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  • Title: [High-flow arteriovenous fistula of the central nervous system associated with hereditary haemorrhagic telangiectasia].
    Author: Sasamori T, Hida K, Asano T, Nakayama N, Kuroda S, Iwasaki Y.
    Journal: No Shinkei Geka; 2009 Jan; 37(1):57-63. PubMed ID: 19175035.
    Abstract:
    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.
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