An Update on Multimodal Ophthalmological Imaging of Diffuse Choroidal Hemangioma in Sturge–Weber Syndrome
Abstract
:1. Introduction
2. Discussion
2.1. Histopathological Findings and Clinical Characteristics of Choroidal Hemangiomas in Sturge-Weber Disease
2.2. Fundus Photography
2.3. Fundus Autofluorescence (FA)
2.4. Fluorescein Angiography (FFA)
2.5. Indocyanine Green Angiography (ICGA)
2.6. Spectral Domain Optical Coherence Tomography (SDOCT)
2.7. Near-Infrared Reflectance (NIR)
2.8. Ultrasound Imaging
2.9. Treatment Strategies for DCHs
3. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Imaging Modality Technical Characteristics | Advantages | Disadvantages | DCH Appearance |
---|---|---|---|
Fundus Photography WL 490–615 nm [78] FOV 45°–200° [78] | Non-invasive; ease of capturing images; both eyes can be assessed simultaneously. | The extent or thickness of the hemangioma is not assessable. Shallow DCH does not significantly alter the fundus appearance. | DCH’s subtle color differences known as “tomato ketchup” appearance. Additional characteristics such as exudative retinal detachments, venous congestion, or hyperplastic RPE changes can be monitored. |
FA WL 488–514 nm [79] FOV 30°–200° [80] | Efficient; non-invasive; useful in evaluating the presence of subretinal fluid or retinal detachment. | The extent or thickness of the hemangioma is not assessable. | Untreated DCH exhibits intrinsic iso-FA or hypo-FA patterns; treated DCH often displays a higher prevalence of hypo-FA traits. Extrinsic DCH findings including RPE hyperplasia, atrophy, and fibrous metaplasia. |
NIR WL 790 nm [79] FOV 30–200° [80] | Efficient; non-invasive. | Further studies for its implication in DCH’s diagnosis and monitoring are necessary. | Greater tissue penetration of NIR allows rapid evaluation of the EPR. A rare case of hyperreflective dots was reported. |
FFA WL 465–530 nm [81] FOV 55°–200° [81] | FFA can be particularly informative in cases where DCH is associated with retinal detachment. | It requires dye administration which rarely can give rise to allergic reactions. It can interfere with renal and hepatic metabolisms. Requires substantial patient collaboration. | FFA has been shown to depict early hyperfluorescence and subsequent late-phase leakage consistent with exudative retinal detachment linked to DCH. |
ICGA WL 790–835 nm [57] FOV 55°–105° [81] | ICGA offers a detailed dynamic visualization of DCH; valuable insights into the intricate choroidal circulation within DCH. | Requires dye administration, which rarely can give rise to allergic reactions. It can interfere with renal and hepatic metabolisms. Requires substantial patient collaboration. | Early phase: vascular network within the hemangioma is quickly filled. Intermediate phase: complete dye filling yields intense hyperfluorescence with a mix of hyper- and hypofluorescent dots on an already luminescent background. Late phase: pronounced hypofluorescence of the tumor when compared to the adjacent choroid emerges as a signature feature. |
SDOCT WL 840–870 nm [56,57,58,59]. | Efficient; non-invasive; allows for the identification of retinal layers and assessment of retinal thickness. | In cases of thickened choroid SDOCT falls short in evaluating the choroidal-scleral junction. | It can reveal the breakdown of Bruch’s membrane and RPE, intraretinal fluid, and subretinal fluid. |
EDI-SDOCT WL 840–870 nm [56,57,58,59]. | Efficient; non-invasive; allows for a better visualization of the choroidal-scleral junction which renders manual choroidal thickness measurement easier. | Not always available in hospital settings. | Choroidal vasculature is visualized on EDI-SDOCT cross sectional images. Profile alterations of the retina and choroid are well visible. |
SSOCT WL 1055–1300 nm [65,66,67]. | Efficient; non-invasive; allows for a better visualization of the choroidal-scleral junction which renders manual choroidal thickness measurement easier. | Expensive technology, rarely available in hospital settings. | Consistent accuracy of SSOCT in measuring hemangioma thickness and effectively distinguishing the lesion from surrounding normal choroid. Profile alterations of the retina and choroid are better evaluated. |
OCTA WL 840–870 nm [56,57,58,59]. | Efficient; non-invasive; it provides insights into lesion characteristics by analyzing density, flow, and vascular patterns. | Further studies for its implication in DCH diagnosis and monitoring are necessary. | Differentiated vascular patterns, like worm-like, spaghetti-shaped, and club-shaped, have been identified within CCH using OCTA. |
US Frequency: A-scan 7–10 MHz B-scan 10–20 MHz [75] | Cost effective, efficient. Helps in distinguishing DCH from malignant choroidal tumors such as melanomas. | It provides two-dimensional images and may not capture the full extent of complex hemangiomas or small lesions. | Benign tumors reveal a thickened choroid with pronounced internal reflectivity. DCH demonstrates a regular internal structure and lacks significant vascularization. DCH appears as a solid, dome-shaped lesion with hyperechogenicity and lacks a posterior shadow. Detects presence of associated features like retinal detachment and superficial calcifications. |
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Ciancimino, C.; Di Pippo, M.; Rullo, D.; Ruggeri, F.; Grassi, F.; Scuderi, G.; Abdolrahimzadeh, S. An Update on Multimodal Ophthalmological Imaging of Diffuse Choroidal Hemangioma in Sturge–Weber Syndrome. Vision 2023, 7, 64. https://doi.org/10.3390/vision7040064
Ciancimino C, Di Pippo M, Rullo D, Ruggeri F, Grassi F, Scuderi G, Abdolrahimzadeh S. An Update on Multimodal Ophthalmological Imaging of Diffuse Choroidal Hemangioma in Sturge–Weber Syndrome. Vision. 2023; 7(4):64. https://doi.org/10.3390/vision7040064
Chicago/Turabian StyleCiancimino, Chiara, Mariachiara Di Pippo, Daria Rullo, Francesco Ruggeri, Flaminia Grassi, Gianluca Scuderi, and Solmaz Abdolrahimzadeh. 2023. "An Update on Multimodal Ophthalmological Imaging of Diffuse Choroidal Hemangioma in Sturge–Weber Syndrome" Vision 7, no. 4: 64. https://doi.org/10.3390/vision7040064