Optic Disc Pit Maculopathy - Case Series, Clinical Approach, and Management.

PURPOSE: The purpose of this study was to analyze the diagnostic and therapeutic approach of five cases with optic disc pit (ODP) maculopathy. MATERIALS AND METHODS: This was a retrospective study of five patients diagnosed with ODP maculopathy. Four of these cases had unilateral involvement, whereas one case had bilateral findings. The medical notes of these individuals were reviewed in order to record the presenting symptoms, clinical signs, visual acuity (VA), imaging, management, and the final visual outcome on their last follow-up appointment. RESULTS: The first patient (53-year-old female) underwent a left pars plana vitrectomy (PPV) combined with inner retinal fenestration, endolaser, and perfluoropropane (C3F8) gas tamponade and her VA improved from 6/24 to 6/9 Snellen. A focal retinal laser treatment was carried out on our second patient leading to decrease of the subretinal fluid but had a poor visual outcome due to the underlying secondary glaucoma from iris melanoma treatment in the past. The third patient was an asymptomatic 7-year-old girl in which the maculopathy resolved spontaneously without any surgical intervention with a final VA of 6/5. The fourth and fifth patients were asymptomatic with good vision in both eyes and were, therefore, only monitored with follow-ups. CONCLUSION: ODP maculopathy remains a challenging clinical entity for a vitreoretinal surgeon. The current management for ODP maculopathy involves surgical procedures with PPV being a common treatment of choice. Spontaneous resolution of ODP maculopathy has also been reported. Our study highlights the contrasting management that can be adopted in the treatment of ODP maculopathy, and there is not one definite treatment for this condition.

DISLODGEMENT OF FLUOCINOLONE ACETONIDE INTRAVITREAL IMPLANT INTO THE INFUSION CANNULA DURING VITRECTOMY FOR RETINAL DETACHMENT.

BACKGROUND/PURPOSE: To report a case of dislodgement of an Iluvien (fluocinolone acetonide) intravitreal implant into the infusion cannula during pars plana vitrectomy for retinal detachment. METHODS: The patient's surgery was video recorded, and the medical notes were reviewed retrospectively. RESULTS: A patient developed a macula off retinal detachment over 1 year after intravitreal injection of Iluvien for diabetic maculopathy. The patient underwent pars plana vitrectomy, removal of implant, and successful retinal reattachment. Although we planned to remove the implant through a sclerostomy, we were not able to localize it after performing peripheral indented vitrectomy. As the intraocular pressure was fluctuating, we suspected that the implant might have dislodged into the infusion cannula. However, despite increasing the intraocular pressure to 60 mmHg and performing repeated fluid-air exchange, we could not eject the implant back into the vitreous cavity. Therefore, after completing the surgery safely, we flushed the infusion cannula with balanced salt solution and we found the implant. CONCLUSION: Increasing the intraocular pressure and performing fluid-air exchange were not sufficient to eject the implant probably because of the strong surface adherence between the infusion cannula and the implant's coating material. We, therefore, recommend removing and flushing the infusion cannula if the implant cannot be localized in the eye. In addition, clinicians should be aware that a fluctuating intraocular pressure might be the first sign of a partially blocked infusion cannula by the implant.

Optic Disc Pit Maculopathy: A Review.

Optic disc pit (ODP) is a rare congenital anomaly of the optic disc that can be associated with maculopathy leading to progressive visual deterioration. The vast majority of cases are sporadic and no obvious factors have been correlated with the development of maculopathy. Optic disc pit maculopathy is defined by the concentration of intraretinal and subretinal fluid at the area of macula. Despite the advances in the imaging of the fundus, the origin of the fluid remains unknown and the exact pathogenesis of the maculopathy is not fully understood. Although some cases have been reported to resolve spontaneously, most cases require surgical intervention in order to treat ODP maculopathy and prevent loss of vision. Currently, there is no definite treatment for these patients and several surgical methods have been described, including pars plana vitrectomy (PPV) (combined with various techniques, such as inner retinal fenestration, autologous fibrin, and glial tissue removal), laser photocoagulation, intravitreal gas injection, and macular buckling. Overall, PPV remains the main form of surgical repair of ODP maculopathy. Although our understanding of the background and the pathophysiology of the disease has significantly improved, more studies are required in order to define the optimal treatment. This review summarizes the potential pathogenesis, as well as the diagnostic and therapeutic approach of ODP maculopathy.

Primary vitreoretinal lymphoma.

Primary vitreoretinal lymphoma (PVRL) is a rare ocular lymphoid malignancy, which consists a subset of primary central system lymphoma (PCNSL) and the most common type of intraocular lymphoma. The involvement of eyes is estimated to be approximately 20% of PCNSL, but the brain involvement may be up to 80% of PVRL. Typically, PVRL is a high grade B-cell malignancy of the retina and needs to be assorted from choroidal low-grade B-cell lymphomas. Very often PVRL masquerades and can be erroneously diagnosed as chronic uveitis, white dot syndromes or other neoplasms. Establishing an accurate diagnosis may involve cytology/pathology, immunohistochemistry, flow cytometry, molecular pathology and cytokine profile analysis. There is inadequate information about PVRL's true incidence, ethnic/geographical variation and pathogenetic mechanisms. The therapeutic approach of PVRL involves aggressive chemotherapy and radiation therapy. Although PVRL tends to have a good response to the initial treatment, the prognosis is poor and the survival restricted due to the high relapse rates and CNS involvement.

Management of retinal detachment in retinoblastoma with globe conserving treatment.

PURPOSE: To investigate the features and outcomes of retinoblastoma (Rb) patients complicated by development of retinal detachment (RD) following systemic chemotherapy with or without local focal consolidation treatment and without external beam radiotherapy (EBRT). METHODS: A retrospective study of all Rb patients between April 2002 and April 2012 at a tertiary hospital center (Birmingham Children's Hospital and Birmingham Midlands Eye Centre, United Kingdom). All eyes that had developed RD during or after systemic treatment with or without local focal consolidation treatment were included in the study. The time interval between the type of treatment of Rb, development of RD and relevant surgical intervention were analyzed. Patients with exudative RD were treated conservatively through observation and patients with rhegmatogenous RD were treated with scleral buckling. Final anatomical retinal reattachment rate and visual acuity outcomes were analyzed. RESULTS: A total of 258 patients were treated for Rb over the 10-year period. One hundred sixty-nine patients were managed with globe conserving treatment. Ten (5.92%) eyes of 10 patients were complicated with RD. Five eyes were exudative or presumed exudative type of RD while the other five eyes were rhegmatogenous or presumed rhegmatogenous RD. In the exudative group, two patients achieved visual acuity (VA) of 0.2 Single Kays (20/32 Snellen), and the other two patients achieved 0.85 and 0.86 Crowded Kays (20/142 and 20/145 Snellen), respectively, after the RD had resolved. The last patient in the group had to be enucleated due to tumor recurrences. The median time for the exudative RD to resolve is 15 weeks (range, 4-36 weeks). In the rhegmatogenous group, 3 of the 4 operated patients achieved retinal reattachment. The final postoperative VA ranged between 0.05 Crowded Kays to 1.84 Crowded Kays (20/22 to 20/1384 Snellen). The other patient was treated conservatively as no retinal breaks were found with previous cryotherapy and thermotherapy. The final VA in this patient was hand movement, and the RD did not reattach. The median time for the rhegmatogenous RD to reattach is 6 weeks (range, 4-8 weeks). There were no intra- and postoperative complications. CONCLUSIONS: With long-term conservative management through observation, exudative Rb after systemic treatment of Rb will tend to resolve by itself. However, supplementary local treatment with cryoretinopexy or laser photocoagulation during the systemic treatment of Rb can lead to an increased risk of rhegmatogenous RD. In these cases, most rhegmatogenous RD are successfully repaired with non-drainage scleral buckling and cryoretinopexy.

A Combination of Surgical Techniques to Repair a Giant Traumatic Macular Hole.

A 38-year-old man with a traumatic full-thickness macular hole (FTMH) presented to our eye casualty department with a sudden deterioration of his right eye vision to hand movements over the past one week. The suspected traumatic FTMH was present since he was 13 years old from a direct impact of a golf ball in his right eye and his best-corrected visual acuity (BCVA) has always remained at 1/60 Snellen vision. On examination, he had a very large FTMH measuring 1635 µm with central foveal retinal detachment. Pars plana vitrectomy combined with large inverted internal limiting membrane (ILM) peel flap, 5000 Cs silicone oil tamponade, and autologous platelets implantation was performed. Follow-up visits revealed that the FTMH was closed under silicone oil. The silicone oil was removed six months after the surgery and the FTMH remained close with the retina remaining attached. His BCVA was restored to his previous baseline level of 1/60 Snellen vision. With the advent of multiple techniques to repair FTMH such as the ILM flaps, we have combined this technique with older proven techniques such as silicone oil tamponade and autologous platelets implantation to close the giant traumatic FTMH. This case study demonstrates that combining techniques can help close a FMTH that is otherwise deemed impossible in the past.

Resource Planning in Glaucoma: A Tool to Evaluate Glaucoma Service Capacity.

BACKGROUND: The National Patient Safety Agency (2009) publication advising timely follow-up of patients with established glaucoma followed several reported instances of visual loss due to postponed appointments and patients lost to follow-up. The Royal College of Ophthalmologists Quality Standards Development Group stated that all hospital appointments should occur within 15% of the intended follow-up period. AIM: To determine whether: 1. Glaucoma follow-up appointments at a teaching hospital occur within the requested time 2. Appointments are requested at appropriate intervals based on the NICE Guidelines 3. The capacity of the glaucoma service is adequate Methods: A two-part audit was undertaken of 98 and 99 consecutive patients respectively attending specialist glaucoma clinics. In the first part, the reasons for delayed appointments were recorded. In the second part the requested follow-up was compared with NICE guidelines where applicable. Based on the findings, changes were implemented and a re-audit of 100 patients was carried out. RESULTS: The initial audit found that although clinical decisions regarding follow-up intervals were 100% compliant with NICE guidelines where applicable, 24% of appointments were delayed beyond 15% of the requested period, due to administrative errors and inadequate capacity, leading to significant clinical deterioration in two patients. Following the introduction of an electronic appointment tracker and increased clinical capacity created by extra clinics and clinicians, the re-audit found a marked decrease in the percentage of appointments being delayed (9%). CONCLUSIONS: This audit is a useful tool to evaluate glaucoma service provision, assist in resource planning for the service and bring about change in a non-confrontational way. It can be widely applied and adapted for use in other medical specialities.

A Mystery of Bilateral Annular Choroidal and Exudative Retinal Detachment with No Systemic Involvement: Is It Part of Vogt-Koyanagi-Harada Disease Spectrum or a New Entity?

A 65-year-old Caucasian male presented to the eye emergency department with bilateral significant visual loss. He was otherwise healthy with no significant past medical history. Ophthalmic history was significant for chronic open-angle glaucoma, for which the patient was using latanoprost once daily to both eyes. There was no preceding history of trauma or ocular surgery and the patient was emmetropic. Two weeks prior to his presentation, he reported a headache, which settled spontaneously. Slit-lamp examination demonstrated bilateral keratic precipitates, bilateral significantly shallow anterior chamber, and bilaterally normal intraocular pressures of 16 mm Hg. Fundal examination was significant for bilateral 360-degree choroidal detachments with exudative retinal detachment involving the maculae. These findings were confirmed using wide-field fundus photography, B-scan ultrasonography, and optical coherence tomography. Fundus fluorescein angiography did not reveal any evidence of retinal vasculitis. Indocyanine green chorioangiography of the posterior pole showed multiple areas of focal choroidal hypoperfusion. Extensive systemic investigation demonstrated no infectious, neoplastic, or inflammatory cause, and the patient did not complain of any systemic symptoms. Treatment with high-dose intravenous methylprednisolone was administered and this brought about complete resolution of both choroidal and retinal detachments, with partial visual recovery.

Case Series Investigating the Efficacy and Safety of Bilateral Fluocinolone Acetonide (ILUVIEN(®)) in Patients with Diabetic Macular Edema: 10 Eyes with 12 Months Follow-up.

INTRODUCTION: This short case series presents the results from 5 patients with bilateral chronic diabetic macular edema (DME), 12 months after they were initially treated with ILUVIEN (®) [0.2 µg/day fluocinolone acetonide (FAc)]. METHODS: Ten eyes from five patients with pseudophakic lenses were investigated. Patients had bilateral, chronic DME and had received prior laser and anti-VEGF therapy. Visual and anatomic outcomes were investigated 12 months post-FAc implant in both eyes. RESULTS: At baseline, central retinal thickness (CRT) was 645.3 ± 176.1 microns (mean ± standard deviation), intraocular pressure (IOP) was 13.7 ± 3.6 mmHg and visual acuity (VA) was 44.5 ± 18.6 Early Treatment Diabetic Retinopathy Study (ETDRS) letters. Mean CRT improved at 6 months (341.7 ± 169.7 microns) and 12 months (287.4 ± 103.1 microns) and there were concurrent improvements in VA (ETDRS letters were 56 ± 16 and 55 ± 16 at 6 and 12 months, respectively). Mean IOP was stable throughout and ≤21 mmHg. Left and right eyes were compared in the 5 patients by plotting changes in CFT, IOP and VA at 12 months, from baseline levels. CONCLUSION: This bilateral case series demonstrates the effectiveness of a sustained, controlled low dose of FAc in the management of bilateral DME over a 12-month period. The FAc implant has shown to work well in treatment of bilateral DME, although longer follow-up of these patients is still needed. FUNDING: Publication charges were funded by Alimera Sciences Ltd.

Case Series Investigating the Efficacy and Safety of Bilateral Fluocinolone Acetonide (ILUVIEN(®)) in Patients with Diabetic Macular Edema.

INTRODUCTION: Patients with diabetic macular edema (DME), a chronic, vision-limiting condition, may be insufficiently responsive to standard-of-care anti-vascular endothelial growth factor (VEGF) and/or laser therapies. One approved treatment for such patients is 0.2 µg/day fluocinolone acetonide (FAc) sustained-release implant; however, data are limited for treatment strategies in patients with bilateral chronic DME insufficiently responsive to standard-of-care therapies. METHODS: Six pseudophakic patients with bilateral, chronic DME previously treated with laser and anti-VEGF therapy (and intravitreal triamcinolone acetonide in 10 eyes) were retrospectively investigated for visual and anatomical outcomes, 6 months post-0.2 µg/day FAc implant in both eyes. RESULTS: At baseline, the mean best corrected visual acuity (BCVA) was approximately 6/38 or 43 [standard deviation (SD) ±17.4] Early Treatment Diabetic Retinopathy Study (ETDRS) letters; mean central retinal thickness (CRT) was 648 µm (SD ±160). Mean change in BCVA was +10 letters (SD ±12.2 letters), with 4/12 eyes maintaining or achieving driving vision (≥70 letters) and 3/12 eyes having unchanged BCVA. CRT was reduced 6 months after 0.2 µg/day FAc implant in 11/12 eyes. The mean intraocular pressure (IOP) was 16.1 mmHg [mean change of 1.1 mmHg (SD ±3.6)]. CONCLUSION: In a real-world setting, 0.2 µg/day FAc implant in both eyes was a feasible, effective choice for patients with severe bilateral DME, without notable increases in IOP. FUNDING: Publication charges were funded by Alimera Sciences Ltd. Medical writing assistance for this study was provided by QXV Communications and funded by Alimera Sciences Ltd.

Use of flucinolone acetonide for patients with diabetic macular oedema: patient selection criteria and early outcomes in real world setting.

INTRODUCTION: Fluocinolone acetonide slow release implant (Iluvien®) was approved in December 2013 in UK for treatment of eyes which are pseudophakic with DMO that is unresponsive to other available therapies. This approval was based on evidence from FAME trials which were conducted at a time when ranibizumab was not available. There is a paucity of data on implementation of guidance on selecting patients for this treatment modality and also on the real world outcome of fluocinolone therapy especially in those patients that have been unresponsive to ranibizumab therapy. METHOD: Retrospective study of consecutive patients treated with fluocinolone between January and August 2014 at three sites were included to evaluate selection criteria used, baseline characteristics and clinical outcomes at 3-month time point. RESULTS: Twenty two pseudophakic eyes of 22 consecutive patients were included. Majority of patients had prior therapy with multiple intravitreal anti-VEGF injections. Four eyes had controlled glaucoma. At baseline mean VA and CRT were 50.7 letters and 631 µm respectively. After 3 months, 18 patients had improved CRT of which 15 of them also had improved VA. No adverse effects were noted. One additional patient required IOP lowering medication. Despite being unresponsive to multiple prior therapies including laser and anti-VEGF injections, switching to fluocinolone achieved treatment benefit. CONCLUSION: The patient level selection criteria proposed by NICE guidance on fluocinolone appeared to be implemented. This data from this study provides new evidence on early outcomes following fluocinolone therapy in eyes with DMO which had not responded to laser and other intravitreal agents.

Neoplastic leptomeningeal disease masquerading as central serous retinopathy. A case report.

A 69-year-old man became aware of people's speech being out of synch with their lip movements alongside persistent headaches, both of which progressively worsened. A few weeks later, he developed progressive and painless visual loss in one eye. Initial neurological evaluation, inflammatory markers and head computed tomography scan were normal. Ophthalmological examination and OCT scan revealed right macular subretinal fluid with choroidal indentation, which prompted urgent further investigations including head MRI revealing extensive leptomeningeal disease. The patient continued to deteriorate and deceased shortly afterwards. This is the first reported case of neoplastic leptomeningeal disease presenting with loss of vision due to choroidal metastasis with localised exudative retinal detachment. Diagnosing neoplastic leptomeningeal disease requires a high index of suspicion from the treating physician. Symptoms may be nonspecific and/or subtle. Combining cerebrospinal fluid cytology from lumbar puncture with contrast-enhanced magnetic resonance imaging of the brain is considered the optimal diagnostic approach.

Sequential surgical repair of a macular hole-related retinal detachment in a hyperopic patient.

PURPOSE: To report a case of macular hole-related retinal detachment in a hyperopic patient and a sequential surgical approach to repair. METHODS: Case report. PATIENTS: A single patient with acute macular hole-related retinal detachment. CONCLUSION: Retinal detachment secondary to macular hole typically occurs in highly myopic patients and is rare in patients with hypermetropia. Surgical repair may involve a combined or sequential approach to close the macular hole.

A case of severe hydroxychloroquine-induced retinal toxicity in a patient with recent onset of renal impairment: a review of the literature on the use of hydroxychloroquine in renal impairment.

We present a case of a 67-year-old female who presented with a twelve-month history of progressive blurred vision in both eyes. The patient was on hydroxychloroquine 200 mg twice a day for eight years for the treatment of scarring alopecia. Two years prior to presenting, the patient was found to have chronic kidney disease stage 3 secondary to hypertension. Examination revealed bilateral reduced visual acuities with attenuated arterioles and pigmentary changes on retinal assessment. Goldmann visual fields showed grossly constricted fields in both eyes. The patient was diagnosed with retinal toxicity secondary to hydroxychloroquine probably potentiated by renal impairment. Risk factors for retinal toxicity secondary to hydroxychloroquine can be broadly divided into dose-related and patient-related factors. Our patient developed severe retinal toxicity despite being on the recommended daily dose (400 mg per day). Although retinal toxicity at this dose has been documented, the development of renal impairment without dose adjustment or close monitoring of visual function is likely to have potentiated retinal toxicity. This case highlights the need to monitor renal function in patients on hydroxychloroquine. Should renal impairment develop, either the drug should be stopped or the dose reduced with close monitoring of visual function by an ophthalmologist.