Susac's syndrome: isolated retinal artery occlusion after 10 years of remission / Síndrome de Susac: oclusão isolada da artéria retiniana após 10 anos de remissão

ABSTRACT Susac's syndrome, or microangiopathy of the retina, inner ear, and brain, is a rare condition characterized by the clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. The complete triad has been documented in 85% of reported cases. At clinical onset, the most common manifestations are central nervous system symptoms, followed by visual symptoms and hearing disturbances. Although the clinical course of Susac's syndrome is usually self-limiting, fluctuating, and monophasic, clinical polycyclic and chronic courses have also been described. Likewise, recurrences of the full triad after more than 10 years of remission have been reported. We describe a 21-year-old woman who presented with branch retinal artery occlusions and magnetic resonance imaging findings compatible with Susac's syndrome without objective hearing loss. After 10 years of remission, the patient complained of visual field loss due to new retinal ischemia. Neither other symptoms nor neuroimaging or audiometry pathologic findings were found during the clinical course.

RESUMO A síndrome de Susac, ou a micro angiopatia da retina, do ouvido interno e do cérebro, é uma condição rara caracterizada pela tríade clínica de encefalopatia, oclusão de ramo da artéria retiniana e perda de audição neuro-sensorial. A tríade completa é documentada em 85% dos casos registrados. No início dos sinais clínicos, a manifestação mais comum relaciona-se ao sistema nervoso central, seguida por sintomas visuais e distúrbios auditivos. Apesar do curso clínico da síndrome de Susac ser usualmente auto limitante, variável e monofásico, cursos clínicos policíclicos e crônicos têm sido também descritos. Do mesmo modo, recorrências da tríade completa após mais de 10 anos de remissão têm sido relatadas. Descrevemos o caso de uma mulher de 21 anos que apresentava oclusões de ramos da artéria retiniana e imagens por ressonância magnética compatíveis com a síndrome de Susac, sem comprometimento objetivo da audição. Dez anos após a remissão, a paciente queixou-se de perda de campo visual devido a uma nova isquemia da retina. Nenhum outro sintoma, ou neuroimagem ou achado audiométrico patológico foi observado durante o curso clínico.

Incidence of anterior segment neovascularization during intravitreal treatment for macular edema secondary to central retinal vein occlusion / Incidência de neovasos de segmento anterior durante o tratamento de edema macular secundário a oclusão da veia central da retina

ABSTRACT Purpose: To analyze the effects of injections of intravitreal triamcinolone acetonide (IVTA) and intravitreal bevacizumab (IVB) on the incidence rates of anterior segment neovascularization (ASN) and neovascular glaucoma (NVG) in patients with macular edema secondary to central retinal vein occlusion (CRVO). Methods: In this prospective, randomized, double-masked, sham-controlled study, 35 patients with macular edema following CRVO were randomized to intravitreal bevacizumab, intravitreal triamcinolone acetonide, or sham injections during the first 6 months of the study. The primary outcome was the incidence rate of ASN at month 6. The secondary outcomes were the mean changes from baseline in best-corrected visual acuity (BCVA) and central foveal thickness (CFT) on optical coherence tomography over time to month 12. Results: ASN developed in 8 (22.86%) eyes, including 5 (62.50%) eyes in the sham group and 3 (37.50%) eyes in the IVTA group, during 12 months of fol low-up (p=0.009). BCVA differed significantly (p<0.05) among the groups only at month 1. CFT did not differ significantly (p<0.05) among the groups over 12 months. NVG required surgery and developed in one eye despite laser treatment. Conclusion: Early treatment with intravitreal antivascular endothelial growth factor therapy decreases the rates of ASN and NVG after CRVO.

RESUMO Objetivo: Analisar as taxas de incidência de neovascularização do segmento anterior (NSA) e de glaucoma neovascular (GNV), em pacientes com edema macular secundário a oclusão de veia central da retina (OVCR), em tratamento com injeções intravítreas de triamcinolona (IVTA) ou bevacizumab (IVB). Métodos: Neste estudo prospectivo, randomizado, duplo mascarado e sham controlado, 35 pacientes com edema macular secundário a OVCR foram randomizados para IVB, IVTA ou para o grupo controle (sham), durante os 6 primeiros meses do estudo. O desfecho primário foi a taxa de incidência de NSA no mês 6. Os desfechos secundários foram alterações médias da acuidade visual corrigida (BCVA) e espessura foveal central (EFC) ao exame de tomografia de coerência óptica, até o mês 12. Resultados: NSA ocorreu em oito (22,86%) olhos, cinco (62,50%) olhos no grupo sham e três (37,50%) olhos no grupo tratado com injeções intravítreas de Triamcinolona, Não houve nenhum caso com NSA no grupo tratado com bevacizumab durante 12 meses de acompanhamento (p=0,009). A BCVA apresentou diferença estatisticamente significante (p<0,05) entre os grupos, somente no mês 1. A EFC não apresentou diferenças estatisticamente significantes (p<0,05) entre os grupos ao longo dos 12 meses. GNV ocorreu em um olho apesar do tratamento com laser e este paciente necessitou de intervenção cirúrgica. Conclusão: O tratamento precoce com injeções intravítreas de Anti VEGF podem diminuir as taxas de neovascularização do segmento anterior e glaucoma neovascular após oclusão de veia central da retina.

Neovascular Glaucoma Due to Branch Retinal Vein Occlusion Combined with Branch Retinal Artery Occlusion

Branch retinal artery occlusion (BRAO) and branch retinal vein occlusion (BRVO) rarely cause neovascular glaucoma (NVG). A 58-year-old woman with hypertension and type 2 diabetic mellitus complained of progressive visual loss in her right eye for the previous 3 months. At initial examination, visual acuity was 20 / 63 in the right eye. Angle neovascularization was observed and the intraocular pressure (IOP) was 30 mmHg in her right eye. Fundus examination and fluorescein angiography showed BRAO combined with BRVO. We immediately injected intravitreal and intracameral bevacizumab in her right eye. The next day, we performed scatter photocoagulation in the nonperfusion area. One month later, visual acuity was 20 / 20 in her right eye and the IOP was 17 mmHg with one topical antiglaucoma agent. The neovascularization had regressed completely. We report a case of unilateral NVG which was caused by BRAO with concomitant BRVO and advise close ophthalmic examination of the iris and angle in BRVO with BRAO.

A Case of Odontogenic Orbital Cellulitis Causing Blindness by Severe Tension Orbit

We report a very rare case of odontogenic orbital cellulitis causing blindness by severe tension orbit. A 41-yr old male patient had visited the hospital due to severe periorbital swelling and nasal stuffiness while he was treated for a periodontal abscess. He was diagnosed with odontogenic sinusitis and orbital cellulitis, and treated with antibiotics. The symptoms were aggravated and emergency sinus drainage was performed. On the next day, a sudden decrease in vision occurred with findings of ischemic optic neuropathy and central retinal artery occlusion. Deformation of the eyeball posterior pole into a cone shape was found from the orbital CT. A high-dose steroid was administered immediately resulting in improvements of periorbital swelling, but the patient's vision had not recovered. Odontogenic orbital cellulitis is relatively rare, but can cause blindness via rapidly progressing tension orbit. Therefore even the simplest of dental problems requires careful attention.

Coagulación intravascular retiniana: como signo clínico de embolización de líquido amniónico / Intravascular coagulation in the retina as clinical sign of embolization of amniotic fluid

La embolización de líquido amniótico es una de las condiciones más infrecuentes pero desvastadora que puede presentarse en embarazadas. La prevalencia se ha reportado de 1 en 80.000, con un alto índice de mortalidad (86%). El diagnóstico esta basado en uno o más de los 4 signos clínicos: colapso cardiovascular, dificultad respiratoria, coma y/o convulsiones y coagulopatía. Coagulación intravascular diseminada puede ocasionar pérdida visual bilateral en el grupo de sobrevivientes, por oclusión del sistema arterial retiniano u coroideo. Los sintomas visuales se presentan usualmente después del desarrollo de síntomas sistémicos; sin embargo, en el caso clínico que se expone a continuación la manifestación visual ocurrió simultaneamente con el desarrollo de las sistémicas. Por tanto, la pérdida visual bilateral en el contexto de los signos clínicos sistémicos expuestos, debe alertar al obstetra sobre la posibilidad de embolización de liquido amniótico.

Amniotic fluid embolism is one of the most devastating and infrequent condition known in pregnant women. The prevalence has been reportd as a 1 in 80.000 deliveries with high mortality rate, almost 86%. The diagnosis is based on one or more of the four symptoms and signs: cardiovascular collapse, respiratory distress, coma/seizures and/or coagulopathy. Disseminated intravascular coagulopathy can cause bilateral visual loss in the group of survivors by occlusion of the retinal artery and/or the choroid system. The visual manifestations appear usually after the development of systemic symptoms, but in the clinical case described below the visual symptoms occurred simultaneously to the development of systemic manifestations. Therefore, bilateral visual loss in the context of the systemic clinical signs exposed, should alert the obstetrician about the possibility of embolization of amniotic fluid.

Intravitreal Bevacizumab for the Treatment of Neovascular Glaucoma Associated With Central Retinal Artery Occlusion

We report three cases of neovascular glaucoma secondary to central retinal artery occlusion (CRAO) which were effectively managed with intravitreal bevacizumab (IVB) followed by panretinal photocoagulation (PRP). Neovascular glaucoma without peripheral anterior synechiae developed between one and five weeks following CRAO onset. All patients received 0.75 mg (0.03 ml) IVB. In all patients, complete regression of the iris and anterior chamber angle neovascularization was confirmed within one week. PRP was applied two weeks after the injection. The follow-up period was four to seven months (average, five months). Intraocular pressure was controlled in all patients using topical antiglaucoma medications alone. However, one patient experienced a recurrence of neovascularization three months after the initial combination treatment. This patient received another IVB injection and additional PRP, and the recurrent neovascularization resolved. There were no local or systemic adverse events in any patients. Therefore, intravitreal bevacizumab may be an effective adjunct in the treatment of neovascular glaucoma associated with CRAO.

Visual Loss in One Eye after Spinal Surgery

PURPOSE: To report a patient who developed an unusual combination of central retinal artery occlusion with ophthalmoplegia following spinal surgery in the prone position. METHODS: A 60-year-old man underwent a cervical spinal surgery in the prone position. Soon after recovery he could not open his right eye and had ocular pain due to the general anesthesia. Upon examination, we determined that he had a central retinal artery occlusion with total ophthalmoplegia. RESULTS: Despite medical treatment, optic atrophy was still present at the following examination. Ptosis and the afferent pupillary defect disappeared and ocular motility was recovered, but visual loss persisted until the last follow-up. CONCLUSIONS: A prolonged prone position during spinal surgery can cause external compression of the eye, causing serious and irreversible injury to the orbital structures. Therefore, if the patient shows postoperative signs of orbital swelling after spinal surgery the condition should be immediately evaluated and treated.

Transthoracic Echocardiography and Carotid Doppler Ultrasound for Detection of Cardiac and Carotid Artery Disease in Patients with Acute Retinal Artery Obstruction

We reviewed the medical records of patients with acute retinal artery obstruction (RAO) and evaluated the importance of transthoracic echocardiography (TTE) and carotid Doppler ultrasound in determining causes of cardiac and carotid artery origin in RAO. A retrospective case study conducted in the Department of Ophthalmology, Inha University Hospital, Korea comprised 26 patients presenting with acute RAO who underwent systemic evaluation, TTE and carotid Doppler ultrasound between June 1, 1997 and December 31, 2003. Among these 26 patients, abnormal cardiac findings were detected in 12 (46%) and abnormal carotid findings in 4 (15%). Furthermore, other risk factors for RAO were found in 2 (8%) and stroke broke out within 7 months after experiencing RAO in 4 (15%) of the 26 patients. In patients with acute RAO, TTE and carotid Doppler ultrasound play an important role in pinpointing the origins of retinal emboli. It is thought that TTE and carotid Doppler ultrasound may be essential examinations for determining the underlying cause, planning treatment strategies, and preventing stroke and death.

Oclusão de artéria e veia central da retina secundária à presumida vasculite herpética como manifestação inicial da AIDS: relato de caso / Central retinal artery and vein occlusion secondary to presumed herpetic vasculitis as the initial manifestation of the aids: a case report

Objetivo: Apresentar o caso de um homem de 42 anos que teve uma Oclusão de Artéria e Veia Central da Retina por Herpes Zoster como manifestação inicial de AIDS. Uma associação fundoscópia rara, cuja história, achados clínicos, fisiopatologia e diagnósticos diferenciais serão aqui discutidos. Local: Hospital CEMA - São Paulo - Brasil. Método: Relato de caso. Resultados: A associação das alterações fundoscópicas típicas com os dados semiológicos ajudaram na elucidação diagnóstica. Conclusão: A AIDS tem aqui relatada mais uma de suas formas de apresentação. As avaliações fundoscópicas seriadas são importantes no diagnóstico precoce das infecções oportunistas e devem ter um papel adjuvante na terapia anti-retroviral, preservando, assim, a saúde visual destes pacientes.

Degeneraçäo coroido-retiniana bilateral näo usual e oclusäo vascular retiniana em sarcoidose-relato de um caso / Unusual bilateral chorioretinal degeneration and retinal vascular occlusion in sarcoidosis-A case reported

Objetivo: apresentar um caso de Degeneraçäo coroido-retiniana näo usual e oclusäo vascular em paciente melanoderma com Sarcoidose. Local: Instituto de Olhos e Laser de Belém (clínica privada). Método: Neste trabalho é relatado um caso de doença diagnosticada pela fundoscopia, angiorretinografia fluoresceínica, Raio X de tórax, tomografia computadorizada de tórax, scan de gálio e dosagem no soro da converting enzima angiotensina 1. Resultado: A associaçäo dos achados de fundoscopia e de angiorretinografia fluoresceínica com exames positivos para sarcoidose, como elevaçäo da converting enzima angiotensina 1 no soro, linfadenopatia bilateral e infiltraçäo parenquimatosa pulmonar. Conclusäo: O paciente apresentou positiva associaçäo da Degeneraçäi coroido-retiniana näo usual e oclusäo vascular retiniana com exames labotarotiais (SACE) e de RaioX e tomografia computadorizada de tórax relacionados à Sarcoidose

Retinopatia vascular associada à Síndrome Antifosfolípide / Vascular occlusive retinopathy associated with anti-phospholipid antibodies

Os autores descrevem 2 casos (4 olhos) de retinopatia vaso-oclusiva periférica severa associada à síndrome antifosfolípide e descrevem também a fisiopatologia e alternativas terapêuticas desta condiçäo especial