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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.

Introduction: Cerebral venous sinus thrombosis (CVST) is a rare but serious condition in both adults and children. Risk factors include thrombophilias, dehydration, and certain inherited conditions like sickle cell trait (SCT). We present a case of CVST in a pediatric patient with SCT to highlight key considerations in diagnosis and management. Case Presentation: A 14-year-old male with SCT presented with worsening headache and vomiting after prolonged sun exposure and dehydration during athletic camp. Imaging revealed right occipital hemorrhage, hydrocephalus, right CSVT, and bilateral cerebellar developmental venous anomalies. Hypercoagulability testing was normal. Diagnostic evaluation included computed tomography, magnetic resonance imaging, MR venography (MRV), and hypercoagulability testing. The patient was treated with an external ventricular drain, platelet transfusion, and anticoagulation. Management also involved hydration, platelet transfusion, supportive care, and multidisciplinary follow-up. Follow-up MRV showed recanalization. Conclusion: This case highlights SCT as a potential CVST risk factor. Timely recognition, evaluation of precipitants like dehydration, supportive care including anticoagulation, and multidisciplinary management are important. An individualized approach is needed to balance thrombosis recurrence and bleeding risks. Patients with SCT require education on risks and prompt evaluation of neurological symptoms to allow early diagnosis and care of CVST. [ABSTRACT FROM AUTHOR]

Objective To explore clinical characteristics and treatment of mixed cerebral cavernous malformations (CCM) with developmental venous anomaly (DVA). Methods The clinical data of a patient with mixed CCM with DVA admitted to Department of Neurosurgery, Peking Union Medical College Hospital in December 2021 were analyzed retrospectively. Result The patient's epilepsy was controlled after resection of cavernous hemangioma and venous malformation in the medial posterior part of the left temporal lobe, and there was no recurrence of postoperative symptoms during the perioperative period. Conclusion Mixed CCM with DVA have no specific manifestations requiring complex therapeutic strategies. [ABSTRACT FROM AUTHOR]

: The intrinsic lesions of the brainstem in children lead to a variety of diseases. Although their differentiation is important; however, their diagnosis is difficult via neuroradiological imaging alone. Herein, we report a pediatric case of a developmental venous anomaly (DVA) sited at the brain stem. The patient’s initial head MRI revealed an intrinsic lesion with ill-defined and brush-like contrast enhancements, which was primarily suspected to be a brain tumor. However, the atypical clinical course of the patient and neuroimaging findings such as the rapid exacerbations and remissions in a short time period, and rack of lesion volume effect observed in MRI, refrain us from proceeding with highly invasive examination and initiating any type of treatment including chemotherapy, radiotherapy and ／ or administration of steroids. Consequently, a complete recovery of symptoms and improvement in neuroimaging findings were achieved during the natural course of the disease. Retrospectively, MRI with contrast mater at the initial visit revealed a DVA traversing across the medulla oblongata, suggesting that the this case’s pathophysiology was based on a venous malformation of the medulla oblongata with stagnant outflow and stenosis, causing increased venous pressure and contributing to the clinical symptoms and findings on neuroradiological imaging. In this study, we report this extremely rare case of DVA with a literature review. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. • Medusa's head sign is useful for diagnosing developmental venous anomalies (DVAs) and can be appreciated on MRI and CT • DVAs are a common cerebral vascular malformation characterized by intramedullary veins converging on a large draining vein • DVAs are part of the normal venous drainage pattern and are most often asymptomatic, but complications can occur [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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.

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.

Vascular malformations of the brain are aberrant vascular connections that are most likely congenital. Cerebral vascular malformations are the umbrella term for multiple conditions, each with different symptoms, signs, and imaging characteristics. These conditions are (1) Arteriovenous malformations, abnormal arteries, and veins; (2) Dural arteriovenous fistula; (3) Developmental venous anomalies; (4) Cavernous malformations, enlarged blood-filled spaces; (5) Cavernous angiomas, abnormal veins; (6) capillary telangiectasias, enlarged capillary-sized vessels; (7) vein of Galen malformations; and (8) mixed malformations. It is important to study the complications of each and their mimics to make an accurate diagnosis. Various imaging features of different vascular malformations seen on MRI are discussed that which would aid in diagnosis and planning management. [ABSTRACT FROM AUTHOR]

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? [ABSTRACT FROM AUTHOR]

Developmental venous anomaly (DVA) is an anatomical variation of the intracranial venous system, usually without clinical repercussion. In most cases, DVAs are incidentally diagnosed and should be considered as benign conditions. In rare circumstances, DVAs may become symptomatic due to mechanical or flow-related etiologies. The authors present three cases of symptomatic arterialized DVAs: a 28-year-old male with hematoma at the splenium of the corpus callosum and intraventricular hemorrhage, a 53-year-old male patient with a history of epileptic seizures starting recently, and a 25-year-old male patient, previously healthy who started with persistent headaches and hemosiderin deposition in brain parenchyma. These rare cases of arterialized DVAs are conditions that can cause symptoms or show more aggressive behavior with bleeding.

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 theDVA as key to avoiding complications in brainstemcavernomamicrosurgery. 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, intraoperativemanipulation, and intrinsic hypercoagulability from COVID-10 infection are potential pathophysiological factors. Improved knowledge of DVAs, brainstemvenous anatomy, and "safe entry zones" will further elucidate the etiology of and the effcacious treatment for this complication. [ABSTRACT FROM AUTHOR]

IntroductionDevelopmental 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.CaseWe 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.ConclusionDelayed 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.

IntroductionDevelopmental 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.MethodsA 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.ResultsA 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.ConclusionThe 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.

Background: Arteriovenous malformation (AVM) and developmental venous anomaly (DVA) rarely coexist. Developing a surgical strategy to treat this co-occurrence is difficult due to the unclear pathogenesis. We report the use of super-selective digital subtraction angiography (DSA) and Three-dimensional (3D) rotational digital subtraction venography (DSV) to develop a surgical strategy for complex AVM draining into a DVA., Case Description: A 58-year-old woman presented with left hemiparesis and unconsciousness. Plain and contrast computed tomography showed a right frontal subcortical hematoma and a heterogeneous contrast lesion anterior to the hematoma, leading to a dilated vessel. The hematoma was removed due to worsening unconsciousness. DSA revealed a right frontal AVM of Spetzler-Martin grade 2 with superficial drainage into a DVA, and 3D-DSV revealed that the intermediate part of the DVA involved normal parenchyma. Interventional transarterial embolization and surgical nidus removal were planned. Preoperative super-selective DSA showed two medullary veins draining from the AVM into the DVA. Thus, we decided to separate the two medullary veins from the nidus. Postoperative angiography revealed complete removal of the AVM and preservation of the DVA., Conclusion: Treating a complex AVM draining into a DVA is challenging; surgeons have to remove only the AVM portion and preserve the DVA. Super-selective DSA and 3D rotational DSV were performed to develop the surgical strategy., Competing Interests: There are no conflicts of interest., (Copyright: © 2024 Surgical Neurology International.)

Amaç: Kavernom ve gelişimsel venöz anomali (GVA) birlikteliği en sık görülen miks tip vasküler malformasyondur. Bu çalışmada kavernom gelişimine etki edebilecek GVA vasküler özellikleri ve epidemiyolojik özellikler araştırılmıştır. Gereç ve Yöntem: Ocak 2016- Şubat 2019 tarihleri arasında hastanemizde kontrastlı beyin manyetik rezonans (MR) görüntüleme yapılan hastalar veri tabanından taranarak bu görüntülerde kavernom ve GVA’sı bulunan hastalar tespit edildi. Bu hastalara ait yaş, cinsiyet, lezyonların yerleşim yeri, kavernom boyutu, GVA’nın tek kesitteki maksimum damar sayısı, toplayıcı venin drenaj yönü ve drenaj yeri belirlendi. Bulgular: Çalışmaya dahil edilen 28 hastanın 15’i kadın (%56) olup, yaş ortalaması 45’ti (17-82). Supratentoryal yerleşim sık olmakla birlikte infratentoryal olanlarda istatistiksel olarak kavernom sayısında anlamlı artış görüldü. Subepandimal drenaj vakaların %61’inde mevcuttu ve bu oran erkeklerde anlamlı düzeydeydi. Z ekseninde toplayıcı ven drenajı yukarıdan aşağı yönde olan GVA’larda eşlik eden kavernom boyutları daha büyüktü. Sonuç: İnfratentoryal yerleşimli, subepandimal vene drene olan GVA’larda kavernom görülme olasılığı daha yüksek olup hastaların MR ile takibi oldukça önemlidir.

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.

An intimate understanding of dynamic angioarchitectural development of vascular malformations involving the intracranium may provide mechanistic insight into the incipient pathogenesis of these lesions. The authors report two patients harboring cerebral developmental venous anomaly (DVA) and three patients harboring sporadically developing cerebral arteriovenous malformations (AVM) in whom the internal cerebral vein (ICV) represented the chief venous egress route. Onyx embolization successfully achieved complete obliteration in all patients harboring cerebral AVMs. Two female patients presenting with a chief complaint of chronic headaches was found to harbor deeply situated DVA draining via the lateral group of direct lateral vein (DLV) egress via the ICV. Three female patients presenting with chronic headaches or intraparenchymal hemorrhage were found to possess cerebral AVMs deriving arterial feeders from the anterior cerebral arteries, with major venous drainage into the ICV via the direct lateral veins or terminal vein. Common drainage of DVAs and AVMs may indicate a common originate genesis. This pattern of venous drainage in the context of seeking to develop a cohesive and coherent model illumining our understanding of the mechanistic incipient pathogenesis of AVMs. [ABSTRACT FROM AUTHOR]

A developmental venous anomaly (DVA) sometimes causes intracerebral hemorrhage, not subarachnoid hemorrhage (SAH). A 45-year-old man had sudden, severe headache. Computed tomography (CT) scan demonstrated diffuse SAH. No cerebral aneurysm or other arterial lesion, except DVA in the left frontal area, was observed after repeated digital subtraction angiography. The bleeding source was diagnosed as the DVA itself. He was treated conservatively. Two weeks later, CT and magnetic resonance imaging scans showed only residual faint hematoma at the DVA’s location. Five years later, no aneurysm or cavernous angioma was observed. Therefore, we encountered a very rare case of SAH due to DVA.

Abstract Background Since the nineteenth century, a great variety of crossed brainstem syndromes (CBS) have been described in the medical literature. A CBS typically combines ipsilateral cranial nerves deficits to contralateral long tracts involvement such as hemiparesis or hemianesthesia. Classical CBS seem in fact not to be so clear-cut entities with up to 20% of patients showing different or unnamed combinations of crossed symptoms. In terms of etiologies, acute brainstem infarction predominates but CBS secondary to hemorrhage, neoplasm, abscess, and demyelination have been described. The aim of this study was to assess the proportion of CBS caused by a bleeding episode arising from a brainstem cavernous malformation (BCM) reported in the literature. Case presentation We present the case of a typical Foville syndrome in a 65-year-old man that was caused by a pontine BCM with extralesional bleeding. Following the first bleeding episode, a conservative management was decided but the patient had eventually to be operated on soon after the second bleeding event. Discussion A literature review was conducted focusing on the five most common CBS (Benedikt, Weber, Foville, Millard-Gubler, Wallenberg) on Medline database from inception to 2020. According to the literature, hemorrhagic BCM account for approximately 7 % of CBS. Microsurgical excision may be indicated after the second bleeding episode but needs to be carefully weighted up against the risks of the surgical procedure and openly discussed with the patient. Conclusions In the setting of a CBS, neuroimaging work-up may not infrequently reveal a BCM requiring complex multidisciplinary team management including neurosurgical advice.

Cavernous malformations are angiographically occult vascular malformations. They are often associated with a developmental venous anomaly through poorly understood mechanisms. We present an unusual case of a gradually enlarging cavernous malformation associated with a developmental venous anomaly with arteriovenous shunting, suggesting venous hypertension or reflux as a potential cause of progressive growth. [ABSTRACT FROM AUTHOR]

Intracranial mixed vascular malformations (MVMs) are defined as any combination of a developmental venous anomaly (DVA), cerebral cavernous malformation (CCM), capillary telangiectasia (CTG), or arteriovenous malformation (AVM) within a single, contiguous lesion. However, most MVMs described in the literature contain only 2 pathologically discrete malformations; juxtaposition of 3 or more abnormalities in a single lesion remains exceedingly rare. We present the case of a 19-month-old female with new onset focal seizures and a 4-cm right basal ganglia lesion initially believed to be an embryonal neoplasm. She subsequently underwent gross total resection (GTR) of the lesion via a transsylvian-transinsular approach. Intraoperatively, the lesion appeared to be heterogenous and highly vascular, with areas of purplish-gray friable tissue. Pathology confirmed the lesion to be a MVM containing a CCM, CTG, and a DVA. This appears to be the first reported case of such a lesion confirmed on pathology in the literature. [ABSTRACT FROM AUTHOR]

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 [ABSTRACT FROM AUTHOR]

Vascular malformations are atypical irregular anomalies of vein with a prevalence of 3% of the general population. Most of them are asymptomatic with an inconclusive etiology and are located on other routine investigations. We present a case report of intraoral manifestations of VA in a 23-year-old male patient with a complaint of a swelling on his right side of cheek for a period 8 months with gradual increase in size and associated symptoms. The lesion was diagnosed as venous angioma following an MRI scan and treated with sclerotherapy. The lesion responded to sclerotherapy and spontaneous remission was evident. No recurrence was noted during 12 months follow up. Early detection is necessary to determine the clinical behaviour and potential dentoalveolar complications of the lesion for timely diagnosis and rapid treatment and can avoid fatal events. [ABSTRACT FROM AUTHOR]

Background: Craniotomy is required for the removal of brainstem cavernous malformations (CMs) with repeated hemorrhage, and this condition is often complicated by an accompanying developmental venous anomaly (DVA). However, a DVA of the brainstem or cerebellum with drainage penetrating the pons is an exceptional finding., Observations: A 57-year-old man presented with double vision. Computed tomography revealed progressive enlargement of the hemorrhage in the dorsal pons. Contrast-enhanced magnetic resonance angiography revealed an expanded transverse vessel penetrating the center of the pons in contact with the CM. Digital subtraction angiography revealed that the DVA, comprising the expanded transpontine vein and some cerebellar medullary veins acting as normal venous drainage, coexisted with the CM. By utilizing the angioarchitecture and intraoperative neuronavigation system data, electrophysiological mapping, and indocyanine green videoangiography, complete removal of the CM was accomplished while preserving the DVA and brain function., Lessons: This study presents the intraoperative images of an expanded transpontine vein as a DVA, which has never been depicted in a live patient before, accompanied by a CM in the dorsal portion of the pons. https://thejns.org/doi/10.3171/CASE24314.

Developmental venous anomaly (DVA) is considered a congenital vascular anatomic variation with normal draining function. Limited cases of DVA with arteriovenous malformation (AVM) in the territory of DVA have been reported, and the etiology of de novo AVM formation remains unknown and speculative. In this case, it is continuously observed that the inappropriate radiosurgery involving the DVA draining veins led to the disruption of DVA radicles and the secondary formation of de novo AVM and hemodynamic aneurysm. This sequential process exhibited in the present case verified that outflow disturbance underlay the potential etiology of AVM formation drained by adjoining DVA. Meanwhile, it also manifested the importance of preserving the integrity of the collecting veins when treating the DVA by any means. [ABSTRACT FROM AUTHOR]

Deep-seated brainstem cavernous malformations (BSCMs) pose a particular challenge for brainstem entry intraoperatively and their treatment can require comprehensive application of neuronavigation, electrophysiological brainstem mapping and monitoring, and full knowledge of safe brainstem entry zones. In the present report, we have introduced a supplementary technical trick for localizing a hidden tiny lesion inside the brainstem when a developmental venous anomaly (DVA) is present on the brainstem surface. A retrospective analysis of a 74-case cohort treated surgically from January 2011 to December 2019 was conducted. We identified 11 patients (14.9%) whose deep-seated BSCMs were exposed and removed following a brainstem surface DVA path as a supplementary technical trick. We have presented 2 typical cases to illustrate the operative nuances. Of the 11 patients, 5 were male and 6 were female. Their average age was 38.0 ± 14.0 years (range, 15–62 years). Most BSCMs were located in the pons (n = 5; 45.5%), followed by the pontomesencephalic area (n = 3; 27.3%), midbrain (n = 2; 18.2%), and medulla oblongata (n = 1; 9.1%). All BSCMs were successfully located and completely removed. In 5 cases, the DVA was impaired after lesion removal (45.5%). However, no aggravated postoperative brainstem edema occurred in any of the 11 patients. After 3.6 ± 2.0 years of follow-up (2 patients were lost to follow-up; follow-up rate, 81.8%), no rebleeding was found, and the modified Rankin scale score of the patients had improved from 2.7 ± 1.1 preoperatively to 1.7 ± 0.9 at follow-up (P = 0.031). The presented method could help surgeons trace deep-seated BSCMs with minimal brainstem parenchyma impairment, avoiding unnecessary aggressive exploration. [ABSTRACT FROM AUTHOR]

Objective: Cavernoma and DVA (Developmental venous anomalies) association is the most common mixed type vascular malformation. In this study, vascular and epidemiological features of DVA that may affect the development of cavernoma were investigated. Material and Methods: Patients with cavernoma and DVA were detected by scanning the patients with contrast-enhanced brain MRI in our hospital between January 2016 and February 2019 from the database. Age, gender, location of lesions, cavernoma size, maximum number of vessels in a single section of DVA, drainage direction and drainage location of the collecting vein of these patients were determined. Results: Of the 28 patients included in the study, 15 were female (56%) and the average age was 45 (17-82). Although supratentorial location was frequent, a statistically significant increase was observed in infratentorial ones. Subependymal drainage was present in 61% of the cases and this rate was significant in men. Cavernoma sizes were larger in DVA with top-down drainage of the collecting vein in the Z-axis. Conclusion: The possibility of cavernoma is higher in DVA that is located infratentorially and draining into the subependymal vein, and the follow-up of patients with MRI is very important. [ABSTRACT FROM AUTHOR]

Hydrocephalus is a pathological buildup of cerebrospinal fluid within the ventricles leading to ventricular enlargement out of proportion to sulci and subarachnoid spaces. Developmental venous anomaly is a common benign and usually asymptomatic congenital cerebrovascular malformation. Hydrocephalus caused by aqueductal developmental venous anomaly is extremely rare. We describe a case of a 47-year-old man who presents with short-term memory impairment who was found to have a developmental venous anomaly draining bilateral medial thalami through a common collector vein that causes aqueductal stenosis and obstructive hydrocephalus.

Abstract Background There are no established theories regarding the role of the cerebellum in dystonia. We report a case of focal limb dystonia secondary to a vasogenic edema of the dentate nucleus caused by a symptomatic developmental venous anomaly. Case presentation A 44-year-old woman presented with sudden onset dystonia in her left arm for 1 week. Brain imaging revealed vasogenic edema in the deep white matter of the left cerebellar hemisphere, including the left dentate nucleus, secondary to a developmental venous anomaly. 18F-fluorodeoxyglucose positron emission tomography images showed hypometabolism in the corresponding cerebellar deep nuclei without the involvement of other brain regions. She was treated with a steroid. At the one-month follow-up, computed tomography scan demonstrated remission of the cerebellar edema, which was thought to be the cause of dystonia. Conclusions This case demonstrates that the cerebellum has an important role in the development of dystonia. Further studies are needed to elucidate the relationship between dystonia and cerebellar dysfunction.

Constitutional mismatch repair deficiency syndrome (CMMRD) is a rare cancer-predisposition syndrome associated with a high risk of developing a spectrum of malignancies in childhood and adolescence, including brain tumours. In this report, we present the case of an 8-year-old boy with acute headache, vomiting and an episode of unconsciousness in whom brain imaging revealed a high-grade glioma (HGG). The possibility of an underlying diagnosis of CMMRD was suspected radiologically on the basis of additional neuroimaging findings, specifically the presence of multiple supratentorial and infratentorial developmental venous anomalies (DVAs) and malformations of cortical development (MCD), namely, heterotopic grey matter. The tumour was debulked and confirmed to be a HGG on histopathology. The suspected diagnosis of CMMRD was confirmed on immunohistochemistry and genetic testing which revealed mutations in PMS2 and MSH6. The combination of a HGG, multiple DVAs and MCD in a paediatric or young adult patient should prompt the neuroradiologist to suggest an underlying diagnosis of CMMRD. A diagnosis of CMMRD has an important treatment and surveillance implications not only for the child but also the family in terms of genetic counselling. [ABSTRACT FROM AUTHOR]

Purpose: Developmental venous anomalies (DVAs) are extreme anatomical venous variations formed by multiple radiating medullary veins, which converge centripetally into a single collecting vein. Their coexistence with symptomatic cavernous malformations (CMs) has been reported in the literature. The aim of this study was to assess the characteristics of DVAs using MRI. Methods: A total of 6948 head MRIs of adult Caucasian patients were retrospectively analyzed to determine the number and locations of DVAs. We collected the data on the termination of the collecting vein, the prevalence of DVA-related CMs, and MRI FLAIR signal-hyperintensity corresponding to the location of the DVA. Results: At least one DVA was identified in 7.46% of the patients. The prevalence decreased with age, with a Pearson correlation coefficient of − 0.7328. A total of 599 DVAs were identified. Multiple DVAs were found in 10.92% of the patients with DVAs. The DVAs were identified more often in the supratentorial region (73.12%, p < 0.0001), and the most common location was the frontal lobe (35.23%). The collecting vein usually drained into the superficial cerebral veins (68.78%). CMs were observed in 4.14% of the patients with DVAs, and the prevalence showed a positive correlation with age. Signal-intensity abnormalities were identified in the vicinity of 5.18% DVAs. Conclusion: Knowledge about characteristics of DVAs and associated anomalies is essential for neuroradiologists and neurosurgeons. The large number of currently available diagnostic studies enables us to assess anatomical variants on a great number of subjects. [ABSTRACT FROM AUTHOR]

Sinus pericranii is a rare vascular anomaly characterized by an abnormal communication between the intra‐ and extracranial venous systems through a calvarial defect(s). We present three cases of congenital sinus pericranii with facial involvement, emphasizing its cutaneous presentation with diagnostic pitfalls and discuss the multidisciplinary management of this vascular anomaly. [ABSTRACT FROM AUTHOR]

Background: Since the nineteenth century, a great variety of crossed brainstem syndromes (CBS) have been described in the medical literature. A CBS typically combines ipsilateral cranial nerves deficits to contralateral long tracts involvement such as hemiparesis or hemianesthesia. Classical CBS seem in fact not to be so clear-cut entities with up to 20% of patients showing different or unnamed combinations of crossed symptoms. In terms of etiologies, acute brainstem infarction predominates but CBS secondary to hemorrhage, neoplasm, abscess, and demyelination have been described. The aim of this study was to assess the proportion of CBS caused by a bleeding episode arising from a brainstem cavernous malformation (BCM) reported in the literature.Case Presentation: We present the case of a typical Foville syndrome in a 65-year-old man that was caused by a pontine BCM with extralesional bleeding. Following the first bleeding episode, a conservative management was decided but the patient had eventually to be operated on soon after the second bleeding event.Discussion: A literature review was conducted focusing on the five most common CBS (Benedikt, Weber, Foville, Millard-Gubler, Wallenberg) on Medline database from inception to 2020. According to the literature, hemorrhagic BCM account for approximately 7 % of CBS. Microsurgical excision may be indicated after the second bleeding episode but needs to be carefully weighted up against the risks of the surgical procedure and openly discussed with the patient.Conclusions: In the setting of a CBS, neuroimaging work-up may not infrequently reveal a BCM requiring complex multidisciplinary team management including neurosurgical advice. [ABSTRACT FROM AUTHOR]

• The incidence of DVA in thalamic glioma was 18.18%. • Some phenotypic coexpression of shared gene may modulate the development of DVA. • Markers such as IDH1 were not associated with the development of DVA. This study aimed to assess the prevalence of developmental venous anomaly (DVA) in patients with thalamic glioma. Furthermore, we explored the association between DVA and some important biomarkers, such as IDH1 mutation, and H3K27M mutation. Patients who received tumor resection in West China Hospital between August 2009 and October 2017 were enrolled. Propensity score matching was conducted based on a logistic regression model and 1:1 matching for case and control was used to generate a new cohort from patients with meningioma. Chi-square test, t -test, univariate and multivariate analyses were employed to assess the prevalence of DVA in thalamic glioma and meningioma and to identify risk factors associated with DVA. Ninety-nine patients with thalamic glioma were enrolled in the current study (male, n = 54; female, n = 45). The mean age was 42.9 ± 15.3 years old. We identified a higher prevalence of DVA in 99 patients with thalamic glioma when compared with 99 patients with meningioma (18.18% vs. 7.07%), which was slightly lower than the prevalence of DVA in glioma reported in previous studies. Furthermore, the distribution of gender, age, and tumor grade in DVA did not reach statistical significance. Chi-square test, univariate and multivariate analyses showed that IDH1 mutation, ATRX mutation, MGMT promoter methylation, p53 mutation, MMP9, EGFR, and Top II positive expression, TERT mutation, and H3K27M mutation were not associated with the development of DVA in thalamic glioma. A higher prevalence of DVA was found in thalamic glioma compared with meningioma. [ABSTRACT FROM AUTHOR]

Purpose: to better understand the pathological process of sinus pericranii and the safety of the surgical intervention. Methods: patients’ archive review of patients who underwent surgical management for sinus pericranii with a confirmed diagnosis and with follow up period greater than 6 months. Results: 6 infants were included; all underwent disconnection surgically with good outcome in all cases. Conclusion: disconnection surgery for sinus pericranii is a safe procedure with a good prognosis.

DVAs are often not well seen on CT angiography unless the angiography is venous phase; they will be seen on CT with contrast. Computed tomography (CT) of his head (not shown) had been reportedly negative. Keywords: Developmental venous anomaly; Sylvian fissure; Magnetic resonance imaging; Subarachnoid hemorrhage EN Developmental venous anomaly Sylvian fissure Magnetic resonance imaging Subarachnoid hemorrhage 222 224 3 06/27/22 20220501 NES 220501 A 9-year-old male was transferred to the Children's Colorado Emergency Department (ED) from another ED after losing consciousness and falling face-first onto a sidewalk while being escorted from a 4th of July parade. [Extracted from the article]

Purpose: to better understand the pathological process of sinus pericranii and the safety of the surgical intervention. Methods: patients' archive review of patients who underwent surgical management for sinus pericranii with a confirmed diagnosis and with follow up period greater than 6 months. Results: 6 infants were included; all underwent disconnection surgically with good outcome in all cases. Conclusion: disconnection surgery for sinus pericranii is a safe procedure with a good prognosis. [ABSTRACT FROM AUTHOR]

Background: Significant advances have been reported recently in the genetic and mechanistic characterization of extracranial venous malformations. However, intracranial purely venous malformations (icVM) analogous to those outside the CNS have not been systematically described. Purpose: We sought to ascertain whether such an entity as icVM could in fact be identified, distinct from previously described CNS venous anomalies and analogous to extracranial venous malformations. Methods: Our prospectively collected pediatric cerebrovascular database was reviewed to identify patients with icVM; 1458 consecutive angiograms and/or angiographic interventions performed on 706 children at our institution from October, 2006 through May, 2019 were evaluated, in addition to outside imaging studies on 192 additional patients sent to our Vascular Anomalies Center for cerebrovascular review during the same time period. Thus, the cohort consisted of 898 children. Results: Nineteen of 898 patients (2.1%) were found to harbor icVM, including 9 (47.3%) with sinus pericranii, 15 (78.9%) with associated large, complex extracranial venous malformations, and 3 (15.7%) with neurocognitive delay. There was no intracranial hemorrhage or venous hypertension seen in the cohort. Asymptomatic venous thrombosis in the superior sagittal sinus was seen in three patients. Conclusion: Venous malformations, both extracranial and icVM, share many characteristics that are distinct from developmental venous anomalies. icVM were not associated with venous hypertension. The underlying genetic mutations involved in the development of icVM, germ-line or somatic, remain to be elucidated, but may very well involve shared mechanisms and pathways with extracranial venous malformations. [ABSTRACT FROM AUTHOR]

Background: Intracranial Hodgkin's lymphoma (HL) is an exceedingly rare condition that is at an increased risk of misdiagnosis and mismanagement, especially when initial radiographic evidence points to an alternative pathology., Observations: The authors describe the case of a 75-year-old female who presented with a posterior fossa lesion initially concerning for a vascular malformation on computed tomography imaging due to perilesional hypervascularity. Subsequent angiography revealed a developmental venous anomaly (DVA) but no arteriovenous shunting. The patient's clinical history combined with magnetic resonance imaging findings prompted a tissue biopsy, which demonstrated a rare case of central nervous system (CNS) HL. The neoangiogenesis of this CNS HL with an adjacent DVA contributed to the original radiographic misdiagnosis of an arteriovenous malformation. HL's angiogenic potential, coupled with the proangiogenic environment induced around DVAs, may have contributed to this rare CNS HL metastasis to the cerebellum. The potential misdiagnosis of posterior fossa CNS HL has also been seen in several prior cases reviewed herein., Lessons: Hypervascular tumors, especially when associated with an adjacent DVA, should also be considered when first evaluating suspected intracranial vascular lesions. Although rare, CNS HL should be included in the differential diagnosis for patients with a prior history of HL. https://thejns.org/doi/10.3171/CASE24238.