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An 81-year-old woman developed painful vision loss to hand motions in the right eye over a several-day period. Dilated fundus examination revealed no acute pathology, but automated perimetry showed a superotemporal visual field defect in the asymptomatic left eye, suggestive of a junctional scotoma. Magnetic resonance imaging demonstrated enhancement of the right optic nerve extending to its junction with the optic chiasm. The patient's vision failed to improve with intravenous corticosteroids, but demonstrated significant improvement with therapeutic plasma exchange. She was subsequently found to be seropositive for aquaporin-4 autoantibodies, confirming the diagnosis of neuromyelitis optic spectrum disorder.

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.

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.

Purpose This study evaluates the temporal evolution of the spatial relationship between the pituitary adenoma transsphenoidal surgical cavity and the adjacent optic chiasm and discusses implications on timing and choice of radiotherapy modality. Methods This retrospective observational review analyzed factors that might influence the postoperative relationship between the surgical cavity and the optic chiasm, including tumor type, craniocaudal tumor and resection cavity dimensions, the preoperative distance between tumor and the optic chiasm, the presence of cavernous sinus invasion, and the choice of intraoperative packing material. Changes observed on magnetic resonance imaging in the preoperative, immediate (within 72 h), and delayed (≥3 months) postoperative periods were compared. Results Sixty-five patient histories were analyzed. Preoperatively, the pituitary adenoma was apposed to the optic chiasm in 43 patients (66%). Postoperatively, 34 patients (52%) in the immediate postoperative period and 54 patients (83%) in the delayed postoperative period had a distance ≥2 mm between the resection cavity and the optic chiasm. This distance provides a greater margin of safety with adjuvant radiosurgery. Preoperative tumor size showed a strong association with postoperative descent of the optic chiasm. Conclusions Preoperative tumor size and degree of mass effect on the optic chiasm predict postoperative changes. In this study, the distance between the resection cavity and the optic chiasm was greater at ≥3 months postoperatively than in the immediate postoperative period, regardless of preoperative mass effect, indicating radiotherapy planning should be deferred to ≥3 months postoperatively when not precluded by aggressive histological characteristics that necessitate more immediate treatment. Precis To investigate the temporal relationship between the postoperative sellar surgical cavity and the adjacent optic apparatus after transsphenoidal resection of pituitary adenomas and the implications for radiotherapy.

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.

A previously healthy 62-year-old African American female presented with a fulminant orbital cellulitis of the right eye with diffuse scleritis and orbital inflammation extending to the optic chiasm on neuroimaging. She was taken for an emergent orbitotomy with an orbital fat biopsy and started on broad-spectrum intravenous (IV) and topical antibiotics. Within 36 hours of presentation, scleral thinning and a corneal melt ensued, ending in enucleation. Intraoperative cultures were positive for Clostridium septicum, leading to a systemic work-up exposing a previously undiagnosed colon adenocarcinoma and metastatic multiple myeloma.

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.

Unexplained or idiopathic pituitary stalk thickening or central diabetes insipidus not only harbours rare occult malignancies in 40% of cases but can also reflect benign congenital defects. Between 2014 and 2019, a multidisciplinary, expert national guideline development group in the UK systematically developed a management flowchart and clinical practice guideline to inform specialist care and improve outcomes in children and young people (aged

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.

Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disease of the central nervous system (CNS) associated with antibodies to aquaporin-4 (AQP4), which has distinct clinical, radiological and pathological features, but also has some overlap with multiple sclerosis and myelin oligodendrocyte glycoprotein (MOG) antibody associated disease. Early recognition of NMOSD is important because of differing responses to both acute and preventive therapy. Magnetic resonance (MR) imaging has proved essential in this process. Key MR imaging clues to the diagnosis of NMOSD are longitudinally extensive lesions of the optic nerve (more than half the length) and spinal cord (three or more vertebral segments), bilateral optic nerve lesions and lesions of the optic chiasm, area postrema, floor of the IV ventricle, periaqueductal grey matter, hypothalamus and walls of the III ventricle. Other NMOSD-specific lesions are denoted by their unique morphology: heterogeneous lesions of the corpus callosum, ‘cloud-like’ gadolinium (Gd)-enhancing white matter lesions and ‘bright spotty’ lesions of the spinal cord. Other lesions described in NMOSD, including linear periventricular peri-ependymal lesions and patch subcortical white matter lesions, may be less specific. The use of advanced MR imaging techniques is yielding further useful information regarding focal degeneration of the thalamus and optic radiation in NMOSD and suggests that paramagnetic rim patterns and changes in normal appearing white matter are specific to MS. MR imaging is crucial in the early recognition of NMOSD and in directing testing for AQP4 antibodies and guiding immediate acute treatment decisions. Increasingly, MR imaging is playing a role in diagnosing seronegative cases of NMOSD.

Monocular visual field defects generally localize at or anterior to the optic chiasm, while homonymous hemianopias localize to the retrochiasmal visual pathway. Highly incongruous visual field defects may be difficult to identify on 24-2 Humphrey visual field testing, and this case demonstrates the value of optical coherence tomography (OCT) ganglion cell-inner plexiform layer (GCIPL) in rapidly localizing the lesion. A 54-year-old woman was found on routine examination to have an isolated superonasal quadrant visual field defect respecting the vertical meridian in the left eye only on Humphrey 24-2 SITA-Fast testing. She had a remote history of significant head trauma. Visual acuity, anterior segment, and fundus examination were normal. OCT revealed a bow-tie atrophy of the retinal nerve fiber layer in the right eye (OD), and binocular homonymous hemi-macular atrophy of OCT GCIPL, confirming the localization was the left retrochiasmal visual pathway. A repeat Humphrey 30-2 SITA-Fast visual field demonstrated that the visual field defect was also present in the OD in a highly incongruous manner. Magnetic resonance imaging of the brain with contrast showed mild atrophy of the left optic tract. This case demonstrates that highly incongruous visual field defects may be difficult to identify on Humphrey 24-2 SITA-Fast visual fields, and OCT GCIPL serves as a rapid way to localize the lesion. More detailed visual field testing including 30-2 programs should be considered in these cases.

Background: Optic neuritis (ON) is often the presenting symptom in inflammatory central nervous system demyelinating disorders. Objective: To compare the frequency and pattern of optic chiasm involvement in patients with aquaporin-4-immunoglobulin G (AQP4-IgG)-associated ON to patients with myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG)-associated ON. Methods: Retrospective review of all patients evaluated at Mayo Clinic, Stanford University and Ramathibodi Hospital who were found to have: (1) ON, (2) either MOG-IgG or AQP4-IgG by cell-based assay, and (3) magnetic resonance imaging (MRI) at the time of ON. MRI was reviewed for contrast enhancement of the optic chiasm and the pattern of involvement. Results: One hundred and fifty-four patients (74 AQP4-IgG and 80 MOG-IgG) were included. Among patients with AQP4-IgG-ON, 20% had chiasmal involvement, compared with 16% of patients with MOG-IgG-ON ( p = 0.66). In patients with chiasmal involvement, longitudinally extensive optic nerve enhancement (from orbit extending to chiasm) was identified in 54% of MOG-IgG-ON patients, compared with 7% of AQP4-IgG-ON patients ( p = 0.01). Conclusion: Chiasmal involvement of MOG-IgG-ON and AQP4-IgG-ON occur at more similar frequencies than previously reported. Furthermore, MOG-IgG-ON chiasmal involvement is more likely to be part of a longitudinally extensive optic nerve lesion.

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.

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.

Purpose To evaluate the validity of electrophysiological tests in the early diagnosis of a ganglion cells and/or optic nerve dysfunction in patients with pituitary microadenoma. Methods 66 eyes, from 33 patients with microadenoma with no evidence of the optic chiasm compression in magnetic resonance imaging (MRI) and the visual impairment in the routine ophthalmological examination, standard static perimetry (24-2 white on white) and optical coherence tomography (HD-OCT), were analysed. The pattern electroretinogram (PERG), standard pattern visual evoked potentials (PVEPs) and multichannel visual evoked potentials (mVEPs) (ISCEV standards) were performed. The results obtained from the electrophysiological tests were compared to the same number of age-matched healthy controls. Results Statistically significant differences between the patients with microadenoma and healthy controls were detected in all electrophysiological tests (p Conclusions In patients with microadenoma, the abnormalities in the electrophysiological tests are registered even without clinical evidence of visual impairment from the routine ophthalmological examination, SAP, OCT and chiasmal compression in MRI. The mVEPs have the most significant role in the diagnosis of the visual pathway dysfunction in patients with microadenoma.

A 67-year-old previously healthy woman presented with progressive visual impairment including bitemporal hemianopsia. A brain magnetic resonance imaging revealed a contrast-enhancing mass in the optic chiasm, spreading along the left optic tract. The patient underwent a transcranial biopsy of the left optical tract that yielded a diagnosis of diffuse large B-cell lymphoma. CT scans of the chest, abdomen, and pelvis, PET-CT, and bone marrow biopsy revealed no evidence of systemic lymphoma. Thus, the final diagnosis was of primary central nervous system lymphoma of the optic chiasm. Systemic treatment was initiated with full response. Six months after the end of the treatment, recurrence at cerebellum parenchyma and left tentorium was recorded. A new systemic treatment achieved full response. A second recurrence was noted in an optical coherence tomography of the right eye, 2 years after the initial diagnosis. The patient was treated with intravitreal methotrexate with initial success, but eventual failure after 10 months. Intravitreal rituximab was used with no effect. The patient was then referred to radiotherapy and underwent external beam radiotherapy with VMAT. There were no acute toxicities to report. After the radiotherapy treatment, at 1-year follow-up, the patient has no evidence of disease. Long-term toxicities were recorded and are considered manageable. The present case emphasizes the role of ocular irradiation as an option in the management of intraocular lymphoma patients, including in the salvage setting, with an acceptable ocular toxicity profile.

Ana Maria Cunha,1 Rodrigo Vilares-Morgado,1 Ana Filipa Moleiro,1 Fernando Falcão-Reis,1,2 Olinda Faria1,2 1Department of Ophthalmology, Centro Hospitalar Universitário de São João, Porto, Portugal; 2Department of Surgery and Physiology, Faculty of Medicine, University of Porto, Porto, PortugalCorrespondence: Ana Maria CunhaDepartment of Ophthalmology, Centro Hospitalar Universitário de São João, Avenida Prof. Hernâni Monteiro, Porto, 4202 – 451, PortugalTel +35 1225512100Fax +35 1225513669Email ana.cunha.18@gmail.comBackground: Leber hereditary optic neuropathy (LHON) is an optic neuropathy of mitochondrial inheritance. Childhood-onset disease is relatively rare and there are limited data on this important patient subgroup.Case Presentation: We present 3 particular presentations of LHON. Patient 1 was an 8-year-old boy admitted to the emergency department reporting a progressive bilateral visual loss and intermittent headaches. Neuro-ophthalmological examination revealed a bilateral pseudopapilledema. Lumbar puncture identified intracranial hypertension and the brain and orbits magnetic resonance imaging showed T2 hyperintensity in the posterior region of the left optic nerve and the optic chiasm. Patient 2 was a 12-year-old boy admitted to the emergency department reporting painless, progressive central vision loss in the right eye. Fundus examination revealed a hyperemic disc and vascular network papillary and peripapillary vascular microdilations. Three months later, the left eye presented visual loss. Patient 3 was a 6-year-old female child referred to the neuro-ophthalmology specialist due to painless central visual loss in both eyes. Her BCVA was 1/10 and counting fingers in right and left eye, respectively, and fundus examination revealed a pallor optic disc in the temporal sector.Discussion: The phenotype of childhood-onset disease may present itself distinct from classical adult-onset LHON. The absence of classical clinical features could lead to initial misdiagnosis. There should exist a high index of suspicion in children presenting unexplained subnormal vision in order to avoid potential diagnostic delays.Keywords: Leber hereditary optic neuropathy, childhood-onset disease, mitochondrial inheritance

Purpose Periorbital tumor location presents a significant challenge with 3-dimensional conformal radiation therapy or intensity modulated radiation therapy due to high tumor dose needed in the setting of close proximity to orbital structures with lower tolerance. Proton beam therapy (PBT) is felt to be an effective modality in such cases due to its sharp dose gradient. Materials and Methods We reviewed our institutional PBT registry and identified 17 patients with tumor epicenters within 2 cm of the eye and optic apparatus treated with passive scatter PBT with comparison volumetric arc therapy plans available. Maximum and mean doses to organs at risk of interest, including optic nerves, optic chiasm, lens, eye ball, pituitary, cochlea, lacrimal gland, and surrounding brain, were compared using the paired Wilcoxon signed rank test. Overall survival was determined using the Kaplan-Meier method. Results Median age was 67. Median follow-up was 19.7 months. Fourteen patients underwent upfront resection and received postoperative radiation and 3 received definitive radiation. One patient received elective neck radiation, 2 underwent reirradiation, and 3 had concurrent chemotherapy. There was a statistically significant reduction in mean dose to the optic nerves and chiasm, brain, pituitary gland, lacrimal glands, and cochlea as well as in the maximum dose to the optic nerves and chiasm, pituitary gland, lacrimal glands, and cochlea with PBT. The 18-month cumulative incidence of local failure was 19.1% and 1-year overall survival was 80.9%. Conclusion Proton beam therapy resulted in significant dose reductions to several periorbital and optic structures compared with volumetric arc therapy. Proton beam therapy appears to be the optimal radiation modality in such cases to minimize risk of toxicity to periorbital organs at risk.

Albinism is a group of rare inherited disorders arising from impairment of melanin biosynthesis. The reduction of melanin synthesis leads to hypopigmentation of the skin and eyes. A wide range of ophthalmic manifestations arise from albinism, including reduction of visual acuity, nystagmus, strabismus, iris translucency, foveal hypoplasia, fundus hypopigmentation, and abnormal decussation of retinal ganglion cell axons at the optic chiasm. Currently, albinism is incurable, and treatment aims either surgically or pharmacologically to optimize vision and protect the skin; however, novel therapies that aim to directly address the molecular errors of albinism, such as l-dihydroxyphenylalanine and nitisinone, are being developed and have entered human trials though with limited success. Experimental gene-based strategies for editing the genetic errors in albinism have also met early success in animal models. The emergence of these new therapeutic modalities represents a new era in the management of albinism. We focus on the known genetic subtypes, clinical assessment, and existing and emerging therapeutic options for the nonsyndromic forms of albinism.

Introduction: Germ Cell Tumors (GCT) represent less than 4% of primary brain tumors. They comprise Germaniums, Non-Germinomatous Germ Cell Tumors and Teratomas. Teratomas represent less than 20% of intracranial GCT. They are tumors of multipotential cells derived from all 3 germ cell layers, frequently arising in midline structures, most commonly in the pineal and suprasellar regions, with a clear excess of male cases and frequently found in children and young adults. We report a case of a mature teratoma in the third ventricle in a 37-year-old male. Case Report: We report a case of a 37-year-old male with a history of headache lasting up to 9 days and refractory to pharmacological treatment. The CT scan revealed a 20mm round hypodense lesion in the anterior third ventricle, with a punctate hyperdensity in the inferior pole causing biventricular hydrocephalus with no periventricular lucency and the MRI showed a well-defined encapsulated mass lesion attached to the roof of the third ventricle, isointense in T1WI with circumferential enhancement and hyperintense in T2WI. Gross total resection was performed. Histopathologic evaluation revealed a mature teratoma. There was no evidence of recurrence on follow up MRI at 2 years. Conclusion: Intracranial teratomas typically originate in midline structures from optic chiasm to pineal region. Presentation after the first two decades of life is exceptional. Complete surgical resection is the only curative treatment for pure mature teratomas. We report the case of a mature teratoma in a 37-year-old male with unusual radiological findings.

Background Radiation therapy for intracranial lesions is constrained by dose to neurologic organs at risk. Case Description We report 2 cases, a newly diagnosed chondrosarcoma and a previously irradiated meningioma, with tumors that abutted the optic chiasm following subtotal resection. Definitive radiotherapy would have required either undercoverage of the tumor or treatment of the chiasm with doses posing an unacceptable risk of blindness. Therefore, the patients underwent open surgery with placement of an abdominal fat autograft to provide space between the tumor and the optic structures at risk. Patients received definitive fractionated stereotactic radiotherapy. For each patient, we retrospectively compared the treated plan (with fat autograft) to a second plan generated using the pre-autograft imaging, maintaining similar tumor coverage. For the chondrosarcoma, the fat autograft reduced the optic chiasm maximum dose by 21% (70.4 Gy to 55.3 Gy). For the reirradiated peri-optic meningioma, the optic chiasm maximum dose was reduced by 10% (50.8 Gy to 45.9 Gy), the left optic nerve by 17% (48.9 Gy to 40.4 Gy), and the right optic nerve by 30% (32.3 Gy to 22.6 Gy). Conclusions We demonstrate the utility of abdominal fat autograft placement to maximize coverage of tumor while minimizing dose to intracranial organs at risk.

Purpose This report describes the clinical course of the traumatic chiasmal syndrome and treatment of diplopia due to the hemifield-slide phenomenon. Case report A 20-year-old male with decreased vision in both eyes following blunt head trauma was referred to the neuro-ophthalmology clinic. Neuroimaging revealed disruption of the optic chiasm and skull base fracture. After partial vision recovery, the patient noticed complete bitemporal hemianopia. Three months later, he was exotropic and complained of diplopia and difficulty in near visual tasks. His symptoms were successfully controlled by injection of botulinum toxin A in both lateral rectus muscles. Conclusions There is some potential for improvement in vision in patients with traumatic chiasmal syndrome. Botulinum toxin could be used in patients with the hemifield-slide phenomenon to predict the sensory outcome of surgery and relieve the bothersome symptoms.

We aimed to assess clinical characteristics of apoplexy of pituitary microadenomas compared to macroadenomas. We retrieved clinical records of patients > 18 years old, hospitalized in Rambam hospital between January 2001 and October 2017, with pituitary apoplexy and follow-up of at least one year. We compared clinical course and outcomes of apoplexy between patients with microadenomas and macroadenomas, and between patients who received conservative or surgical treatment. Statistical analysis was done using Fisher’s exact and Mann–Whitney tests. Twenty-seven patients with pituitary apoplexy were included in the final analysis: mean age was 40.7 ± 12.5 years, 13(48%) were female, 7(26%) had microadenomas, and 21(78%) harbored clinically nonfunctioning pituitary adenomas. Upon admission, hyponatremia, random cortisol level of

Hemianopia, loss of vision in half of the visual field, results from damage to the visual pathway posterior to the optic chiasm. Despite negative effects on quality of life, few rehabilitation options are currently available. Recently, several long-term training programs have been developed that show visual improvement within the blind field, although little is known of the underlying neural changes. Here, we have investigated functional and structural changes in the brain associated with visual rehabilitation. Seven human participants with occipital lobe damage enrolled in a visual training program to distinguish which of two intervals contained a drifting Gabor patch presented within the blind field. Participants performed ∼25 min of training each day for 3–6 months and undertook psychophysical tests and a magnetic resonance imaging scan before and after training. A control group undertook psychophysical tests before and after an equivalent period without training. Participants who were not at ceiling on baseline tests showed on average 9.6% improvement in Gabor detection, 8.3% in detection of moving dots, and 9.9% improvement in direction discrimination after training. Importantly, psychophysical improvement only correlated with improvement in Humphrey perimetry in the trained region of the visual field. Whole-brain analysis showed an increased neural response to moving stimuli in the blind visual field in motion area V5/hMT. Using a region-of-interest approach, training had a significant effect on the blood oxygenation level-dependent signal compared with baseline. Moreover, baseline V5/hMT activity was correlated to the amount of improvement in visual sensitivity using psychophysical and perimetry tests. This study, identifying a critical role for V5/hMT in boosting visual function, may allow us to determine which patients may benefit most from training and design adjunct interventions to increase training effects. SIGNIFICANCE STATEMENT Homonymous visual field loss is a common consequence of brain injury and is estimated to affect more than 230,000 people in the United Kingdom. Despite its high prevalence and well-described impact on quality of life, treatments to improve visual sensitivity remain experimental, and deficits are considered permanent after 6 months. Our study shows that behavioral changes following vision rehabilitation are associated with enhanced visually-evoked occipital activity to stimuli in the blind visual field. Unlike previous behavioral studies, we observe clinical changes that are specific to the trained region of vision. This lends significant weight to such training paradigms and offers a mechanism by which visual function can be improved despite damage to the primary visual pathway.

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.

A 62-year-old woman suffering from pulmonary Mycobacterium avium complex (MAC) disease was admitted to our hospital with fever, visual impairment, and lower limb weakness. MRI detected lesions in the optic chiasm and spinal cord extending the length of 6 vertebrae. The anti-aquaporin 4 (AQP4) antibody titer determined by ELISA was elevated to 8.3 IU/l. On the basis of these findings, the patient was diagnosed as having neuromyelitis optica (NMO), when chest CT also demonstrated exacerbation of pulmonary lesions. Methylprednisolone pulse therapy and double-filtered plasma exchange ameliorated the symptoms, and the EDSS score improved from 8.5 to 6.5. Six months later, visual impairment recurred, although ELISA showed that the anti-AQP4 antibody titer had become undetectable. Also, the CSF interleukin-6 (IL-6) level was elevated to 34.8 pg/ml. There have been few reports of NMO associated with pulmonary MAC disease. An increase of IL-6 is considered to exacerbate the clinical picture of NMO, whereas it may suppress progression of the pulmonary MAC disease. Exacerbation of the pulmonary MAC disease and the following internal counteraction with IL-6 may have resulted in a NMO relapse. The present patient was therefore administered eculizumab but not satralizumab, a humanized anti-IL-6 receptor antibody, for prevention of NMO recurrence.

Purpose: The aim of this study was to determine the alteration in ganglion cell complex and its relationship with retinal nerve fiber layer (RNFL) thickness as measured by spectral-domain optical coherence tomography (OCT) in pituitary adenoma cases and also its correlation with visual field (VF). Methods: This is a prospective comparative study wherein detailed neuro-ophthalmic examination including perimetry, RNFL and ganglion cell layer inner plexiform layer (GCL-IPL) thickness were measured preoperatively in the cases of pituitary adenoma with chiasmal compression with visual symptoms and field changes who were planned for neuro-surgical intervention. These parameters were repeated 1 year after the surgery. GCL-IPL, RNFL parameters were compared with controls and were correlated with VF mean deviation (MD). The diagnostic power of GCL-IPL was tested using the receiver operating characteristic (ROC) curve. Healthy age and sex-matched controls without any ocular and systemic abnormality were taken for comparison. Results: Twenty-four patients qualified the inclusion criteria. A significant thinning of GCL-IPL (P = 0.002) and RNFL (P = 0.039) was noticed in the pituitary adenoma group. GCL-IPL (r = 0.780 P < 0.001) and RNFL (r = 0.669, P < 0.001) were significantly correlated with the MD. The ROC curve values of GCL-IPL were 0.859 (95% confidence interval 0.744% to 0.973) and of RNFL were 0.731 (95% confidence interval 0.585–0.877). The diagnostic ability of GCL-IPL was more as compared to the RNFL analysis, although it was statistically insignificant (P = 0.122). Conclusion: GCL-IPL measurements on the OCT are a sensitive tool to detect early anterior visual pathway changes in chiasmal compression for pituitary adenoma patients.

Introduction: Paediatric tumours in the sellar and parasellar regions present clinical and surgical challenges due to anatomical position and behaviour. We illustrate a rare case which caused obstructive hydrocephalus. Case Presentation: The study included a 14-year-old girl with a glioneuronal tumour (40 mm) originating from the optic chiasm, obliterating the aqueduct, with consequent triventricular hydrocephalus. The patient underwent extended endoscopic endonasal surgery and repair of the skull-base deficiency using a multi-layer technique with fascia lata. The 12-month follow-up showed no complications or recurrences, with recovery in visual acuity. Conclusion: The immediate placement of external ventricular drainage, in combination with an extended trans-sphenoidal approach, is a safe and feasible option to treat suprasellar paediatric lesions with hydrocephalus.

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.

Background: Meningioma is common primary central nervous system tumors. Twenty-five percent of all meningioma consist of skull base meningioma. Planum sphenoidale meningiomas are rare. Planum sphenoidale meningiomas can extend into adjacent areas. Approximately two thirds of patients complain of failing vision in one eye as the first symptom. Case Report: A 32-year-old woman presented with 6-month history of progressively worsening blurred of both of vision. She also complained her smell ability was reduced for 3 months. She had headache for 6 months. The pain was worsening in the morning. She is conscious. A neurologic examination revealed bilateral hyposmia and visual deficits but no weakness. Visus of oculo dextra was 1/300 and visus of oculo sinistra was no light perception. Magnetic resonance imaging (MRI) intravena contrast of brain revealed a large extra-axial mass measured ±6,2x5,9x6 cm centred on planum sphenoidale displacing both frontal lobes. She had an operation of tumor removal with cranio-orbito-zygotomy approach. The tumor, which measured ±7cmx7cmx6 cm, was succesfully removed completely. She gets improvement of smell ability and both visual postoperatively. The histopathology of the tumor revealed meningioma WHO grade I. Discussion: Planum sphenoidale meningiomas present a frequently encountered pathology of the anterior skull base. These meningiomas give rise to an early visual disturbance with relatively slow progression. Displacement of the olfactory tracts and optic chiasm occur when the meningioma extends into the paranasal sinuses and nasal cavity. Clinical presentation and diagnosis often occur in the late stage. Anosmia is one of common finding on physical examination. Postoperative improvement of visual symptoms depends on the preoperative duration of those symptoms.

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.

Background Cystic lesions in the sellar region include a variety of entities, such as craniopharyngioma, Rathke cleft cyst (RCC), intrasellar arachnoid cyst, cystic pituitary adenomas, cholesterol granulomas (CGs), and xanthogranulomas (XGs). The distinction among them remains a preoperative challenge due to similarities in their clinical and radiologic findings. Case Description We describe 3 cases with cystic sellar lesions. The first patient is a woman who presented with headache and hormonal disturbances, including high levels of prolactin, with a sellar and suprasellar cystic lesion discovered on magnetic resonance imaging. She was initially treated with dopamine agonists with normalization of prolactin levels but no changes on the size of the lesion. She underwent an endoscopic endonasal resection and the histology resulted in a CG/XG. The second patient is a woman who consulted for an incidentally discovered sellar cyst. During the follow-up, the lesion demonstrated enlargement with compression of the optic chiasm. With a preoperative diagnosis of RCC, the lesion was removed through an endoscopic endonasal transsellar approach. Final pathologic diagnosis was consistent with CG/XG. The third case was that of a man who presented with refractory headaches and vision loss, with a sellar/suprasellar cystic lesion on magnetic resonance imaging. He underwent endoscopic endonasal transsellar surgery for resection of what preoperatively was thought to be a giant RCC; final pathology again was consistent with CG/XG. Conclusions CG/XG is an uncommon pathology with unspecific clinical and radiologic features. However, this pathology should be considered in the differential diagnosis of mixed cystic/solid lesions in the sellar region.

Lesions affecting the body of the optic chiasm typically produce bitemporal hemianopia. the blood supply comes from the anterior communicating artery, anterior cerebral, posterior communicating, posterior cerebral, and basilar arteries. We herein report a young patient admitted to the emergency department with acute confusion, left-sided hemiparesis, hemihypoesthesia, and dysarthria. Bitemporal hemianopia was detected after resolution of confusion. on cranial magnetic resonance imaging (MRI), infarction in the right anterolateral thalamus in the territory of tuberothalamic artery (TA) and in posterior chiasma in the territory of the posterior communicating artery (PCoA) was revealed. Cerebral MR angiography showed luminal irregularity of the PCoA. the patient was presented to draw attention to the rare entity ischemic chiasmal syndrome.

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.

Craniopharyngiomas can invade surrounding structures, including the optic chiasm and hypothalamus. In such cases, subtotal resection is often preferred to limit perioperative morbidity and mortality; however, subtotal resection is associated with high rates of recurrence. Recurrent craniopharyngioma is typically treated with another subtotal resection and adjuvant radiotherapy. We present a case of a patient found to have a large craniopharyngioma compressing the optic chiasm, hypothalamus and left cavernous sinus. She underwent surgical debulking but developed recurrence shortly thereafter. Subsequently, she underwent a second debulking surgery, followed by fractionated stereotactic radiotherapy (SRT). Results show that she exhibited an impressive response to SRT with further tumor shrinkage, while remaining clinically well. This case demonstrates the efficacy of SRT in salvage of recurrent craniopharyngioma.

Most pituitary adenomas are slow-growing and benign, which means they are not cancer and do not spread to other parts of the body. They generally have a slow but severe impact on vision due to compression of the optic nerves, optic chiasm, and cavernous sinus. However, as they grow big they can put pressure on nearby structures, such as the nerves that connect the eyes to the brain and cause symptoms. Pituitary adenomas are the third most common intracranial tumor and arise from the pituitary gland. An individual case is presented in this paper with radiological evidence (MRI) of a large lobulated intrasellar mass (3.0 cm transversely, 3.5 cm craniocaudally and 2 cm anteroposteriorly) with supra-sellar extension is causing elevation of the floor of the 3rd ventricle of the brain. Pituitary macroadenoma and hemorrhagic foei, pituitary apoplexy, and pituitary adenomas were founded by different CT scan and MRI reports in different laboratories. The patient was treated with constitutional homoeopathic medicines – Natrum muriticum-m/1, 16 doses up to Natrum muriticum-m/20, 16 doses each from 26/04/2014 to 07/08/2017. Before treatment imaging was done several times from 6/04/2011 to 12/01/2012showed complete resolution of the pituitary adenoma and during treatment3 follow-up imaging was done on 25/07/2015 and 06/08/2017. This case report reviews the clinical presentation, homoeopathic management, and treatment of the major classification of pituitary adenomas and call attention to the need for repertorization in individualized homoeopathic prescription. It is hoped that if this type of clinical research continues in the future, homeopathy will have a breakthrough result and it’s symptomatic medical treatment will play a beneficial role in the near future from the deadly evils of various types of chemotherapy, radiotherapy, or combined therapy.

Background: Caniopharyngiomas in spite of being benign tumors they have a high rate of recurrence of which cysts constitute major component. Ommaya Reservoir System (ORS) is a simple manoeuvre for percutaneous drainage of cysts. Aim of Study: To evaluate the efficacy of using C-arm as a guiding tool for placement of Ommaya reservoir tube in predominantly cystic recurrent craniopharyngiomas. Patients and Methods: This study included 8 patients with clinically symptomatic and radiologically confirmed recurrent craniopharyngioma with significant cystic part. These patients were treated by C-arm guided insertion of Ommaya reservoir tube. The technique is described in details. CT brain was obtained in the first post-operative night to assess the position of the tube. Post-operative clinical and radiological evaluations were compared to the pre-operative. Results: This study included 6 males and 2 females, ranging in age from 5 years to 38 years with mean age of 18.4 years. Headache was the most common presenting complaint, followed by visual impairment. All surgeries were performed with C-arm guidance and post-operative CT brain documented satisfactory position of the tube in 7 cases and only one case required second look surgery for repositioning of the tube with the same technique. Significant post-operative improve-ment of headache and visual symptoms were encountered in all cases due to decompression of the optic chiasm by cyst drainage. Conclusion: C-arm assisted insertion of Ommaya reservoir tube is a reliable method that can minimize the errors of free hand technique and of special value in low facility centers.

Background Intraorbital arteriovenous fistulas (AVFs) are rare lesions, and their treatment is challenging. We have presented a case of an intraorbital AVF treated with endovascular embolization through the surgically accessed basal vein of Rosenthal. Case Description A 53-year-old man had been referred to our hospital for an aneurysm-like lesion that was compressing the left optic chiasm. A left internal carotid angiogram demonstrated an orbital AVF fed by a distal segment of the ophthalmic arteries and the anterior branch of the inferolateral trunk that was drained solely into a tortuous basal vein of Rosenthal through a bridging vein of the left optic nerve sheath. Considering the risk of hemorrhagic complications during transvenous manipulation and visual complication in cases of transarterial embolization, combined surgical and transvenous embolization was attempted through the translocated basal vein of Rosenthal to bypass the dangerous path to the fistula. The lesion was directly catheterized through the translocated basal vein of Rosenthal after confirming visual tolerance to brief drainage occlusion using visual evoked potential monitoring, resulting in successful fistula obliteration using detachable coils. Conclusion Direct catheterization of the translocated deep draining vein was useful to bypass the dangerous access to the fistula and could be a feasible alternative strategy for treating selected AVFs.

Objectives Septo‐optic dysplasia (SOD) is a clinical syndrome characterized by varying combinations of optic nerve hypoplasia, pituitary gland hypoplasia and abnormal cavum septi pellucidi. It is suspected on prenatal imaging when there is non‐visualization or hypoplasia of the septal leaflets. Long‐term postnatal outcomes of fetuses with prenatally suspected SOD have been documented poorly. The aims of this study were to describe the natural history of deficient septal leaflets, to quantify the incidence of postnatally confirmed SOD and to document the visual, endocrine and long‐term neurodevelopmental outcomes of these infants. Methods This was an observational retrospective study of all fetuses with prenatal imaging showing isolated septal agenesis, assessed at a single tertiary center over an 11‐year period. Pregnancy, delivery and neonatal outcomes and pre‐ and postnatal imaging findings were reviewed. Neonatal evaluations or fetal autopsy reports were assessed for confirmation of SOD. Ophthalmologic, endocrine, genetic and long‐term developmental evaluations were assessed. Imaging findings and outcome were compared between infants with and those without postnatally confirmed SOD. Results Of 214 fetuses presenting with septal absence on prenatal ultrasound and magnetic resonance imaging (MRI), 18 (8.4%) were classified as having suspected isolated septal agenesis suspicious for SOD. Uniform prenatal MRI findings in cases with suspected SOD included remnants of the leaflets of the cavum septi pellucidi, fused forniceal columns, normal olfactory bulbs and tracts and a normal optic chiasm. Twelve fetuses were liveborn and five (27.8%) had postnatally confirmed SOD. Only two of these five fetuses had additional prenatal imaging features (pituitary cyst, microphthalmia and optic nerve hypoplasia) supporting a diagnosis of SOD. The other three confirmed SOD cases had no predictive prenatal or postnatal imaging findings that reliably differentiated them from cases without confirmed SOD. Visual and endocrine impairments were present in two (40%) and four (80%) cases with confirmed SOD, respectively. In those with visual and/or endocrine impairment, developmental delay (median age at follow‐up, 2.5 (interquartile range, 2.5–7.0) years) was common (80%) and mostly severe. Neonates with isolated septal agenesis and a lack of visual or endocrine abnormalities to confirm SOD had normal development. Conclusions Only a quarter of fetuses with isolated septal agenesis suggestive of SOD will have postnatal confirmation of the diagnosis. Clinical manifestations of SOD are variable, but neurodevelopmental delay may be more prevalent than thought formerly. © 2020 Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

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.

Radiotherapy-related visual decline is a significant concern in survivors of childhood cancer; however, data establishing the dose-response relationship between dose to the optic apparatus and visual acuity decline in children are sparse. We aimed to determine this relationship in a cohort of children treated with proton therapy.We identified 458 children with 875 eyes at risk treated with proton therapy for intracranial malignancy between December 2006 and September 2018. Eyes were considered at risk if either the ipsilateral optic nerve or optic chiasm received ≥30 GyRBE to 0.1 cmExcluding children with tumor progression, no patient experienced complete vision loss. The actuarial 5-year rate of any visual acuity decline was 2.6% (95% confidence interval [CI]: 1.5%-4.6%). The dose to 0.1 cmVisual acuity decline following radiotherapy for intracranial malignancies in children is rare. A dose of approximately 56 GyRBE to 0.1 cm

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.

In this report, the authors demonstrated that idiopathic pituitary hyperplasia (PH) can cause complete bitemporal hemianopia and amenorrhea, even in the setting of mild anatomical compression of the optic chiasm and normal pituitary function. Furthermore, complete resolution of symptoms can be achieved with surgical decompression. PH can occur in the setting of pregnancy or end-organ insufficiency, as well as with medications such as oral contraceptives and antipsychotics, or it can be idiopathic. It is often found incidentally, and surgical intervention is usually unnecessary, as the disorder rarely progresses and can be managed by treating the underlying etiology. Here, the authors present the case of a 24-year-old woman with no significant prior medical history, who presented with bitemporal hemianopia and amenorrhea. Imaging revealed an enlarged pituitary gland that was contacting, but not compressing, the optic chiasm, and pituitary hormone tests were all within normal limits. The patient underwent surgical decompression of the sella turcica and exploration of the gland through an endoscopic endonasal transsphenoidal approach. Pathology results demonstrated PH. A postoperative visual field examination revealed complete resolution of the bitemporal hemianopia, and menstruation resumed 3 days later. The patient remains asymptomatic with no hormonal deficits.

Purpose To describe the trans-occipital asymmetries of pattern and flash visual evoked potentials (VEPs), in an infant with MRI findings of unilateral optic nerve aplasia and hemi-chiasm dysplasia. Methods A child with suspected left cystic microphthalmia, left microcornea, left unilateral optic nerve aplasia, and hemi-chiasm underwent a multi-channel VEP assessment with pattern reversal, pattern onset, and flash stimulation at the age of 16 weeks. Results There was no VEP evidence of any post-retinal visual pathway activation from left eye with optic nerve aplasia. The VEP trans-occipital distribution from the functional right eye was skewed markedly across the midline, in keeping with significant misrouting of optic nerve fibres at the chiasm. This was supported by the anatomical trajectory of the optic chiasm and tracts seen on MRI. Conclusion This infant has chiasmal misrouting in association with unilateral optic nerve aplasia and unilateral microphthalmos. Chiasmal misrouting has not been found in patients with microphthalmos or anophthalmos, but has been reported after early eye loss in animal models. Our findings contribute to our understanding of the discrepancy between the visual pathway physiology of human unilateral microphthalmia and animal models.

Objective Thyroid-stimulating hormone (TSH)-secreting pituitary adenoma (PA) is an extremely rare functioning form of PA that accounts for 0.7–2% of all such cases. The previously reported outcomes of the surgical removal of TSH-PA are poor. Owing to its extremely low incidence, most available reports on TSH-PA are case reports or small case series. Thus, we investigated the clinical and endocrinological outcomes of surgically treated TSH-PA through our institutional series. Methods We retrospectively reviewed 14 consecutive cases of surgically treated TSH-PA, focusing on the clinical, radiological, surgical, and endocrinological data. Results There were seven male (50%) and seven female (50%) patients. The mean age was 42.5 years (range, 19–63). The mean tumor size was 16.6 mm (range, 4–30). Optic chiasm compression was noted in six patients (42.9%), and no patient showed cavernous sinus invasion. Thirteen of 14 patients (92.8%) underwent transnasal transsphenoidal approach (TSA), and one patient underwent TSA followed by transcranial approach for residual tumor removal. Thirteen of 14 patients (92.8%) showed endocrinological remission; all patients who experienced remission showed subnormal levels of TSH (

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.