High-flow arteriovenous fistula of the central nervous system associated with hereditary haemorrhagic telangiectasia

The authors reported three cases of high flow arteriovenous fistula (AVF) associated with hereditary haemorrhagic telangiectasia (HHT). The first case was a 9-month-old boy who presented with subarachnoid hemorrhage (SAH). Digital subtraction angiography (DSA) revealed a perimedullary AVF at the cervical spine and the fistula was successfully interrupted by transarterial embolization with no additional neurological deficits. The second case was his mother, a 29-year-old women. She also had history of epistaxis, so imaging screens of her central nervous system (CNS) were undertaken. MRI showed a giant intracerebral varix associated with a high-flow pial AVF. This patient was treated by surgery and postoperative CT angiography showed extinction of AVF completely. The third case was an 8-year-old girl with a family history of HHT. She presented with gradual progression of paraparesis, sensory disturbance in the bilateral lower extremities and bladder dysfunction. MRI disclosed a perimedullary AVF with a large varix at the L3/4 level. The AVF was successfully interrupted by transarterial embolization with platinum coils. Post-embolization DSA showed complete disappearance of the AVF. The existence of HHT must always be considered a possibility when AVF of the CNS is diagnosed.