In the sixth episode of Clinical Conversations in Retina, Daniel Epshtein, OD, FAAO, and Joseph J. Pizzimenti, OD, FAAO, FORS, FNAP, professor at the Rosenberg School of Optometry and past president of the Optometric Retina Society, discuss central serous chorioretinopathy (CSCR) and its diagnosis, treatments, and link to the pachychoroid spectrum.
Classifying central serous chorioretinopathy
The wide variety of phenotypic presentations of CSCR and the lack of a standardized nomenclature often make it difficult to precisely diagnose CSCR.
The ambiguity of various terms, such as acute, persistent, and chronic, can vary amongst different eyecare practitioners (ECPs) and various research groups.
Standardizing the classification of CSCR
The Central Serous Chorioretinopathy International Group was formed in order to outline a standardized method to classify CSCR and address some of the difficulties of CSCR diagnosis.1 This standardization clarifies the various expressions of CSCR that make it difficult to classify.
In practice, however, both Dr. Pizzimenti and Dr. Epshtein note that their clinical approach rarely adheres to the exact steps outlined by the group. The standardized method offers a clear clinical definition of CSCR, describing it as a focal detachment of sensory retina and/or the retinal pigment epithelium that is typically formed over thickened, dysfunctional choroid.
With its various phenotypes, CSCR is more of a family of diseases than one condition, states Dr. Pizzimenti.
Diagnosing patients with CSCR
Dr. Pizzimenti starts the diagnosis process with a thorough history of the patient, identifying any common risk factors of CSCR, including recent steroid use, past pregnancy, sleep apnea, and kidney/heart/bone marrow transplants. Testosterone and Saw Palmetto use are also potential risk factors that Dr. Pizzimenti takes into consideration.
Imaging modalities to identify CSCR
Next, a clinical exam can help identify any serous retinal detachments and pigmentary disturbances. This can be done through optical coherence tomography (OCT), preferably with enhanced imaging, to closely examine the choroid.
1. Optical coherence tomography
Many OCTs can perform enhanced depth imaging, and Dr. Epshtein encourages ECPs to check if their OCT has this feature. It is important to further investigate any lesions that might be suggestive of macular neovascularization with OCT angiography or dye-based angiography.
2. Fundus autofluorescence
Fundus autofluorescence is another imaging method that can help identify retinal pigment epithelium changes, which can be useful for determining chronicity and aid in prognostication of CSCR. Large descending tracts imply that CSCR has been present for some time, maybe a few months.
ECPs may also form a better idea of what the patient’s vision may be if the serous retinal detachment resolves. The appearance of hypo-autofluorescence and lots of pigmentary disturbances implies a worse condition than iso-autofluorescence with minimal pigmentary disturbances.
3. Fluorescein angiography
Fluorescein angiography can assist in concluding if the condition is more localized or diffused. For instance, a localized, extrafoveal hyperfluorescence can be treated with a thermal or photodynamic laser. Though it may leave a relative scotoma, a thermal laser may photocoagulate the affected retina and resolve fluid egress.
Two scenarios for CSCR
Dr. Epshtein and Dr. Pizzimenti consider a hypothetical patient who is having their first documented episode of CSCR with the following characteristics:
- Serous retinal detachment
- Thickened choroid
- Few visible changes in fundus autofluorescence
- No testosterone supplementation
- No pertinent medical history
In such a patient, Dr. Pizzimenti would look for retinal pigmentary abnormalities, including a search for areas of retinal pigment epithelium (RPE) atrophy in both eyes and shallow areas of subretinal fluid.
The state of the outer retina can be examined by observing the ellipsoid zone band to ensure it is intact. The doctors look to rule out choroidal neovascularization (CNV)—using standard OCT or OCT angiography—and any exogenous or endogenous hypercortisolism.
If the patient does not have CNV, then the patient can be evaluated again in 1 to 2 months to monitor any partial or complete resolution. Dr. Pizzimenti would recommend that the patient oversee their stress levels to manage the production of endogenous glucocorticoids, which would affect the progression of the condition.
Managing recurrent CSCR
More aggressive treatments are considered for repeat and recurrent CSCR episodes. Dye-based angiography may be necessary to inform treatment options. The longer a serous retinal or RPE detachment is permitted to linger, the higher the likelihood of functional vision loss.
Treatment must be individualized for each patient, utilizing both clinical and diagnostic data to guide treatment. Patients who exhibit signs of functional vision loss must be referred for treatment.
CSCR and the pachychoroid spectrum
There is compelling data demonstrating that CSCR patients tend to have an especially thick choroid,2 leading Dr. Pizzimenti to believe that the pachychoroid spectrum is somehow associated with the pathogenesis of CSCR.
Dr. Epshtein has noted that he detects pachychoroid pigment epitheliopathy in the fellow eyes of CSCR patients 90% to 95% of the time, suggesting that this is most likely a bilateral asymmetric condition and both eyes should be examined.
This does not significantly change the method of treatment but can assist in differential diagnosis. Measuring an unusually thick choroid can allow ECPs to narrow treatment options. With advancing research regarding CSCR, better treatments for CSCR will hopefully emerge soon.
Conclusion
There are many factors to consider when classifying CSCR and various tests to help identify signs of CSCR. Care for CSCR must be individualized to each patient, relying on factors such as vision, retinal/RPE alterations, and history of previous episodes.
Newer theories suggesting that CSCR is part of the pachychoroid spectrum will hopefully advance our understanding of the disease, allowing us to provide better treatments for our patients with CSCR.
