Giant anterior sacral meningocele associated with hydroureteronephrosis and renal injury: illustrative case.

BACKGROUND: Anterior sacral meningocele (ASM) is a defect in the closure of the neural tube. Patients can be asymptomatic or present with genitourinary, neurological, reproductive, or colorectal dysfunction. Magnetic resonance imaging (MRI) is the gold standard test because it can assess communication between the spinal subarachnoid space and the lesion and identify other abnormalities. Surgical correction is the definitive treatment because untreated cases have a mortality rate of more than 30%. OBSERVATIONS: A 24-year-old woman with Marfan syndrome presented with polyuria, recurrent urinary tract infections, and renal injury for 3 months along with a globose abdomen, with a palpable mass in the middle and lower third of the abdomen that was massive on percussion. MRI showed an ASM consisting of two cystic lesions measuring 15.4 × 14.3 × 15.8 and 6.7 × 6.1 × 5.9 cm, respectively, compressing the distal third of the right ureter and causing a hydroureteronephrosis. Drainage and ligature of the cystic lesion were performed. The urinary outcome was excellent, with full recovery after surgery. LESSONS: ASM should be suspected in all abdominal masses with progressive symptoms in the setting of Marfan syndrome. Computed tomography and MRI are important to investigate genitourinary anomalies or other types of dysraphism to guide the best surgical approach.

Optimizing optical coherence tomography and histopathology correlation in retinal imaging.

OBJECTIVE: To develop a methodology to correlate optical coherence tomography (OCT) images and histopathological sections from the same eye. Part 1: To determine the best fixative for optimal OCT and histopathological analysis in post-mortem eyes. Part 2: A protocol is proposed to correlate histopathological features and OCT scans from the same post-mortem eyes. DESIGN: Experimental study. PARTICIPANTS: Part 1: Twenty-three rabbit eyes and 14 post-mortem human eyes. Part 2: Nineteen post-mortem human eyes. METHODS: Part 1: Six different fixatives were tested, and specimens were evaluated on 4 criteria: globe shape, structure opacification, retinal detachment, and nuclear details. Part 2: Based on the findings from Part 1, fixed human eyes were imaged using OCT. Orientation-controlled histopathological processing was performed to obtain serial tissue sections from paraffin embedded tissue, which were matched to corresponding OCT images. RESULTS: Part 1: Of the 6 fixatives, 2% glutaraldehyde and Davidson's solution met the proposed criteria in rabbit eyes. Of these, glutaraldehyde showed similar results in human eyes and was selected for Part 2. Part 2: Using anatomical landmarks, cross-sectional histopathological sections in the same orientation as the OCT images were correlated to their corresponding OCT images. Retinal lesions such as a macular hole, an epiretinal membrane, and the presence of drusen were easily correlated, proving the reliability of our methodology. Moreover, the photoreceptor's inner/outer junction was correlated to a hyperreflective band on OCT. CONCLUSIONS: A standardized protocol was developed to correlate OCT images and histopathological findings by generating serial cross-sections of the retina, which can be used to better understand otherwise ambiguous OCT findings.

Ten-Year Follow-Up after Bilateral Submacular Neovascular Membrane Removal in a Case of Autosomal Recessive Bestrophinopathy.

Herein, we report the case of an 8-year-old girl who presented in December 2000 with a submacular neovascular membrane in the right eye, with a clinical diagnosis of Best disease. At that time, she underwent pars plana vitrectomy (PPV) with removal of the subretinal choroidal neovascularization (CNV). Her vision improved from 20/200 to 20/25. Four years later, a new CNV developed in the other eye. Initially, she underwent unsuccessful photodynamic therapy. As her vision worsened, she underwent a second, this time successful, PPV with membrane removal in the left eye, with vision improving to 20/30. Ten years later, she returned complaining of vision loss over the last year. Her vision was 20/200 OU, and optical coherence tomography demonstrated very large intraretinal cystoid spaces resembling bilateral macular schisis. Four ranibizumab injections as well as dorzolamide eye drops were tried, both without success. Finally, she underwent PPV with internal limiting membrane peeling and gas-fluid exchange in the left eye. One month later, the macula appeared flat and vision had improved to 20/60. The same procedure was performed 1 year later for the right eye, with vision improving to 20/80. One year later, mild cystic spaces developed again in both eyes, although much smaller than previously observed. Her vision remained stable.

Coinfection by Vaccinia virus and an Orf virus-like parapoxvirus in an outbreak of vesicular disease in dairy cows in midwestern Brazil.

The current report describes an outbreak of vesicular disease affecting dairy cows in midwestern Brazil in which a coinfection with 2 poxviruses-Vaccinia virus (VACV) and a parapoxvirus-was demonstrated. Milking cows presented vesicles, painful reddish or whitish papules, and scabby proliferative lesions in the teats and udder, in a clinical course of approximately 10-21 days. Histologically, multifocal areas of moderate to severe acanthosis, spongiosis, hypergranulosis, and parakeratotic or orthokeratotic hyperkeratosis with adjacent focally extensive ulcers were observed in the epidermis. Rounded eosinophilic inclusion bodies were observed in the cytoplasm of epithelial cells of areas with acanthosis or necrosis. Moderate inflammatory infiltrate of lymphocytes, plasma cells, neutrophils, and macrophages were observed in some dermal areas. Two people milking the affected cows developed lesions on the hands, painful papules which progressed to ulcerative and scabby lesions in 4-7 days. Electron microscopy of scabs from 1 cow revealed the concomitant presence of orthopoxvirus and parapoxvirus particles. Scabs from 2 cows were positive by polymerase chain reaction for the parapoxvirus B2L gene; 1 of the scabs was also positive for the VACV vgf gene. Nucleotide sequencing of the B2L amplicon revealed a similarity of 96-99% with Orf virus (ORFV) and lower identity with Pseudocowpox virus (92-95%) and Bovine papular stomatitis virus (85-86%). Nucleotide sequencing of a region of parapoxvirus DNA polymerase gene revealed a high similarity (98-100%) with ORFV sequences. Thus, an unusual coinfection with VACV and a parapoxvirus, likely ORFV, was demonstrated in the outbreak.

Long-term results of repeated anti-vascular endothelial growth factor therapy in eyes with retinal pigment epithelial tears.

PURPOSE: To evaluate the long-term results of retinal pigment epithelium tears in eyes treated with repeated anti-vascular endothelial growth factor (VEGF) therapy. METHODS: Five patients with retinal pigment epithelial tears (without foveal center involvement) after anti-VEGF injection were studied retrospectively. Mean follow-up time was 52 months, with measurements of visual acuity and evaluation of macular findings by angiography and optical coherence tomography during this period. All eyes had a persistent submacular neovascular membrane 30 days after the tear. An anti-VEGF drug was reinjected until the membranes stopped leaking. RESULTS: The mean initial visual acuity immediately after the tear was 20/160, and the mean final visual acuity was 20/60. The number of anti-VEGF reinjections varied from two to eight during the follow-up period. Long-term optical coherence tomography analysis showed reduced fluid and remodeling of the torn retinal pigment epithelium. CONCLUSION: Long-term visual results with repeated anti-VEGF therapy are not as devastating as suggested previously. Visual acuity and metamorphopsia improve with time as long as the neovascular membrane is inactive. Optical coherence tomography changes in the macular area reflect the visual acuity improvement.

Traumas oculopalpebrais no serviço de pronto-atendimento oftalmológico do Hospital Universitário Evangélico de Curitiba / Oculopalpebral traumas in the ophthalmology emergency service of the Evangelic University Hospital of Curitiba

Objetivo: Avaliar o perfil epidemiológico dos pacientes vítimas de traumas oculopalpebrais na cidade de Curitiba. Métodos: Foram estudados, prospectivamente, 622 pacientes que procuraram o Serviço de Pronto-Atendimento Oftalmológico do Hospital Universitário Evangélico de Curitiba no período de março a junho de 1998. Resultados: Houve predomínio de indivíduos do sexo masculino (6 homens: 1 mulher) e da faixa etária de 20 a 50 anos, compreendendo 73,3 por cento dos atendimentos. Os traumas oculopalpebrais mais freqüentes foram corpo estranho corneoconjuntival (54,6 por cento), ceratoconjuntivite química (12,1 por cento) e trauma contuso (8,8 por cento), sendo que 67,4 por cento dos traumas ocorreram no ambiente de trabalho e 21,2 por cento no ambiente doméstico. Verificou-se que a maioria dos pacientes com trauma oculopalpebral (67 por cento) procurou o serviço médico especializado nas primeiras 24 horas pós-trauma, embora a proteçäo ocular tenha sido pouco utilizada no trabalho e sendo praticamente inexistente em ambiente doméstico. Conclusöes: Os autores enfatizam a grande repercussäp socioeconômica causada pelos traumas oculopalpebrais e a necessidade de alertar órgäos governamentais, médicos especialistas ou näo, representantes de classes, empresários, trabalhadores e a populaçäo em geral quanto à importância da prevençäo de acidentes oculopalpebrais como forma de proteger a saúde ocular e optimizar os recursos humanos em nossa sociedade.

Hipertelorismo de Greig: relato de um caso / Hypertelorism of Greig: a case report

O hipertelorismo de Greig é uma disostose congênita, devida a anomalia do desenvolvimento dos ossos do crânio e face, caracterizada pelo aumento da separaçäo entre as órbitas. Outras alteraçöes anatômicas podem associar-se ao hipertelorismo, entre elas, a microftalmia, a microcórnea, o coloboma de disco óptico, as escleras azuladas, a paralisia dos músculos retos laterais. Dentiçäo anormal, palato em ogiva, sindactilia, alargamento das falanges, acrocianose e clavículas rudimentares säo outras alteraçöes associadas ao hipertelorismo de Greig. Neste estudos, os autores relatam 1 caso de hipertelorismo de Greig com algumas das alteraçöes acima, bem como, catarata e coloboma de íris em ambos os olhos

Trauma ocular penetrante grave e bilateral: revisäo de 10 casos / Bilateral and severe penetrating ocular trauma: report of 10 cases

Dez casos de ferimentos oculares penetrantes, graves e bilaterais, foram analisados retrospectivamente quanto à epidemiologia, apresentaçäo clínica, tratamento e resultado visual final. Todos os casos foram decorrentes de acidentes automobilísticos em que os pacientes se encontravam no banco dianteiro dos veículos sem o uso de cintos de segurança. Somente 2 pacientes apresentaram traumatismos graves em outros órgäos, os demais apresentaram apenas ferimentos de face junto ao trauma ocular. Quando do trauma penetrante somente foi feita a reconstituiçäo microscópica do globo ocular. Cinco olhos foram enucleados de imediato devido a perda total ou quase total do conteúdo intraocular, com laceraçöes que frequentemente se extendiam até o nervo óptico. Todos os olhos foram submetidos a vitrectomia pars plana e a outros procedimentos adicinais, dependendo de cada caso, entre 1 e 4 semanas após a reconstituiçäo inicial do globo, mas antes da vitrectomia, era apenas de projeçäo luminosa no melhor olho. O tempo de seguimento pós-operatório variou de 3 a 18 meses. Todos os olhos operados obtiveram melhora da acuidade visual que variou de acordo com o tipo da lesäo pré-existente. A importância do uso de cintos de segurança, inclusive no trânsito urbano, e da utilizaçäo da vitrectomia no tratamento destes casos é aqui discutida