Glioma of the optic nerve and chiasm: a case report / Glioma óptico-quiasmático: um relato de caso

ABSTRACT This article reports the case of an 11-year-old male patient with a history of proptosis and low progressive visual acuity in the left eye. He presented with a best corrected visual acuity of 20/25 in the right eye and light perception in the left eye. Exotropia and limitation in adduction were observed in the left eye. On automated perimetry, inferiortemporal quadrantopsia was observed in the right eye, while total scotoma was observed in the left eye. On magnetic resonance imaging, there was an expansive lesion in the left optic nerve, extending to the brainstem with chiasmatic involvement. This article aims to report a case of optic pathway glioma, as well as to discuss its clinical findings and their interconnection with the current literature.

RESUMO Este artigo relata o caso de um paciente do sexo masculino, 11 anos de idade, com história de proptose e baixa de acuidade visual progressiva. Ao exame oftalmológico apresentava melhor acuidade visual de 20/25 em olho direito e percepção de luz em olho esquerdo. Existia exotropia e limitação à adução no olho esquerdo. À campimetria automatizada, observou-se quadrantopsia temporal inferior em olho direito e escotoma total em olho esquerdo. À ressonância magnética, evidenciou-se lesão expansiva em trajeto do nervo óptico esquerdo estendendo-se até região do tronco encefálico, com acometimento quiasmático. O objetivo deste artigo é relatar o glioma de vias ópticas, bem como discutir os achados e sua interligação com a literatura atual.

[Results of radiation therapy for optic nerve sheath meningioma]. / Rezul'taty luchevogo lecheniya meningiomy obolochki zritel'nogo nerva.

Optic nerve sheath meningioma (ONSM) is a slow-growing benign tumor that tends to extend intracranially. The tumor is characterized by vision disturbance, optic nerve atrophy and edema. At present, radiotherapy is recommended as the treatment of choice for ONSM. PURPOSE: To assess the neuro-ophthalmic symptomatology of ONSM and how it changes in response to radiotherapy. MATERIAL AND METHODS: In the period from 2004 to 2018, 112 patients with ONSM aged 17 to 76 years underwent stereotactic radiotherapy. In 91 patients, visual functions varied from light perception to 1.0; in 21 patients the affected eye was blind. The prospective follow-up of 103 patients lasted 6 months to 10 years (mean follow-up duration was 57 months). Studied patients underwent either conventional fractionated radiotherapy with Novalis (n=88) or hypofractionation radiotherapy with CyberKnife (n=24). RESULTS: According to ophthalmological examination, in 60.5% of cases an improvement of visual functions was observed, 39.5% of patients had visual functions remain stable at the initial level. No vision impairment during irradiation was detected. Vision deterioration in the long-term follow-up was observed in 2 patients: extended tumor growth in one case and radio-induced retinopathy in the other. CONCLUSION: The study showed that this method of treatment for ONSM is effective and safe, allowing preservation of visual functions and in some cases - its improvement, while tumor growth remains under control.

Role of the optic nerve sheath diameter in the assessment of the effectiveness of decompressive surgery after malignant middle cerebral artery infarction / Papel do diâmetro da bainha do nervo óptico na avaliação da eficácia da cirurgia descompressiva após infarto maligno da artéria cerebral média

Abstract Background After a case of stroke, intracranial pressure (ICP) must be measured and monitored, and the gold standard method for that is through an invasive technique using an intraventricular or intraparenchymal device. However, The ICP can also be assessed through a non-invasive method, comprised of the measurement of the optic nerve sheath diameter (ONSD) through ultrasound (US). Objective To evaluate the ICP of patients who underwent wide decompressive craniectomy after middle cerebral artery (MCA) infarction via preoperative and postoperative ONSD measurements. Methods A total of 17 patients, aged between 34 and 70 years, diagnosed with malignant MCA infarction with radiological edema and mid-line shift, who underwent decompressive surgery, were eligible. From the records, we collected data on age, sex, preoperative and postoperative Glasgow Coma Scale (GCS) scores, National Institutes of Health Stroke Scale (NIHSS) score, the degree of disability in the preoperative period and three months postoperatively through the scores on the Modified Rankin Scale (MRS), and the preoperative and postoperative midline shift measured by computed tomography (CT) scans of the brain. Results Preoperatively, the mean GCS score was of 8 (range: 7.7-9.2), whereas it was found to be of 12 (range 10-14) on the first postoperative day (p = 0.001). The mean preoperative NIHSS score was of 21.36 ± 2.70 and, on the first postoperative day, it was of 5.30 ± 0.75 (p < 0.001). As for the midline shift, the mean preoperative value was of 1.33 ± 0.75 cm, and, on the first postoperative day, 0.36 ± 0.40 cm (p < 0.001). And, regarding the ONSD, the mean preoperative measurement was of 5.5 ± 0.1 mm, and, on the first postoperative day, it was of 5 ± 0.9 mm (p < 0.001). Conclusion The ocular US measurement of the ONSD for the preoperative and postoperative monitoring of the ICP seems to be a practical and useful method.

Resumo Antecedentes Após um acidente vascular cerebral (AVC), a pressão intracraniana (PIC) deve ser medida e monitorada, e o método padrão-ouro para isso é um procedimento invasivo por meio de um dispositivo intraventricular ou intraparenquimal. No entanto, a PIC também pode ser avaliada por um método não invasivo, composto da medida do diâmetro da bainha do nervo óptico (DBNO) por ultrassom (US). Objetivo Avaliar a PIC de pacientes submetidos a craniectomia descompressiva ampla após infarto da artéria cerebral média (ACM) por meio das medidas do DBNO nos períodos pré e pós-operatório. Métodos Um total de 17 pacientes, com idades entre 34 e 70 anos, diagnosticados com infarto maligno da ACM com edema radiológico e deslocamento da linha média, e que foram submetidos a cirurgia descompressiva, eram elegíveis. A partir dos prontuários, coletamos informações relativas à idade, gênero, pontuações pré e pósoperatória na Escala de Coma de Glasgow (ECG), pontuação na escala de AVC dos National Institutes of Health (NIH), o grau de incapacidade no pré-operatório e após três meses da operação pelas pontuações na Escala de Rankin Modificada (ERM), e o desvio da linha média no pré e pós-operatório medido por tomografia computadorizada (TC) cerebral. Resultados No pré-operatório, a pontuação média na ECG foi de 8 (variação: 7,7-9,2), e, no primeiro dia do pós-operatório, 12 variação 10-14) (p = 0,001). A pontuação média na escala dos NIH foi de 21,36 ± 2,70 no pré-operatório, e de 5,30 ± 0,75 no primeiro dia de pós-operatório (p < 0,00 1). Quanto ao desvio da linha média, no préoperatório ele teve uma média de 1,33 ± 0,75 cm, e de 0,36 ± 0,40 cm no primeiro dia de pós-operatório (p < 0,001). E o DBNO apresentou uma média pré-operatória de 5,5 ± 0,1 mm, e de 5 ± 0,9 mm no primeiro dia de pós-operatório (p < 0,001). Conclusão A mensuração ocular do DBNO por US para o monitoramento da PIC no pré e no pós-operatório parece ser um método prático e útil.

The association between intracranial pressure and optic nerve sheath diameter on patients with head trauma / Associação entre a pressão intracraniana e o diâmetro da bainha do nervo óptico em pacientes com traumatismo cranioencefálico

Abstract Background: Although intracranial pressure (ICP) monitoring is the gold standard method for measuring intracranial pressure after traumatic brain injury, optic nerve sheath diameter (ONSD) measurement with ultrasound (US) is also used in the evaluation of ICP. Objective: To investigate the association between a series of OSND measurements by US and changes in clinical presentation of the patient. Methods: Prospective study including 162 patients with traumatic brain injury. Age, sex, cerebral CT findings, ONSD levels by US at minutes 0, 60, and 120, Glasgow Coma Scale (GCS) within same period, change of consciousness, treatment, and mortality data were reviewed. The association of ONSD levels with GCS, change of consciousness, treatment, and mortality was evaluated. Results: There was no difference in ONSD changes in the patients' sample within the period (p=0.326). ONSD significantly increased in patients who died (p<0.001), but not in those who survived (p=0.938). There was no significant change in ONSD of the patients who received anti-edema therapy (p=801), but significantly increased ONSD values were found in those who received anti-edema therapy (p=0.03). Patients without change of consciousness did not have any significant change in ONSD (p=0.672), but ONSD values increased in patients who consciousness became worse, and decreased in those who presented a recovery (respectively, p<0.001, p=0.002). A negative correlation was detected between ONSD values and GSC values measured at primary, secondary, and tertiary time periods (for all p<0.001). Conclusions: ONSD follow-up may be useful to monitor ICP increase in patients with acute traumatic brain injury.

RESUMO Antecedentes: Embora o monitoramento da pressão intracraniana (PIC) seja o método padrão-ouro para medir a pressão intracraniana após lesão encefálica traumática, a medição do diâmetro da bainha do nervo óptico (DBNO) com ultrassom (US) também é usada na avaliação da PIC. Objetivo: Investigar a associação entre uma série de medidas de DBNO por US e mudanças na apresentação clínica do paciente. Métodos: Estudo prospectivo incluindo 162 pacientes com traumatismo cranioencefálico. Idade, sexo, achados de TC cerebral, níveis de DBNO por US nos minutos 0, 60 e 120, Escala de Coma de Glasgow (GCS) no mesmo período, mudança de consciência, tratamento e dados de mortalidade foram revisados. A associação dos níveis de DBNO com GCS, mudança de consciência, tratamento e mortalidade foi avaliada. Resultados: Não houve diferença nas mudanças de DBNO na amostra de pacientes no período (p=0,326). O DBNO aumentou significativamente em pacientes que morreram (p<0,001), mas não naqueles que sobreviveram (p=0,938). Não houve mudança significativa no DBNO dos pacientes que receberam terapia antiedema (p=801), mas valores significativamente aumentados de DBNO foram encontrados naqueles que receberam terapia antiedema (p=0,03). Pacientes sem alteração da consciência não tiveram alteração significativa no DBNO (p=0,672), mas os valores do DBNO aumentaram nos pacientes que pioraram a consciência e diminuíram naqueles que apresentaram recuperação (respectivamente, p<0,001, p=0,002). Detectou-se correlação negativa entre os valores de DBNO e os valores de GSC medidos nos períodos primário, secundário e terciário (para todos, p<0,001). Conclusões: O acompanhamento do DBNO pode ser útil para monitorar o aumento da PIC em pacientes com lesão cerebral traumática aguda.

Misunderstandings and controversies in the diagnosis and treatment of optic nerve sheath meningioma / 中华眼底病杂志

Optic nerve sheath meningiomas (ONSM) account for about 1%-2% of all meningiomas. Most of them are benign and the disease progresses slowly. ONSM is a relatively rare intraorbital benign tumor in clinical practice. Due to its close relationship with the optic nerve, the diagnosis and treatment are special, so there are many controversies and misunderstandings in the diagnosis and treatment of ONSM. ONSM is often misdiagnosed as acute optic nerve papillitis, optic nerve atrophy, ischemic optic neuropathy, acute retrobolic optic neuritis, optic disc vasculitis or optic fatigue due to its similar clinical features to other optic nerve diseases such as optic neuritis. The treatment includes observation, surgical treatment and radiotherapy, and appropriate treatment should be selected for different patients according to the changes of their condition. Therefore, understanding of the controversies and misunderstandings in the diagnosis and treatment of ONSM is of great clinical significance for timely and accurate diagnosis, appropriate treatment and improvement of the prognosis of patients.

von Hippel-Lindau Syndrome: Genetic Study of Case With a Rare Pathogenic Variant With Optic Nerve Hemangioblastoma, a Rare Phenotypic Expression.

von Hippel-Lindau syndrome (VHLS) is a rare, autosomal dominant genetic disease with high penetrance and variable phenotypic expression caused by variants in the VHL gene. VHLS is associated with the presence of vascular tumors, often hemangioblastoma of the central nervous system, retina, or spinal cord and, less frequently, pancreatic cystic neoplasm, pancreatic neuroendocrine tumor, clear cell carcinoma of the kidney, endolymphatic sac tumor, pheochromocytoma, and paraganglioma. The authors report a case of a patient with VHLS with a rare pathogenic variant in the VHL gene and with an optic nerve hemangioblastoma, a rare phenotypic expression. Case report: A 49-year-old woman was diagnosed with cystic neoplasm of the pancreas, renal cell carcinoma of the right kidney, and hemangioblastoma of the left optic nerve. The patient's family history revealed siblings with VHLS manifestations. The index case was her mother who died at age 63 of clear cell renal carcinoma. The information was obtained by consulting the patient's medical register and by interviews with the patient and her relatives. The presence of left optic nerve hemangioblastoma was suggested by CT scan of the skull and orbit. The sequencing of the VHL gene was performed in the peripheral blood by the polymerase chain reaction (PCR) technique, and the duplication and deletion research was performed using the multiplex ligation-dependent probe amplification (MPLA) technique. The presence of a rare pathogenic variant c.263G> A (p.Trp88Ter) was observed in heterozygosity in the VHL gene that determined a premature stop codon. CT scan of the skull and orbits suggested the presence of HB in the optic nerve of the left eye. The results of the CT scan of the skull and orbits show thickening with tortuosity of the left optic nerve, with a small area of nodular enhancement. The right optic nerve had a conserved aspect. Conclusion: This is the fourth case described of this rare pathogenic variant of the VHL gene, according to the Human Gene Mutation Database and VHLdb database records and with an optic nerve hemangioblastoma of the optic nerve, a very rare phenotypic expression of the VHLS.

Analysis of clinical characteristics of optic nerve metastases / 中华眼底病杂志

Objective To explore the clinical features,primary lesions and prognosis of optic nerve metastases.Methods Seven patients (11 eyes) with optic nerve metastatic tumor diagnosed by the examinations of ophthalmology,laboratory and pathology in Chinese PLA General Hospital from April 2015 to September 2017 were included in this study.All patients underwent BCVA,flash VEP,OCT,orbital MRI,serum tumor marker,cerebral spinal fluid detection and PET-CT.Histopathological examination of primary or near superficial metastases was performed.The follow-up period was ranged from 16 to 44 months,with the mean of 23.0 ± 10.9 months.The clinical characteristics,primary tumor,imaging features,treatment and clinical prognosis in the patients were analyzed.Results Among 7 patients,there were 5 males and 2 females,with the mean age of 53.90± 14.99 years;3 patients with unilateral optic nerve involvement,4 patients with bilateral optic nerve involvement;5 patients (71.4％) first diagnosed in ophthalmology.Five patients (45.5％) were misdiagnosed as optic neuritis,optic disc edema in 6 eyes (54.5％).All of them appear loss of visual acuity,including 8 eyes (72.7％) with BCVA＜ 0.1,2 eyes (18.2％) with BCVA 0.1-0.5,1 eye (9.1％) with BCVA ＞ 0.5.MRI results show that 1 patient with intraorbital segment,1 patient with internal segment of optic canal,4patients with intracranial segment,1 patient with intracranial segment and optic chiasma involved simultaneously,4 patients involving surrounding tissue.There were 4 patients (57.1％) with lung cancer,2 patients (28.6％) with kidney cancer,1 patient (14.3％) with gastric cance;6 patients (85.7％) with metastasis from other sites,2 patients with brain metastasis (1 patient with meningitis carcinomatosa).There were 2 patients (28.6％) with previous primary cancer surgery.After diagnosis,1 patient received chemotherapy,1 patient received radiotherapy,5 patients gave up treatment.At the end of follow-up,1 patient (1 eye) of chemotherapy with BCVA increased by 2 line;1 patient (2 eyes) of radiotherapy with no change in BCVA;of the 5 patients who gave up treatment,1 patient died of disease,1 patient lost follow-up,and 3 patients (4 eyes) had no change in BCVA.Conclusions With atypically clinical manifestations,the optic nerve metastases easily misdiagnosed as optic neuritis,and with poor therapeutic effect.Primary lesions are mostly found in lung cancer.

Optic nerve sheath meningiomas: prevalence, impact, and management strategies.

Optic nerve sheath meningiomas are rare benign neoplasms of the meninges surrounding the optic nerve. They are a significant cause of morbidity. While the mortality rate is practically zero, these tumors can blind or disfigure patients. Given that the clinical course can be variable, and treatment has the capacity to cause morbidity itself, the management of these patients can be difficult. We review the literature to discuss the prevalence of optic nerve sheath meningiomas, the association with neurofibromatosis type 2, natural history, and management options and strategies.

Meningioma de las vainas del nervio óptico / Meningioma of the optic nerve sheaths

RESUMEN Introducción: los meningiomas de la vaina del nervio óptico son tumores meningoteliales originados de células aracnoideas que producen disminución de la visión y proptosis con un compromiso ocular variable. Presentación del caso: se trata de una paciente de 65 años de edad con antecedentes de hipertensión arterial que ingresó por proptosis, disminución de la visión y quemosis conjuntival del ojo derecho, evidenciándose en el examen del fondo de ojo la presencia de disco óptico pequeño de bordes definidos de coloración normal, sin compromiso del grosor de la capa de fibras nerviosas de la retina con dilatación vascular moderada a predominio venoso sin evidencia de alteraciones maculares, hemorragias ni exudados. Los estudios campimétricos evidenciaron la presencia de escotoma altitudinal con toma del cuadrante temporal inferior y el estudio de los potenciales evocados visuales mostró caída de su amplitud. Mediante el estudio de resonancia magnética nuclear se observó la existencia de una imagen isointensa homogénea de bordes bien definidos, que no comprometía la estructura axonal del nervio óptico y que respeta la porción intracanalicular e intracraneal, confirmándose el diagnóstico de meningioma de las vainas del nervio óptico. Conclusiones: Se confirma el diagnóstico de meningioma de las vainas del nervio óptico del ojo derecho en una paciente con proptosis unilateral y quemosis conjuntival, lo que constituye una inusual forma de presentación con manifestaciones oftalmológicas.

ABSTRACT Introduction: meningiomas of the optic nerve sheath are meningothelial tumors originating from arachnoid cells that produce decrease of vision and proptosis with variable ocular involvement. Case report: a 65-year-old female patient with a history of hypertension who was admitted due to proptosis, decrease of vision and conjunctival chemosis of the right eye; fundus of the eye examination revealed the presence of a small optic disc of defined edges of normal coloration, without compromise of the thickness of the layer of nervous fibers of the retina with moderate vascular dilatation to venous predominance without evidence of macular alterations, hemorrhages or exudates. The campimetric studies showed the presence of altitudinal scotoma with lower temporal quadrant capture, and the study of the visual evoked potentials showed fall of its amplitude. Through the study of nuclear magnetic resonance (NMR, the existence of a homogeneous isointense image of well-defined edges was observed, which did not compromise the axonal structure of the optic nerve and which respects the intracanalicular and intracranial portion that confirmed the diagnosis of meningioma of the optic nerve sheaths. Conclusions: the diagnosis of meningioma of the optic nerve sheaths on the right eye was confirmed in a patient with unilateral proptosis and conjunctival chemosis, which constitutes an unusual presentation with ophthalmological manifestations.

Optic neuropathy in the context of leukemia or lymphoma: diagnostic approach to a neuro-oncologic emergency.

BACKGROUND: Optic neuropathy in the context of leukemia or lymphoma has a broad differential diagnosis, including infiltration, infection, inflammation, compression, and medication effects. Confirming the underlying etiology in a timely manner is crucial as, while infiltration carries a poor prognosis, treatment modalities can have serious consequences themselves. METHODS: A review of the literature was conducted for cases of isolated optic neuropathy in the context of leukemia or lymphoma, in which the underlying etiology remained unclear following initial clinical examination and neuroimaging. Clinical, radiological, and pathological characteristics of the cases are summarized. RESULTS: Ninety-two cases meeting inclusion criteria were identified. Leukemic or lymphomatous infiltration was the presumed diagnosis in 72% of the reports, indicating this is the most likely etiology in such cases. The remaining reports were attributed to inflammation, infection, or drug toxicity. For illustrative purposes, the previously unpublished case of an 11-year-old girl with remitted T lymphoblastic lymphoma is presented. She suffered recurrence in the form of isolated left optic nerve infiltration that required transconjunctival biopsy to confirm diagnosis. CONCLUSIONS: Optic nerve infiltration by leukemia or lymphoma requires both diagnostic certainty and urgent management. Recommendations are made for a step-wise, yet rapid investigative approach that may ultimately require biopsy of the optic nerve.

[Apropos of 5 cases of optic nerve tumors diagnosed during a 6-year-period]. / À propos de 5 cas de tumeurs du nerf optique sur une période de 6ans.

INTRODUCTION: Optic nerve tumors are uncommon and rarely described. By way of five patients treated at the University Hospital of Clermont-Ferrand, France, over a six-year-period, we discuss their clinical and radiological characteristics, their treatment and their course. OBSERVATIONS: We report two cases of optic nerve glioma in small children, two cases of optic nerve sheath meningioma and a 57-year-old patient treated for lung cancer with an optic nerve metastasis and choroidal and brain metastases. The diagnosis was confirmed by imaging in all patients and histologically for the cases of optic nerve glioma. RESULTS: The children with glioma were exclusively treated with chemotherapy, the two women with meningioma exclusively with radiation therapy, and the man with the optic nerve metastasis by chemotherapy and radiotherapy. The tumors were stabilized in all cases except for a progression in one case of glioma, one year after treatment. DISCUSSION: Gliomas and meningiomas are the most frequent tumors; other tumors are rare. Through a literature review and many illustrations, we discuss epidemiologic, clinical and radiologic characteristics, treatment and course of these uncommon tumors. CONCLUSION: The diagnosis of optic nerve tumors must be considered in cases of anterior or posterior progressive optic neuropathy. Treatment must be a compromise between effective treatment of the tumor and preservation of visual function. Decisions are made through multidisciplinary consultations, in which the role of the ophthalmologist is crucial for the diagnosis and success of the treatment.

Autofluorescence and spectral-domain optical coherence tomography of optic disk melanocytoma / Autofluorescência e tomografia de coerência óptica de domínio espectral do melanocitoma do disco óptico

The authors report fundus autofluorescence (FAF) and spectral-domain optical coherence tomography (OCT) findings of two consecutive patients who presented with optic disk melanocytoma (ODM). A retrospective study was performed by reviewing medical records and ophthalmic imaging examinations. Optical coherence tomography findings were sloped and brightly reflective anterior tumor surface, adjacent retinal desorganization and abrupt posterior optical shadowing. Vitreous seeds were found in one patient. Fundus autofluorescence revealed outstanding hypoautofluorescence at the tumor area and isoautofluorescence at the remaining retina. Optical coherence tomography findings of the reported cases are consistent with those reported in the reviewed literature. Fundus autofluorescence has been used in the assessment of choroidal melanocytic tumors, but not yet in melanocytomas. We assume that this is the first report of these findings and believe that when its pattern has become clearly defined, fundus autofluorescence will be a useful tool to avoid misdiagnosis in suspicious cases and for follow-up.

Os autores descrevem os achados do exame de autofluorescência do fundo de olho (AF) e da tomografia de coerência óptica (TCO) de domínio-espectral em dois pacientes consecutivos apresentando melanocitoma disco óptico (MDO) em um estudo retrospectivo, por revisão dos prontuários e exames oftalmológicos de imagem. Os achados no exame de tomografia de coerência óptica foram hiperrefletividade e elevação da superfície anterior da lesão, desorganização da retina adjacente, e, sombreamento óptico posterior. Sementes vítreas foram encontrados em um paciente. O exame de autofluorêscencia do fundo de olho revelou hipoautofluorescência marcante na área do tumor e isoautofluorescência nas demais áreas da retina. As características encontradas na tomografia de coerência óptica dos pacientes apresentados são consistentes com os achados relatados na literatura atual. A autofluorêscencia do fundo de olho tem sido utilizada na avaliação de tumores melanocíticos da coroide, mas ainda não em melanocitomas. Acreditamos que este seja a primeira descrição dos achados de autofluorêscencia do fundo de olho em pacientes com melanocitoma de disco óptico e que, quando seu padrão de tornar-se claramente definido, a autofluorêscencia do fundo de olho será uma ferramenta útil para evitar erros de diagnóstico em casos suspeitos.

Optic nerve sheath meningioma in the first decade of life: case report and review of the literature.

BACKGROUND/AIM: Tumors of the optic nerve are mostly either optic nerve gliomas or optic nerve sheath meningiomas (ONSMs). While gliomas occur in children, most meningiomas are slow-growing tumors affecting middle-aged individuals with progressive visual loss, proptosis, disc edema and optociliary veins on fundus examination. ONSMs are extremely rare in children, with only 14 cases reported in children under the age of 10 years. The purpose of this study is to describe an additional case and review clinical, radiologic and histopathological findings that can help differentiate such tumors in children and allow an early and accurate diagnosis. CASE REPORT: An 8-year-old girl had a 2-year history of progressive proptosis and total visual loss on the right side. A computed tomography scan revealed a well-defined intraconal mass with perioptic calcification. Magnetic resonance imaging showed a tumor surrounding the optic nerve and extending intracranially, with enhancement following gadolinium injection. The patient was submitted to fronto-orbital craniotomy for complete tumor excision. Histological studies identified the neoplasm as ONSM. CONCLUSIONS: Though unusual, ONSMs may occur in children and, in these cases, present a much more aggressive behavior than in adults. ONSM should therefore be differentiated from optic glioma in children because of its aggressive behavior and need for different treatment modalities.

Melanocitoma associado à membrana neovascular sub-retiniana / Melanocytoma associated to subretinal neovascular membrane

Relatamos caso de melanocitoma de disco óptico associado à membrana neovascular sub-retiniana. O paciente apresentava diminuição da visão, presença de descolamento seroso de retina, com hemorragia e exsudação e foi encaminhado com o diagnóstico de melanoma de coróide e sugestão de enucleação. São discutidas as principais características dos dois tumores e os detalhes do diagnóstico diferencial.

We report a case of optic disc melonocytoma associated to subretinal neovascular membrane. The patient presented with low visual acuity, serous retinal detachment with haemorrhage and exudates and was referred with the diagnosis of melanoma and an orientation to enucleate the eye. We discuss the major aspects of both tumors and the details of differential diagnostic.

Melanocitoma do nervo óptico: [revisão] / Optic disk melanocytoma: bibliographic review: [review]

O melanocitoma do disco óptico é bem conhecido como uma variante do nervo melanocítico que geralmente ocorre no disco óptico. Historicamente o tumor foi confundido com melanoma maligno. Histopatologicamente é composto por células redondas densamente pigmentadas com citoplasma abundante e pequenos nucléolos. O melanocitoma é considerado um tumor benigno, estacionário e com pouca predisposição de sofrer malignação. Na maioria dos casos são assintomáticos, mas podem apresentar defeitos pupilares e no campo visual. É importante salientar que a transformação maligna em melanoma ocorre em 1 a 2 por cento dos casos. Assim, os oftalmologistas devem familiarizar-se com o melanocitoma do disco óptico e os pacientes afetados devem ser acompanhados periodicamente.

Melanocytoma of the optic disc is a well known variant of melanocytic nevus that usually occurs as a deeply pigmented lesion on the head of the optic disc. Historically, this tumor has often been confused with malignant melanoma. Histopathologically, it is composed of deeply pigmented round oval cells with abundant cytoplasm and small, round, bland nuclei. Melanocytomas is a benign, stationary tumors, with almost no propensity to undergo malignant transformation. Most cases that occur on the optic disc are visually asymptomatic, but they can cause an afferent pupillary and visual field defects. Importantly, it can exhibit malignant transformation into melanoma in 1 to 2 percent of the cases. The affected patients should have periodic follow up.

Preoperative diagnosis and microsurgery of the optic gliomas:retrospective analysis of 7 patients / 北京大学学报(医学版)

Objective: To summarize the clinical characteristics and therapeutic experience of optic gliomas. Methods: The clinical data of 7 patients of our department in the past 3 years were analyzed. Results: In this series of optic glioma patients, the average age was 18.7 years, the ratio of men to women was 4∶3,and 100% of the tumors were sub-totally removed. Diabetes inspidus was the most comman postoperative complication. Conclusion: The optic glioma is benign intracranial tumor with good prognosis. The key point of treatment is surgical resection combined with proper postoperative radiotherapy.