Ocular Histoplasmosis Syndrome: morphology and clinical spectrum

Abstract

 

Purpose

 

To report the clinical, imaging, and treatment characteristics of patients with ocular histoplasmosis syndrome (OHS)

 

Methods

 

Retrospective, longitudinal study evaluating consecutive patients with OHS. Clinical and imaging characteristics were analyzed. Chorioretinal lesions were further analyzed with SD-OCT in all patients within the study period.

 

Results

 

313 eyes of 157 patients were diagnosed with OHS. Mean age was 51.7±17.7 years. 67% of patients had a follow-up equal or greater than 6 months. 41% of eyes had an active choroidal neovascularization (CNV) on SD-OCT. 14% of eyes had disciform scars at presentation. 67% had type II CNV. CNV types were not associated with worse initial (p=0.162) or final (p=0.151) BCVA. The presence of macular OHS lesions was not associated with the presence of active CNV(p=0.583). 112/169 treated eyes (66%) received anti VEGF therapy. Mean final BCVA was significantly better than initial BCVA among eyes that received anti VEGF therapy (p<0.001)

 

Conclusions

 

Up to 55% of eyes developed some type of CNV or associated sequelae. OHS CNVs may present as any type of CNV, but are predominantly type II. Location of the CNV plays a more important role in final BCVA than type of CNV. Anti VEGF treatment is a viable option for subfoveal and juxtafoveal CNVs, similar to previous reports

 

 

Figure 1. Classic triad of OHS. A. Color fundus photograph of the right eye demonstrating typical peripapillary atrophy (PPA), and two centrally pigmented, macular histo spots. B. Left eye with circumferential PPA and prominent subretinal hemorrhage. C. Fundus autofluorescence corresponding with image in A.; Macular histo spots are easier to identify than on color photographs (arrows). D. Fundus autofluorescence corresponding with image in B.; a large area of hypoautofluorescence corresponds to the areas of subretinal hemorrhage and subretinal fluid observed in B. A macular histo spot is clearly identified (arrow).

 

 

Figure 2. CNV types on cross-sectional SD-OCT images. A. Sub-RPE CNV. Irregularly elevated, undisrupted RPE hyperreflective band involving the foveola and extending nasally. B. Subretinal CNV. A large accumulation of fluid occupies the space between the neurosensory retina and a disrupted RPE. C. Retinal angiomatous CNV. A combination of multiple intrarretinal and subretinal accumulation of fluid is present underneath the fovea.

 

Keywords: 688 retina • 552 imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound) • 462 clinical (human) or epidemiologic studies: outcomes/complications