Long-Term Ranibizumab Treatment in Neovascular Age-Related Macular Degeneration: A Belgian Subanalysis from the Global Real-World LUMINOUSTM Study.

PURPOSE: To evaluate long-term, real-world treatment patterns and outcomes of ranibizumab 0.5 mg for neovascular age-related macular degeneration (nAMD) in a Belgian cohort. PATIENTS AND METHODS: This Belgian (BE) cohort of the 5-year global observational LUMINOUS study included 229 patients with nAMD. Outcomes included visual acuity (VA), central retinal thickness (CRT) and safety. RESULTS: The mean age was 79.5±7.7 years. The majority of patients (67.7%) were female and all patients were Caucasian. Most patients previously received ranibizumab with only 17.5% of patients being treatment-naïve. The injection frequency declined over time irrespective of prior treatment status (p<0.0001), with treatment-naïve eyes receiving a mean of 4.2±2.9 yearly injections and prior-ranibizumab eyes 3.6±2.7. Regression analysis confirmed first-year VA increases for treatment-naïve eyes (p=0.002) followed by a slight decrease of -1.8 letters per year. For prior-ranibizumab eyes, the visual changes over 1 year were statistically non-significant (p=0.90) but declined slightly after year one (p<0.0001). Anatomically, the CRT of treatment-naïve eyes decreased over time from baseline (p<0.0001), whereas the CRT of prior-ranibizumab eyes remained stable (p=0.43). No new safety findings were identified. CONCLUSION: LUMINOUS-BE reconfirms the well-characterized benefit-risk profile of ranibizumab for nAMD treatment. The observed low injection frequency reflects a need for more rigorous treatment in real-world settings. CLINICAL TRIAL REGISTRATION: NCT01318941.

Early diagnosis and successful treatment of paraneoplastic melanocytic proliferation.

BACKGROUND: Paraneoplastic melanocytic proliferation (bilateral diffuse uveal melanocytic proliferation, BDUMP) is a rare but devastating disease that causes progressive visual loss in patients who usually have an occult malignancy. Visual loss occurs as a result of paraneoplastic changes in the uveal tissue. METHODS: In a masked fashion, the serum of two patients with BDUMP was evaluated for the presence of cultured melanocyte elongation and proliferation (CMEP) factor using cultured human melanocytes. We evaluated the efficacy of plasmapheresis as a treatment modality early in the disease in conjunction with radiation and chemotherapy. RESULTS: The serum of the first case patient was investigated after plasmapheresis and did not demonstrate proliferation of cultured human melanocytes. The serum of the second case was evaluated prior to treatment with plasmapheresis and did induce this proliferation. These findings are in accordance with the diminution of CMEP factor after plasmapheresis. Treatment with plasmapheresis managed to stabilise the ocular disease progression in both patients. CONCLUSIONS: In the past, visual loss due to paraneoplastic melanocytic proliferation was considered progressive and irreversible. We treated two patients successfully with plasmapheresis and demonstrated a relation between CMEP factor in the serum of these patients and proliferation of cultured melanocytes.

Effect of pegaptanib sodium 0.3†mg intravitreal injections (Macugen) in intraocular pressure: posthoc analysis from V.I.S.I.O.N. study.

OBJECTIVE: To assess the rate of pegaptanib-associated sustained intraocular pressure (IOP) elevation. METHODS: A posthoc analysis was conducted on all IOP measurements, except the immediate 30-min postinjection, from all subjects randomised to pegaptanib 0.3 mg or sham injections continuously in the first 2 years of the Vascular endothelial growth factor Inhibition Study in Ocular Neovascularisation (V.I.S.I.O.N.) study. Measurements were taken with Goldmann applanation tonometer or Tonopen, except at baseline and in cases of an IOP reading >30 mm Hg when a Goldmann applanation tonometer was mandatory. RESULTS: Of 221 subjects, IOP measurements ≥22 mm Hg were seen in 28/114 and 23/107 subjects of the pegaptanib and sham subgroups, respectively (p=0.6338) and measurements ≥24 mm Hg were observed in eight and eight subjects in the pegaptanib and sham groups, respectively. More than two measurements ≥22 mm Hg occurred in six and 10 subjects (p=0.3025), and more than two measurements ≥24 mm Hg were observed in one and four subjects in the pegaptanib and sham groups, respectively. One patient with sustained IOP elevation in the pegaptanib study group, and four in the sham group, had IOP lowering medication added during the course of the study. No subject required glaucoma surgery. CONCLUSIONS: In V.I.S.I.O.N., after 2 years, there was no evidence of sustained IOP elevation associated with pegaptanib 0.3 mg use. TRIAL REGISTRATION NUMBER: NCT00321997.

Retinal pigment epithelial detachments and tears, and progressive retinal degeneration in light chain deposition disease.

BACKGROUND/PURPOSE: Light-chain deposition disease (LCDD) is a rare condition characterised by deposition of monoclonal immunoglobulin light chains (LCs) in tissues, resulting in varying degrees of organ dysfunction. This study reports the characteristic clinical ocular findings seen in advanced LCDD upon development of ocular fundus changes. This is the first report to describe this entity in vivo in a series of patients. METHODS: A case series of ocular fundus changes in three patients with kidney biopsy-proven LCDD. All patients underwent best corrected visual acuity (BCVA) exam, perimetry, colour fundus photography and fluorescein angiography; two patients underwent indocyanine green angiography, optical coherence tomography, ultrasound and electroretinography; and one patient underwent fundus autofluorescence. RESULTS: Three patients, 53-60 years old at initial presentation, were studied. All three presented with night blindness, poor dark adaptation, metamorphopsia and visual loss. Examination revealed serous and serohaemorrhagic detachments, multiple retinal pigment epithelial (RPE) tears, diffuse RPE degeneration and progressive fibrotic changes. Neither choroidal neovascularisation nor other vascular abnormalities were present. Final best corrected visual acuity (BCVA) ranged from 20/40 to 20/300. CONCLUSIONS: Progressive LC deposition in the fundus seems to damage RPE pump function with flow disturbance between choroid and retina. This pathogenesis can explain the evolution to RPE detachments and subsequent rips and progressive retinal malfunction.

Flecked retina, choroidopathy, and retinal pigment epithelial tear in familial pulmonary arterial hypertension.

BACKGROUND: Thanks to advancement in treatment modalities, the medial survival rate of patients with familial pulmonary arterial hypertension (FPAH) has been improved. Unfortunately, ocular complications because of the chronically elevated systemic venous pressure become more frequent. METHODS: The authors report new FPAH-associated fundus changes in a 50-year-old male patient treated with sildenafil. The anomalies were studied with autofluorescence, fluorescein and indocyanine green angiography, and spectral optical coherence tomography including enhanced depth imaging. RESULTS: Loss of vision and cystoid macular edema were associated with a flecked retina, a thick choroid, central serous choroidopathy-like changes in both eyes and with retinal pigment epithelium detachments and a retinal pigment epithelium tear in the right eye. CONCLUSION: Treatment of ocular findings associated with FPAH mainly involves optimal control of pulmonary arterial hypertension and ophthalmic supportive treatment toward preventing acute ocular complications. The ocular complications occur as a result of enhanced pressure in the superior vena cava and in the ophthalmic veins, resulting in dilation of the ocular veins and congestion of the choroid. Sildenafil treatment in FPAH may enhance the congestion of the choroid and can induce central serous choroidopathy-like changes. A flecked retina, central serous choroidopathy-like changes, and retinal pigment epithelium tear are rare complications of FPAH.

Diving-related visual loss in the setting of angioid streaks: report of two cases.

PURPOSE: The purpose of this study was to report diving-related visual loss in the setting of angioid streaks. METHODS: Observational case reports of two patients with angioid streaks suffering sudden visual loss immediately after diving. RESULTS: Two young adult male patients presented with visual loss after diving headfirst. Funduscopy revealed angioid streaks, peau d'orange, subretinal hemorrhages, and ruptures of Bruch membrane. Choroidal neovascularization developed during follow-up. Both patients had an otherwise uneventful personal and familial medical history. CONCLUSION: In patients with angioid streaks, diving headfirst can lead to subretinal hemorrhages and traumatic ruptures in Bruch membrane and increase the risk of maculopathy. Ophthalmologists should caution patients with angioid streaks against diving headfirst.

Vertical transmission of macular telangiectasia type 2.

PURPOSE: The purpose of this study was to report vertical transmission of macular telangiectasia type 2 and type 2 diabetes mellitus in 3 families. METHODS: In this retrospective interventional case series, the charts of patients with inherited macular telangiectasia type 2 were reviewed. A large spectrum of presentations of macular telangiectasia type 2 was observed and has been studied with different techniques including best-corrected visual acuity, microperimetry, confocal blue reflectance fundus autofluorescence, fluorescein angiography, and time domain and spectral domain optical coherence tomography. RESULTS: Vertical transmission of macular telangiectasia type 2 and associated type 2 diabetes mellitus is described in 3 families. Symptomatic as well as asymptomatic eyes with macular telangiectasia type 2 were identified. In 2 families, a mother and son experienced visual loss and were diagnosed with macular telangiectasia type 2. All 4 patients had type 2 diabetes. Diabetic retinopathy was observed in one mother and her son. In the third family, the index patient was diagnosed macular telangiectasia type 2 after complaints of metamorphopsia. She and her family members had type 2 diabetes mellitus, and further screening of her family revealed familial macular telangiectasia type 2. None of the patients were treated for macular telangiectasia type 2. CONCLUSION: Macular telangiectasia type 2 may be more common than previously assumed, as vision can remain preserved and patients may go undiagnosed. Screening of family members is indicated, and detection of mild anomalies is possible using fundus autofluorescence and spectral domain optical coherence tomography.