Ocular Coherence Tomographic and Clinical Characteristics in Patients of Punctuate Inner Choroidopathy Associated with Zonal Outer Retinopathy

Abstract

Optical coherence tomography is helpful in the diagnosis of patients suffering zonal ocular outer retinopathy associated with punctate inner choroidopathy. All those patients responded well to immunosuppressive agents.

Idiopathic Multifocal Choroiditis/Punctate Inner Choroidopathy With Acute Photoreceptor Loss or Dysfunction Out of Proportion to Clinically Visible Lesions

“…13,14,19 The original Gass case series, which first described AZOOR, included patients with MFC lesions. 20 Spaide et al reported 11 patients with MFC and blind spot enlargement and found that the scotomata correlated with the loss of the photoreceptor outer segments on SD-OCT. 16 Chen et al reported in their series of PIC patients that the scotomata seen in these patients also corresponded to the photoreceptor-loss visible on SD-OCT. 10 Kramer et al found that these areas of photoreceptor-loss in patients with MFC corresponded to hyperautofluorescence, which was also verified by reports from Jung et al and Freund et al 9,11,12 All these findings are consistent with our observations. The underlying cause of the hyperautofluorescence in areas of photoreceptor attenuation associated with MFC/PIC remains unclear.…”

“…This atrophy often progressed over time despite therapy. 8 In contrast to the current observations, in these patients foveal sparing was common until late in the disease course and the majority of the patients maintained good VA. 8 In our cohort, 3 cases (case 3, 6 and 8) recovered similar to the reports of Kramer et al, Spaide et al and Chen et al, 3,10,11 two while undergoing systemic corticosteroid and anti-VEGF treatment and one patient recovered without treatment and retained a very small discrete relative paracentral scotoma. Two of our cases (case 5+7) presented with already existing central vision- and corresponding photoreceptor loss and with extinguished or diminished cone and rod response on ERG testing.…”

“…9,11,17 However, in previous reports the photoreceptor-attenuation and hyperautofluorescence resolved completely under immune modulating therapy and BCVA returned back to normal. 3,9,10 In our series, BCVA fully recovered only in 3 out of 8 cases. The remaining patients displayed persistent decreased BCVA and photoreceptor attenuation.…”

“…9, 17 Choroidal hypo-perfusion, choroidal ischemia or a blockage-effect were assumed to cause this finding. 17, 24–26 Hyperreflectivity in the Henle’s layer corresponding to the hypofluorescent area in this case and the fact that increased choroidal thickness is found in areas corresponding to the photoreceptor irregularity in MFC/PIC with secondary photoreceptor loss in the acute inflammatory stage, 10 made us believe that a blockage effect rather than choroidal hypo-perfusion is responsible for the hypofluorescence on ICGA found in our patient.…”

“…Previously some patients with MFC/PIC with disruption of the ellipsoid and interdigitation zones seen on SD-OCT in clinically uninvolved areas within macula have been described. 3,9,10 In these cases, the outer retinal structures recovered and the hyper-autofluorescence seen on fundus autofluorescence (FAF) faded with corticosteroid treatment. 3,9,10 …”