Peripapillary Choroidal Neovascularization Research Articles

Photodynamic therapy: current role in the treatment of chorioretinal conditions.

Verteporfin photodynamic therapy (vPDT) is a selective vaso-occlusive treatment that targets choroidal vascular abnormalities. It was initially developed to treat neovascular age-related macular degeneration using the 'standard' vPDT protocol (verteporfin 6 mg/m(2), vPDT laser fluence 50 J/cm(2)). vPDT therapy has subsequently evolved as an important treatment modality for a range of other chorioretinal conditions including choroidal haemangioma, central serous chorioretinopathy, polypoidal choroidal vasculopathy, and peripapillary choroidal neovascularisation. Various 'safety-enhanced' vPDT protocols have been devised to optimise treatment outcomes, typically using reduced dose verteporfin (verteporfin 3 mg/m(2)) or reduced fluence vPDT (vPDT laser fluence 25 J/cm(2)). This paper reviews the current role of vPDT therapy in the treatment of chorioretinal conditions.

Causes and Prognosis of Visual Acuity Loss at the Time of Initial Presentation in Idiopathic Intracranial Hypertension.

To determine the etiology and prognosis of visual acuity loss in idiopathic intracranial hypertension (IIH) at presentation and to provide objective measures to predict visual outcome. A retrospective review of 660 patients with IIH (2009-2013) identified 31 patients (4.7%) with 48 eyes having best-corrected visual acuity (BCVA) of 20/25 or worse on initial presentation. Fundus photography, optical coherence tomography (OCT) of the optic disc and macula, and perimetry were used to determine the causes and prognosis of vision loss. Segmentation of the macula OCT was performed using the Iowa Reference Algorithm to determine the retinal ganglion cell-inner plexiform layer complex (GCL-IPL) thickness. Outer retinal changes alone caused decreased BCVA at initial presentation in 22 eyes (46%): subretinal fluid in 16, chorioretinal folds in 5, and peripapillary choroidal neovascularization in 1. The vision loss was reversible except for some eyes with chorioretinal folds. Optic neuropathy alone caused decreased BCVA in 10 eyes (21%) and coexisting outer retinal changes and optic neuropathy caused decreased BCVA in 16 eyes (33%). A GCL-IPL thickness less than or equal to 70 μm at initial presentation or progressive thinning of greater than or equal to 10 μm within 2 to 3 weeks compared with baseline correlated with poor visual outcome. Visual acuity loss in IIH can be caused by both outer retinal changes and optic neuropathy. Vision loss from outer retinal changes is mostly reversible. The outcome of patients with coexisting outer retinal changes and optic neuropathy or optic neuropathy alone depends on the degree of optic neuropathy, which can be predicted by the GCL-IPL thickness.

Optical Coherence Tomography Features of POEMS Syndrome and Castleman Disease-associated Papillopathy

Purpose: To report ocular symptoms and optical coherence tomography (OCT) features of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome.Methods: Case report of two Caucasian patients with POEMS, one of whom also had multicentric Castleman disease.Results: Both patients presented with edema on both optic disks, bilateral macular edema was seen in case 1, and peripapillary choroidal neovascularization was seen on the right side in case 2. OCT confirmed papilledema involving all retinal layers, with additional bilateral peripapillary serous retinal detachment involving the macula in both cases, and peripapillary CNV in case 2. Changes in retinal nerve fiber layer thickness, total peripapillary retinal thickness, and macular detachment were followed with OCT over the course of the disease and after administration of systemic therapy.Conclusion: OCT examination of the optic nerve head and the macula has an important additional role in the diagnosis and follow-up of POEMS syndrome and Castleman disease.

Optic disc drusen and peripapillary subretinal neovascular membranes in children

AbstractPurpose Choroidal neovascular membranes are a rare cause of decreased vision in children with optic disc drusen. It can cause central vision loss by subfoveal progression of the choroidal neovascularization, serous macular detachment, or submacular hemorrhage.Methods We report two cases of optic nerve head drusen in childhood. A 6 year‐old girl and a 12 year‐old boy were both refered to our hospital for optic disc edema with unilateral visual lossResults Fundus examination showed pseudopapillary edema in the two cases. A pediatric check‐up with neurologic and cardiovascular exams and CT scan were performed. B‐Scan ultrasonography showed the presence of the optic nerve head drusen in both eyes but wasn’t identified on CT scan in the two cases. In our cases, optic disc drusen causes peripapillary haemorrhages proceeding from the same mechanism with a juxtapapillary choroidal neovascularization. Because of the location of the hemorrhage only monitoring was recommended. Partial spontaneous visual acuity improvement in 6 months was observed with regression of haemorrhage.Conclusion Optic nerve head drusen are calcified deposits arising from the prelaminar portion of the optic nerve, they have a reported prevalence of 0.4% in children. Although usually benign, their presence can sometimes be associated with serious ocular conditions and cause amblyopia. They have been reported to cause myriad local vascular complications, including central retinal artery and vein occlusion and peripapillary choroidal neovascularization.

Intravitreal Bevacizumab in the Treatment of Peripapillary Choroidal Neovascular Membrane Secondary to Idiopathic Intracranial Hypertension

A 14 year-old Caucasian boy with idiopathic intracranial hypertension (IIH) presented with blurred vision in his left eye. Visual acuity was 20/20, right eye, and 20/80, left eye, and funduscopy revealed bilateral papilledema. In addition, there was peripapillary choroidal neovascular membrane (PPCNVM) in the left eye. Oral acetazolamide improved the symptoms and signs of IIH, but seven weeks later, acuity remained 20/80, left eye, with an increase in subretinal hemorrhage. Two weeks following an intravitreal injection of bevacizumab, visual acuity on the left had improved to 20/30 with resolution of subretinal hemorrhage and fibrosis of PPCNVM. After an additional 2 weeks, visual acuity improved to 20/20, and there has been no sign of recurrence over 3.5 years of follow-up.

Peripapillary choroidal neovascularisation in the context of ocular syphilis is sensitive to combination antibiotic and corticosteroid treatment

Peripapillary choroidal neovascularisation (CNV) in the context of ocular syphilis is exceptional and little is known about its natural history and optimal therapeutic management. We report here a case of right eye peripapillary CNV with subretinal fluid encroaching on the fovea in a patient with mild bilateral ocular inflammation and cystoid macular oedema (CMO) in his contralateral eye. Extensive investigations revealed positive serology for active syphilitic infection. The patient received treatment with intravenous benzylpenicillin according to the algorithm for neurosyphilis complemented with oral corticosteroids as prophylaxis against Jarisch-Herxsheimer reaction for a period of 17 days. On the 15th day, receding of subretinal fluid in his right eye to a small pocket around the optic disc was identified as well as resolution of left eye CMO. We suggest that a course of antibiotic treatment for neurosyphilis with the addition of oral corticosteroids may be an effective therapeutic option for CNV in the context of ocular syphilis and thus more invasive treatment approaches are not warranted.

Histopathology of Peripapillary Choroidal Neovascularization

The different therapeutic responses observed among choroidal neovascularization (CNV) of different etiologies, ages, and locations might be related to the presence of varied mediators. Two surgically removed peripapillary CNVs from two different patients were analyzed. One of the patients had received one intravitreous injection of bevacizumab 3 months earlier. CNV was analyzed using conventional histology and immunohistochemistry. Histological analysis showed intense neovascularization and epithelial and glial components. Vascular endothelial growth factor (VEGF) receptors were found in the endothelial cells and the epithelial cells of the CNV. VEGF was expressed in the patient who had not been previously treated with anti-VEGF. The CNV was deeply infiltrated by glial cells and invaded by microglial cells in one case. VEGF and VEGF receptors may be expressed, suggesting that therapies aiming at VEGF may be efficient only for a subtype of CNV and at a certain time point of their evolution.

Peripapillary choroidal neovascularization in best disease.

Best disease is an autosomal dominant retinal dystrophy with a variable phenotypic expression. Clinically, it is characterized by a vitelliform lesion in the macula because of the deposition of yellow material in a dome-shaped configuration, believed to be lipofuscin that accumulates within and beneath the retinal pigment epithelium. Best disease is occasionally complicated by the development of choroidal neovascularization (CNV), which typically occurs in the macula. We report a case of peripapillary CNV in Best disease. Interventional case report. A 12-year-old boy who was previously diagnosed with Best disease was treated with reduced fluence photodynamic therapy for subfoveal CNV in the right eye. After 2 months, he presented with peripapillary CNV in the left eye, which was treated with repeated sessions of reduced fluence photodynamic therapy. Ophthalmologists must be aware that peripapillary CNV may occasionally complicate Best disease and can be successfully treated with photodynamic therapy.

Eclipse photodynamic therapy for a presumed peripapillary metastatic tumor.

We report a successful use of a modified photodynamic therapy (PDT) termed Eclipse PDT in treating a patient with peripapillary metastatic choroidal tumor. Optic disk protection effect was measured with different colored paper disk attached to the reflecting mirror of the laser machine. Black paper disk was chosen to perform Eclipse PDT because of its maximal blocking effect. A patient with peripapillary metastatic choroidal tumor was treated using this method, and the postoperative outcome was favorable, with improvement in visual acuity and resolution of subretinal fluid. With the new technique, treatment can be modified according to the lesion's shape and location without damaging the optic disk. Eclipse PDT can further extend the indication of PDT treatment to peripapillary choroidal neovascularization as well as choroidal tumors close to the optic disk.

Photodynamic therapy for unilateral idiopathic peripapillary choroidal neovascularization in a child

A 10-year-old girl presented with visual loss in her right eye. Best-corrected visual acuity was 20/50 in the right eye and 20/20 in the left eye. Dilated funduscopic examination revealed a yellowish elevated lesion near the optic disc with macular edema and hemorrhage on the inferotemporal vascular arcade in the right eye. Fluorescein angiography showed a hyperfluorescent lesion consistent with choroidal neovascularization (CNV) and optical coherence tomography (OCT) showed a peripapillary lesion with subretinal fluid elevating the neurosensory retina in the macular area. With a diagnosis of unilateral idiopathic peripapillary CNV, the patient underwent photodynamic therapy (PDT) with verteporfin. At the four month follow-up, visual acuity increased to 20/25 and OCT showed peripapillary scar formation and total resolution of the subretinal fluid. At the one year follow-up, visual acuity and fundus were stable without recurrence of the lesion. PDT for peripapillary CNV in children has not previously been reported. Although it seems to be a favorable treatment option for peripapillary lesions, there is a lack of knowledge about long-term follow-up in pediatric cases.

Regression of Peripapillary Choroidal Neovascularization after Oscillatory Transpupillary Thermotherapy and Anti-VEGF Pharmacotherapy

We prospectively evaluated a new treatment for recalcitrant choroidal neovascularization (CNV) in 4 patients. We used an infrared laser (810 nm) in oscillatory thermotherapy (OTT) mode combined with indocyanine green (ICG) dye, utilizing the beneficial effect of both thermotherapy and photodynamic therapy. We describe preliminary experiences with ICG-assisted OTT (I-OTT) combined with intravitreal bevacizumab/dexamethasone for refractory peripapillary CNV resistant to standard therapy. Clinical examination, funduscopy, fluorescein angiography, and optical coherence tomography were performed at baseline and postoperatively. Infrared laser spot size was approximately one-half the lesion size (oscillation 2-3 Hz). Intravitreal injections of bevacizumab (1.25 mg) and dexamethasone (1000 µg) were done during the same visit. Mean follow-up was 12.5 months (range 5-17). Mean energy level was 325 mW (range 200-500) in oscillatory mode (2-3 Hz/sec) pre- and post-ICG infusion. Indocyanine green dose was approximately 1 mg/kg (75 mg/patient). All patients had a single treatment. Mean visual acuity improved in 1 patient from 20/60 to 20/30 and remained the same in the other 3 (20/20, 20/40, and 20/400). At final examination, there was no evidence of clinical or angiographic activity of CNV. Indocyanine green-assisted OTT has the potential to treat CNV in wet age-related macular degeneration. It may reduce thermal side effects and eliminate or reduce the need for frequent intravitreal treatment. We postulate that I-OTT has a synergistic effect of thermal energy combined with a weak photosensitizer (ICG) applied in an individualized manner, which minimizes thermal damage to retinal and choroidal tissue. Additional anti-vascular endothelial growth factor pharmacotherapy enhances the effect of I-OTT on abnormal new vessels.

Intravitreal bevacizumab treatment of bilateral peripapillary choroidal neovascularization from optic nerve head drusen

Choroidal neovascular membranes are a rare cause of decreased vision in children with optic nerve head drusen. We present a case of bilateral choroidal neovascular membranes associated with optic nerve head drusen in a 5-year-old boy who was successfully treated with a combination of focal laser photocoagulation and intravitreal bevacizumab.

Effect of Intravitreal Ranibizumab in the Treatment of Peripapillary Choroidal Neovascularisation

Intravitreal ranibizumab therapy is widely used in treatment of subfoveal choroidal neovascularisation (CNV) in age-related macular degeneration. We wanted to study the effect of intravitreal ranibizumab therapy in peripapillary CNV. A prospective recording of treatment outcomes in twelve eyes (12 patients) with peripapillary CNV with intravitreal injections of ranibizumab was performed. The patients received a series of 3 injections 4–6 weeks apart, and then a new ophthalmic examination was made including OCT and further therapy was given if the peripapillary CNV was still active. Nine patients had idiopathic peripapillary CNV, and in 3 patients it was associated to age-related macular degeneration. Followup had to be at least 6 months. The mean follow-up time was 15.9 (range 9–27) months and the mean number of injections 6.2 (3–10). In 10 patients treatment had resulted in an inactivation of the peripapillary CNV, but 3 of them had reactivation, while 2 patients had no inactivation. Currently, 5 patients are continuous to receive treatment. VA improved in 10 patients. Intravitreal ranibizumab therapy appears to be effective in patients with peripapillary CNV, but in some cases there is repeated reactivation or continuous activity of the peripapillary CNV.

Peripapillary choroidal neovascularization in congenital retinoschisis.

To report a case of peripapillary choroidal neovascularization (CNV) associated with congenital retinoschisis. Case report. A 5-year-old white boy presented with regressed peripapillary CNV in the right eye along with peripheral retinoschisis in the same eye. The left eye presented with macular, lamellar, and peripheral retinoschisis changes. Optical coherence tomography and fluorescein angiography confirmed the presence of regressed peripapillary CNV in the right eye and also revealed retinal nonperfusion within the peripheral retinoschisis cavity in the right eye. The authors report a case of peripapillary CNV associated with congenital retinoschisis. The authors hypothesize that peripheral retinal nonperfusion in the right eye may have contributed to the development of CNV in that eye.

Peripapillary choroidal neovascularization associated with kenny syndrome.

To report a case of Kenny-Caffey syndrome with peripapillary choroidal neovascularization. An observational case report with clinical findings demonstrated by wide-field retinal photographic and angiographic evaluation of a patient with Kenny-Caffey syndrome. Wide-field retinal photography and fluorescein angiography showed characteristic findings seen in Kenny-Caffey syndrome (crowded optic nerves, retinal vascular tortuosity). In addition, the previously undocumented finding of peripapillary choroidal neovascularization is demonstrated. The development of peripapillary choroidal neovascularization can occur in patients with Kenny-Caffey syndrome. The development of this finding may be secondary to abnormalities of the optic nerve inherent in this rare disorder.

Treatment of Peripapillary Choroidal Neovascularization with Intravitreal Bevacizumab

Peripapillary choroidal neovascularization (CNV) is an uncommon condition and often shows a growth tendency towards the fovea during spontaneous progression that threatens visual acuity. Treatment of peripapillary CNV is difficult. The authors report results of intravitreal bevacizumab therapy for peripapillary CNV. Four patients with CNV located in the temporal or superior peripapillary area received intravitreal bevacizumab injections. Ophthalmologic examinations including OCT were performed at baseline and at 6-week intervals. Fluorescein angiography was performed at baseline and depending on clinical and OCT findings. The mean follow-up was 34+/-20 (22-69) weeks. The patients received an average of 3.5+/-3.1 (1-8) injections. In all patients fluorescein angiography showed inactivation of peripapillary CNV. No further increase in size was observed in any of the patients. The OCT showed a decrease of intraretinal and subretinal fluid. No intraocular or systemic side effects were observed. In this series of patients, intravitreal bevacizumab appears to be efficacious. A progression of peripapillary CNV could be prevented in all patients and the lesion was successfully inactivated. Anti-VEGF treatment with bevacizumab represents a promising therapy option for peripapillary CNV.

Intérêt des injections intravitréennes de ranibizumab dans le traitement des néovascularisations choroïdiennes péripapillaires étendues

To evaluate the safety and explore the efficacy of intravitreal injection of ranibizumab 0.5mg in treatment of large peripapillary choroidal neovascularization. This single-center, prospective, interventional, noncomparative, nonrandomized clinical case series study included six consecutive patients with large or recurrent peripapillary choroidal neovascularization. Ranibizumab 0.5mg was administered at baseline, and then monthly following the development of hemorrhage or subretinal fluid associated with visual symptoms. The response to treatment was assessed in terms of changes in lesion characteristics as measured by FA and change in visual acuity (VA). Six patients (seven eyes with peripapillary choroidal neovascularization) were enrolled. three of the seven eyes had choroidal neovascularization associated with angioid streaks, two had idiopathic neovascularization, and the last eye had peripapillary neovascular membrane associated with age-related macular degeneration. The median follow-up was 12 months (+/-3 months). At 1 year, the best-corrected visual acuity improved from +0.74 log Mar to +0.45 log Mar (p=0.0431), with resolution of intraretinal fluid. On average, four intravitreal injections of ranibizumab (0.5mg) were required during this year. Laser photocoagulation treatment, photodynamic therapy, or subretinal surgery are limited by the high recurrence rate, the difficulty in assessing the true extent of the lesion, and the difficulty in improving visual function. Intravitreal ranibizumab injections appeared to be a good therapeutic option for patients with peripapillary CNV in the short term. Long-term follow-up of these patients will aid in assessing the sustained effect of the drug.

Extracción quirúrgica de la neovascularización coroidea peripapilar en pacientes jóvenes

To analyze anatomical and visual outcomes after subretinal surgery for peripapillary choroidal neovascularization (CNV) not associated with age-related macular degeneration (AMD). Five eyes from five patients with peripapillary CNV who had been treated by subretinal surgery were retrospectively analyzed. The patients averaged 32 years of age (range, 11 to 49, SD 14.5 years) and mean follow-up was 31.4 months (range, 8 to 48, SD 20.8 months). Best corrected visual acuity was 0.05 (range, 0.001 to 0.16, SD 0.07) before surgery and 0.64 (range, 0.2 to 0.9, SD 0.26) after surgery (p=0.005; Student's t test for paired data). Neither intraoperative nor postoperative incidences occurred. Surgical removal may be considered one of the therapeutic options to treat peripapillary CNV not related to AMD.

Regression of a Peripapillary Neovascular Membrane Following Lumbar Peritoneal Shunting in a Case of Idiopathic Intracranial Hypertension

This report concerns a case of Idiopathic Intracranial Hypertension (IIH) in a man treated with lumbar peritoneal shunting. Despite resolution of papilloedema a central scotoma remained and evaluation in the retinal department revealed peripapillary choroidal neovascularisation (CNV). During 10 months follow up fundus appearance and Optical Coherence Tomographic (OCT) findings improved although his visual acuity did not change. The case is presented as an example of improvement of a neovascular membrane following treatment of raised intracranial pressure.

Intravitreal Bevacizumab in Inflammatory Ocular Neovascularization

To assess the role of bevacizumab in inflammatory ocular neovascularization. Retrospective, multicenter, consecutive case series of inflammatory ocular neovascularization. Patients with inflammatory ocular neovascularization of varying causes for whom standard therapy failed were treated with intravitreal injection of bevacizumab. Main outcome measures included improvement of best-corrected visual acuity (BCVA) expressed in logarithm of minimum angle of resolution units, response of inflammatory ocular neovascularization by funduscopy and angiography, and decrease in central foveal thickness as measured by optical coherence tomography at the three-month follow-up. At the three-month follow-up, 84 eyes of 79 patients had been treated with a mean of 1.3 injections (range, one to three). Thirty-four eyes showed juxtafoveal choroidal neovascularization (CNV), 34 eyes showed subfoveal CNV, eight eyes showed peripapillary CNV, and 11 eyes showed neovascularization of the disc (NVD) or neovascularization elsewhere (NVE). BCVA improved 2.4 lines from 0.68 (6/28 or 20/94) to 0.44 (6/17 or 20/55) (P < .001). BCVA improved by one to three lines in 34.5% of the eyes, by four to six lines in 16.7% of the eyes, and by more than six lines in 14.2% of the eyes. Function was unchanged in 23.8% of the eyes. BCVA worsened in 10.7% (zero to three lines in 7.1%, more than four lines in 3.6%). Central foveal thickness decreased from baseline 346 to 252 microm (P < .001). For CNV, 32 eyes (43.2%) had complete regression after the injection, 27 (36.5%) had partial regression, five (6.8%) had no response, and 10 eyes (13.5%) were not evaluated by the contributors. For NVD or NVE, seven eyes (63.6%) had complete regression of new vessels and four eyes (36.4%) had partial regression after the injection. Intravitreal bevacizumab led to short-term significant visual improvement and regression of inflammatory ocular neovascularization in a wide variety of inflammatory ocular diseases.