An 11-year-old patient, previously diagnosed with pigmentitis fugax, came to a review consultation complaining of blurred vision, dizziness and episodes of amaurosisx in recent days.
Examination showed a VA of 0.7 in BE, with no changes compared to previous controls.
No changes in eye movements or diplopia.
The eye fundus showed bilateral papilledema, pigment dispersion of the retro-ecutorial spicular type in the four quadrants and maculae of normal appearance.
VC showed peripheral restriction typical of pigmentitis.
OCT showed a large increase in mean RNF thickness of 546 μm in the RE and 442 μm in the LE.
Analytical and neuroimaging studies were normal.
Lumbar puncture showed normal biochemistry and a pressure of 30 cm H20.
BMI: 29.19, overweight (97th percentile).
In all cases an oral treatment was established with prednisone at a dose of 1.5 mg/kg/day, acetazolamide 250 mg/8 hours, potassium supplement and gastric protection by our Pediatric Department.
In the first two cases, papilledema and paresis of the LV pair improved after the first week.
Controls were performed with OCT at one week, 15 days, one month and subsequently monthly.
Figure 2 shows the evolution of OCT in case number 2, a progressive decrease in the thickness of the RNFL was observed.
In case number 3, papilledema was maintained for eight weeks.
The maximum follow-up was 9 months.
Corticosteroids began to be withdrawn in descending order when clinical symptoms improved and there was a significant decrease in the thickness of the RNFL on OCT and acetazolamide when measurements of NFC at 110 μm were observed on OCT.
In the first two cases this occurred around the third month, in case number 3, treatment lasted 6 months.
In case number 1, after 3 months without treatment, OCT detected a small transient increase in RNFL that, as it did not produce symptoms, did not require treatment.
However, in case number 3, a decrease in the thickness of the RNFL up to 75 μm in the right eye and 67 μm in the left eye has been observed after two months of authentic treatment.
In long-term follow-up 1 year, neither in case 1 nor in case 2 any significant recurrence requiring retreatment was detected by OCT.
