Longitudinal changes in optic disc cupping from the baseline in chiasmal lesion optic neuropathy and glaucoma.

We aimed to investigate the changes in cupping in chiasmal lesion optic neuropathy (chON) compared to baseline optic disc and glaucoma. We used a novel study design to enroll patients who had fundus photographs incidentally taken during routine health check-ups prior to the onset of optic neuropathy. In 31 eyes (21 patients) with chON and 33 eyes (30 patients) with glaucoma, we investigated the change in cup-to-disc (C/D) area from the baseline to overt cupping using flicker analysis. Compared to the baseline, 23 eyes (74.2%) had increased cup size and 3 (9.7%) had vascular configuration changes in the chONgroup; in contrast, all glaucoma eyes exhibited changes in cup size and vascular configuration. The increase in C/D area ratio was significantly smaller in chON (0.04 ± 0.04) compared to glaucoma (0.10 ± 0.04, P < 0.001); the minimum residual neuroretinal rim width showed a more pronounced difference (29.7 ± 8.2% vs 7.1 ± 3.9%, P < 0.001). The changes distributed predominantly towards the nasal direction in chON, contrasting the changes to the arcuate fibers in glaucoma. In conclusion, our results provide the first longitudinal evidence of true pathological cupping in chONcompared to photographically disease-free baseline. The marked difference in the residual minimum rim width reaffirms the importance of rim obliteration in the differential diagnosis between the two diseases.

Microsurgical Resection of a Parachiasmatic Craniopharyngioma via a Left-Sided Pterional Transsylvian Approach.

Craniopharyngiomas are histologically benign tumors that originate from squamous rests along the pituitary stalk. They make up approximately 1.2% to 4.6% of all intracranial tumors and do not show significant differences in occurrence based on sex. Adamantinomatous craniopharyngiomas have 2 peaks of incidence, commonly observed in patients from ages 5 to 15 years and again from 45 to 60 years. In contrast, papillary craniopharyngiomas mainly affect adults in their fifth and sixth decades of life.1 The "malignancy" of craniopharyngiomas is attributed to their location and the challenges associated with achieving complete removal because they can manifest in the sellar, parachiasmatic, and intraventricular regions or a combination of these.2,3 Various approaches have been used to resect these tumors.4,5 Radical resection offers the most promising option for disease control, potential cure, and the ability to transform the disease from lethal to survivable in children, allowing for a functional adult life.2,3 Meticulous evaluation is crucial to determine the appropriate approach and side, with particular emphasis on closely examining the relationship between the tumor and optic pathways (nerve, chiasm, tract), which are frequently involved. This assessment should also include the tumor's relationship with other crucial structures, such as the hypothalamus and adjacent arteries, to ensure that the strategy is adjusted accordingly to further minimize the risk of postoperative morbidity. Video 1 demonstrates a left-sided pterional transsylvian approach to remove a parachiasmatic craniopharyngioma involving the left optic chiasm and tract.

Prechiasmatic Transection of the Unilateral Dodge Class â Optic Pathway Glioma without Neurofibromatosis Type 1: Technical Description and Clinical Prognosis.

OBJECTIVE: For unilateral Dodge Class Ⅰ optic pathway glioma (OPG-uDCⅠ) without neurofibromatosis type 1, unilateral isolated optic nerve gliomas before the optic chiasm have been confirmed to possibly cause visual deterioration and poor prognosis. For this type of highly selective localized tumor, we explored surgery as the only treatment method. This article retrospectively analyzed and summarized the clinical data of this case series, with the aim of exploring the main technical details and clinical prognosis. METHODS: Included were patients with OPG-uDCⅠ without neurofibromatosis type 1 and experiencing vision loss on the affected side. The fronto-orbital approach was used, which was mainly divided into 3 parts: intraorbital, optic canal, and intracranial. All patients underwent prechiasmatic resection without any adjuvant treatments. The follow-up period was 3 months after surgery, and magnetic resonance imaging and contralateral visual acuity were reviewed annually after surgery. RESULTS: All OPG-uDCⅠ cases were completely removed without any adjuvant treatments, and there was no recurrence during the follow-up period. Pathological results showed that, except for 1 adult patient with pilomyxoid astrocytoma (World Health Organization grade Ⅱ), the others all had pilocytic astrocytoma (World Health Organization grade Ⅰ). Five patients experienced transient ptosis, and all recovered 3 months after surgery. CONCLUSIONS: For OPG-uDCⅠ without neurofibromatosis type 1, radical prechiasmatic resection of the tumor is possible, without the need for postoperative radiotherapy and chemotherapy.

Objective Assessment of Visual Field Defects Caused by Optic Chiasm and Its Posterior Visual Pathway Injury.

OBJECTIVES: To investigate the characteristics and objective assessment method of visual field defects caused by optic chiasm and its posterior visual pathway injury. METHODS: Typical cases of visual field defects caused by injuries to the optic chiasm, optic tracts, optic radiations, and visual cortex were selected. Visual field examinations, visual evoked potential (VEP) and multifocal visual evolved potential (mfVEP) measurements, craniocerebral CT/MRI, and retinal optical coherence tomography (OCT) were performed, respectively, and the aforementioned visual electrophysiological and neuroimaging indicators were analyzed comprehensively. RESULTS: The electrophysiological manifestations of visual field defects caused by optic chiasm injuries were bitemporal hemianopsia mfVEP abnormalities. The visual field defects caused by optic tract, optic radiation, and visual cortex injuries were all manifested homonymous hemianopsia mfVEP abnormalities contralateral to the lesion. Mild relative afferent pupil disorder (RAPD) and characteristic optic nerve atrophy were observed in hemianopsia patients with optic tract injuries, but not in patients with optic radiation or visual cortex injuries. Neuroimaging could provide morphological evidence of damages to the optic chiasm and its posterior visual pathway. CONCLUSIONS: Visual field defects caused by optic chiasm, optic tract, optic radiation, and visual cortex injuries have their respective characteristics. The combined application of mfVEP and static visual field measurements, in combination with neuroimaging, can maximize the assessment of the location and degree of visual pathway damage, providing an effective scheme for the identification of such injuries.

CHIASM-Net: Artificial Intelligence-Based Direct Identification of Chiasmal Abnormalities in Albinism.

Purpose: Albinism is a congenital disorder affecting pigmentation levels, structure, and function of the visual system. The identification of anatomical changes typical for people with albinism (PWA), such as optic chiasm malformations, could become an important component of diagnostics. Here, we tested an application of convolutional neural networks (CNNs) for this purpose. Methods: We established and evaluated a CNN, referred to as CHIASM-Net, for the detection of chiasmal malformations from anatomic magnetic resonance (MR) images of the brain. CHIASM-Net, composed of encoding and classification modules, was developed using MR images of controls (n = 1708) and PWA (n = 32). Evaluation involved 8-fold cross validation involving accuracy, precision, recall, and F1-score metrics and was performed on a subset of controls and PWA samples excluded from the training. In addition to quantitative metrics, we used Explainable AI (XAI) methods that granted insights into factors driving the predictions of CHIASM-Net. Results: The results for the scenario indicated an accuracy of 85 ± 14%, precision of 90 ± 14% and recall of 81 ± 18%. XAI methods revealed that the predictions of CHIASM-Net are driven by optic-chiasm white matter and by the optic tracts. Conclusions: CHIASM-Net was demonstrated to use relevant regions of the optic chiasm for albinism detection from magnetic resonance imaging (MRI) brain anatomies. This indicates the strong potential of CNN-based approaches for visual pathway analysis and ultimately diagnostics.

Chiasmal Compression by the Third Ventricle in Obstructive Hydrocephalus: Case Report and Review of the Literature.

The authors review the phenomenon of third ventricular dilation causing chiasmal compression and vision loss, emphasize the need for further study given continued poor outcomes, and, in a patient case, illustrate the value of obtaining magnetic resonance imaging and nerve and macular optical coherence tomography in a patient with an unclear mechanism of vision loss. [J Pediatr Ophthalmol Strabismus. 2023;60(5):e49-e54.].

Atrophy of the optic chiasm is associated with microvascular diabetic complications in type 1 diabetes.

Introduction: Diabetic neuropathy and diabetic eye disease are well known complications of type 1 diabetes. We hypothesized that chronic hyperglycemia also damages the optic tract, which can be measured using routine magnetic resonance imaging. Our aim was to compare morphological differences in the optic tract between individuals with type 1 diabetes and healthy control subjects. Associations between optic tract atrophy and metabolic measures, cerebrovascular and microvascular diabetic complications were further studied among individuals with type 1 diabetes. Methods: We included 188 subjects with type 1 diabetes and 30 healthy controls, all recruited as part of the Finnish Diabetic Nephropathy Study. All participants underwent a clinical examination, biochemical work-up, and brain magnetic resonance imaging (MRI). Two different raters manually measured the optic tract. Results: The coronal area of the optic chiasm was smaller among those with type 1 diabetes compared to non-diabetic controls (median area 24.7 [21.0-28.5] vs 30.0 [26.7-33.3] mm2, p<0.001). In participants with type 1 diabetes, a smaller chiasmatic area was associated with duration of diabetes, glycated hemoglobin, and body mass index. Diabetic eye disease, kidney disease, neuropathy and the presence of cerebral microbleeds (CMBs) in brain MRI were associated with smaller chiasmatic size (p<0.05 for all). Conclusion: Individuals with type 1 diabetes had smaller optic chiasms than healthy controls, suggesting that diabetic neurodegenerative changes extend to the optic nerve tract. This hypothesis was further supported by the association of smaller chiasm with chronic hyperglycemia, duration of diabetes, diabetic microvascular complications, as well as and CMBs in individuals with type 1 diabetes.

Investigation of Demyelination and Remyelination Processes of the Compressed Optic Chiasm: An Experimental Model.

AIM: To demonstrated demyelination and remyelination of the optic nerve histologically by electron microscopy in an experimental model similar to the compression of pituitary adenomas on the optic chiasm. MATERIAL AND METHODS: The rats were fixed to a stereotaxic device under deep anesthesia, and a balloon catheter was placed under the optic chiasm through a burr hole which was in front of the bregma in accordance with the brain atlas of rats. The animals were divided into five groups (n=8): control, mild compression demyelination, severe compression demyelination, mild compression remyelination, severe compression remyelination. The fine structures of the tissues obtained were evaluated using electron microscopy. RESULTS: We found a significant difference in the severity of degeneration when comparing group 1 with group 5 (p < 0.001); there was no degeneration in group 1 rats and severe degeneration in all of the group 5 rats. Oligodendrocytes were found in all rats in group 1 and none of the rats in no group 2. The nuclei were preserved in the group 1 rats but damaged in all of the group 5 rats. There were no lymphocytes or erythrocytes in group 1 and all positives in group 5. CONCLUSION: This technique, which induced degeneration without causing damage to the optic nerve with toxic or chemical agents, revealed Wallerian degeneration similar to tumoral compression. After compression relief, the optic nerve remyelination process can be better understood, particularly for sellar lesions. In our opinion, this model may guide future experiments to identify protocols to induce and accelerate remyelination.

Portable MRI to assess optic chiasm decompression after endoscopic endonasal resection of sellar and suprasellar lesions.

OBJECTIVE: Low-field portable MRI (pMRI) is a recent technological advancement with potential for broad applications. Compared with conventional MRI, pMRI is less resource-intensive with regard to operational costs and scan time. The application of pMRI in neurosurgical oncology has not been previously described. The goal of this study was to demonstrate the efficacy of pMRI in assessing optic nerve decompression after endoscopic endonasal surgery for sellar and suprasellar pathologies. METHODS: Patients who underwent endoscopic endonasal surgery for sellar and suprasellar lesions at a single institution and for whom pMRI and routine MRI were performed postoperatively were retrospectively reviewed to compare the two imaging systems. To assess the relative resolution of pMRI compared with MRI, the distance from the optic chiasm to the top of the third ventricle was measured, and the measurements were compared between paired equivalent slices on T2-weighted coronal images. The inter- and intrarater correlations were analyzed. RESULTS: Twelve patients were included in this study (10 with pituitary adenomas and 2 with craniopharyngiomas) with varying degrees of optic chiasm compression on preoperative imaging. Measurements were averaged across raters before calculating agreement between pMRI and MRI, which demonstrated significant interrater reliability (intraclass correlation coefficient [ICC] = 0.78, p < 0.01). Agreement between raters within the pMRI measurements was also significantly reliable (ICC = 0.93, p < 0.01). Finally, a linear mixed-effects model was specified to demonstrate that MRI measurement could be predicted using the pMRI measurement with the patient and rater set as random effects (pMRI ß coefficient = 0.80, p < 0.01). CONCLUSIONS: The results of this study suggest that resolution of pMRI is comparable to that of conventional MRI in assessing the optic chiasm position in relation to the third ventricle. Portable MRI sufficiently demonstrates decompression of the optic chiasm after endoscopic endonasal surgery. It can be an alternative strategy in cases in which cost, scan-time considerations, or lack of intraoperative MRI availability may preclude the ability to assess adequate optic nerve decompression after endoscopic endonasal surgery for sellar and suprasellar lesions.

[Opportunities and challenges in the diagnosis and treatment of optic chiasm lesions: a clinical and research perspective].

The optic chiasm is a critical component of the visual pathway, and lesions in the pituitary and sellar regions can cause irreversible damage to a patient's visual function, resulting in a significant decrease in their quality of life. As a result, neuro-ophthalmology evaluation is a crucial part of the multidisciplinary treatment of pituitary diseases. However, due to the significant variation in the anatomical structure of the optic chiasm and the sellar region, as well as the complexity of the injury mechanism, chiasm injury can result in diverse manifestations and severity levels, which can make it difficult to correlate with anatomical parameters. In recent years, research has increasingly focused on the early recognition of optic chiasm compression, the prediction of visual function after intervention, and the long-term neurodegenerative effects, while optical coherence tomography (OCT), electrophysiological examinations, and functional magnetic resonance imaging are currently the most commonly used methods for evaluating sellar region lesions. However, the role of these methods, represented by OCT, in clinical diagnosis and treatment, still lacks high-level clinical evidence support, and the evaluation and prediction of optic chiasm function remain key areas for further study. In addition to compression lesions, lesions such as inflammation, infiltration, and demyelination in the sellar region, caused by systemic multi-system diseases, can also lead to visual function damage and require recognition in clinical practice.

Microphthalmia, aphakia and agenesis of the optic chiasm and tract in a wild fox squirrel (Sciurusniger).

We describe gross and histopathological features of multiple ocular and neuro-ophthalmic abnormalities in a fox squirrel (Sciurus niger). Ophthalmic findings included severe bilateral microphthalmos, with the right eye more affected than the left. Histopathology confirmed severe microphthalmia, aphakia, disorganized retinal tissue and small optic nerves, as well as agenesis of the optic chiasm and optic tract. This combination of neuro-ophthalmic abnormalities has not been previously described in wild animals.

Decussating axons segregate within the anterior core of the primate optic chiasm.

BACKGROUND: The axons of ganglion cells in the nasal retina decussate at the optic chiasm. It is unclear why tumours cause more injury to crossing nasal fibres, thereby giving rise to temporal visual field loss in each eye. To address this issue, the course of fibres through the optic chiasm was examined following injection of a different fluorescent tracer into each eye of a monkey. METHODS: Under general anaesthesia, cholera toxin subunit B-Alexa Fluor 488 was injected into the right eye and cholera toxin subunit B-Alexa Fluor 594 was injected into the left eye of a single normal adult male rhesus monkey. After a week's survival for anterograde transport, serial coronal sections through the primary optic pathway were examined. RESULTS: A zone within the core of the anterior and mid portions of the optic chiasm was comprised entirely of crossing fibres. This zone of decussation was delineated by segregated, interwoven sheets of green (right eye) and red (left eye) fibres. It expanded steadily to fill more of the optic chiasm as fibres coursed posteriorly towards the optic tracts. Eventually, crossed fibres became completely intermingled with uncrossed fibres, so that ocular separation was lost. CONCLUSIONS: A distinct, central compartment located within the anterior two-thirds of the optic chiasm contains only crossing fibres. Sellar tumours focus their compressive force on this portion of the structure, explaining why they so often produce visual field loss in the temporal fields.

Cis-p-tau plays crucial role in lysolecithin-induced demyelination and subsequent axonopathy in mouse optic chiasm.

Multiple sclerosis (MS) is an autoimmune demyelinating disease that leads to axon degeneration as the major cause of everlasting neurological disability. The cis-phosphorylated tau (cis-p-tau) is an isoform of tau phosphorylated on threonine 231 and causes tau fails to bind micro-tubules and promotes assembly. It gains toxic function and forms tangles in the cell which finally leads to cell death. An antibody raised against cis- p-tau (cis mAb) detects this isoform and induces its clearance. Here, we investigated the formation of cis-p-tau in a lysophosphatidylcholine (LPC)-induced prolonged demyelination model as well as the beneficial effects of its clearance using cis mAb. Cis -p-tau was increased in the lesion site, especially in axons and microglia. Behavioral and functional studies were performed using visual cliff test, visual placing test, and visual evoked potential recording. Cis-p-tau clearance resulted in decreased gliosis, protected myelin and reduced axon degeneration. Analysis of behavioral and electrophysiological data showed that clearance of cis-p-tau by cis mAb treatment improved the visual acuity along with the integrity of the optic pathway. Our results highlight the opportunity of using cis mAb as a new therapy for protecting myelin and axons in patients suffering from MS.

Compression of the optic chiasm is associated with reduced photoentrainment of the central biological clock.

Objective: Pituitary tumours that compress the optic chiasm are associated with long-term alterations in sleep-wake rhythm. This may result from damage to intrinsically photosensitive retinal ganglion cells (ipRGCs) projecting from the retina to the hypothalamic suprachiasmatic nucleus via the optic chiasm to ensure photoentrainment (i.e. synchronisation to the 24-h solar cycle through light). To test this hypothesis, we compared the post-illumination pupil response (PIPR), a direct indicator of ipRGC function, between hypopituitarism patients with and without a history of optic chiasm compression. Design: Observational study, comparing two predefined groups. Methods: We studied 49 patients with adequately substituted hypopituitarism: 25 patients with previous optic chiasm compression causing visual disturbances (CC+ group) and 24 patients without (CC- group). The PIPR was assessed by chromatic pupillometry and expressed as the relative change between baseline and post-blue-light stimulus pupil diameter. Objective and subjective sleep parameters were obtained using polysomnography, actigraphy, and questionnaires. Results: Post-blue-light stimulus pupillary constriction was less sustained in CC+ patients compared with CC- patients, resulting in a significantly smaller extended PIPR (mean difference: 8.1%, 95% CI: 2.2-13.9%, P = 0.008, Cohen's d = 0.78). Sleep-wake timing was consistently later in CC+ patients, without differences in sleep duration, efficiency, or other rest-activity rhythm features. Subjective sleep did not differ between groups. Conclusion: Previous optic chiasm compression due to a pituitary tumour in patients with hypopituitarism is associated with an attenuated PIPR and delayed sleep timing. Together, these data suggest that ipRGC function and consequently photoentrainment of the central biological clock is impaired in patients with a history of optic chiasm compression.

Visual recovery following surgical intervention for pituitary apoplexy correlated with preoperative optical coherence tomography.

Pituitary apoplexy is a rare but potentially fatal endocrinological emergency which can be difficult to diagnose as presenting symptoms vary significantly. Optimal management requires early diagnosis and collaboration between ophthalmology, endocrinology and neurosurgical services. We present a case of pituitary apoplexy in a 52-year-old Caucasian female who was referred by her optometrist to Palmerston North Hospital Eye Clinic with a three-week history of sudden onset moderate bifrontal headaches, two weeks of non-specific peripheral visual changes and dense bitemporal inferior quadrantanopia on formal visual field testing. Ocular motility and slit lamp examination were unremarkable and retinal nerve fibre layer (RNFL) was relatively preserved on optical coherence tomography (OCT). MRI demonstrated a haemorrhagic pituitary macroadenoma elevating and compressing the optic chiasm without cavernous extension. Blood tests revealed mild hypothyroidism, hypocortisolism, hypogonadotropism and hyperprolactinaemia. The patient was commenced on hydrocortisone and levothyroxine replacement and proceeded for urgent transsphenoidal tumour resection at Wellington Regional Hospital. Histology revealed a non-functioning macroadenoma. The patient was asymptomatic and visual field tests had normalised three weeks post-operatively. Six weeks post-operatively, thyroid function and cortisol levels were normal and replacement therapies were ceased.

Diagnostic and Prognostic Utility of Optical Coherence Tomography in Patients With Sellar/Suprasellar Lesions with Chiasm Impingement: A Systematic Review/ Meta-Analyses.

BACKGROUND: In this review, we appraised the current literature on the utility of optical coherence tomography (OCT) as a diagnostic and predictive factor for postoperative visual function outcomes in patients with sellar/suprasellar lesions with chiasmal impingement. METHODS: A systematic search of PubMed, CINAHL, Scopus, and Cochrane Library was conducted following PRISMA guidelines. Included studies described diagnostic or prognostic utility of OCT in patients with sellar/suprasellar lesions with chiasmal impingement. Meta-analysis was represented as mean difference (MD) with 95% confidence intervals. Meta-regression was performed to determine predictive factors of visual outcomes. RESULTS: Forty-eight articles were identified for final pooled analysis, representing a total of 2435 patients with compressive sellar/suprasellar lesions and 952 healthy controls. Mean age was 43.3 (11.4) years, with 1494 (48.8%) male and 1566 (51.2%) female patients. Mean retinal nerve fiber layer (RNFL) was significantly different in the study population compared with healthy controls (75.8 µ [13.2] vs. 91.4 µ [10.8], P < 0.00001). The nasal segment of RNFL had the largest mean difference (MD -9.76 [-12.39, -7.13], P < 0.0001). Visual acuity, visual field mean deviation, and visual field pattern standard deviation all showed significant differences between the study population and healthy controls as well (P < 0.0001). Meta-regressions showed significant predictive capability of preoperative RNFL in determining visual function outcome (P < 0.05). CONCLUSIONS: Our findings provide promising support for the growing evidence that OCT parameters can be utilized as both a diagnostic and prognostic tool for patients with compression of the optic apparatus. There is a need for further studies to gain a better understanding of OCTs and to improve patient outcomes.

Junctional Scotoma and Patterns of Visual Field Defects Produced by Lesions Involving the Optic Chiasm.

BACKGROUND: Lesions of the optic chiasm (OC) typically produce bitemporal hemianopia (BTH) on visual field (VF) testing, whereas lesions located at the nasal optic nerve-chiasmal (ON-OC) junction have been proposed to produce junctional scotoma (JXS), a central defect in the ipsilateral eye with temporal field loss in the contralateral eye. In this study, we investigated whether the pattern of VF loss in patients with chiasmal compression predicted the appearance of the causative lesion on neuroimaging and described the clinical presentation of these patients with different types of VF defect. METHODS: Retrospective chart review of patients seen in tertiary neuro-ophthalmology practice over 6 consecutive years with lesions abutting or displacing the OC was performed. Lesion size and location relative to the OC on neuroimaging was determined and correlated with VF defects as well as optical coherence tomography (OCT) of the peripapillary retinal nerve fiber layer and macular ganglion cell complex (GCC). RESULTS: Fifty-three patients were enrolled. VFs demonstrated JXS (n = 18), BTH (n = 14), monocular VF defect (n = 4), and no VF defect (n = 17); 64.7% of cases with normal VFs had radiologic OC compression. Lesion volume was highest in the JXS group, and these patients also had the poorest presenting visual acuity. All patients with JXS showed involvement of the ON-OC junction; however, not all cases showed compression of the OC from the nasal direction (15 of 18), and 17 of 18 also showed compression of one or both prechiasmatic ONs. Compression of the ON-OC junction was also seen in 79% of BTH, 100% of monocular VF defect, and 59% of no VF defect cases. Fifty percent of patients with normal VFs already had thinning of the GCC on OCT. GCC thinning was most pronounced nasally in the BTH group, but diffuse bilateral thinning was found in 38% of cases compared with 60% of JXS. VFs improved in 6 of 6 patients with BTH but only in 5 of 8 JXS cases after treatment. CONCLUSIONS: JXS is more often seen with larger lesions and when there is compression of both the prechiasmatic ON and ON-OC junction. These patients have worse presenting visual acuity and poorer outcomes. Not all patients with radiologic compression had VF defects, although 50% of patients with normal VFs had evidence of compression on the macular GCC analysis, emphasizing the importance of macular OCT in the evaluation of patients with lesions involving the OC.

Cavernous Malformation of the Optic Nerve and Chiasm: Prompt Suspicion and Surgery Matter.

BACKGROUND: Cavernous malformations (CMs) of the optic nerve and chiasm are extremely rare, accounting for less than 1% of all intracranial CMs. Acute, subacute, or progressive visual loss from CM may occur with or without hemorrhage. Prompt surgical excision of the CM offers the best hope to improve or stabilize vision. Given its rarity, optic nerve and chiasm CMs may not be readily suspected. We provide 3 cases of optic nerve and chiasm CM, highlighting key neuroimaging features and the importance of expedited intervention. METHODS: Case records of the neuro-ophthalmology clinics of the Bascom Palmer Eye Institute and the University of Colorado, and literature review of reported cases of optic CM. RESULTS: A 49-year-old woman reported acute progressive painless vision loss in the right eye. MRI showed a suprasellar mass with heterogeneity in signal involving the right prechiasmatic optic nerve. Surgical excision of the CM 5 days after onset of visual loss improved vision from 20/300 to 20/30. A 29-year-old woman with acute painless blurred vision in the right eye had anterior chiasmal junctional visual field defects corresponding to a heterogeneously minimally enhancing mass with blood products enlarging the optic chiasm and proximal right optic nerve. Surgical excision of the CM 8 weeks after onset of visual loss improved vision from 20/40 to 20/15 with improved visual fields. A 33-year-old woman with a history of familial multiple CMs, diagnosed at age 18, reported new-onset severe headache followed by blurred vision. MRI showed a hemorrhagic lesion of the optic chiasm and right optic tract. She was 20/20 in each eye with a reported left superior homonymous hemianopia. No intervention was recommended. Vision of the right eye worsened to 20/400 2 months later. The patient was followed over 13 years, and the MRI and visual function remained unchanged. Literature review yielded 87 optic CM cases occurring across gender and nearly all ages with visual loss and headache as the most common presenting symptoms. Optic chiasm is the most common site of involvement (79%). Nearly 95% of reported CM cases were treated with surgery with 81% with improved vision and 1% with worsened vision. CONCLUSION: MRI features are critical to the diagnosis of optic nerve and chiasm CM and may mimic other lesions. A high index of suspicion by the neuro-ophthalmologist and neuroradiologist leads to early recognition and intervention. Given optic CM displaces and does not infiltrate neural tissue, expedited surgical resection by a neurosurgeon after consideration of other diagnostic possibilities improves visual function in most cases.

Necrosis of the optic nerve and chiasm with safe radiation doses: Report of two rare cases.

INTRODUCTION: Radiation-induced optic neuropathy (RION) is still a devastating complication of brain and skull base radiation that has no effective treatment up until today, thus uttermost caution must be taken in treating patients that brain radiotherapy is needed. We present two cases of RION that happened in seemingly safe radiation doses. CASE DESCRIPTION: A 48-year-old female with a history of pleomorphic pituitary adenoma developed bilateral and painless loss of vision 10 months after radiation to the brain; the total radiation dose was 45 Gy in 25 fractions and no other risk factors of RION were found. Magnetic resonance imaging of the brain depicted bilateral prechiasmatic optic nerve enhancement with involvement of the optic chiasm. Treatment with high doses of corticosteroids was unsuccessful. A 62-year-old female with a history of lung adenocarcinoma and brain metastases presented with a 1-month history of decreased vision in both eyes. He had undergone whole-brain radiotherapy with a total dose of 30 Gy over 10 fractions and concurrent chemotherapy with cisplatin and pemetrexed. Brain magnetic resonance imaging (MRI) with contrast showed bilateral intracranial optic nerve enhancement. CONCLUSIONS: This is the second case report of RION in a patient with a history of brain radiotherapy and concurrent chemotherapy with pemetrexed. History of chiasmal compression, concurrent use of chemotherapeutic agents, and high fraction size (despite the safety of total radiation dose) were possible contributing risk factors to develop RION in our cases. Hence, adjusting the radiation dose according to the presence of these risk factors is recommended.

Molecular Profile of a Pituitary Rhabdomyosarcoma Arising From a Pituitary Macroadenoma: A Case Report.

Pituitary sarcoma arising in association with pituitary adenoma is an uncommon finding. Most cases of secondary sarcoma have been noted to arise with a median interval of 10.5 years post radiation. In this case report, we describe a 77-year-old man with an incidental discovery of a pituitary macroadenoma on magnetic resonance imaging (MRI) and underwent radiotherapy. Three years after radiation treatment, there was an acute change in clinical symptoms and increase in tumor size and mass effect on the optic chiasm which prompted surgical resection. A pituitary adenoma along with a separate spindle-cell sarcomatous component was identified in histology. Immunohistochemical stain for muscle markers confirmed a development of pituitary rhabdomyosarcoma (RMS). Molecular profiling of the tumor identified mutations in TP53, ATRX, LZTR1, and NF1. Despite its rarity, characterization of pituitary RMS with immunohistochemistry and molecular studies may provide an insight to its pathophysiological relationship with pituitary adenoma.