Vein of Galen aneurysm in an adult: case report

Abstract

-Vein of Galen aneurysm is a rare pathology, representing less than 1% of intracranial vascular malformations. We report on a 65 years-old man who experienced a generalized tonic-clonic seizure. Brain imaging showed a large calcified expanding mass in the pineal region, confirming the diagnosis of a vein of Galen aneurysm. Because of the spontaneous thrombosis of the malformation, there was no need for microsurgical or endovascular treatment and he is been regularly followed since that.KEY WORDS: vein of Galen aneurysm, vein of Galen malformation, cerebral vein, congenital vascular malformation. Aneurisma da veia de Galeno em adulto: relato de casoRESUMO -Aneurisma da veia de Galeno é patologia rara, representando menos de 1% das malformações vasculares intracranianas. Apresentamos o caso de um homem de 65 anos que teve episódio de crise convulsiva tônico-clônica generalizada. Exames de imagem evidenciaram grande processo expansivo calcificado na região pineal, confirmando o diagnóstico de aneurisma trombosado de veia de Galeno. Devido à trombose espontânea da malformação, foi excluída a possibilidade de tratamento endovascular bem como microcirúrgico, mantendo-se o acompanhamento clínico. PALAVRAS-CHAVE: aneurisma da veia de Galeno, mal-formação da veia de Galeno, veias cerebrais, malformação vascular congênita.Vein of Galen aneurysm (VGA) is a rare vascular malformation representing less than 1% of vascular intracranial abnormalities. It is a congenital process frequently detected between the 6 th and 11 th months of gestational age 1 , during early childhood or neonatal period 2 . This finding in adult age is very rare, presenting or not symptoms throughout childhood 3 . Clinical manifestations can present at any age in the form of heart failure, delayed neuropsychomotor development, hydrocephalus and seizures 4 .We describe a case of VGA reporting clinical symptoms, radiological findings and management relating to current published literature. CASEA 65 year-old man was brought to the emergency room at the University Hospital of the Pontifical Catholic University of Paraná, Brazil because of generalized tonic-clonic seizures. On admission, he was treated with anticonvulsant therapy for status epilepticus. Past medical history was significant for treated high blood pressure and heart failure diagnosed late in life. Also significant for an episode of ischemic cerebrovascular disease two years before, resulting in left hemiparesis. During childhood and adult life there was no neuropsycomotor development delay, no signs of cardiopathy, no neurological signs or symptoms as well as no history of epilepsy.CT scan showed a calcified expanding process in the pineal region (Fig 1). MRI showed a heterogenic roundshaped mass localized in the supra-vermian cistern with dimensions of 35x32x30 mm showing high signal in T2-weight imaging (Fig 2). The MRI images were suggestive of a thrombosed VGA. Cerebral angiography showed a thrombosed VGA also (Fig 3).Considering the fact that the aneurysm was already thrombosed ...

Vein of Galen Aneurysms: Presentation and Endovascular Management

“…True VGAMs have been reported in adults [27,28,29,30]. It seems that fluid dynamics may play a role in controlling the flow condition at this particular fistula and in postponing its clinical presentation to adulthood [29].…”

“…The patient was an intelligent university student that was discovered accidentally to have such a VGAM; the particular flow pattern of this anomaly made the patient asymptomatic till adulthood. Other cases of adult VGAM remained asymptomatic till they presented suddenly with hemorrhage [28] or epilepsy after spontaneous thrombosis [30]. …”

“…VGAMs occurring in adults can also result from venous ectasia due to high flow condition caused by deeply seated arteriovenous malformations or tentorial/clival dural arteriovenous fistulae drained directly to the vein of Galen or one of its tributaries [30,31]. The treatment options in these situations are not directed toward the vein of Galen itself but rather involve attacking the fistula directly by different methods that include transarterial embolization, microsurgery and additional radiosurgery in some cases in a staged fashion [32].…”

Sinus Thrombosis after Endovascular Treatment of Vein of Galen Aneurysmal Malformation

“…The VGAM are characterized by abnormal arterial connections to the median prosencephalic vein, the embryonic precursor of the cerebral magna vein [1,3,4] . Its incidence is rare and is estimated to be around 1% of all intracranial vascular lesions [1,[4][5][6][7] . Clinical presentation is mainly in childhood [1,4] , but some cases are diagnosed in adults [7] .…”

“…Its incidence is rare and is estimated to be around 1% of all intracranial vascular lesions [1,[4][5][6][7] . Clinical presentation is mainly in childhood [1,4] , but some cases are diagnosed in adults [7] . VGAM are usually manifested during the neonatal period with severe congestive heart failure, as well as hydrocephalus, epileptic seizures, development delay, and hemorrhage [4,6] .…”