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To report a case of a pituitary macroadenoma that presented with bilateral intraretinal hyporeflective spaces located primarily in the inner nuclear layer on the macular SD-optical coherence tomography.Case report.Based on the findings of a macular SD-optical coherence tomography, a 53-year-old man was considered as having cystoid macular edema secondary to a retinovascular condition. He was treated with intravitreal vascular endothelial growth factor inhibitors and corticosteroids with no improvement. Confrontation visual fields suggested a bitemporal defect. MRI confirmed the presence of a nonsecreting pituitary macroadenoma compressing the optic chiasm.Not all intraretinal hyporeflective spaces in the optical coherence tomography are secondary to a retinal vascular condition. Hyporeflective spaces in the inner nuclear layer on optical coherence tomography imaging that do not leak on fluorescein angiography may be secondary to optic atrophy from chiasmal compression.

(1) Background: The normal A1 segment of the anterior cerebral artery (ACA) has a supraoptic course. The proximal infraoptic course of an A1 segment leaving the internal carotid artery (ICA) near the origin of the ophthalmic artery is a rare possibility. This study aimed to determine the prevalence and detailed anatomy of infraoptic A1 segments. (2) Methods: We retrospectively studied 145 computed tomography angiograms from 92 male and 53 female cases, with ages varying from 61 to 78 y.o. (3) Results: In 21/145 cases, infraoptic or infrachiasmatic horizontal-medial courses of A1 segments that emerged distally from the ICA were found. Distal infraoptic A1 segments were bilateral in 16/145 cases and unilateral in 3/145 cases. Infrachiasmatic A1 segments were found bilaterally in 2/145 male cases. All the infraoptic/infrachiasmatic A1 segments left long ICAs with low bifurcations. In 7/34 sides with distal infraoptic or infrachiasmatic A1 segments, supracarotid courses were present. In one female, the right A1 segment had an anterior supraclinoid, supracarotid and infraoptic course. In two female cases with a bilateral distal infraoptic A1, the segment was almost contacting the respective posterior cerebral artery. (4) Conclusions: In cases with dolicho(ectatic) ICAs, the A1 segments could have infraoptic and supracarotid courses the neurosurgeons should be aware of.

Primary lymphoma of the visual pathway is rare, especially at the chiasm. Very few cases have been reported. The lesion is frequently confused with an optic–hypothalamic glioma. A 55-year-old man was found disoriented at his home by a friend and evaluated with a brain MRI which demonstrated an expansile mass located at the optic chiasm and hypothalamus level. The principal differential was a high-grade hypothalamic glioma due to the contrast enhancement. A biopsy of the chiasmal lesion was performed. Histological diagnosis of the lesion was compatible with a diffuse large B cell lymphoma. He was started on methotrexate and rituximab; however, his clinical course kept deteriorating, and he died 64 days after his presentation. All prior cases of primary lymphoma of the chiasm are reviewed.

An 84-year-old man presented with a frontal headache and easy bruising. He had a background history of a pituitary macroadenoma, diagnosed incidentally a year earlier. Investigations showed haemorrhage into the pituitary macroadenoma leading to a diagnosis of pituitary apoplexy in the context of low platelet count secondary to immune thrombocytopaenia. He was treated with intravenous hydrocortisone, platelet transfusion, intravenous immunoglobulin and high-dose steroid. Neurosurgical intervention was not indicated initially. Five days into his admission, he developed bilateral ptosis and ophthalmoplegia. MRI confirmed further haemorrhage associated with compression of the optic chiasm. He was transferred to a tertiary neurosurgical centre where he underwent urgent surgical decompression. To date, there has been minor improvement in his neurological symptoms. Management of this patient required considerable multidisciplinary teamwork between the clinics of endocrinology, haematology, neurosurgery, ophthalmology and geriatrics.

Context Characterization of the clinical features and natural history of nonfunctioning pituitary microadenomas (NFPmAs) is limited by heterogeneous and small-scale studies. Objective To characterize the clinical presentation and natural history of NFPmAs and evaluate if imaging follow-up interval can be extended. Methods Retrospective single-center cohort study (years 2006-2021) of conservatively managed patients with NFPmAs. Initial symptoms, pituitary function, and tumor size were assessed. A change in NFPmA size ≥2 mm, as determined by pituitary or brain magnetic resonance imaging (MRI), was considered significant. Results There were 347 patients in the study cohort. Headache (78.4%) and fatigue (70.0%) were commonly reported despite no evidence of mass effect or significant pituitary hypofunction. Pituitary deficiencies at baseline were rare, with hypogonadism being most common (5.1%). During a median imaging follow-up period of 29 months (range 3-154), 8.1% of NFPmAs grew. Growth incidence was 2.1 per 100 person-years with a mean and median time to growth of 38.1 (SD ± 36.4) and 24.5 (interquartile range 12.0-70.8) months, respectively. Tumor growth was mild and not associated with new pituitary deficiencies or visual deficits. Conclusion These data indicate that the natural history of NFPmAs is overall benign. Consequently, we propose that the initial MRI follow-up timeline for NFPmAs can be extended up to 3 years unless a lesion is close to the optic chiasm, there are worrisome mass effect symptoms, or new pituitary deficiencies.

Objective Secondary adrenal insufficiency (sAI) is a severe endocrinologic complication associated with nonfunctioning pituitary adenoma (NFPA). However, its prevalence is not simply related to tumor size. In this study, we aimed to detect the clinical characteristics of NFPAs that cause sAI. Methods We retrospectively investigated the clinical data of 218 patients with newly diagnosed macro NFPA between April 2011 and March 2020. The patients for whom endocrinologists had prescribed hydrocortisone after comprehensive endocrinologic evaluation were defined as having sAI. The 7 clinical factors analyzed for association with sAI were age, sex, presence of neurologic symptoms, hospitalization for emergency management of pituitary apoplexy, degree of optic chiasm compression, and Knosp grades on both sides. Results Seventy-three patients (33%) were classified into the sAI group. Multinomial logistic regression showed the strongest correlation between sAI and Knosp grade on the less extending side (P = 0.0001), followed by sex (male) (P = 0.0013) and pituitary apoplexy (P = 0.098). Tumors that extended bilaterally and had Knosp grades of 1–3 were frequently observed in sAI and were common in males. Conclusions The NFPAs that occupy the sella space and compress the walls on both sides of the cavernous sinus, but do not penetrate them, have a higher risk of developing sAI. This type is more common in males and is seen even in patients without visual field disturbances. This clinical finding will be beneficial in management of patients with NFPA.

Objectives COVID-19 is a multisystemic disease. Ophthalmological abnormalities are relatively rare among COVID-19-infected patients. The aim of our study was to report orbital and visual pathways MRI findings in a nationwide multicenter cohort of patients with severe COVID-19. Methods This IRB-approved retrospective multi-center study included participants presenting with severe COVID-19, who underwent brain MRI from March 4th to May 1st 2020. Two neuroradiologists (“blinded”), blinded to all data, individually analyzed morphological MRIs focusing on the orbits and the visual pathways. A second consensus reading session was performed in the case of disagreement between both readers. Clinical and ophthalmological data were compared to MRI findings. Descriptive statistical analysis and interobserver agreement for MRI reading using non-weighted Cohen kappa statistics were performed. Results 129 participants (43 [33%] women and 86 [67%] men, mean age 63 ± 14 years) were included in the study. 17/129 (13%) patients had abnormal MRI findings of the orbit or visual pathways. 11/17 (65%) patients had a FLAIR-WI hyperintense optic disc. 6/17 (35%) patients had abnormal signal of at least one of the visual pathway structures: 6/6 (100%) of the optic nerve, 1/6 (17%) of the optic chiasm, 2/6 (33%) of the optic tract and 1/6 (17%) of the optic radiations. Conclusions Our study showed that a substantial number of patients with severe COVID-19 presented with abnormal MRI findings of the orbit or visual pathways, which might lead to potentially severe visual impairment.

The retinogeniculate visual pathway (RGVP) conveys visual information from the retina to the lateral geniculate nucleus. The RGVP has four subdivisions, including two decussating and two nondecussating pathways that cannot be identified on conventional structural magnetic resonance imaging (MRI). Diffusion MRI tractography has the potential to trace these subdivisions and is increasingly used to study the RGVP. However, it is not yet known which fiber tracking strategy is most suitable for RGVP reconstruction. In this study, four tractography methods are compared, including constrained spherical deconvolution (CSD) based probabilistic (iFOD1) and deterministic (SD‐Stream) methods, and multi‐fiber (UKF‐2T) and single‐fiber (UKF‐1T) unscented Kalman filter (UKF) methods. Experiments use diffusion MRI data from 57 subjects in the Human Connectome Project. The RGVP is identified using regions of interest created by two clinical experts. Quantitative anatomical measurements and expert anatomical judgment are used to assess the advantages and limitations of the four tractography methods. Overall, we conclude that UKF‐2T and iFOD1 produce the best RGVP reconstruction results. The iFOD1 method can better quantitatively estimate the percentage of decussating fibers, while the UKF‐2T method produces reconstructed RGVPs that are judged to better correspond to the known anatomy and have the highest spatial overlap across subjects. Overall, we find that it is challenging for current tractography methods to both accurately track RGVP fibers that correspond to known anatomy and produce an approximately correct percentage of decussating fibers. We suggest that future algorithm development for RGVP tractography should take consideration of both of these two points., In this work, we investigate the performance of four tractography methods (SD‐Stream, iFOD1, UKF‐1T, and UKF‐2T) for reconstruction of the complete retinogeniculate visual pathway (RGVP). Anatomical measurement and expert rating assessments shows that the iFOD1 method can better quantitatively estimate the percentage of decussating fibers, while the UKF‐2T method produces reconstructed RGVPs that are judged to better correspond to the known anatomy. However, it is challenging for current tractography methods to both accurately track RGVP fibers that correspond to known anatomy and produce an approximately correct percentage of decussating fibers.

Significant restoration of visual function can occur following pituitary tumor resection, although the time course of visual recovery remains poorly understood. This single-centre, two-year, prospective cohort study investigated the temporal patterns of visual recovery in consecutive patients undergoing pituitary tumor resection, between 2009 and 2018. Eyes were stratified based on pre-operative optical coherence tomography (OCT) retinal nerve fibre layer (RNFL) thickness measurements, with thin RNFL being defined as those within the fifth-percentile of age-matched normative values, and normal RNFL as those above the fifth-percentile. Visual function and OCT parameters were assessed pre-operatively, and at 6 weeks, 6 months, and 2 years post-operatively. 456 eyes of 228 patients (mean ± SD age, 53 ± 15 years) were included, of which 114 (25%) eyes had thin RNFL pre-operatively. Visual field recovery was observed in both groups during the first 6 weeks post-operatively (all Q ≤ 0.02), although improvements in visual field parameters between 6 weeks to 6 months were limited to eyes with thin RNFL (both Q 0.05). No further improvements in visual function were detected beyond 6 months in both groups (both Q 0.50). Similar trends were observed in linear regression analysis according to baseline visual function in both groups. In summary, eyes with normal RNFL thickness at baseline experienced most of their recovery within the first six weeks following surgery, while eyes with thin RNFL exhibited gradual improvements during the first six months. These findings have important implications when providing patient counselling and prognostication in the pre-operative setting.

The efficacy of stereotactic radiotherapy (SRT) has been well established for postoperative residual and recurrent nonfunctioning pituitary adenomas (NFPAs). However, the risk of visual impairment due to SRT for lesions adjacent to the optic pathways remains a topic of debate. Herein, we evaluated the long-term clinical outcomes of hypofractionated stereotactic radiotherapy (HFSRT) for perioptic NFPAs. From December 2002 to November 2015, 32 patients (18 males and 14 females; median age 63 years; range, 36–83 years) with residual or recurrent NFPAs abutting or displacing the optic nerve and/or chiasm (ONC) were treated with HFSRT. The median marginal dose was 31.3 Gy (range, 17.2–39.6) in 8 fractions (range, 6–15). Magnetic resonance imaging (MRI) and visual and hormonal examinations were performed before and after HFSRT. The median follow-up period was 99.5 months (range, 9–191). According to MRI findings at the last follow-up, the tumor size had decreased in 28 (88%) of 32 patients, was unchanged in 3 (9%), and had increased in 1 (3%). The successful tumor size control rate was 97%. Visual functions remained unchanged in 19 (60%) out of 32 patients, improved in 11 (34%), and deteriorated in 2 (6%). Two patients had deteriorated visual functions; no complications occurred because of the HFSRT. One patient developed hypopituitarism that required hormone replacement therapy. The result of this long-term follow-up study suggests that HFSRT is safe and effective for the treatment of NFPAs occurring adjacent to the ONC.

To evaluate the ophthalmic features of Rathke's cleft cyst (RCC) and its association with radiological characteristics.In this retrospective single-center study, patients who showed typical findings suggestive of RCC on magnetic resonance imaging (MRI) and underwent relevant ophthalmic examination were recruited retrospectively. Patients were stratified into two groups according to the presence or absence of ophthalmic symptoms related to RCC. We reviewed patients' demographic information, initial symptoms, endocrinological status, ophthalmic features, and characteristics of MRI. Height, size and location of RCC, as well as the optic chiasm displacement assessed from MRI.Thirty-three patients (20 women and 13 men) were included in this study from among 335 patients with RCC on MRI. Fifteen patients had ophthalmic manifestation related to the cyst (Ophthalmic group), whereas 18 patients were not (Non-ophthalmic group). Headache was the most common initial symptom (15 patients, 45.5%), followed by visual disturbance (7, 21.2%), diplopia (1, 3.0%), retro-orbital pain (1, 3.0%), galactorrhea (1, 3.0%), and peripheral extremity discomfort (1, 3.0%). In seven asymptomatic patients (21.2%), the lesion was an incidental finding during a regular medical examination. Ophthalmic manifestation included visual field defect (14 patients, 93.3%) and diplopia (1 patient, 6.7%). The height, volume, and the coronal and sagittal displacements were larger in the ophthalmic group (Appropriate ophthalmic examinations are warranted in patients with RCC, and treatment should be actively considered in patients with ophthalmic manifestations.

Objectives Prolactinoma is the most common cause of pituitary tumours. Current medical guidelines recommend dopamine agonists (cabergoline or bromocriptine) as the initial therapy for prolactinoma. However, surgical removal can also be considered in selected cases, such as patients with macroadenomas with local complications (bleeding or optic chiasm pressure) or those not responding to medical treatment. Methods The present retrospective study included patients with prolactinomas (n=43; female, 24; male, 19) who were primarily managed with medical (n=32) or surgical (n=11) treatment. Results Macroadenoma (n=29.67%) was commonly detected in both genders (female, 54%; male, 84%). Moreover, the mean pre-treatment prolactin levels were similar in both genders (female, 683.3 ± 1347 ng/mL; male, 685.4 ± 805 ng/mL; p=0.226). Surgically treated patients had a greater reduction in tumour size (27.7 ± 17.9 mm pre-treatment vs. 8.72 ± 14.2 mm post-treatment) than non-surgically treated ones (12.5 ± 7.5 mm pre-treatment vs. 4.1 ± 4.2 mm post-treatment; p=0.00). However, the decrease in prolactin levels was similar between the two patient groups (p=0.108). During the follow-up period (10.6 ± 7.0 years), the average cabergoline dose of the patients was 1.42 ± 1.47 mcg/week. Conclusions Although a surgical approach was considered for selected cases of prolactinoma, the average dose used for medical treatment was highly inadequate for the patients in the present study.

A 12-year-old boy being followed up for amblyopia presented to our hospital with visual disturbance in the left eye. The patient's best corrected visual acuity on Snellen chart was 1.0 in the right eye and 0.3 in the left eye. Increased horizontal cup-to-disc ratio was detected on dilated fundus examination. Retinal nerve fiber layer measurement showed diffuse nerve fiber loss and visual field test showed bitemporal hemianopsia. Magnetic resonance imaging revealed a lesion that filled and widened the sella and suprasellar cistern and compressed the optic chiasm. The patient was operated with transcranial approach. The pathologic examination revealed craniopharyngioma.

Our recent report demonstrated that hesperetin (Hst) as a citrus flavonoid, significantly reduces the levels of demyelination in optic chiasm of rats. Previous evidence also indicated that nano-hesperetin (nano-Hst) possesses beneficial impacts in experimental models of Alzheimer's disease and autism. In this study, the effects of nano-Hst on latency of visual signals, demyelination levels, glial activation, and expression of Olig2 and MBP were evaluated in lysolecithin (LPC)-induced demyelination model. Focal demyelination was induced by injection of LPC (1%, 2 μL) into the rat optic chiasm. Animals received oral administration of nano-Hst at dose of 20 mg/kg for 14 or 21 days post LPC injection. Visual evoked potential (VEP) recording showed that nano-Hst reduces the latency of visual signals and ameliorates the extent of demyelination areas and glial activation. Expression levels of the Olig2 and MBP were also significantly increased in nano-Hst treated rats. Overall, our data suggest that nano-Hst reduces the latency of visual signals through its protective effects on myelin sheath, amelioration of glial activation, and enhancement of endogenous remyelination.

Wilbrand's knee of the optic chiasm refers to crossing fibers from one optic nerve that stray for a short distance into the opposite optic nerve before joining the optic tract. This loop of aberrant axons, although small, has generated much controversy. In a previous study, labeling of the optic pathway in normal monkeys with a radioactive tracer revealed no Wilbrand's knee. Monocular enucleation induced a typical knee to form. These findings suggested that Wilbrand's knee is absent normally, but appears after atrophy of one optic nerve. This conclusion has been challenged by images showing Wilbrand's knee in the normal human chiasm using anisotropic light scattering. It has also been resisted by some clinicians who believe that Wilbrand's knee is necessary to explain the anterior chiasmal syndrome. Early in his distinguished career, William F. Hoyt examined the fiber organization of the monkey optic nerve and chiasm. He found no evidence for Wilbrand's knee and rejected its importance for the topical diagnosis of chiasmal lesions. His conclusion is supported by new data showing that anisotropic light scattering is not a reliable method for tracing axons. Hence, that method has given a misleading impression that Wilbrand's knee exists in normal subjects. Although Wilbrand's knee has fascinated neuro-ophthalmologists for more than a century, it is an inconsequential structure that develops only after a longstanding monocular optic neuropathy.

Aim. The aim of the study was to determine the frequency of pathologies which can mimic normal-tension glaucoma (NTG), observed in neuroimaging of NTG patients, and to evaluate the frequency of pathologies in determined additional indications for neuroimaging. Material and Methods. The studied group consisted of 126 NTG patients who met at least one of the following criteria: unilateral NTG, damage in the visual field (VF) inconsistent with optic disc appearance, fast VF progression, worsening of visual acuity, predominant optic disc pallor rather than optic disc excavation, diagnosis under the age of 50, and scotoma in VF restricted by a vertical line. The patients included in the research underwent MRI scans of the brain and both orbits. Results. After neuroimaging, the results of 29 (23%) patients were qualified as positive; 18 (14.2%) of the identified pathologies were found to clinically affect the visual pathway. The most frequent brain pathology was intracranial meningiomas, observed in 4 patients (3.1%), followed by optic nerve sheath meningiomas diagnosed in 3 cases (2.4%), and brain glioma in 1 patient (0.8%). Pituitary gland adenomas were described in 6 patients (4.5%); 3 of the tumours were in contact with the optic chiasm. 53 (40%) patients had minimal ischemic changes in different regions of the brain. In the case of worsening BCVA or fast VF progression, the frequency of positive results was the highest (50% and 40%), whereas in the case of diagnosis at a young age and unilateral involvement, neuropathology was the rarest (0% and 6.9%). Conclusions. In the case of NTG, the decision to perform neuroimaging should be made after a detailed assessment of clinical status, rather in the event of finding the signs of possible compressive optic neuropathy than as an obligatory procedure for every patient.

Silicone oil insertion is a common modality in vitreoretinal surgeries. The purpose of this study is to conduct a systematic review to summarize the uncommon complication of extraocular silicone oil migration.Following the PRISMA guidelines, a systematic review of the literature was performed on January 11, 2020, using PubMed and EMBASE with the following terms: "silicone oil," "eye," and "migrat*."A total of 69 patients-68 patients from 59 articles and one case from our institution-were included in the final analysis. The median age was 54 years (range, 9-92) and 40 patients (57.9%) were men. Orbital migration was reported in 34 patients, and retrolaminar migration (including optic nerve, optic chiasm, suprasellar, subarachnoid space, intraventricular spaces) was reported in 35 patients. Orbital migration group had more aphakics (p = 0.007), implanted glaucoma drainage device (p = 0.005), scleral buckle (p = 0.000), history of trauma-related indications for pars plana vitrectomy (p = 0.000), shorter silicone oil endotamponade time (p = 0.008), more symptomatic (p = 0.000), and requiring surgical intervention (p = 0.000). Retrolaminar migration group had older patients (p = 0.016) and more diabetics (p = 0.041).Systematic review sheds light on plausible risk factors on site of silicone oil migration. Majority of orbital cases are symptomatic and require intervention while retrolaminar cases are incidental and can be managed conservatively. Awareness of this complication can help guide clinicians predict which patients would likely need surgical intervention. Graphical abstract.

Purpose: The aim of the present study is to assess the predictive value of the suprasellar volume (SSV) of nonfunctioning pituitary adenomas (NFPAs) for visual field (VF) impairment in order to guide clinical decision-making and improve neurosurgical management. Methods: Two independent samples of patients with NFPAs (exploratory population N = 50, testing population N = 98) were included in the present study. In the first phase, we determined the optimal cut-off value of the SSV correlating with VF deficits in the exploratory population. In the second phase, we then studied the accuracy of identified cut-off in predicting a VF deficit in the testing population. Results: In the exploratory population, the optimal cut-off value of the SSV to determine the presence of a VF deficit was 1.5mL. Sensitivity and specificity of the cut-off were 81.3 and 100%, respectively. The positive predictive value (PPV) and the negative predictive value (NPV) were 100 and 75%, respectively. When we checked the identified cut-off score on the testing population, we found a sensitivity of 71% and a specificity of 100%. The PPV and NPV were 100 and 59.2%, respectively. In six cases with VF defects and SSV inferior to 1.5mL, the displacement of optic chiasm was in superior position. Conclusion: The SSV may represent an accurate method in routinely clinical practice for predicting VF deficit in patients affected by NFPA.

Neuroinflammation, glial activation, and oxidative injury are the main pathological mechanisms of demyelination in multiple sclerosis (MS). Arbutin, a natural polyphenol compound, possesses antioxidant, anti-inflammatory, and neuroprotective properties whose therapeutic potential has not been studied in the experimental animal models of MS. In the present study, the efficiency of arbutin on lysolecthin (LPC)-induced local demyelination model was investigated. Demyelination was induced by micro-injection of 2 μl LPC (1%) into the rat optic chiasm and the treated group received daily injection of arbutin (50 mg/kg, i.p) during 2 weeks. Visual-evoked potential (VEP) recordings were used to functionally assess the visual pathway. Gene expression analysis was done to evaluate the arbutin effect on the inflammatory, stress oxidative-related mediators, and myelin markers. The myelin-specific staining was performed to assess demyelination and GFAP staining as an astrocyte marker. We found that arbutin significantly reduced P1-latency of VEPs waves and demyelination at 7 and 14 days post-demyelination. Arbutin decreased inflammatory cytokines (IL-1B, IL-17, TNF-α) and iNOS mRNA expression level. In addition, the expression level of anti-inflammatory cytokine (IL-10) and antioxidant mediators (Nrf-2 and HO-1) was enhanced by arbutin treatment. Arbutin increased MBP and Olig2 expression levels in demyelination context. Finally, arbutin attenuated GFAP as an astrocyte marker. Finally, this study demonstrates that arbutin improves functional recovery and myelin repair in the demyelinated optic chiasm through attenuation of inflammation, astrocyte activation, and oxidative stress. These findings might open new promising avenues for treating demyelinating disorders such as multiple sclerosis. Graphical abstract.

Objectives We aimed to evaluate optic chiasm (OC) measures as potential imaging marker for anterior optic pathway damage assessment in the context of neuromyelitis optica spectrum disorders (NMOSD). Materials and method This cross-sectional study included 39 patients exclusively with aquaporin 4-IgG seropositive NMOSD of which 25 patients had a history of optic neuritis (NMOSD-ON) and 37 age- and sex-matched healthy controls (HC). OC heights, width, and area were measured using standard 3D T1-weighted MRI. Sensitivity of these measures to detect neurodegeneration in the anterior optic pathway was assessed in receiver operating characteristics analyses. Correlation coefficients were used to assess associations with structural measures of the anterior optic pathway (optic nerve dimensions, retinal ganglion cell loss) and clinical measures (visual function and disease duration). Results OC heights and area were significantly smaller in NMOSD-ON compared to HC (NMOSD-ON vs. HC p smaller than 22.5 mm2 yielded a sensitivity of 0.92 and a specificity of 0.92 in separating chiasms of NMOSD-ON from HC. OC area correlated well with structural and clinical measures in NMOSD-ON: optic nerve diameter (r = 0.4, p = 0.047), peripapillary retinal nerve fiber layer thickness (r = 0.59, p = 0.003), global visual acuity (r = − 0.57, p = 0.013), and diseases duration (r = − 0.5, p = 0.012). Conclusion Our results suggest that OC measures are promising and easily accessible imaging markers for the assessment of anterior optic pathway damage. Key Points • Optic chiasm dimensions were smaller in neuromyelitis optica spectrum disorder patients compared to healthy controls. • Optic chiasm dimensions are associated with retinal measures and visual dysfunction. • The optic chiasm might be used as an easily accessible imaging marker of neurodegeneration in the anterior optic pathway with potential functional relevance.

We have quantitatively evaluated the macular and peripapillary microvascular changes in eyes with chiasmal compression caused by brain tumors compared with healthy control eyes using optical coherence tomography angiography (OCT-A) and correlated them with other ocular parameters. This cross-sectional study involved the analysis of 36 eyes of 36 patients with chiasmal compression and age and refractive error-matched 35 healthy control eyes. OCT-A was used to generate microvascular images of the superficial and deep retinal capillary plexus (SRCP, DRCP) and the radial peripapillary capillary (RPC) segment in the macula and peripapillary areas. Automated segmentation and vessel density measurements facilitated the analysis of each layer. Macular OCT-A analysis revealed a significant reduction in vessel density in the SRCP (P = 0.004) of the nasal quadrant (P P = 0.019) in the eyes with chiasmal compression compared with the control eyes. The RPC segment vessel density has also been significantly reduced in the eyes with chiasmal compression (P

The effect of metals on the functioning of the human eye is multifactorial and includes enzyme activity modulation, trace metal metabolic pathways changes, and cytotoxic activity. Functional dysfunctions appear mostly as a result of the accumulation of toxic xenobiotic metals or disturbances of micronutrients’ homeostasis. So far, the affinity of selected metals to eye tissues, i.e., the cornea, choroid, lens, and anterior chamber fluid, has been most studied. However, it is known that many eye symptoms are related to damage to the optic nerve. In order to fill this gap, the aim of the study is to perform a multi-element analysis of tissue collected postmortem from optic chiasm and optic nerves. A total of 178 samples from 107 subjects were tested. The concentrations of 51 elements were quantified by inductively coupled plasma mass spectrometry (ICP-MS) after the wet-mineralization step. In terms of elemental composition, the optic chiasm is dominated by two trace elements, i.e., iron (Fe) and zinc (Zn), besides macro-elements Ca, K, Na, P, and Mg. The subjects formed a homogeneous cluster (over 70% subjects) with the highest accumulation of aluminum (Al). The remaining two departing clusters were characterized by an increased content of most of the elements, including toxic elements such as bismuth (Bi), uranium (U), lead (Pb), chromium (Cr), and cadmium (Cd). Changes in elemental composition with age were analyzed statistically for the selected groups, i.e., females, males, and subjects with alcohol use disorder (AUD) and without AUD. A tendency of women to lose Se, Cu, Zn, Fe with age was observed, and a disturbed Ca/Mg, Na/K ratio in subjects with AUD. Although the observed trends were not statistically significant, they shed new light on the risks and possible pathologies associated with metal neurotoxicity in the visual tract.

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.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.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], P0.00001). The nasal segment of RNFL had the largest mean difference (MD -9.76 [-12.39, -7.13], P0.0001). 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 (P0.0001). Meta-regressions showed significant predictive capability of preoperative RNFL in determining visual function outcome (P0.05).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.

BACKGROUND Tumors involving the sellar area often cause visual disorders due to optic nerves/chiasm compression, so that surgery is required. However the likelihood of visual improvement/restoration is variable. Optical coherence tomography (OCT) is a valid diagnostic tool for defining pathological conditions of the optic apparatus. We herein investigated the role of the SD-OCT and the best corrected visual acuity in predicting postoperative visual recovery, in patients complaining of chiasm compression due to sellar-suprasellar lesions. MATERIALS AND METHODS We retrospectively analyzed 20 patients (7 females and 13 males; mean age 50,8 ± 17,87 years, range 11-83), with optic chiasm/nerve compression that underwent endoscopic endonasal approach at the Division of Neurosurgery of the Universita degli Studi di Napoli "Federico II", Naples - Italy, between June 2014 and February 2015. Pre-operative ophthalmological assessment focused on BCVA Snellen charts, Standard Automated Perimetry test and SD-OCT. Test were repeated at two weeks, one and three months after surgery. RESULTS A significant thickness reduction in all the patients, as compared to the control group was noted: GCC thickness reduction remained mostly unchanged postoperatively. 42.9% of the patients had altered visual acuity (VA), while the Perimetry revealed 90% of altered preoperative MD values (MD> -2.00): perimetry improvement was noted in 93% of cases and visual acuity in 91.3% of cases. A direct correlation between preoperative retinal status and functional recovery was identified considering the values of pRNFL and GCC. CONCLUSIONS OCT provides valuable information in regards to the entity of compression also in patients without any visual field defects. Although several factors should be taken into consideration, we retain that preoperative RNFL and GCC thickness values can be claimed as a reliable predictors of visual recovery in those patients presenting chiasm compression.

Fingolimod (FTY720) is an oral immunosuppressive compound that has been prescribed to multiple sclerosis (MS) patients since 2010. The lipophilicity and low molecular weight of FTY720 allows it to cross blood brain barrier (BBB) and exert both peripheral and central effects. Previous reports showed that intranasal (IN) administration of drugs are the preferred non-invasive route, which bypasses BBB and improves their delivery and bioavailability in the central nervous system (CNS). Therefore, we aimed to compare the effects of IN and oral administrations of FTY720 on astrocyte activation and demyelination levels of optic chiasm in a focal demyelination model.The experimental model was induced by injection of 2 µL lysolecithin 1% into the optic chiasm of male Wistar rats. The rats were treated by oral gavage or intranasal drop of FTY720 at dose of 0.3 mg/kg for 14 days. Astrocyte activation was analyzed using GFAP immunostaining, extent of demyelination, and myelination levels were measured by fluoromyelin staining, and MOG immunostaining, respectively. Then, the concentration of FTY720 was measured by high performance liquid chromatography (HPLC) method in brain tissues.Our data showed that IN administration of FTY720 significantly decreases astrocyte activation and demyelination levels in the optic chiasm compared to the oral administration route. In addition, the concentration of FTY720 was higher in the brain tissue of IN receiving rats compared to the oral treated group.It seems that IN administration of FTY720 may be a preferred route to decline the central inflammation and demyelination levels in the MS patients.

SIGNIFICANCE Pituitary apoplexy is a syndrome with a varied appearance, which carries a significant risk of morbidity and mortality. It is important to recognize the potential numerous symptoms and clinical findings, urgently investigate with the proper neuroimaging tests, and coordinate care with the appropriate specialists without delay. PURPOSE This study aimed to describe a patient with worsening headache and ophthalmoparesis attributable to pituitary apoplexy who initially had reportedly a normal neuroimaging result and were diagnosed with migraine. CASE REPORT A 39-year-old Hispanic man with a history of migraine developed a new and worsening headache. He presented to a hospital emergency department where he underwent a non-contrast-enhanced computed tomography and MRI, whose results were subsequently interpreted as normal. His headache was attributed to migraine, and he was medicated as such and discharged. Three days later, he developed horizontal and vertical diplopia and sought a second opinion. His visual acuity and visual fields were normal. He manifested a right pupil-sparing, external partial cranial nerve III palsy and concurrent right sixth nerve palsy. He also complained of worsening headache and lethargy. He was immediately referred for contrast-enhanced MRI and magnetic resonance angiography with suspicion of pituitary apoplexy. Subsequent imaging revealed a hemorrhagic pituitary macroadenoma consistent with pituitary apoplexy that was expanding laterally into the right cavernous sinus. He underwent immediate neurosurgical surgical repair. CONCLUSIONS New or worsening headache with signs and symptoms of hypopituitarism should immediately be investigated for pituitary apoplexy. Other possible findings include involvement of cranial nerves III through VI because of cavernous sinus involvement and visual deficits if the optic chiasm or intracranial portion of the optic nerve is involved. However, growth may be lateral, and no visual deficits may be found, as in this case. Multiple concurrent cranial neuropathies should increase suspicion for cavernous sinus involvement.

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.

Background Distal catheterization in the ophthalmic artery beyond the origin of the central retinal artery has been attempted to avoid visual complications in cases of transarterial embolization (TAE). Although avoiding visual complications is important, extraocular complications have been rarely reported and discussed. Here, we report a case of an intraorbital arteriovenous fistula (AVF) presenting with impaired extraocular movement after a provocation test and discuss the potential risks associated with TAE at the third segment of the ophthalmic artery. Case Description A 53-year-old man was referred to our hospital for a newly diagnosed vascular lesion on the left optic chiasm. A left internal carotid angiogram revealed an intraorbital AVF fed by distal branches of the left ophthalmic artery taking a recurrent course toward the proximal ophthalmic artery, and the anterior branches of the inferior lateral trunk draining into the tortuous basal vein of Rosenthal with a varix. Neither the cavernous sinus nor the superior ophthalmic vein was opacified as draining routes of this lesion. A provocation test was done for diagnostic and therapeutic purposes at the third segment of the ophthalmic artery. During the test, the patient developed a transient impaired adduction of the left eye without any visual field deficit; therefore, TAE was avoided. The patient made an uneventful recovery and was recommended a transcranial transvenous embolization for a radical treatment. Conclusions In intraorbital hypervascular lesions, occlusion of the third segment of the ophthalmic artery is associated with a potential risk of extraocular complications.

To avoid deterioration of visual function, extended endoscopic endonasal transsphenoidal surgery (TSS) for craniopharyngioma was performed with visual evoked potential (VEP) monitoring using light-emitting diodes (LEDs). The position of the optic chiasm was carefully evaluated on the preoperative midsagittal magnetic resonance (MR) images. Intraoperatively, direct and sharp dissection of the tumor from the optic chiasm was performed under VEP monitoring with LEDs through extended endoscopic endonasal TSS. If the VEP finding changed and became unstable, the operator were informed and stopped the surgical manipulation for the optic chiasm to recover. After 5–10 min, recovery of VEP findings was checked and the procedure resumed. Extended endoscopic endonasal TSS with VEP monitoring was performed in consecutive 7 adult patients with newly diagnosed suprasellar craniopharyngiomas with maximum diameters of 25–41 mm (mean 33.7 mm). VEPs were stable throughout the surgery in 5 cases, but showed temporary instability and amplitude decrease in 2 cases, although the VEPs had recovered at the end of the surgery. Visual function, evaluated using visual impairment score, was improved after surgery in all patients. Gross total removal was achieved in 5 cases, and subtotal removal (90%) in 2 cases. Intraoperative VEP monitoring is the only way to test visual function during surgery, and may be important and helpful in extended endoscopic endonasal TSS, which requires direct dissection between the optic nerve and craniopharyngioma under the endoscope.

Introduction: The aim of the study was to determine which microperimetry and contrast sensitivity test parameters would prove the most valuable during diagnosing optic chiasm compression due to pituitary adenomas. Material and methods: A control group comprised healthy individuals (Group 1). Patients with pituitary macroadenoma were divided into two groups: Group 2 — absent optic chiasm compression; and Group 3 — present optic chiasm compression detected on contrastenhanced magnetic resonance imaging (MRI). Each group comprised 20 patients (40 eyes), i.e. a total of 60 patients (120 eyes) were examined. A complete ocular examination, intraocular pressure, microperimetry, contrast sensitivity test, kinetic Goldmann visual field, and static Octopus visual field test were performed. Results: Group 1 and 2 variables showed no statistically significant differences with respect to the mean sensitivity (MS) and mean defect (MD) in microperimetry. After dividing the microperimetry area into quadrants, a difference was shown in the mean sensitivity of the lower-nasal quadrant (MS LN) and mean defect of the lower-nasal quadrant (MD LN) between those groups. Receiver operating characteristic (ROC) curves analysis revealed that the microperimetry parameter — MS LN as well as row D and E contrast sensitivity test could be highly specific in the assessment of early damage of the optic nerve in patients suffering from pituitary adenoma. Conclusions: Microperimetry and contrast sensitivity test are non-invasive diagnostic investigations adjunctive to MRI, which facilitate detection of early chiasmal compression caused by pituitary adenomas.

Background The mechanisms of pendular seesaw nystagmus (SSN) remain unknown. Methods We evaluated modulation of pendular SSN by removal of visual fixation, convergence, and positional changes in 2 patients, one with bitemporal hemianopia due to a traumatic damage of the optic chiasm and the other with platybasia compressing the medulla and lower cerebellum. Results In both patients, the pendular SSN markedly decreased or disappeared with convergence, without visual fixation in darkness, during static head tilt toward each shoulder while sitting and while supine. Conclusions The similar patterns of nystagmus modulation observed in our patients with a different etiology indicate a common role of both visual and otolithic inputs in generating pendular SSN.

The authors report the case of a 75-year-old man with preexisting glaucoma and recurrent retinal detachment who underwent intraocular silicone oil placement OD resulting in subsequent retrolaminar silicone oil migration to the optic chiasm and vision loss OS. MRI showed silicone oil tracking posteriorly along the right optic nerve to the chiasm. He was placed on high-dose corticosteroids and underwent a successful optic nerve sheath fenestration with improvement of vision in the contralateral eye. Clinicians should be cognizant of the potential for translaminar posterior migration of intraocular silicone oil, as well as the utility of optic nerve sheath fenestration to decompress the anterior visual pathways and restore vision.

Meningiomas are reasonably common benign intracranial tumours. Those treated surgically are not necessarily the same as those addressed by radiotherapy, in terms of patient factors and anatomical location. Using "lack of progression" as the criterion of benefit, in this population of 612 treated lesions (541 patients), the 10 year rate is 93% by any of the radiotherapy means used. Single fraction stereotactic radiosurgery (SRS) was used for smaller meningiomas, with fractionated radiotherapy for large volume tumours, and/or those in contact with the optic chiasm/nerve. Using median volume as the variable, larger tumours were as adequately controlled as smaller tumours. Symptomatic improvement was achieved in the majority of patients with worsened neurological deficit in5% of patients. Subtotal resection followed by radiotherapy provides little advantage over radiotherapy alone. Local control is achieved in close to two-thirds of patients with Grade 2 or 3 meningiomas. More likely amongst the patients who have radiotherapy alone there will be patients with Grade 2 or 3 tumours (but are not biopsied) who have good long-term control. Surgery is very appropriate where complete resection (with low morbidity) can be achieved, and/or to decompress those tumours with pressure features. Informed consent can include a discussion regarding the role of radiotherapy.

This study determines the impact of change in aeration in sinonasal cavities on the robustness of passive‐scattering proton therapy plans in patients with sinonasal and nasopharyngeal malignancies. Fourteen patients, each with one planning CT and one CT acquired during radiotherapy were studied. Repeat and planning CTs were rigidly aligned and contours were transferred using deformable registration. The amount of air, tumor, and fluid within the cavity containing the tumor were measured on both CTs. The original plans were recalculated on the repeat CT. Dosimetric changes were measured for the targets and critical structures. Median decrease in gross tumor volume (GTV) was 19.8% and correlated with the time of rescan. The median change in air content was 7.1% and correlated with the tumor shrinkage. The median of the mean dose D mean change was +0.4% for GTV and +0.3% for clinical target volume. Median change in the maximum dose D max of the critical structures were as follows: optic chiasm +0.66%, left optic nerve +0.12%, right optic nerve +0.38%, brainstem +0.6%. The dose to the GTV decreased by more than 5% in 1 case, and the dose to critical structure(s) increased by more than 5% in three cases. These four patients had sinonasal cancers and were treated with anterior proton fields that directly transversed through the involved sinus cavities. The change in dose in the replanning was strongly correlated with the change in aeration (P = 0.02). We found that the change in aeration in the vicinity of the target and the arrangement of proton beams affected the robustness of proton plan.

Background Charles Bonnet Syndrome (CBS) is a rare clinical entity that is classically composed of visual hallucinations in the context of an altered optic pathway with preservation of reality judgment. This case aims to present the association of visual hallucinations with complex alterations of the nervous structures adjacent to the visual pathway and an atypical clinical presentation, thus explaining the possible mechanisms involved in the generation of these symptoms. Case presentation A 43-year-old man presents seeking care due to visual hallucinations with partial preservation of reality judgment and symptoms compatible with a major depressive disorder, including irritability and diminished hygiene habits. He has a history of complete gradual loss of vision and hyposmia. Due to poor treatment response during hospitalization, an MRI was obtained, which showed a frontal tumor lesion with meningioma characteristics adjacent to the olfactory groove and compression of the optic chiasm. He underwent surgical resection of the lesion, which remitted the psychotic symptoms, but preserving the visual limitation and depressive symptoms. Conclusions The presence of visual hallucinations, without other psychotic features as delusions, is a focus of attention for basic structural pathologies in the central nervous system. Affection at any level of the visual pathway can cause CBS. When finding atypical symptoms, a more in-depth evaluation should be made to allow optimization of the diagnosis and treatment.

Purpose The purpose of this study was to investigate the changes in structural spectral-domain optical coherence tomography (SD-OCT), OCT Angiography (OCTA) parameters, and visual acuity, 1 year after endoscopic endonasal approach for the removal of an intra-suprasellar pituitary adenoma compressing optic chiasm and compare outcomes with 48 hours postoperative data. Methods Sixteen eyes of eight patients (4 males, 4 females, mean age 52 ± 11 years) were enrolled in this prospective study. The primary outcome was to evaluate the changes over time before and after surgery, analyzing the Best Corrected Visual Acuity (BCVA), Ganglion Cell Complex (GCC), Retinal Nerve Fiber Layer (RNFL) thicknesses, the retinal vessel density (VD) of Superficial Capillary Plexus (SCP), Deep Capillary Plexus (DCP), Radial Peripapillary Capillary (RPC) and the Foveal Avascular Zone (FAZ). The secondary outcome was to identify potential biomarkers that could predict visual acuity changes after 1-year follow-up. Results When comparing SD-OCT and OCTA measurements obtained after 1 year with those observed 48 hours after surgery, GCC and RNFL were significantly improved. After a significant reduction at 48 hours, GCC thickness showed a significant increase at 1 year after surgery (p = 0.007), while a significant restoration of RNFL thickness was found at 1 year (p = 0.005), as well as the VD of SCP, DCP, and RPC values. FAZ area did not change over time. BCVA significantly improved at each time after surgery (p = 0.037, p = 0.013). A statistically significant correlation was found between the preoperative BCVA, VD of SCP, DCP, RPC, and the postoperative BCVA at 1 year (p = 0.017, p = 0.029, p = 0.031, p = 0.023). Conclusion SD-OCT and OCTA provide helpful information to identify the retinal structural and vascular improvements 1 year after surgery. OCTA parameters could serve as potential predictive markers for visual acuity recovery at long-term follow-up.

Optic chiasm cavernous malformations (CMs) are rare. Patients with these CMs typically present with progressive vision loss and are treated via microsurgical resection. We present a middle-aged man with a CM of the optic chiasm and right optic nerve treated via microsurgical resection. We used a minipterional-transsylvian approach. The CM capsule was incised at the lateral surface of the optic chiasm, and the lesion was entered. The malformation was debulked and resected in a piecemeal fashion. A plane was developed circumferentially around the CM borders to separate it from the surrounding neural tissue until removal was complete. At 6-week follow-up, the patient reported stabilized vision in his right eye, and a transient postoperative deficit in his left eye had fully resolved. Postoperative magnetic resonance imaging showed that gross total resection was achieved. Optic apparatus CMs are approached using technical principles similar to brainstem CM resections.

We evaluated postoperative retinal thickness in pediatric and juvenile craniopharyngioma (CP) patients with chiasmal compression using optical coherence tomography (OCT) auto-segmentation. We included 18 eyes of 18 pediatric or juvenile patients with CP and 20 healthy controls. Each thickness of the macular retinal nerve fiber layer (RNFL), ganglion cell layer (GCL), inner plexiform layer (IPL), inner nuclear layer, outer plexiform layer, outer nuclear layer, and photoreceptor layer was compared between the CP patients and healthy controls. There was significant thinning in the macular RNFL (estimates [μm], superior, − 10.68; inferior, − 7.24; nasal, − 14.22), all quadrants of GCL (superior, − 16.53; inferior, − 14.37; nasal, − 24.34; temporal, − 9.91) and IPL (superior, − 11.45; inferior, − 9.76; nasal, − 15.25; temporal, − 4.97) in pediatric and juvenile CP patients postoperatively compared to healthy control eyes after adjusting for age and refractive errors. Thickness reduction in the average and nasal quadrant of RNFL, GCL, and IPL was associated with peripapillary RNFL thickness, and reduced nasal quadrant GCL and IPL thicknesses were associated with postoperative visual field defects. In pediatric and juvenile patients with CP, decreased inner retinal layer thickness following chiasmal compression was observed. The changes in retinal structures were closely related to peripapillary RNFL thinning and functional outcomes.

We evaluated changes in parafoveal and peripapillary vessel density in chiasmal compression after decompression surgery using optical coherence tomography angiography (OCT-A). Sixty-two eyes with chiasmal compression for which preoperative and postoperative (4–6 months) OCT, OCT-A, visual field (VF), and comprehensive ophthalmic data were available, and 44 healthy eyes were evaluated. Vessel densities of the superficial retinal capillary plexus (SRCP), deep retinal capillary plexus (DRCP), and radial peripapillary capillary (RPC) segment were assessed using OCT-A. The postoperative measurements were compared with preoperative data. Preoperative peripapillary retinal nerve fiber layer, macular ganglion cell-inner plexiform layer thickness, and vessel densities of SRCP and RPC segments in patients’ eyes were significantly reduced compared to those of healthy controls (P P P = 0.0052, and P = 0.0085, respectively). Vessel densities were significantly decreased in the SRCP (P P = 0.0017), and RPC segments (P r = − 0.3195, P = 0.0114) and DRCP (r = − 0.5165, P r = − 0.2586, P = 0.0424). These findings indicate that decreased perfusion around the optic nerve head and on the macula associated with chiasmal compression could further progress after decompression surgery. Further functional and longer-term clinical studies are needed to elucidate the clinical implications of these findings.

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.

Occurrences of suprasellar central nervous system (CNS) embryonal tumors in adults are extremely rare. Hemorrhagic onset is further uncommon, with only anecdotic cases reported in the literature. The authors describe the case of a 57-year-old man affected by a suprasellar CNS embryonal tumor, with hemorrhagic onset and a unique diffusion pattern along the optic pathways. Background Occurrences of suprasellar central nervous system (CNS) embryonal tumors in adults are extremely rare. Hemorrhagic onset is further uncommon, with only anecdotic cases reported in the literature. The authors describe the case of a 57-year-old man affected by a suprasellar CNS embryonal tumor, with hemorrhagic onset and a unique diffusion pattern along the optic pathways. Material and Methods A 57-year-old man presenting with acute visual acuity worsening and left homonymous hemianopia was referred to our hospital. Neuroradiologic studies demonstrated an infiltrating, high-grade lesion involving the optic chiasm and right retrochiasmatic pathways with a hemorrhagic area in the ipsilateral pulvinar. Results The patient underwent microsurgical biopsy. Pathologic assessment confirmed the diagnosis of CNS embryonal tumor, not otherwise specified (NOS) according to the 2016 World Health Organization (WHO) classification of CNS tumors. The patient was referred to a multimodal adjuvant treatment; he eventually died 4 months after surgery. Competent literature has been systematically reviewed in the light of the relevant changes made in the last version of the WHO classification. Conclusion Embryonal tumors should be considered in the differential diagnosis for sellar and suprasellar space-occupying lesions, despite the rarity of the disease and the uncommon features at time of presentation. As per our knowledge, this is the first case ever described of hemorrhagic suprasellar embryonal tumor with a diffusion pattern along white matter fibers. Histogenesis, biomolecular and neuroradiologic features, and classification of embryonal tumors are an open field of research, with considerable implications for the definition of better diagnostic pitfalls and therapeutic regimens.

Purpose To evaluate the ability of confocal near-infrared reflectance (NIR) to diagnose retrograde microcystic maculopathy (RMM) in eyes with temporal visual field (VF) loss and optic atrophy from chiasmal compression. To compare NIR findings with optical coherence tomography (OCT) findings in the same group of patients. Methods Thirty-four eyes (26 patients) with temporal VF loss from chiasmal compression and 41 healthy eyes (22 controls) underwent NIR fundus photography, and macular OCT scanning. VF loss was estimated and retinal layers thickness were measured on OCT. Two examiners blinded to the diagnosis randomly examined NIR images for the presence of hyporeflective abnormality (HA) and OCT scans for the presence of microcystic macular abnormalities (MMA). The total average and hemi-macular HA area and number of microcysts were determined. The groups were compared and the level of agreement was estimated. Results The OCT-measured macular retinal nerve fiber and ganglion cell layers were thinner and the inner nuclear layer was thicker in patients compared to controls. HA and MMA were detected in 22 and 12 patient eyes, respectively, and in 0 controls (p Conclusions RMM is a frequent finding in eyes with severe VF loss from long-standing chiasmal compression. NIR photography appears to be more sensitive than OCT for detecting RMM and may be useful as screening method for its presence.

Objective Opto-chiasmatic (OC) cavernous malformations are sporadic lesions that are often misdiagnosed clinically and radiologically. Presenting symptoms range from incidental findings to the more frequent and dramatic "chiasmal apoplexy." The present study aims to evaluate the potential role of arachnoidal membranes of the basal cisterns in the onset of OC apoplexy. A possible mechanism resembling a compartment syndrome is discussed through the description of two cases of bleeding cavernomas. Methods We describe clinical, radiological, intraoperative findings in two cases of young patients presenting with OC apoplexy from bleeding cavernoma. The first was a 38-year-old man diagnosed with optic neuritis at the first episode of visual acuity deterioration. The second patient was a 22 -year-old woman who suffered two OC apoplexy episodes from a recurrence, which also presented with bleeding. Results Both patients were operated on via pterional craniotomy and presented a postoperative improvement of visual symptoms. The second patient experienced deterioration 30 months after surgical resection due to rebleeding from a recurrence and required a second operation. Follow-up revealed a good recovery of visual disturbances; MRI at 6 and 3 years showed in both patients an apparent complete removal of the cavernous malformations. Conclusion The cisternal environment where OC cavernous malformations develop and the paradigm of a compartment syndrome could explain the clinical presentation variability. This very rare subset of cavernomas would benefit from a classification system using ad hoc neuroimaging protocols and consistent indications.

We report a case of primary central nervous system lymphoma(PCNSL)originating from an optic chiasma, which was difficult to diagnose but was finally diagnosed by biopsy. A 62-year-old immunocompetent man presented with bilateral visual field disturbance, hypopituitarism, and diabetes insipidus;an optic chiasm lesion was detected on MRI. After starting steroid supplementation for adrenal insufficiency, visual field disturbance immediately improved. Since the lesion completely disappeared three months after its onset, it became the follow-up without histological confirmation. Six months after the onset, visual field disturbance progressed, and the lesion recurred. We performed a left optic nerve biopsy to maintain the right visual field, which remained partially. The pathology was PCNSL. We performed postoperative chemoradiotherapy, and the patient showed remission and improvement of the visual field. Isolated PCNSLs arising from optic chiasma are very rare. The diagnosis of optic chiasm lesions is difficult due to their similarity with a variety of inflammatory/autoimmune disease and neoplastic lesions. When a lymphoma is considered to be differentiated, early biopsy should be performed before administering a steroid. The approach and sampling site to prevent the function are also important for biopsy.

According to reports from the literature,1,2 depending on the location where cavernomas appear, range from the very common locations to unusual. Cavernous malformations arising from the optic nerve and chiasm are rare, with only few cases reported to date.3-5 We present a case of a 28-yr-old man who suddenly started with sever visual loss in the right eye and homonymous lateral hemianopia in the left eye. Because of the acute symptomatology, a brain MRI was immediately performed in order to diagnose the etiology. The MRI showed a chiasmatic mass with right extension, heterogeneous on T1 and T2 sequences, without enhancement after gadolinium. The surgery was carried out a week after the diagnosis. A right pterional transsylvian approach was performed and the cavernoma was resected with microsurgical maneuvers, preserving the optic nerve fibers, chiasm, and optic tract. The patient evolved favorably, improving the visual deficit in the postoperative period as can be observed in the postoperative visual field study 7 mo after the surgery. The patient signed an informed consent for the procedure and agreed with the use of his images and surgical video for research and academic purposes. Our surgical case emphasizes the importance of a prompt diagnosis and surgery for chiasmatic cavernomas3 associated to visual loss, providing early decompression of the optic apparatus and improvement of the visual field defects after surgery.

Purpose To evaluate whether the occurrence of chiasmal herniation coincides with visual field (VF) deterioration and to compare the course of VF defects in patients with and without radiological chiasmal herniation following treatment of pituitary adenoma. Methods This retrospective cohort study included 48 pituitary macroadenoma patients with chiasm compression, divided into three groups: Group 1 (N = 12), downward displaced optic chiasm and deteriorated VFs; Group 2 (N = 16), downward displaced optic chiasm; Group 3 (N = 20), control-group matched for tumour size and follow-up VFs, in mean deviation (dB). VFs were compared over time and a severity index, Chiasm Herniation Scale (CHS), for herniation based on radiological parameters was designed. Results After treatment, all groups showed improvement of VFs (Gr1: 2.97 dB p = 0.097, Gr2: 4.52 dB p = 0.001 and Gr3: 5.16 dB p = 0.000), followed by long-term gradual deterioration. The course of VFs between patients with and without herniation was not significantly different (p = 0.143), neither was there a difference in the course before and after herniation (p = 0.297). The median time till onset of herniation was 40 months (IQR 6 month-10 years) and did not significantly differ (p = 0.172) between the groups. There was no relation between VFs and the degree of herniation (p = 0.729). Conclusion Herniation does not appear to have clinical relevance with respect to VF outcome. The newly designed CHS is the first scoring system to quantify the severity of herniation and, in the absence of alternatives, may be useful to describe MRI findings to serve future added value in larger sized outcome studies.

Aim: The purpose of the study is to perform a dosimetric analysis of the doses received by planning target volume and organ at risks in the postoperative glioblastoma by using 3D-conformal radiotherapy to a total dose of 60 Gy in 30 fractions. Materials & Methods: All patients received concurrent temozolomide every day, and this was followed by adjuvant temozolomide of 5 days of treatment per month. Results: More than 98% of patients were treated with a dose of 60 Gy. Doses were analyzed for the normal whole brain, tumor volume, as well as all the organs at risk. Conclusion: Given the grave prognosis and the limited survival of glioblastoma despite the best treatment available, makes 3D-conformal radiotherapy an equally acceptable treatment option.

Ethambutol is an integral part of Antitubercular therapy (ATT) and is often associated with optic neuropathy, However, neuroimaging of ethambutol induced optic neuropathy has been sparsely reported in the literature. We describe the case of a 45-year male patient, diagnosed as Tuberculous spondylodiscitis and was on ATT. Four months after ATT initiation, he presented with visual blurring in both the eyes with bitemporal hemianopia and central scotomas. Visual evoked potential (VEP) revealed prolonged latencies in N75 and P100 waveforms bilaterally. Magnetic Resonance Imaging (MRI) showed optic chiasma and bilateral optic tract hyperintensities on 3D Fluid Attenuated Inversion Recovery (FLAIR) and 3D Double Inversion Recovery (DIR) sequences. Ethambutol was discontinued immediately. On follow-up after 8 weeks, visual acuity reversed back to normal in both eyes.

Objective CTNNB1-targeted inhibitor is demonstrated to be an effective neoadjuvant therapy in adamantinomatous craniopharyngioma (ACP) patients and cystic degeneration is a canonical sign of pediatric ACP. This study aimed to investigate the relationship between the cystic performances and CTNNB1 mutation (CTNNB1 MUT) status so as to analyze the possible diagnostic criteria of CTNNB1 MUT in pediatric cystic ACP (PCACP). Methods Patient’s population, clinical characteristics, tissue samples and MRI data were collected and summarized in PCACP patients. The results were compared between CTNNB1 MUT and CTNNB1 wild-type (WT) groups according to the Sanger sequencing. MRI features of the cyst were also recorded. The receiving operating characteristic (ROC) curve analysis was applied to evaluate the differential diagnostic value. Results 19 of the 61 patients manifested CTNNB1 MUT PCACP and 42 patients were CTNNB1 WT PCACP. Multiple cysts, irregular shape of cyst, hypo-intense interior signal of cyst on non-contrast T1W1, compression with optic chiasm and pituitary stalk and enhancement signal of cystic wall have been demonstrated in CTNNB1 MUT PCACP patients on MRI. Only the Area under the curve (AUC) values of quantity of cyst, shape of cyst and interior signal of cyst on non-contrast T1W1 were over 0.7. For criteria based on the combination of the 6 characteristic features, the AUC value was 0.928. Conclusion Preoperative MRI may provide an effective value in predicting PCACP patients with CTNNB1 MUT and offer potential evidence for preoperative management with molecular targeted agents.