Graefes Arch Clin Exp Ophthalmol DOI 10.1007/s00417-015-2987-1
LETTER TO THE EDITOR
Choroidal involvement in Rosai–Dorfman syndrome may be depicted and followed using enhanced depth imaging optical coherence tomography (EDI-OCT) Janelle Fassbender 1 & Shlomit Schaal 1
Received: 23 January 2015 / Accepted: 2 March 2015 # Springer-Verlag Berlin Heidelberg 2015
Dear Editor, We read with interest the recently published case report by Vermeulen et al. [1] regarding BRosai–Dorfman disease presenting as choroidal melanoma^. We previously published a similar presentation of a patient that underwent systemic work-up for bilateral choroidal masses [2]. Our patient initially presented with minimal visual complaints and bestcorrected visual acuity of 20/20 OU. Sub-papillomacular bundle, amelanotic choroidal masses OU were discovered on dilated fundus exam. Initial work-up demonstrated focal pigment epithelial detachment OD but overall intact outer retinal layers OU on spectral domain optical coherence tomography (SD-OCT). Unlike the case report by Vermeulen et al. (2013), our patient had positive systemic findings, including bilateral renal masses. These masses were accessible to biopsy and revealed S100- and CD163-positive lymphocytes and negative immunostaining for melanoma-specific markers, confirming a diagnosis of Rosai–Dorfman syndrome (RDS). This patient recently completed 6 years of follow-up in our clinic with stable visual acuity and only mild progression of the choroidal masses (Fig. 1a, b). For follow-up, we utilized the enhanced depth imaging technique of spectral domain OCT (EDI-OCT; Heidelberg; Heidelberg, Germany) to further evaluate the lesions. OCT studies revealed smooth, sloping, and homogenous masses with extensive choroidal involvement (Fig. 1c, d). The overlying retinal architecture
* Shlomit Schaal [email protected] 1
Department of Ophthalmology and Visual Sciences, University of Louisville, 301 E. Muhammad Ali Boulevard, Louisville, KY 40202, USA
remained intact in spite of anterior displacement of the foveal pit OD. Sinus histiocytosis with massive lymphadenopathy or RDS was first described by Rosai and Dorfman [3]. Uveal involvement in RDS has been rarely reported in the literature and in the largest case series [4], the founding authors describe 13 out of 113 cases with ophthalmic manifestations. Only one of these involved the uvea. Since this time, a consecutive study of pathological specimen of the orbit revealed a 0.09 % incidence of uveal RDS [5]. With new advancements in choroidal imaging, we now have the opportunity to further characterize this intriguing choroidal pathology to hopefully avoid the outcome Vermeulen et al. encountered. Detailed choroidal imaging in standard OCT falls short due to various factors, including increased displacement from zero delay, inherent limitations in Fourier domain systems, and wavelength-dependent light scatter within the image path [6]. Fortunately, standard spectral domain-OCT (Heidelberg and Cirrus; Carl Zeiss, Jena, Germany) is able to overcome these factors by making a fairly simple adjustment: moving the instrument closer to the eye. This moves the target closer to zero-delay, where the inverted image rather than the real, upright image is displayed resulting in focused illumination on the choroid rather than the inner retina. Several studies have recently been published evaluating choroidal masses using EDI-OCT. Although higher-volume studies have found a less than 50 % rate of images acceptable for analysis, important characteristics of melanoma versus nevus were elucidated, including choroidal shadowing below a choroidal nevus and choriocapillaris thinning above [7]. Shields et al. [8] also compared choroidal nevus to small choroidal melanoma on EDI-OCT, the latter of which frequently causes subretinal fluid, subretinal lipofuscin deposition, and a shaggy overlying photoreceptor layer. In our patient, the
Graefes Arch Clin Exp Ophthalmol
Fig. 1 Color fundus photography and enhanced depth imaging OCT of bilateral choroidal RDS after 6 years of follow-up. a Recent color fundus photo of right eye demonstrating homogenous ring-like yellow choroidal mass. b Color photo of left eye demonstrating a similar choroidal lesion.
c Enhanced depth imaging OCT (EDI-OCT) of right eye reveals a large pigment epithelial detachment overlying a deep choroidal mass characterized by smooth contour. d EDI-OCT of left eye demonstrating similar choroidal lesion. Photoreceptor layers are preserved in both eyes
choroidal RDS lesions appear more similar to a choroidal nevus on EDI-OCT (Fig. 1c, d). Choroidal masses in RDS are rare, yet they may present in a uni- or bilateral fashion and cause visual field defects. These must be distinguished from aggressive lesions such as melanoma or metastasis and systemic work-up is imperative. Advancements in choroidal imaging, i.e., EDI-OCT, may help to avoid drastic measures such as enucleation to differentiate benign from malignant lesions.
Albrecht Graefes Arch Klin Exp Ophthalmol 251(1):295–299. doi: 10.1007/s00417-012-2175-5 Barak Y, Tong MG, Purkiss TJ, Schaal S (2012) Rosai–Dorfman disease diagnosed because of bilateral choroidal masses. Retin Cases Brief Rep 6(3):326–329. doi:10.1097/ICB.0b013e3182378dd8 Rosai J, Dorfman RF (1969) Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 87(1):63–70 Foucar E, Rosai J, Dorfman RF (1979) The ophthalmologic manifestations of sinus histiocytosis with massive lymphadenopathy. Am J Ophthalmol 87(3):354–367 Vemuganti GK, Naik MN, Honavar SG (2008) Rosai–Dorfman disease of the orbit. J Hematol Oncol 1:7. doi:10.1186/17568722-1-7 Wong IY, Koizumi H, Lai WW (2011) Enhanced depth imaging optical coherence tomography. Ophthalmic Surg Lasers Imaging Off J Int Soc Imaging Eye 42(Suppl):S75–S84. doi:10.3928/ 15428877-20110627-07 Shah SU, Kaliki S, Shields CL, Ferenczy SR, Harmon SA, Shields JA (2012) Enhanced depth imaging optical coherence tomography of choroidal nevus in 104 cases. Ophthalmology 119(5):1066–1072. doi:10.1016/j.ophtha.2011.11.001 Shields CL, Kaliki S, Rojanaporn D, Ferenczy SR, Shields JA (2012) Enhanced depth imaging optical coherence tomography of small choroidal melanoma: comparison with choroidal nevus. Arch Ophthalmol 130(7):850–856. doi:10.1001/archophthalmol.2012. 1135
Acknowledgments This study was supported in part by an unrestricted grant from Research to Prevent Blindness.
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Conflict of interest None of the authors have any conflicts of interest to disclose.
References 1.
Vermeulen TL, Isaacs TW, Spagnolo D, Amanuel B (2013) Rosai– Dorfman disease presenting as choroidal melanoma: a case report and review of the literature. Graefes Arch Clin Exp Ophthalmol
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LETTER TO THE EDITOR
Choroidal involvement in Rosai–Dorfman syndrome may be depicted and followed using enhanced depth imaging optical coherence tomography (EDI-OCT) Janelle Fassbender 1 & Shlomit Schaal 1
Received: 23 January 2015 / Accepted: 2 March 2015 # Springer-Verlag Berlin Heidelberg 2015
Dear Editor, We read with interest the recently published case report by Vermeulen et al. [1] regarding BRosai–Dorfman disease presenting as choroidal melanoma^. We previously published a similar presentation of a patient that underwent systemic work-up for bilateral choroidal masses [2]. Our patient initially presented with minimal visual complaints and bestcorrected visual acuity of 20/20 OU. Sub-papillomacular bundle, amelanotic choroidal masses OU were discovered on dilated fundus exam. Initial work-up demonstrated focal pigment epithelial detachment OD but overall intact outer retinal layers OU on spectral domain optical coherence tomography (SD-OCT). Unlike the case report by Vermeulen et al. (2013), our patient had positive systemic findings, including bilateral renal masses. These masses were accessible to biopsy and revealed S100- and CD163-positive lymphocytes and negative immunostaining for melanoma-specific markers, confirming a diagnosis of Rosai–Dorfman syndrome (RDS). This patient recently completed 6 years of follow-up in our clinic with stable visual acuity and only mild progression of the choroidal masses (Fig. 1a, b). For follow-up, we utilized the enhanced depth imaging technique of spectral domain OCT (EDI-OCT; Heidelberg; Heidelberg, Germany) to further evaluate the lesions. OCT studies revealed smooth, sloping, and homogenous masses with extensive choroidal involvement (Fig. 1c, d). The overlying retinal architecture
* Shlomit Schaal [email protected] 1
Department of Ophthalmology and Visual Sciences, University of Louisville, 301 E. Muhammad Ali Boulevard, Louisville, KY 40202, USA
remained intact in spite of anterior displacement of the foveal pit OD. Sinus histiocytosis with massive lymphadenopathy or RDS was first described by Rosai and Dorfman [3]. Uveal involvement in RDS has been rarely reported in the literature and in the largest case series [4], the founding authors describe 13 out of 113 cases with ophthalmic manifestations. Only one of these involved the uvea. Since this time, a consecutive study of pathological specimen of the orbit revealed a 0.09 % incidence of uveal RDS [5]. With new advancements in choroidal imaging, we now have the opportunity to further characterize this intriguing choroidal pathology to hopefully avoid the outcome Vermeulen et al. encountered. Detailed choroidal imaging in standard OCT falls short due to various factors, including increased displacement from zero delay, inherent limitations in Fourier domain systems, and wavelength-dependent light scatter within the image path [6]. Fortunately, standard spectral domain-OCT (Heidelberg and Cirrus; Carl Zeiss, Jena, Germany) is able to overcome these factors by making a fairly simple adjustment: moving the instrument closer to the eye. This moves the target closer to zero-delay, where the inverted image rather than the real, upright image is displayed resulting in focused illumination on the choroid rather than the inner retina. Several studies have recently been published evaluating choroidal masses using EDI-OCT. Although higher-volume studies have found a less than 50 % rate of images acceptable for analysis, important characteristics of melanoma versus nevus were elucidated, including choroidal shadowing below a choroidal nevus and choriocapillaris thinning above [7]. Shields et al. [8] also compared choroidal nevus to small choroidal melanoma on EDI-OCT, the latter of which frequently causes subretinal fluid, subretinal lipofuscin deposition, and a shaggy overlying photoreceptor layer. In our patient, the
Graefes Arch Clin Exp Ophthalmol
Fig. 1 Color fundus photography and enhanced depth imaging OCT of bilateral choroidal RDS after 6 years of follow-up. a Recent color fundus photo of right eye demonstrating homogenous ring-like yellow choroidal mass. b Color photo of left eye demonstrating a similar choroidal lesion.
c Enhanced depth imaging OCT (EDI-OCT) of right eye reveals a large pigment epithelial detachment overlying a deep choroidal mass characterized by smooth contour. d EDI-OCT of left eye demonstrating similar choroidal lesion. Photoreceptor layers are preserved in both eyes
choroidal RDS lesions appear more similar to a choroidal nevus on EDI-OCT (Fig. 1c, d). Choroidal masses in RDS are rare, yet they may present in a uni- or bilateral fashion and cause visual field defects. These must be distinguished from aggressive lesions such as melanoma or metastasis and systemic work-up is imperative. Advancements in choroidal imaging, i.e., EDI-OCT, may help to avoid drastic measures such as enucleation to differentiate benign from malignant lesions.
Albrecht Graefes Arch Klin Exp Ophthalmol 251(1):295–299. doi: 10.1007/s00417-012-2175-5 Barak Y, Tong MG, Purkiss TJ, Schaal S (2012) Rosai–Dorfman disease diagnosed because of bilateral choroidal masses. Retin Cases Brief Rep 6(3):326–329. doi:10.1097/ICB.0b013e3182378dd8 Rosai J, Dorfman RF (1969) Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 87(1):63–70 Foucar E, Rosai J, Dorfman RF (1979) The ophthalmologic manifestations of sinus histiocytosis with massive lymphadenopathy. Am J Ophthalmol 87(3):354–367 Vemuganti GK, Naik MN, Honavar SG (2008) Rosai–Dorfman disease of the orbit. J Hematol Oncol 1:7. doi:10.1186/17568722-1-7 Wong IY, Koizumi H, Lai WW (2011) Enhanced depth imaging optical coherence tomography. Ophthalmic Surg Lasers Imaging Off J Int Soc Imaging Eye 42(Suppl):S75–S84. doi:10.3928/ 15428877-20110627-07 Shah SU, Kaliki S, Shields CL, Ferenczy SR, Harmon SA, Shields JA (2012) Enhanced depth imaging optical coherence tomography of choroidal nevus in 104 cases. Ophthalmology 119(5):1066–1072. doi:10.1016/j.ophtha.2011.11.001 Shields CL, Kaliki S, Rojanaporn D, Ferenczy SR, Shields JA (2012) Enhanced depth imaging optical coherence tomography of small choroidal melanoma: comparison with choroidal nevus. Arch Ophthalmol 130(7):850–856. doi:10.1001/archophthalmol.2012. 1135
Acknowledgments This study was supported in part by an unrestricted grant from Research to Prevent Blindness.
2.
3.
4.
5.
6.
Conflict of interest None of the authors have any conflicts of interest to disclose.
References 1.
Vermeulen TL, Isaacs TW, Spagnolo D, Amanuel B (2013) Rosai– Dorfman disease presenting as choroidal melanoma: a case report and review of the literature. Graefes Arch Clin Exp Ophthalmol
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8.
