Evaluation of commotio retinae in orbital fractures / Avaliação com commotio retinae (Edema de Berlim) em fraturas orbitárias

ABSTRACT Purpose: This study aimed to evaluate the mechanisms of injury and types of orbital fractures and their relation to concurrent commotio retinae. Methods: This retrospective study evaluated the records of patients with orbital fractures whose diagnoses had been confirmed by computer tomography between July 2017 and September 2019. Patient demographics, the circumstances of injury, ophthalmic examination results, and radiological findings were tabulated. Statistical analysis of the data used two-tailed student's t-tests, chi-squared tests, and odds ratio calculations. Statistical significance was set at p<0.05. Results: Of the 204 patients with orbital fractures included in this study, 154 (75.5%) were male. The mean age was 42.1 years. Orbital fractures involving one orbital wall (58.8%) were more common than those affecting multiple walls (41.2%). The majority of fractures affected the inferior wall (60.3%), with the medial walls being the next most frequently affected (19.6%). The most common cause of injury was assault (59.3%), and the second most common was falls (24%). Commotio retinae was observed in 20.1% of orbital fracture cases and was most associated with injuries caused by assault (OR=5.22, p<0.001) and least associated with those caused by falls (OR=0.06, p<0.001). Eye movement restrictions were more common in central than peripheral commotio (OR=3.79, p=0.015) and with medial wall fractures than fractures to other orbital walls (OR=7.16, p<0.001). The odds of commotio were not found to be higher in patients with multi-walled orbital fractures than in those with single-walled fractures (p=0.967). Conclusions: In the study population, assault was the most common cause of orbital fractures and resulted in commotio retinae than other causes. Ophthalmologists should be aware of the likelihood of commotio retinae in patients with orbital fractures resulting from assault, regardless of the extent of the patient's injuries.

RESUMO Objetivo: Este estudo visou avaliar os mecanismos da lesão e os tipos de fraturas orbitárias e sua relação com commotio retinae simultânea. Métodos: Este estudo retrospectivo avaliou registros de pacientes com fraturas orbitárias cujos diagnósticos foram confirmados por tomografia computadorizada entre julho de 2017 e setembro de 2019. Foram registrados os dados demográficos, circunstâncias da lesão, os resultados do exame oftalmológico e achados radiológicos. A análise estatística dos dados usou os testes de t-Student bicaudal, qui-quadrado e cálculos de odds ratio. O significado estatístico foi fixada em p<0,05. Resultados: Dos 204 pacientes com fraturas orbitárias incluídos neste estudo, 154 (75,5%) eram sexo masculino (75,5%). A média de idade foi de 42,1 anos. As fraturas orbitárias envolvendo uma parede orbital (58,8%) foram mais comuns do que as que acometeram várias paredes (41,2%). A maioria das fraturas acometeu a parede inferior (60,3%), sendo as paredes mediais as próximas mais frequentemente afetadas (19,6%). A causda mais comum de lesão foi agressão (59,3%), e a segunda mais comum foi queda (24%). A commotio retinae foi observada em 20,1% dos casos de fratura orbital e foi mais associada a lesões causadas por agressão (OR=5,22, p<0,001) e menos associada com aquelas causadas por quedas (OR=0,06, p<0,001). As restrições de movimentos oculares eram mais comuns na comoção central do que na periférica (OR=3,79, p=0,015) e com fraturas da parede medial do que com fraturas de outras paredes orbitais (OR=7,16, p<0,001). As chances de comoção não foram maiores em pacientes com fraturas orbitais de paredes múltiplas do que naqueles com fraturas de parede simples (p=0,967). Conclusões: Na população do estudo, a agressão foi a causa mais comum de fraturas orbitais e resultou em commotio retinae mais grave do que qualquer outra causa. Os oftalmologistas devem estar cientes da probabilidade de commotio retinae em pacientes com fraturas orbitais resultantes de agressão, independentemente da extensão das lesões do paciente.

Purtscher-like retinopathy in a patient with COVID-19-associated coagulopathy / Retinopatia tipo Purtscher em paciente com coagulopatia associada ao COVID-19

ABSTRACT The most frequently reported ophthalmic manifestation of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is conjunctivitis. We have described a case of Purtscher-like retinopathy in a patient with severe coronavirus disease 2019 (COVID-19)-associated coagulopathy. A young woman with multiple comorbidities was admitted for COVID-19-related acute respiratory distress syndrome. Her course was complicated by fungemia. Ophthalmic examination revealed bilateral posterior pole, intraretinal lesions and fluconazole was added for presumed fungal retinitis. At 1-week follow-up, widespread peripapillary cotton-wool spots and hemorrhages suggestive of Purtscher-like retinopathy were observed. The levels of D-dimers, fibrinogen, and C-reactive protein were markedly elevated prior to our consultation, indicating preceding prothrombotic and pro-inflammatory states. Subsequent venous duplex revealed deep venous thrombosis in the right subclavian and internal jugular veins. Von Willebrand factor indices were markedly elevated, suggesting severe COVID-19-associated coagulopathy. Purtscher-like retinopathy, a rare occlusive microangiopathy has been described in various pro-inflammatory and prothrombotic conditions. To the best of our knowledge, this is the first report of Purtscher-like retinopathy in COVID-19-associated coagulopathy.

RESUMO A manifestação oftálmica mais frequentemente re­latada da infecção por SARS-CoV-2 é a conjuntivite. Trata-se de estudo de caso de retinopatia tipo Purtscher em uma paciente com coagulopatia grave associada ao COVID-19. Uma jovem com múltiplas comorbidades foi admitida por síndrome do desconforto respiratório agudo relacionado ao COVID-19. Seu quadro foi complicado pela fungemia. O exame oftálmico revelou pólo posterior bilateral, lesões intraretinianas e o fluconazol foi adicionado para tratar a retinite fúngica presumida. No decorrer de uma semana, manchas largas peripapilares de algodão e hemorragias sugestivas de retinopatia tipo Purtscher foram observadas. Os dímeros D, o fibrinogênio e a proteína c-reativa estavam acentuadamente elevados antes da nossa consulta, indicando um estado pró-trombótico e pró-inflamatório precedente. O duplex venoso subsequente revelou trombose venosa profunda nas veias subclávia direita e jugular interna. Os índices de fatores von Willebrand estavam marcadamente elevados, sugerindo coagulopatia grave associada ao COVID-19. A retinopatia tipo Purtscher, uma microangiopatia oclusiva rara foi descrita em várias condições pró-inflamatórias e pró-trombóticas. Para nosso conhecimento, este é o primeiro relatório de retinopatia tipo Purtscher com coagulopatia associada ao COVID-19.

Characteristics of ocular trauma in the United States / Características de trauma ocular nos Estados Unidos

ABSTRACT Purpose: We aimed to study the characteristics of ocular trauma, an important but largely preventable global cause of blindness, in the United States. Methods: Retrospective chart review of the National Trauma Data Bank (2008-2014) was performed. All patients with ocular trauma were identified using ICD-9CM codes. The collected data were statistically analyzed with student's t-test, Chi-squared test, and logistic regression analysis performed using the SPSS software. The significance was set at p<0.05. Results: It was found that 316,485 (5.93%) of the 5,336,575 admitted trauma patients had ocular injuries. Their mean (SD) age was 41.8 (23) years, and most of them were men (69.4%). Race/ethnicity distribution was White 66.1%, Black 15.1%, and Hispanic 12.3%. The common injuries were orbital 39.5% and eye/adnexa contusions 34%. Associated traumatic brain injury was present in 58.2%. The frequent mechanisms were falls 25.5%, motor vehicle accident-occupant 21.8%, and struck by/against 17.6%. Patients <21 years of age had higher odds of cut/pierce injuries (OR=3.29, 95%CI=3.07-3.51) than the other age groups, those aged 21-64 years had higher odds of motor vehicle accident-cyclist (OR=4.95, 95%CI=4.71-5.19), and those >65 years had higher odds of falls (OR=16.75, 95%CI=16.39-17.12); p<0.001. The Blacks had a greater likelihood of firearm injuries (OR=3.24, 95%CI=3.10-3.39) than the other racial/ethnic groups, the Hispanics experienced more of cut/pierce injuries (OR=2.01, 95%CI=1.85-2.18), and the Whites experienced more of falls (OR=2.3, 95%CI=2.3-2.4); p<0.001. The Blacks (OR=3.41, 95%CI=3.34-3.48) and Hispanics (OR=1.75, 95%CI=1.71-1.79) mostly suffered assaults, while the Whites suffered unintentional injuries (OR=2.78 95%CI=2.74-2.84); p<0.001. Optic nerve/visual pathway injuries had the greatest association with very severe injury severity scores (OR=3.27, 95%CI=3.05-3.49) and severe Glasgow Coma Scores (OR=3.30, 95%CI=3.08-3.54); p<0.001. The mortality rate was 3.9%. Conclusions: Male preponderance and falls, motor vehicle accident-occupant, and struck by/against mechanisms agree with the previous reports. The identified demographic patterns underscore the need to develop group-specific preventive measures.

RESUMO Objetivo: O trauma ocular é uma causa importante e amplamente evitável de cegueira em todo o mundo. Nosso objetivo é estudar suas características nos EUA. Métodos: Revisão retrospectiva do National Trauma Data Bank (2008-2014). Todos os pacientes com trauma ocular foram identificados com códigos CID-9CM. Os dados coletados foram analisados estatísticamente e o teste t de student foi utilizado. As análises qui-quadrado e de regressão logística foram realizadas com o software SPSS. A significância foi estabelecida em p<0,05. Resultados: 316.485 (5,93%) de 5.336.575 pacientes internados com trauma, apresentaram lesões oculares. A média (DP) de idade foi de 41,8 (23) anos. A maioria era do sexo masculino (69,4%). A distribuição raça/etnia foi branca: 66,1%, negra; 15,1% e hispânica: 12,3%. As lesões comuns foram orbitárias: 39,5% e contusões dos olhos/anexos: 34%. A maioria (58,2%) teve lesão cerebral traumática. Os mecanismos frequentes foram: quedas: 25,5%, acidente com veículos motorizados: 21,8% e acidentes atingidos por algo/contra algo: 17,6%. Os pacientes <21 anos apresentaram chance aumentada de lesões com corte/perfuração (RC=3,29; IC95% = 3,07-3,51) do que outras faixas etárias, aqueles entre 21-64 anos responderam por acidente automobilístico-ciclista: (RC=4,95; IC95% = 4,71-5,19) e aqueles >65 anos foram vítimas de quedas (RC=16,75; IC 95% = 16,39-17,12); p<0,001. Os negros apresentaram maior probabilidade de lesões por arma de fogo (RC=3,24; IC95% = 3,10-3,39) do que outras raças/etnias e os hispânicos tiveram mais lesões de corte/perfuração (RC=2,01; IC95%= 1,85-2,18) enquanto os brancos tiveram mais quedas: (RC=2,3; IC95%= 2,3-2,4); p<0,001. Os negros (RC=3,41; IC95% = 3,34-3,48) e os hispânicos (RC=1,75; IC95% = 1,71-1,79) sofreram principalmente agressões e os brancos tiveram lesões não intencionais (RC=2,78; IC95% 2,74-2,84); p<0,001. Lesões do nervo óptico/via visual apresentaram maior associação com escore de gravidade de lesão muito grave (RC=3,27; IC95%= 3,05-3,49) e escores graves de Coma de Glasgow (RC=3,30; IC95%= 3,08-3,54); p<0,001. A taxa de mortalidade foi de 3,9%. Conclusões: Houve preponderância masculina a quedas, a acidentes com veículo motorizado atingidos por algo/contra algo conforme os relatos anteriores. Os padrões demográficos identificados realçam a necessidade de desenvolver medidas de prevenção específicas para os diferentes grupos.

Central retinal artery occlusion associated with patent foramen ovale: a case report and literature review / Oclusão da artéria central da retina associada ao forame oval patente: relato de caso e revisão de literatura

ABSTRACT Patent foramen ovale might cause cryptogenic strokes, including retinal artery occlusion. Herein, we describe a previously healthy young man who presented with central retinal artery occlusion in the setting of patent foramen ovale and explore the need for transesophageal echocardiogram for its diagnosis. Cardiovascular workup and neuroimaging were unremarkable. Transthoracic echocardiogram bubble study revealed a right to left atrial shunt and subsequent transesophageal echocardiogram disclosed patent foramen ovale. This congenital cardiac anomaly was the likely conduit for a thrombo-embolic central retinal artery occlusion. We identified seven patients with patent foramen ovale associated with central retinal artery occlusion in the literature. Transthoracic echocardiogram was diagnostic in only one patient (14.3%), whereas transesophageal echocardiogram was required to reveal patent foramen ovale in the remaining six (85.7%). Our case and the previous reports support the link between central retinal artery occlusion and patent foramen ovale. Therefore, providers should consider the more sensitive transesophageal echocardiogram during the initial evaluation of young patients without immediately identifiable causes of retinal artery occlusion.

RESUMO O forame oval patente pode estar associado a derrames criptogênicos que incluem a oclusão da artéria retiniana. Descrevemos aqui um jovem previamente saudável que apresentou oclusão da artéria central da retina associada ao forame oval patente, sendo considerado portanto, a necessidade de um ecocardiograma transesofágico para seu diagnóstico. A avaliação cardiovascular e a neuroimagem não foram significativas. O estudo da bolha no ecocardiograma transtorácico revelou um shunt atrial direito-esquerdo e o ecocardiograma transesofágico subsequente revelou um forame oval patente. Esta anomalia cardíaca congênita foi o provável conduíte para uma oclusão tromboembólica da artéria central retiniana Na literatura, foram identificadossete pacientes com forame oval patente associado à oclusão da artéria central retiniana. O ecocardiograma transtorácico diagnosticou apenas um paciente (14,3%), enquanto o ecocardiograma transesofágico foi necessário para revelar o forame oval patente nos seis casos restantes (85,7%). Nosso caso e relatos anteriores suportam a ligação entre a oclusão da artéria central retiniana e o forame oval patente. Os profissionais devem considerar, como sendo mais sensível, o ecocardiograma transesofágico na avaliação inicial de pacientes jovens sem causas imediatamente identificáveis de oclusões da artéria retiniana.

Impact of Anxiety Levels and Sleep Patterns on Perceived Pain During Intravitreal Bevacizumab Injections.

BACKGROUND AND OBJECTIVE: To evaluate the impact of anxiety and sleep patterns on intravitreal injection pain. PATIENTS AND METHODS: This prospective, noninterventional study surveyed patients scheduled for intravitreal injection by two retinal surgeons. A standard intravitreal injection technique was used. Patients filled out pre-procedure General Anxiety Disorder-7 (GAD-7) and Pittsburgh Sleep Quality Index (PSQI) questionnaires. Post-procedural pain was assessed with a visual analogue scale. Quality and hours of sleep and anxiety levels were correlated with perceived intravitreal injection pain. RESULTS: A total of 140 patients met inclusion criteria. Mean ± standard deviation scores were 4.9 ± 5.6 for the GAD-7, 6.3 ± 4.1 for the PSQI, and 3.69 ± 2.64 for intravitreal injection pain. Anxiety correlated with intravitreal injection pain (rho = 0.25; P = .003). Previous night's sleep (rho = -0.16; P = .057) and poor sleep quality (rho = 0.14; P = .11) were weakly correlated. Regression analysis revealed anxiety was the only significant predictor of intravitreal injection pain. A 1-point increase in anxiety predicted a 0.10-point increase in intravitreal injection pain (B = 0.10, P = .032). CONCLUSIONS: Anxiety level was the best predictor of perceived pain. This has implications for pre-procedural anxiety screening. [Ophthalmic Surgery Lasers Imaging Retina. 2021;52:498-504.].

Patterns of ocular trauma in elderly patients in an urban population-the Bronx experience / Padrões de trauma ocular em pacientes idosos em uma população urbana - a experiência de Bronx

ABSTRACT Purpose: To evaluate the characteristics of ocular injuries among elderly patients admitted to an urban level I trauma center because of major trauma from 2008 to 2015. Methods: A retrospective chart review was conducted of patients aged >65 years admitted with ocular injuries that were identified with ICD-9 codes. Tabulated data were analyzed using the Student's paired t-test, the chi-squared test, and regression analysis using STATA/MP-12 software. Significance was set at p<0.05. Results: Of a total of 861 patients, 221 (25.7%) admitted for major trauma and ocular injuries were elderly. The mean age of these patients was 80.3 years (median =79.2 years; interquartile range=63.8-94.6 years). Of these patients, 40.7% were males and 59.3% were females. The males were younger than the females (mean age, 77.3 vs. 82.4 years, respectively, p<0.001). Race was documented as white (30.8%), black (13.6%), and "other" (54.3%), with 67.5% of the "other" group (36.7% overall) identified as Hispanic. The most frequent injuries were contusion of the eye/adnexa (68.2%), orbital wall fractures (22.2%), and an open wound of the ocular adnexa (18.1%). Males had a 2.64-fold greater risk of orbital wall fractures (95% confidence interval [CI]=1.38-5.05, p<0.003). Patients with orbital wall fractures had higher injury severity scores than those without (95% CI=14.1-20.9 vs. 6.8-8.6, respectively, p<0.001). The most common injuries were falls (77.8%) and pedestrian/motor vehicle accidents (6.8%). Falls occurred mostly at home (51.7%), on the street (13.9%), and in hospitals/nursing homes (12.2%). Those falling at home were older than those falling at other locations (95% CI=81.8-85.4 vs. 77.0-80.6 years, respectively, p<0.002). Conclusions: Ocular injuries in elderly Bronx patients most commonly occurred in females due to falls in the home/nursing home setting. Public health measures addressing identifiable individual and environmental risks in these common locations would be most beneficial in reducing the incidence of ocular injuries in this population.

RESUMO Objetivo: Avaliar as características das lesões oculares de idosos nas internações por grandes traumatismos em um centro urbano de trauma nível I de 2008 a 2015. Métodos: Realizou-se uma revisão retrospectiva de prontuários de pacientes com mais de 65 anos internados com lesões oculares identificados com os códigos CID-9. Os dados tabulados foram analisados com o teste t de Student, teste qui-quadrado e análise de regressão, utilizando o software STATA/MP-12. A significância estatística foi fixada em p<0,05. Resultados: Duzentos e vinte e um (25,7%) pacientes de um total de 861, admitidos por traumatismo craniano importante e lesões oculares, eram idosos. A idade média era de 80,3 anos (mediana=79,2; intervalo interquartil=63,8-94,6). 40,7% eram do sexo masculino e 59,3% do feminino. Os homens eram menos idosos (média=77,3) do que as mulheres (média=82,4), p<0,001. A raça foi documentada como branca (30,8%), negra (13,6%) e "outra" (54,3%); 67,5% dos "outros" (36,7% no geral) identificados como hispânicos. As lesões mais frequentes foram contusão do olho/anexos (68,2%), fraturas da parede orbital (22,2%) e ferida aberta dos anexos oculares (18,1%). Os homens tiveram 2,64 mais chances de fraturas da parede orbital (95% CI=1,38-5,05; p<0,003). Pacientes com fraturas da parede orbital tiveram maiores escores de gravidade da lesão (95% CI=14,1-20,9) do que aqueles sem fraturas (96% IC=6,8-8,6), p<0,001. Os mecanismos comuns foram quedas (77,8%) e acidentes a pé com veículos automotores (6,8%). As quedas ocorreram principal mente em casa (51,7%), na rua (13,9%) e em hospitais/lares de idosos (12,2%). Aqueles que caíram em casa eram mais velhos (IC 95%=81,8-85,4) do que os que tiveram quedas em outros locais (IC 95%=77,0-80,6), p<0,002. Conclusões: Lesões oculares em pacientes idosos de Bronx foram mais comuns no sexo feminino e devido a quedas que ocorreram em casa/lar de idosos. Medidas de saúde pública direcionadas a riscos individuais e ambientais identificáveis nesses locais comuns seriam mais benéficas na redução de lesões oculares nessa população.

Antiphospholipid syndrome and neurofibromatosis type I: a coincidence or new association? / Síndrome antifosfolípide e neurofibromatose tipo I: uma coincidência ou nova associação?

ABSTRACT - Numerous studies have reported on structural vascular anomalies and ischemia associated with neurofibromatosis type 1 that are thought to stem from dysfunction of neurofibromin, the neurofibromatosis type 1 protein. Documented cases of associated antiphospholipid syndrome fulfilling the accepted diagnostic criteria are exceptionally rare, with only three cases reported in the literature. Here, we report on a patient with neurofibromatosis type 1 and a history of spontaneous abortions presenting with sudden vision loss in the right eye and swelling of the optic nerve head. Fluorescein angiography indicated anterior ischemic optic neuropathy. Brain magnetic resonance imaging revealed findings consistent with left cavernous sinus meningioma. Serologic testing demonstrated persistently elevated anti-b2-glycoprotein antibodies. Her findings suggested antiphospholipid syndrome with concomitant clinical and laboratory evidence of antiphospholipid syndrome: frequent abortions, a vaso-occlusive episode, and persistently elevated antiphospholipid syndrome antibodies. To our knowledge, this case represents the first neuro-ophthalmic manifestation of antiphospholipid syndrome associated with neurofibromatosis type 1.

RESUMO - Inúmeros estudos têm relatado anomalias vasculares estruturais e isquemia associada com à neurofibromatose tipo 1 que, acredita-se, resultam da disfunção da neurofibromina, a proteína tipo 1 da neurofibromatose. Casos documentados de síndrome antifosfolípide associada que atendem aos critérios diagnósticos aceitos são excepcionalmente raros, com apenas três casos relatados na literatura. Aqui, relatamos um paciente com neurofibromatose tipo 1 e histórico de abortos espontâneos apresentando perda repentina de visão no olho direito e edema de cabeça do nervo óptico. A angiofluoresceínografia indicou neuropatia óptica isquêmica anterior. Ressonância magnética cerebral revelou achados compatíveis com meningioma do seio cavernoso esquerdo. O teste sorológico demonstrou anticorpos anti-b2 glicoproteína persistentemente elevados. Seus achados sugerem síndrome antifosfolípide com evidências clínicas e laboratoriais concomitantes de síndrome antifosfolipídica: abortos frequentes, episódio vaso-oclusivo e anticorpos antifosfolípides persistentemente elevados. Pelo nosso conhecimento, este caso pode representar a primeira manifestação neuro-oftálmica da síndrome antifosfolípide associada à neurofibromatose tipo 1.

Antiphospholipid syndrome and neurofibromatosis type I: a coincidence or new association?

Numerous studies have reported on structural vascular anomalies and ischemia associated with neurofibromatosis type 1 that are thought to stem from dysfunction of neurofibromin, the neurofibromatosis type 1 protein. Documented cases of associated antiphospholipid syndrome fulfilling the accepted diagnostic criteria are exceptionally rare, with only three cases reported in the literature. Here, we report on a patient with neurofibromatosis type 1 and a history of spontaneous abortions presenting with sudden vision loss in the right eye and swelling of the optic nerve head. Fluorescein angiography indicated anterior ischemic optic neuropathy. Brain magnetic resonance imaging revealed findings consistent with left cavernous sinus meningioma. Serologic testing demonstrated persistently elevated anti-b2-glycoprotein antibodies. Her findings suggested antiphospholipid syndrome with concomitant clinical and laboratory evidence of antiphospholipid syndrome: frequent abortions, a vaso-occlusive episode, and persistently elevated antiphospholipid syndrome antibodies. To our knowledge, this case represents the first neuro-ophthalmic manifestation of antiphospholipid syndrome associated with neurofibromatosis type 1.

Etiology of optic atrophy: a prospective observational study from Saudi Arabia.

BACKGROUND: Optic atrophy (OA) represents permanent retinal ganglion cell loss warranting study to establish etiology. OBJECTIVES: To describe neurogenic causes of OA. DESIGN: Prospective, observational. SETTING: Tertiary care center, Riyadh, Saudi Arabia. PATIENTS AND METHODS: We included consecutive patients of all ages with OA caused by lesions affecting the visual pathways who were referred over a 9-month period (November 2013 to July 2014). Diagnosis was based on visual acuity, ophthalmoscopic features and ancillary tests. Patient demographics, results of a clinical examination, test data and etiology were recorded. For each cause of OA, both gender and age group were analyzed as potential risk factors using simple univariate logistic regression. OA associated with glaucoma and retinal diseases was excluded. MAIN OUTCOME MEASURE: Description of causes of OA. RESULTS: Two hundred and four patients and 353 eyes met inclusion criteria. The median age was 27 years (range 3 months-77 years; interquartile range, 27 years) among 111(54.4%) females and 93(45.6%) males, with no statistically significant difference in age of presentation between the genders. The majority of lesions were bilateral (n=151, 74%). Tumors were the most common cause, accounting for 127 (62.2%) cases. These occurred mostly in adults (72.4%) compared to the pediatric group (OR=3.3, 95% CI: 1.79-6.03; P < .001). Hereditary neoplasia (OR=5.55; 95% CI: 1.67-18.42; P=.005) and metabolic diseases (OR=17.57; 95% CI: 2.15-143.62; P=.007) were more common causes in the pediatric group. There were no significant associations between gender or visual acuity and etiology of OA. In developed nations, OA is frequently the result of ischemia and neuritis. We found many other causes, especially orbital and intracranial tumors. CONCLUSIONS: The frequency of tumors as the cause of OA may represent a higher incidence of aggressive tumors coupled with poor recognition/acknowledgement of symptoms and limited access, resulting in late presentations. LIMITATIONS: These findings may reflect bias from selective referrals to a tertiary center and may not represent all of Saudi Arabia.

Late Recognition of a Case of Oculo-Auriculo-Vertebral Spectrum.

PURPOSE: To report the late recognition of a case oculo-auriculo-vertebral spectrum (OAVS). CASE REPORT: A 14-year-old male patient with a history of spinal scoliosis and anal atresia presented with a right ocular growth that was increasing in size and causing irritation. He was discovered to have a right limbal dermoid and forniceal lipodermoid. All visual functions were normal and the rest of the anterior and posterior segments were unremarkable. External examination revealed facial asymmetry and ipsilateral hemifacial microsomia and a pre-auricular skin tag. A diagnosis of oculo-auriculo-vertebral spectrum (OAVS) or Goldenhar syndrome was made. CONCLUSIONS: Growth of a limbal dermoid helped identify oculo-auriculo-vertebral spectrum, a disorder with a wide phenotypic spectrum that can affect all systems. Early recognition and syndromic designation is imperative; full pediatric survey of suspected cases will help rule out the life-threatening manifestations of the spectrum.

Chronic Myelogenous Leukemia Relapse Presenting With Central Nervous System Blast Crisis and Bilateral Optic Nerve Infiltration.

Bilateral, simultaneous optic nerve sheath infiltration as a manifestation of leukemia relapse is very rare. A 45-year-old woman with chronic myelogenous leukemia was successfully treated to cytogenetic bone marrow remission 1 year previously and maintained on imatinib. She developed total bilateral blindness with marked, bilateral optic disc edema and evidence of bilateral optic nerve infiltration on magnetic resonance imaging. Cerebrospinal fluid cytology confirmed central nervous system (CNS) blast crisis. She recovered visual acuity of 20/20 in the right eye, and 20/25 in the left eye with salvage systemic and intrathecal chemotherapy before radiation therapy. Our report underscores the importance of timely and aggressive intervention of blast crisis of the CNS and the need for CNS penetrating induction and maintenance therapy.

Morning Glory Syndrome with Carotid and Middle Cerebral Artery Vasculopathy.

PURPOSE: To report a case of incidental asymptomatic atypical morning glory syndrome (MGS) with concomitant ipsilateral carotid and middle cerebral dysgenesis. CASE REPORT: A 6-year-old child was discovered to have incidental findings of MGS, with atypia. All visual functions were normal including vision and stereopsis. Neuroimaging revealed ipsilateral carotid and middle cerebral vascular narrowing without associated collateral vessels or cerebral ischemia commonly seen in Moyamoya disease. Subsequent annual examinations have been stable, without signs of progression. CONCLUSIONS: This case demonstrates disparity between structural aberrations and final visual and neurological function and reinforces the association between MGS and intracranial vascular disruption. Full ancillary ophthalmic and neuroimaging studies should be performed in all patients with MGS with interval reassessments, even when the patient is asymptomatic and functionally intact.

Signet ring cell adenocarcinoma and bilateral leptomeningeal involvement of optic nerve sheaths.

Signet ring cell adenocarcinoma has a propensity for leptomeningeal carcinomatosis, and although bilateral optic nerve involvement is rare, this may occur with or without obvious signs of diffuse leptomeningeal involvement. We describe a 41-year-old woman who presented with a brief history of simultaneous bilateral visual deterioration and a distended abdomen. Examination revealed bilateral no light perception vision and bilateral optic disc edema. Radiologic work-up showed large multiple pelvic masses involving the ovaries, multifocal boney deposits, and widespread central nervous system carcinomatosis, involving the optic nerves and the first, fifth, and eighth cranial nerves. Biopsy of an ovarian mass demonstrated islands of signet ring cells. Signet cell adenocarcinomatous infiltration of the leptomeningeal space should be considered in cases of bilateral simultaneous vision loss with signs suggestive of leptomeningeal infiltration of the optic nerve sheath.