Paraproteinemic maculopathy.

PURPOSE: Paraproteinemia relates to monoclonal gammopathy-producing pathologic antibodies with serous macular detachment being an uncommon ocular manifestation. We ascertained the clinical course of maculopathy in paraproteinemia and investigated the effect of various therapeutic methods on the resolution of subretinal deposits. DESIGN: Multicenter, retrospective, observational case series. PARTICIPANTS: The records of patients with paraproteinemia with optical coherence tomography (OCT) documentation of serous macular detachment were reviewed. METHODS: Data collection included coexisting morbidity, rheology data (immunoglobulin level, hematocrit, and blood viscosity), clinical examination results, and OCT findings. MAIN OUTCOME MEASURES: Best-corrected visual acuity (BCVA), height and basal area of the serous macular detachment, and systemic versus local therapies. RESULTS: A total of 33 cases were collected: 10 new and 23 previously reported in the literature. Diabetes was present in 7 patients, systemic hypertension in 9 patients, and anemia in 18. Mean initial immunoglobulin level was 6497 mg/dl, and mean serum viscosity was 5.5 centipoise (cP). Mean logarithm of the minimum angle of resolution initial vs. final BCVA was 0.55 (Snellen equivalent, 20/71) vs. 0.45 (20/56) in the right eye and 0.38 (20/48) vs. 0.50 (20/63) in the left eye. After mean follow-up of 7 months (range, 0-51 months). Systemic therapies included plasmapheresis (18), chemotherapy (30), blood transfusions (2), transplantation of progenitor hematopoietic cells (2), and oral rituximab (10). Immunoglobulin levels normalized in 8 patients and were unchanged in 1 after plasmapheresis, chemotherapy, or both. Ocular therapy in 8 patients included vitrectomy (1), laser photocoagulation (4), intravitreal bevacizumab (5), intravitreal triamcinolone (2), intravitreal dexamethasone implant (1), intravitreal rituximab (1), and sub-Tenon corticosteroid (1). The maculopathy resolved partially or completely in 17 patients and worsened or remained unchanged in 14 patients over median follow-up of 7 months. Maculopathy was unilateral in 9 cases and occurred at a lower initial immunoglobulin level in diabetics. There was a positive correlation between area of the detachment and serum viscosity. CONCLUSIONS: Paraproteinemic maculopathy can be unilateral. Decreasing the blood immunoglobulin level is the primary goal of therapy for paraproteinemic maculopathy, and this can be achieved by a systemic route. Coexisting diabetes facilitates leakage of immunoglobulins at lower levels than in nondiabetics.

Photodynamic therapy for symptomatic subretinal fluid related to choroidal nevus.

PURPOSE: The purpose of this article is to evaluate the role of photodynamic therapy (PDT) for symptomatic choroidal nevus with subretinal fluid (SRF) extending to the fovea. METHODS: Retrospective review of the medical records of all patients who underwent PDT for a symptomatic choroidal nevus with SRF. RESULTS: Seventeen patients were included in the study. The mean initial visual acuity was 20/80 (range, counting fingers to 20/20). The mean initial tumor thickness was 1.23 mm (range, 0.66-1.93 mm). All tumors presented at least 2 risk factors for growing (including orange pigment, symptoms, peripapillary location, SRF, and thickness >2 mm). The mean number of PDT sessions was 1.41 (range, 1-3). The mean final visual acuity improved to 20/60 (range, counting fingers to 20/20). Subretinal fluid was reduced in all eyes (100%) and had completely disappeared in 9 eyes (53%) after PDT. Of 9 cases with complete resolution of the SRF, 2 patients (22%) presented recurrence. The mean final tumor thickness increased to 1.24 mm (range, 0.66-2.01 mm) at a mean follow-up of 22.47 months (range, 6-60 months). Tumor thickness increased in 3 eyes (18%) and remained unchanged in 13 eyes (76%), and 1 lesion (6%) shrank down to a flat chorioretinal scar. CONCLUSION: Photodynamic therapy is a good treatment option to reduce SRF in symptomatic choroidal nevus with serous macular detachment. Further growth into melanoma was observed in 18% of cases. Thus, PDT may not allow a good local tumor control. Longer follow-up is required to determine its value in these patients.

Ocular findings in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome.

OBJECTIVE: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare disorder. This study investigated the types of ocular signs and symptoms in patients with POEMS and any systemic factors that may be associated with development of such ocular findings. DESIGN: Case series from tertiary referral center. PARTICIPANTS: A total of 33 patients with POEMS syndrome underwent at least 1 ophthalmologic examination and were included in the study. METHODS: A 10-year retrospective chart review of patients diagnosed with POEMS syndrome was performed. MAIN OUTCOME MEASURES: Visual symptoms, visual acuity, presence of optic disc edema (ODE), and levels of systemic factors (including plasma vascular endothelial growth factor [VEGF], plasma interlukin-6 [IL-6], and raised intracranial pressure) and their relationship to ODE. RESULTS: Five of the patients (15%) reported diplopia, 15 patients (45%) had blurred vision, and 3 patients (9%) had ocular pain. The most common ocular finding was bilateral ODE in 17 patients (52%). Of the patients with ODE, 5 (29%) were asymptomatic at the first ocular examination. Among patients with ODE, there was a significant difference (P = 0.03) between the mean plasma VEGF level at the time of diagnosis of the ODE compared with when the ODE resolved. There was no difference in plasma IL-6 levels between people with and without ODE. Patients with ODE had a higher mean lumbar puncture opening pressure (276±14 mm H(2)O; normal range, 100-250 mm H(2)0) than patients without ODE, although the difference was not statistically significant (P = 0.08). CONCLUSIONS: Optic disc edema is a common finding in patients with POEMS. Because patients can be asymptomatic, eye examinations should be performed in all patients with POEMS. There may be an association between elevated VEGF and intracranial pressure and ODE; further studies are required.

Clinical magnification and residual refraction after implantation of a double intraocular lens system in patients with macular degeneration.

PURPOSE: To evaluate the efficacy of a standard double intraocular lens (IOL) system (IOL-Vip) in patients with low vision and central scotoma due to macular degeneration and assess the predictability of the residual refraction and magnification. SETTING: Ophthalmology Department, Hospital General Universitario, Valencia, Spain. METHODS: This interventional prospective noncomparative case series comprised 13 consecutive surgical procedures in 10 patients with central scotoma. Follow-up was 12 months. Evaluation included the difference between preoperative and postoperative best corrected visual acuity (BCVA), refraction, position of the IOLs, endothelial cell density, and occurrence of postoperative complications. Residual refraction and eye magnification were calculated using a theory developed in a previous study, and the values were compared with the clinical results. RESULTS: The mean BCVA was 1.37 logMAR preoperatively and 0.68 logMAR 1 year postoperatively. The mean best corrected clinical gain was 44%. There was no statistically significant difference between the clinically evaluated and theoretically calculated residual refractions (P = .17). No intraoperative or postoperative complications occurred. CONCLUSIONS: Implantation of the double IOL system improved BCVA in patients with low vision due to advanced maculopathy. The results were best in myopic patients (long eyes); patients with hyperopia (short eyes) had high residual refraction. The postoperative clinical gain and residual refraction were predictable, showing the feasibility of implanting a customized double IOL.

Intravitreal bevacizumab (Avastin) injection in ocular ischemic syndrome.

PURPOSE: To determine whether bevacizumab may improve anatomic and visual outcomes in patients with ocular ischemic syndrome (OIS). DESIGN: Interventional case reports. METHODS: Two patients with OIS presenting with unilateral ocular pain, iris neovascularization, and macular edema. Intravitreal injection of bevacizumab (1.25 mg). The main outcome measures were postinjection best-corrected visual acuity (BCVA), intraocular pressure (IOP), angiographic findings, and optical coherence tomography (OCT) findings and complications. RESULTS: One week after treatment, both patients demonstrated regression of the iris neovascularization and improvement of the macular edema, with no changes in BCVA and IOP. One eye was reinjected at four months. After three and seven months, no significant or systemic adverse events were observed, and no signs of new iris neovascularization were present. CONCLUSIONS: Intravitreal bevacizumab may be useful for the treatment of eyes with iris neovascularization and macular edema secondary to OIS.

High-dose infliximab prophylaxis in endotoxin-induced uveitis.

PURPOSE: The aim of this study to analyze the preventive effect of high-dose infliximab in endotoxin-induced uveitis (EIU) in rabbits. METHODS: An experimental study was conducted on 64 rabbits. Salmonella typhimurium lipopolysaccharide endotoxin was intravitreally injected. Infliximab was intravenously (i.v.) injected 24 h before the intravitreal injection (20 mg/kg). The animals were randomly assigned to five groups: group A, saline intravitreal injection; group B, Infliximab i.v. group C, infliximab + saline; group D, intravitreal endotoxin and group E, infliximab i.v. + intravitreal endotoxin. With two masked observers, a microscopic examination of aqueous humor (cells, tumor necrosis factor [TNF] alpha) and aqueous protein level were performed 24 h after an endotoxin injection and 48 h after an infliximab infusion. RESULTS: Infliximab treatment, at a dose of 20 mg/kg, significantly improved all the parameters. Inflammatory cell infiltration was significantly reduced in the iris, ciliary body, and anterior chamber (U Mann-Whitney test, P = 0.01). Associated with a lower level of TNF-alpha and protein exudate in aqueous humor (U Mann-Whitney test, P = 0.01). CONCLUSIONS: Infliximab, at a dose of 20 mg/kg, is effective in the prophylaxis of the EIU.

Photodynamic therapy of symptomatic choroidal nevi.

PURPOSE: To evaluate the role of photodynamic therapy (PDT) for patients with symptomatic choroidal nevi involving the fovea or located near the fovea with subretinal fluid extending to the fovea. MATERIALS AND METHODS: Retrospective review of five patients who underwent PDT for choroidal nevi at two separate centers in Ankara and Barcelona. RESULTS: The mean initial logMAR visual acuity was 0.5 (range: 0 to 1.5). The mean largest tumor base diameter was 3.2 mm (range: 2.1-4.5 mm) and the mean tumor thickness was 1.1 mm (range: 0.7-1.6 mm). The mean number of PDT sessions was 1.6 (range:1-3). The mean final tumor thickness was 1.0 mm (range: 0-1.6 mm) at a mean follow-up of 19 months (range: 12-32 months). The mean final logMAR visual acuity was 0.4 (range: 0-1.5). Subfoveal fluid disappeared or decreased significantly in 4 of 5 eyes (80%) after PDT. CONCLUSIONS: PDT led to resolution of subretinal fluid with preservation of visual acuity in many symptomatic choroidal nevi in this study. Careful case selection is important as PDT of indeterminate pigmented tumors may delay the diagnosis and treatment of an early choroidal melanoma and thereby increase the risk for metastasis.

Serous retinal detachment as a presenting feature of chronic myelogenous leukemia.

PURPOSE: The purpose of this study was to report a case of diffuse unilateral retinal detachment as an initial symptom of chronic myelogenous leukemia in a 35-year-old woman. METHODS: This is a case report of a 35-year-old white woman who presented with painless acute visual loss in her left eye. RESULTS: A 35-year-old white woman presented with acute visual loss in her left eye. She had no systemic or ophthalmological medical history. Slit-lamp examination was normal in both eyes. Fundus examination of the left eye showed not only multiple retinal hemorrhages but also a diffuse serous retinal detachment affecting the nasal aspect of the retina and the posterior pole. The leukocyte count was 124,000/mm (99% blasts), and the Philadelphia chromosome was positive, an abnormality that is associated with chronic myelogenous leukemia. CONCLUSION: Serous retinal detachment is an uncommon feature among leukemias. Chronic myelogenous leukemia may remain asymptomatic until a blastic phase occurs. Prompt recognition of the disease leads to early systemic chemotherapy and may help to restore visual function.

Fundus autofluorescence of choroidal melanocytic lesions and the effect of treatment.

PURPOSE: To correlate fundus autofluorescence (FAF) patterns in choroidal melanocytic lesions with changes present on the surface of such lesions, including lipofuscin, hyperpigmentation, drusen, and fibrous metaplasia, and to describe the effect of treatment on FAF. METHODS: Retrospective chart review of 23 consecutive patients with choroidal nevi and melanoma who underwent FAF photography. The correlation between increased FAF patterns and foci of lipofuscin, hyperpigmentation, drusen, or fibrous metaplasia was defined as a complete correlation, partial correlation, or no correlation. The posttreatment FAF photographs of 6 patients with choroidal melanoma who were managed with plaque radiotherapy or plaque radiotherapy and transpupillary thermotherapy were also analyzed. RESULTS: Lipofuscin was present in 13 tumors, hyperpigmentation in 9 tumors, drusen in 6 tumors, and fibrous metaplasia in 4 tumors. A complete correlation between increased FAF and lipofuscin was found in 8 tumors (61.5%), a partial correlation in 3 tumors (23.1%), and no correlation in 2 tumors (15.4%). A complete correlation between hyperpigmentation and increased FAF was found in 5 tumors (55.6%), a partial correlation in 3 tumors (33.3%), and no correlation in 1 tumor (11.1%). A partial correlation was found between drusen and increased FAF in all 4 tumors. A partial correlation was found between fibrous metaplasia and increased FAF in all 3 tumors. Following treatment, increased FAF was observed in 6 choroidal melanomas owing to an increase in lipofuscin and hyperpigmentation. CONCLUSIONS: Choroidal melanocytic lesions with overlying lipofuscin and hyperpigmentation are associated with increased FAF in about 90% of cases. Fundus autofluorescence photography may be helpful in evaluating small melanocytic tumors, since lipofuscin is a risk factor for growth. Following treatment, choroidal melanomas may show increased FAF.