venous system seem to play a pivotal role in their post-natal genesis and development.

Case report: Delayed outflow obstruction of a DVA: A rare complication of brainstem cavernoma surgery.

Introduction: Developmental venous anomalies (DVAs) are considered variants of normal transmedullary veins. Their association with cavernous malformations is reported to increase the risk of hemorrhage. Expert consensus recommends meticulous planning with MR imaging, use of anatomical "safe zones", intraoperative monitoring of long tracts and cranial nerve nuclei, and preservation of the DVA as key to avoiding complications in brainstem cavernoma microsurgery. Symptomatic outflow restriction of DVA is rare, with the few reported cases in the literature restricted to DVAs in the supratentorial compartment. Case: We present a case report of the resection of a pontine cavernoma complicated by delayed outflow obstruction of the associated DVA. A female patient in her 20's presented with progressive left-sided hemisensory disturbance and mild hemiparesis. MRI revealed two pontine cavernomas associated with interconnected DVA and hematoma. The symptomatic cavernoma was resected via the infrafacial corridor. Despite the preservation of the DVA, the patient developed delayed deterioration secondary to venous hemorrhagic infarction. We discuss the imaging and surgical anatomy pertinent to brainstem cavernoma surgery, as well as the literature exploring the management of symptomatic infratentorial DVA occlusion. Conclusion: Delayed symptomatic pontine venous congestive edema is extremely rare following cavernoma surgery. DVA outflow restriction from a post-operative cavity, intraoperative manipulation, and intrinsic hypercoagulability from COVID-10 infection are potential pathophysiological factors. Improved knowledge of DVAs, brainstem venous anatomy, and "safe entry zones" will further elucidate the etiology of and the efficacious treatment for this complication.

Epilepsy and cerebral developmental venous anomalies (DVAs): A systematic review.

OBJECTIVE: Cerebral developmental venous anomalies (DVAs) are frequent and rarely symptomatic. When symptomatic, DVAs may present with seizures; however, little is known about the characteristics of DVA-related epilepsy. In this systematic review, we aim to describe the clinical and paraclinical features of patients with DVA-related epilepsy. METHODS: This review was registered on PROSPERO (CRD42021218711). We searched the MEDLINE/PubMed and Scopus databases for case reports/series on patients with DVAs complicated by seizures. Studies describing patients with a potentially epileptogenic comorbid lesion close to their seizure focus were excluded. Descriptive statistical analyses were performed to synthetize patient characteristics. The methodological quality of each study was evaluated using a standardized appraisal tool. RESULTS: In total, 66 patients were included from 39 articles. The frontal lobe was the most common location for DVAs. The superior sagittal sinus drained half of the DVAs. Seizures were inaugural in most cases, and the most common manifestations accompanying seizures were headaches. EEG was abnormal in 93% of cases, but only 26% had characteristic epileptic spikes. More than half the patients suffered a medical complication due to their DVA, with hemorrhage and thrombosis being the most common. Refractory seizures were encountered in 19% of individuals. At 12 months of follow-up, 75% of patients were seizure free. Most included studies were at low risk of bias. SIGNIFICANCE: Epilepsy can be a complication of DVAs; these DVAs are mostly frontal or parietal, draining via the superior sagittal sinus or the vein of Galen.

Developmental venous anomaly thrombosis in a patient with coronavirus disease 2019-associated hypercoagulability: illustrative case.

BACKGROUND: Spontaneous thrombosis of a developmental venous abnormality (DVA) is a rare complication associated with hypercoagulability. The objective of this case report is to describe an association between DVA thrombosis and mild coronavirus disease 2019 (COVID-19) infection in a vaccinated patient. OBSERVATIONS: A 28-year-old male with hypertension presented with severe headache and left-sided hemiparesis. Five weeks prior to presentation, the patient experienced mild respiratory symptoms and tested positive for COVID-19. Admission brain computed tomography (CT) showed a large right parieto-occipital intracerebral hemorrhage with surrounding edema. CT venography and catheter angiography showed a thrombosed DVA with associated venous infarction as the hemorrhage etiology. He was treated with decompressive hemicraniectomy, external ventricular drain placement, and systemic anticoagulation. The patient was functionally independent (modified Rankin Scale score, 2) at 4-month follow-up. Hypercoagulability work-up was unremarkable. LESSONS: Delayed DVA thrombosis after the COVID-19 infectious period may represent an association between the infection and a protracted systemic viral-induced hypercoagulable state. The severity of COVID-19 symptomatology does not appear to correlate with risk of DVA thrombosis. Young patients with a recent history of COVID-19 infection who present with venous infarction should be evaluated for an underlying thrombosed DVA.

Awake craniotomy removal of a corticospinal tract developmental venous anomaly hemorrhage: A case report.

Developmental venous anomalies (DVAs) are composed of mature venous vessels that lack malformed or neoplastic elements. Although the hemorrhage risk is considered negligible, some patients may have neurological symptoms attributable to acute infarction or intracranial hemorrhage secondary to thrombosis, in the absence of a coexisting cavernous malformation. We report the case of a 42-year-old patient who presented with acute left-hand paresis secondary to a subcortical hemorrhage. This bleeding originated from a DVA in the corticospinal tract area and was surgically drained through an awake craniotomy. To accomplish this, we used a trans-precentral sulcus approach. After the complete removal of the coagulum, small venous channels appeared, which were coagulated. No associated cavernoma was found. Although the main DVA trunk was left patent, no signs of ischemia or venous infarction were observed after coagulating the small venous channels found inside the hematoma cavity. Two weeks after the procedure, the patient's hand function improved, and he was able to resume desktop work. DVA-associated hemorrhage within the cortico-spinal tract could be safely removed with modern awake mapping techniques. This technique allowed the patient to rapidly improve his hand function.

Cerebellar cavernous angioma associated with developmental venous anomaly presenting as a cerebellopontine angle neoplasm in CT scan.

Association of developmental venous anomaly (DVA) and cavernous malformation (CM) of the posterior fossa is rare. If bleeding occurs, it must be assigned to the CM. Developmental venous anomalies, considered as normal vascular variation, are rarely symptomatic. Indeed, the CMs are true malformations and have a high bleeding potential.

Angioarchitecture of the hemorrhagic developmental venous anomaly with stenosis of the collecting vein and cavernous malformation: a case report.

We herein report a case of developmental venous anomaly (DVA) with venous congestion caused by stenosis of the collecting vein that presented with intracerebral hemorrhage (ICH). A 74-year-old woman was referred to our hospital a few days after the onset of motor aphasia. Computed tomography (CT) and magnetic resonance imaging (MRI) showed ICH in the left frontal lobe. Angiography revealed DVA in the left frontal lobe in the late venous phase. Stenosis of the collecting vein of DVA at the entrance to the superior sagittal sinus was detected and accompanied by cavernous malformation (CM) beside DVA. Cone-beam CT revealed the absence of the left septal vein and hypoplastic transverse caudate veins. The patient was treated by blood pressure management and no additional neurological symptoms were detected. DVA develops to compensate for the absence of pial or deep venous systems, and generally benign and clinically asymptomatic. However, the outflow restriction of DVA causes chronic venous hypertension and the formation of CM. These abnormalities are considered to occur during post-natal life and may result in ICH. The risk of hemorrhage needs to be considered in cases of DVA with restricted venous outflow or CM.

Cerebrofacial venous metameric syndrome type 2+3: face is the index of brain.

We describe a rare case of a 20-year-old man who presented with an extensive facial and orbital venous malformation associated with multiple intracranial venous malformations. The co-existence of cerebrofacial venous malformations points towards a common final pathway in development of these malformations. Our findings are consistent with few previous similar case descriptions. In addition, we describe some novel observations which, to the best of our knowledge, have not been described in the literature. This case reinforces the concept of metameric and segmental distribution of cerebrofacial vasculature, and the aberrations thereof leading to the metameric venous malformations, as proposed by Lasjaunias et al.

Developmental Venous Anomaly Presenting as an Acute Stroke Mimic.

Several mimics of acute ischemic stroke may complicate the decision to administer intravenous thrombolysis. Developmental venous anomalies (DVA) are fairly common variants of normal cerebral vasculature and may occasionally present with acute focal neurologic symptoms. We report a case of DVA presenting with the acute onset of focal neurologic deficits and focal hypodensities on the CT of the brain, resulting in a dilemma regarding whether to administer intravenous tissue-type plasminogen activator (IV tPA) for stroke thrombolysis. Recognition of subtle brain changes on the CT that were inconsistent with an acute ischemic stroke led to further imaging and a definitive diagnosis. Developmental venous anomalies should be considered in the differential diagnosis of acute ischemic stroke.