The Effect of Treatment of Idiopathic Intracranial Hypertension on Prevalence of Retinal and Choroidal Folds

Abstract

Purpose We described 3 types of folds in the retina and a crease in the outer retina associated with papilledema due to idiopathic intracranial hypertension (IIH) at presentation. We report the change in folds relative to treatment of IIH over the six months. Design/Study Subjects Substudy of a randomized clinical trial. Methods Study eyes of subjects assigned to acetazolamide (ACZ, n=44) or placebo (PLB, n=43) had spectral domain optical coherence tomography (SDOCT) images of the optic disc and macula regions at baseline, 3 and 6 months. Images were evaluated for peripapillary wrinkles (PPW), retinal folds (RF), choroidal folds (CF), and creases using transaxial and en face views. The optic nerve head (ONH) shape, retinal nerve fiber layer (RNFL) thickness, ONH volume, and papilledema grade were measured. Outcome Determination of the presence or absence of PPW, RF, CF and creases. Results At presentation, except for an increase of PPW in ACZ eyes (64% vs 28%); both treatment groups were matched for all OCT features. At 6 months, ACZ-treated, but not PLB-treated, eyes had fewer folds of all types (p < 0.01), with a 57% reduction in frequency of RF. Creases did not resolve. Resolution of RF, but not of PPW and CF, was associated with significant reduction in RNFL thickness, ONH volume and papilledema grade. Conclusions The various types of retinal folds associated with papilledema reflect biodynamic processes and show an ACZ treatment effect. Persistence of these folds despite marked improvement in ONH swelling suggests permanent changes in the affected retinal tissues. Trial registration: clinicaltrials.gov identifier: NCT01003639

Update on the Diagnosis and Treatment of Idiopathic Intracranial Hypertension

“…Other nonspecific findings of intracranial hypertension on fundus exam include venous stasis, hyperemia, hemorrhages, infarcts, cotton wool spots, choroidal and retinal folds, and absence of spontaneous retinal venous pulsations. 18,[29][30][31] In chronic untreated cases, optic disc edema can be replaced by optic atrophy due to damage to the retinal ganglion cells.…”

Nonarteritic Anterior Ischemic Optic Neuropathy Induced Retinal Folds and Deformations

“…RF diminished with treatment that reduced ONH swelling, RNFL thickness, and cerebrospinal fluid pressure at 6 months. 12 PPW and outer RF eventually resolved over a longer interval and CF persisted over a longer interval despite treatment.…”

“…We recently reported several types of retinal and choroidal folds (CF) in patients with papilledema due to idiopathic intracranial hypertension (IIH) and how these folds change over time and with therapy. 11 , 12 These folds have several stereotypic patterns: (1) peripapillary wrinkles (PPW), consisting of closely spaced concentric or spiral folds within a half disc diameter of the ONH and confined to the retinal nerve fiber layer (RNFL); (2) horizontal or radially oriented RF greater than one half disc diameter from the optic disc and predominantly affecting the middle and inner retinal layers in the posterior pole that spared the outer retina and choroid; (3) choroidal folds (CF), which almost always are associated with overlying RF, and (4) peripapillary outer RF characterized by deeply furrowed “creases” (“high water marks”). 13 The pattern of folds correlated with two structural determinates: PPW and outer RF were associated with the magnitude of disc edema (thickened RNFL and volume), whereas CF correlated with the degree of anterior displacement of the peripapillary tissues and lamina cribrosa.…”

Automated image-based estimation of severity and cause of optic disc edema

“…Max vessel segment tortuosity 26 Location of tortuosity trend derivative equal to 1 27 Tortuosity trend parameter the number of upper end-points ( Fig. 6.4).…”

“…The retinal area around the optic disc sometimes responds to the biomechanical physical stress of swelling by exhibiting visible wrinkles (or folds) of the tissue around the optic disc [26]. The presence and variety of retinal and choroidal folds [27] is congenital (i.e.…”