Review

Authors: Luis Fernando Sánchez-Espino, Marta Ivars, Javier Antoñanzas...

Sturge-Weber syndrome (SWS) is a congenital, sporadic, and rare neurocutaneous disorder, characterized by the presence of a facial port-wine birthmark (PWB), glaucoma, and neurological manifestations including leptomeningeal angiomatosis and seizures. It is caused by a postzygotic, somatic, gain-of-function variant of the gene, and more recently, the gene in association with distinctive clinical features. Neuroimaging can help identify and stratify patients at risk for significant complications allowing closer follow-up; although no presymptomatic treatment has been demonstrated to be effective to date, these patients could benefit from early treatment and/or supportive interventions. Choroid plexus (CP) thickness measurements in brain magnetic resonance imaging (MRI) have a high sensitivity and specificity for early and incipient changes in SWS. In contrast, the absence of pathologic findings makes it possible to rule out associated neurological involvement and leads to periodical observation, with new imaging studies only in cases of new clinical signs/symptoms. Periodic ophthalmological examination is also recommended every 3 months during the first year and yearly afterwards to monitor for glaucoma and choroidal hemangiomas. Treatment for SWS depends on the extent and areas that are affected. These include laser surgery for PWB, anticonvulsants in the case of brain involvement, with either seizures or abnormal EEG, and medical treatment or surgery for glaucoma. Sirolimus has been used in a limited number of patients and appears to be a safe and potentially effective treatment for cutaneous and extra-cutaneous features, however controlled clinical studies have not been carried out. Better knowledge of molecular pathways will help to develop future targeted treatments.

PubMed: 37124240
DOI: 10.2147/TACG.S363685

Review

Authors: Saeed Karimi, Ramin Nourinia, Arman Mashayekhi...

Circumscribed choroidal hemangiomas are benign vascular hamartomas without systemic associations. Generally, they are orange-red elevated masses, which are found posterior to the equator. Lesions are usually solitary and unilateral. Overlying subretinal fluid, serous retinal detachment and cystoid macular edema are common findings. Intravenous fluorescein angiography, indocyanine green angiography, ultrasonography, optical coherence tomography and enhanced depth imaging are helpful ancillary tests for diagnosis of circumscribed choroidal hemangiomas. Asymptomatic circumscribed choroidal hemangiomas do not require treatment. For symptomatic lesions with exudative retinal detachment or cystoid macular edema, photodynamic therapy has emerged as the treatment of choice with high rates of tumor regression, subretinal fluid resorption and minimal complications. Lens-sparing external beam radiotherapy, plaque brachytherapy, proton beam therapy, stereotactic radiosurgery, transpupillary thermotherapy, laser photocoagulation and anti-VEGF injections are other treatment modalities.

PubMed: 26730320
DOI: 10.4103/2008-322X.170353

Review

Authors: Mrittika Sen, Santosh G Honavar

Circumscribed choroidal hemangioma is a benign vascular tumor which presents in middle-aged adults with progressive diminution of vision, metamorphopsia, floaters, and visual field defects. Diagnosis is based on the characteristic clinical features. It is an orange-red, usually solitary, tumor situated in the posterior pole. The visual symptoms are because of the associated subretinal fluid, cystoid macular edema, and, in long-standing cases, retinal pigment epithelium changes, subretinal fibrosis and retinoschisis. It must be distinguished from the more ominous amelanotic melanoma and choroidal metastasis. Diagnostic tools such as ultrasound, fundus fluorescein angiography, indocyanine green angiography, and optical coherence tomography are helpful in cases with diagnostic dilemma. Treatment is indicated in symptomatic cases. The management of choroidal hemangioma has evolved over the years beginning with laser photocoagulation to transpupillary thermotherapy, photodynamic therapy, plaque brachytherapy and external beam radiotherapy. No one therapeutic option holds superiority over the other. In this article, we review the epidemiology, clinical manifestations and treatment of the circumscribed variant of choroidal hemangioma.

Topics: Brachytherapy; Choroid Neoplasms; Coloring Agents; Fluorescein Angiography; Hemangioma; Humans; Hyperthermia, Induced; Indocyanine Green; Laser Coagulation; Photochemotherapy; Tomography, Optical Coherence; Ultrasonography

PubMed: 31755430
DOI: 10.4103/ijo.IJO_2036_19

Authors: Youssef Abdelmassih, Georges Azar, Sophie Bonnin...

The aim of the study is to report on the indocyanine green angiography (ICGA) and OCT findings in patients hospitalized for severe COVID infection. In this observational prospective monocentric cohort study, we included patients hospitalized for severe COVID infection. The main outcomes were ICGA and OCT findings. A total of 14 patients with a mean age of 58.2 ± 11.4 years and a male predominance (9/14 patients; 64%) were included. The main ICGA findings included hypofluorescent spots in 19 eyes (68%), intervortex shunts in 10 eyes (36%), and characteristic "hemangioma-like" lesions in five eyes (18%). "Hemangioma-like" lesions were both unique and unilateral, and showed no washout on the late phase of the angiogram. The main OCT findings included focal choroidal thickening in seven eyes (25%), caverns in six eyes (21%) and paracentral acute middle maculopathy lesions in one eye (4%). All patients hospitalized for severe COVID infection had anomalies on ICGA and OCT. Lesions to both retinal and choroidal vasculature were found. These anomalies could be secondary to vascular involvement related directly or indirectly to the SARS-CoV2 virus.

PubMed: 34682810
DOI: 10.3390/jcm10204686

Review

Authors: A D Singh

Phototherapy can be translated to mean 'light or radiant energy-induced treatment.' Lasers have become the exclusive source of light or radiant energy for all applications of phototherapy. Depending on the wavelength, intensity, and duration of exposure, tissues can either absorb the energy (photocoagulation, thermotherapy, and photodynamic therapy (PDT)) or undergo ionization (photodisruption). For phototherapy to be effective, the energy has to be absorbed by tissues or more specifically by naturally occurring pigment (xanthophyll, haemoglobin, and melanin) within them. In tissues or tumours that lack natural pigment, dyes (verteporphin, Visudyne) with narrow absorption spectrum can be injected intravenously that act as focal absorbent of laser energy after they have preferentially localized within the tumour. Ocular phototherapy has broad applications in treatment of ocular tumours. Laser photocoagulation, thermotherapy, and PDT can be delivered with low rates of complications and with ease in the outpatient setting. Review of the current literature suggests excellent results when these treatments are applied for benign tumours, particularly for vascular tumours such as circumscribed choroidal haemangioma. For primary malignant tumours, such as choroidal melanoma, thermotherapy, and PDT do not offer local tumour control rates that are equivalent or higher than those achieved with plaque or proton radiation therapy. However, for secondary malignant tumours (choroidal metastases), thermotherapy and PDT can be applied as a palliative treatment. Greater experience is necessary to fully comprehend risks, comparative benefits, and complication of ocular phototherapy of ocular tumours.

Topics: Eye Neoplasms; Humans; Laser Therapy; Lasers; Light Coagulation; Photochemotherapy; Phototherapy

PubMed: 23238445
DOI: 10.1038/eye.2012.258

Review

Authors: Federico Di Staso, Mariachiara Di Pippo, Solmaz Abdolrahimzadeh...

Choroidal neovascularizations are historically associated with exudative macular degeneration, nonetheless, they have been observed in nevus, melanoma, osteoma, and hemangioma involving the choroid and retina. This review aimed to elucidate the possible origins of neovascular membranes by examining in vivo and in vitro models compared to real clinical cases. Among the several potential mechanisms examined, particular attention was paid to histologic alterations and molecular cascades. Physical or biochemical resistance to vascular invasion from the choroid offered by Bruch's membrane, the role of fibroblast growth factor 2 and vascular endothelial growth factor, resident or recruited stem-like/progenitor cells, and other angiogenic promoters were taken into account. Even if the exact mechanisms are still partially obscure, experimental models are progressively enhancing our understanding of neovascularization etiology. Choroidal neovascularization (CNV) over melanoma, osteoma, and other tumors is not rare and is not contraindicative of malignancy as previously believed. In addition, CNV may represent a late complication of either benign or malignant choroidal tumors, stressing the importance of a long follow-up.

Topics: Humans; Choroid Neoplasms; Vascular Endothelial Growth Factor A; Macular Degeneration; Choroidal Neovascularization; Retina; Choroid; Retinal Neovascularization; Melanoma; Osteoma

PubMed: 36674579
DOI: 10.3390/ijms24021064

Authors: Paul T Finger

Topics: Choroid Neoplasms; Hemangioma; Humans

PubMed: 31007211
DOI: 10.4103/ijo.IJO_707_19

Review

Authors: Waleed M Alshehri, Badr O AlAhmadi, Fatima Alhumaid...

Circumscribed choroidal hemangioma (CCH) is a sort of non-malignant hamartomatous tumor that occurs in the choroidal layer of the eye. It is a rare condition that affects people between their second and fourth decades of life, leading to significant deterioration of vision. One of the most catastrophic consequences of CCH is exudative retinal detachment (ERD), which has a severe impact on vision. This review aims to comprehensively assess the safety and efficacy of photodynamic therapy (PDT) using verteporfin as a therapeutic approach. Using the eligibility criteria, we analyzed the findings of 18 published articles from PubMed, Web of Science, Scopus, and Cochrane. The standard PDT protocol was used in all included studies, except two (one used half-dose, the other one used the double-dose) with an average of 1-2 sessions. PDT induced substantial tumor regression, with a mean thickness range from 0 to 2.3 mm. However, this contrasted with a previous study that reported a thickness of 3.46 mm as an indication of PDT failure. The mean tumor diameter varied from 4.8 mm to total tumor flattening. A suboptimal effect with a mean diameter ranging from 6mm to 8mm was found in two clinical studies. Significant improvement in vision was observed during the last follow-up, ranging from a normalization of Best Corrected Visual Acuity (BCVA) 20/20 to 20/80; counting finger vision persisted in two patients even after treatment. PDT successfully achieved complete subretinal fluid (SRF) resolution in 14 studies and resolved ERD in nine articles. Most studies did not report serious adverse events, but some reported macular atrophy, microcystic degeneration of the retina, transient visual disturbances, Retinal pigmented epithelium (RPE) metaplasia, and cystic degeneration of the retina. This systemic review demonstrated PDT's effectiveness and safety as a first-line management modality for CCH. Photodynamic therapy efficiently induced tumor regression, resulting in a notable reduction in both tumor diameter and thickness, with optimal efficacy to improve vision and resolution of the consequences of CCH, such as SRF and ERD.

PubMed: 38222120
DOI: 10.7759/cureus.50461

Review

Authors: C Camarillo Gómez, I Sánchez Ronco, Jl Encinas...

Uveal metastases are the most frequent malign intraocular tumour, of which more than 80% are localized in the choroids. This, together with the progressive increase in its incidence, makes its study and review necessary for a correct diagnosis and treatment in current clinical practice. Etiology varies according to the sex of the patient: lung carcinoma metastasises most frequently in men and breast carcinoma in women. These tend to multifocality and are generally localized in the posterior pole. Fifty percent of cases follow an asymptomatic development, but they can cause loss of vision, scotomas, metamorphopsias and photopsias. Charactersitic ophthamoscopic examination shows a placoid, homogenous choroidal lesion with a creamy appearance. The differential diagnosis must consider the amelanotic nevus, choroidal amelanotic melanoma, choroidal haemangioma, rear scleritis, choroidal osteoma, chorioretinitis, Harada's disease, rhegmatogenous retina detachment, uveal effusion syndrome, and serous central chorioretinopathy. An exhaustive history and complete ophthalmological examination are essential to the diagnosis, to which fluorescein angiography, ocular echography, fine needle puncture aspiration (FNPA), computerized tomography and magnetic resonance can be added as complementary tests. Treatment of these tumours is usually the systemic treatment of the primary tumour; the possibilities of local treatment are observation, external radiotherapy, transpupillary thermotherapy and enucleation.

Topics: Choroid Neoplasms; Humans

PubMed: 19169300
DOI: No ID Found

Review

Authors: Zuyi Yang, Dianzhe Tian, Zhixuan Xie...

Because of its benign nature and rarity, circumscribed choroidal hemangioma (CCH) often receives limited attention, leading to a high rate of misdiagnosis and a lack of standardized treatment protocols. We provide a thorough clarification of the demographics, clinical features, diagnosis, management, and prognosis of CCH. We conducted a systematic search of the PubMed, EMBASE, and Ovid databases up to December, 2023, to identify relevant studies. The study included 106 studies encompassing 3854 patients with CCH. The demographic profile revealed a male preponderance (62 %, 95 % CI 61-64 %) and a peak incidence in the working-age population (30-50 years, 48 %, 95 % CI 39-57 %), with the right eye being involved in 50 % of cases (95 % CI 48-53 %). Clinically, the most common symptom was decreased vision (90 %, 95 % CI 78-99 %), followed by blurred vision, visual field defect, and metamorphopsia. Fundus examination frequently revealed an orange-colored tumor (80 %, 95 % CI 58-96 %) located subfoveally (48 %, 95 % CI 42-53 %), often accompanied by subretinal fluid (SRF) (84 %, 95 % CI 78-89 %) and sometimes exudative retinal detachment (69 %, 95 % CI 51-85 %). The proportion of correctly diagnosed CCH upon first presentation was 13 % (95 % CI 3-26 %), with CCH commonly misdiagnosed as unspecified choroidal tumors, choroidal metastasis, and central serous chorioretinopathy. Photodynamic therapy (PDT) was the most widely researched treatment, taking up 18 % (95 % CI 8-31 %), followed by observation, transpupillary thermotherapy (TTT), and laser photocoagulation. When no treatment was applied, 33 % of patients experienced visual acuity improvement, which increased to 76 % (95 % CI 58-90 %) with radiotherapy and 58 % (95 % CI 50-67 %) with PDT. In terms of tumor shrinkage, radiotherapy was most effective (100 %), with PDT close behind (95 % CI 96-100 %), and TTT at 63 % (95 % CI 45-80 %). PDT led to SRF resolution in 89 % (95 % CI 77-97 %) of patients and cystoid macular edema (CME) resolution in 73 % (95 % CI 38-97 %), while radiotherapy achieved the highest efficacy with 100 % SRF resolution (95 % CI: 99-100 %) and 100 % CME resolution (95 % CI: 83-100 %). Complication rates were highest with radiotherapy (14 %, 95 % CI 5-25 %) and PDT (9 %, 95 % CI 4-16 %). First-line treatment failure rates were highest for TTT (71 %, 95 % CI 44-92 %) and laser (70 %, 95 % CI 28-99 %), with radiotherapy showing the lowest rate (0 %, 95 % CI 0-2 %). Recurrence rates were highest for laser (68 %, 95 % CI 17-100 %) and TTT (62 %, 95 % CI 26-93 %), whereas radiotherapy had the lowest recurrence rate (0 %, 95 % CI 0-1 %). CCH predominantly affects the working-age male population, often leading to vision impairment and SRF. The diagnosis of CCH remains challenging, with low accuracy and frequent misdiagnoses. While PDT is the most widely researched treatment, radiotherapy offers superior outcomes in visual acuity, tumor shrinkage, and resolution of SRF and CME, though it carries higher complication rates. This study highlights the need for improved diagnostic accuracy and a balanced approach to treatment.

Topics: Humans; Choroid Neoplasms; Hemangioma; Prognosis; Visual Acuity; Fluorescein Angiography; Tomography, Optical Coherence; Photochemotherapy; Disease Management; Choroid

PubMed: 39862992
DOI: 10.1016/j.survophthal.2025.01.001