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A preschool girl presented with sudden-onset bilateral painless loss of vision from 2 days prior. Child’s examination showed light perception vision, sluggishly reacting pupils, otherwise normal anterior segment, healthy optic disc and retina in both eyes. MRI of brain and orbit with contrast revealed thickened left part of the optic chiasm with contrast enhancement extending proximally to bilateral optic tract and hyperintensities in the left thalamus and periventricular white mater. Considering the topographical distribution of lesions in the brain, neuromyelitis optica spectrum disorder was suspected. The child was started on intravenous methylprednisolone followed by tapering oral steroids. Serological testing was positive for myelin oligodendrocyte glycoprotein (MOG) and negative for aquaporin-4 antibodies. This case represents an unusual case of MOG associated demyelination disorder where the distribution of lesions showed chiasmal involvement along with optic tract, thalamus and deep white mater lesions.

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

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.

(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.

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.

The purpose of this study was to examine if the use of a bolus helmet when treating the head with intensity modulated proton therapy (IMPT) will maintain organs at risk (OAR) sparing while improving the clinical target volume (CTV) coverage. A bolus helmet is a device that aims to improve on the traditionally used range shifter in proton therapy by improving dose distribution characteristics. Ten patients were retrospectively selected who had 2 separate treatment planning scans performed, a scan with the bolus helmet and a second scan without. Plans were created using both scans. Dose to organs at risk (OAR) including the left optic nerve, right optic nerve, optic chiasm, and normal brain minus CTV (brain-CTV), as well as CTV coverage were compared between the 2 plans. The use of the bolus helmet displayed lower mean OAR doses as well as higher CTV coverage, suggesting that use of the bolus helmet provides benefit when treating the head with IMPT.

Multiple sclerosis (MS) is an inflammatory demyelinating disease leading not only to physical disability but also to cognitive dysfunction. The aim of our study was to test cognitive functions of MS patients with mild relapsing-remitting form and to find out the relationship between cognitive functions and brain volumetry.52 patients (RRMSp) and 23 age-related healthy participants (CON) were enrolled. Mild disability was defined by mean EDSS 2.4 (≤ 4.0), and by median of disease duration 5.2 years. Cognitive status was tested using Single Digit Modality Test (SDMT). Brain volumetry was processed in FreeSurfer 2.0.0.RRMSp patients showed significantly lower SDMT score than CON. SDMT results correlated positively with volume of thalamus, putamen and nc. caudate, and negatively with optic chiasma volume. Compared with CON, RRMSp presented with significantly lower volume in left and right nc. accumbent, cuneus and insular GM, right putamen, total brain cortical grey matter (GM), white matters hypointensities, and 3rd ventricular widths.To our best knowledge, this is the first study that presents results showing a correlation of lower SDMT with higher optic chiasma volume, due to its subclinical chronic demyelination. We confirmed that GM atrophy is involved in cognitive functions in MS (Tab. 3, Fig. 2, Ref. 73).

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.

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.

Purpose Anatomical changes, such as shrinkage and aeration, can affect dose distribution in proton therapy (PT) for maxillary sinus carcinoma (MSC). These changes can affect the dose to the target and organs at risk (OARs); however, when these changes occur during PT is unclear. This study aimed to investigate the dosimetric impact of anatomical changes during PT. Materials and Methods Fifteen patients with MSC were enrolled in this study. Initial PT plans were generated based on initial computed tomography (CT) images. Several repeat CT images were obtained to confirm anatomical changes during PT. Evaluation PT plans were generated by copying initial PT plans to repeat CT images. The dose differences of the target and OARs were evaluated by comparing both the plans. Results At 3–4 weeks after the initiation of PT, the target volume reduced by approximately 10% as compared with the initial volume. Consequently, the target volumes gradually varied until the end of treatment. The value of V95 (volume that received 95% of the prescription dose) in the clinical target volume of the evaluation PT plan was similar to that of the initial PT plan. However, the dose to OARs, such as the contralateral optic nerve, contralateral eyeball, brainstem, and optic chiasm, increased significantly from the middle to the later phases of the treatment course. In contrast, there was a slight dose difference in the ipsilateral optic apparatus. Conclusion The trend analysis in this study showed that anatomical changes appeared 3–4 weeks after the start of PT, and the dose to the OARs tended to increase. Therefore, it is recommended to check the status of tumor 3–4 weeks after the start of treatment to avoid the deterioration of dose distribution due to these changes.

The link between ancient Greek medicine and the Arabic translation period in the 9th century cannot be understood without studying the contributions of Syriac scholars. With their mastery of Greek and the related Semitic languages of Syriac and Arabic, they initiated a scientific translation process with methods that prevail to this day. In this paper, we reviewed Hunayn Ibn Isshaq's Ten Treatises on the Eye to elucidate the original contributions of the Syriac physicians to the field of neurologic surgery. We analyzed the oldest known diagram of orbital anatomy along with Hunayn's genuine ideas on the optic nerve anatomy and pathology, optic chiasm, afferent pupillary reflex, and papilledema and venous congestion. We also reviewed the neurosurgical elements found in the Syriac Book of Medicines including the thought process in localizing neurologic deficits based on clinical experience and anatomic dissections and the earliest recorded description of brachial plexus pathology.

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.

The optic nerve is known to be one of the largest nerve bundles in the human central nervous system. There have been many studies of optic nerve imaging and post-processing that have provided insights into pathophysiology of optic neuritis related to multiple sclerosis and neuromyelitis optica spectrum disorder, glaucoma, and Leber's hereditary optic neuropathy. There are many challenges in optic nerve imaging, due to the morphology of the nerve through its course to the optic chiasm, its mobility due to eye movements and the high signal from cerebrospinal fluid and orbital fat surrounding the optic nerve. Recently, many advanced and fast imaging sequences have been used with post-processing techniques in attempts to produce higher resolution images of the optic nerve for evaluating various diseases. Magnetic resonance imaging (MRI) is one of the most common imaging methodologies for the optic nerve. This review paper will focus on recent MRI advances in optic nerve imaging and explain several post-processing techniques being used for analysis of optic nerve images. Finally, some challenges and potential for future optic nerve studies will be discussed.

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.

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.

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.

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.

Examining individuals with Leber's hereditary optic neuropathy (LHON) provides a rare opportunity to understand how changes in mitochondrial DNA and loss of vision can be related to changes in organization of the whole-brain structural network architecture. In comparison with the previous neuroimaging studies with LHON participants, which were focused mainly on analyzing changes which occur in different areas of the patient's brain, network analysis not only makes it possible to observe single white matter fibers' aberrations but also the whole-brain nature of these changes. The purpose of our study was to better understand whole-brain neural network changes in LHON participants and see the correlation between the clinical data and the changes. To achieve this, we examined fifteen LHON patients and seventeen age-matched healthy subjects with the usage of ultra-high filed 7T magnetic resonance imaging (MRI). Basing on the analysis on MRI diffusion tensor imaging (DTI) data, whole-brain structural neural networks were reconstructed with the use of the minimum spanning tree algorithm (MST) for every participant. Our results revealed that the structural network in LHON participants was altered at both the local and the global level. The global network structures of LHON subjects were less centralized with path-like organization and there was an imbalance in the main hub centrality. Moreover, the inspection of nodes and hubs in terms of their anatomical placement revealed that in the LHON participants the prominent hubs were located within the basal ganglia (i.e. bilateral caudate, left pallidum), which differed them from healthy controls. An analysis of the relationships between the global MST metrics and LHON participants' clinical characteristics revealed significant correlations between the global network metrics and the duration of illness. Furthermore, the nodal parameters of the optic chiasm were significantly correlated with the duration of illness and the averaged thickness of the right retinal nerve fiber layer (RNFL). These findings clearly showed that the progression of the disease is accompanied by alterations within the brain network structure and its efficiency.

PurposeCritical flicker fusion frequency (CFF) is a short but sensitive method for evaluating optic nerve function. We measured CFF in patients with pituitary neuroendocrine tumors (Pit-NETs) to assess its usefulness.MethodsData from 184 patients with nonfunctioning Pit-NETs, who had been treated with transsphenoidal surgery and had no medical history of eye diseases, was used in this retrospective study. Visual acuity decline (VAD) was defined as > 0.10 reduction in logMAR visual acuity and CFF decline (CFD) was defined as CFF value ResultsContact between the optic nerve or chiasma and the tumor was present and absent in 161 and 23 patients, respectively. In patients showing contact, the difference in CFF between the left and right eyes was larger (P = 0.0008), and the optimal cut-off value using the receiver operating characteristic curve was 3 Hz. Therefore, ≥ 3 Hz was considered positive for CFF laterality (CFL), the most prevalent condition. Tumor height was lower in patients with CFL positivity compared to those with VAD or VFD (P ConclusionChanges in CFL permit early detection of Pit-NETs. Our results indicate that CFF laterality can be seen in the early stages of compressive optic neuropathy due to Pit-NET.

[Background] Surgical decompression to the optic-chiasmatic region in craneofacial fibrous dysplasia (CFD) must be performed safely to improve or stabilize visual loss., [Method] We describe a technical nuance when facing on a huge, deformed skull with potentially imbricated dura mater. Craniectomy was performed in concentric arches allowing to expose surgical field and elevated step by step. Bilateral micro-decompression was performed after without difficulties., [Conclusions] Decompressing both optic nerves using this technique is safe and relatively simple to perform.

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.

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.

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.

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.

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.

The visual system, consisting of the eyes and the visual pathways of the brain, receives and interprets light from the environment so that we can perceive the world around us. A wide variety of disorders can affect human vision, ranging from ocular to neurologic to systemic in nature. While other noninvasive imaging techniques such as optical coherence tomography and ultrasound can image particular sections of the visual system, magnetic resonance imaging (MRI) offers high resolution without depth limitations. MRI also gives superior soft-tissue contrast throughout the entire pathway compared to computed tomography. By leveraging different imaging sequences, MRI is uniquely capable of unveiling the intricate processes of ocular anatomy, tissue physiology, and neurological function in the human visual system from the microscopic to macroscopic levels. In this review we discuss how structural, metabolic, and functional MRI can be used in the clinical assessment of normal and pathologic states in the anatomic structures of the visual system, including the eyes, optic nerves, optic chiasm, optic tracts, visual brain nuclei, optic radiations, and visual cortical areas. We detail a selection of recent clinical applications of MRI at each position along the visual pathways, including the evaluation of pathology, plasticity, and the potential for restoration, as well as its limitations and key areas of ongoing exploration. Our discussion of the current and future developments in MR ocular and neuroimaging highlights its potential impact on our ability to understand visual function in new detail and to improve our protection and treatment of anatomic structures that are integral to this fundamental sensory system. LEVEL OF EVIDENCE 3: TECHNICAL EFFICACY STAGE 3: .

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.

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.

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.

BACKGROUND Investigation of visual field defects (VFDs) is important to decide the treatment and to predict the prognosis in patients with a pituitary mass. The aim of this study was to evaluate the correlation among 2 diagnostic modalities-MRI and optical coherence tomography (OCT)-and VFDs. METHODS Consecutive patients who showed the presence of a pituitary mass on MRI and in whom ophthalmic examinations were performed were recruited. Height and volume of the mass, sagittal and coronal displacement of optic chiasm, and the direction of mass expansion were measured. Patients were divided into 2 groups according to the presence (VFD group) or absence of VFDs (no VFDs group [NVFD]). The correlation among MRI parameters, OCT parameters, and VFDs were examined, and the diagnostic values of MRI and OCT and the combined value of the 2 modalities were analyzed. RESULTS Forty-one patients were included. The greatest coefficients of determination were observed between the sagittal displacement and pattern standard deviation (PSD) (R2 = 0.3661, P < 0.001) and between the inferonasal ganglion cell-inner plexiform layer (GCIPL) and PSD (R2 = 0.4079, P < 0.001). The height and the size of the mass in the VFD group were significantly greater as 57% and 148%, respectively, and the VFD group had more severe chiasmal displacement both in the sagittal (165%) and in the coronal (178%) plane (large effect in all). All macular GCIPLs were thinner in the NVFD group (range 9%-26%, large effect), whereas only temporal (25%) and average (11%) values were among peripapillary retinal nerve fiber layers. CONCLUSIONS The highest correlations with the degree of the VFD were seen in the sagittal displacement of optic chiasm and the inferonasal GCIPL, and these parameters were correlated concurrently. Both modalities showed a good diagnostic value for discriminating VFDs.

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.