Developmental Venous Anomaly Research Articles

Unilateral Internuclear Ophthalmoplegia and Upbeat Nystagmus Due to a Rapidly Enlarging Cavernous Malformation and Associated Developmental Venous Anomaly.

Unilateral Internuclear Ophthalmoplegia and Upbeat Nystagmus Due to a Rapidly Enlarging Cavernous Malformation and Associated Developmental Venous Anomaly.

Ultrasonography monitoring with Superb Microvascular Imaging during cerebrovascular surgery

IntroductionUltrasonography (US) is used as a real-time dynamic imaging modality during neurosurgery. A novel Doppler US technique, Superb Microvascular Imaging (SMI), can be used to visualize low-velocity flow of small vessels at high resolution with high frame rates. We visualized vessel flow using this US SMI technique and contrast agent during cerebrovascular surgery. MethodsForty-three patients with an unruptured cerebral aneurysm (control), ischemic and hemorrhagic moyamoya disease, carotid artery stenosis, hemangioblastoma, severe stenosis of the middle cerebral artery, venous angioma, and intracerebral hemorrhage (ICH) underwent neurosurgery with US SMI monitoring using a contrast agent. The diameter, length, and number of penetrating vessels were analyzed in patients with an unruptured cerebral aneurysm (control), moyamoya disease, and ICH. ResultsDiameter and length of cerebral penetrating vessels were significantly increased in patients with moyamoya disease and ICH compared to control patients. The number of penetrating vessels was increased in moyamoya disease patients compared to control and ICH patients. In hemorrhagic moyamoya disease, flow in the penetrating vessels originated from a deep periventricular point and extended to the cerebral surface. Pulsatile cerebral aneurysms during clipping surgery and carotid artery stenosis during carotid endarterectomy were easily identified by SMI. Drastically increased vessel flow in patients with a hemangioblastoma or a venous angioma was observed. ConclusionUsing the US SMI technique and contrast agent, we obtained useful flow information of the vascular disease structure and intracerebral deep small vessels during cerebrovascular surgery. Further quantitative analysis will be informative and helpful for cerebrovascular surgery.

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

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). 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. MVD for HFS caused by a DVA is a safe procedure and can be effective at reducing spasm frequency and severity.

Cerebellar Dysfunctions Associated with a Developmental Venous Anomaly

A developmental venous anomaly (DVA) is a congenital vascular malformation characterized by dilated medullary veins. In the majority of cases, a DVA is asymptomatic and typically, incidentally diagnosed on magnetic resonance imaging and angiography. However, when a DVA is located in the cerebellum, patients may experience symptoms such as hearing disturbance, tinnitus, and dizziness, which are often attributed to mechanical or flow-related issues. In this report, we present the case of a 70-year-old patient who complained of dizziness, along with cerebellar dysfunctions, due to a DVA.

Rupture of Developmental Venous Anomaly

Rupture of Developmental Venous Anomaly

Prevalence of Developmental Venous Anomalies in Association with Sporadic Cavernous Malformations on 7T MRI.

The etiology of sporadic cavernous malformations is not well-understood. However, recent evidence suggests that they may arise from a developmental venous anomaly. The goal of this study was to evaluate the prevalence of developmental venous anomalies associated with sporadic cavernous malformations using 7T MR imaging. We retrospectively identified patients with a sporadic cavernous malformation imaged with 7T MR imaging between August 2019 and July 2022. Two raters determined whether a developmental venous anomaly was associated with each malformation. The study included 59 patients with a total of 61 cavernous malformations. Of the sixty-one, 44 (72%) had an associated developmental venous anomaly. An associated anomaly was most common for cavernous malformations in the brainstem (88%) compared with the cerebral hemispheres or cerebellum (60%-67%). By means of high-quality 7T imaging, most patients with a sporadic cavernous malformation were found to have an associated developmental venous anomaly. These findings support the hypothesis that cavernous malformations may arise secondary to hemodynamic abnormalities.

Clinical case of 9-month observation of orbital venous varicose with intraluminal thrombus using radiologic methods

A rare clinical case of thrombosis of varicose superior ophthalmic vein in combination with a unilateral venous anomaly of the subcortical region of the brain with successful antithrombotic therapy is presented. Varicose veins of the superior ophthalmic vein are rare (2% of all orbital formations) and are a risk factor for thrombosis.Material and methods. A 41-year-old patient with verified thrombosis of unilateral orbital venous varicose veins was observed for 9 months. Initially, MSCT of the brain and MSCT angiography were performed, then dynamic monitoring was carried out by performing MRI of the brain and MR venography, and finally an ultrasound examination of the orbit was performed.Description of a clinical case. The clinical presentation consisted of a slight non-pulsatile unilateral exophthalmos, mild ptosis, periorbital soft tissue edema, subconjunctival hemorrhage, paraorbital hematoma, and venous dysfunction in the fundus. MSCT and MSCT angiography revealed a thrombus inside the sac of orbital venous varicose veins. The disease was probably secondary in nature, given that it was combined with venous angioma of the subcortical region on the same side. During 9 months of observation, to monitor the effectiveness of therapy, MRI was performed three times before and after contrast enhancement, which showed a gradual decrease in exophthalmos, the size of the thrombus and the varicocele sac, which was accompanied by a gradual improvement in the clinical picture until subjective recovery.Conclusion. MRI was as good an imaging modality as MSCT, and, in our opinion, more preferable due to the absence of radiation exposure and the need for iodine contrast agent.

Approaches to the Middle Cerebellar Peduncle for Resection of Pontine Cavernomas.

The expanded retrosigmoid approach with splitting of the horizontal cerebellar fissure provides a more direct and shorter route for central and dorsolateral pontine lesions while minimizing retraction of tracts, nuclei, and cerebellum. 1-4. The middle cerebellar peduncle is partially covered by the petrosal surface of the cerebellum. The horizontal cerebellar fissure (petrosal fissure) divides the petrosal surface of the cerebellar hemisphere into superior and inferior parts. Splitting the petrosal fissure separates the superior and inferior petrosal surfaces and exposes the posterolateral middle cerebellar peduncle (posterior and lateral to the root entry zone of CN5). 1-4. Expanded retrosigmoid craniotomy is performed, including unroofing of the sigmoid sinus; petrosal fissure is split to expose the posterolateral middle cerebellar peduncle; entry point for resection of the cavernoma is identified; nims stimulator stimulator is used to confirm the absence of tracts and nuclei; myelotomy is performed; and cavernoma and its draining vein (but not the developmental venous anomaly) are removed using a combination of traction and countertraction against gliotic plane. Wide splitting of the horizontal cerebellar fissure minimizes retraction or resection of the cerebellum and offers the best angle of attack. Knowledge of brainstem anatomy and use of intraoperative navigation are critical to avoid complications. Far lateral through the middle cerebellar peduncle is a variant that can be used to resect pontine cavernomas if a caudocranial trajectory is preferred.The patient consented to the procedure and to the publication of her image.

Abstract 113: A Curious Case of Macrovascular Arteriovenous Shunting in Glioblastoma Multiforme

Introduction Arteriovenous shunting has been described in glioblastoma multiforme (GBM), also known as WHO grade 4 astrocytoma. This case report demonstrates the diagnostic dilemma of GBM, which was subsequently found to have macrovascular arteriovenous shunting. The lesion was initially treated as an ischemic stroke for months. Methods A 65‐year‐old‐man with a past medical history of hypertension, hyperlipidemia, and diabetes presented to a community hospital in February 2023 with worsening left‐sided weakness. The patient started having left‐sided weakness around December 2023, but no work‐up had been completed. The exam was notable for spastic hemiparesis. Results At the time of initial presentation, a non‐contrasted head computed tomography (NCHCT) was interpreted as normal. Magnetic resonance imaging (MRI) of the brain showed diffusion restriction and fluid attenuated inversion recovery (FLAIR) hyperintensity in the right corona radiata and was interpreted as a subacute right corona radiata and gangliocapsular infarct. Computed tomography angiogram (CTA) of the head and neck was unremarkable. He was discharged on aspirin and a high‐intensity statin. He re‐presented in April 2023 with sepsis and was found to have multiple pressure ulcers; his left‐sided weakness had worsened despite a course of inpatient rehabilitation. A repeat NCHCT demonstrated hyperdensity within the suspected infarct, interpreted as hemorrhagic transformation. The next day, he was found to be difficult to arouse and dysarthric. He was subsequently transferred to our institution for further management. On arrival, a CTA of the head and neck was performed, which suggested that the previously described hyperdensity was possibly a venous varix, and a thrombosed developmental venous anomaly or arteriovenous malformation was suggested. The patient then underwent an MRI brain with and without contrast, which noted that there was an increase in size of a heterogeneously enhancing mass centered in the posterior right basal ganglia/corona radiata with extension into right thalamus, right cerebral peduncle, and posterior right body of corpus callosum. The MRI findings were most consistent with GBM. Given the diagnostic uncertainty, the patient underwent a cerebral angiogram, which demonstrated a hypervascular posterior right basal ganglia mass with arteriovenous shunting from the middle cerebral artery to the internal cerebral veins. Shortly after, the patient underwent a brain biopsy, which demonstrated GBM (IDH‐wildtype, WHO grade 4). The patient was ultimately transitioned to hospice. Conclusion This case highlights the presence of macrovascular shunting in GBM. Intratumoral arteriovenous shunting in GBM has been described, and when present, can lead to delay in treatment due to the broadened differential diagnosis. This patient was first treated for an ischemic stroke with subsequent hemorrhagic transformation. Further imaging suggested a venous varix, thrombosed developmental venous anomaly, or arteriovenous malformation, but the combination of the cerebral angiogram and MRI led to the definitive diagnosis in a patient with a short survival period.

뇌교 및 연수 해면상혈관종에 대한 telovelar 접근법의 수술 결과 및 뉘앙스

Background: Brainstem cavernous malformations (CMs) are characterized by dilated sinusoidal channels related to capillary telangiectasia and developmental venous anomalies. Brainstem CM hemorrhages present with focal neurological symptoms, according to their invasive lesions. Surgical removal should be considered for symptomatic and recurrent bleeding of brainstem CMs. Despite the risk of surgery with highly eloquent tissue, surgical removal should be performed to protect patients from stepwise decline due to recurrent hemorrhage. The approaches for treating brainstem CMs are retrosigmoid, far lateral, midline suboccipital with or without telovelar, orbitozygomatic, and supracerebellar-infratentorial. Pontine and medullary CMs are approached via retrosigmoid, far lateral, and suboccipital craniotomies, with or without the telovelar approach. 
 Materials and Methods: The telovelar approach is the standard approach used for pontine brain-stem CMs. Patients with brainstem CMs treated using the telovelar approach were enrolled from three institutions. 
 Results: All three patients had no further neurological deficits after surgery. 
 Conclusions: The surgical results suggest that the telovelar approach for pons and pontomedul-lary junction brainstem CMs is safe and effective.

Neurologiniai ir psichiatriniai simptomai, sukelti įgimtos veninės anomalijos

One of the most common vascular brain malformations is developmental venous anomaly (DVA). This anomaly is usually considered to be asymptomatic but there are some reports in the literature about DVA causing neurological symptoms due to neurovascular compression, obstructive hydrocephalus, venous infarction, or intracerebral hemorrhage.There are no publications on DVA causing psychiatric symptoms. This paper presents a clinical case of DVA in which the patient develops a mood disorder along with neurological symptoms that are typical for a parietal brain lesion. Along with this clinical case, a review of literature is presented which includes classification of vascular brain malformations, prevalence of DVA, clinical manifestations, and characteristics in imaging studies. The article also reviews the functions of the parietal lobe, discusses mood disorders possibly related to parietal lesions, and briefly introduces cyclothymic disorder.

Developmental venous malformation: A “Do-Not-Touch’’ lesion

The most common slow-flow venous malformation in the brain are Developmental Venous Anomalies (also known as DVAs).

Neuro-image: developmental venous anomaly and central vein sign in multiple sclerosis.

Neuro-image: developmental venous anomaly and central vein sign in multiple sclerosis.

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.

Assigning developmental venous anomaly thrombosis to SARS-CoV-2 only when the causal connection has been proven.

Assigning developmental venous anomaly thrombosis to SARS-CoV-2 only when the causal connection has been proven.

Arterial Spin-Labeling MR Imaging for the Differential Diagnosis of Venous-Predominant AVMs and Developmental Venous Anomalies.

Venous-predominant AVMs are almost identical in appearance to developmental venous anomalies on conventional MR imaging. Herein, we compared and analyzed arterial spin-labeling findings in patients with developmental venous anomalies or venous-predominant AVMs, using DSA as the criterion standard. We retrospectively collected patients with either DVAs or venous-predominant AVMs, each available on both DSA and arterial spin-labeling images. Arterial spin-labeling imaging was visually assessed for the presence of hyperintense signal. CBF measured at the most representative section was normalized to the contralateral gray matter. The temporal phase of developmental venous anomalies or venous-predominant AVMs was measured on DSA as a delay between the first appearance of the intracranial artery and the lesion. Correlation between the normalized CBF and the temporal phase was evaluated. Analysis of 15 lesions (13 patients) resulted in categorization into 3 groups: typical venous-predominant AVMs (temporal phase, <2 seconds), intermediate group (temporal phase between 2.5 and 5 seconds), and classic developmental venous anomalies (temporal phase, >10 seconds). Arterial spin-labeling signal was markedly increased in the typical venous-predominant AVM group, while there was no discernible signal in the classic developmental venous anomaly group. In the intermediate group, however, 3 of 6 lesions showed mildly increased arterial spin-labeling signal. The normalized CBF on arterial spin-labeling and the temporal phase on DSA were moderately negatively correlated: r(13) = 0.66, P = .008. Arterial spin-labeling may predict the presence and amount of arteriovenous shunting in venous-predominant AVMs, and using arterial spin-labeling enables confirmation of typical venous-predominant AVMs without DSA. However, lesions with an intermediate amount of shunting suggest a spectrum of vascular malformations ranging from purely vein-draining developmental venous anomalies to venous-predominant AVMs with overt arteriovenous shunting.

Developmental venous anomalies look like a spider’s feet

Developmental venous anomalies look like a spider’s feet

Symptomatic Developmental Venous Anomaly: State-of-the-Art Review on Genetics, Pathophysiology, and Imaging Approach to Diagnosis.

Developmental venous anomalies (DVAs) are the most common slow-flow venous malformation in the brain. Most DVAs are benign. Uncommonly, DVAs can become symptomatic, leading to a variety of different pathologies. DVAs can vary significantly in size, location, and angioarchitecture, and imaging evaluation of symptomatic developmental venous anomalies requires a systematic approach. In this review, we aimed to provide neuroradiologists with a succinct overview of the genetics and categorization of symptomatic DVAs based on the pathogenesis, which forms the foundation for a tailored neuroimaging approach to assist in diagnosis and management.

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

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. 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. 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. 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.

Mesencephalic developmental venous anomaly causing obstructive hydrocephalus: illustrative case.

Developmental venous anomalies (DVAs) are congenital anatomical variants of the normal deep parenchymal veins. DVAs are occasionally found incidentally on brain imaging, and most cases are asymptomatic. However, they rarely cause central nervous disorders. Herein, a case of mesencephalic DVA that caused aqueduct stenosis and hydrocephalus and discuss its diagnosis and treatment is reported. The patient was a 48-year-old female who presented with depression. Computed tomography and magnetic resonance imaging (MRI) of the head revealed obstructive hydrocephalus. Contrast-enhanced MRI revealed an abnormally distended linear region with enhancement on the top of the cerebral aqueduct, which was confirmed as a DVA by digital subtraction angiography. An endoscopic third ventriculostomy (ETV) was performed to improve the patient's symptoms. Intraoperative endoscopic imaging showed obstruction of the cerebral aqueduct by the DVA. This report describes a rare case of obstructive hydrocephalus caused by DVA. It highlights the usefulness of contrast-enhanced MRI for diagnosing cerebral aqueduct obstructions due to DVAs and the effectiveness of ETV as a treatment option.