TY - JOUR
T1 - STELLATE MULTIFORM AMELANOTIC CHOROIDOPATHY
T2 - Clinical and Multimodal Imaging Features
AU - Ramtohul, Prithvi
AU - Pellegrini, Marco
AU - Pichi, Francesco
AU - Preziosa, Chiara
AU - Marchese, Alessandro
AU - Cicinelli, Maria Vittoria
AU - Miserocchi, Elisabetta
AU - Mundae, Rusdeep
AU - Mrejen, Sarah
AU - Rofagha, Soraya
AU - Mein, Calvin E.
AU - Mein, Luke
AU - Ober, Michael D.
AU - Cunha de Souza, Eduardo
AU - Cohen, Salomon Yves
AU - van Dijk, Elon H. C.
AU - Jampol, Lee
AU - Boon, Camiel J. F.
AU - Freund, K. Bailey
N1 - Funding Information: This work was supported by The Macula Foundation Inc., New York, New York. P. Ramtohul was supported by The Philippe Foundation. Publisher Copyright: © Lippincott Williams & Wilkins.
PY - 2023/9/1
Y1 - 2023/9/1
N2 - PURPOSE: To describe the clinical and multimodal imaging features of stellate multiform amelanotic choroidopathy (SMACH; also known as serous maculopathy due to aspecific choroidopathy). METHODS: Retrospective observational case series of eyes presenting with SMACH. Multimodal imaging including fundus photography, optical coherence tomography (OCT), OCT angiography (OCTA), and indocyanine green angiography (ICGA) was analyzed. RESULTS: Eighteen eyes from 18 patients (mean age: 28 ± 19 years) were included. The mean follow-up duration was 9 years. Ophthalmoscopy showed a yellowish orange, dendriform choroidal lesion. At presentation, subretinal fluid (SRF) was seen in 10 of 18 cases (56%). Eight patients (44%) showed no evidence of SRF during a mean follow-up of 6 years. Cross-sectional OCT showed hyperreflective fibrous-like changes within the inner choroid with choriocapillaris flow preservation on OCTA. En face OCT showed a hyperreflective choroidal lesion with finger-like projections oriented in a stellate configuration. On ICGA, SMACH showed early and late hypofluorescence. None of the cases showed lesion growth. CONCLUSION: SMACH seems to be a unilateral choroidopathy characterized by distinctive multimodal imaging features. As SRF was absent in some cases, while a dendriform pattern was a consistent finding in all eyes, the authors propose renaming this entity "stellate multiform amelanotic choroidopathy," a name that retains its previous abbreviation "SMACH."
AB - PURPOSE: To describe the clinical and multimodal imaging features of stellate multiform amelanotic choroidopathy (SMACH; also known as serous maculopathy due to aspecific choroidopathy). METHODS: Retrospective observational case series of eyes presenting with SMACH. Multimodal imaging including fundus photography, optical coherence tomography (OCT), OCT angiography (OCTA), and indocyanine green angiography (ICGA) was analyzed. RESULTS: Eighteen eyes from 18 patients (mean age: 28 ± 19 years) were included. The mean follow-up duration was 9 years. Ophthalmoscopy showed a yellowish orange, dendriform choroidal lesion. At presentation, subretinal fluid (SRF) was seen in 10 of 18 cases (56%). Eight patients (44%) showed no evidence of SRF during a mean follow-up of 6 years. Cross-sectional OCT showed hyperreflective fibrous-like changes within the inner choroid with choriocapillaris flow preservation on OCTA. En face OCT showed a hyperreflective choroidal lesion with finger-like projections oriented in a stellate configuration. On ICGA, SMACH showed early and late hypofluorescence. None of the cases showed lesion growth. CONCLUSION: SMACH seems to be a unilateral choroidopathy characterized by distinctive multimodal imaging features. As SRF was absent in some cases, while a dendriform pattern was a consistent finding in all eyes, the authors propose renaming this entity "stellate multiform amelanotic choroidopathy," a name that retains its previous abbreviation "SMACH."
KW - SMACH
KW - multimodal imaging
KW - optical coherence tomography
KW - stellate multiform amelanotic choroidopathy
UR - http://www.scopus.com/inward/record.url?scp=85168789410&partnerID=8YFLogxK
U2 - https://doi.org/10.1097/IAE.0000000000003826
DO - https://doi.org/10.1097/IAE.0000000000003826
M3 - Article
C2 - 37127025
SN - 0275-004X
VL - 43
SP - 1448
EP - 1461
JO - Retina (Philadelphia, Pa.)
JF - Retina (Philadelphia, Pa.)
IS - 9
ER -