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PURPOSE To report a case of a good visual outcome in a patient with bilateral multifocal syphilitic chorioretinitis, despite the late diagnosis. METHODS Ophthalmologic examination, multimodal imaging, including fundus photography, angiography, and optical coherence tomography. RESULTS The authors describe a 47-year-old heterosexual man with a bilateral multifocal syphilitic chorioretinitis that was lately diagnosed and despite that had a good visual outcome. CONCLUSION The diagnosis of ocular syphilis is a challenge; however, once it has been made, even lately, it is eminently treatable.

Background and Aims: Metabolic disorders can present ophthalmologic changes. Congenital Generalized Lipodystrophy (CGL) is characterized by severe metabolic manifestations such as insulin resistance, diabetes and hypertriglyceridemia, but there are few data published about ophthalmologic findings in this condition. The purpose of this study is to describe ocular abnormalities in patients with CGL. Materials and Methods: It was a cross sectional study with 15 patients with CGL of both sex, aged between 2 and 29 years old. We evaluated the symptoms of surface eye disease and visual acuity. In the slit lamp, were evaluated the anterior segment of the eye, the break up time of the tear film with fluorescein and the corneal findings. Under mydriasis, the eye fundus examination was performed. Results: All subjects had dyslipidemia: 15/15 (100%) hypertriglyceridemia; 15/15 (100%) low HDL-c; 4/15 (26,7%) and high levels of LDL-c. Diabetes was presented in 7/15 (46,7%) cases. Symptoms of surface eye disease (blurred vision, pruritus, hyperemia or dry eye sensation) were presented in 9/15 (60%) patients and 8/15 (53%) had refractive errors: 5/8 (62,5%) astigmatism, 2/8 (25%) myopia and 1/8 (13%) myopia and astigmatism. In the slit lamp, 12/15 (80%) presented anterior blepharitis (seborrheic or meibomite), 13/15 (87%) decrease in the break up time of the tear (less than 8 seconds) and 5/15 (33,3%) keratitis. In the fundoscopy, 2/15 (13,3%) presented retinopathy (one with nonproliferative diabetic retinopathy and another presented proliferative diabetic retinopathy). Conclusion: These findings demonstrate high frequency of blepharites and its complications in patients with CGL, even in young subjects without diabetes. These data allow us to speculate that the presence of abnormalities in the anterior segment of the eye may be a marker of metabolic disorders as dyslipidemia and insulin resistance. Disclosure V.O. Fernandes: None. L.M.A. Gomes: None. A.D.R. Montenegro: None. C.M.M. Ponte: None. L.A.A. Batista: None. R.E.M. Aragão: None. J.V. Silva: None. R.M. Montenegro: None.

Adult-onset foveomacular vitelliform dystrophy is a rare disease. It shares heritance features with Best disease. Its onset is in the 3rd and 5th decade, and it is characterized by subretinal deposition of yellowish material in the foveal area. Visual acuity ranges from 20/25 to 20/50, which can be seen in routine examination. Patient remains with good visual function throughout theirs lives. Typically the electro-oculogram may be normal or subnormal. We present a case of adult-onset vitelliform macular dystrophy, diagnosed in a patient with complaint of bilateral blurred vision.

Endogenous endophthalmitis is a rare, and frequently devastating, ophthalmic disease. It occurs mostly in immunocompromised patients, or those with diabetes mellitus, cancer or intravenous drugs users. Candida infection is the most common cause of endogenous endophthalmitis. Ocular candidiasis develops within days to weeks of fungemia. The association of treatment for pancreatitis with endophthalmitis is unusual. Treatment with broad-spectrum antibiotics and total parenteral nutrition may explain endogenous endophthalmitis. We report the case of a patient with pancreatitis treated with broad-spectrum antibiotics and total parenteral nutrition who developed bilateral presumed Candida endogenous endophthalmitis that was successfully treated with vitrectomy and intravitreal amphotericin B.

Optic disc pit is a congenital anomaly of the optic nerve frequently associated with macula detachment. It has a poor visual prognosis if left untreated. The treatment of the optic disc remains controversial and includes the use of laser along the edge of the optic nerve, vitrectomy with gas tamponade with or without removal of posterior hyaloid. We report a case of a 19-year-old female with a longstanding macula detachment due to optic disc pit who was treated with vitrectomy with removal of posterior hyaloid, gas tamponade, and laser. Despite longstanding macular detachment the final visual acuity was 20/25.

Susac's syndrome is a rare disease attribuited to a microangiopathy involving the arterioles of the cochlea, retina and brain. Encefalopathy, hearing loss, and visual deficits are the hallmarks of the disease. Visual loss is due to multiple, recurrent branch arterial retinal occlusions. We report a case of a 20-year-old women with Susac syndrome presented with peripheral vestibular syndrome, hearing loss, ataxia, vertigo, and vision loss due occlusion of the retinal branch artery.

Visceral Leishmaniasis, also know as Kala-azar, is a parasitic tropical disease caused by protozoa of the genus Leishmania donovani. It is an endemic disease in many countries. It affects approximately 1,5 million people every year, and when associated with mal-nutrition and co-infection it may be fatal. Fever, hepatosplenomegaly, and pancytopenia is its typical clinical picture. Ocular manifestations of Kalaazar are relatively rare and can affect either anterior or posterior segment of the eye. We report a patient with kala-azar presenting intraretinal hemorrhages that regress completely after the successful treatment for visceral leishmaniasis.

Dengue fever is a well known viral infection transmitted through the bite of an infected Aedes aegypti and Aedes albopictus mosquito. Its geographic distribution covers over 100 countries. Clinical presentation is variable and depends upon the age and immunological status of the patient. It can be classified as asymptomatic illness, dengue fever, dengue hemorrhagic or dengue shock syndrome. Ocular manifestations of dengue have been described, however there are few reports on neurological involvement. We hereby report two cases of bilateral optic neuritis after dengue viral infection. Both of them rapidly recovered the visual acuity without treatment.

Von Hippel-Lindau (VHL) e uma sindrome tumoral autossomica dominante. Esses tumores incluem hemangioblastoma da retina e sistema nervoso central (CSN), carcinoma de celulas renais, feocromocitoma, tumores de pâncreas, cistoadenoma de rins, pâncreas e epididimo. Os sintomas mais comuns sao perda da visao, aumento da pressao intracraniana, deficits neurologicos, aumento da pressao arterial sistemica paradoxal e dor local. Relatamos o caso de um paciente com perda de visao e historia de hemangiomas cerebelares cujo diagnostico de VHL foi feito apos exame oftalmologico.

O artigo descreve um caso de vasculopatia polipoidal idiopatica da coroide (VPIC) e demonstra o papel relevante da indocianinografia no diagnostico diferencial com outras causas de neovascularizacao subretiniana, particularmente degeneracao macular relacionada a idade (DMRI), bem como avalia os aspectos extremos na evolucao da doenca em um unico paciente. Paciente de 54 anos, sexo masculino, cor parda, com queixas de baixa de acuidade visual subita em OD, foi submetido a exame oftalmologico, pelo qual se constatou hemorragia vitrea em OD e, posteriormente, descolamento sero-hemorragico do epitelio pigmentario da retina em OE. A angiofluoresceinografia e indocianinografia mostraram quadro compativel com vasculopatia polipoidal idiopatica da coroide. O paciente foi submetido a vitrectomia via pars plana em OD e foi acompanhado por periodo de 5 meses, evoluindo com perda de visao secundaria a descolamento de retina e cicatriz macular disciforme em OD. No olho contralateral, ocorreu involucao da vasculopatia com preservacao de acuidade visual 20/25. A vasculopatia polipoidal idiopatica da coroide e entidade clinica cujas anormalidades do fundo de olho se sobrepoem as manifestacoes da degeneracao macular relacionada a idade exsudativa. A indocianinografia e de vital importância no diagnostico diferencial entre as duas doencas que tem evolucao e prognostico distintos. Alem disso, consideramos este caso muito ilustrativo pelas diferentes nuances com que a doenca se manifestou e evoluiu em um unico paciente.

Extraintestinal manifestations of Crohn's disease are common. Although ocular complications of Crohn's disease are infrequent, most ocular manifestations include iritis, uveitis, episcleritis, scleritis and conjuntivitis. We report a patient who developed uveitis two years before diagnose of Crohn's disease.

Siderosis bulbi is a sight-threatening condition characterized by a pigmentary and degenerative change in the eye that follows the intra-ocular retention of a foreign body containing iron. The main cause of ocular siderosis is penetrating trauma mostly by hammering a steel object without or with poor eye protection. Herein, we report a case of siderosis bulbi after penetrating trauma that was lately treated with vitrectomy to remove the foreign body, and despite treatment resulted in ocular globe atrophy.

Relatam-se os casos de dois irmãos consanguíneos com síndrome de Sjögren- Larsson, enfatizando a importância clínica do exame oftalmológico. BPLS, masculino, 11 anos e MBLS, feminino, 10 anos, irmãos de pais não-consanguíneos, apresentando ictiose congênita, diplegia espástica e retardo mental. Ao exame oftalmológico, apresentavam miopia, fotofobia, baixa acuidade visual. A fundoscopia, presença de cristais branco-amarelados em área foveal e parafoveal em ambos os olhos. Aconselhamento genético foi realizado. O manejo foi de suporte. A Síndrome de Sjögren-Larsson é uma rara doença autossômica recessiva em que há 100% de penetrância. Síndrome de Sjögren-Larsson é classicamente caracterizada por ictiose, espasticidade e deficiência mental. A doença é causada por mutações no gene aldeído desidrogenase. As alterações oculares observadas são geralmente bilaterais, cristais branco-amarelados em área retiniana, que aparecem nos dois primeiros anos de vida e que vão aumentando em número com a idade. As anormalidades oculares não têm relação com a severidade da ictiose ou com as anormalidades neurológicas. Acredita-se que as lesões oftalmológicas sejam um sinal patognomônico da síndrome. É necessário enfatizar a importância do diagnóstico precoce e possibilidades de tratamento dietético.

Tay-Sachs disease is an autosomal recessive disorder of sphingolipid metabolism, caused by enzime hexosaminidase A deficiency that leads to an accumulation of GM2 in neurocytes which results in progressive loss of neurological function. The accumulation of lipid in retinal ganglion cells that leads to a chalk-white appearance of the fundus called "cherry red spot" is the hallmark of Tay-Sachs disease. It is also seen in others neurometabolic diseases as well as in central retinal artery occlusion. This case reports a child with Tay-Sachs disease in a family with four previous similar deaths without diagnostic. Tay-Sachs é uma doença autossômica recessiva, caracterizada pela deficiência da enzima hexosaminidase A levando ao acúmulo de esfingolipídios (GM2) em células neuronais que resulta em uma perda progressiva da função neurológica. O acúmulo de lipídios em células ganglionais da retina leva a uma aparência de mácula em cereja, característica do fundo de olho de pessoas acometidas. "Mácula em cereja" também pode ser vista em outras doenças neurometabólicas e em oclusão da artéria central da retina. Este trabalho relata o caso de um paciente com doença de Tay-Sachs em uma família com história de quatro óbitos por causas semelhantes sem diagnóstico.

Cryptococcal meningitis is caused by the yeast Cryptococcus neoformans. Two varieties are recognized: var. gattii and var. neoformans. It is usually associated with immunosuppressive states, particularly HIV infection. Cryptococcal infection of the central nervous system is uncommon in immunocompetent children and difficult to diagnose. Ocular complications are common. Optic disc swelling was found in 33%. The following report describes a case of meningitis caused by C. neoformans var. gattii in an 8 year-old immunocompetent child who developed optic atrophy. The patient was treated with amphotericin B associated with corticosteroids. Possible therapeutic strategies aimed at reducing visual loss in cryptococcal meningitis have great importance to avoid this important morbidity.

Fundus flavimaculatus is a progressive, bilateral, hereditary retinal dystrophy characterized by ill-defined, yellowish, pisiform flecks at the level of the retinal pigment epithelium. Since the disease process is at level of the retinal pigment epithelium, it is not surprising that subretinal macular neovascularizations might occur. Nevertheless, they have been rarely reported as complications of the disease. The following report describes a case of fundus flavimaculatus that progressed with blurred vision by a subretinal macular neovascularization.

Unilateral retinitis pigmentosa (URP) is a rare tapetoretinal dystrophy affecting only one eye. Criteria are necessary to make a correct diagnosis of URP: exclude all infective etiologies, check that the clinical signs of retinitis are present in the affected eye and the total absence of any signs or symptoms of retinitis pigmentosa in the fellow eye. Electroretinogram (ERG) and electro-oculogram (EOG) are useful for the correct diagnosis. In this paper we report one case.

Optic neuropathy due to cat scratch disease is a relatively infrequent occurrence associated with macular star formation and is characterized by sudden painless loss of vision mostly unilateral. Bartonella henselae is well recognized as the etiologic agent in cat scratch disease. Ocular complications of the disease occur in up to 10% of patients and include neuroretinitis. Ocular bartonelosis is usually self-limited with complete or near-complete recovery of vision in otherwise healthy patients. A case of a boy with neuroretinitis caused by B. henselae is reported.

Acute retinal necrosis (ARN) is an uncommon necrotizing, fulminant retinopathy caused by the herpes simplex virus types 1 or 2 or by the varicella zoster vírus with visually devastating consequences. Generally it occurs in patients who are systemically healthy, but occasionally occurs in immunocompromised host. We report a case of bilateral ARN in a patient with AIDS with a good final visual outcome.

Metastatic tumors are the most common intraocular malignances and choroid is by far the most common site. Breast and lung cancer are the first cause in women and men respectively. We report the case of a 71-year old woman who had choroidal tumor in her left eye. Further image body scans demonstrated several lesions in both sides of the lungs with dissemination to other organs. Diagnosis of a brochioloalveolar carcinoma established after a biopsy carried out. The patient died before initiating a proper treatment.