STELLATE MULTIFORM AMELANOTIC CHOROIDOPATHY
Retina, vol. 43, pp. 1448–1461
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."
Authors 19
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Prithvi Ramtohul corresponding
Vitreous Retina Macula Consultants of New York
Affiliation as printed
Vitreous Retina Macula Consultants of New York, New York, New York;
Vitreous Retina Macula Consultants of New York, New York, New York, USA
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University of Milan · Luigi Sacco Hospital
Affiliation as printed
Department of Biomedical and Clinical Science “Luigi Sacco,” Eye Clinic, Luigi Sacco Hospital, University of Milan, Milan, Italy;
Department of Biomedical and Clinical Science "Luigi Sacco," Eye Clinic, Luigi Sacco Hospital, University of Milan, Milan, Italy
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Cleveland Clinic Lerner College of Medicine · Cleveland Clinic Abu Dhabi · Case Western Reserve University
Affiliation as printed
Eye Institute, Cleveland Clinic Abu Dhabi, Al Maryah Island, Abu Dhabi, United Arab Emirates. Cleveland Clinic Lerner College of Medicine, Case Western Reserve University, Cleveland, Ohio;
Eye Institute, Cleveland Clinic Abu Dhabi, Al Maryah Island, Abu Dhabi, United Arab Emirates. Cleveland Clinic Lerner College of Medicine, Case Western Reserve University, Cleveland, OH, USA
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University of Milan · Luigi Sacco Hospital
Affiliation as printed
Department of Biomedical and Clinical Science “Luigi Sacco,” Eye Clinic, Luigi Sacco Hospital, University of Milan, Milan, Italy;
Department of Biomedical and Clinical Science "Luigi Sacco," Eye Clinic, Luigi Sacco Hospital, University of Milan, Milan, Italy
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Northwestern University · IRCCS Ospedale San Raffaele · Istituto di Ricovero e Cura a Carattere Scientifico San Raffaele
Affiliation as printed
Department of Ophthalmology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois;
Department of Ophthalmology, IRCCS San Raffaele Scientific Institute, Milan, Italy;
Department of Ophthalmology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois
Department of Ophthalmology, IRCCS San Raffaele Scientific Institute, Milan, Italy
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Maria Vittoria Cicinelli corresponding
Vita-Salute San Raffaele University · IRCCS Ospedale San Raffaele · Istituto di Ricovero e Cura a Carattere Scientifico San Raffaele
Affiliation as printed
Department of Ophthalmology, IRCCS San Raffaele Scientific Institute, Milan, Italy;
School of Medicine, Vita-Salute San Raffaele University, Milan, Italy;
Department of Ophthalmology, IRCCS San Raffaele Scientific Institute, Milan, Italy
School of Medicine, Vita-Salute San Raffaele University, Milan, Italy
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Elisabetta Miserocchi corresponding
Vita-Salute San Raffaele University · IRCCS Ospedale San Raffaele · Istituto di Ricovero e Cura a Carattere Scientifico San Raffaele
Affiliation as printed
Department of Ophthalmology, IRCCS San Raffaele Scientific Institute, Milan, Italy;
School of Medicine, Vita-Salute San Raffaele University, Milan, Italy;
Department of Ophthalmology, IRCCS San Raffaele Scientific Institute, Milan, Italy
School of Medicine, Vita-Salute San Raffaele University, Milan, Italy
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Rusdeep S. Mundae corresponding
Vitreous Retina Macula Consultants of New York
Affiliation as printed
Vitreous Retina Macula Consultants of New York, New York, New York;
Vitreous Retina Macula Consultants of New York, New York, New York, USA
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Centre Ophtalmologique d'Imagerie et de Laser
Affiliation as printed
Ophthalmic Center for Imaging and Laser, Paris, France;
Ophthalmic Center for Imaging and Laser, Paris, France
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University of California, San Francisco
Affiliation as printed
Department of Ophthalmology, University of California, San Francisco, San Francisco, California;
East Bay Retina Consultants, Inc, Oakland, California;
Department of Ophthalmology, University of California, San Francisco, San Francisco, California
East Bay Retina Consultants, Inc, Oakland, California; Department of Ophthalmology, University of California, San Francisco, San Francisco, California
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Affiliation as printed
Retinal Consultants of San Antonio, San Antonio, Texas;
Retinal Consultants of San Antonio, San Antonio, Texas
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Affiliation as printed
Retinal Consultants of San Antonio, San Antonio, Texas;
Retinal Consultants of San Antonio, San Antonio, Texas
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Michael D. Ober corresponding
Affiliation as printed
Department of Ophthalmology, Henry Ford Health Systems, Detroit, Michigan;
Department of Ophthalmology, Henry Ford Health Systems, Detroit, Michigan
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Affiliation as printed
Department of Ophthalmology, University of São Paulo (USP), São Paulo, Brazil;
Department of Ophthalmology, University of São Paulo (USP), SP, Brazil
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IRCCS Ospedale San Raffaele · Istituto di Ricovero e Cura a Carattere Scientifico San Raffaele
Affiliation as printed
Department of Ophthalmology, IRCCS San Raffaele Scientific Institute, Milan, Italy;
Department of Ophthalmology, IRCCS San Raffaele Scientific Institute, Milan, Italy
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Leiden University Medical Center
Affiliation as printed
Department of Ophthalmology, Leiden University Medical Center, Leiden, the Netherlands;
Department of Ophthalmology, Leiden University Medical Center, Leiden, the Netherlands
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Affiliation as printed
Department of Ophthalmology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois;
Department of Ophthalmology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois
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Leiden University Medical Center · Amsterdam University Medical Centers
Affiliation as printed
Department of Ophthalmology, Amsterdam University Medical Centers, Amsterdam, the Netherlands; and
Department of Ophthalmology, Leiden University Medical Center, Leiden, the Netherlands;
Department of Ophthalmology, Amsterdam University Medical Centers, Amsterdam, the Netherlands
Department of Ophthalmology, Leiden University Medical Center, Leiden, the Netherlands
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K. Bailey Freund corresponding
Vitreous Retina Macula Consultants of New York · New York University
Affiliation as printed
Department of Ophthalmology, NYU Grossman School of Medicine, New York, New York
Vitreous Retina Macula Consultants of New York, New York, New York;
Department of Ophthalmology, NYU Grossman School of Medicine, New York, New York, USA
Vitreous Retina Macula Consultants of New York, New York, New York, USA
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