Retinal Capillary Hemangioma Research Articles

Vitreoretinal Surgery for Retinal Capillary Hemangiomas With Retinal Detachment.

Vitreoretinal Surgery for Retinal Capillary Hemangiomas With Retinal Detachment.

Tumors of the Retina, Optic Disc and Retinal Pigment Epithelium

Vascular tumors of the retina include retinal capillary hemangioma (retinal hemangioblastoma), retinal cavernous hemangioma, retinal racemose hemangioma, and vasoproliferative tumor of the retina. Although they are benign and rare tumors, vascular tumors of the retina are important due to the potential for vision loss through ocular complications, as well as the possibility of morbidity and mortality from accompanying systemic diseases. Ophthalmologists have an important role in diagnosing and implementing necessary screening and treatments. This article will describe the vascular tumors of the retina and optic nerve head.

Multimodal Imaging and Functional Assessment in a Young Patient Presenting With Concomitant Unilateral Retinal Capillary Hemangioma and Presumed Retinal Pigment Epithelium Dysgenesis.

Romano, Francesco MD FEBO1; Preziosa, Chiara MD FEBO1; Staurenghi, Giovanni MD FARVO1; Pellegrini, Marco MD1 Author Information

Combined Rhegmatogenous and Tractional Retinal Detachment in Solitary Retinal Capillary Hemangioma

Combined Rhegmatogenous and Tractional Retinal Detachment in Solitary Retinal Capillary Hemangioma

Hamartomas of the Retina and Optic Disc.

Hamartomas are local malformation of cells that demonstrate abnormal proliferation in the area where they are normally present. Retinal and optic disc hamartomas include astrocytic hamartoma, congenital hypertrophy of the retinal pigment epithelium (CHRPE), simple congenital hamartoma of the retinal pigment epithelium (CSHRPE), combined hamartoma of the retina and retinal pigment epithelium (CHRRPE), retinal hemangioblastoma (retinal capillary hemangioma), and retinal cavernous hemangioma. Retinal and optic disc hamartomas can be observed sporadically as well as with systemic associations. Astrocytic hamartoma usually appears as a flat, transparent yellowish lesion. CHRPE is a round, pigmented, and flat lesion. CSHRPE usually presents as a dark black macular tumor. CHRRPE consists of vascular, glial, and pigment epithelial components, which can demonstrate peripapillary, macular, and peripheral localization. Retinal hemangioblastoma is a vascular tumor, red-pink in color with tortuous and dilated afferent and efferent vessels, typically located in the peripheral retina or optic disc. Retinal cavernous hemangioma is characterized by the formation of thin-walled saccular angiomatous structures in the retina or optic nerve head resembling concord grapes. Ultrasonography, fundus autofluorescence, optical coherence tomography, optical coherence tomography angiography, and fluorescein angiography methods are used in the diagnosis of retinal and optic disc hamartomas. Some retinal and optic disc hamartomas do not require treatment. However, complications including vitreous hemorrhage, macular exudation, retinal detachment, macular hole, epiretinal membrane, and choroidal neovascularization require treatment.

RetinoblastomainAdults: Clinical Features, Gene Mutations and Treatment Outcomes: A Study of Six Cases.

PurposeTo report six Asian adult patients with retinoblastoma (RB).DesignRetrospective and observational small case series.ParticipantsSix patients with a white dome-shaped tumor of the retina were evaluated from May 10, 1995, to September 10, 2021.Main Outcome MeasuresInitial tumor and associated fundus features, pathology, gene mutation, treatment, tumor course on follow-up, and salvage globe outcome.ResultsThe six affected Asian patients consisted of three men and three women. The mean age at the time of diagnosis was 36.5 years (median: 31 years, range: 20-55 years). All patients were unilateral. In all cases, the tumors were white, dome-shaped, with full-thickness retinal involvement, and mushroom-like protrusions into the vitreous cavity. The mean tumor thickness measured by ultrasonography was 4.5 mm (median: 3.2 mm, range: 3.2-6.8 mm). Associated characteristic symptoms included dilated retinal feeding artery and draining vein (100%), surrounding subretinal infiltration (83%), exudative retinal detachment (83%), and vitreous seeds (67%). Local tumor resection was performed in three patients, I-125 plaque brachytherapy combined with transpupillary thermotherapy (TTT) and intravitreous injection of melphalan (combination treatment) in one patient, I-125 plaque brachytherapy in two patients, and enucleation in one (20%) patient. RB1 gene testing was carried out on four patients and pathological diagnosis on five patients. Genetic analysis revealed that the RB1 mutation was a mosaic c.709dupG (p.Glu237GlyfsTer4) duplication in one patient, a mosaic c.763C>T(p.Arg255Ter) mutation in another patient, while the remaining two patients were RB1 negative. At the end of the follow-up, none of the patients had developed tumor-related metastasis or died. The findings were consistent in all patients who had an adequate follow-up. This study focused on this rare lesion to distinguish it from other intraocular white lesions in adults, including choroidal osteoma, vitreoretinal lymphoma, and retinal capillary hemangioma, all of which are different clinical entities.ConclusionIn adults, RB is typically a white, full-thickness retinal mass that is unilateral, often combining with retinal feeding vessels, subretinal infiltration, and vitreous seeds. Genetic studies on adult-onset RB are essential and still require elucidation. Despite RB being a malignant tumor, patient survival was minimally affected.

A Case Report of Intraocular Radiotherapy with Strontium-90 for Juxtapapillary Retinal Capillary Haemangioma

Juxtapapillary retinal capillary haemangiomas are rare benign vascular tumors located adjacent to the optic disc. They may occur sporadically or in association with von Hippel-Lindau disease. Treatment modalities for juxtapapillary retinal capillary haemangiomas vary from observation to aggressive vitreoretinal surgery. A combination of different treatment modalities is often tried with modest success. We report a patient diagnosed with right-sided juxtapapillary retinal capillary haemangioma at the age of 36 in an amblyopic eye previously treated with patching and surgery. In 2013, at the age of 45 he presented with increasing shadow in his right visual field. The ophthalmological examination revealed a right-sided subtotal serous retinal detachment with 6.2×5.6×3.5 mm juxtapapilllary retinal capillary haemangioma and visual acuity of hand movements. He underwent vitreoretinal surgery, radiation of juxtapapillary retinal capillary haemangioma with intraocular strontium applicator and insertion of silicone oil, all in one session. We describe a novel treatment, the intraocular radiation with strontium. This can be applied in complicated cases where vitrectomy is necessary.

Tractional retinal detachment and juxtapapillary retinal capillary hemangioma in a 6-year-old girl: A case report

A 6-year-old girl with visual impairment in the right eye (OD) was referred for an eye evaluation. The fundus of the OD showed a fibrotic orange endophytic lesion located adjacent to the optic disc. In retinal optical coherence tomography, a local tractional retinal detachment and choroidal neovascular membrane were observed together also with the presence of subretinal fluid. Due to the vision of the OD evolved to nonlight perception in the following exam, enucleation was performed. The pathology report was correlated with hemangioblastoma. Herein, we describe a case of a young girl with a retinal hemangioblastoma with quick evolution and without prior systemic diagnosis.

Ophthalmological Aspects of von-Hippel–Lindau Syndrome

ABSTRACT Background: von Hippel–Lindau (VHL) syndrome is a multisystem neoplastic disorder involving eyes, central nervous system, kidneys, spine, and other tissues. A retinal capillary hemangioma (RCH) is the earliest manifestation of the VHL disease in most cases. Areas covered:This paper aims to provide an up-to-date review of the current literature about von Hippel–Lindau syndrome. Molecular background, systemic and ocular features of the diseases as well as the utility of newer imaging modalities in diagnosis and monitoring of ocular VHL disease have been described. Besides, we have discussed newer treatment modalities and therapeutic targets. Conclusion: Modern imaging technologies like optical coherence tomography and optical coherence tomography angiography are tools of the trade, in making an appropriate diagnosis and monitoring disease activity and response to treatment. Peripheral RCH may be treated using laser photocoagulation in tumors up to 3000 µm. Vascular endothelial growth factor suppression can help in reducing tumor activity and stabilize the tumor size; however, it does not regress the RCH.

Case series of retinal capillary hemangioma in patients with and without von hippel lindau disease

Case series of retinal capillary hemangioma in patients with and without von hippel lindau disease

Regression of a juxtapapillary retinal capillary hemangioma after verteporfin photodynamic therapy

Regression of a juxtapapillary retinal capillary hemangioma after verteporfin photodynamic therapy

Phthisis bulbi and retinal capillary haemangioma associated with papilloedema due to recurrent bilateral cerebellar haemangioblastomas – A case of Von Hippel Lindau syndrome

Von Hippel Lindau (VHL) syndrome is an autosomal dominant condition caused by mutation of VHL gene. It is characterized by vascular tumors of eye, central nervous system, visceral cysts and tumors. Here we report a case of VHL in a young male patient who presented with phthisis bulbi in the right eye and retinal capillary haemangioma in the left eye. Systemic features of raised intracranial pressure was present along with bilateral abduction deficit and florid disc edema in the left eye. There was a polypoidal reddish mass in the inferotemporal quadrant, with a dilated draining vein, indicating retinal capillary hemangioma. The phthisical right eye was speculated to result from untreated retinal capillary hemangioma, culminating in exudative retinal detachment. Urinary Vanillyl Mandelic Acid suggested pheochromocytoma. Imaging modalities revealed multiple cerebellar hemangioblastomas, pheochromocytoma and pancreatic cysts. Screening of family members revealed no evidence of systemic manifestations, suggesting the possibility of sporadic mutation. Retinal capillary hemangioma is the commonest and earliest manifestation of VHL. The ocular complications include intraretinal exudation and exudative retinal detachment, secondary angiomatosis, epiretinal membrane, vitreous hemorrhage, retinal neovascularization, tractional retinal detachment and macular ectopia. Our case report emphasizes that retinal examination in patients with suspected VHL is not only a screening tool for the presence of capillary hemangioma but also for regular monitoring and prevention of vision threatening complications. Keywords: Von Hippel Lindau (VHL) syndrome, Retinal capillary hemangioma, Cerebellar hemangioblastomas, Pheochromocytoma.

Proven and less studied hematopoietic and vasoactive growth factors in retinal capillary hemangioma

Pathogenesis of retinal capillary hemangioma has not been sufficiently studied at the present time. Therefore, the study of cytokine levels in biological fluids seems to be very relevant in order to increase knowledge about the mechanisms of the disease development and searching for targeted therapies. The content of hematopoietic and vasoactive growth factors in blood serum, lacrimal fluid, and vitreous body was studied in patients with retinal capillary hemangioma. A total of 26 patients with retinal angiomatosis were examined. The samples of blood serum (n = 23) and lacrimal fluid (n = 10) from practically healthy people aged 22 to 46 (27.4±1.4 years) were used as a control. To perform comparative assessment of cytokine concentrations in the vitreous body of patients with retinal capillary hemangioma, were used samples of the vitreous body from 6 patients (average age 33±4.7 years; from 21 to 49 years) with rhegmatogenous retinal detachment. To measure the cytokine concentrations, we applied multiplex analysis technique using the xMAP platform with LuminexxPONENT 3.1 program and ProcartaPlex sets (eBioscience, Austria). A detailed characteristic of vasoactive factors in capillary retinal hemangioma was obtained as a result of this work. Some disorders in chemokine regulation were identified. There was a significant increase in serum concentrations of three vasoactive factors, i.e., PDGF-BB, HGF, and PIGF-1, with a decrease in chemokines (MCP-1, MIP-1α, and MIP-1β). The frequencies of PIGF-1 and MIP-1α detection also significantly differed from the control group. SCF was significantly more often determined in patients with retinal angiomatosis only at the systemic level. Correlations between PDGF-BB and PIGF-1, as well as PIGF-1 and MIP-1β were shown. A significant increase in VEGF-A, HGF, VEGF-D, as well as MCP-1 concentrations was shown in the lacrimal fluid. The inversion of PDGF-BB concentrations in serum and lacrimal fluid was noted. Analysis of intraocular cytokine levels revealed a significant increase in VEGF-A and HGF concentrations, with marked decrease in MIP-1α and MIP-1β. PDGF-BB in 100% of cases was determined only in vitreous body of patients with retinal angiomatosis. With respect to the revealed characteristic shifts of HGF/SF intraocular production in retinal capillary hemangioma, it seems relevant to search ways for its inhibition, thus providing potential basis for a new therapeutic strategy in treatment of retinal angiomatosis.

Photodynamic Therapy in Ocular Oncology.

Over the past two decades, we have witnessed the increasing use of photodynamic therapy (PDT) in the field of ocular oncology. Based on a review of the literature and our own experience, we herein review the role of PDT for the management of intraocular tumors. The discussion includes two main topics. First, we discuss the application of PDT for benign tumors, including circumscribed choroidal hemangioma, choroidal osteoma, retinal astrocytoma, retinal capillary hemangioma (retinal hemangioblastoma), and retinal vasoproliferative tumor. Second, we assess the role of PDT for malignant tumors, including choroidal melanoma and choroidal metastasis.

Swept Source OCT Angiography of Optic Nerve Head Retinal Capillary Hemangioma

Swept Source OCT Angiography of Optic Nerve Head Retinal Capillary Hemangioma

Visual prognosis based staging for retinal capillary hemangioma

Purpose . To analyze the literature to determine the visual prognosis of eyes affected by retinal angiomatosis of Von-Hippel Lindau disease (VHL). Study Type . Retrospective literature analysis. Methods . Medline and PubMed searches were performed for publications on retinal capillary hemangioma (CRH). Data was collected on patient age of presentation, CRH laterality, location, treatment, and progression. Statistical analyses using Pearson Chi-Square test, likelihood ratio, and Fischer’s exact tests were performed for the effect of these characteristics on visual acuity outcomes. Results . Vision outcomes need to be based on current technology to be clinically relevant. Therefore, only significant publications from 1960 to 2019 were included. Of these, 5 clinical case series included, a total of 427 cases. Of these 69.4 % (58–94.1 %) cases were unilateral CRH, 30.6 % (5.9–42 %) were bilateral, 18.1 % (10.9–23.5 %) were juxtapapillary in location. Major factors affecting visual acuity were age at onset (p = 0.03), location (p < 0.0001) and multifocality (p = 0.0005). Of interest, CRH-related vision loss was independent of the presence of VHL disease (p = 0.157). Conclusion . In this study, age at onset, location, multifocality of CRH were the most important predictors of vision loss, which were used to create a vision-outcome based classification system. This information can be used to counsel patients and for informed consent.

Multimodal Imaging of a Case of Juxtapapillary Retinal Capillary Hemangioma

Multimodal Imaging of a Case of Juxtapapillary Retinal Capillary Hemangioma

Multiple Retinal Capillary Hemangioma Associated with NeurofibromatosisType 1

Multiple Retinal Capillary Hemangioma Associated with NeurofibromatosisType 1

Management of capillary Retinal capillary hemangiomas: Case report and review of literature

Management of capillary Retinal capillary hemangiomas: Case report and review of literature

Detection of retinal microvascular changes in von Hippel-Lindau disease using optical coherence tomography angiography.

PurposeVon Hippel-Lindau (VHL) disease is a hereditary disorder that can lead to ophthalmic manifestations, including retinal capillary hemangioma (RCH). The diagnosis of RCH is often guided by wide-field fluorescein angiography. In some cases, optical coherence tomography angiography (OCT-A) serves as a non-invasive alternative to FA. Herein, we used OCT-A to examine the macular microvasculature in patients with VHL disease.SubjectsSubjects were selected from patients with a diagnosis of VHL. The control group included eyes without retinal diagnosis from patients with an episode of unilateral retinal detachment or trauma and age ≤ 50 years old.MethodsSubjects were scanned on the Optovue RTVue-XR device to acquire 3mm x 3mm OCT-A images of the superficial (SCP) and deep capillary plexus (DCP). SCP and DCP vessel density (VD) were calculated after the images were binarized. Furthermore, for subjects with RCH, each OCT-A image was divided equally into four quadrants. SCP and DCP VD of quadrants with RCH were compared to those without RCH. T-tests were performed for statistical analysis.Results67 eyes with a history of VHL disease were included as study subjects, while 16 eyes were included as controls. Significant increases in VD were found in patients with VHL disease for both the SCP (p = 0.0441) and DCP (p = 0.0344). When comparing quadrants with associated RCH development to those without, we found no significant difference in SCP VD (p = 0.160) or DCP VD (p = 0.484).ConclusionsOCT-A can detect changes in the retinal microvasculature in the macula of patients with VHL disease. OCT-A imaging may be an additional tool for screening and early detection of patients at risk of developing ocular complications of VHL disease. Future studies should explore subtle progression on OCT-A associated with the pathogenesis and development of RCH, particularly with larger scan patterns.