Title

Stellate Multiform Amelanotic Choroidopathy (SMACH). Clinical and Multimodal Imaging Features

Authors

Prithvi Ramtohul
Marco Pellegrini
Francesco Pichi
Chiara Preziosa
Alessandro Marchese
Maria Vittoria Cicinelli
Elisabetta Miserocchi
Rusdeep Mundae
Sarah Mrejen
Soraya Rofagha
Calvin E. Mein
Luke Mein
Michael D. Ober, Henry Ford Health
Eduardo Cunha de Souza
Salomon Yves Cohen
Elon H. C van Dijk
Lee Jampol
Camiel J. F Boon
K. Bailey Freund

Recommended Citation

Ramtohul P, Pellegrini M, Pichi F, Preziosa C, Marchese A, Cicinelli MV, Miserocchi E, Mundae R, Mrejen S, Rofagha S, Mein CE, Mein L, Ober MD, Cunha de Souza E, Cohen SY, van Dijk EHC, Jampol L, Boon CJF, and Freund KB. Stellate Multiform Amelanotic Choroidopathy (SMACH). Clinical and Multimodal Imaging Features. Retina 2023.

Document Type

Article

Publication Date

5-1-2023

Publication Title

Retina (Philadelphia, Pa.)

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 10/18 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.

CONCLUSIONS: SMACH appears 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, we propose renaming this entity "stellate multiform amelanotic choroidopathy", a name which retains its prior abbreviation "SMACH".

PubMed ID

37127025

ePublication

ePub ahead of print