GROSS TOTAL RESECTION IN A RARE CASE OF OPTIC NERVE ASTROCYTOMA: A CASE REPORT.

The authors present a 3-year-old female with increasing proptosis and absent vision in the right eye. Chemotherapy had done for 3months. But her ailments lingered. The right eye exhibited severe proptosis and poor vision, whereas the left eye was normal with 20/20 vision. Preoperative MRI revealed a dumbbell-shaped tumor in the intra-orbital and intra-cranial section of the right optic nerve. A lateral supra-orbital approach was used to dissect the dumbbell-shaped tumor and the right optic nerve. No remnant of the tumor was discovered during a follow-up examination. The case study demonstrates how to identify and treat ONA surgically. However, we need further research on optic nerve PA to gain a better understanding of their behavior. While gross total resection (GTR) is usually curative, tumors in deep locations may be unresectable and require alternative therapeutic procedures. Additionally, the case study emphasizes the importance of additional research on early detection and prevention.

[Hypofractionated stereotactic irradiation for optic nerve glioma]. / Stereotaksicheskoe obluchenie gliom zritel'nogo nerva v rezhime gipofraktsionirovaniya.

Optic nerve glioma is a rather rare tumor. It predominantly arises in pediatric patients, including those with type I neurofibromatosis. This neoplasm is accompanied by decreased visual function and exophthalmos. Treatment strategy is individualized depending on age, volume and spread of tumor, as well as severity of clinical manifestations. Possible treatment options are surgical resection, chemotherapy, radiotherapy and their combination. Radiotherapy can be recommended for patients with intact visual functions, no severe proptosis and trophic lesions. Classic fractionation mode is used as a standard. Currently, the possibility of hypofractionated irradiation is being considered. OBJECTIVE: To evaluate safety and efficacy of hypofractionated radiotherapy in patients with optic nerve glioma. MATERIAL AND METHODS: Sixteen patients with optic nerve gliomas underwent hypofractionated stereotactic irradiation (CyberKnife) between May 2014 and October 2019. Single focal dose was 5.5 Gy. There were 5 fractions up to total focal dose of 27.5 Gy. The sample enrolled 14 children with a median age of 4 years (range 23 months - 13 years) and 2 adults aged 47 and 66 years, respectively. Median of tumor volume was 2.77 cm3 (range 1.69-10.01 cm3). RESULTS: Tumor growth control was achieved in all patients, partial remission was observed in 5 (32%) patients. None patient had deterioration of visual function. Improvement of visual acuity was noted in 3 (19%) cases. Visual field enlargement occurred in 4 (67%) out of 6 patients who were preoperatively examined. After irradiation, proptosis decreased by ≥ 1 mm in 9 (60%) out of 15 patients.

Pilocytic astrocytoma of the optic nerve with intracystic hemorrhage in an adult: illustrative case.

BACKGROUND: Optic pathway gliomas are uncommon, accounting for 3-5% of childhood brain tumors, and are mostly classified as pilocytic astrocytomas (PAs). PAs of the optic nerve are particularly rare in adults. OBSERVATIONS: The authors presented the case of PA of the left optic nerve in a 49-year-old woman along with detailed pathological and molecular analyses and sequential magnetic resonance imaging. The tumor had progressed during 5 years of follow-up along with cyst formation and intracystic hemorrhage; it had a thick capsule and contained xanthochromic fluid. The boundary between tumor and optic nerve was unclear. B-type Raf kinase (BRAF) V600E point mutations or translocations, IDH1-R132H mutations, loss of alpha-thalassemia/mental retardation X-linked, and 1p/19q codeletion were negative. LESSONS: BRAF alterations in pediatric PAs of the optic nerve are less frequent than those observed in PAs in other lesions; the same molecular pattern was observed in the adult case, without changes in BRAF. Surgical management should be indicated only in cases with severely impaired vision or disfigurement because there is no clear border between the tumor and optic nerve. Further discussion is needed to optimize the treatment for adult optic pathway gliomas, including radiotherapy, chemotherapy, and molecular-targeted therapies, in addition to surgical intervention.

An elusive case of malignant optic nerve glioma with a suprasellar mass.

A 67-year-old man with multiple comorbidities presented with 3 weeks of orbital pain, proptosis, and no light perception vision in the left eye. Examination was notable for a left orbital apex syndrome with CN III, VI palsies, an optic neuropathy, and central retinal vein occlusion. Magnetic resonance imaging of the orbits was notable for extensive enhancement, enlargement, and T2 hyperintensity of the optic nerve, with perineural sheath enhancement, and chiasmal hyperintensity. Inflammatory workup and lumbar puncture were unremarkable. No improvement was seen after a 3-day course of intravenous solumedrol. Initial optic nerve biopsy revealed necrotic nerve tissue, macrophage infiltration, increased vascularization, and peripheral gliosis. The volume of tissue was inadequate for genomic analysis. The patient was lost to follow-up but returned 5 months later with right-sided vision loss. Repeat neuroimaging showed a new suprasellar mass and progressive expansion and enhancement of both optic nerves. Biopsies of the suprasellar mass and left nerve at this time were consistent with a high-grade glial neoplasm, WHO grade IV. This is a rare case of glioblastoma involving the optic nerves and suprasellar region. In such cases, molecular profiling can improve diagnosis and may provide for targeted treatments in the future.

A Rare Case of Orbital Adenoid Cystic Carcinoma Mimicking as Optic Nerve Glioma.

Adenoid cystic carcinoma is an uncommon epithelial cell tumour that usually originates from glands. They arise from the upper respiratory tract, lungs, mammary glands, and skin, but most commonly from the salivary glands and lacrimal glands. Our article reports a 53-year-old individual presenting with a history of diminution of visual acuity over the past one and a half months, also associated with a right-sided headache and throbbing eye pain not relieved by medication. Examination revealed right axial proptosis, ptosis, and visual acuity of 6/36, right afferent pupillary defect (RAPD), restriction of ocular movements in supraduction, dextroelevation, and abduction. The fellow eye was completely normal. MRI revealed an enhancing lesion in the retrobulbar area of the right orbit indicative of optic nerve glioma of stage 2. The patient underwent orbito-zygomatic craniotomy with subtotal excision of the mass by a neurosurgeon. Following surgery, histopathological examination of the excised tumour revealed features consistent with adenoid cystic carcinoma. On the third post-operative day, the subject's vision improved to counting fingers at 3 metres, and extraocular movements were regained.

Neurological manifestations of neurofibromatosis type 1: our experience.

INTRODUCTION: Neurofibromatosis type 1 (NF1) is a progressive multisystem disorder following an autosomal dominant inheritance pattern that presents with multiple neurological manifestations. METHODS: We reviewed medical histories of patients with NF1 followed up at our hospital's paediatric neurology department from May 1990 to 31 December 2018. We collected data on neurological symptoms. RESULTS: A total of 128 patients with NF1 were identified. Mean age (SD) at NF1 diagnosis was 4.43 (3.38) years (range, 0.5-14.5 years). There was a slight female predominance (53.1%). Macrocephaly (head circumference over 2 SDs above average for age) was present in 37.5% of cases. Attention-deficit/hyperactivity disorder was recorded in 28.9% of patients (37): combined type in 20 patients, predominantly inattentive in 15, and predominantly impulsive/hyperactive in 2. Other manifestations included headache (18.6%), cognitive impairment (7.8%), motor deficit (6.2%), and epilepsy (4.68%). Brain MRI was performed in 85 patients, revealing T2-weighted hyperintensities in the basal ganglia and/or cerebellum in 60 patients (70.5%), Chiari malformation type 1 in 4 cases, and arachnoid cysts in 3. Optic nerve gliomas were identified by MRI in 22 patients (25.8%). Other MRI findings included plexiform neurofibromas (9.3%) and central nervous system gliomas (3.1%). CONCLUSIONS: The neurological manifestations identified in our sample are consistent with those reported in the literature. Effective transfer strategies from paediatric neurology departments and subsequent clinical follow-up by adult neurology departments are needed to prevent loss to follow-up in adulthood.

A rare MRI finding of NF-1: perineural arachnoidal gliomatosis.

Optic pathway gliomas are the most common central nervous system neoplasms in patients with neurofibromatosis type 1. Perineural arachnoidal gliomatosis is a rare and distinctive growth pattern of optic nerve glioma, in which the tumor infiltrates through the pia mater and pre-dominantly involves the subarachnoid space around the optic nerve. Here, we report an 8-year-old girl with perineural arachnoidal gliomatosis associated with neurofibromatosis type 1.

Manifestaciones neurológicas en neurofibromatosis tipo 1. Nuestra experiencia / Neurological manifestations of neurofibromatosis type 1: our experience

Introducción: La neurofibromatosis tipo 1 (NF1) es un desorden progresivo multisistémico de herencia autosómica dominante que presenta numerosas manifestaciones neurológicas. Métodos: Revisión de historias clínicas de pacientes afectos de NF1 controlados en una Unidad de Neuropediatría de mayo de 1990 a 31 de diciembre de 2018 y sus manifestaciones neurológicas asociadas. Resultados: Se revisaron 128 pacientes afectos de NF1. Edad media al diagnóstico de NF1, 4,43 años ± 3,38 SDS (rango 6 meses-14,5 años) con discreto predominio femenino (53,1%). Se asocia macrocefalia (PC> 2SDS) en el 37,5% de los casos. TDAH en el 28,9% de los casos (37), subtipo combinado 20, inatento 15 casos y predominantemente hiperactivo 2 casos. Otras manifestaciones incluyen; cefalea (18,7%), déficit cognitivo (7,8%), afectación motora (6,2%) y epilepsia (4,68%). Se realizó RM cerebral a 85 pacientes, mostrando 60 (70,5%) hiperseñales en T2 en ganglios basales y/o cerebelo, junto con otras alteraciones como Chiari I (4 casos) y quistes aracnoideos (3 casos). Se identificaron gliomas de nervio óptico en 22 casos (25,8%). Otros hallazgos diagnosticados por RM incluyen neurofibromas plexiformes (9,3%) y otros gliomas localizados en sistema nervioso central (3,1%). Conclusiones: Las manifestaciones neurológicas encontradas concuerdan con lo recogido en la literatura. El seguimiento de estos pacientes se pierde en la edad adulta, siendo necesario establecer adecuadas estrategias de transferencia y posterior seguimiento de pacientes a los servicios de adultos. (AU)

Introduction: Neurofibromatosis type 1 (NF1) is a progressive multisystem disorder following an autosomal dominant inheritance pattern that presents with multiple neurological manifestations. Methods: We reviewed medical histories of patients with NF1 followed up at our hospital's paediatric neurology department from May 1990 to 31 December 2018. We collected data on neurological symptoms. Results: A total of 128 patients with NF1 were identified. Mean age (SD) at NF1 diagnosis was 4.43 (3.38) years (range, 0.5-14.5 years). There was a slight female predominance (53.1%). Macrocephaly (head circumference over 2 SDs above average for age) was present in 37.5% of cases. Attention-deficit/hyperactivity disorder was recorded in 28.9% of patients (37): combined type in 20 patients, predominantly inattentive in 15, and predominantly impulsive/hyperactive in 2. Other manifestations included headache (18.6%), cognitive impairment (7.8%), motor deficit (6.2%), and epilepsy (4.68%). Brain MRI was performed in 85 patients, revealing T2-weighted hyperintensities in the basal ganglia and/or cerebellum in 60 patients (70.5%), Chiari malformation type 1 in 4 cases, and arachnoid cysts in 3. Optic nerve gliomas were identified by MRI in 22 patients (25.8%). Other MRI findings included plexiform neurofibromas (9.3%) and central nervous system gliomas (3.1%). Conclusions: The neurological manifestations identified in our sample are consistent with those reported in the literature. Effective transfer strategies from paediatric neurology departments and subsequent clinical follow-up by adult neurology departments are needed to prevent loss to follow-up in adulthood. (AU)

Visual Outcomes After Treatment for Sporadic Optic Pathway Gliomas in Pediatric Patients: A Systematic Review.

OBJECTIVE: Optic pathway gliomas (OPGs) typically occur in the first decade of life and 40%-50% are not associated with neurofibromatosis 1 (NF1) (sporadic). Management strategies are often patient specific because of the variable and unpredictable course. No study has summarized the effect of treatment strategies on visual outcomes in the subset of pediatric patients with sporadic OPG. METHODS: We conducted a systematic review to determine the nature of visual outcomes in pediatric patients with sporadic, non-NF1-associated OPG using the PubMed, Embase, Scopus, Cochrane, and CINAHL Plus databases. Visual outcomes were categorized as improved, unchanged, or deteriorated. RESULTS: Of 1316 results, 31 articles were included. Treatment indications are unknown with full clinical detail. A total of 45.2% (14/31) reported deteriorated outcomes after treatment, 35.5% (11/31) no change, and 19.4% (6/31) improvement. Of radiotherapy studies, 50.0% (4/8) found no change, 37.5% (3/8) deterioration, and 12.5% (1/8) improvement. Of chemotherapy studies, 35.7% (5/14) each showed improvement and deterioration, whereas 28.6% (4/14) showed no change. Of surgical studies, 62.5% (5/8) indicated deterioration, and 37/5% (3/8) indicated no change. The singular study examining observation reported deterioration in visual outcomes. Factors associated with poor visual outcomes included signs and symptoms of visual decline at presentation, involvement of the intraorbital optic nerve, and intracranial hypertension requiring surgery. Causality cannot be determined from systematic review. CONCLUSIONS: Most studies showed that vision in pediatric patients with sporadic OPG is stable to poor after observation, chemotherapy, radiotherapy, or surgery. Chemotherapy may be associated with most favorable visual outcomes.

Evaluation of optic nerve diameters in individuals with neurofibromatosis and comparison of normative values in different pediatric age groups.

AIM: Our study aimed to report the normative values for optic nerve diameter in different age groups in MR imaging (MRI) in the pediatric population and to find a cut-off value for diagnosis in different age groups to be used for the diagnosis of optic glioma in patients with Neurofibromatosis 1(NF1). MATERIALS-METHODS: Orbital MRI obtained from 2011 to 2021 for children with and without NF1 were reviewed. Patients were divided into three groups: NF1 with glioma (group 1, n = 38), NF1 without glioma (group 2, n = 57), and healthy controls (group 3, n = 295). Two radiologists assessed diameter and tortuosity using validated criteria. The optic nerve measurements were obtained by two radiologists in two plans (axial and coronal sections) at five locations; retroocular, midsegment, and prechiasmatic segment on axial plane and retroocular segment and chiasmatic on coronal plane. RESULTS: Optic nerves were divided into 4 age groups: 0-2 years, 2-6 years, 6-12 years, and 12-18 years. It was observed that optic nerve diameters increased with age in healthy individuals. In subjects in groups 1 and 2, the mean diameter of the optic nerve was significantly greater at all locations compared with control individuals. Tortuosity scores were significantly associated in NF1 subjects with optic glioma than in NF1 subjects without optic glioma. CONCLUSION: We present the normative values obtained by measuring optic nerve diameters in pediatric populations (0-18 years) on MRI of our center. A rapid increase in optic nerve diameter was observed in the first 6 years of life, followed by a slower increase. Quantitative reference values for optic nerve diameter will benefit the development of objective diagnostic criteria for optic nerve gliomas (ONGs) secondary to NF1.

Orbital indications.

GKNS for uveal melanoma has become a recognized valued treatment which avoids enucleation of the eye. In the early days, the eccentric location of these tumors made treatment difficult, but Gamma Knife Perfexion has solved that problem. It is known that larger tumors and tumors with an anterior location have a worse prognosis. GKNS has also been of rather unexpected benefit in optic nerve gliomas that require treatment. Choroidal hemangiomas may benefit from treatment as can secondary glaucoma. It has also been found to be beneficial in the treatment of thyrotoxic ophthalmopathy.

Imaging of the Primary Visual Pathway based on Visual Deficits.

Vision loss can occur due to a variety of etiologies along the primary visual pathway. Understanding the anatomic organization of the visual pathway, which spans the globe to the occipital cortex, can help tailor neuroimaging to identify the cause of visual dysfunction. In this review, relevant anatomy and optimization of computed tomography and magnetic resonance imaging techniques will be described. This will be followed by a discussion of imaging findings related to pathologies at each functional anatomic level.

Grating visual acuity impairment assessed by sweep visually evoked potentials in children with optic pathway tumors unable to perform optotype acuity tests / Deficiência na acuidade visual de grades avaliada pelos potenciais visuais evocados de varredura em crianças com tumores da via óptica incapazes de informar a acuidade de optotipos

ABSTRACT Purpose: To determine visual impairment due to optic pathway tumors in children unable to perform recognition acuity tests. Methods: Grating visual acuity scores, in logMAR, were obtained by sweep visually evoked potentials (SVEP) in children with optic pathway tumors. The binocular grating visual acuity deficit was calculated by comparison with age-based norms and then assigned to categories of visual impairment as mild (from 0.10 to 0.39 logMAR), moderate (from 0.40 to 0.79 logMAR), or severe (≥0.80 logMAR). Interocular differences were calculated by subtraction and considered increased if >0.10 logMAR. Results: The participants were 25 children (13 boys; mean ± SD age, 35.1 ± 25.9 months; median age, 32.0 months) with optic pathway tumors (24 gliomas and 1 embryonal tumor), mostly located at the hypothalamic-chiasmatic transition (n=21; 84.0%) with visual abnormalities reported by parents (n=17; 68.0%). The mean grating acuity deficit was 0.60 ± 0.36 logMAR (median, 0.56 logMAR). Visual impairment was detected in all cases and was classified as mild in 10 (40.0%), moderate in 8 (32.0%), and severe in 7 (28.0%) children, along with increased interocular differences (>0.1 logMAR) (n=16; 64.0%). The remarkable ophthalmological abnormalities were nystagmus (n=17; 68.0%), optic disc cupping and/or pallor (n=13; 52.0%), strabismus (n=12; 48.0%), and poor visual behavior (n=9; 36.0%). Conclusion: In children with optic pathway tumors who were unable to perform recognition acuity tests, it was possible to quantify visual impairment by sweep-visually evoked potentials and to evaluate interocular differences in acuity. The severity of age-based grating visual acuity deficit and interocular differences was in accordance with ophthalmological abnormalities and neuroimaging results. Grating visual acuity deficit is useful for characterizing visual status in children with optic pathway tumors and for supporting neuro-oncologic management.(AU)

RESUMO Objetivo: Determinar o grau de deficiência visual em crianças com tumores da via óptica incapazes de informar a acuidade visual de reconhecimento. Método: A acuidade visual de grades, em logMAR, foi estimada por potenciais visuais evocados de varredura em crianças com tumores das vias ópticas. O déficit da acuidade visual de grades binocular foi calculado em relação ao valor mediano normativo esperado para a idade e a deficiência visual, classificada como leve (0,10 a 0,39 logMAR), moderada (0,40 a 0,79 logMAR) ou grave (≥0,80 logMAR). Diferenças inter-oculares foram calculadas por subtração e consideradas aumentadas se >0,10 logMAR. Resultados: Foram avaliadas 25 crianças (13 meninos; média de idade ± DP=35,1± 25,9 meses; mediana=32,0 meses) com tumores da via óptica (24 gliomas e 1 tumor embrionário) localizados particularmente na transição hipotalâmico-quiasmática (n=21; 84,0%) e com anormalidades visuais detectadas pelos pais (n=17; 68,0%). A média do déficit da acuidade de grades foi 0,60 ± 0,36 logMAR (mediana=0,56 logMAR). Observou-se deficiência visual leve em 10 (40,0%), moderada em 8 (32,0%) e grave em 7 (28,0%), além de aumento da diferença interocular da acuidade visual (n=16; 64,0%). As principais alterações oftalmológicas encontradas foram: nistagmo (n=17; 68,0%), aumento da escavação do disco óptico e/ou palidez (n=13; 52,0%), estrabismo (n=12; 48,0%) e comportamento visual pobre (n=9; 36,0%). Conclusão: Em crianças com tumor da via óptica e incapazes de responder aos testes de acuidade visual de reconhecimento, foi possível quantificar deficiência visual por meio dos potenciais visuais evocados de varredura e avaliar a diferença interocular da acuidade visual de grades. A gravidade do déficit da acuidade visual de grades relacionado à idade e a diferença interocular da acuidade visual de grades foram congruentes com alterações oftalmológicas e neuroimagem. O déficit da acuidade visual de grades foi útil à caracterização do estado visual em crianças com tumores da via óptica e ao embasamento da assistência neuro-oncológica.(AU)

Optic Nerve Gliomas.

Objectives To describe the diagnostic and management features of optic nerve gliomas. Design Literature review. Results Optic nerve gliomas are generally benign in the pediatric age group although they are usually malignant and aggressive in adults. As such, the mechanisms by which these lesions are diagnosed, the systemic implications, the goals of intervention, and the nature of therapeutic management all differ between these tumors. Conclusions This article addresses these lesions and discusses the diagnostic and therapeutic paradigms by which they may be approached.

Occurrence of Second Primary Malignancies in Patients With Primary Optic Nerve Gliomas: A Surveillance, Epidemiology, and End Results Analysis.

Background/Aim: Advanced understanding of screening and therapeutic modalities acts as provision for increased survival in patients diagnosed with optic nerve gliomas. Secondary primary malignancies (SPMs) in patients diagnosed with primary optic nerve glioma (OPG) are currently an uncharacterized frontier. This US national database analysis highlights the incidences of SPMs in patients diagnosed with primary OPG. Materials and Methods: Standardized incidence ratios (SIR) and excess absolute risk (EAR) for SPMs were calculated using the SEER-specific multiple outcome analysis. 95% SIR confidence intervals were calculated with statistical significance achieved at p<0.05. Results: SPMs originating from soft tissues (including the heart) (SIR=33.23, CI=6.85-97.11; EAR=5.07), breast (SIR=4.99, CI=1.36-12.77; EAR=5.57), female breast (SIR=5.03, CI=1.37-12.89; EAR=5.58), brain (SIR=105.38, CI=65.23-161.08; EAR=36.23), cranial nerves (SIR=103.29, CI=12.51-373.12; EAR=3.45), non-lymphocytic leukemia (SIR=15.05, CI=1.82-54.37; EAR=3.25), myeloid and monocytic leukemia (SIR=16.26, CI=1.97-58.75; EAR=3.27), and Kaposi's sarcoma (SIR=79.88, CI=2.02-445.08; EAR=1.72) demonstrated significantly increased SIR. Overall, the values for cumulative SPM (SIR=6.04, CI=4.33-8.19; EAR=59.60) highlight the overall significance in incidence of SPM in patients diagnosed with OPG. Conclusion: Clinical decision-making should reconcile enhanced propensities for development of SPM.

Optic Nerve Glioma in Neurofibromatosis: Radiological Clues to Diagnosis in a Young Child.

A 13-month-old boy presented with delay in attainment of milestones. On examination, he had café au lait spots and central hypotonia. MRI brain with contrast revealed bilateral bulky enhancing optic nerves with involvement of chiasma. Mid-orbit downward kinking and pseudo-cerebrospinal fluid sign classical of optic nerve glioma (OPG) with neurofibromatosis1 (NF1) could be well appreciated in the MRI. Correct identification of OPG may help in the diagnosis of NF1 in a young child, as other phenotypic features appear later in childhood.

Acute angle plication of optic nerve glioma as a mechanism of rapidly progressive visual loss.

Purpose: The majority of pediatric patients with optic nerve gliomas (ONG) remain asymptomatic; however, a subset of patients suffer rapid, irreversible visual loss. The purpose of this study was to determine anatomic differences of ONG noted either by imaging or by intra-operative observation between patients with gradual visual dysfunction and those with rapid visual loss. Methods: A retrospective review was performed in patients with visual loss secondary to ONG. The clinical records, pathology, and radiographic images were reviewed for all patients. The degree of folding or plication of the optic nerve (ON) was determined by analyzing the most acute angle present in the course of the ON. Outcome measures: The primary outcome measure was the angle of plication or folding of the ON and the relationship of this to the course of visual dysfunction. Results: Six patients with ONG were included in the study. A structural difference in the ON was identified in four patients with rapid vision loss compared with two patients with more gradual visual dysfunction. In patients with rapid progressive visual loss, the ONG had a 90-degree or more acute plication of the ON. Those with more gradual visual loss had more obtuse bends in the ONG. Conclusions: We have identified that the intrinsic structure of the ONG may contribute to the rare but devastatingly rapid progression of visual dysfunction in some patients. Recognizing these changes may guide clinicians to intervene prior to the development of irreversible visual loss.

Malignant optic nerve glioma in a young woman with 7 year follow up without recurrence.

PURPOSE: To report diagnostic and management challenges of a case of WHO Grade III glioma of the optic nerve occurring in an unusually young patient with more than 7 years of survival without recurrence. OBSERVATIONS: An 18-year-old woman reported rapidly progressive vision loss in the right eye in the setting of a right optic nerve lesion, central retinal artery occlusion, central retinal vein occlusion, and neovascularization of the optic disc. An orbital MRI with contrast demonstrated enhancement of the intraocular, intraorbital, and intracanalicular portion of the right optic nerve. Biopsy of a portion of the intraorbital optic nerve was negative, however, biopsy of the intracranial optic nerve confirmed WHO Grade III glioma (anaplastic astrocytoma). Although the tumor was excised, there remained positive margins at the optic chiasm. The patient was then managed with a combination of radiation and temozolomide. Postoperatively, the initial neovascularization of the optic nerve that had resolved, re-emerged with gliosis. In this setting a concern for intraorbital tumor arose and the globe was enucleated, definitively ruling out neoplasm. The patient has remained tumor free seven years after resection. CONCLUSIONS AND IMPORTANCE: Malignant optic pathway glioma is rare and carries a high 5-year mortality rate. Diagnosis can be elusive given orbital MRI with contrast often appears to be non-specific. Inflammatory changes can be confounding such that a biopsy in the respective area will yield a negative pathologic result. Repeat biopsy is recommended if clinical suspicion is high. Combination treatment of optic nerve tumor resection, temozolomide and radiation has been effective in treating this patient who continues to be followed closely and has had no clinical or radiographic evidence of recurrence in over 7 years. The re-emergence of neovascularization with gliosis/fibrosis of the optic nerve, was driven by ischemia and further precipitated by radiation. To our knowledge this patient represents the youngest reported case of malignant optic nerve glioma with the longest reported survival in the literature to date (over seven years).

Acute post-partum vision loss due to pilocytic astrocytoma.

PURPOSE: We report an unusual case of a thirty-three-year-old woman who presented with acute unilateral vision loss following pregnancy and was ultimately discovered to have a pilocytic astrocytoma of the optic nerve. OBSERVATIONS: A thirty-three-year-old previously healthy female presented one month following Caesarean section with unilateral vision loss. She was found to have significantly decreased visual acuity, an afferent pupil deficit, proptosis, optic nerve edema and choroidal folds. Imaging revealed a large lesion of the optic nerve. Biopsy was performed and pathologic analysis revealed a pilocytic astrocytoma, WHO grade 1. The patient opted for close observation without further management and demonstrated mild improvement in visual function. CONCLUSIONS/IMPORTANCE: Optic pathway gliomas (OPG) most commonly present in the pediatric patient population with painless proptosis, slowly progressive vision loss, and clinical findings of chronic optic neuropathy.( Farazdaghi et al., 2019 Sep) 1 Acute presentations of this disease in adulthood are rare. This case demonstrates a rare case of acute optic nerve glioma presentation during the post-partum period.