Occlusive Cytomegalovirus Panuveitis following Intravitreal Dexamethasone Implant.

PURPOSE: To report a case of unilateral cytomegalovirus (CMV) panuveitis with occlusive vasculitis following injection of intravitreal dexamethasone implant in a patient with type 2 diabetes mellitus. METHODS: Observational case report. RESULTS: A 60-year-old immunocompetent male with well-controlled type 2 diabetes mellitus was treated with intravitreal dexamethasone implant for recurrent uveitis that was responsive to steroids. 3 months after implantation, patient develops panuveitis with occlusive vasculitis. Anterior chamber tap confirms diagnosis of cytomegalovirus retinitis. Intravitreal foscarnet and oral valganciclovir led to quiescent disease. CONCLUSIONS: Patients treated with local immunosuppressants should be monitored carefully to assess treatment response and complications, even in the absence of frank immunodeficiency. Quantitative viral PCR can be an effective way to monitor treatment response to antiviral therapy.

Biologic agents in experimental autoimmune uveitis.

Experimental uveitis models were developed in an effort to elucidate the pathogenesis of human uveitis. The therapeutic effects of numerous anti-inflammatory agents including corticosteroids and immunomodulatory agents including biologic response modifiers have been investigated in both experimental and human uveitis. Monoclonal antibodies to tumor necrosis factor alpha and anti-interleukins, among others, demonstrate efficacy and are employed in the treatment of uveitis refractory to conventional immunomodulatory agents.

Spectral domain optical coherence tomography findings in patients with acute syphilitic posterior placoid chorioretinopathy.

PURPOSE: To describe the appearance of acute syphilitic posterior placoid chorioretinitis, a rare ocular manifestation of syphilis, on spectral domain optical coherence tomography (SD OCT) both before and after treatment. METHODS: Ophthalmic examination and imaging studies of 30 eyes of 19 confirmed cases were analyzed both at the time of presentation and at each follow-up visit. Patients with SD OCT and fluorescein angiography at the time of presentation, and at least three documented follow-up visits after initiation of therapy, were included in the study. Standard treatment of neurosyphilis was given to each patient, including 4 million units of penicillin G administered intravenously every 4 hours for 14 days. RESULTS: Fundus examination and imaging studies were consistent with previous reports and confirmed the diagnosis of acute syphilitic posterior placoid chorioretinitis. In 13 eyes (43.3%), baseline SD OCT scans were performed within 1 to 2 days of presentation and revealed a small amount of subretinal fluid, disruption of the inner segment/outer segment junction, and hyperreflective thickening of the retinal pigment epithelium (RPE). All 30 eyes were again scanned between Days 7 and 9 after presentation and revealed loss of the inner segment/outer segment and OS/RPE bands, and irregular hyperreflectivity of the RPE with prominent nodular elevations but without subretinal fluid. Early disruption of the external limiting membrane and punctate choroidal hyperreflectivity were seen in 1 of the 30 eyes (3.3%) and 14 of the 30 eyes (46.6%), respectively. Vision improved and the outer retinal abnormalities normalized in 28 of the 30 eyes (93.3%) after the treatment of neurosyphilis. The external limiting membrane, inner segment/outer segment band, and/or linear outer segment/RPE junction remained substantially abnormal despite treatment in 2 eyes left with 20/200 vision. CONCLUSION: Patients with acute syphilitic posterior placoid chorioretinitis show characteristic outer retinal abnormalities on SD OCT imaging, including disruption of the inner segment/outer segment band, nodular thickening of the RPE with loss of the linear outer segment/RPE junction, and, in some cases, loss of the external limiting membrane, accumulation of subretinal fluid, and punctate hyperreflectivity in the choroid. Vision improved and these abnormalities reversed after treatment of neurosyphilis in most of the patients. Persistently, poor vision despite treatment was associated with long-term loss or disruption of outer retinal anatomy on SD OCT.

Proliferative vitreoretinopathy: current and emerging treatments.

Proliferative vitreoretinopathy is a disease process that follows the proliferation of ectopic cell sheets in the vitreous and/or periretinal area, causing periretinal membrane formation and traction, in patients with rhegmatogenous retinal detachments. Currently, vitreous surgery is the standard treatment; however, the results aren't satisfactory given the vision loss that ensues and that redetachment is relatively common. It is becoming clearer that there exists an interplay between various cytokines/growth factors, matrix proteins, and the different cell types that drive the undesirable formation of periretinal membranes. This fundamental understanding is aiding in identifying different adjunct agents that can block the cellular events intrinsic to proliferative vitreoretinopathy. In this review, we describe the current understanding on the pathogenesis and discuss how the fundamental understanding of the biochemical/molecular events is instrumental in developing the novel treatment strategies that are also highlighted.

Orbital Burkitt's Lymphoma: An Aggressive Presentation.

Objective. To present the case of an aggressive orbital Burkitt's lymphoma. Methods. Chart review. Case Presentation. A 24-year-old Haitian man came to our clinic complaining of rapidly progressive right eye proptosis. On examination, a large friable exophytic mass with necrotic areas and exudative/hemorrhagic secretions was noted protruding from his right orbit. A biopsy revealed the characteristic "starry-sky" appearance of a Burkitt lymphoma. The patient died shortly after due to complications from systemic involvement. Discussion. This case is meant to raise physicians' awareness on the healthcare situation in some underdeveloped countries, emphasizing the importance of education in preventive medicine.

Presumed sturge weber syndrome in a haitian boy: a case of delayed diagnosis.

Purpose. To report an untypical presentation of a presumed Sturge-Weber Syndrome (SWS), and to highlight the indispensable value of thorough clinical examination as primary means for proper diagnosis and management. Methods. Chart review. Results. A 7 year-old boy, with a long history of ocular symptoms and an unspecified ocular surgery, presents with a painful blind left eye. Based on clinical examination, the suspicion of SWS was raised. The presentation was not typical in the sense that no evident port-wine stain was observed on the face. However, facial asymmetry and gum discoloration were guiding clinical clues to pursue further investigations. Unfortunately, due to poor treatment response, the patient underwent enucleation. Tissue pathology revealed diffuse choroidal hemangiomas, consistent with the diagnosis of SWS. Conclusion. SWS presents with hamartomatous malformations and venous dilation affecting the skin, central nervous system and eye. The ocular involvement may vary, with the most common complications being glaucoma, buphthalmos and diffuse choroidal hemangiomas. This case report helps remind physicians of the importance of a thorough clinical examination, and highlights the ophthalmologists' responsibility of examining beyond the eye.

Clinical and ultrasound biomicroscopy features associated with growth in iris melanocytic lesions.

PURPOSE: To determine the clinical and ultrasound biomicroscopy (UBM) features associated with growth in iris melanocytic lesions. STUDY DESIGN: Retrospective case series analysis. METHODS: We included all iris melanocytic lesions that were monitored between January 2005 and November 2009. At the end of the analysis, 44 eyes of 44 patients were included in the final analysis. The clinical features analyzed were: iris color, largest base diameter, radial location of the lesion epicenter, circumferential location of the lesion epicenter, lesion configuration, lesion pigmentation, intrinsic vascularity within the lesion, presence of associated pigmentation, the impact on the pupil, presence of iris atrophy, and lesion-induced localized cataracts. The UBM features included lesion thickness, presence of corneal touch, presence of surface plaque, internal structure, and internal reflectivity. Regression analysis was performed to define the features associated with growth. RESULTS: Twenty-three percent of the lesions showed documented growth. Mean follow-up was 21.4 months (range: 10-48). Clinical features associated with growth were a large basal diameter at baseline (P = .004) and inferior location (P = .004). UBM features associated with growth were: a greater baseline thickness (P = .01), presence of corneal touch (P = .007), an irregular internal structure (P = .0001), and the presence of dots and linear streaks (P < .0001). Clinical features that were not associated with growth were the radial location of the lesion in the iris (P > .999), lesion configuration (P > .999), lesion pigmentation (P > .999), the presence of pigment dispersion (P = .70), iris freckles (P = .15), corectopia (P > .999), ectropion (P > .999), and intrinsic vascularity (P = .70). UBM features not associated with growth were the presence of a surface plaque (P = .07) and the internal reflectivity (P = .77). CONCLUSION: Substantial growth in iris melanocytic lesions is associated with original larger basal diameter and inferior lesion location. On UBM growth is associated with greater original thickness, presence of corneal touch, and an irregular internal structure. Presence of these features could modify the frequency of observation of those lesions.

A case of acute multifocal inner retinitis associated with nonspecific viral illness.

PURPOSE: To describe a patient with sudden unilateral loss of vision associated with white inner retinal lesions and a history of a nonspecific viral infection. METHODS: Case report. We reviewed the medical record and retinal angiogram of one patient. RESULTS: The patient presented with a sudden loss of visual acuity in the left eye. Fundus examination revealed multiple white inner retinal lesions and hyperemic optic disks in both eyes. Spontaneous improvement of visual acuity and resolution of the retinal lesions were noted over the ensuing weeks. CONCLUSION: Retinitis can present as a nonspecific response to various infectious agents. We herein report a case of acute multifocal inner retinitis associated with a nonspecific viral illness.

Uveal metastatic disease: current and new treatment options (review).

Choroidal metastasis represents the most common form of intraocular malignancies. It may occur in up to 10% of patients with systemic metastasis with almost half of the patients developing central nervous system disease. The most common primary sites of ocular metastasis are breast cancer in women and lung cancer in men. In most cases, these lesions tend to be asymptomatic and are not evaluated by an ophthalmologist. The diagnosis is generally made by the history of present or prior malignancies and an ophthalmological examination with slit-lamp biomicroscopy and indirect ophthalmoscopy. As with other malignancies, management may vary with each patient. Small tumors, that do not compromise the vision and that have responded previously to systemic treatment, may be closely observed. For larger lesions and for symptomatic ones, external beam radiation offers an excellent alternative to save the eye and stabilize vision. Bevacizumab (Avastin), a potent monoclonal antibody that has also been employed for the treatment of ocular vaso-proliferative diseases, has been used in the treatment of choroidal metastasis and has shown promising results.

Clinical and pathologic characteristics of biopsy-proven iris melanoma: a multicenter international study.

OBJECTIVE: To collaborate with multiple centers to identify representative epidemiological, clinical, and pathologic characteristics of melanoma of the iris. This international, multicenter, Internet-assisted study in ophthalmic oncology demonstrates the collaboration among eye cancer specialists to stage and describe the clinical and pathologic characteristics of biopsy-proven melanoma of the iris. METHODS: A computer program was created to allow for Internet-assisted multicenter, privacy-protected, online data entry. Eight eye cancer centers in 6 countries performed retrospective chart reviews. Statistical analysis included patient and tumor characteristics, ocular and angle abnormalities, management, histopathology, and outcomes. RESULTS: A total of 131 patients with iris melanoma (mean age, 64 years [range, 20-100 years]) were found to have blue-gray (62.2%), green-hazel (29.1%), or brown (8.7%) irides. Iris melanoma color was brown (65.6%), amelanotic (9.9%), and multicolored (6.9%). A mean of 2.5 clock hours of iris was visibly involved with melanoma, typically centered at the 6-o'clock meridian. Presentations included iritis, glaucoma, hyphema, and sector cataract. High-frequency ultrasonography revealed a largest mean tumor diameter of 4.9 mm, a mean maximum tumor thickness of 1.9 mm, angle blunting (52%), iris root disinsertion (9%), and posterior iris pigment epithelium displacement (9%). Using the American Joint Commission on Cancer-International Union Against Cancer classification, we identified 56% of tumors as T1, 34% of tumors as T2, 2% of tumors as T3, and 1% of tumors as T4. Histopathologic grades were G1-spindle (54%), G2-mixed (28%), G3-epithelioid (5%), and undetermined (13%) cell types. Primary treatment involved radiation (26%) and surgery (64%). Kaplan-Meier analysis found a 10.7% risk of metastatic melanoma at 5 years. CONCLUSIONS: Iris melanomas were most likely to be brown and found in the inferior quadrants of patients with light irides. Typically small and unifocal, melanomas are commonly associated with angle blunting and spindle cell histopathology. This multicenter, Internet-based, international study successfully pooled data and extracted information on biopsy-proven melanoma of the iris.

Current treatments for radiation retinopathy.

BACKGROUND: to review the currently available therapeutic modalities for radiation retinopathy (RR), including newer investigational interventions directed towards specific aspects of the pathophysiology of this refractory complication. METHODS: a review of the literature encompassing the pathogenesis of RR and the current therapeutic modalities available was performed. RESULTS: RR is a chronic and progressive condition that results from exposure to any source of radiation. It might be secondary to radiation treatment of intraocular tumors such as choroidal melanomas, retinoblastomas, and choroidal metastasis, or from unavoidable exposure to excessive radiation from the treatment of extraocular tumors like cephalic, nasopharyngeal, orbital, and paranasal malignancies. After the results of the Collaborative Ocular Melanoma Study, most of the choroidal melanomas are being treated with plaque brachytherapy increasing by that the incidence of this radiation complication. RR has been reported to occur in as many as 60% of eyes treated with plaque radiation, with higher rates associated with larger tumors. Initially, the condition manifests as a radiation vasculopathy clinically seen as microaneurysms and telangiectases, with posterior development of retinal hard exudates and hemorrhages, macular edema, neovascularization and tractional retinal detachment. Regrettably, the management of these eyes remains limited. Photodynamic therapy, laser photocoagulation, oral pentoxyphylline and hyperbaric oxygen have been attempted as treatment modalities with inconclusive results. Intravitreal injections of anti-vascular endothelial growth factor such as bevacizumab, ranibizumab and pegaptanib sodium have been recently used, also with variable results. DISCUSSION: RR is a common vision threatening complication following radiation therapy. The available therapeutic options are limited and show unsatisfactory results. Further large investigative studies are required for developing better therapeutic as well as preventive treatment strategies.

Surgical treatment of ocular toxocariasis: anatomic and functional results in 45 patients.

PURPOSE: To evaluate the anatomic and functional outcome after surgery in patients with complicated ocular toxocariasis. METHODS: A retrospective review of the medical records of pediatric patients who underwent a surgical procedure for ocular toxocariasis from July 1990 to January 2005. Patients with postoperative follow-up shorter than 6 months were excluded from the study. Best-corrected visual acuity (BCVA) was arbitrarily divided into 5 groups: 1) no light perception (NLP); 2) light perception (LP) and hand motion (HM); 3) finger counting (FC) to 20/400; 4) 20/300 to 20/60; and 5) 20/50 to 20/20. RESULTS: Forty-five patients with a mean age of 8.1 years (range 6-10) were included in the study. The ocular toxocariasis presentation most commonly encountered was peripheral granuloma (38%). Twenty-one eyes presented with BCVA in the LP/HM group, 18 (40%) eyes in the FC to 20/400 group, 5 (12%) eyes in the 20/300 to 20/60 group, and 1 (5%) eye in the 20/50 to 20/20 group. The most common surgical procedure was pars plana vitrectomy (PPV) in 58% of the eyes. Four eyes (9%) presented with postoperative BCVA of NLP, 2 eyes (4%) in the LP/HM group, 12 eyes (27%) in the FC to 20/400 group, 19 eyes (42%) in the 20/300 to 20/60 group, and 8 eyes (18%) in the 20/50 to 20/20 group. CONCLUSIONS: Surgical treatment of severe ocular complications secondary to toxocara infections results in satisfactory anatomic results and may improve the overall visual outcome of these patients.

Ocular parasitic diseases: a review on toxocariasis and diffuse unilateral subacute neuroretinitis.

Parasitic infections may damage various ocular tissues, thereby causing visual dysfunction. In 1950, Wilder described the first case in which larval forms of nematodal intestinal roundworms (Ascaridoidea: Ascaris, Toxocara, Ancylostoma, Necator, and Strongyloides) were implicated as a cause of intraocular disease. This review focuses on two disorders associated with parasitic infections: ocular toxocariasis and diffuse unilateral subacute neuroretinitis.

Selective and pan-blockade agents in the anti-angiogenic treatment of proliferative diabetic retinopathy: a literature summary.

Diabetes mellitus is a major health concern in the modern world. Several sight-threatening ocular conditions are included in the array of health problems associated with this disease. Understandably, 2 of the more sight-threatening problems, proliferative diabetic retinopathy (PDR) and diabetic macular edema (DME), have received a great deal of attention in recent years. Pivotal studies, such as the Early Treatment Diabetic Retinopathy Study and the Diabetic Retinopathy Study, have established laser photocoagulation as the accepted treatment modality. The last decade has seen a surge in clinical data supporting the use of pharmacologic therapy in place of the often damaging laser therapy. Supporting data are based on the establishment of vascular endothelial growth factor (VEGF) as a key facilitator of disease progression in diabetic retinopathy. We will discuss the advantages and disadvantages of both selective and pan-blockade anti-VEGF agents available today in an effort to help guide physicians wishing to use these agents to treat PDR and DME.

Microperimetry findings in patients with birdshot chorioretinopathy.

OBJECTIVE: To assess the role of microperimetry-1 (MP1) as an ancillary tool in patients with birdshot chorioretinopathy (BSCR). DESIGN: Observational cross-sectional study. PARTICIPANTS: Twenty-three eyes of 23 patients. METHODS: A review of medical records was conducted of patients with BSCR seen at our institution, from January 2008 to August 2008, on whom MP1 had been performed. Of the 23 eyes included in the study, 15 eyes were identified as having HLA-A29 positive BSCR; 8 eyes with no known ocular pathology were used in the analysis as the control group. The clinical status was assessed by biomicroscopy, indirect ophthalmoscopy, and fluorescein angiography. RESULTS: When eyes with active disease were compared with eyes with inactive disease there was a statistically significant difference (p = 0.001) between them in the number of points below 16 dB. The difference was also statistically significant (p = 0.04) when it was adjusted for visual acuity, associated disease, and age. When eyes of patients with inactive disease were compared with control eyes, there was a statistically significant difference (p = 0.01) in the number of points below 16 dB, suggesting that not all patients may recover their full retinal sensitivities. When eyes of patients with active disease were compared with controls there was a statistically significant difference (p = 0.01) between them in the number of points below 16 dB after adjusting for age, visual acuity, and associated disease (macular edema and epiretinal membrane). CONCLUSIONS: Microperimetric quantification of macular sensitivity in patients with BSCR may provide an ancillary tool to evaluate activity and may help to assess visual impairment in these patients.