From the Department of Ophthalmology, Vita-Salute University, San Raffaele Scientific Institute, Milan, Italy.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Luisa Pierro, MD, Department of Ophthalmology, San Raffaele Scientific Institute, Via Olgettina, 60 20132 Milan, Italy. E-mail: firstname.lastname@example.org
A diagnosis of choroidal metastasis is usually based on patient’s clinical history, the tumor’s ophthalmoscopic appearance, and instrumental imaging results such as ultrasonography, fluorescein angiography, fundus autofluorescence, indocyanine green angiography, and optical coherence tomography. Spectral-domain optical coherence tomography (SD-OCT) has recently provided additional useful information in clinical diagnostic procedures.
We describe a case in which suspected choroidal metastasis, supported by several instrumental imaging techniques, presented peculiar OCT features at different stages that successfully guided us to primary tumor identification.
A healthy 46-year-old man presented for gradual impairment of vision in the left eye during the previous 4 months. Best-corrected visual acuity was 20/20 in the right eye and 20/100 in the left eye. Fundus observation showed a small serous neuroepithelial elevation confined in the upper-temporal quadrant of the right eye (Fig. 1A) and a patchy pigmented lesion with yellowish subretinal spots at the posterior pole with serous inferior retinal detachment in the left eye (Fig. 1B). The features were consistent with leopard spot retinopathy.
Figure 1. (A) Small serous neuroepiihelial elevation confined in the upper-temporal quadrant of the right eye. (B) Leopard spot retinopathy at the posterior pole with serous inferior retinal detachment in the left eye.
Fluorescein angiography showed a late dimly hyperfluorescent lesion in the right eye, corresponding to the small serous retinal detachment (Fig. 2A). Fluorescein angiography in the left eye showed a wide hyperfluorescent area at the posterior pole with patchy hypofluorescent spots that were unchanged during the course of the examination (Fig. 2B), and indocyanine green angiography revealed a granular hypofluorescence in the same area. Fundus autofluorescence of the right eye was unremarkable, whereas in the left eye it showed an inverted pattern compared with fluorescein angiography: a hypoautofluorescent area at the posterior pole with hyperautofluorescent spots corresponding to the hypofluorescent spots on fluorescein angiography (Fig. 3). SD-OCT of the right eye confirmed the macula-sparing serous neuroepithelial elevation over normal retinal pigment epithelium (RPE) (Fig. 4A). SD-OCT of the left eye showed in the context of leopard spot retinopathy a pattern of hyperintense irregular spots within the photoreceptor layer and the RPE, subretinal fluid, and marked irregularity of the RPE, with thickening and gross undulation (Fig. 4B). Ultrasonography showed increased choroidal thickness without evident solid lesion and confined macula-on serous retinal detachment in the left eye (Fig. 5).
Figure 2. (A) Late dimly hyperfluorescent lesion in the right eye superotemporal to the macula, corresponding to the small serous retinal detachment. (B) Wide hyperfluorescent area at the posterior pole of the left eye with patchy hypofluorescent spots that were unchanged during the course of the examination.
Figure 3. Fundus autofluorescence of the left eye shows an inverted pattern compared with fluorescein angiography.
Figure 4. (A) Spectral-domain optical coherence tomography (SD-OCT) of the right eye in the context of leopard spot retinopathy shows a pattern of hyperintense irregular spots in the context of the photoreceptor layer and in the retinal pigment epithelium (RPE), subretinal fluid, and marked irregularity of the RPE, with thickening and gross undulation. (B) SD-OCT of the left eye shows a macula-sparing serous neuroepithelial elevation over normal retinal pigment epithelium.
Figure 5. Ultrasonography of the left eye show increased choroidal thickness without evident solid lesion and confined macula-on serous retinal detachment.
It was recommended that urgent whole-body computerized tomography scan and complete blood examination be performed. The patient was admitted to our department 60 days later because of acute onset of cough and fever.
Further ophthalmologic evaluation was performed during hospitalization. Best-corrected visual acuity was 20/30 in the right eye and 20/200 in the left eye. Mild reduction of the serous neuroepithelial elevation in the right eye was noted. Fundus autofluorescence and fluorescein angiography demonstrated a new round granular hyperfluorescent area in this region (Fig. 6), whereas SD-OCT scan highlighted the same pattern of the posterior pole of the left eye: irregularity and thickening of the RPE (Fig. 7). The left eye was unchanged.
Figure 6. New round granular hyperfluorescent area in the right eye.
Figure 7. Spectral-domain optical coherence tomography scan of the right eye over the new granular hyperfluorescent lesion, showing the same pattern of the posterior pole in the left eye: irregularity and thickening of the retinal pigment epithelium.
Further investigations were consistent with lung adenocarcinoma, with vertebral and sellar region metastases.
None of the imaging techniques is evidently diagnostic for choroidal metastasis if considered alone. The SD-OCT pattern in choroidal metastasis has been recently demonstrated as hyperintense irregular spots in the photoreceptor layer and in the context of the RPE, subretinal fluid, marked irregularity of the RPE, with thickening and gross undulation.1,2 In agreement with Arevalo et al., we recognized on SD-OCT the anterior displacement of the photoreceptor layer by subretinal fluid overlying a hyperreflective thickened RPE-choriocapillaris complex.2
Our patient’s SD-OCT result fit with these patterns, and we associated it for the first time in the literature with leopard spot retinopathy.3 This retinopathy has been indeed reported to be associated with leukemia, the systemic form of large-cell non-Hodgkin lymphoma, chronic central serous chorioretinopathy, hypertensive choroidopathy, organ transplant chorioretinopathy, idiopathic uveal effusion syndrome, neonatal adrenoleukodystrophy, and systemic carcinoma with bilateral diffuse uveal melanocytic proliferation.4–10 Cohen et al. reported few cases of the idiopathic form of unilateral leopard spot retinopathy.11
Hence we consider our case clinically interesting because we associated a choroidal metastasis with leopard spot retinopathy, and because we could observe the natural history of choroidal metastases over a 3-month period due to the presence of disease in both eyes at different stages. We described an SD-OCT picture of early choroidal metastasis: early acute phases were characterized by exudation and serous neuroepithelial detachment underlined by apparently intact RPE, without any further photoreceptor alteration. Later phases showed fluid reabsorption and irregularity of the RPE, with thickening and undulation; funduscopic features of leopard spot were noted in the context of this RPE alteration. We hypothesize that neuroretinal detachment and subretinal fluid of the early stages is a result of tumor cell activity, possibly through choroidal vascular hyperpermeability and RPE barrier disruption.12 In addition, later stage pattern could be characterized by direct infiltration of tumor cell mass underneath the RPE, causing its irregularity and thickening.
OCT could not be considered an exhaustive diagnostic tool in case of suspected metastasis, but we emphasize the central role of SD-OCT among instrumental imaging procedures and for final successful diagnosis. In the absence of an evident etiology, a choroidal metastasis should be always ruled out with an SD-OCT finding of serous neuroepithelial detachment underlined by apparently intact RPE, even in the absence of solid lesions detected by ultrasonography. A metastasis should also be ruled out in case of leopard spot retinopathy in apparently healthy patients.
- Natesh S, Chin KJ, Finger PT. Choroidal metastases fundus autofluorescence imaging: correlation to clinical, OCT, and fluorescein angiographic findings. Ophthalmic Surg Lasers Imaging. 2010;41:406–412.
- Arevalo JF, Fernandez CF, Garcia RA. Optical coherence tomography characteristics of choroidal metastasis. Ophthalmology. 2005; 112:1612–1619.
- Okuda T, Higashide T, Wakabayashi Y, Nishimura A, Sugiyama K. Fundus autofluorescence and spectral-domain optical coherence tomography findings of leopard spots in nanophthalmic uveal effusion syndrome. Graefes Arch Clin Exp Ophthalmol. 2010;248: 1199–1202.
- Barr CC, Zimmerman LE, Curtin VT, Font RL. Bilateral diffuse melanocytic uveal tumors associated with systemic malignant neoplasms: a recently recognized syndrome. Arch Ophthalmol. 1982;100:249–255.
- Lyons CJ, Castano G, McCormick AQ, Applegarth D. Leopard spot retinal pigmentation in infancy indicating a peroxisomal disorder. Br J Ophthalmol. 2004;88:191–192.
- Robert MP, Faure C, Reman O, Miocque S. Leopard spot retinopathy: an early clinical marker of leukaemia recurrence?Ann Hematol. 2008;87:927–929.
- Nakamura T, Miyauchi O, Mizota A, Adachi-Usami E. Bilateral leopard spot fundus of hypertensive choroidopathy and its visual function. Ophthalmologica. 2002;216:228–229.
- Iida T, Spaide RF, Haas A, Yannuzzi LA, Jampol LM, Lesser RL. Leopard-spot pattern of yellowish subretinal deposits in central serous chorioretinopathy. Arch Ophthalmol. 2002;120:37–42.
- Elagouz M, Stanescu-Segall D, Jackson TL. Uveal effusion syndrome. Surv Ophthalmol. 2010;55:134–145.
- Wheatcroft S, Watts P, McAllister J. Leopard spot retina. Eye. 1993; 7:189–190.
- Cohen SY, Massin P, Quentel G. Clinicopathologic reports, case reports, and small case series: unilateral, idiopathic leopard-spot lesion of the retinal pigment epithelium. Arch Ophthalmol. 2002;120:512–516.
- Lee SY, Joe SG, Kim JG, Chung H, Yoon YH. Optical coherence tomography evaluation of detached macula from rhegmatogenous retinal detachment and central serous chorioretinopathy. Am J Ophthalmol. 2008;145:1071–1076.