Optic disc drusen are acellular calcific deposits occurring in small, crowded optic discs with abnormal vasculature. Evidence suggests axoplasmic transport alteration and axonal degeneration are involved in disc drusen formation. In affected patients, the number and size of disc drusen are highly variable, and the drusen may be visible near the disc surface or buried within the disc, causing them to appear as pseudopapilledema. B-scan echography is the most sensitive method for detecting disc drusen. Most patients with disc drusen are asymptomatic, but progressive visual field loss and vascular complications, including anterior ischemic optic neuropathy and choroidal neovascularization, may occur. Optic disc drusen have no established effective treatment. Diagnosing disc drusen correctly is important to avoid unnecessary work-up and to avoid overlooking potential serious conditions such as true papilledema. Disc drusen patients with more-than-expected visual field defects or progressive visual loss should have work-up to exclude other causes.
Purpose To evaluate the clinical utility of spectral domain optical coherence tomography (SD-OCT) in differentiating mild papilledema from buried optic nerve head drusen (ONHD). Design Comparative case series. Participants 16 eyes of 9 patients with ultrasound-proven buried ONHD, 12 eyes of 6 patients with less than or equal to Frisén grade 2 papilledema due to idiopathic intracranial hypertension. 2 normal fellow eyes of patients with buried ONHD were included. Methods A raster scan on the optic nerve and retinal nerve fiber layer (RNFL) thickness analysis was performed on each eye using SD-OCT. Eight eyes underwent enhanced depth imaging SD-OCT. Images were assessed qualitatively and quantitatively to identify differentiating features between buried ONHD and papilledema. Five clinicians trained with a tutorial and masked to the underlying diagnosis reviewed the SD-OCT images of each eye independently to determine the diagnosis. Main outcome measures Differences in RNFL thickness in each quadrant between the two groups, and diagnostic accuracy of five independent clinicians based on the SD-OCT images alone. Results We found no statistically significant difference in RNFL thickness between buried ONHD and papilledema in any of the four quadrants. Diagnostic accuracy among the readers was low and ranged from 50–64%. The kappa coefficient of agreement among the readers was 0.35 (95% Confidence interval: 0.19, 0.54). Conclusions SD-OCT is not clinically reliable in differentiating buried ONHD and mild papilledema.
OCT is a valuable tool in evaluating the peripapillary RNFL in both glaucomatous and nonglaucomatous optic neuropathies. This technology may be used for both research and clinical purposes to assess disease progression in optic neuropathies and diseases that affect the central nervous system. OCT-measured RNFL thickness remains complimentary to the clinical examination skills in the evaluation of nonglaucomatous optic neuropathies.
A 20-year-old man with a history of pre-B cell acute lymphocytic leukemia (ALL) presented with optic perineuritis of the right eye while undergoing chemotherapy. Evaluation failed to reveal an infectious or neoplastic cause, and the patient improved with oral corticosteroid treatment. He returned 10 weeks later with complete loss of vision in the right eye. Optic nerve biopsy revealed leukemic infiltration of the optic nerve, and the patient was treated for central nervous system (CNS) relapse of ALL. Transient optic perineuritis may be the initial manifestation of CNS involvement of pre-B cell ALL.
Objective. To correlate visual and neurologic clinical scores and treatment of optic neuritis and multiple sclerosis (MS) patients with assays of serum phosphorylated neurofilament heavy chain (pNF-H) and optical coherence tomography (OCT) measurements of axonal loss. Design/Methods. The Optic Neuritis Treatment Trial (ONTT) randomized 457 patients with acute optic neuritis to intravenous methylprednisolone (IVMP) followed by oral prednisone, oral prednisone or placebo treatment arms. We quantified serum pNF-H levels in 175 ONTT patients 5 years after study entry. We performed OCT measurements of macular volume and the retinal nerve fiber layer (RNFL) in a subset of 51 patients at year 15. Results. Elevated pNF-H levels at year 5 correlated to poorer visual function at study entry. Lower 15 year macular volumes and RNFL thickness correlated better with follow-up than with baseline visual function measures. With IVMP treatment, 15 year RNFL differences of the fellow eye (FE) minus the affected eye (SE) RNFLFEmSE correlated with five-year pNF-H levels. PNF-H was reduced by half with IVMP relative to placebo or by 40% relative to prednisone. Conclusions/Relevance. Acute optic neuritis patients who have more severe visual loss during initial presentation have a higher incidence of axonal loss that was slightly suppressed with IVMP treatment.
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