RETINAL ASTROCYTIC HAMARTOMA: Optical Coherence Tomography Classification and Correlation With Tuberous Sclerosis Complex.

PURPOSE: To propose a classification of retinal astrocytic hamartoma based on spectral domain optical coherence tomography and correlate each class with systemic manifestations of tuberous sclerosis complex. METHODS: Retrospective chart review conducted at four international referral medical retina centers. There were 43 consecutive patients with an established diagnosis of tuberous sclerosis complex based on presence of at least 2 major or 1 major and 2 minor features of the diagnostic criteria. Clinical and spectral domain optical coherence tomography features regarding retinal astrocytic hamartoma were documented. RESULTS: The mean patient age at presentation was 16.2 years. The retinal astrocytic hamartoma was classified as Type I (n = 41), Type II (n = 25), Type III (n = 20), or Type IV (n = 12). Patients with Type II showed greater number of cutaneous fibrous plaques (odds ratio = 64.8; 92% confidence interval: 64.2-65; P < 0.001); those with Type III displayed higher incidence of subependymal giant-cell astrocytomas (odds ratio = 43.2; 95% confidence interval: 43.0-43.3; P < 0.001); and those with Type IV showed higher incidence of pulmonary lymphangiomyomatosis (odds ratio = 126; 95% confidence interval: 122-128; P < 0.001). CONCLUSION: Retinal astrocytic hamartoma can be classified into four morphologic groups, based on spectral domain optical coherence tomography. There are important systemic tuberous sclerosis complex correlations with each class.

Diabetic retinopathy: current and new treatment options.

Diabetes mellitus has become a major health concern worldwide and its incidence is projected to increase. Diabetic macular edema (DME) and proliferative diabetic retinopathy (PDR) are considered the most sight-threatening ocular complications in these patients. Pivotal studies, such as the Early Treatment Diabetic Retinopathy Study (ETDRS) and the Diabetic Retinopathy Study (DRS), have established macular and pan-retinal laser as the gold-standard of treatment for these complications. The recent discovery of the vascular endothelial growth factor (VEGF) and its role in the development of proliferative disease, has led to a movement towards treating PDR and DME with anti-angiogenic medications alone or in conjunction with the gold-standard of care. Due to the severity of the diabetic ocular complications and the rising incidence of diabetes worldwide, it is important for the non-ophthalmologist care provider to be informed of the new treatments available for these conditions in an effort to better guide their patients. In this review, I will discuss the importance of these new methods of treatment as well as the significance of systemic glucose control, vitreous surgery and laser photocoagulation.

Diabetic papillopathy: current and new treatment options.

Diabetes mellitus is a growing global epidemic. Patients with this disease present with a variety of health conditions, including a number of ocular complications that threaten vision, such as proliferative diabetic retinopathy and macular edema. Diabetic papillopathy, another potential ocular complication from diabetes, is a self-limiting, sometimes bilateral disease that may affect both type 1 and type 2 diabetics. It is characterized by optic disc swelling caused by vascular leakage and axonal edema in and around the optic nerve head. Occasionally, it may be accompanied by intraretinal hemorrhages and hard exudates. Diabetic papillopathy tends to be mild and is usually associated with good visual prognosis; however, there are some cases in which permanent visual impairment can develop. The pathogenesis remains largely unknown, but there has been evidence suggestive of its associations with a small cup/disc ratio and rapid reduction in glycemia. There is no validated therapy for diabetic papillopathy; however, current case reports have shown promising results after local injections of corticosteroids as well as bevacizumab (Avastin), a potent monoclonal antibody that has been employed for the treatment of ocular vaso-proliferative diseases such as choroidal neovascular membranes associated with age-related macular degeneration and proliferative diabetic retinopathy.

Confluent laser photocoagulation for the treatment of retinopathy of prematurity.

PURPOSE: To describe the efficacy, outcomes, and complications of confluent laser technique in a cohort of premature infants with threshold retinopathy of prematurity. Laser photocoagulation has a good treatment outcome in the management of retinopathy of prematurity; however, the number and density of laser spots remains debatable. Laser treatment can be done in a scattered pattern, a near-confluent pattern, or a confluent treatment pattern. METHODS: A retrospective chart review of patients with threshold retinopathy of prematurity treated between 2003 and 2006 was conducted. Confluent laser treatment was applied anterior to the ridge extending to the ora serrata 360 degrees . Rate of progression, frequency of retreatment, postoperative complications, structural outcomes, and refractive error were evaluated. RESULTS: A total of 100 eyes from 51 patients were included. Mean gestational age was 28 weeks (range: 23 to 32 weeks) and mean birth weight was 1,065 g (range: 477 to 1,905 g). Patients had a mean follow-up of 13 months (range: 6 to 50 months). Progression to stage 4 or 5 occurred only in a total of 6 eyes (6%). Postoperative complications included cataract, vitreous hemorrhage, corneal edema, and macular dragging. Mean spherical equivalent at the last follow-up visit was -3.80 diopters (range: -19.00 to+4.00 diopters). CONCLUSION: Patients treated with confluent laser photocoagulation had a low rate of progression to stage 4 or 5 retinopathy of prematurity. Also, the need for additional laser treatment was small, with rates of complications and structural outcomes comparable to previous reports using a nonconfluent laser pattern.

Current trends in the management of ocular symptoms in Adamantiades-Behçet's disease.

Adamantiades-Behçet's disease (ABD) is a multisystemic vasculitic disease. It is most prevalent in the Eastern Mediterranean countries and the Eastern region of Asia. Its effect on the eye can range from mild to debilitating, resulting in total blindness. A necrotizing and obliterative vasculitis affects both arteries and veins of organs. Recurrent attacks of uveitis, oral aphthous ulcers, skin lesions, and genital ulcers are common. Topical and systemic corticosteroids have been the mainstay in the treatment of ocular inflammation for many years; however, due to the several known side effects of corticosteroids and thanks to scientific advances, more novel approaches to ABD treatment have been emerging. Antimetabolites such as methotrexate and azathioprine have been utilized with the latter showing positive results. Chlorambucil has been utilized effectively for ocular manifestations of ABD. Interferon alpha has shown encouraging results in the management of refractory ocular inflammation associated with ABD, either alone or in combination with other immunosuppressive agents. Surgical interventions to deal with complications from ABD can be safely done if adequate control of inflammation is achieved peri-operatively. Early detection and aggressive treatment, when needed, have proven to be essential in the management of this relentlessly explosive disease.

Pegaptanib sodium for the treatment of proliferative diabetic retinopathy and diabetic macular edema.

Diabetes mellitus is a growing health concern world-wide. Patients with this disease present with a variety of health conditions, including a number of sight-threatening ocular pathologies. Proliferative diabetic retinopathy (PDR) and diabetic macula edema (DME) are common diseases that cause substantial vision impairment in diabetic patients. There has been a strong focus on studying the epidemiology and treatment of these diseases. The recent discovery of vascular endothelial growth factor (VEGF) and its role in the development of proliferative disease, has led to a movement towards treating PDR and DME with anti-angiogenic medications in conjunction with the standard of care. In this review we present a summary of the origination and progression of PDR and DME. This will be followed by a review of clinical data surrounding new anti-angiogenic treatment modalities.

Choroidal melanoma masquerading as panuveitis in a patient with multiple myeloma.

BACKGROUND: Choroidal melanoma can sometimes present with associated anterior uveitis or panuveitis that can mask the underlying intraocular neoplasm and confound the diagnosis. METHODS: Retrospective chart review. RESULTS: A 58-year-old woman presented with recurrent episodes of hypopyon, severe panuveitis, and a choroidal mass lesion in the right eye. Extensive systemic workup led to a diagnosis of multiple myeloma. Despite successful treatment of myeloma, the choroidal mass persisted and increased in size. Transscleral biopsy of the mass lesion revealed primary malignant choroidal melanoma. CONCLUSION: This case demonstrates that choroidal melanoma can present as masquerade syndrome with significant intraocular inflammation. Such cases pose a significant diagnostic dilemma to the clinician. Transscleral choroidal biopsy can provide critical information that is essential to establishing a definitive diagnosis and treatment plan.

Ocular cysticercosis of the anterior segment.

A 6-year-old girl presented with signs of severe anterior uveitis. After initiating treatment, a cyst like lesion was observed in the anterior chamber that led to the diagnosis of ocular cysticercosis that was ultimately confirmed with histopathologic analysis. Ocular cysticercosis usually affects the extraocular muscle. Infection of the anterior chamber has been described, although to a lesser extent. Because of the usually poor visual prognosis and the controversy in treatment, physicians should be aware of this disease and its different variants of presentation.