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Observational Study
. 2023 Sep 1;43(9):1448-1461.
doi: 10.1097/IAE.0000000000003826.

STELLATE MULTIFORM AMELANOTIC CHOROIDOPATHY: Clinical and Multimodal Imaging Features

Prithvi Ramtohul  1 , Marco Pellegrini  2 , Francesco Pichi  3 , Chiara Preziosa  2 , Alessandro Marchese  4   5 , Maria Vittoria Cicinelli  5   6 , Elisabetta Miserocchi  5   6 , Rusdeep Mundae  1 , Sarah Mrejen  7 , Soraya Rofagha  8   9 , Calvin E Mein  10 , Luke Mein  10 , Michael D Ober  11 , Eduardo Cunha de Souza  12 , Salomon Yves Cohen  5 , Elon H C van Dijk  13 , Lee Jampol  4 , Camiel J F Boon  13   14 , K Bailey Freund  1   15
Affiliations
Observational Study

STELLATE MULTIFORM AMELANOTIC CHOROIDOPATHY: Clinical and Multimodal Imaging Features

Prithvi Ramtohul et al. Retina. .

Abstract

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."

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References

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