[Combination of intravascular surgery and surgical operation for occipital subcutaneous arteriovenous fistula in a patient with neurofibromatosis type I].
ABSTRACT We presented a case of neurofibromatosis type I (NF-1) associated with an extracranial arteriovenous fistula (AVF) fed by the occipital and vertebral artery. A 20-year-old man was referred to our hospital because of an occipital subcutaneous pulsatile mass. A CT scan showed a huge subcutaneous enhanced mass. Angiography revealed that the occipital AVF was fed by bilateral occipital arteries, the left ascending pharyngeal artery, the left middle cerebral artery, and the left vertebral artery with-abundant communication with the subcutaneous veins. Endovascular treatment by using both coil and glue (Eudragid) embolization via the occipital artery successfully obliterated the AVF. Subsequently surgical operation was performed. Postoperative angiography showed the disappearance of the AVF. Combination of intravascular surgery and surgical operation should be considered as an effective treatment for NF-1 associated with AVF.
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ABSTRACT: Vascular abnormalities associated with neurofibromatosis Type 1 (NF-1) are well known, but arteriovenous fistulae (AVF) are rare and usually involve vertebral AVF. A case of intercostal AVF associated with NF-1 and manifesting as congestive myelopathy is described. A 62-year-old woman with a medical history of NF-1 experienced gradually worsening gait disturbance. Neurological examination at the time of admission found spastic paraparesis, decrease of sensation in the bilateral lower extremities, and vesicorectal disturbance. Magnetic resonance imaging scans revealed flow void sign and a congestive lesion of the thoracolumbar spinal cord, as well as a huge mass in the paravertebral region at T10-T12. Spinal angiography revealed an intercostal AVF with a large paravertebral varix, which drained to the spinal medullary vein and caused dilation of the intradural venous plexus of the spinal cord resulting in congestive myelopathy. Direct surgery was selected for the occlusion of the draining point of the medullary vein, located in the entry of the dura. The patient underwent hemilaminectomy at T10-T12, and the dural entry of the draining vein was occluded. Postoperative angiography revealed no dilated venous plexus of the spinal cord and reduced paravertebral varix. The symptoms were gradually relieved after the surgery. The present case of a very rare intercostal AVF with an unusual manifestation of congestive myelopathy occurred in a relatively rare association with NF-1. Accurate evaluation of the vascular anomaly allowed selection of the appropriate surgical method.Neurosurgery 10/2007; 61(3):E656-7; discussion E657. · 2.53 Impact Factor
- The Journal of Dermatology 03/2006; 33(2):158-9. · 1.77 Impact Factor
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ABSTRACT: Neurofibromatosis type 1 (NF-1) is one of the most common inherited diseases and as an autosomal dominant genetic disorder results from NF-1 gene mutation with 100% penetration and wide phenotypic variability. The disease can involve a wide variety of tissues derived from all three embryonic layers. NF-1 vasculopathy has been described primarily in peripheral arteries, but arteries supplying the CNS may also be involved. Of those, extracranial vertebral involvement is the commonest and most important. A series of four patients with NF-1 and vascular disease of the vertebral artery is described with a review of the pathophysiology, vascular phenotypes, their management and the pertinent literature.Interventional Neuroradiology 12/2007; 13(4):315-28. · 0.77 Impact Factor