Tumor de hipófise em paciente com hidrocefalia: um desafio diagnóstico / Pituitary tumor in a patient with hydrocephalus: a diagnostic challenge

RESUMO A hidrocefalia é definida como a dilatação ventricular pelo aumento da pressão intraventricular e intracraniana quando não tratada ou por insucesso do tratamento. Muitas vezes, leva ao dano das vias ópticas, podendo causar atrofia óptica, devido à proximidade dessas vias com o ventrículo lateral quando ocorre a dilatação. Assim como a hidrocefalia pode levar à atrofia óptica, outras patologias também podem. Tumores hipofisários compartilham desse mesmo sinal, além de causar hemianospsia bitemporal quando o tumor comprime quiasma óptico. Ademais, a hemianopsia bitemporal é o distúrbio visual mais comum encontrado em pacientes com tumor de hipófise. Os tumores de hipófise, por exemplo, geram manifestações clínicas que podem estar relacionadas à disfunção da glândula ou aos efeitos mecânicos da expansão tumoral. Sinais e sintomas visuais estão mais ligados ao efeito mecânico do tumor. Assim, muitas vezes, o paciente procura o oftalmologista antes do endocrinologista. Neste caso, analisaremos uma paciente portadora de hidrocefalia que apresentava, concomitantemente, um tumor hipofisário, e a investigação oftalmológica fez toda a diferença no tratamento da paciente.

ABSTRACT Hydrocephalus is defined as ventricular dilation caused by increased intraventricular and intracranial pressure when untreated or due to treatment failure. Optical pathways can often cause optic atrophy due to the proximity to the lateral hazard when dilation occurs. Hydrocephalus can lead to optic atrophy, as well as other pathologies. Pituitary tumors share this same sign, in addition to causing bitemporal hemianopia when it compresses the optic chiasm. In addition, bitemporal hemianopia is the visual disturbance most commonly found in patients with pituitary tumors. Pituitary tumors, for example, have clinical manifestations that may be related to gland dysfunction, or to mechanisms of tumor expansion. Visual signs and symptoms are more linked to the mechanical effect of the tumor. Therefore, the patient usually seeks the ophthalmologist before the endocrinologist. In this case, we analyzed a patient with hydrocephalus who presented, at the same time, a pituitary tumor, and the ophthalmological investigation made all the difference in the treatment of the patient.

Using ImageJ to Evaluate Optic Disc Pallor in Traumatic Optic Neuropathy

PURPOSE: To evaluate optic disc pallor using ImageJ in traumatic optic neuropathy (TON). METHODS: This study examined unilateral TON patients. The optic disc was divided into 4 quadrants (temporal, superior, nasal, and inferior), consistent with the quadrants on optical coherence tomography (OCT) retinal nerve fiber layer (RNFL) thickness maps. Optic disc photography was performed and disc pallor was quantified using gray scale photographic images imported into ImageJ software. The correlation between optic disc pallor and RNFL thickness was examined in each quadrant. RESULTS: A total of 35 patients (31 male, 4 female) were enrolled in the study. The mean participant age was 34.8 +/- 15.0 years (range, 5 to 63 years). Overall RNFL thickness decreased in 6 patients, with thinning most often occurring in the inferior quadrant (28 of 35 eyes). There was a significant correlation between optic disc pallor and RNFL thickness (superior, rho = -0.358, p = 0.04; inferior, rho = -0.345, p = 0.04; nasal, rho = -0.417, p = 0.01; temporal, rho = -0.390, p = 0.02). The highest level of correspondence between disc pallor and RNFL thickness values outside of the normative 95th percentiles was 39.3% and occurred in the inferior quadrant. CONCLUSIONS: Optic disc pallor in TON was quantified with ImageJ and was significantly correlated with RNFL thickness abnormalities. Thus, ImageJ evaluations of disc pallor may be useful for evaluating RNFL thinning, as verified by OCT RNFL analyses.

Achados oculares em pacientes com mucopolissacaridoses / Ocular findings in patients with mucopolysaccharidosis

OBJETIVO: O objetivo deste estudo foi determinar a prevalência e gravidade das complicações oculares em pacientes com mucopolissacaridoses (MPS). MÉTODOS: Vinte e nove pacientes com diagnóstico de mucopolissacaridoses foram estudados. Foram avaliados: idade, sexo, acuidade visual, presença de estrabismo, erros refrativos, exame de fundo de olho, pressão intraocular, espessura corneal central e ultrassonografia ocular. RESULTADOS: Foram avaliados três pacientes com MPS I (12 por cento), 11 pacientes com MPS II (37,9 por cento), um paciente com MPS III (3,4 por cento) e 14 pacientes com MPS VI (48,3 por cento). A média de idade foi de 9,5 anos (DP 5,5). Observou-se hipermetropia em 88,5 por cento (23 pacientes) e astigmatismo em 51,7 por cento (15 pacientes). A média da acuidade visual corrigida foi de 0,45 logMAR (DP 0,68). A média do equivalente esférico foi +3,57 D (DP 2,46) e da pressão intraocular foi 17 mmHg (DP 3,9). Os achados mais comuns foram: espessamento palpebral 24,1 por cento (7 pacientes); opacidade da córnea, 55,2 por cento dos casos (16 pacientes); atrofia do nervo óptico, 23,1 por cento (6 pacientes); dobras radiais na retina 24 por cento (7 pacientes). O fundo de olho não foi examinado em 3 pacientes devido à opacidade de córnea. A média da espessura do complexo esclera-retina-coroide (ERC) medida por ultrassom foi de 1,78 mm (DP 0,51). CONCLUSÃO: Os achados oftalmológicos mais proeminentes foram espessamento palpebral, diminuição da acuidade visual, hipermetropia moderada, opacidade da córnea, dobras radiais na retina perimacular e atrofia do nervo óptico.

PURPOSE: The objective of this study was to determine the prevalence and severity of ocular complications in patients with mucopolysaccharidosis (MPS). METHODS: Twenty-nine patients with diagnosis of mucopolysaccharidosis were studied. Age, gender, visual acuity, presence of strabismus, refractive error, fundus examination, intraocular pressure, central corneal thickness and ocular echography were assessed for each individual. RESULTS: There were three patients with MPS I (12 percent), eleven patients with MPS II (37.9 percent), one patient with MPS III (3.4 percent) and fourteen patients with MPS VI (48.3 percent). Mean age was 9.5 years (ranged from 1.2 to 20 years, DP 5.5). Refraction was available in 26 patients, from which 88.5 percent (23 patients) were hyperopic, and 53.8 percent (14 patients) presented astigmatism. Best corrected visual acuity was available in 18 patients and the mean was 0.45 logMAR (DP 0.68). The mean spherical equivalent was +3.57 D (SD 2.46) and intraocular pressure was 17 mmHg (SD 3.9). The most common findings were: eyelid thickening in 24.1 percent (7 patients); corneal opacity in 55.2 percent of cases (16 patients); optic nerve atrophy in 23.1 percent (6 patients); and radial folds in the retina in 24 percent (7 patients). The fundus was examined in 26 out of 29 patients because corneal opacity avoided the exam in 3 of them. The average thickness of the complex sclera-retina-choroid (SRC was 1.78 mm (SD 0.51). CONCLUSION: The most prominent ophthalmologic findings were eyelid thickening, decreased visual acuity, high hyperopia, corneal opacity, perimacular radial folds in the retina and optic nerve atrophy.

Aniridia associated with congenital aphakia and secondary glaucoma.

We report a case of aniridia associated with congenital aphakia and secondary glaucoma. A 35-year-old male presented with aniridia, congenital aphakia and secondary glaucoma in both eyes. After an unsuccessful medical management, he underwent trabeculectomy with mitomycin C and anterior vitrectomy under local anesthesia in his left eye. Postoperatively, at the end of six months, intraocular pressure (IOP) in his left eye was controlled without medications. This case highlights the rare association of aniridia with congenital aphakia and secondary glaucoma.

Image of the traumatic optic atrophy detected with optical coherence tomography and the vision function defection / 法医学杂志

OBJECTIVE@#To analyze the unilateral image of the traumatic optic nerve atrophy using optical coherence tomography (OCT) and to explore the relationship between the optic nerve atrophy and visual acuity or visual field.@*METHODS@#Fifteen cases with traumatic optic nerve atrophy were studied. All cases were tested by routine opthalmological examination, visual evoked potentials (VEP) and optic disc examination with OCT. In cases with visual acuity more than 0.1 the visual field was tested.@*RESULTS@#All cases had visual acuity and visual field defect at various levels and optic disc retinal nerve fiber layer (RNFL) atrophy.@*CONCLUSION@#The traumatic optic atrophy begins in the temporal area of optic disc. The nasal area's atrophy occurs at the last and is the mildest. The more serious is the optic nerve atrophy, the worse is the vision function impairment. OCT is a valuable technique for evaluating the optic nerve atrophy and has good correlation with the visual function.

External ocular manifestation of Wolfram syndrome

Wolfram syndrome is a neurodegenerative disorder usually diagnosed in childhood. The ocular manifestation of wolfram syndrome is mainly characterized by bilateral optic atrophy; however, pigmentary retinopathy has been reported in some cases. We present a case with new external ocular manifestation of wolfram syndrome not previously reported in the literature. A 22-year-old retarded man clinically diagnosed as having wolfram syndrome presented with ocular discomfort in his both eyes for a 4-year period, being aggravated recently. On examination, he was emmetrope with best corrected visual acuity of about two meter count finger in each eye. Pupillary response was sluggish but equal in both eyes. Ocular motility was normal and there was no considerable sign of nystagmus; however, there were disturbed blinking reflex, lagophthalmus, and poor bell's phenomenon. Slit lamp examination showed bilateral symmetrical saucer-like depression of the corneal surface adjacent to the limbos that is known to be deled formation. The result of Schemer test was out of the normal limit, in favor of dry eye. So, dry eye due to blinking disturbances in patients with wolfram syndrome as a neurodegenerative disorder may lead to external ocular manifestations, requiring conservative treatments

evaluation for isolated unexplained optic atrophy: implications for Saudi Arabia

To describe the evaluation of isolated optic atrophy based upon the individual clinical presentation, history and exam of the patient and a directed work up for disorders which are endemic to the patient population of interest. Review of literature and expert opinion. The evaluation of optic atrophy can be difficult and can lead to many expensive or unnecessary tests. The majority of patients with optic atrophy have historical or examination findings that allow the clinician to make an etiologic diagnosis or provide specific guidance for a focused laboratory or radiographic evaluation. Neuro-imaging, preferably cranial magnetic resonance imaging [MRI] with and without contrast and including orbital fat suppression is generally considered the first line evaluation for all patients with unexplained optic atrophy. Directed laboratory or other radiographic testing should be individualized based upon the prevalence of specific disorders in the patient population of interest

Atrofia retinal posterapia fotodinámica en pacientes miópicos con membrana neovascular coroidea / Retinal atrophy post-photodynamic therapy in myopic patients with choroidal neovascular membrane

Describir una complicación retinal en pacientes con Membrana Neovascular Coroidea (MNVC) miópica tratados con Terapia Fotodinámica (TFD) con Verteporfin. Dentro de una serie retrospectiva de 78 ojos con MNVC miópica, se describen 2 casos clínicos de pacientes que desarrollaron atrofia retinal posterior al tratamiento con TFD con Verteporfin en la zona expuesta. Ambos pacientes tenían alteraciones atróficas extensas en su ojo contralateral que no había sido tratado previamente. Se estudiaron posibles factores de riesgo para esta complicación.

Evaluation of the etiology of ocular globe atrophy or loss

Este estudo investigou a etiologia dos casos de atrofia e perda do globo ocular em pacientes atendidos no ambulatório de prótese buco-maxilo-facial de duas Faculdades de Odontologia do estado de São Paulo. Um total de 238 pacientes e seus prontuários foram examinados, e a etiologia e incidência dos casos de atrofia e perda do globo ocular foram avaliados considerando gênero, faixa etária, lado afetado e tipo de cirurgia oftalmológica realizada. As perdas por etiologia traumática foram as de maior incidência (57,14%), seguidas das perdas por etiologia patogênica (36,13%) e com menor incidência para as perdas por causas congênitas (5,04%). Comparando-se os gêneros, houve predominância da população masculina (61.76%; p<0,01). A faixa etária mais freqüentemente atingida foi a de 21 a 40 anos (42.01%; p<0,01). Em todas as etiologias estudadas, a cirurgia mais empregada para remoção do globo ocular foi a enucleação (66.38%; p<0,01). Houve predominância da perda do globo ocular do lado esquerdo (55.04%), mas não houve diferença estatisticamente significante.

Scleroderma, stroke, optic neuropathy: a rare association.

A known case of scleroderma presented with right hemiparesis, focal seizures, optic atrophy and gangrene of digits. There was no evidence of peripheral nerve or muscle involvement. MRI showed multifocal infarcts in both cerebral hemispheres. MR angiography revealed poor flow in bilateral carotid arteries with collateralization from posterior circulation. She improved with phenytoin, nifedipine, antibiotics and immunosuppressants. The rarity of central nervous system affliction in scleroderma and large vessel vasculitis is discussed along with review of literature.

Atrofia Óptica e Doença de Camurati-Engelmann / Optic atrophy and Camurati-Engelmann disease

Objetivo: Relato de caso de atrofia óptica bilateral secundária à Doença de Camurati-Engelmann. Local: Clivan - Instituto de Oftalmologia. Métodos: Os autores descrevem uma criança de 10 meses com nistagmo, estrabismo, atrofia óptica bilateral e alterações esqueléticas. Os exames clínico e radiológico permitiram o diagnóstico da displasia diafisária progressiva - Doença de Camurati-Engelmann. Conclusão: A proliferação óssea na Doença de Camurati-Engelmann pode causar compressão do canal óptico e atrofia óptica. A atrofia óptica na criança, quando detectada, deve ser prontamente investigada a fim de evitar a progressão do dano funcional.

Evaluation of optic nerve atrophy in children

Evaluation of causes and incidence of optic atrophy in children. Revision of patients records through a period from 1990 to 2000. Clinical examination, computed tomography, magnetic resonance image, and histopathological examination were used to establish the diagnosis. one hundred children of unilateral or bilateral optic nerve atrophy were subjected of this study. Seventy children the cause of optic atrophy was known and thirty children had optic atrophy with no identified cause. Established of diagnosis of optic atrophy was based on neur-imaging studies. The most common cause of optic atrophy was tumors especially optic nerve glioma

Cardiac and ocular manifestations in Egyptian patients with mucopolysaccharidoses

Cardiac and ocular manifestations were evaluated in 21 patients clinically suspected of mucopolysaccharidosis. After electrophoresis analysis of urinary glycoaminoglycans, 3 patients were excluded because their results did not correlate with any known type of mucopolysaccharidosis. Echocardiography revealed abnormal findings in 11 patients [61.1%]. The mitral valve was the most commonly affected valve; 7 patients [38.9%] had thickened mitral valve and 6 had mitral regurge. Corneal opacities were found in 3 patients [16.7%] and progressive increase in intraocular pressure in 1 patient [5.6%], while fundus examination showed early optic atrophy in 1 patient [5.6%] and bilateral papilloedema in 2 patients [11.1%]

Presentation of primary open angle glaucoma at Liaquat medical college eye hospital, Hyderabad

To evaluate the presentation of primary open angle glaucoma in hospital admission. Design: Two hundred thirty three glaucoma patients were admitted from January 1995 to April 1996, where 74 patients were primary open angle glaucoma type. Setting: Department of Ophthalmology Unit 1, Eye Hospital, Liaquat Medical College Jamshoro, Hyderabad. Seventy four patients of primary open angle glaucoma. Main outcome measures: Primary open angle glaucoma [POAG] patients presented for treatment in advanced stage of optic nerve head damage. Of the 223 glaucomas, 74 patients had POAG. Of the 74 patients, 31 [41.89%] were male and 43 [58.10%] female. Eight [10.8 1%] of the 74 patients were below 40 years age while all others were above 40 years age. Seven [5.40%] of the 148 eyes had intraocular pressure [I.O.P.] near 20 mmHg, while rest of eyes had I.O.P. above 21 mmHg. Thirty [20.27%] eyes of the 148 eyes had glaucomatous optic atrophy. Majority of primary open angle glaucoma patients presented for their treatment at very late stage. Out of the 148 eyes, 54 eyes were in advanced [.8 and.9 cup disc ratio] stage of optic disc cupping and 30 eyes had complete glaucomatous optic atrophy

Pseudo-hipoparatireoidismo-osteodistrofia hereditária de Albright: relato de um caso / Pseudohypoparathyroidism - Albright's hereditary osteodystrophy

Descriçäo de um caso de diagnóstico tardio de pseudo-hipoparatireoidismo, onde säo apresentadas alteraçöes oftalmológicas como catarata e atrofia óptica. Há pouca mençäo a esta síndrome na literatura oftalmológica, entretanto o oftalmologista é frequentemente o primeiro médico a ser procurado pelo paciente. Os principais sinais e sintomas para um diagnóstico precoce säo discutidos