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.

Spontaneous thrombosis of type II vein of Galen aneurysmal malformation: a case report.

Vein of Galen malformations (VGAMs) are rare and complex congenital brain vascular anomalies that pose significant diagnostic and treatment challenges. The natural history of this type of vascular anomaly is very poor, with many patients succumbing to complications such as congestive heart failure, hydrocephalus, and brain parenchymal injury. Although the clinical course of most VGAMs was considered unfortunate, with meticulous imaging, a group of lesions with a more placid presentation and course can be identified.

Oculomotor Nerve Palsy Induced by a Cerebral Developmental Venous Anomaly: A Case Report and Comprehensive Review.

BACKGROUND Symptoms caused by developmental venous anomalies (DVAs) are usually mild and unspecific. Despite the benign nature of DVAs, they can occasionally be symptomatic. CASE REPORT A 67-year-old woman presented with sudden diplopia and left eyelid ptosis for 10 days. A neurologic examination revealed left complete oculomotor nerve palsy. Other neurologic deficits, including eye pain or pulsatile tinnitus, were not detected. Furthermore, the visual acuity was normal. Additionally, no retinal hemorrhage, venous dilatation, or fundus tortuosity were observed. No ischemia lesions or neoplasms were observed in MRI, and no widening or enhancement of the cavernous sinus was detected in post-contrast T1-weighted images, but magnetic resonance tomography cerebral angiography (MRTA) detected an offending vessel compressing the left oculomotor nerve in the fossa interpeduncular. We hypothesized that oculomotor nerve palsy (ONP) was caused by an abnormal arterial structure. However, digital subtraction angiography (DSA) revealed no aneurysm or abnormal arterial structure in the arterial phase, while a tortuous and dilated collecting vein was detected in the venous phase, connecting the left temporal lobe to the left cavernous sinus. This indicated a typical caput medusae appearance, suggesting the mechanism of oculomotor palsy caused by compressive impairment of the DVA. The patient refused microvascular decompression surgery, and ONP persisted after 30 days. Management was conservative, with spontaneous resolution at 60 days and no recurrence during the 2-year follow-up. CONCLUSIONS ONP is rarely caused by DVAs, which are easily ignored due to their benign nature. Cerebral vein examinations are advised for patients exhibiting clinical symptoms of unknown etiology.

Vein of Galen aneurysmal malformation: does size affect outcome?

PURPOSE: To validate a semiautomated method for segmenting vein of Galen aneurysmal malformations (VGAM) and to assess the relationship between VGAM volume and other angioarchitectural features, cardiological findings, and outcomes. METHODS: In this retrospective study, we selected all subjects with VGAM admitted to the Gaslini Children's Hospital between 2009 and 2022. Clinical data were retrieved from electronic charts. We compared 3D-Slicer segmented VGAM volumes obtained by two independent observers using phase-contrast MR venography to those obtained with manual measurements performed on T2-weighted images. The relationship between VGAM volumes and clinical and neuroimaging features was then explored. RESULTS: Forty-three subjects with VGAM (22 males, mean age 6.56 days) were included in the study. Manual and semiautomated VGAM volumes were well correlated for both readers (r = 0.86 and 0.82, respectively). Regarding reproducibility, the inter-rater interclass correlation coefficients were 0.885 for the manual method and 0.992 for the semiautomated method (p < 0.001). The standard error for repeated measures was lower for the semiautomated method (0.04 versus 0.40 of manual method). Higher VGAM volume was associated with superior sagittal sinus narrowing, jugular bulb stenosis, and aqueductal stenosis (p < 0.05). A weak correlation was found between VGAM volume and straight sinus dilatation (r = 0.331) and superior sagittal sinus index (r = - 0.325). No significant associations were found with cardiac findings, post-embolization complications, and outcome (p > 0.05). CONCLUSIONS: Semiautomated VGAM volumetry is feasible and reliable with improved reproducibility compared to the manual method. VGAM volume is not a prognostic factor for clinical outcome, but it is related to other venous findings with potential hemodynamic effects.

Echocardiography evaluation of neonatal vein of Galen aneurysmal malformation.

BACKGROUND: In neonatal vein of Galen aneurysmal malformation, vein of Galen aneurysmal malformation echocardiography remains the mainstay for early detection and explains various haemodynamic changes occurring due to a large systemic arterio-venous shunt. However, there is limited evidence of echocardiography in risk stratifying neonatal vein of Galen aneurysmal malformation vein of Galen aneurysmal malformation. The objective of this study was to identify echocardiographic parameters that could be associated with major outcomes and guide timing of neuro-intervention. METHODS: In this retrospective chart review, infants < 28 days of age with the diagnosis of vein of Galen aneurysmal malformation vein of Galen aneurysmal malformation were included. Demographic, clinical, and echocardiographic parameters were compared in neonates who survived or died with neonatal presentation. A risk algorithm model based on key echocardiographic parameters was developed to determine those who are at risk of early death. RESULTS: Of the 19 neonates included, with median birth weight 3.1 kg (IQR 2.58-3.36), nine (47%) neonates died at median age of 5 days (IQR 4-17). All neonates showed retrograde diastolic flow at the level of descending aorta by colour Doppler on the first post-natal echocardiogram at median age of 2 days (IQR 1-5.5). An aortic antegrade-to-retrograde velocity time integral ratio of < 1.5 and supra-systemic pulmonary artery pressure had 100% positive predictive value of death (p = 0.029), whereas aortic antegrade-to-retrograde velocity time integral ratio of > 1.5 and sub-systemic pulmonary artery pressure had 100% positive predictive value of survival (p = 0.029). CONCLUSION: Combination of aorta antegrade-to-retrograde velocity time integral ratio and degree of pulmonary hypertension on the first post-natal echocardiogram may help stratify the severity of disease and guide optimal timing for neuro-intervention for neonatal vein of Galen aneurysmal malformation.

Contribution of Deep Cerebral Venous Anomaly to the Emergence of Nonaneurysmal Subarachnoid Hemorrhage as Opposed to Aneurysmal Subarachnoid Hemorrhage.

BACKGROUND: The exact reason of nonaneurysmal subarachnoid hemorrhage (SAH) is an enigma. The aim of this study is to identify if type III deep cerebral venous drainage is exclusively prevalent in patients with nonaneurysmal SAH and to enumerate the predictors of poorer outcome in these patients. METHODS: All patients of age >18 years, presented at our centre with spontaneous SAH on noncontrast computed tomography head and were divided into 2 groups, aneurysmal and nonaneurysmal SAH after 4-vessel DSA. Based on the deep venous drainage pattern on both sides, basal venous drainage was found and classified into 3 types: type I, type II, and type III. The 3 groups were pitted against one another. Regression analysis were performed to predict the occurrence of nonaneurysmal-SAH with different types of basal vein. RESULTS: There were 100 nonaneurysmal SAH cases and 103 aneurysmal SAH cases. The mean age of presentation was 47.8 ± 13.55 years with slight male predominance (52%). The patients with type III venous drainage have 2 times more risk of developing nonaneurysmal SAH (95% confidence interval = 1.21-4.31) as compared to those with aneurysmal SAH. On multivariate analysis, type III basal venous drainage, worse Hunt and Hess grade at presentation, extensive bleeding were predictors of an adverse outcome. CONCLUSIONS: The presence of type III venous distribution is associated with a 2-fold increase in the probability of having nonaneurysmal SAH, as well as a 3-fold increase in the risk of developing poorer neurological sequelae.

Doppler Ultrasound Flow Reversal in the Superior Sagittal Sinus to Detect Cerebral Venous Congestion in Vein of Galen Malformation.

BACKGROUND AND PURPOSE: Vein of Galen malformation is a rare congenital cerebrovascular malformation. In affected patients, increased cerebral venous pressure constitutes an important etiologic factor for the development of brain parenchymal damage. The aim of this study was to investigate the potential of serial cerebral venous Doppler measurements to detect and monitor increased cerebral venous pressure. MATERIALS AND METHODS: This was a retrospective monocentric analysis of ultrasound examinations within the first 9 months of life in patients with vein of Galen malformation admitted at <28 days of life. Categorization of perfusion waveforms in the superficial cerebral sinus and veins into 6 patterns was based on antero- and retrograde flow components. We performed an analysis of flow profiles across time and correlation with disease severity, clinical interventions, and congestion damage on cerebral MR imaging. RESULTS: The study included 44 Doppler ultrasound examinations of the superior sagittal sinus and 36 examinations of the cortical veins from 7 patients. Doppler flow profiles before interventional therapy correlated with disease severity determined by the Bicêtre Neonatal Evaluation Score (Spearman ρ = -0.97, P = < .001). At this time, 4 of 7 patients (57.1%) showed a retrograde flow component in the superior sagittal sinus, whereas after embolization, none of the 6 treated patients presented with a retrograde flow component. Only patients with a high retrograde flow component (equal or more than one-third retrograde flow, n = 2) showed severe venous congestion damage on cerebral MR imaging. CONCLUSIONS: Flow profiles in the superficial cerebral sinus and veins appear to be a useful tool to noninvasively detect and monitor cerebral venous congestion in vein of Galen malformation.

Angiographic predictors of spontaneous obliteration of transarterial partially embolized brain arteriovenous malformations.

BACKGROUND: Spontaneous obliteration of cerebral arteriovenous malformations is uncommon but could occur after partial embolization. METHODS: A retrospective study of 140 patients that underwent embolization for cerebral AVMs from 2005 to August 2019 using liquid embolic agents. The angiographic outcome of patients was classified as regard complete embolization, partial embolization, and complete obliteration after partial embolization. The parameters studied included size, location, number of arterial feeders, number of draining veins, rupture status, embolic agent, and patient factors as well. RESULTS: The study patients included 74 (53%) females and 66 (47%) males. Their age ranged from 7 to 43 years old. One hundred and eight patients (77%) presented with hemorrhage. The AVM grades were grade II in 57 (40.7%) patients and grade III in 56 (39.3%) patients. Sixty-one (43.57%) patients were treated by n-Butyl Cyanoacrylate and 71 (50.71%) patients were treated with Onyx, and both materials were used together in 8 cases. Follow-up angiography was done from 6 to 36 months after embolization. The rate of complete occlusion in all patients was 61.43% (86 patients). There were three groups of patients, the first group had complete occlusion of the nidus at the time of embolization and included 68 (48.57%) patients. The second group had partial embolization with partial occlusion of the nidus 54 patients (38.57%). The 3rd group included 18 patients (12.85%) with complete nidal occlusion on follow up after partial embolization. The delay in the venous drainage of the AVM to the late arterial phase or early venous phase with flow stasis was a significant predictor of future obliteration on follow up after partial embolization. Other significant parameters that were associated with the progressive disappearance of the AVM nidus on follow up after partial embolization are presentation with hemorrhage, AVMs size less than 3 cm, the presence of single draining or double draining veins, superficial venous drainage, and one or 2 arterial feeders. CONCLUSION: Spontaneous closure of intracranial arteriovenous malformations after partial embolization may be encountered in cases of stasis of flow during embolization procedure with a delay of the venous drainage. A long-term follow-up of more cases over many years is required to confirm the validity of this conclusion.

Vein of Galen aneurysmal malformation: rationalizing medical management of neonatal heart failure.

Neonates who present in high output heart failure secondary to vein of Galen aneurysmal malformation can be difficult to manage medically due to the complex physiology that results from the large shunt through the malformation. Though the cardiac function is often normal, right ventricular dilation, severe pulmonary hypertension, and systemic steal can result in inadequate organ perfusion and shock. This report recommends medical management for stabilization of neonates prior to definitive management with endovascular embolization. IMPACT: Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial arteriovenous malformation, which can present in the neonatal period with high output heart failure. Heart failure secondary to VGAM is often difficult to manage and is associated with high mortality and morbidity. Despite optimal medical management, many patients require urgent endovascular embolization for stabilization of their heart failure. This report offers discrete recommendations that can be used by clinicians as guidelines for the medical management of heart failure in newborns with VGAM.

Pial arteriovenous fistula resembling the vein of Galen aneurysmal malformation, treated with staged endovascular embolization: a case report.

A 41-month-old boy was presented to our hospital because of an intracranial mass suspected of cerebrovascular malformation. He was admitted and received cerebral angiography. The angiography result confirmed the intracranial mass was the dilated vein of Galen resulting from a pial arteriovenous fistula, which quite resembling the vein of Galen aneurysmal malformation. Considering one-time embolization of the fistula may greatly change the distribution of intracranial blood flow, we decided to perform staged embolization. In the first stage, we partially embolized the fistula, resulting in a sharp decrease in blood flow to the lesion. The second intervention was performed one month later, and completely embolized the fistula. The boy recoverd well and returned to normal childhood without any neurological deficits. Follow-up MR images obtained at 10 months after the last procedure showing total obliteration of the pAVF, gradually shrinking of the varix, and remodeling of the vein of Galen.

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.

Fine, Vascular Network Formation in Patients with Vein of Galen Aneurysmal Malformation.

BACKGROUND AND PURPOSE: A vein of Galen aneurysmal malformation is known to present with recruitment of dural feeders and, in our cohort, a fine, vascular network formation. The vessels we have observed differ from dural vascular recruitment in that they produce a hairlike, collateral network of vessels. We reviewed treatment courses of vein of Galen aneurysmal malformation treatments in a series of 36 cases that displayed a fine, vascular network formation. MATERIALS AND METHODS: We retrospectively analyzed 36 cases of vein of Galen aneurysmal malformation, including tectal/thalamic AVMs, treated at our center from January 2004 to September 2021, and reviewed fine, vascular network formations in the subarachnoid space and subependymal zone alongside the vein of Galen aneurysmal malformation. RESULTS: Patients at first endovascular treatment ranged from neonates to 157 months (median age, 4.3 months). Patients with preinterventional fine, vascular network formations were significantly older at the initial angiogram than patients with postinterventional fine, vascular network formations (P < .05). On average, for 20 control choroidal/mural vein of Galen aneurysmal malformations whose treatment course had been completed and in which no plexiform network was visualized, a mean of 2.63 (SD, 1.64) treatments were required to achieve a radiographic cure. For the 36 choroidal/mural vein of Galen aneurysmal malformations whose treatment course had been completed and in which a fine, vascular network formation was visualized, a mean of 5.94 (SD, 2.73) treatments were required to achieve a radiographic cure (P < .01). CONCLUSIONS: Development of a fine, vascular network formation is an acquired and reversible phenomenon that differs from typical dural vessel recruitment, given the hairlike nature of the network and its rapid onset postinterventionally. It typically resolves after completion of treatment, and this resolution correlates with closure of the vein. We recommend that neurointerventionalists avoid delays in treatment wherever possible to reduce the likelihood of a fine, vascular network formation.

Brain Injury in Fetuses with Vein of Galen Malformation and Nongalenic Arteriovenous Fistulas: Static Snapshot or a Portent of More?

BACKGROUND AND PURPOSE: Brain injury in fetuses with vein of Galen malformations and nongalenic AVFs is a rare complication whose appearance, course, and prognosis are poorly studied. We sought to characterize the MR imaging features and examine associations with postnatal outcome. MATERIALS AND METHODS: This was a retrospective analysis of fetal MRIs of subjects with vein of Galen malformation and nongalenic arteriovenous fistulas. Two pediatric neuroradiologists independently reviewed examinations to determine the presence of abnormalities on structural imaging (T1 volumetric interpolated breath-hold examination and T2-HASTE), DWI, and T2*-weighted images; discrepancies were adjudicated by a third reviewer. Radiologic progression of injury was determined by additional fetal or neonatal MRIs. A simple composite score evaluating poor neonatal clinical outcome as either intubation or death by postnatal day 2 was also queried. A body fetal imager evaluated the presence of systemic findings of right heart strain. RESULTS: Forty-nine fetal MR imaging examinations corresponding to 31 subjects (27 vein of Galen malformations and 4 nongalenic AVF cases) were analyzed. Injury was observed in 8 subjects (26%) with 14 fetal examinations; the mean gestational age at identification of injury was 32.2 (SD 4.9) weeks. Structural abnormalities were present in all subjects with injury; restricted diffusion, in 5/7 subjects with available data; and T2* abnormalities, in all subjects with available data (n = 7). Radiologic progression was documented in all cases with follow-up imaging (n = 7). All subjects with fetal brain injury had a poor neonatal clinical outcome. CONCLUSIONS: Brain injury in fetuses with vein of Galen malformation and nongalenic AVFs shows a combination of structural abnormalities, restricted diffusion, and blooming on T2* images. Injury appears to portend a poor prognosis, with relentless progression and a likely association with adverse neonatal outcomes.

The outcome of the vein of Galen aneurysmal malformation cases diagnosed prenatally.

Vein of Galen aneurysmal malformation (VGAM) is a rare foetal anomaly associated with neurodevelopment delay, cardiac failure, and even perinatal death. We aimed to assess prenatal features of VGAM and describe postnatal outcomes. This was a retrospective study involving six foetuses diagnosed with VGAM prenatally in two centres. All of the cases underwent foetal neurosonography and echocardiography. The presence of ventriculomegaly, intracranial haemorrhage and cardiac failure was recorded. Pregnancy and neonatal outcome information were obtained from medical records. The mean gestational age at diagnosis was 31.1 ± 5.1 weeks, and the mean size of VGAM was 29.2 ± 5.2 × 26.4 ± 3.3 mm. Ventriculomegaly was detected in five of six (83.3%) cases. Intracranial haemorrhage was present in five (83.3%) cases. Cardiac failure was shown in four (66.6%) foetuses. Three foetuses underwent termination of pregnancy (TOP); in two cases, neonatal death occurred. One patient was treated with endovascular embolisation, and there was no cardiac problem or neurodevelopment delay. Prenatally diagnosed VGAM have a poor prognosis, mainly if a cardiac failure or neurological consequences (intracranial haemorrhage, hydrocephaly) are present in utero.Impact StatementWhat is already known on this subject? VGAM is the most common cerebral arteriovenous malformation detected prenatally, and it can lead to severe consequences in the perinatal period.What do the results of this study add? The accuracy of foetal neurosonography is excellent for detecting VGAM and associated brain abnormalities. Foetal echocardiography is mandatory for the prediction of prognosisWhat are the implications of these findings for clinical practice and/or further research? VGAM is associated with severe brain injury, cardiac failure, and the prognosis is generally poor. We need predictors to identify those expected to benefit from postnatal therapy.

Endoscopic third ventriculostomy in an infant with vein of Galen aneurysmal malformation treated by endovascular occlusion: Case report and a review of literature.

INTRODUCTION: Vein of Galen aneurysmal malformations (VGAMs) can, through multiple mechanisms, complicate with hydrocephalus (HCP). It is generally agreed that management strategies in this scenario should focus on endovascular embolizations. Treatment options for non-responders, however, have been only scarcely reported upon. CASE PRESENTATION: We present a nine-month-old boy with a mural type VGAM complicated by HCP. Despite endovascular occlusion of the sole feeder, the child exhibited hydrocephalus progression prompting an Endoscopic Third Ventriculostomy (ETV). This procedure restored a cerebrospinal fluid (CSF) circulation otherwise impaired by aqueduct obstruction. Later, a new feeder arose and a second embolization was ultimately needed in order to achieve VGAM regression. Throughout four years of follow up, the child attained all developmental marks. DISCUSSION/CONCLUSION: VGAMs are prone to hydrocephalus development as there is both an underlying venous congestion and a mechanical, obstructive component. Although there is a rationale for addressing both components, the underlying AV shunts and subsequent venous pressure elevations usually determine failure of traditional CSF shunting strategies. It is therefore challenging to manage HCP in patients who failed to improve following endovascular embolizations. For such cases, ETV stands as an elegant minimal invasive alternative with potential to provide a more physiologic drainage route and thus better allow for neurological development.

Transvenous embolization of vein of galen aneurysmal malformations using the "Chapot pressure cooker" technique.

METHODS: Two patients, one 5-year-old and one 7-year-old, both presented with congestive heart failure in the newborn period and were subsequently treated in the newborn period with multiple, staged TAEs with n-BCA for choroidal VGAMs. RESULTS: We achieved progressive reduction in shunting and flow but were unable to accomplish complete closure of the malformation: in both patients, a small residual with numerous perforators persisted. The decision was made to perform TVE using the CHPC. In this technique, a guiding catheter is placed transjugular into the straight sinus (SS). One or two detachable tip microcatheters are advanced to the origin of the SS. Another microcatheter is advanced and the tip placed between the distal marker and the detachment zone of the former. Coils and n-BCA are used to prevent reflux of Onyx. CONCLUSIONS: In this study, we recognized two important factors of traditional VGAM treatment that may cause interventionalists to consider the ChPC to treat VGAM: (1) without liquid embolic, deployed coils may not occlude the fistula entirely. (2) There is the concern of causing delayed bleeding should the arterial component of the fistula rupture. ChPC ameliorates these issues by offering complete closure of the fistula with liquid embolic material in TVE.