Progressive peritumoral edema defining the optic fibers and resulting in reversible visual loss

Abstract

Hemangioblastomas are frequently associated with peritumoral edema caused by extravasation of plasma ultra-filtrate 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 near-complete 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.

Optic Nerve Haemangioblastoma: Signs of Chronicity

“…As was seen in our case, Baggenstos and colleagues [14] showed resolution of bilateral optic tract oedema after optic nerve haemangioblastoma resection, confirming the lesion as the source. Visual deficits in the contralateral eye were seen to improve after tumour removal, again suggesting that oedema was the cause of compromise, rather than ischaemia or mass effect.…”

Hemangioblastoma of the optic nerve

“…5,14 A more contemporary finding associated with cerebral and optic nerve hemangioblastomas is peritumoral edema. 3,24,38 As retrobulbar hemangioblastomas are often supplied by branches of the ophthalmic artery, the high blood volume and permeability of these tumors results in a disproportionately large amount of edema for the size of the lesion. 25 This edema tends to propagate along low-resistance white matter pathways, and can be demonstrated on serial imaging.…”

Aggressive granular cell tumor of the neurohypophysis with optic tract edema and invasion into third ventricle

“…The relationship between OTE and visual outcomes was assessed in a few studies, but results were inconsistent. [1,3,11] Using optical coherence tomography, a recent study evaluated retinal nerve fiber layer thickness in addition to visual acuity and visual field in patients with sellar and suprasellar lesions. The study demonstrated worse visual function and poor visual improvement postoperatively in patients who had OTE.…”