Progressive peritumoral edema defining the optic fibers and resulting in reversible visual loss.
Hemangioblastomas are frequently associated with peritumoral edema caused by extravasation of plasma ultrafiltrate through permeable neoplastic vessels. The authors report the clinical and imaging findings in a 62-year-old man with von Hippel-Lindau disease who presented with rapid (within 24 hours) loss of color vision and nearcomplete loss of left eye vision (acuity too poor to test). Serial MR imaging demonstrated a stable vascular tumor in the medioinferior aspect of the left optic nerve, associated with progressive edema extending from the nerve through to the bilateral optic radiations. Complete resection of the lesion was performed via an extended transsphenoidal approach, and histological examination confirmed the lesion was a hemangioblastoma. Postoperatively, the patient recovered color vision and had improvement in visual acuity (20/320). Serial imaging in this unique case captured the progressive extravasation of peritumoral edema that tracked and defined the parallel white matter tracts of first- and second-order neurons of the optic system, causing vision loss. Tumor resection led to resolution of the edema and improvement in visual function.
Baggenstos
Martin
M
Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA.
Chew
Emily
E
Butman
John A
JA
Oldfield
Edward H
EH
Lonser
Russell R
RR
D001929:Brain Edema / Q000209:etiology / Q000473:pathology*; D003118:Color Perception; D018325:Hemangioblastoma / Q000150:complications / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009900:Optic Nerve / Q000473:pathology*; D020127:Recovery of Function; D012160:Retina / Q000473:pathology; D015354:Vision, Low / Q000473:pathology*; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000473:pathology* / Q000601:surgery