Choroidal Hemangioma Research Articles

Overlap Syndrome: A case with features of both Sturge Weber and klippel trenaunay phacomatoses

Background: Sturge Weber and Klippel Trenaunay syndromes are mesodermal phacomatoses occurring sporadically. We report a rare case with overlapping features of both syndromes. Case report: An eight-year-old girl complains of low vision and pain in left eye for nine months. Examination revealed port wine stain on left side of face, fingertips and toes present since birth. Length of both legs was symmetrical with skin hypertrophy. Visual acuity was light perception and lid swelling with hyphema noticed in left eye. BScan showed retinal detachment with choroidal haemangioma. CT scan showed orbital inflammation. The haemangioma was treated with Intravitreal Bevacizumab followed by vitrectomy with subretinal fluid drainage and focal laser. Radiotherapy was given to the left orbit. Conclusion: Nevus flammeus, choroidal haemangioma, retinal detachment and neovascular glaucoma consistent with Sturge Weber syndrome. Soft tissue hypertrophy with dilated veins is compatible with Klippel Trenaunay syndrome.

Ruthenium-106 plaque brachytherapy for the treatment of diffuse choroidal hemangioma in Sturge-Weber syndrome.

To evaluate the efficacy of ruthenium-106 plaque brachytherapy for the treatment of diffuse choroidal hemangioma (DCH) in Sturge-Weber syndrome (SWS). A total of 8 patients with DCH in SWS managed with plaque brachytherapy were retrospectively included. Patients were treated with ruthenium-106 plaque therapy (median apex dose: 83 Gy) at the thickest tumor region. On follow-up, we recorded the tumor thickness, the best-corrected visual acuity (BCVA), subretinal fluid (SRF) status, and complications following treatment. At a median follow-up of 43mo, tumor regression was observed in all cases, with a complete resolution of SRF and reduction in tumor-thickness. No radiation complications were recorded during the follow up time. Ruthenium-106 plaque therapy to the thickest portion of the tumor seems to be a useful treatment in patients with DCH in SWS.

High-dose ruthenium-106 plaque therapy for circumscribed choroidal hemangioma: a retrospective study of 25 Chinese patients.

To evaluate the efficacy and safety of ruthenium-106 (106Ru) plaque radiotherapy at a dose (>50 Gy) higher than recommended (29-50 Gy) for treatment of circumscribed choroidal hemangioma (CCH) in Chinese patients. This retrospective study included 25 symptomatic CCH patients undergoing 106Ru plaque brachytherapy involving 25 eyes between January 2005 and August 2016. Ophthalmic examination was performed at the baseline and at each post-treatment follow-up visit, using best-corrected visual acuity (BCVA), dilated fundus examination, and B-scan ultrasonography. The primary efficacy outcome measures included the changes in BCVA and hemangioma dimensions at the last followup visit from the baseline. The mean follow-up duration was 28.0±26.6 (range, 12-110)mo. All the hemangiomas were located in the posterior pole except for two involving the fovea. The mean apex dose of 106Ru plaque radiotherapy was 84.4±19.7 Gy. The mean BCVA improved from 41.4±29.3 (0-97) at the baseline to 53.0±33.8 (0-97) ETDRS letters at the last visit (P=0.01). The mean hemangioma height declined from 3.98±0.88 (2.40-5.50) mm to 0.84±1.63 (0-6.47) mm (P≤0.001), and the greatest linear diameter (GLD) reduced from 9.36±2.23 (6.80-15.00) to 7.40±2.45 (0-13.00) mm (P≤0.001). Hemangioma size increased in one (4%) eye with a worsened vision, and subretinal fluid completely resolved in all but one patient (4%). Radiation-related retinopathy was observed in two patients at post-treatment 9 and 11mo, respectively. 106Ru plaque brachytherapy at a dose (>50 Gy) higher than recommended (29-50 Gy) is an effective treatment regimen for symptomatic CCH associated with significantly improved visual acuity and a favorable safety profile in Chinese patients.

Effect of prolonged photodynamic therapy irradiation time combined with intravitreal injection of ranibizumab in the treatment of circumscribed choroidal hemangioma

Objective To observe the clinical effect of prolonged photodynamic therapy (PDT) irradiation time combined with intravitreal injection of ranibizumab in the treatment of circumscribed choroidal hemangioma (CCH). Methods A retrospective clinical study. From March 2012 to March 2018, 51 eyes of 51 patients diagnosed in Shenzhen Eye Hospital were included in the study. Among the patients, the tumor of 36 eyes were located in macular area, of 15 eyes were located outside macular area (near center or around optic disc). All patients underwent BCVA, color fundus photography, FFA, ocular B-scan ultrasonography and OCT examinations. The BCVA examination was performed using the international standard visual acuity chart, which was converted into logMAR visual acuity. OCT showed 48 eyes with macular serous retinal detachment. of 36 eyes with tumor located in macular area, the logMAR BCVA was 0.05±0.05, the tumor thickness was 4.5± 2.2 mm, the diameter of tumor was 9.7±3.6 mm. Of 15 eyes with tumor located outside macular area, the logMAR BCVA was 0.32±0.15, the tumor thickness was 3.8±1.4 mm, the diameter of tumor was 7.7± 1.9 mm. PDT was performed for all eyes with the irradiation time of 123 s. After 48 h, all patients received intravitreal injections of 0.5 mg ranibizumab (0.05 ml). At 1, 3 and 6 months after treatment, the same equipment and methods before treatment were used for related examination. BCVA, subretinal effusion (SRF), tumor leakage and size changes were observed. BCVA, tumor thickness and diameter before and after treatment were compared by t test. Results At 6 months after treatment, the tumor was becoming smaller without scar formation. FFA showed that the blood vessels in the tumor were sparse compared with those before treatment, and the fluorescence leakage domain was reduced. OCT showed 43 eyes of macular serous detachment were treated after the combined treatment. The logMAR BCVA were 0.16±0.15 and 0.55±0.21 of the eyes with tumor located in or outside macular area, respectively. The difference of logMAR BCVA between before and after treatment was significant (t=-2.511, -2.676; P=0.036, 0.040). Both the tumor thickness (t=3.416, 3.055; P=0.011, 0.028) and diameter (t=4.385, 4.171; P=0.002, 0.009) of CCH patients were significantly reduced compared with that before treatment. Conclusion The tumor of CCH can be reduced by prolonged PDT irradiation time combined with intravitreal injection of ranibizumab. Key words: Photochemotherapy; Choroid neoplasms/therapy; Hemangioma/therapy; Angiogenesis inhibitors/therapeutic use

A case of Choroidal Hemangioma in pregnancy.

We report a 29 year old female gravid 1 with 28 weeks of gestation was referred by her obstetrician for blurred vision in her left eye from the past 3 days. On examination patient manifested a facial hemangioma over left face and lid. Fundus examination of left eye revealed a choroidal hemangioma which increased in size during the course of pregnancy. No intervention was done and the tumour regressed completely after pregnancy. Observation alone may be indicated in cases of choroidal hemangioma demonstrating sub-retinal fluid in pregnancy. Circumscribed choroidal hemangioma can manifest in Sturge-Weber syndrome rather typically found diffuse type.

Effectiveness and Safety of Intravitreal Injection of Conbercept as an Initial Treatment for Exudative Circumscribed Choroidal Hemangioma

Objective: To investigate the effectiveness and safety of intravitreal injection of conbercept (IVC) as the initial treatment for exudative circumscribed choroidal hemangioma (CCH). Methods: Forty-two eyes of 42 patients received 3 monthly IVC (0.5 mg/0.05 mL) as the initial treatment. Three months later, the patients were assessed for further treatment including observation, reinjection of conbercept, laser photocoagulation (if the lesion was 3,000 μm away from the macular fovea), or photodynamic therapy (PDT; if the lesion was under the macular fovea). Anatomical and functional responses including best corrected visual acuity (BCVA), central foveal thickness (CFT), and tumor size were analyzed. Results: Twenty-three patients (54.76%) were sensitive to the monotherapy of IVC. Fourteen patients (33.33%) were insensitive to IVC and underwent rescue laser photocoagulation, and 5 patients (11.90%) underwent rescue PDT due to insensitivity to IVC treatment at 3 months. For subgroup analysis, although no statistical difference was found for BCVA at any follow-up time point compared to baseline, an increasing tendency of BCVA was found in the IVC group (p> 0.05). The mean CFT decreased significantly from 427.13 ± 214.74 μm at baseline to 259.83 ± 61.68 μm at 6 months in the IVC group (p< 0.05). No influence on tumor size was found in the IVC group. Conclusion: IVC as the initial treatment might be an option for exudative CCH.

Indocyanine green angiography in the diagnostics of choroidal tumors

To study the use of ultrasound color Doppler imaging and indocyanine green (ICG) angiography for identification of malignant vasculature of choroidal tumors. The study included 46 patients (46 eyes) with tumors of the choroid: 19 men and 26 women aged 22 to 89 years, average age 53.2±17.1 years. All patients underwent indocyanine green angiography and Doppler ultrasound imaging. At the time of examination, thickness of the tumors ranged from 1.1 mm to 3.0 mm, and the base diameter from 4.0 mm to 13.0 mm. Choroidal melanoma was diagnosed in 27 patients, choroidal nevus in 10, choroidal hemangioma in 5, and choroidal metastasis in 4 patients. The results of indocyanine green angiography demonstrate that the pathological malignant vasculature was determined: nevi was diagnosed in 7 out of 10 patients, choroidal melanoma in 16 out of 27, metastasis in 2 out of 4, and hemangioma in none of the 5 patients. Pathological malignant vasculature was determined in most patients with choroidal melanoma (77.8%), in all patients with choroidal hemangioma and in 3 patients with choroidal metastases. Ultrasound imaging revealed malignant vasculature in 15 of 27 (56%) patients with choroidal melanoma, it could be identified in all patients with hemangioma in 2 patients and with choroidal metastases, and in none patients with choroidal nevi. Indocyanine green angiography helps verify malignant vasculature in 77.8% of patients with small and medium sized choroidal melanomas. Angioarchitecture of choroidal tumors visualized with indocyanine green angiography is characterized by variability of patterns, which should be taken into account in the differential diagnosis of various pathological diseases.

Sonologic similarity in the differential diagnosis of pediatric neovascular glaucoma

B-mode ultrasonography is widely used in ophthalmology to assess the retina and vitreous when there is a media opacity or very small pupil size, which prevents direct examination of fundus. Unlike other specialties which request radiologists to perform ultrasonography as part of their investigation, we ophthalmologists are able to perform B-scan by our side of the department as and when required. Here, we report a pediatric case in which B-scan happened to be done by the radiologists; further details enhanced with Doppler uptake resulted in a diagnostic dilemma to both ophthalmologists and radiologists. A 10-year-old male child presented with severe pain and redness in the left eye with repeated vomiting. Ocular examination revealed neovascular glaucoma in the left eye with an absent perception of light. Ultrasound B-scan performed by a radiologist showed a linear hyperechoic structure extending from the posterior aspect of the lens to the optic disc with Doppler uptake, which they suggested as a possibility of persistent hyperplastic primary vitreous (PHPV). However, evaluation of previous treatment records revealed an earlier diagnosis of circumscribed choroidal hemangioma with bullous retinal detachment. Evaluation of pediatric neovascular glaucoma thought about as PHPV yielded a surprising diagnosis of choroidal hemangioma with retinal detachment. Hence, the latter needs to be kept in mind, although the radiological findings mislead to diagnose PHPV.

Phakomatosis pigmentovascularis with sturge–Weber syndrome

An 11-year-old boy presented with port-wine stain on the right side of the face and pigmentation of the sclera and iris in the left eye (LE). Fundus examination revealed inferior rim excavation of disc with orangish red tomato ketchup appearance of background retina suggestive of diffuse choroidal hemangioma in the right eye (RE) and normal retina in the LE. Ultrasonography showed increased thickness of retinochoroidal scleral complex, and swept-source optical coherence tomography showed choroidal thickening in the RE. He was diagnosed to have phakomatosis pigmentovascularis (PPV) with Sturge–Weber syndrome and glaucoma. PPV is a rare congenital disorder characterized by the presence of capillary malformation and pigmentary nevi. PPV can present with or without systemic involvement. Those without systemic involvement need to be followed up closely as they can manifest later in life.

Отграниченная гемангиома хориоидеи: особенности течения, лечение

Отграниченная гемангиома хориоидеи: особенности течения, лечение

Лечение ограниченной гемангиомы хориоидеи: термотерапия или брахитерапия. Сравнительный анализ

Purpose. To carry out a comparative analysis of results in the treatment of the circumscribed choroidal hemangioma (CCH) by methods of diode-laser transpupillary thermotherapy and brachytherapy. Material and methods. The data of treatment of 185 patients (185 eyes) with circumscribed choroidal hemangioma were analyzed, previously the patients were divided into groups by type of treatment. For the comparative analysis, the groups were standardized by tumor thickness, length of the base, tumor localization, follow-up period, initial visual acuity, frequency of detectable retinal detachment. As a result, the group of diode-laser thermotherapy consisted of 54, and the group of brachytherapy – 33 individuals. Results. After the treatment, the average visual acuity after treatment in the group of transpupillary thermotherapy was significantly higher (0.47) than in the group of brachytherapy (0.2) (p=0.0004). It was noted that the improvement of visual acuity after transpupillary thermotherapy was achieved in 27 (53%) cases more often than after brachytherapy – in 5 cases (15%) (p=0.01 χ 2 criterion). Each of the treatment types led to the resorption of the subretinal transudate and the reposition of the detached retina in all cases. In the evaluation of changes in average visual acuity before and after the treatment in each group, there were noted statistically significant differences in the group of transpupillary thermotherapy (0.33 before the treatment, 0.47 after the treatment; p=0.046) and their absence in the group of brachytherapy (0.22 before treatment, 0.2 after treatment; p=0.7). Conclusion. Diode laser transpupillary thermotherapy leads to better functional results compared with brachytherapy. Taking into consideration the absence of surgical trauma in transpupillary thermotherapy and an increased risk of complications in brachytherapy, the priority in the choice of a treatment method for CCH should be given to transpupillary thermotherapy.

Focal ossification as one of the reasons for erroneous diagnosis of chorioretinal lesions

Focal calcifications of the retina and choroid occur usually in such well-known tumors as: retinoblastoma, choroidal osteoma, choroidal hemangioma, retinal astrocytoma. In addition, cases of idiopathic or secondary calcification are known, the most common of them is sclerochoroidal calcification. The article provides a detailed analysis of the clinical and tomographic pictures of ossifying conditions occurring in adults. It is shown that, in addition to a different ophthalmoscopic picture, these conditions are characterized by a different level of localization of the pathological calcification zone and a different stage of retinal damage.

Small choroidal melanoma and pseudomelanomas: methods of differential diagnostics. Part 1

Choroidal melanoma is a malignant tumor prone to early metastasis. Its clinical picture is polymorphic, as a result of which it can simulate many diseases of the fundus, which are referred to in the literature as pseudomelanomas. Among these are: choroidal nevus, localized choroidal hemangioma, melanocytomas, choroidal metastatic carcinoma, congenital hypertrophy of retinal pigment epithelium, late stage of age-related macular degeneration, focal retinal choroiditis, organized subretinal hemorrhage, retinal hemangiomas. However, studies comparing the clinical signs of all listed pseudomelanomas and small choroidal melanoma are few. The first part of the review describes the ophthalmoscopic picture of the small choroidal melanoma and pseudomelanomas.

A case of choroidal osteoma in a 5‐year‐old child

AbstractPurposeTo report a case of choroidal osteoma (CO) in a 5‐year‐old boy.MethodsCase reportResultsA 5‐year‐old child presented with decreased vision in both eyes. His best‐corrected visual acuity (BCVA) was 20/50 in the right eye (OD) and 20/100 in the left eye (OS). The anterior segment was normal in both eyes. Cycloplegic refraction was +2.50 ‐2.25 x 10 in the OD and +3.25 ‐2.50 x 180 in the OS. Funduscopy of the OS revealed a large subretinal, yellowish lesion in the juxta‐papillary area. B‐scan ultrasonography of the OS revealed a dense echogenic plaque with acoustic shadowing. The axial CT scan also showed a calcified lesion in the posterior pole. Optical coherence tomography (OCT) was normal at the fovea but revealed subretinal fluid (SRF) at the superior temporal vascular arcade. Spectacle correction was prescribed and alternating part‐time occlusion was performed. The patient was asked to come for regular follow‐up. Six months later his BCVA was 20/25 in both eyes, no variations of the mass were noted and the SRF disappeared.ConclusionsOC is a rare, benign ossifying tumor. It is typically found in healthy young women, mostly unilateral, in the posterior pole of the eye, near the optic disc. It is relatively rare in children. OC must be differentiated mainly from amelanotic choroidal melanoma, choroidal nevus, choroidal hemangioma, choroidal metastasis, sclerochoroidal calcification and posterior scleritis. In children it can pose a serious diagnostic challenge as it can mimic other lesions at posterior pole, such us retinoblastoma, retinochoroidal coloboma, and toxoplasmic retinochoroiditis. Asymptomatic or stable CO can be observed. For choroidal neovascular membranes photodynamic therapy and laser treatment have been used in the past. More recently, anti‐VEGF treatments have been employed with success.

Ocular manifestations of Sturge–Weber syndrome

Sturge-Weber syndrome (SWS) is a rare, congenital disease which frequently involves the eye. It is important that ophthalmologists recognize this syndrome and are aware of its range of ocular manifestations. The aim of this article is to present our understanding of the pathogenesis and clinical manifestations of this syndrome and provide updated information on the treatment of SWS glaucoma and choroidal hemangioma. SWS glaucoma usually fails medical management. Surgical options include angle procedures, filtering procedures, device placement, and combination procedures. Combination procedures have become popular in this population due to the single procedure failure rate of angle surgery and the complications associated with device implantation. Choroidal hemangioma is best treated by photodynamic therapy. Lifelong monitoring for ocular complications related to SWS is essential. There is a need for consensus guidelines on care and surveillance of patients with SWS to provide the best care for these patients.

Management of Diffuse Choroidal Hemangioma with Plaque Brachytherapy

Management of Diffuse Choroidal Hemangioma with Plaque Brachytherapy

Gamma Knife Radiosurgery for Choroidal Hemangioma: A Single-Institute Series

Choroidal hemangioma (CH) is a benign vascular tumor that induces subretinal fluid collection or exudative retinal detachment and consequent visual symptoms. Current standard treatments for CH include cryotherapy, diathermy, photocoagulation, photodynamic therapy, transpupillary thermotherapy, and radiation therapy. Stereotactic radiosurgery has recently been applied to the treatment of CH because of its characteristic stiff dose-fall-off and accuracy. We have adopted gamma knife radiosurgery (GKRS) to treat CH and have retrospectively assessed tumor volume reductions and improvements to visual acuity achieved thereby. Fourteen patients with CHs were treated with GKRS from November 2006 to December 2017. Eight patients had circumscribed CH, and 6 exhibited diffuse CHs and were diagnosed with Sturge-Weber syndrome. The mean age of patients was 27.1 years (range: 8-68 years) and the mean duration of clinical or radiological follow-up was 40.2 months (range: 5-105 months). The mean volume of the tumors at the time of GKRS was 533.5 mm3 (range: 124-1150 mm3), and the mean prescribed marginal dose was 11.6 Gy (range: 10-16 Gy) with 50% isodose lines. The tumor volume decreased by the last follow-up in all patients. The visual acuity improved in 9 patients (64%) and decreased in 1 (7%). Six patients (43%) required trans-pars plana vitrectomy before or after GKRS. There were no symptomatic complications from radiation injury during the follow-up periods. GKRS could be an acceptable alternative treatment for symptomatic CH when standard therapy is not feasible.

Circumscribed Choroidal Hemangioma: Visual Outcome in the Pre-Photodynamic Therapy Era versus Photodynamic Therapy Era in 458 Cases

To analyze visual outcomes after treatment of choroidal hemangioma in the pre-photodynamic therapy (PDT) era versus PDT era. Retrospective, comparative case series. A total of 458 patients with circumscribed choroidal hemangioma. Comparison of hemangioma managed in the pre-PDT (1967-2001) era versus PDT (2002-2018) era. Visual acuity outcome. A total of 458 tumors were treated over this 51-year period. A comparison (pre-PDT [n= 220 cases] vs. PDT [n= 238 cases]) revealed PDT era patients were of older mean age (48.9 vs. 53.8 years, P= 0.002) and were more likely to have systemic hypertension (17.7% vs. 33.8%, P < 0.001), tumor location in the macula (57.4% vs. 67.5%, P= 0.01), subretinal fluid on OCT (33.3% vs. 70.7%, P= 0.01), and greater extent of overlying lipofuscin (P= 0.001). Findings of tumor basal diameter and thickness and fluorescein and indocyanine green angiography were no different in the 2 eras. Treatment (pre-PDT vs. PDT) included argon laser photocoagulation (42.1% vs. 0.4%), PDT (0% vs. 43.8%), transpupillary thermotherapy (0% vs. 0.4%), plaque radiotherapy (7.0% vs. 5.2%), external beam radiotherapy (1.4% vs. 1.3%), enucleation (0.9% vs. 0.4%), and observation (48.6% vs. 47.6%). After treatment, patients in the PDT era demonstrated better mean logarithm of the minimum angle of resolution visual acuity (1.28 vs. 0.51, P < 0.001) (Snellen equivalent 20/400 vs. 20/63, P < 0.001). Final visual acuity was ≥20/40 for those with entering vision of ≥20/40 (59.6% vs. 74.7%, P= 0.001) and for those with entering vision of 20/50-20/200 (25.4% vs. 47.3%, P < 0.001). Management of choroidal hemangioma in the PDT era has allowed for significantly better visual outcome compared with the pre-PDT era, with mean final visual acuity of 20/400 (pre-PDT era) versus 20/63 (PDT era).

GNAQ Q209R Mutations Are Highly Specific for Circumscribed Choroidal Hemangioma.

Several tumors, including uveal melanoma, show somatic mutations of GNAQ/GNA11. Circumscribed choroidal hemangioma is a benign tumor that becomes symptomatic in adulthood. In some patients, morphologic examination of biopsies is required for differential diagnosis between amelanotic choroidal melanoma and circumscribed choroidal hemangioma. Here, we report the results of GNAQ/GNA11 mutation analysis in samples from circumscribed choroidal hemangioma. Deep amplicon sequencing (Illumina MiSeq, San Diego, CA, USA) of positions R183 and Q209 of GNAQ and GNA11 in tissue samples from 33 patients with histologically diagnosed circumscribed choroidal hemangioma. All patients underwent biopsy or enucleation at our clinic between 2008 and 2018. To enable detection of variant alleles at low fractions, read depth exceeded 15,000-fold. DNA for genetic analysis was prepared from either snap-frozen (n = 22) or FFPE (n = 11) tissue samples. Samples from 28/33 patients (85%) showed a somatic missense mutation of GNAQ (c.626 A > G) predicted to result in p.Q209R. Variant allele fraction was variable (range 2.3% to 28%). Variants of GNAQ resulting in p.Q209 are characteristic for circumscribed choroidal hemangiomas. It appears that the GNAQ mutation spectrum in this tumor is narrow, possibly restricted to p.Q209R. Moreover, the spectrum is distinct from that of uveal melanoma, in which alterations resulting in p.Q209R are very rare.

Choroidal Vascular Pattern in Cases of Sturge-Weber Syndrome

To study the choroidal vascular pattern in patients with Sturge-Weber syndrome (SWS) using swept-source OCT (SS-OCT). Prospective comparative observational study. All patients with SWS with no history of prior treatment for posterior segment pathology were included. Both eyes of all patients were studied using fundus imaging, SS-OCT, fundus fluorescein angiography (FFA), and indocyanine green angiography (ICG) by 2 independent observers. The FFA and ICG were screened for any vascular abnormalities. The SS-OCT was evaluated for choroidal changes. A total of 34 eyes of 17 patients with diagnosed SWS in the age group 9 to 26 years were studied. The FFA and ICG in 7 and 11 patients, respectively, showed some vascular abnormalities. SS-OCT was performed in all patients. The diffuse choroidal hemangioma (DCH) was characterized by loss of the choroidal vascular pattern, increase in the choroidal thickness and loss of visualization of the sclerochoroidal interface. Based on the FFA, ICG, and SS-OCT imaging, there were 3 patients with no DCH, 5 with bilateral DCH, and the remaining 9 patients had unilateral DCH. The detection rate was 50% clinically: 52.94% with FFA, 82.35% with ICG, and 86.36% with SS-OCT. There was substantial agreement between the 2 observers for all 3 investigations. SS-OCT is a reliable noninvasive imaging modality for early diagnosis and follow-up of DCH over time.