Multimodal imaging of sarcoid choroidal granulomas
© Modi et al.; licensee Springer. 2013
Received: 30 June 2013
Accepted: 8 August 2013
Published: 23 August 2013
Enhanced-depth imaging optical coherence tomography (EDI-OCT) provides high-resolution imaging of the choroid. Herein, we report multimodal imaging, including EDI-OCT, of a case of sarcoid choroidal granulomas.
A 63-year-old female with biopsy-supported sarcoidosis presented with unilateral multifocal choroidal granulomas. Enhanced-depth imaging optical coherence tomography (EDI-OCT) demonstrated a homogenous hyporeflective choroidal lesion with choriocapillaris thinning and sparing of the surrounding choroid. The patient was started on oral steroids with a weekly taper schedule. Within 5 weeks, the choroidal lesions had clinically resolved with return of normal-appearing choroidal architecture on EDI-OCT. Indocyanine green angiography, however, demonstrated hypofluoresence at the sites of choroidal granulomas 11 months after the clinical resolution, suggesting a longstanding choroidal perfusion deficit undetected by OCT.
Choroidal imaging via EDI-OCT provides detailed morphologic information of sarcoid granulomas and can accurately demonstrate structural resolution of the lesions.
KeywordsChoroidal granuloma Sarcoidosis Imaging Enhanced-depth imaging optical coherence tomography Indocyanine green
This is a longitudinal case report of a patient with unilateral multifocal choroidal granulomas in the setting of systemic sarcoidosis. Multimodal imaging was used to aid in diagnosis and follow through resolution.
Choroidal granulomas in the absence of anterior uveitis are a rare but well-recognized manifestation of sarcoidosis, occurring in approximately 5% of patients with ocular sarcoidosis . Accurate imaging to correctly differentiate granulomas from amelanotic melanomas and choroidal metastasis is critical prior to developing a treatment plan. Enhanced-depth imaging optical coherence tomography (EDI-OCT), a novel imaging modality that provides excellent morphologic details of the choroid, has recently been used to evaluate choroidal pathology in various disease states including central serous chorioretinopathy , myopia , macular degeneration , choroidal tumors , and a Toxocara optic nerve granuloma . However, EDI-OCT imaging of choroidal granulomas in the setting of sarcoidosis has not been previously reported.
Herein, we report a case of unilateral multifocal choroidal sarcoid granulomas that underwent multimodal imaging including EDI-OCT, fluorescein angiography (FA), and indocyanine green angiography (ICG).
Report of a case
A 63-year-old black female with recently diagnosed biopsy-supported systemic sarcoidosis involving the lungs and skin presented with an insidious, painless decrease in vision of the right eye with metamorphopsia over a 3-month period. A recent medical evaluation revealed lymphadenopathy on chest computed tomography, erythema nodosum of the lower extremities, and a skin biopsy demonstrating noncaseating granulomas consistent with sarcoidosis. She had not started any systemic immunosuppression at the time of presentation. The patient consented for this case to be presented.
Choroidal granulomas manifesting as the sole lesion in ocular sarcoidosis has been previously described [1, 7–10]. Desai et al. reported on the largest case series of choroidal granulomas in nine patients with biopsy-proven sarcoidosis . Eight patients presented with solitary choroidal granulomas, and one patient presented with multifocal involvement. All the patients were started on oral prednisone with nine out of nine eyes demonstrating a reduction in size of the granuloma and two out of nine eyes demonstrating complete resolution. Five of the eyes experienced a recurrence, however, with an average time to recurrence of 7.6 months .
EDI-OCT imaging of the choroid, in this case, provided accurate morphologic characterization of the choroidal granuloma and its subsequent resolution. Salman et al. previously characterized tuberculous granulomas via a Stratus OCT (Carl Zeiss Meditec Inc., Dublin, CA, USA), which demonstrated the relationship of the anterior granuloma with the RPE and retina . However, the morphology and the posterior margin were not well characterized. The resolving ability of EDI-OCT for choroidal lesions has been reported as 1 mm in depth, allowing for accurate qualitative and quantitative evaluation of small choroidal lesions too small to be characterized via ultrasound . Additionally, EDI-OCT may provide the ability to detect interval change of the granuloma size in response to treatment and, potentially, early recurrence.
After 1 month of treatment on oral steroids, the granulomas in this patient clinically disappeared with residual RPE mottling and hypopigmentation over the sites of prior granulomas. Repeat imaging via EDI-OCT demonstrated resolution of the lesion with an apparent return of normal choroidal architecture. The ICG, however, continued to demonstrate hypofluorescence in the involved sites 11 months after clinical resolution. It is possible that this may represent a longstanding choroidal perfusion deficit that cannot be resolved via OCT. Subsequent EDI-OCT has not demonstrated early recurrence of these lesions, and the patient will continue maintenance therapy with low-dose steroids.
In conclusion, choroidal imaging via EDI-OCT provides detailed morphologic information of sarcoid granulomas and can accurately demonstrate structural resolution of the lesions. Further studies are required to identify defining features that may aid in differentiation from other inflammatory and neoplastic processes.
YSM is a second year ophthalmology resident at the Bascom Palmer Eye Institute. He has a clinical and academic interest in uveitis, choroidal tumors, and vitreoretinal surgery. AE is a third year medical student at the University of Miami School of Medicine. She is interested in ophthalmology. SB was a former Uveitis fellow at the Bascom Palmer Eye Institute under the auspices of Dr. Janet Davis and Dr. Thomas Albini; she is currently pursuing a vitreoretinal fellowship at Cornell University. JWH is a professor of Ophthalmology at the Bascom Palmer Eye Institute. His clinical interests include ocular oncology and vitreoretinal diseases and surgery. TA is an associate professor of Ophthalmology at the Bascom Palmer Eye Institute. His clinical interests include uveitis and vitreoretinal diseases and surgery.
Best-corrected visual acuity
Enhanced-depth imaging optical coherence tomography
Indocyanine green angiography
Retinal pigment epithelium
Spectral domain optical coherence tomography
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