An Update on Multimodal Ophthalmological Imaging of Diffuse Choroidal Hemangioma in Sturge–Weber Syndrome

Sturge–Weber syndrome (SWS) is characterized by facial port-wine stains, leptomeningeal hemangiomas, and prominent ocular manifestations such as glaucoma and diffuse choroidal hemangiomas (DCHs). Imaging modalities are critical for diagnosing and longitudinally monitoring DCHs in SWS. Fundus photogr...

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Main Authors: Chiara Ciancimino, Mariachiara Di Pippo, Daria Rullo, Francesco Ruggeri, Flaminia Grassi, Gianluca Scuderi, Solmaz Abdolrahimzadeh
Format: Article
Language:English
Published: MDPI AG 2023-10-01
Series:Vision
Subjects:
Online Access:https://www.mdpi.com/2411-5150/7/4/64
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author Chiara Ciancimino
Mariachiara Di Pippo
Daria Rullo
Francesco Ruggeri
Flaminia Grassi
Gianluca Scuderi
Solmaz Abdolrahimzadeh
author_facet Chiara Ciancimino
Mariachiara Di Pippo
Daria Rullo
Francesco Ruggeri
Flaminia Grassi
Gianluca Scuderi
Solmaz Abdolrahimzadeh
author_sort Chiara Ciancimino
collection DOAJ
description Sturge–Weber syndrome (SWS) is characterized by facial port-wine stains, leptomeningeal hemangiomas, and prominent ocular manifestations such as glaucoma and diffuse choroidal hemangiomas (DCHs). Imaging modalities are critical for diagnosing and longitudinally monitoring DCHs in SWS. Fundus photography is fundamental in assessing both eyes simultaneously, fluorescein angiography and indocyanine green angiography effectively map the retinal and choroidal circulation, and ultrasonography offers essential structural insights into the choroid and retina. NIR imaging reveals subtle retinal pigment changes, often overlooked in standard fundus examination. Enhanced depth imaging spectral domain optical coherence tomography (EDI-SDOCT) and swept-source OCT (SSOCT) improve the visualization of the choroidal-scleral boundary, essential for DCH characterization. The potential of OCT angiography (OCTA) is under exploration, particularly its role in predicting signs of disease progression or worsening, as well as potential new biomarkers such as the choroidal vascularity index (CVI). The present review aims to provide an update on multimodal imaging of DCHs in SWS.
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spelling doaj.art-ce6e4468521445fcadbfb04b3a8207362023-12-22T14:49:32ZengMDPI AGVision2411-51502023-10-01746410.3390/vision7040064An Update on Multimodal Ophthalmological Imaging of Diffuse Choroidal Hemangioma in Sturge–Weber SyndromeChiara Ciancimino0Mariachiara Di Pippo1Daria Rullo2Francesco Ruggeri3Flaminia Grassi4Gianluca Scuderi5Solmaz Abdolrahimzadeh6Ophthalmology Unit, Neurosciences, Mental Health, and Sense Organs (@NESMOS) Department, Faculty of Medicine and Psychology, University of Rome Sapienza, St. Andrea Hospital, 00189 Rome, ItalyOphthalmology Unit, Neurosciences, Mental Health, and Sense Organs (@NESMOS) Department, Faculty of Medicine and Psychology, University of Rome Sapienza, St. Andrea Hospital, 00189 Rome, ItalyOphthalmology Unit, Neurosciences, Mental Health, and Sense Organs (@NESMOS) Department, Faculty of Medicine and Psychology, University of Rome Sapienza, St. Andrea Hospital, 00189 Rome, ItalyOphthalmology Unit, Neurosciences, Mental Health, and Sense Organs (@NESMOS) Department, Faculty of Medicine and Psychology, University of Rome Sapienza, St. Andrea Hospital, 00189 Rome, ItalyOphthalmology Unit, Neurosciences, Mental Health, and Sense Organs (@NESMOS) Department, Faculty of Medicine and Psychology, University of Rome Sapienza, St. Andrea Hospital, 00189 Rome, ItalyOphthalmology Unit, Neurosciences, Mental Health, and Sense Organs (@NESMOS) Department, Faculty of Medicine and Psychology, University of Rome Sapienza, St. Andrea Hospital, 00189 Rome, ItalyOphthalmology Unit, Neurosciences, Mental Health, and Sense Organs (@NESMOS) Department, Faculty of Medicine and Psychology, University of Rome Sapienza, St. Andrea Hospital, 00189 Rome, ItalySturge–Weber syndrome (SWS) is characterized by facial port-wine stains, leptomeningeal hemangiomas, and prominent ocular manifestations such as glaucoma and diffuse choroidal hemangiomas (DCHs). Imaging modalities are critical for diagnosing and longitudinally monitoring DCHs in SWS. Fundus photography is fundamental in assessing both eyes simultaneously, fluorescein angiography and indocyanine green angiography effectively map the retinal and choroidal circulation, and ultrasonography offers essential structural insights into the choroid and retina. NIR imaging reveals subtle retinal pigment changes, often overlooked in standard fundus examination. Enhanced depth imaging spectral domain optical coherence tomography (EDI-SDOCT) and swept-source OCT (SSOCT) improve the visualization of the choroidal-scleral boundary, essential for DCH characterization. The potential of OCT angiography (OCTA) is under exploration, particularly its role in predicting signs of disease progression or worsening, as well as potential new biomarkers such as the choroidal vascularity index (CVI). The present review aims to provide an update on multimodal imaging of DCHs in SWS.https://www.mdpi.com/2411-5150/7/4/64diffuse choroidal hemangiomaSturge–Weber syndromemultimodal imagingfundusenhanced depth imagingspectral domain optical coherence tomography (OCT)
spellingShingle Chiara Ciancimino
Mariachiara Di Pippo
Daria Rullo
Francesco Ruggeri
Flaminia Grassi
Gianluca Scuderi
Solmaz Abdolrahimzadeh
An Update on Multimodal Ophthalmological Imaging of Diffuse Choroidal Hemangioma in Sturge–Weber Syndrome
Vision
diffuse choroidal hemangioma
Sturge–Weber syndrome
multimodal imaging
fundus
enhanced depth imaging
spectral domain optical coherence tomography (OCT)
title An Update on Multimodal Ophthalmological Imaging of Diffuse Choroidal Hemangioma in Sturge–Weber Syndrome
title_full An Update on Multimodal Ophthalmological Imaging of Diffuse Choroidal Hemangioma in Sturge–Weber Syndrome
title_fullStr An Update on Multimodal Ophthalmological Imaging of Diffuse Choroidal Hemangioma in Sturge–Weber Syndrome
title_full_unstemmed An Update on Multimodal Ophthalmological Imaging of Diffuse Choroidal Hemangioma in Sturge–Weber Syndrome
title_short An Update on Multimodal Ophthalmological Imaging of Diffuse Choroidal Hemangioma in Sturge–Weber Syndrome
title_sort update on multimodal ophthalmological imaging of diffuse choroidal hemangioma in sturge weber syndrome
topic diffuse choroidal hemangioma
Sturge–Weber syndrome
multimodal imaging
fundus
enhanced depth imaging
spectral domain optical coherence tomography (OCT)
url https://www.mdpi.com/2411-5150/7/4/64
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