The vascular tumors of the retina and choroid comprise a diverse group of congenital and acquired lesions. The major vascular tumors of the retina include retinal capillary hemangioma, cavernous hemangioma of the retina, retinal vasoproliferative tumor, and racemose hemangiomatosis of the retina or Wyburn-Mason syndrome. Choroidal vascular tumors include circumscribed choroidal hemangioma and diffuse choroidal hemangioma. While classified as benign, visual symptoms secondary to exudative retinal detachment and a variety of other mechanisms are common and are a major source of long-term visual disability. While many therapeutic modalities exist, treatment of symptomatic cases can be challenging. Of particular importance, many of the vascular tumors of the retina and choroid have significant associations with systemic disease. As ocular symptoms are often the most common presenting disease manifestation, the ophthalmologist plays an important role in accurate and early diagnosis. The ability to initiate prompt screening and treatment in appropriate cases is critical. In the following article, the key clinical and diagnostic features of the major retinal and choroidal vascular tumors, their systemic associations, and the literature pertaining to the most currently available treatment strategies are reviewed.

Purpose : To establish the characteristics of secondary retinal and retinal pigment epithelial (RPE) changes associated with the presence of choroidal melanoma and choroidal nevus as documented by optical coherence tomography (OCT) and fundus autofluorescence (FAF). Materials and Methods : PubMed review of major English publications examining the correlation between clinical characteristics of choroidal melanoma and nevus with OCT and FAF findings. Results : The intrinsic properties of choroidal melanoma, as well as overlying RPE changes, drusen, and lipofuscin are best characterized by FAF, while OCT is more sensitive for the identification of subretinal and intraretinal fluid as well as atrophy, degeneration, and photoreceptor loss in the neurosensory retina. Conclusions : Secondary retinal changes associated with choroidal melanocytic lesions can be documented by OCT and FAF. OCT-evident changes are observed more often with choroidal melanoma than choroidal nevus. OCT is better suited to identify the overlying retinal detachment and edema, even before these findings are clinically apparent. FAF is most useful in documenting the presence of lipofuscin, a finding that represents one of the important criteria in differentiating small choroidal melanoma from benign choroidal nevus.

Purpose and Design: To describe an unusual clinical finding seen in children undergoing intra-arterial chemotherapy for retinoblastoma. Materials and Methods: A retrospective review of 69 eyes of 63 patients receiving intra-arterial chemotherapy over a 3-year period. Charts and photographs of 69 consecutive cases were reviewed, and data were collected on patients with clinical evidence of a hyperemic cutaneous periocular abnormality following the procedure. Results: A blanching erythematous and edematous patch was noted in the periocular region in 16% (11 of 69) of the children who received intraarterial chemotherapy. The plaque extended into the region of the supertrochlear and medial marginal artery distribution on the ipsilateral side of the intra-arterial chemotherapy. All patches of erythema spontaneously resolved within 3 months following completion of the intra-arterial chemotherapy. Conclusion: Periocular erythema and swelling is a self-limited clinical finding associated with intra-arterial chemotherapy in a small number of patients.

Surgical resection of uveal melanomas is an alternative eye-salvaging approach to the more commonly used irradiation techniques. There are two surgical resection techniques: Transscleral resection or "Exoresection" via a partial lamellar sclerouvectomy and "Endoresection" via a pars plana vitrectomy. While exoresection is more applicable to anteriorly located tumors with ciliary body and/or iris involvement, endoresection is more suitable for posteriorly located tumor without ciliary body involvement. Both approaches are suitable for large tumors with >8 mm in thickness. In general, eyes containing these large tumors have a very dismal prognosis regarding long-term visual function, eye retention, and irradiation-induced side effects. By removing the tumor burden from the eye, histopathologic and cytogenetic information of the tumor is available and complications associated with the so-called toxic tumor syndrome are avoided. However, both types of surgical resection are challenging surgical procedures, bearing the risk of early and late postoperative complications.

Retinoblastoma (RB) is an embryonic neoplasm of retinal origin. For many years, scientists have sought the fundamental origins of tumorigenesis, with the ultimate hope of discovering a cure. Indeed, these efforts have led to a significant understanding that multiple molecular and genetic aberrations, such as uncontrolled proliferation and the inhibition of apoptosis that contribute to the canonical characteristics of tumor biology. Despite these advances, a thorough understanding, such as the precise cells, which are the targets of neoplastic transformation, especially in solid tumors, is currently lacking. The focus of this review is to emphasize the molecular defects involved in the RB tumor progression and mechanisms associated with inhibition of tumor cell apoptotic processes. This review also discusses the importance of target molecules characterization and their potential therapeutic or prognostic use in RB disease.

Idiopathic juxtafoveolar retinal telangiectasis (IJFT), also known as parafoveal telangiectasis or idiopathic macular telangiectasia, refers to a heterogeneous group of well-recognized clinical entities characterized by telangiectatic alterations of the juxtafoveolar capillary network of one or both eyes, but which differ in appearance, presumed pathogenesis, and management strategies. Classically, three groups of IJFT are identified. Group I is unilateral easily visible telangiectasis occurring predominantly in males, and causing visual loss as a result of macular edema. Group II, the most common, is bilateral occurring in both middle-aged men and women, and presenting with telangiectasis that is more difficult to detect on biomicroscopy, but with characteristic and diagnostic angiographic and optical coherence tomography features. Vision loss is due to retinal atrophy, not exudation, and subretinal neovascularization is common. Group III is very rare characterized predominantly by progressive obliteration of the perifoveal capillary network, occurring usually in association with a medical or neurologic disease. This paper presents a current review of juxtafoveolar retinal telangiectasis, reviewing the classification of these entities and focusing primarily on the two most common types encountered in clinical practice, i.e., groups I and II, describing their clinical features, histopathology, natural history, complications, latest results from imaging modalities and functional studies, differential diagnosis, and treatment modalities.

Objective and Design: A retrospective review study was designed to describe five cases of optic disc melanocytomas with tumor-related visual impairment. Participants: Five patients with optic disc melanocytoma presented with visual complaints to a tertiary eye hospital in Saudi Arabia. Materials and Methods: Demographic and clinical data were analyzed, including the results of ocular examination, lesion laterality, best-corrected Snellen visual acuity, pupillary reflex, visual field testing, color fundus photography, fundus fluorescein angiography, and ophthalmic ultrasound. Results: Visual dysfunction secondary to optic disc melanocytoma was identified. Case 1 had macular star edema with mild tumor enlargement, Case 2 had optic atrophy, Case 3 had juxtapapillary choroidal neovascular membrane with macular involvement, Case 4 had optic disc swelling with an enlarged blind spot, and Case 5 had a large altitudinal visual field defect. Conclusion: Although melanocytomas of the optic disc tend to have a benign behavior with slow evolution and stable vision, they may adversely affect visual function through a variety of mechanisms.

Purpose: This study was designed to determine the rate and pattern of vitreo-retinal diseases at a tertiary eye care center in South-eastern Nigeria. Materials and Methods: The outpatient register at the Eye Clinic of the University of Nigeria Teaching Hospital, Enugu, was retrospectively examined to identify all new patients registered between January 2004 and December 2008. A chart review of subjects with vitreo-retinal disease was conducted to record relevant demographic and clinical data including the needs for vitreo-retinal care. Descriptive and analytical statistics were performed. A P-value <0.001 (one degree of freedom) was considered statistically significant. Results: Of the 8,239 new patients reported during the period, 326 subjects (males- 59.3%; females- 40.7%; sex ratio = 1.1:1) aged 49.3 ± 16.8 years (range 3-82 years) had vitreo-retinal disease. The rate of vitreo-retinal disease was 3.9%. The rate was higher in subjects above 40 years old (P < 0.001), but did not differ between sexes (P = 0.469). Diabetic retinopathy (24.9%), hypertensive retinopathy (13.3%), and age-related macular degeneration (10.7%) were the leading vitreo-retinal diseases. Blindness from vitreo-retinal disease was bilateral in 6.1% of subjects and unilateral in 17.5% of subjects. The common co-morbidities were ocular conditions such as refractive error (19.8%), cataract (14.2%), and glaucoma (10.4%); and systemic conditions such as diabetes mellitus (14.6%) and hypertension (13.2%). Conclusions: The rate of vitreo-retinal diseases among new ophthalmic outpatients at UNTH, Enugu, is 3.9%. Retinal vascular disorders and age-related maculopathy are the leading retinal diseases. At UNTH, resource needs for vitreo-retinal care are urgent including retinal photography/angiography, laser photocoagulation, intra-vitreal pharmacotherapy, and vitreo-retinal surgery.

Purpose: To compare the functional and anatomical outcomes following intravitreal triamcinolone acetonide vs. intravitreal bevacizumab (Avastin) treatment for diffuse diabetic macular edema. Materials and Methods: In this prospective, randomized study, subjects were divided into two groups: 24 eyes that received intravitreal injection of 4 mg/0.1 mL triamcinolone acetonide (IVTA group) and 24 eyes received intravitreal injection of 1.25 mg/0.05 mL bevacizumab (IVB group). Changes in best corrected visual acuity (BCVA), intraocular pressure (IOP), baseline fluorescein angiography and optical coherence tomography measurements were evaluated in both groups. Follow-up visits out to three months from baseline are reported. Results: One month after treatment, baseline foveal thickness decreased from 452 μ to 299 μ in the IVTA group and from 292 μ to 270 μ in the IVB group. BCVA increased by two or more lines in 58.3% of eyes in the IVTA group and there was no similar improvement in the IVB group. In the IVTA group, a transient increase in IOP (27-43 mmHg) occurred in four cases (16.7%), which was successfully controlled with topical medications. There were no complications in the IVB group. Conclusion: Short term outcomes indicate that intravitreal injection of bevacizumab was not associated with surgical complications compared to triamcinolone acetonide. Triamcinolone acetonide appears to be more effective treatment for diabetic macular edema than bevacizumab.

Purpose: To describe six patients (six eyes) who developed an eccentric macular hole after surgery for idiopathic epimacular proliferation. Materials and Methods: Review of records from six patients who developed eccentric macular holes postoperatively following vitrectomy in 107 consecutive cases with peeling of the epimacular proliferation and internal limiting membrane (ILM) from June 2004 to January 2009, Results: Eccentric macular holes were developed from nine days to eight months (mean, 3.1 months) after epimacular proliferation peeling. The ILM was peeled in addition to the epimacular proliferation in five of the six cases. Of the six eccentric macular holes, four were located temporal to the fovea, one was located superior to the fovea, and one was located nasal to the fovea. Final visual acuities after a mean follow-up period of 17.3 months were 20/20 in two eyes, 20/25 in one eye, 20/40 in two eyes, and 5/200 in one eye. The eye with the eccentric macular hole nasal to the fovea had the poorest final visual acuity of 5/200. Conclusion: Eccentric macular holes occurring after vitrectomy to remove epimacular proliferation is an uncommon postoperative finding. Various explanations have been suggested for the etiology of these holes, but there is no consensus. We suggested that the ILM tear should be initiated with a diamond dusted knife to reduce the likelihood of injury to the underlying Muller cells that may contribute to the formation of eccentric macular holes.

Purpose: To evaluate the prevalence of diabetic retinopathy (DR) in the urban and rural areas of Al Hasa region of Saudi Arabia and to determine risk factors related to DR. Materials and Methods: This study was conducted on patient attending primary health care centers between July 2007 and June 2009. A retrospective chart review was conducted on subjects with diabetes mellitus greater than 18 years old. Ophthalmologists examined DR status through dilated pupils by using direct, indirect, and slit lamp bio-microscopy. Frequencies, percentage, and their 95% confidence intervals (CIs) were calculated. Odd's ratio was used to associate DR with possible risk factors. A P value less than 0.05 was considered statistically significant. Results: The prevalence of DR among 473 diabetic subjects was 30% (95% CI: 25.80-34.20). The odd ratios (ORs) of DR among diabetic residing in an urban area was significantly higher than diabetics residing in rural areas [OR = 1.94 (95% CI of OR 0.82-2.89)]. DR was associated to the duration of diabetes (adjusted OR = 1.70), uncontrolled blood sugar level (adjusted OR = 1.96), hyperlipidemia (adjusted OR = 2.04), and hypercholesterolemia (adjusted OR = 2.80). Conclusions: DR appears to be a public health problem in the Al Hasa district of Saudi Arabia, and a planned approach is required to avoid severe visual impairment in patients with diabetes mellitus. Primary prevention and early detection could be implemented through primary health centers and non-ophthalmologists.

Objective: This study was designed to present our early experience with intravitreal bevacizumab combined with laser treatment for retinopathy of prematurity (ROP) at a single institution over a 13-month-period. Methods: A retrospective case series of eight children with ROP who received intravitreal bevacizumab combined with laser treatment between June 2007 and July 2008 were reported. A chart review was conducted to evaluate if stability of the ROP lesion had been achieved. Main information collected included data on demographics, gestational age, birth weight, length of stay in neonatal intensive care unit, and stage of ROP. Results: Fifteen eyes of eight subjects were treated. One eye did not receive any treatment due to complete retinal detachment. The median age at treatment was 8 weeks (range, 6 weeks to 1 year). The most common stage of ROP was 3+. All eyes remained stable at 1 year or later after treatment. Conclusion: Intravitreal bevacizumab in conjunction with laser treatment had promising results at our institution. We recommend prospective, randomized, controlled clinical trials to compare the effect of laser treatment alone, of bevacizumab treatment alone (at different doses), and of combined bevacizumab and laser treatment.

Purpose: To report a patient with small juxtapapillary choroidal melanoma with chromosome 3 monosomy treated with I ¹²⁵ plaque and transpupillary thermotherapy (TTT). A 64-year-old Caucasian male presented with painless blurred vision of the left eye. Ocular examination disclosed a small juxtapapillary choroidal melanocytic tumor with overlying subretinal fluid and orange pigment. Ultrasound showed an elevated choroidal mass of 2 mm thickness with low reflectivity on A-scan and hollowness on B scan, consistent with a small choroidal melanoma. The patient was treated with plaque I ¹²⁵ radiotherapy combined with one session of TTT. Genetic testing of the tumor cells obtained by fine needle aspiration biopsy showed chromosome 3 monosomy. At 1 year after treatment, the tumor was regressed with resolution of subretinal fluid and 20/40 visual acuity. A small choroidal melanoma can manifest monosomy of chromosome 3, a known predictive factor for the development of systemic metastasis.

We report a case of multiple evanescent white dot syndrome (MEWDS) that presented with putative idiopathic choroidal neovascularisation (ICNV) before showing angiographic signs typical of MEWDS. A 16-year-old male presented with unilateral metamorphopsias and visual loss in his left eye. ICNV with subretinal hemorrhage was diagnosed and treated with intravitreal Avastin^® . Fifteen days later, regression of choriodal neovascularization (CNV) was documented together with the appearance of fluorescein angiography (FA) and indocyanine green angiography (ICGA) signs typical for MEWDS, that included faint mottled FA hyperfluorescence in the mid-peripheral fundus, irregularly shaped mid-peripheral ICGA dark areas in the intermediate angiographic phase that were clearly delineated in the late phase as well as peripapillary hypofluorescence. Fundus examination appeared completely normal during the follow-up except for the CNV hemorrhage noted at the initial visit. This case demonstrates the need to consider ICNV as a diagnosis of exclusion until inflammatory causes have been eliminated. In this case, the underlying occult inflammatory condition would have been missed without the ICGA data that clearly showed signs of MEWDS that was supported by FA findings.

Isolated cilioretinal artery occlusion is a rarely reported initial manifestation of polycythemia vera. In this study, we reported a case of a 65-year-old man with polycythemia vera with cilioretinal artery occlusion as an initial manifestation.

Optic disc pit is an excavation of the optic nerve head usually seen in association with other abnormalities of the optic nerve, peripapillary retina, or posterior vitreous detachment. In 50% the cases, it might be associated with serous macular detachment. The prevailing theory that explains this disorder is that subretinal fluid which is derived from liquefied vitreous passes through the optic disc pit and elevates the macula. In this study, we report a case of serous macular detachment complicating optic disc pit in a young male patient treated surgically without internal limiting membrane peeling and showed dramatic improvement of vision after 1 year.

Despite initial concerns regarding systemic complications, the use of intravitreal antivascular endothelial growth factor (anti-VEGF) agents for ocular disease is rapidly expanding worldwide, in terms of both the number of patients injected and its indications. To our knowledge, there are no cases in the literature reporting erectile dysfunction following the use of intravitreal bevacizumab. We postulate an organic mechanism for impaired erectile function due to systemically absorbed intravitreal bevacizumab. We describe a case of erectile dysfunction following intravitreal bevacizumab administration. Color fundus photos, fluorescein angiogram and optical coherence tomography images are presented. A 40-year-old male underwent intravitreal bevacizumab therapy for macular edema secondary to a branch retinal vein occlusion. He subsequently developed transient erectile dysfunction after each of his two bevacizumab injections. His only comorbidity was mild hypertension. Erectile dysfunction may be a side effect of intravitreal bevacizumab. The erectile dysfunction could be organic and/or psychogenic in etiology.