TY - JOUR
T1 - Focal scleral nodule
T2 - a new name for solitary idiopathic choroiditis and unifocal helioid choroiditis
AU - Fung, Adrian T.
AU - Waldstein, Sebastian M.
AU - Gal-Or, Orly
AU - Pellegrini, Marco
AU - Preziosa, Chiara
AU - Shields, Jerry A.
AU - Welch, R. Joel
AU - Dolz-Marco, Rosa
AU - Sarraf, David
AU - Nagiel, Aaron
AU - Lalane, Robert
AU - Jung, Jesse J.
AU - Ghazi, Nicola G.
AU - Ramtohul, Prithvi
AU - Arnold, Jennifer J.
AU - Sakurada, Yoichi
AU - Choudhry, Netan
AU - Balaratnasingam, Chandrakumar
AU - Freund, K. Bailey
AU - Shields, Carol L.
PY - 2020/11/1
Y1 - 2020/11/1
N2 - Purpose: To evaluate multimodal imaging findings of solitary idiopathic choroiditis (SIC; also known as unifocal helioid choroiditis) to clarify its origin, anatomic location, and natural course. Design: Multicenter retrospective observational case series. Participants: Sixty-three patients with SIC in 1 eye. Methods: Demographic and clinical data were collected. Multimodal imaging included color fundus photography, OCT (including swept-source OCT), OCT angiography (OCTA), fundus autofluorescence, fluorescein and indocyanine green angiography, and B-scan ultrasonography. Main Outcome Measures: Standardized grading of imaging features. Results: Mean age at presentation was 56 ± 15 years (range, 12–83 years). Mean follow-up duration in 39 patients was 39 ± 55 months (range, 1 month–25 years). The lesions measured a mean of 2.4 × 2.1 mm in basal diameter, were located inferior (64%) or nasal to the optic disc, and appeared yellow (53%). No systemic associations were found. The lesions all appeared as an elevated subretinal mass, with OCT demonstrating all lesions to be confined to the sclera, not the choroid. On OCT, the deep lesion margin was visible in 12 eyes with a mean lesion thickness of 0.6 mm. Overlying choroidal thinning or absence was seen in 95% (mean choroidal thickness, 28 ± 35 μm). Mild subretinal fluid was observed overlying the lesions in 9 patients (14%). Retinal pigment epithelial disruption and overlying retinal thinning was observed in 56% and 57%, respectively. OCT angiography was performed in 13 eyes and demonstrated associated choroidal and lesional flow voids. Four lesions (6%) were identified at the macula, leading to visual loss in 1 patient. One lesion demonstrated growth and another lesion showed spontaneous resolution. Conclusions: In this largest series to date, multimodal imaging of SIC demonstrated a scleral location in all patients. The yellow and white clinical appearance may be related to scleral unmasking resulting from atrophy of overlying tissues. Additional associated features included documentation of deep margin on swept-source OCT, trace subretinal fluid in a few patients, and OCTA evidence of lesional flow voids. Because of the scleral location of this lesion in every patient, a new name, focal scleral nodule, is proposed.
AB - Purpose: To evaluate multimodal imaging findings of solitary idiopathic choroiditis (SIC; also known as unifocal helioid choroiditis) to clarify its origin, anatomic location, and natural course. Design: Multicenter retrospective observational case series. Participants: Sixty-three patients with SIC in 1 eye. Methods: Demographic and clinical data were collected. Multimodal imaging included color fundus photography, OCT (including swept-source OCT), OCT angiography (OCTA), fundus autofluorescence, fluorescein and indocyanine green angiography, and B-scan ultrasonography. Main Outcome Measures: Standardized grading of imaging features. Results: Mean age at presentation was 56 ± 15 years (range, 12–83 years). Mean follow-up duration in 39 patients was 39 ± 55 months (range, 1 month–25 years). The lesions measured a mean of 2.4 × 2.1 mm in basal diameter, were located inferior (64%) or nasal to the optic disc, and appeared yellow (53%). No systemic associations were found. The lesions all appeared as an elevated subretinal mass, with OCT demonstrating all lesions to be confined to the sclera, not the choroid. On OCT, the deep lesion margin was visible in 12 eyes with a mean lesion thickness of 0.6 mm. Overlying choroidal thinning or absence was seen in 95% (mean choroidal thickness, 28 ± 35 μm). Mild subretinal fluid was observed overlying the lesions in 9 patients (14%). Retinal pigment epithelial disruption and overlying retinal thinning was observed in 56% and 57%, respectively. OCT angiography was performed in 13 eyes and demonstrated associated choroidal and lesional flow voids. Four lesions (6%) were identified at the macula, leading to visual loss in 1 patient. One lesion demonstrated growth and another lesion showed spontaneous resolution. Conclusions: In this largest series to date, multimodal imaging of SIC demonstrated a scleral location in all patients. The yellow and white clinical appearance may be related to scleral unmasking resulting from atrophy of overlying tissues. Additional associated features included documentation of deep margin on swept-source OCT, trace subretinal fluid in a few patients, and OCTA evidence of lesional flow voids. Because of the scleral location of this lesion in every patient, a new name, focal scleral nodule, is proposed.
UR - http://www.scopus.com/inward/record.url?scp=85085745289&partnerID=8YFLogxK
U2 - 10.1016/j.ophtha.2020.04.018
DO - 10.1016/j.ophtha.2020.04.018
M3 - Article
C2 - 32507351
AN - SCOPUS:85085745289
SN - 0161-6420
VL - 127
SP - 1567
EP - 1577
JO - Ophthalmology
JF - Ophthalmology
IS - 11
ER -