Introduction :
The vein of Galen aneurysmal malformations (VGAM) are vascular anomalies associated with an enlarged great cerebral vein. Lasjaunias et al. [1] classified them in two groups: true VGAM and dilatations of the vein of Galen, secondary to parenchymatous high flow arteriovenous malformation draining to this vein [2]. According to the angioarchitecture, VGAM are divided in choroidal and mural types [1,3]. We present a case of Choroidal type of VGAM presenting in early childhood achieving near total occlusion after a staged endovascular treatment.
Case Report :
A 9 yr old girl presented with increased head circumference , prominent facial veins since birth, progressive headache since 2 months .There is a history of unreasonable fever ,delayed developmental milestones, decreased scholastic performance with frequent episodes of redness of right eye since age of 1 year .
Antenatal course of mother was uneventful but for oligohydramnios, large head of foetus (antenatal scan) because of which child was delivered by LSCS .Patient was investigated elsewhere in view of her complaints and was diagnosed to have Choroidal type of VGAM .She underwent endovascular embolisation twice followed by GKRT at age of 2 and 4 yrs respectively.
Anthropometric measurements of child ( height, weight, BMI ) were appropriate for her age (50th percentile).She had an elongated face with frontal bossing and increased head circumference (56 cms).There is marked prominence of facial veins bilaterally with a warm face and neck.(Fig 1)
Investigation with chest radiograph showed mild enlargement of cardiac silhouette and encephalic magnetic resonance (MR) evidenced dilated vein of Galen (Fig 2). Digital subtraction angiography (DSA) showed partially embolised choroidal type of VGAM, with a high flow fistula fed by ectatic bilateral choroidal arteries, pericallosal arteries, bilateral Pcom, left A1 (ACA).(Fig 3)
An uneventful trans-arterial embolization of major feeders was performed with 70% NBCA glue,lipoidal mixture achieving near total obliteration (Fig 4,5,6,7)
Postoperatively patient evolution was satisfactory with gradual improvement during her hospital stay. She was discharged on Day 7 with no fresh complaints or deficits (Bicetre score -4)
Patient was adviced for follow up visits after 3,6 months for control MR brain and check DSA.
Discussion :
The VGAM are rare, with incidence estimated around 1% of all intracranial vascular lesions [3,4]. They classically manifest during neonatal period with severe congestive heart failure, but also can present with hydrocephalus, epileptic seizures, development delay and hemorrhage [3,4]. In the embryologic life the median prosencephalic vein drains choroidal plexus into a primitive accessory sinus, called falcine sinus [2]. The median prosencephalic vein normally regresses around 11th week of fetal development and its caudal residual remains as cerebri magna vein [2].
The persistence of median prosencephalic vein is associated with fistulous connections to the choroidal arteries, which cause dilatation of the vein of Galen due to direct blood flow and venous hypertension [5-8]. Usually the sinus rectus is absent, and the malformation drains to a persistent falcine sinus [2].
In the present case, the choroidal multifeeder AVM was seen draining into dilated vein of galen and then into ectatic straight sinus, torcula, transverse sinus, sigmoid sinuses respectively.
As per the classification of Lajausnias et al. the choroidal type usually has many high flow fistulas from choroidal ,pericallosal arteries, thalamoperforating and subependymal branches. Such configuration would usually lead to heart failure at neonatal period [2,3]. The mural type has major blood supply from posterior choroidal arteries and causes macrocephaly, development delay but for few cardiac symptoms [3]. Hydrocephalus may also occur due to venous hypertension blocking the absorption of cerebrospinal fluid
Key words: VEIN OF GALEN , POSTERIOR CHOROIDAL FEEDERS , ENDOVASCULAR , NBCA , GLUE CAST
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