Study finds strong correlation between chorioretinal folds, systemic disease
CRFs are most easily detected with fluorescein angiography.
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Chorioretinal folds appear to be present on angiography and in descriptions of various disease states, yet they are greatly under-recognized, according to researchers at Emory.
“As one begins to recognize this clinical entity, some of the subtleties of its presentation enable an earlier ophthalmic diagnosis, as well as being able to suspect and diagnose the associated systemic disorders,” OSN Retina/Vitreous Board Member Timothy W. Olsen, MD, said.
In an observational, retrospective case series of 57 eyes with chorioretinal folds (CRFs), more than 50% of patients had some form of autoimmune disorder, foremost rheumatoid arthritis, posterior scleritis, thyroid eye disease and inflammatory bowel disease. Other inflammatory disorders — thyroid disease, systemic lupus erythematosus, psoriasis, polymyalgia rheumatica and vitiligo — are also associated, according to the study.
“The highly variable pattern of disease presentation in the choroid combined with the wide variety seen in disease etiology makes the presence of chorioretinal folds an important sign for clinicians to both recognize and evaluate carefully,” study co-author Olsen told Ocular Surgery News.
“Elevated intracranial pressures may also induce CRFs,” Olsen said. “However, our referral bias may have removed cases associated with increased intracranial pressure, as some of these may be outside of the referral patterns of a retina clinic and instead referred to neuro-ophthalmology. Nevertheless, this etiology should also be suspected, especially when there is a hyperopic shift or associated headaches. Lymphoma should also be kept in mind.”
The study authors said they were surprised that only 15% of cases were categorized as idiopathic.
“In the past, a much higher percentage of cases would be considered idiopathic,” Olsen said. “With improved diagnostic capabilities and new associated entities (eg, Lyme disease), we are now better at detecting disease etiologies.”
Fluorescein angiography
According to Olsen, chorioretinal folds are most easily detected using fluorescein angiography.
“During slit lamp biomicroscopy, we suggest using a thin, bright slit beam directed toward the vascular arcades,” he said. “Examination of the dark area adjacent to the beam of light highlights the CRFs, as they assume a glassy, boggy appearance, likely due to intrastromal fluid accumulation.”
Chorioretinal folds are more apparent just subjacent of the retina that is being directly illuminated, a technique that is much like using indirect illumination when examining corneal pathology, Olsen said.
Underlying disorders
Once a systemic disorder is detected, patients should be referred and treated.
“However, structural issues of the globe, usually hyperopia, may not require treatment outside of common refractive management, whereas uveal effusion syndrome may require treatment, depending upon severity,” Olsen said.
The study highlighted three key phenotypes in the evolution of a CRF-related maculopathy, which is slow to progress and directly associated with aging, according to Olsen. Ten study eyes had stage 3 CRF-related maculopathy, and four of those eyes had associated choroidal neovascularization.
“Treatments should be directed at detecting and managing the underlying systemic disorder. Only after an exhaustive search should the CRFs be designated as idiopathic, a category that continues to decline in relative proportion to other diagnoses and systemic disorders,” Olsen said.
Olsen said chorioretinal folds are commonly overlooked, including stage 3 CRF-related maculopathy that resembles the occult forms of exudative age-related macular degeneration.
“However, we found that the need for anti-VEGF therapy seems to be much less for CRFs than for AMD,” he said. In the case of a leakage pattern in the fovea that resembles occult choroidal neovascularization associated with AMD in a patient with rheumatoid arthritis, the etiology could be CRF-related maculopathy.
“Furthermore, the CRF-related maculopathy has a leakage pattern that may require fewer anti-VEGF injections,” Olsen said.
Detecting an underlying disorder in patients with chorioretinal folds has the potential for reducing morbidity and mortality, Olsen said. – by Bob Kronemyer