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Knowledge of pathologic patterns is necessary in medical retina, especially patterns that are unusual or rare. Pattern recognition skills are essential in medical retina. Lawrence Yannuzzi, MD, highlights four patterns that he believes are relevant in clinical practice and should be recognized–and not missed–by retinal specialists.
Reviewed by Orly Gal-Or, MD, Jesse McCann, MD, PhD, and Lawrence A. Yannuzzi, MD
Take-home: Knowledge of pathologic patterns is necessary in medical retina, especially patterns that are unusual or rare.
Pattern recognition skills are essential in medical retina. Lawrence Yannuzzi, MD, highlights four patterns that he believes are relevant in clinical practice and should be recognized–and not missed–by retinal specialists. The four patterns are:
A: Late-phase ICGA shows a dark lesion with an irregular curly edge circumferentially, a chrysanthemum lesion in a multifocal choroiditis. (Images courtesy of Lawrence Yannuzzi, MD)Multifocal choroiditis is a chronic, progressive, bilateral inflammatory chorioretinopathy that affects healthy, myopic white women and is not associated with any systemic or ocular diseases. Affected eyes have multiple punched-out chorioretinal lesions, peripapillary atrophy, and curvilinear chorioretinal streaks in the periphery.
These eyes are at high risk of secondary choroidal neovascularization (CNV), according to Dr. Yannuzzi. Dr. Yannuzzi is vice chairman and director of the LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear, and Throat Hospital, and founder of Vitreous Retina Macula Consultants of New York.
Dr. Yannuzzi described the case of a 37-year-old woman who presented with acute visual loss in her left eye. On clinical examination, three concentric areas consistent with an old fibrous scar, more recent proliferation, and a fresh bleeding membrane were observed. Spots and chrysanthemum lesions were visible in the periphery.
Chrysanthemum lesions are solid with curly edges circumferentially that stain with fluorescein angiography (FA). Indocyanine green angiography (ICGA), while not necessary for diagnosis, accentuates the number and density of the spots.
Optical coherence tomography (OCT) of the lesion shows some hyper-reflectivity underneath the retinal pigment epithelium (RPE), although as the disease is choroidal in nature, it does involve the RPE. This patient was observed.
B: Fundus autofluorescence imaging of an annular catenary curve lesion in a patient with chronic CSC.Central serous chorioretinopathy (CSC), which gives rise to neurosensory detachment at the posterior pole in otherwise healthy adults, is characterized by a number of different patterns: orange-appearing pigment epithelial detachments (PEDs), focal leaks with a “smoke stack” sign on FA, gravitating tracts predisposing to a bullous detachment, a thick choroid, subretinal fibrin, and RPE breaks, rips, and avulsions.
Various RPE features can leave a legacy that is best imaged on fundus autofluorescence (FAF). The newly described catenary curve pattern is characterized by lacy changes in the RPE on FAF.1 The ring-like arrangement occurs secondary to gravitational effects.
RPE blowouts in polypoidal choroidal vasculopathy
C: FA of a blowout in the pigment epithelium that caused a focal leak in a patient with PCV.
The diagnosis of CSC or polypoidal choroidal vasculopathy (PCV) can be challenging.2,3 A case of unusual CSC presentation was that of a 48-year-old man who had 3 D to 4 D of myopia with a thick choroid and no exudative changes.
On OCT, shallow irregular detachment of the pigment epithelium was seen. OCT angiography can detect type 1 neovascularization that has infiltrated the subpigment epithelial area.
After 9 years, the patient became symptomatic and presented with an irregular PED on OCT. Irregular PEDs should raise the suspicion for PCV.
On FA, a smoke stack at the margin of a PED is unusual in CSC. Blowout in the pigment epithelium caused a focal leak. On ICGA, staining in the central macula revealed occult neovascularization.
En-face OCT angiography showed polyps with a branching vascular network. The patient was treated with an injection of an anti-vascular endothelial growth factor drug that resulted in the flattening of the PED.
Stellate nonhereditary idiopathic foveomacular retinoschisis
D: En-face OCT of SNIFR.
A 26-year-old man presented with complaints of metamorphopsia in his left eye. Color photography showed a loss of transparency in the peripapillary area.
On FA, staining of the optic disc without leakage is observed. Near infrared imaging shows a schisis-like cavity with a geometric distribution around the disc.
Evaluation ruled out an optic pit that was not seen on enhanced, depth-imaging OCT of the disc, vitreous traction, compressive optic neuropathy, and x-linked retinoschisis. The patient was diagnosed with stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR).
This entity is a diagnosis of exclusion. It is unilateral in the peripapillary area and affects the outer plexiform layer.4 Some areas of SNIFR decrease after posterior vitreous detachment.
1. Dansingani KK, Balaratnasingam C, Mrejen S, et al. Annular lesions and catenary forms in chronic central serous chorioretinopathy. Am J Ophthalmol 2016;166:60-67.
2. Yannuzzi LA, Freund KB, Goldbaum M, et al. Polypoidal choroidal vasculopathy masquerading as central serous chorioretinopathy . Ophthalmology 2000; 107:767-777.
3. Le D, Yannuzzi LA, Spaide RF, et al. Microrips of retinal pigment epithelium. Arch Ophthalmol.1992;110:1443-1449.
4. Ober MD, Freund KB, Shah M, et al. Stellate nonhereditary idiopathic foveomacular retinoschisis Ophthalmology 2014;121:1406-1413.
Lawrence Yannuzzi, MD
Dr. Yannuzzi has no financial interest in any aspect of this report. This article was adapted from a presentation that Dr. Yannuzzi delivered at the Precision Ophthalmology 2016 meeting.