Vasoproliferative Tumor Secondary to Sarcoidosis-Associated Intermediate Uveitis.

We report the visual and clinical outcomes of a middle-aged woman who presented with exudative retinal detachment (ERD) secondary to a vasoproliferative tumor (VPT) in an eye with sarcoidosis-associated intermediate uveitis. A 55-year-old woman previously diagnosed with sarcoidosis presented with decreased vision in the left eye (LE). Visual acuity in the LE was counting fingers. She had active vitritis, and a peripheral retinal vascular mass was noted in the superotemporal periphery. The mass was associated with ERD involving the posterior pole. The patient was managed with systemic and intravitreal steroids, and cyclosporine was subsequently added as a steroid-sparing agent. Because of recurrence of ERD, the patient underwent pars plana vitrectomy, and cryotherapy and laser photocoagulation were applied to the VPT. Two months postoperatively, visual acuity in the LE improved to 6/10. There was marked regression of the VPT and total resolution of the ERD. In conclusion, we report a favorable visual and clinical outcome in a patient with VPT-associated ERD who responded to a combination of medical therapy and surgical intervention. VPT may lead to different remote complications, so timely diagnosis of these tumors and proper management of their complications is warranted.

Epiretinal Membrane and Vitreomacular Traction Associated With Vasoproliferative Tumor in Macular Telangiectasia Type 2: A Case Report.

We report a case of a 62-year-old man who presented with blurred and distorted vision in both eyes. Fundus examination revealed a fibrous band-like membrane emanating from the disc to the foveal center in the right eye, aneurysmal gray parafoveal lesions in both eyes, and an inferotemporal peripheral vascular tumor in the right eye. The presence of an epiretinal membrane with vitreomacular traction in this patient has led to the diagnosis of an incidental peripheral vascular tumor. To our knowledge, there are no reports describing an association between macular telangiectasia type 2 and epiretinal membrane formation with vitreomacular traction due to a vasoproliferative tumor.

Vasoproliferative retinal tumors: Clinical presentation and treatment outcome.

OBJECTIVE: To study the clinical presentation and treatment outcomes of patients with vasoproliferative retinal tumor (VPRT) in India. METHODS: Retrospective study of 50 eyes in 47 patients. RESULTS: Of the 50 eyes, 25 (50%) were primary and 25 (50%) were secondary. Common ocular pathologies related to secondary VPRT included retinal detachment (n = 8, 32%) and Coats disease (n = 7, 28%). Overall, the mean age at presentation was 35 years (range; 3-74 years) and included 30 (64%) males and 17 (36%) females. The lesions were unilateral in 44 (94%) patients. Secondary retinal features included intra/subretinal exudation (n = 41, 82%), vascularity (n = 32, 64%), subretinal fluid (n = 21, 42%), retinal neovascularisation (n = 9, 18%) and vitreous hemorrhage (n = 8, 16%). Thirty-four eyes (68%) underwent cryotherapy, of which 16 eyes (47%) received adjunct intravitreal anti-VEGF (12 eyes) or corticosteroid injection (4 eyes). Primary surgery included vitrectomy with/without encirclage and cryotherapy (n = 12, 24%) and plaque brachytherapy (n = 3, 6%). At last follow-up (mean 9.7 months, range 3-67 months), 42 eyes (84%) had complete tumor regression with no improvement in visual outcomes when comparing presenting and final best corrected visual acuity. Comparing primary versus secondary VPRT, secondary VPRT presented a decade earlier (31 vs 40 years), needed surgical intervention (48% vs 20%), had poor tumor control (72% vs 92%) and worse visual outcomes (p < 0.05). CONCLUSION: VPRTs commonly present as a unilateral lesions in the adult population with equal prevalence of primary and secondary variants. Compared with primary VPRT, secondary VPRTs require surgical treatment and are associated with poor visual outcome.

Progress in diagnosis and treatment of retinal vasoproliferative tumor / 中华实验眼科杂志

Retinal vasoproliferative tumor (RVPT) is a rare benign glial proliferative tumor of unknown origin.The fundus is typically characterized by yellow or pink tumor-like lesions of the retina around the inferior temporal region, often accompanied by subretinal fluid, exudation, epiretinal membrane and other complications.Typical RVPT showed high fluorescence on fundus fluorescein angiography, diffuse leakage in venous phase and late stage, uneven middle or high reflection signal on B-ultrasound, and it was not difficult to diagnose combined with fundus changes.Atypical RVPT should be differentiated from retinal capillary hemangioma caused by von Hippel-Lindau disease, Coats disease and familial exudative vitreoretinopathy.The pathogenesis of RVPT is unclear, but histopathology shows that it may be the reactive proliferation of glial cells.At present, there is no unified treatment plan for RVPT, mainly cryotherapy, laser photocoagulation, local radiotherapy, transpupillary thermal therapy or photodynamic therapy for tumor.For patients with vitreous hemorrhage, vitreoretinal surgery is feasible.This article reviewed the clinical features, diagnosis and differential diagnosis, pathology and pathogenesis, and treatment progress of RVPT.

EN BLOC RESECTION OF RETINAL VASOPROLIFERATIVE TUMOR USING 23G VITRECTOMY. A CASE REPORT.

PURPOSE: Retinal vasoproliferative tumor is one of the benign vascular tumors which in advanced stages leads to exudative retinal detachment with the formation of epiretinal and subretinal membranes. In such advanced stages, one of the therapeutic options is pars plana vitrectomy. This article presents the case of a patient on whom was performed 23-gauge pars plana phacovitrectomy with en bloc resection of the tumor followed by histological confirmation. CASE REPORT: A 70-year-old patient with a one-year history of unilateral loss of vision in his left eye was admitted to our clinic for examination in February 2018. At admission, the best corrected visual acuity in the right eye was 1.0, and in the left eye was light perception. Based on the clinical picture, sonographic examination of the eye, and fluorescein angiography, the patient was diagnosed with a retinal vasoproliferative tumor. Due to the advanced stage of disease, we proceeded with surgical intervention. We performed 23-gauge phacovitrectomy with a bloc resection of the tumor. Subsequent histological examination confirmed the presence of the presumed tumor. The follow-up exam a few months later showed a completely attached retina with silicone oil tamponade, without exudative retinopathy. However, the best corrected visual acuity improved only slightly to the ability to count fingers at one meter. CONCLUSION: Pars plana vitrectomy with en bloc resection of retinal vasoproliferative tumor is one of the therapeutic modalities in advanced stages.

Case Report: Exacerbation and Spontaneous Separation of the Epiretinal Membrane Following Laser Photocoagulation of a Vasoproliferative Tumor of the Retina.

The present report concerns a rare vasoproliferative tumor of the retina (VPTR) combined with a severe case of secondary epiretinal membrane (ERM). A 56-year-old male patient was diagnosed with VPTR and secondary ERM of the left eye. The patient underwent two rounds of laser photocoagulation (LP) of the tumor. The exacerbation of the ERM was observed after the first round of LP, while spontaneous separation over the five-month follow-up period was noted after the second round of LP. Thus, LP may represent a viable alternative treatment approach for VPTR combined with severe ERM.

A case of primary vasoproliferative tumor with full-thickness macular hole.

A 53-year-old female presented with defective vision in the left eye of one week duration with a best-corrected visual acuity (BCVA) of 6/9. Fundus examination showed a primary retinal vasoproliferative tumor (RVPT) at the inferotemporal quadrant and a small full-thickness macular hole (MH). The patient underwent cryotherapy for RVPT, followed later by vitrectomy with internal limiting membrane peeling and gas tamponade. The tumor regressed and the MH was closed with BCVA of 6/9. RVPT can present with remote macular complications like epiretinal membrane, cystoid macular edema, macular exudates, and rarely full-thickness MH. Management should be directed towards the tumor as well as the complication.

Neovascular Glaucoma in Children: A case series and a review of the literature.

PURPOSE: To study the uncommon causes and treatment options for neovascular glaucoma in children. PATIENTS AND METHODS: A review of the literature on neovascular glaucoma in children was conducted and we present three cases of neovascular glaucoma in children. RESULTS: We present three cases of neovascular glaucoma: two cases were secondary to a retinal vasoproliferative tumor-one to neurofibromatosis type 1 and the other to exudative retinopathy secondary to mild retinopathy of prematurity-and one case was secondary to a central retina vein occlusion secondary to an optic nerve glioma. Vision in the affected eye was severely impaired in all the children. CONCLUSION: The diagnosis and treatment of neovascular glaucoma in children is challenging and often a complication of a systemic or late-stage ocular condition. An appropriate diagnosis and estimation of the visual potential are essential to determine the correct treatment, especially in young children.

Surgery for Combined Hamartoma of the Retina and Retinal Pigment Epithelium.

There is no consensus on whether and when surgical treatment is indicated for combined hamartoma of the retina and retinal pigment epithelium (CHRRPE). We aim to discuss the benefits of surgical intervention and techniques that may improve the outcome. A 24-year-old man experienced progressive visual loss for 6 months in his left eye due to CHRRPE. At presentation, visual acuity was 1.3 LogMAR and fundoscopy revealed extensive tractional pre- and epiretinal membranes, subretinal exudation, and a vasoproliferative tumor in the inferior periphery. A complete vitrectomy was performed, while paying special attention to vitreous shaving at the vitreous base and removal of vitreoschisis-induced vitreous cortex remnants (VCR) from the retinal surface posterior to the vitreous base. Tractional membranes and internal limiting membrane were peeled, and the vasoproliferative tumor was excised. Silicone oil tamponade was removed 11 weeks after surgery. No intra- or postoperative complications occurred. Visual acuity improved to 0.8 LogMAR and remained stable for 48-month follow-up. Vitreoretinal surgery can prevent complications that occur with CHRRPE. In addition, visual function may improve even if the initial visual acuity is low. Timely and complete vitrectomy with extensive membranectomy and detection and removal of VCR is recommended to avoid complications in challenging CHRRPE.

Vitreociliary Block in a Patient With Uveitis and Previous Laser Posterior Capsulotomy.

PURPOSE: To report a case of vitreociliary block (VCB) six months after a laser posterior capsulotomy (LPC). CASE REPORT: A 25-year-old man with uveitis, retinal vasoproliferative tumor, cataract, and acute angle-closure glaucoma due to pupillary seclusion, which required laser iridotomies, implantation of an Ahmed valve, phacoemulsification, and LPC. Six months after capsulotomy, he presented a generalized flattening of the anterior chamber (AC) and ocular hypertension, with patent iridotomies. Hyperechoic anterior hyaloid and hypoechoic spaces in the vitreous were seen in ultrasound imaging. The VCB did not respond to pharmacological treatment and was solved immediately after laser hyaloidotomy. CONCLUSION: There are three cases of VCB after LPC described in the literature. Our patient presented a chronic inflammatory process that generated an inflammatory membrane at the level of the anterior hyaloid with adhesion to the ciliary processes, causing posterior misdirection of the aqueous humor, decreased permeability of the anterior hyaloid, and finally, VCB.

CyberKnife Stereotactic Radiotherapy in Secondary Vasoproliferative Tumor of the Retina.

A 16-year-old boy with X-linked retinoschisis was examined for the visual decline on his left eye. Upon examination, a vasopermeable elevated mass lesion was determined at the lower temporal retinal periphery associated with surrounding arc-like hard exudates which is consistent with a vasoactive proliferative tumor of the retina. After discussing the therapeutic options with the patient and his parents, 3 successive dexamethasone implants 6 months apart were administered. As no satisfactory regression was noticed, CyberKnife stereotactic radiosurgery (SRS) was performed. Regression of the exudative tumor was obtained in follow-up examinations at 12 and 24 months after the SRS session and the thickness of the lesion was markedly reduced.

Clinical spectrum, genetic associations and management outcomes of Coats-like exudative retinal vasculopathy in autosomal recessive retinitis pigmentosa.

Background: Coats-like retinal vasculopathy in retinitis pigmentosa (RP) is rare. This study describes its clinical spectrum, management outcomes and genetic associations in patients with autosomal recessive RP (arRP).Materials and methods: Retrospective review of ophthalmic, multimodal imaging, genetic findings and treatment outcomes of arRP patients who developed Coats-like features. Identification of patients included searching a retinal dystrophy registry of 798 patients.Results: Ten eyes of six patients with arRP (4 males, 2 females, mean age 33 years) demonstrated Coats-like features, namely inferotemporal peripheral retinal telangiectasis combined with unilateral inferotemporal vasoproliferative tumor (VPT) in 4 eyes. Exudative retinal detachment (ERD) developed in five eyes of which four had VPT. Ablation of the vasculopathy using retinal laser photocoagulation and/or cryotherapy in eight eyes, allowed ERD and/or lipid exudation to decrease in seven eyes despite incomplete vasculopathy regression. Additional intravitreal triamcinolone acetonide injection in one eye failed to regress the ERD and associated VPT. Observation in one eye caused increased exudation. Six mutations, including three novel mutations, were found in CRB1, CNGB1, RPGR, and TULP1.Conclusions: Coats-like features in arRP range from retinal telangiectasis to VPTs with extensive ERD and occur predominantly in the inferotemporal retinal periphery. In addition to their classic association with CRB1 mutations, other genes are implicated. To the best of our knowledge, this is the first report describing CNGB1 mutations in Coats-like RP. Awareness of the vasculopathy spectrum is important, and timely ablation of the vasculopathy with long-term monitoring is recommended to prevent additional visual loss in RP patients.

Vascular abnormalities in uveitis.

Inflammation can involve several ocular structures, including the sclera, retina, and uvea, and cause vascular changes in these tissues. Although retinal vasculitis is the most common finding associated with uveitis involving the posterior segment, other vascular abnormalities may be seen in the retina. These include capillary nonperfusion and ischemia, vascular occlusions, preretinal neovascularization, microaneurysms and macroaneurysms, and telangiectasia. Moreover, vasoproliferative tumors and subsequent coat-like response can develop secondary to uveitis. Fluorescein angiography is ideal for the investigation of retinal vascular leakage and neovascularization, while optical coherence tomography angiography can provide depth resolved images from the superficial and deep capillary plexus and can demonstrate vascular remodeling. Choroidal vascular abnormalities primarily develop in the choriocapillaris or in the choroidal stroma and can appear as flow void in optical coherence tomography angiography and filling defect and vascular leakage in indocyanine green angiography. Extensive choriocapillaris nonperfusion in the presence of choroidal inflammation can increase the risk of choroidal neovascular membrane development. Iris vascular changes may manifest as dilation of vessels in stroma due to inflammation or rubeosis that is usually from ischemia in retinal periphery secondary to chronic inflammation. More severe forms of scleral inflammation, such as necrotizing scleritis, are associated with vascular occlusion in the deep episcleral plexus, which can lead to necrosis of sclera layer and uveal exposure.

Retinal vasoproliferative tumor in intermediate uveitis: A case report.

INTRODUCTION: Retinal vasoproliférative tumors (VPTs) have been reported as uncommon complications of intermediate Uveitis. CASE DESCRIPTION: A patient consulted for a gradually decreased vision in the right eye (RE). The examination of the RE found a corrected visual acuity at 20/100 with normal eye pressure measured. The anterior segment was deep and quiet and 2+ vitreous haze was found. Funduscopy showed a VPT. Retinal fluorescein angiography of the RE revealed macular cystoid edema also objectified by the Optical Coherence Tomography (OCT). Intermediate Uveitis was considered idiopathic. The patient received a cryoapplication and was put on oral corticosteroid therapy with improvement of visual acuity and a regress of both vitreous inflammation and cystoid macular edema. CONCLUSION: The association of a VPT with intermediate uveitis represents a real diagnostic and therapeutic challenge and imposes rigorous care and monitoring strategy combining internist and ophthalmologist.

Macular Hole Formation in Eye After Cryotherapy and Intravitreal Bevacizumab Treatment for Vasoproliferative Tumor.

PURPOSE: To report a case of a full-thickness macular hole (MH) that developed after cryotherapy and intravitreal bevacizumab injection (IVB) to treat a retinal vasoproliferative tumor (VPT). METHODS: Case report of a man with a retinal VPT. RESULTS: A 64-year-old Japanese man complained of blurred vision in his right eye. At the initial examination, his best-corrected visual acuity (BCVA) was 20/25 in the right eye and 20/20 in the left eye. Ophthalmoscopy showed a VPT in the lower peripheral retina of the right eye. An exudative retinal detachment and hard exudates were seen around the tumor. Cryotherapy and intravitreal injections of bevacizumab (IVB) were performed. Although the exudative changes were reduced, a MH developed two months after the initial IVB treatment. He underwent 25-gauge pars plana vitrectomy, and the MH was closed. His postoperative BCVA was 20/32 and the VPT was inactive. The reduced BCVA was due to damage of the outer retinal layers. CONCLUSION: Our findings indicate that cryotherapy and IVB are effective treatments for VPT although the possibility of developing a MH should be considered.

Ru-106 plaque radiotherapy for vasoproliferative tumors of retina: a 15-year single-center experience.

PURPOSE: This study evaluates the outcomes of ruthenium-106 (Ru-106) plaque brachytherapy for vasoproliferative tumors (VPTs) of the ocular fundus in a single referral ocular oncology center. METHODS: The clinical charts of all patients diagnosed with VPT who underwent plaque radiotherapy from 2002 to 2017 were reviewed. Clinical features, types of treatment, outcomes and complications were evaluated. RESULTS: Of 46 patients with VPT diagnosis in our ocular oncology clinic, 25 (54.34%) cases were treated with Ru-106 plaque brachytherapy. Eleven patients (44%) were male, and the mean age at the time of diagnosis was 40.92 ± 13.11 years. The mean follow-up time was 47.56 ± 36.87 months. Inferotemporal quadrant was the most common site of the tumor (64.00%). The mean delivered apex and scleral dose was 101.56 ± 6.51 and 412.26 ± 113.66 Gray (Gy), respectively. Initial tumor length, width and thickness were 10.26 ± 3.42, 8.05 ± 2.83 and 4.27 ± 1.10 mm, respectively. The mean tumor thickness decreased to 2.60 ± 0.63 mm, postoperatively. Complete resolution of subretinal fluid around the tumor was achieved in 81.80% of cases. Visual acuity was more than 20/400 in 64% of patients before treatment and 60% of patients at last follow-up. CONCLUSION: Our study showed that Ru-106 plaque radiotherapy is an effective and safe method of treatment in VPTs.

Intravitreal dexamethasone implant with retinal photocoagulation for adult-onset Coats' disease.

PURPOSE: To report a case of adult-onset Coats' disease with secondary retinal vasoproliferative tumor managed with dexamethasone intravitreal implant and retinal photocoagulation. METHODS: Case study. RESULTS: A 41-year-old female with counting finger vision was diagnosed with Coats' disease with secondary retinal vasoproliferative tumor in right eye. Fundus examination revealed exudative retinopathy involving posterior pole and a retinal tumor located in the inferotemporal quadrant. Optical coherence tomography scan confirmed massive exudative neurosensory detachment and fundus fluorescein angiography showed areas of telangiectatic vessels with capillary non-perfusion. Intravitreal injection of dexamethasone implant was done initially followed by laser photocoagulation when the detachment resolved. There was significant improvement in patient's visual acuity with no further recurrence of exudation. CONCLUSION: Intravitreal dexamethasone implant Ozurdex(®) (Allergan, Inc., Irvine, Calif., USA) may be an effective initial therapeutic approach for Coats' disease with massive exudation.

Variable Presentations of Peripheral Exudative Hemorrhagic Chorioretinopathy.

Three cases of peripheral exudative hemorrhagic chorioretinopathy (PEHCR), which mimicked other retinal pathologies, were reported. Different preliminary diagnoses were made initially, but thorough examination combined with the appropriate investigations led to the final diagnosis of PEHCR. Despite the rare occurrence of PEHCR, it must be included in the differential diagnosis for peripheral retinal diseases.

The Relationship Between Vasoproliferative Tumor and Uveitis in a Multiple Sclerosis Patient: A Case Report and Review of the Literature

Vasoproliferative retinal tumor (VPRT) is a rare, benign lesion with a variable clinical course depending on the individual. Favorable outcomes are obtained with early diagnosis and treatment of patients with VPRT. In this case report, we present a case of concomitant VPRT and multiple sclerosis along with our management of uveitis and secondary glaucoma that presumably developed following cryotherapy for VPRT.