Intracerebral pial arteriovenous fistula with large venous varix: A rare case report
A 24-year-old right-handed male, without history of head trauma or alcohol abuse, was admitted to our department with 6-year history of repeated episodes of generalized tonic-clonic seizure. In spite of anticonvulsant therapy, the attack became more frequent. There was no history suggestive of a prior subarachnoid hemorrhage or transient ischemic events. The patient had no mucocutaneous telangiectasia or episodes of recurrent epistaxis. None of his family members including siblings and parents suffered from such type of illness. On examination, he was alert and behaving normally with no focal neurological deficit. There were no signs of meningism. General physical examination showed normal results. Routine laboratory tests were within normal limits and inter-ictal electroencephalogram revealed no abnormality.\nOn computed tomography (CT) scan, a large well circumscribed round smooth walled strong and uniformly enhancing hyper dense space occupying lesion with peripheral wall calcification was found in left frontal lobe []. The lesion was causing significant mass effect with midline shift. A left frontal well defined flow void of size 60 × 66 mm communicating with the superior sagittal sinus (SSS) through dilated cortical vein with feeder supply from left ACA was observed in the cranial magnetic resonance imaging (MRI) with angiogram []. Possibility of pial AVF with large venous varix was considered. 128 Multi slice cerebral CT angiography (CTA) revealed a large left frontal AVF supplied by the distal left ACA draining through a dilated cortical vein into the SSS []. A large variceal dilatation of the proximal venous end measuring nearly 6.5 cm in diameter with wall calcification was noted in association with the AVF.\nThe patient underwent a left frontal craniotomy crossing midline. On reflecting the dura to opposite side, a globular soft pinkish pulsatile varix (≈ 6 × 6 cm) was encountered superficially in left frontal lobe [Figure and ] close to the anterior falx with dilated draining vein reaching up to the anterior part of the SSS. A fistulous connection between the mass and left ACA was noted, which confirmed the presence of AVF. As the mass was posing significant mass effect with very hard and thick wall, simple occlusion of feeding artery did not result into disappearance of the AVF. Hence total excision of the AVF mass along with the venous varix was performed [] after ligating the primary feeding artery. The patient had an unremarkable postoperative course with no fresh focal neurological deficit. A CT scan on second postoperative day demonstrated a large filling defect in the area of the varix excision [Figure and ]. Postoperative CTA done at the time of discharge showed complete obliteration of the AVF and reconstitution of the distal ACA [Figure and ]. He has been followed up for 6 months and has been seizure-free since then on a daily dosage of 300 mg of phenytoin.