PMC6388523_01
(a) Macular OCT showing extensive macular thinning with normal structure, (b and c) Ganglion cell layer OCT showing near total macular GCL loss with central loss. (d) Optic nerve OCT showing severe optic atrophy.
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F1
Macular OCT showed extensive macular thinning with normal structure [Figure 1a]. Ganglion cell layer (GCL) OCT revealed nearly complete GCL loss including central loss OU [Figures 1b and c]. IIH, 75% specific for IICP [Figure 1d]. In Pattern 4 IIH, there remains a hint of temporal wings, secondary to cerebrospinal fluid in the temporal retina.
Brain MRI (a) Diffusion weighted showing extensive fluid around optic nerves. (b) Hygroma, unusual in a child. (c) Greater than 2-mm of CSF in optic nerve near globe.
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F2
However, in one axial cut, we interpreted more than 2 mm of CSF in the optic nerve near the globe [Figures 2a and c] and a small hygroma, which is very unusual at a young age and may be a sign of increased ICP [Figure 2b].
(a) OCT macula at presentation. (b and c) OCT of GCL showing improvement in ganglion cell layers with a central opening explaining the improved visual acuity. (d) The decrease in ANFL due to decrease in ICP and decreased papilledema.
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F3
OCT of the macula showed consistent thinning as before [Figure 3a]. OCT of GCL showed improvement in GCL with a central opening, explaining the improved visual acuity in both eyes [Figures 3b and c]. On optic nerve OCT, ANFL decreased to 40 microns OD and 44 microns OS [Figure 3d].
(a) OCT macula. (b and c) GCL OCT. (d) OCT optic nerve. Stable condition from four months to one year of treatment, some recovery of ANFL from the initial drop after four months.
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F4
Exactly one year later, VA and OCTs [Figure 4] were stable, despite some increase in ANFL from the original improvement seen after four months of treatment, and some non-significant thickening of macula and thus increased GCL.