Patterns of cytomegalovirus retinitis at a tertiary referral center in Turkey.

PURPOSE: To analyze predisposing conditions in Turkish patients with CMV retinitis and to compare HIV-positive and HIV-negative patients. METHODS: We reviewed medical charts and ocular images of 41 patients with CMV retinitis diagnosed between 1996 and 2019. RESULTS: Eleven patients (27%) had HIV infection and 30 were immunocompromised from diverse causes. Initial visual acuity, type, zone, and extent of CMV retinitis, and response to anti-CMV treatment were not significantly different between the two groups. Vitreous haze and panretinal occlusive vasculopathy were the presenting features only in non-HIV patients, seen in 34% and 16% of eyes, respectively. Although not statistically significant, recurrent CMV retinitis was more common in non-HIV patients (17.4% vs. 4.3%/eye-year) and immune recovery uveitis was more common in HIV patients (43% vs. 26%/eye-year). Visual outcomes were similar. Final visual acuity of 1 logMAR or worse was significantly associated with the recurrence of CMV retinitis (odds ratio 9.67; p = 0.01) and also with the occurrence of immune recovery uveitis (odds ratio 4.31; p = 0.058). CONCLUSIONS: Diverse immunocompromising conditions are more commonly associated with CMV retinitis than HIV infection in Turkish patients. Intraocular inflammation was more commonly associated with active retinitis in non-HIV patients and immune recovery uveitis was more common in HIV patients.

Evaluation of emulsified silicone oil with spectral domain-optical coherence tomography and fluorescein angiography.

BACKGROUND: To report emulsified particles and related complications after removal of silicone oil (SO) by spectral domain-optical coherence tomography (SD-OCT) and fluorescein angiography (FA). METHODS: A prospective, single-center investigation was performed using SD-OCT, FA and flare cell photometry measurements to evaluate in a cohort of eyes with emulsified silicone particles after removal of long-term SO tamponade. RESULTS: Nineteen eyes of the 19 patients with emulsified silicone particles detected in ophthalmoscopic exam were included in the study: 13 (68%) were male, and 6 (31.6%) were female, and their mean age was 52.75 ± 16.06 years. Preoperative diagnoses were rhegmatogenous retinal detachment in all eyes. The mean duration of the silicone oil's presence in the eye was found as 12.63 ± 4.87 (7-26) months. SD-OCT examination postoperatively showed small, round and hyper-reflective structures compatible with emulsified silicone particles on preretinal surface in 7 (36.8%) eyes, intraretinally in 6 (31.6%) eyes and in the vitreous in 12 (63%) eyes. FA imaging demonstrated pearl-like hyper-fluorescence at early and late phases on the large blood vessels in 9 (47.4%) eyes. Late phase hyper-fluorescence due to leakage on the macula was observed in 17 (89.5%) eyes. Diffuse peripheral leakage and hyper-fluorescence was detected in 11 (57.9%) eyes. Flare levels were higher in eyes with emulsified SO compared to unaffected fellow eyes (p < 0.05). CONCLUSIONS: Emulsified silicone particles that remain in the eye may lead to retinal complications and inflammation in addition to their known complications such as keratopathy and glaucoma. The particles that lead to inflammation and presence of inflammation may be examined by imaging methods such as SD-OCT and FA. Removing the SO before emulsification would be suitable for avoiding such complications.

Acute macular neuroretinopathy misdiagnosed as optic neuritis.

This study aims to report a case of acute macular neuroretinopathy (AMN) misdiagnosed as optic neuritis. The methods include review of the clinical, photographic, angiographic, visual field, infrared imaging, and optical coherence tomography records of a patient with AMN. A 30-year-old female who was initially diagnosed with optic neuritis and at first was treated with systemic corticosteroid because of this misdiagnosis. She was admitted with no decrease in her complaint of central scotoma in both eyes. Her visual acuities were 20/20 and fundoscopy showed reddish brownish pigment changes in the macula of both eyes. Infrared (IR) imaging disclosed bilateral hyporeflective lesions, optical coherence tomography revealed a disrupted inner segment-outer segment junction of the right eye, and visual field assessment showed central scotomas in both eyes. Three years later, fundus examination of the hyporeflective lesions by IR imaging revealed that the lesions had decreased and were only slightly perceptible. This rare case is presented to focus the attention of clinicians and to emphasize the importance of careful ophthalmic examination and imaging techniques.

Optic neuritis and rapidly progressive necrotizing retinitis as the initial signs of subacute sclerosing panencephalitis: a case report with clinical and histopathologic findings.

We report a case of subacute sclerosing panencephalitis (SSPE) presenting first with optic neuritis and rapidly progressive necrotizing retinitis at the posterior pole. We reviewed the clinical, laboratory, photographic, angiographic, and histopathologic records of a patient with SSPE. A 15-year-old girl was referred after rapid loss of vision due to optic neuritis and macular necrosis in the right eye. She had a history of cardiac valve surgery, but had no systemic symptoms and extensive work-up was unrewarding. Contralateral involvement with rapidly progressive optic neuritis and macular necrotizing retinitis prompted retinochoroidal biopsy of the right eye, which revealed necrosis of inner retinal layers and perivascular lymphoplasmocytic infiltration with intact choroid and outer retina without any findings of inclusion bodies, microorganisms, or atypical cells. The diagnosis was based on histopathologic findings consistent with SSPE, and detection of elevated measles antibody titers in cerebrospinal fluid and serum. It was further confirmed by development of typical electroencephalography pattern at 6 months and neurological symptoms at 4-year follow-up. Clinicians need to be aware that optic neuritis and necrotizing retinitis at the posterior pole may be the presenting features of SSPE.

Expert recommendations for the management of diabetic macular edema with intravitreal dexamethasone implant: A Turkish Delphi study.

PURPOSE: To provide consensus on the clinical use of intravitreal dexamethasone (DEX) implant for the treatment of diabetic macular edema (DME) in Turkey. METHODS: A panel of 22 retina experts prepared 77 statements of recommendation, and 80 retinal specialists practicing in Turkey were chosen to vote either in support or against each one. A Delphi-based method was used through which the ophthalmologists were able to view all of the results anonymously after two rounds and modify their subsequent answers. The survey was conducted via a mini website, and statements without consensus were resent to the specialists with the latest vote results a week later. RESULTS: A total of 72 ophthalmologists answered the first and second round questions. After the first stage, consensus was achieved on 55 of the statements, leaving 22 without agreement. After the second stage, consensus was reached on 11 of the remaining statements. Strong consensus was achieved on statements regarding the etiopathogenesis of DME and the first-line indications and safety of the DEX implant procedure. The panel recommended the use of DEX implant for patients with an arterial thromboembolic event in the last three months and also agreed that pro re nata DEX implant treatment not only provides better outcomes for DME patients but also reduces the treatment burden for those who could not receive an adequate number of anti-vascular endothelial growth factor (VEGF) injections. CONCLUSION: This study provides clinical consensus and recommendations about the use of DEX implant in the clinical practice of DME management in Turkey.

How challenging can it be to treat Behçet uveitis?

A 20-year-old male Behçet uveitis (BU) patient presented with visual acuities (VAs) of hand movement in OD and counting fingers at 1 m in OS following treatment with corticosteroid monotherapy elsewhere. He had active intraocular inflammation OU along with macular hole and retinal detachment in OS. Infliximab (IFX) was started and vitreoretinal surgery was performed. He had infusion reaction with IFX, hepatotoxicity and depression with interferon, and resistance to adalimumab and tocilizumab therapies. Cytomegalovirus retinitis developed in OD following intravitreal dexamethasone implant and endophthalmitis developed in OS. At the 33rd month of follow-up, the patient was in clinical remission; however, there was persistent angiographic inflammation under certolizumab pegol, cyclosporine, mycophenolate mofetil, and low-dose prednisolone treatment. The left eye was phthisical and VA was 0.4 in OD. Immunomodulatory treatment is given based on the severity of inflammation in BU and needs to be closely monitored for efficacy and adverse effects.

Preoperative and Postoperative Factors Affecting Functional Success in Anatomically Successful Retinal Detachment Surgery.

PURPOSE: To investigate preoperative and postoperative factors affecting functional success in anatomically successful retinal detachment surgery. METHODS: Seventy-five eyes of 75 patients with rhegmatogenous retinal detachment who underwent anatomically successful surgery from 2014 to 2019 with more than 1 year follow-up were included in the study. Demographic characteristics, ocular examination findings, preoperative and postoperative spectral domain optic coherence tomography images were retrospectively evaluated. RESULTS: The mean age was 53.9 ± 17 years (range, 11-85 years). The mean follow-up period was 36.7 ± 16 months (range, 14-72 months). The mean best-corrected visual acuity of the patients before surgery was 1.35 ± 1.24 and at postoperative 12 months was 0.66 ± 0.5 logarithm of the minimum angle of resolution. Patients who were operated in 7 days of visual symptoms onset were found to have better visual acuity at the first and subsequent postoperative exams. Preoperative proliferative vitreoretinopathy, vitreous hemorrhage, and extent of retinal detachment were found to have a negative effect on functional success at 12 months. Regeneration of the outer retinal layers had a positive effect on visual acuity at 12 months but did not significantly increase visual acuity after 12 months. Multivariate analysis showed that preoperative external limiting membrane integrity was significantly associated with better functional outcomes. CONCLUSIONS: Early intervention, presence of preoperative external limiting membrane integrity, and restoration of postoperative outer retinal layers positively affected functional success. The presence of preoperative vitreous hemorrhage, preoperative proliferative vitreoretinopathy, and the extent of retinal detachment had a negative effect on prognosis at 12 months.

Interferon Alpha-2a for the Treatment of Cystoid Macular Edema Secondary to Acute Retinal Necrosis.

PURPOSE: To report the results of interferon (IFN) α-2a treatment in patients with cystoid macular edema (CME) secondary to acute retinal necrosis (ARN). METHODS: We reviewed the records of seven patients (eight eyes) who received IFNα-2a for post-ARN CME. The initial dose of IFNα-2a was 3 MIU/day and it could be tapered down to 3 MIU twice a week. Efficacy was assessed by central macular thickness (CMT) on spectral-domain optical coherence tomography and visual acuity. RESULTS: Age range of seven patients (four men, three women) was 36-74 years. Mean CMT decreased from 477.9 ± 167.5 µm to 367.3 ± 120.5 µm at first week, and vision improved up to five lines in five eyes. CME relapsed after cessation of IFNα-2a in all and improved following reinstitution of treatment. Treatment was discontinued in one patient because of depression. Three patients electively discontinued treatment due to poor tolerability or lack of functional improvement. CONCLUSION: IFNα-2a is an effective therapeutic option for post-ARN CME, though side effects such as fatigue, elevated liver enzymes, neutropenia, and depression may limit tolerability. Lower initial doses may be a better tolerated.

Association Between Prognosis of Acute Retinal Necrosis and Retinal Involvement.

Objectives: The aims of this study were to describe the clinical presentation and treatment modalities of acute retinal necrosis (ARN) and to evaluate complications and clinical outcomes according to the extent of retinal involvement at initial presentation. Materials and Methods: The medical records of 52 patients diagnosed with ARN were reviewed and 48 were included in the study. Patients were categorized into two groups according to the extent of retinitis at presentation: retinal involvement of 1-2 quadrants (Group A) or 3-4 quadrants (Group B). Results: The mean age of the 14 women and 34 men at presentation was 51.3±13.6 years (range: 27-78). There were 40 unilateral and 8 bilateral cases. There were 11 eyes (19.6%) in Group A and 45 eyes (80.4%) in Group B. Eleven patients (22.9%) had a history of herpes simplex virus/varicella-zoster virus infection. One patient in Group A and 11 patients in Group B had received local or systemic corticosteroid therapy without concomitant antiviral treatment before referral. The median follow-up period was 29 months (range: 1-209) in Group A and 8.5 months (range: 0.75-209) in Group B. Mean visual acuity (VA) at presentation was 0.42±0.55 LogMAR (range: 0-2.0) in Group A and 1.28±0.95 LogMAR (range: 0-2.9) in Group B (p<0.05). The presence of endothelial keratic precipitates at presentation was significantly different between two groups (p=0.021). Retinal detachment (RD) occurred in 1 eye (9.1%) in Group A and 30 eyes (66.7%) in Group B (p<0.001). Optic disc pallor was seen in 36.4% (4/11) of eyes in Group A and 71.1% (32/45) of eyes in Group B (p=0.033). Other ocular complications were not significantly different between two groups. Mean final visual acuity was 0.29±0.41 LogMAR in Group A and 1.61±0.90 LogMAR in Group B (p<0.05). Conclusion: The extent of retinal involvement at presentation affects visual outcomes and this shows the importance of early diagnosis and early initiation of antiviral treatment.

Real-life experience of ranibizumab therapy for neovascular age-related macular degeneration from Turkey.

AIM: To report the real-life experience and clinical results of intravitreal ranibizumab injections to neovascular age-related macular degeneration (nAMD) in a single institution in Turkey. METHODS: A total of 101 eyes of 89 patients with nAMD treated with intravitreal ranibizumab injection, followed up for at least 24mo between 2009 and June 2014, which were evaluated retrospectively. A pro re nata (PRN) treatment protocol was performed after the patients had received three, monthly loading injections. Best corrected visual acuity (BCVA) and central macular thickness measurements were evaluated at baseline and 3, 6, 12, 18, and 24mo. Number of injections and visits were also recorded. RESULTS: Of the 89 patients, 34 (38.2%) were male and 55 (61.8%) were female and the mean age was 74.0±9.5 (52-91)y. The mean follow-up period was 24.82±4.4 (24-29)mo. Mean number of visits was 8.4±1.12 (7-12) in the first year and 6.6±1.33 (4-12) in the second year. The mean number of injections was 5.8±1.6 (3-10) and 4.2±2.2 (0-9) in the first and second year, respectively. The mean BCVA was 59±15.8 letters at baseline by the Early Treatment Diabetic Retinopathy Study (ETDRS) chart. The mean BCVA at 3, 12, and 24mo was 70.3±15.9, 67.9±14.3 and 67.3±16.9 letters, respectively. Improvement in visual acuity for each of the visits from baseline was found to be statistically significant (P<0.01). Visual acuity in 9 eyes at month 3, 7 eyes at month 12, and 13 eyes at month 24 did not change. The mean central macular thickness (CMT) was 437.99±164.78 µm at baseline. The mean CMT was 348.05±138.47 µm, 349.27±139.79 µm, and 344.13±146.30 µm at months 3, 12, and 24, respectively. The decrease in CMT for each of the visits from baseline was found to be statistically significant (P<0.01). CONCLUSION: Anatomical and functional achievement are obtained in our study, but the mean number of injections and visits are found to be lower than the findings reported in randomized controlled clinical trials in the literature. However, the mean number of injections and visits in our study are compatible with the findings reported in real-life experience studies in the literature.

All Types of Age-related Macular Degeneration in One Patient.

Herein, we describe a neovascular age-related macular degeneration patient with retinal angiomatous proliferation (RAP) and polypoidal choroidal vasculopathy (PCV) coexisting in the same eye at the time of diagnosis. A 55-year-old woman presented with a history of decreased vision in her left eye. Fundoscopy, fluorescein and indocyanine green angiography, and optical coherence tomography imaging revealed RAP and PCV lesions in her left eye at first diagnosis. The patient received intravitreal ranibizumab therapy but developed tachyphylaxis after the first dose despite having three monthly doses. Switching to intravitreal aflibercept injection in our case resulted in anatomic and functional improvement.

Atypical Central Serous Chorioretinopathy.

Bullous central serous chorioretinopathy (CSCR) is a rare variant of CSCR characterized by severe serous retinal detachment which especially involves the inferior quadrants. Corticosteroid therapy administered for systemic or ocular misdiagnoses may induce and exacerbate CSCR. The purpose of this study was to report diagnosis and treatment results of an unusual case of bullous CSCR induced by systemic and periocular corticosteroid therapy received at another medical center due to a misdiagnosis of Vogt-Koyanagi-Harada disease.

Endogenous Brucella endophthalmitis: A case report.

Brucellosis may be associated with a wide range of ophthalmic manifestations including endophthalmitis, which is a sight-threatening condition that needs to be rapidly recognized and treated to avoid permanent visual loss. A 26-year-old female with a 6-month history of vision loss in the left eye was treated with high dose systemic corticosteroids and azathioprine with an initial misdiagnosis elsewhere. A dense vitreous haze with opacities at the posterior hyaloid and a wide area of retinochoroiditis led to the diagnosis of endogenous endophthalmitis at presentation to us. The vitreous sample and blood cultures demonstrated growth of Brucella melitensis. She received 6 months of systemic antibiotherapy, which resulted in resolution of inflammation; however, visual acuity remained poor due to irreversible damage. Infectious etiology, including brucellosis in endemic countries, has to be considered in the differential diagnosis before administering immunomodulatory therapy in patients with panuveitis of unknown origin.

Neovascularization of the optic disc in Behçet's disease.

PURPOSE: To investigate the mechanisms involved in the development of neovascularization of the optic disc (NVD) in Behçet's disease and to evaluate the effects of medical and laser treatments. METHODS: This is a retrospective study of 26 consecutive Behçet's patients (38 eyes) with NVD who presented between 1990 and 2004 at our university hospital. Information on age at presentation, sex, disease duration, laterality of NVD, ocular findings, fluorescein angiographic findings, visual acuity, medical treatment, laser photocoagulation, surgical procedures, and the follow-up period was collected. Eyes with diffuse capillary leakage on fluorescein angiography were defined as having inflammation-induced NVD, and eyes with extensive retinal capillary nonperfusion were defined as having ischemia-induced NVD. RESULTS: Eighteen patients were male, and eight were female. Mean age at presentation was 25.4 +/- 4.9 years. Median disease duration was 5.5 months. Median follow-up was 24 months. Twelve patients had bilateral NVD; 14 had unilateral NVD. Inflammation-induced NVD was seen in 87% of the eyes, and ischemia-induced NVD in 13%. Initial treatment with high-dose corticosteroids combined with conventional immunosuppressive agents was effective in 45% of the eyes with inflammation-induced NVD. Retinal laser photocoagulation was effective in three of five eyes with ischemia-induced NVD. Treatment with interferon alpha-2a resulted in resolution of NVD in all seven patients who received this agent for inflammation- or ischemia-induced persistent NVD. Final visual acuity was less than 0.1 in 24% of the eyes. CONCLUSIONS: Inflammatory mechanisms seem to predominate in the pathogenesis of NVD in Behçet's disease. The results of this study suggest that the response to intensive anti-inflammatory and conventional immunosuppressive treatment is not satisfactory; retinal laser photocoagulation may be ineffective even in eyes with retinal ischemia, but interferon alpha-2a seems to be effective for the treatment of Behçet's patients with NVD.

Focal Choroidal Excavation.

Focal choroidal excavation is a choroidal pit that can be detected by optical coherence tomography. Central serous chorioretinopathy, choroidal neovascularization and polypoidal choroidal vasculopathy are pathologies associated with focal choroidal excavation. In this article, we present the follow-up and treatment outcomes of three eyes of two patients with focal choroidal excavation.

Silent polypoidal choroidal vasculopathy in a patient with angioid streaks.

We present a case of silent polypoidal choroidal vasculopathy (PCV) in a patient with angioid streaks. PCV was detected during a routine ophthalmic examination and confirmed by fluorescein angiography, indocyanine green angiography, and optical coherence tomography. After 2 years of follow-up, the PCV remained silent without any complications. We report this rare coexistence and review literature on this topic.

Multimodal imaging of membranoproliferative glomerulonephritis type II.

To report the fundus findings on multimodal imaging of a membranoproliferative glomerulonephritis type II (MPGNII) patient. A 41-year-old woman had a medical history of renal transplantation for MPGNII 5 years prior to presentation to our clinic for ophthalmic examination. Fundus examination showed macular retinal pigment epithelial changes and large peripapillary atrophy in the right eye, and yellowish bilateral drusen-like deposits peripherally. Fundus fluorescein angiography and indocyanine green angiography revealed multifocal hyperfluorescence in the peripheral retina with accompanying deposits. A hyperautofluorescence pattern was seen in the fundus autofluorescence (FAF) images of the deposits. Spectral domain optical coherence tomography (SD-OCT) scans of the drusen-like material showed retinal pigment epithelium (RPE) elevations with moderate hyperreflectivity underneath. Multiple imaging modalities of the posterior segment can provide detailed information about the ocular involvement of MPGNII and help us to confirm the diagnosis and to follow the lesions.

Peripapillary Retinoschisis in Glaucoma Patients.

Purpose. To investigate peripapillary retinoschisis and its effect on retinal nerve fiber layer (RNFL) thickness measurements by using spectral-domain optical coherence tomography (SD-OCT) in glaucomatous eyes. Methods. Circumpapillary RNFL (cpRNFL) B-scan images of 940 glaucoma patients (Group 1) and 801 glaucoma-suspect patients (Group 2) obtained by SD-OCT were reviewed. The structural and clinical characteristics of the retinoschisis were investigated. The RNFL thickness measurements taken at the time of retinoschisis diagnosis and at the follow-up visits were also compared. Results. Twenty-nine retinoschisis areas were found in 26 of the 940 glaucoma patients (3.1%) in Group 1 and seven areas were found in 801 patients (0.87%) in Group 2. In glaucomatous eyes, the retinoschisis was attached to the optic disc and overlapped with the RNFL defect. At the time of retinoschisis, the RNFL thickness was statistically greater in the inferior temporal quadrant when compared with the follow-up scans (p < 0.001). No macular involvement or retinal detachment was observed. Conclusion. The present study investigated 33 peripapillary retinoschisis patients. Increase in RNFL thickness measurements was observed at the time of retinoschisis. It is important to examine the cpRNFL B-scan images of glaucoma patients so that the RNFL thickness is not overestimated.

Clinical Features and Course of Patients with Peripheral Exudative Hemorrhagic Chorioretinopathy.

OBJECTIVES: To evaluate the clinical characteristics of patients who were followed in our clinic with the diagnosis of peripheral exudative hemorrhagic chorioretinopathy (PEHC). MATERIALS AND METHODS: Medical records of 12 patients who were diagnosed with PEHC in Istanbul University Istanbul Faculty of Medicine, Department of Ophthalmology between July 2006 and June 2014 were reviewed retrospectively. RESULTS: This study included 21 eyes of 12 patients. Four (33.3%) of the patients were male and 8 (66.7%) were female and ages ranged between 73 and 89 years. Eight (66.7%) of the patients were referred to us with the diagnosis of choroidal mass. Unilateral involvement was found in 3 and bilateral involvement in 9 patients. Temporal quadrants were involved in all eyes. Fifteen eyes (71.4%) had subretinal hemorrhage and hemorrhagic/serous retinal pigment epithelial detachment, 11 (52.4%) had lipid exudation, 5 (23.8%) had chronic retinal pigment epithelium alterations, 2 (9.5%) had subretinal fibrosis and 1 (4.8%) had vitreous hemorrhage. PEHC lesions were accompanied by drusen in 11 eyes (52.4%), geographic atrophy in 2 eyes (9.5%), and choroidal neovascularization scar in 2 eyes (9.5%). Treatment was done in both eyes of a patient for lesions which threatened the macula, in a patient with bilateral macular edema and in a patient with vitreous hemorrhage. The remaining eyes were followed-up without any treatment because the lesions did not threaten the macula and they showed no progression during follow-up. CONCLUSION: PEHC is a degenerative disease of peripheral retina that is seen in older patients, and signs of age-related macular degeneration (AMD) may accompany this pathology. Especially in patients with AMD findings, the peripheral retina must be evaluated carefully for existing PEHC lesions.

Evaluation of Follow-Up and Treatment Results in Coats' Disease.

OBJECTIVES: The aim of this study was to evaluate the clinical features, follow-up and treatment results of patients diagnosed with Coats' disease. MATERIALS AND METHODS: Medical records of 27 patients diagnosed with Coats' disease in our clinic were reviewed retrospectively. All patients underwent complete ophthalmological examination and fundus photography was taken. Disease stage and treatment methods used were recorded. RESULTS: Twenty-seven eyes of 27 patients were included in the study. Mean age was 9.03 years; 21 patients were male and 6 were female. Three patients were older than 18 years old. Based on the Shields classification, 1 (3.7%) eye was stage 2A, 4 (14.8%) eyes were stage 2B, 6 (22.2%) were stage 3A1, 3 (11.1%) were stage 3A2, 1 (3.7%) was stage 3B, 4 (14.8%) were stage 4 and 8 (29.6%) were stage 5. Fourteen patients underwent treatment, 12 of whom had combined therapy. The most common treatment modalities were laser photocoagulation and cryotherapy. Encircling band was done in one patient and pars plana vitrectomy in 3 patients. Enucleation was done in 5 patients. CONCLUSION: Coats' disease is a chronic disease and main goal of treatment is to eliminate the vascular anomalies and their complications using repetitive combination therapies. Treatment in the early stages can lead to functional success, and in advanced stages can result in a salvageable eye.