 Hi, everyone. This is Dr. Aviya Grawal from Maharashtra Institute of Medical Sciences and Research, Milana Ambala. And I'll be presenting a paper on a very rare topic, that is vein of gallin aneurysmal malformations, which seeks to be an insight for the neuro interventional radiologist to undo the gallin nod for surgeons. Coming to the abstract of the people, vein of gallin malformations are a very rare and unique entity, which forms the congenital malformations of the cerebral vasculature. They are due to the arterial venous fistulous connections of mishap and arteries in brain, with a persistent embryonic precursor of the vein of gallin, which is called the median present catholic vein of Marcosky, instead of connecting with the capillates. So we'll be reporting two cases today. The first case is that of a middle-aged man with intractable occipital headache. And the second case demonstrates the antinatal diagnosis of vein of gallin malformations in association with ventricular megalia and cardium megalia in routine antinatal scan at 30 weeks of the station in a twin pregnancy. Considering the rarity of these lesions, there are very few studies that have been able to adequately diagnose VOGM. So this makes the study very essential, radically essential for continuing developments in the diagnostic aspects of diagnosing and managing these lesions. Ultrason is the primary screening modality for vein of gallin malformations, followed by MRI, which is the diagnosis of choice. Coming to the introduction, vein of gallin malformations are a very rare arterial venous fistulous, which constitute less than 1% of all cerebral vasculomal formations. And they represent only 30% of the symptomatic vasculomal formations in the pediatric age. These lesions can be characterized by the presence of arterial venous shunts, keeping menopause and catholic vein patent, causing high flow pressure-related aneurysmal dilatation in the midline, forming a large venous pouch just behind the third ventricle. Steen heel in 1895 made the first reference to the gallelic malformation referring it to as varix aneurysm. Though these lesions are very uncommon, they are of special interest to the interventional neural radiologist, because endovascular therapy has alone itself to be proved as an effective and the only safe therapeutic modality. These lesions have been termed as the gaudier not for cerebral vascular surgery because of the major problems being faced during the surgery, which are as follows. These malformations are very deep-seated and have high flow shunt across them, which makes it very difficult during the surgery. They have got poor myelination of the brain pattern. Their tendency to tear easily on retraction and the ventricular shunting may worsen the cerebral venous hypertension. The case one is that of a 35-year-old man who presented with intractable occipital headache for the last five months. The patient was being evaluated for headache when his vitals were within normal limits and all the lab investigations were also normal. The patient during physical examination showed a GCS of 15 by 15 with optimal ophthalmological examination and there was no significant history of any seizure or loss of consciousness or ENTP, vomiting or any other. So, the cause tended to be a query, but then he went to CEMR brain with MRA scan, which revealed a well-subscribed T2A-hypo intense oval midline lesion, which measured approximately 30 into 25 into 23 MF, just dorsal to the third ventricle, as shown in Figure 1. In continuity with dilated straight sinuses and the dilated bilateral transverse sinuses that's resistive of vein of gallen aneurysmal malformation. This malformation also caused mass effect on membrane causing compression of aqueduct resulting in aqueductal stenosis and thus upstream hydrocephalus. This is CEMR T2 weighted and T1 weighted post contrast image showing aneurysmal dilatation of the midline median present catholic vein just behind the third ventricle which was resistive of the vein of gallen on axial sections. Figure 2 suggests the flare and the T2 T1 weighted post contrast images of median present catholic vein on societal sections showing their drainage into the straight sinuses and the tortula of hair of ally with aqueductal compression on midbrain and result in upstream hydrocephalus. Figure 3 is an MRI scan which shows multiple arterial feeders from bilateral posterior cerebral arteries and the basilar arteries attaching to the aneurysmal arterial venous aneurysm without any intervening needle status the capillaries. So as these malformations are direct connection between the arteries and veins there can be multiple arterial feeders which originated from bilateral PCAs and basilar arteries in this case and they were drained by right internal cerebral vein and the right basal vein of lozental with evidence of multiple dilated venous channels from inferior societal sinuses and superficial cortical veins as shown in Figure 4. Due to the high rate of morbidity and risk ratio associated with vein of gallen malformations the patient and his family members chose to continue on supportive treatment and observation. This is another image which shows a midline dorsal venous pouch of vein of gallen malformation just dorsal to the third ventricle showing a flow void on MRI scan on societal T2 weighted image with multiple arterial feeders and dilated venous channels and sinuses at specifically two different distinct levels. There was another case where a twin pregnant twin pregnancy woman presented for antenatal scan at 30 weeks of gestation. She was 28 year old and in fetus B we could evaluate these things. There was a tubular dilated anechoic structure draining blood from the dilated falsine sinus into the transverse sinus. The image B shows the pulsatile and the turbulent flow on color Doppler which was characteristic and consistent with vein of gallen malformation. So on transumptominal scan of fetus B, neurosynographically it was revealed that a tubular dilated anechoic structure was forcing from the spenium of the corpus callus towards the cisterna magnum showing a pulsatile and turbulent blood flow draining the dilated falsine sinuses and the tubular filofilla into the dilated transverse sinuses which was suggestive and consistent with vein of gallen anniversal malformation with mild cerebral ventricle omega as the association. Cross section of the fetal B chest showed slightly enlarged area of fetal heart in relation to the area of chest which was suggestive of mild cardiomegaly. These findings of cerebral venous hypertension and cardiomegaly were consistent with vein of gallen for which counseling and neurosurgical concentration of the parents was done. Coming to the discussion of the paper, development of the talent catholic chloride plexus is accompanied by simultaneous differentiation of the transit venous structure which brings these chloride plexuses and has been designated as median present catholic vein or the primitive internal cerebral vein. By the 11th week there's formation of paired internal cerebral veins which annexes or drains the venous drainage of these chloride plexuses. Formation of these internal cerebral veins results in regression of the median present catholic vein which was the primitive structure except for its most cordal part which joins the internal cerebral veins to form the vein of gallen. There's a flowchart which we have drafted explaining the pathophysiology of the vein of gallen malformations. So what happens is normally the developing internal cerebral veins, they annex the drainage of the fetal chloride plexuses and the median present catholic vein progresses. However, after birth there is high pressure from the arteries communicating with the venous system directly in case of these malformations as the capillaries are lacking. After birth there is exclusion of the low resistance of the placental circulation as well. There is a resultant abrupt increase of flow across these malformations resulting in vein of gallen malformation or the patent median present catholic vein. So this abrupt increase in the flow across this arterial venous malformation is can be can be due to three main reasons, being restriction of the venous drainage or there is or there being a decreased diastolic pressure in the aorta or a compensatory increase in cardiac output to maintain the perfusion. So restriction of venous drainage could be due to poorly developed venous drainage system or the secondary venous stenosis, venous occlusion or a high flow of emission. This all results in a cerebral venous hypertension. This system of creating a hypertension at the cerebral venous level causes the main neurological effects. So we'll be classifying the pathophysiology into the neurological effects and the cardiological effects. So cerebral venous hypertension causes the neurological events which mean cerebral edema, hydrocephalus, microcephaly, hypoxia, headaches, seizures, intracranial bleed and subarachnoid harm, which is focal neurological deficit and many more. There can also be a progressive cerebral parankimal damage from the venous hypertension or hypoxia which may lead to mental retardation, cognitive impairment and delayed milestones or failure to threats. There can also be a collateral drainage into cavernous sinuses as a compensation which can lead to increased flow in facial veins and derogatory venous plexuses forming their prominence and epistaxies or proctosis. If there is a decreased diastolic pressure in the aorta or if there is a compensatory increase in cardiac output to maintain the perfusion, we'll have the features of cardiac menstrual stations which mean pulmonary hypertension, high output cardiac failure and cardiometalline. There can also be reduced subendocardial or coronary flow leading to myocardial dishingion and if there is excessive flow across the pulmonary circulation it would lead to pulmonary hypertension. There will also be increased venous return in compensation to the increased cardiac output which may lead to motioning of blood from right to left side especially across the patent ductus arteriosus and the patent foramatove leading to cyanosis. So these were the pathophysiological features and their clinical manifestations. Principal feeders of the malformation are those that supply the telakorida and the quadrigeminal plate. These include the anterior or the present catholic group which consists of anterior cerebral anterior choroidal middle cerebral and posterior lateral choroidal arteries and the posterior or the mesent catholic group which consists of posterior medial choroidal posterior thalamoperforating quadrigeminal and superior cerebellar arteries. There can be abnormal flow through the fistulas which retards the normal involution of the embryonic vein that is the medial person catholic vein and prohibits the development of the vein of gallus. This non-reguration of medial person catholic vein which drains the shunt lacks a fibrous solid elastic wall and it lies free in the subarachromatic space therefore ballooning out to a larger size especially dorsal to the vertebral. So coming to the modalities for screening and diagnosing and diagnosis of vein of gallium malformations ultrasound it aids in the identification of vein of gallium especially in third trimester and which being a modality of choice and the only modality to screen in antinatal phenates and it helps in differentiation from other non-vascular space occupying lesions along with assessing the status of the fetal cardiovascular system or neurological system. It demonstrates a sonalus and venous sac as a mass located posterior to third ventricle with pulsatile flow and differentiates it from other midline cystic lesions as it was in the in this paper as well. On axial brain CT there can be features of cerebral parenchymal damage in the form of a few cerebral atrophy peri ventricular white matter I put in cities and dilated ventricular system. They can also be well defied multi-lobby littered intensity enhancing lesions within the system of vellum interposable. MRI is the modality of choice for these malformations demonstrating the location precise location of this fistula and the presence of the midas maybe the arterial as well as the venous and the relationship between different pathological components. MR angiography is used as a non-invasive tool and it is an alternative to diagnostic angiographic studies. However, angiography remains the gold standard for the diagnosis of vein of gallium malformations especially in evaluating small feeders subline the fistula as well as the dynamic aspects of the venous drainage of the normal vein and of this arterial venous shunt. These malformations can also be associated with other syndromes especially like turner syndrome, blue rubber syndrome, supineuronumeridine digits, hypospadias and some cardiac manifestations like transposition of great vessels or eartic stenosis and right side area of the clutch. This image shows the ideal treatment which is being followed nowadays for the vein of gallium malformations which involves the embolization and the endovascular therapy especially by neurointerventional radiologists. So a micro catheter is inserted and advanced up to the embolization site where glue and other embolizing agents are injected into the vessels causing the regression. However, aggressive medical management is needed in most cases mostly to postpone the intervention till up to 5 to 6 months of age when the intervention becomes easier and safer. In cases of congestive cardiac failure in a new unit whose refractory to medical treatment, emergency embolization is the need of indication. In units not presenting with cardiac failure, the aim would be to prevent the consequences of cerebral venous hypertension and thus promote normal cerebral development. Arterial venous fistulas can also be occluded on the arterial side using embolic agents such as coils, cyanocrylates and attachable balloons. They can also be dealt with embolization of the venous sac as in case of transvenous and trans-tortular coil embolization which are used to achieve low reduction across the malformations and it is the technique of choice in patients with multiple fistulas. It results in retrograde from bosses obliterating the fistulas and thus the malformations. This is an image post intervention showing how the treatment was performed and the prognosis of the patient wherein the normal brain circulation was bestowed after embolization and it led to reduction in the size of the malformation gradually over time. So concluding the paper, these entities, vein of gallant malformations, have a very varied and life-threatening clinical presentations with a characteristic distinctive complex angioarchitecture which is very essential for their early diagnosis especially for a caring physician who could understand the embryological and the pathophysiological aspects and manage these lesions appropriately. Both in prenatal and neonatal period or at the time of definitive intervention managing these lesions is of course challenging. Thus in near future and even at current scenario the role of imaging especially as a neuro interventional radiologist it is very essential in making these lesions potentially curable and helping the patients to have a better prognosis and low mobility. Thank you.