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Frontiers in Surgery 2022Hemorrhage into optic pathway-hypothalamic glioma (OPHG) is rare. Variable clinical presentations and outcomes are associated with such pathology. We aim to present two... (Review)
Review
BACKGROUND
Hemorrhage into optic pathway-hypothalamic glioma (OPHG) is rare. Variable clinical presentations and outcomes are associated with such pathology. We aim to present two infants presented with OPHG and a systematic review of the literature.
METHODS
We describe two cases of infants presenting with sudden decreased vision, poor feeding, and irritability due to OPHG. Both patients underwent urgent craniotomy and subtotal resection followed by chemotherapy. We systematically reviewed the literature using PubMed, Google Scholar, and Embase. In addition, we included all English published reports for all ages discussing the optic pathway (optic nerve and optic chiasm) or hypothalamic glioma associated with hemorrhage from the year of the first reported case (1970) to January 2022.
RESULTS
Of 17,949, 44 articles met the inclusion criteria of this review. A total of 56 cases were described with a mean of 21.35 years (0.5-70), with the male gender 52% and the female gender 45%. The hemorrhage location was sellar/suprasellar in 43% cases. Histopathology of included cases was pilocytic astrocytoma in 41%, followed by pilomyxoid astrocytoma in 16% cases. The outcome was unfavorable; 37.5% cases showed improvement, whereas 18% cases resulted in death.
CONCLUSION
Apoplexy of the OPHG can be fatal and associated with poor outcomes. A systematic review of the literature has shown that younger age, pilocytic or pilomexyoid astrocytoma histopathology, and chiasmal/hypothalamic locations are associated with a higher risk of intertumoral hemorrhage and poor prognosis. Further genetic studies for OPHG may provide information for high-risk patients.
PubMed: 35733436
DOI: 10.3389/fsurg.2022.891556 -
Frontiers in Medicine 2022Optic tract lesions (OTL) are often difficult to diagnose. We suggest an algorithm to simplify the often-challenging diagnosis of OTL. Clinical and imaging data were...
Optic tract lesions (OTL) are often difficult to diagnose. We suggest an algorithm to simplify the often-challenging diagnosis of OTL. Clinical and imaging data were retrospectively collected from the electronic files of 6 patients diagnosed with OTL at a tertiary medical center in 2016-2020. The series included 4 children and 2 adults with an OTL caused by a glioma ( = 5) or motor vehicle accident ( = 1). Magnetic resonance imaging (MRI) revealed a suprasellar glioma involving the chiasm and tract alone (n = 1) and the ipsilateral optic nerve (n = 2) and only optic tract (3). Perimetry showed incongruent homonymous hemianopia in 3 patients. In two patients, perimetry could only be performed in one eye, and demonstrated hemianopia. In one patient perimetry was unreliable. Fundus examination revealed bow-tie atrophy in all patients. On optical coherence tomography (OCT) of the peripapillary retinal nerve fiber layer (RNFL) horizontal thinning was observed in the contralateral eye ( = 6). By presenting the information in a predefined order-visual field damage, OCT RNFL thickness, and MRI-the diagnosis could be easily reached even in children, and when other structures like the chiasm were involved. Fundus photographs easily detect bow tie atrophy in children. Systematic presentation of the data in a predefined order can ease the diagnostic process of OTLs.
PubMed: 36388882
DOI: 10.3389/fmed.2022.1029829 -
American Journal of Ophthalmology Case... Mar 2020To present a case of glioblastoma multiforme which initially presented with only ophthalmic manifestations.
PURPOSE
To present a case of glioblastoma multiforme which initially presented with only ophthalmic manifestations.
OBSERVATIONS
A 48-year-old man presented with decreased vision and pain with eye movements of the right eye. MRI of the brain showed increased T2/FLAIR signal involving the right optic nerve with no other identified abnormalities. He was treated with intravenous steroids for presumed optic neuritis. His visual acuity then rapidly worsened to no light perception, with new orbital apex symptoms including central retinal artery and vein occlusions and inferior division third and fourth nerve palsies. Repeat MRI with contrast showed perineural enhancement surrounding the right optic nerve and markedly reduced diffusion along its entire course. After an unrevealing initial workup and then a 7 month period during which the patient refused follow up, he re-presented with left sided weakness, headache, and confusion. Repeat brain MRI revealed a large mass involving the right optic nerve, optic chiasm, basal ganglia, corpus callosum and brainstem. Biopsy led to a diagnosis of WHO grade IV glioblastoma multiforme. The disease was poorly responsive to temozolomide, bevacizumab and external beam radiation, and the patient passed away 5 months later.
CONCLUSIONS AND IMPORTANCE
Malignant optic glioma of adulthood is a challenging diagnosis with a poor prognosis. This rare case highlights the importance of maintaining neoplasm in the differential for optic neuritis masqueraders.
PubMed: 32395666
DOI: 10.1016/j.ajoc.2020.100594 -
Case Reports in Ophthalmology 2022A 29-year-old girl presented complaining of acute bilateral visual loss associated with mild headache and retrobulbar pain. She was diagnosed with chiasmal optic...
A 29-year-old girl presented complaining of acute bilateral visual loss associated with mild headache and retrobulbar pain. She was diagnosed with chiasmal optic neuritis caused by multiple sclerosis. Her visual acuity and visual field defect promptly improved after steroid therapy. However optical coherence tomography angiography showed a progressive reduction of superficial capillary plexus density of the retina and optic nerve consistent with the progressive impairment of the retinal ganglionar cell layer. Contrary to chiasmal compression, in chiasmal optic neuritis, the superficial capillary plexus density reduction is diffuse and does not reflect the peculiar anatomy of the chiasm.
PubMed: 35950028
DOI: 10.1159/000525465 -
Clinical Ophthalmology (Auckland, N.Z.) 2022Albinism describes a heterogeneous group of genetically determined disorders characterized by disrupted synthesis of melanin and a range of developmental ocular... (Review)
Review
Albinism describes a heterogeneous group of genetically determined disorders characterized by disrupted synthesis of melanin and a range of developmental ocular abnormalities. The main ocular features common to both oculocutaneous albinism (OCA), and ocular albinism (OA) include reduced visual acuity, refractive errors, foveal hypoplasia, congenital nystagmus, iris and fundus hypopigmentation and visual pathway misrouting, but clinical signs vary and there is phenotypic overlap with other pathologies. This study reviews the prevalence, genetics and ocular manifestations of OCA and OA, including abnormal development of the optic chiasm. The role of visual electrophysiology in the detection of chiasmal dysfunction and visual pathway misrouting is emphasized, highlighting how age-associated changes in visual evoked potential (VEP) test results must be considered to enable accurate diagnosis, and illustrated further by the inclusion of novel VEP data in genetically confirmed cases. Differential diagnosis is considered in the context of suspected retinal and other disorders, including rare syndromes that may masquerade as albinism.
PubMed: 35637898
DOI: 10.2147/OPTH.S329282 -
Cell Reports Aug 2023The balance of contralateral and ipsilateral retinogeniculate projections is critical for binocular vision, but the transcriptional programs regulating this process...
The balance of contralateral and ipsilateral retinogeniculate projections is critical for binocular vision, but the transcriptional programs regulating this process remain ill defined. Here we show that the Pou class homeobox protein POU3F1 is expressed in nascent mouse contralateral retinal ganglion cells (cRGCs) but not ipsilateral RGCs (iRGCs). Upon Pou3f1 inactivation, the proportion of cRGCs is reduced in favor of iRGCs, leading to abnormal projection ratios at the optic chiasm. Conversely, misexpression of Pou3f1 in progenitors increases the production of cRGCs. Using CUT&RUN and RNA sequencing in gain- and loss-of-function assays, we demonstrate that POU3F1 regulates expression of several key members of the cRGC gene regulatory network. Finally, we report that POU3F1 is sufficient to induce RGC-like cell production, even in late-stage retinal progenitors of Atoh7 knockout mice. This work uncovers POU3F1 as a regulator of the cRGC transcriptional program, opening possibilities for optic nerve regenerative therapies.
PubMed: 37590135
DOI: 10.1016/j.celrep.2023.112985 -
Neurology India 2022
Topics: Antitubercular Agents; Ethambutol; Humans; Optic Chiasm; Optic Nerve; Optic Nerve Diseases
PubMed: 36076719
DOI: 10.4103/0028-3886.355154 -
International Journal of Particle... 2021Periorbital tumor location presents a significant challenge with 3-dimensional conformal radiation therapy or intensity modulated radiation therapy due to high tumor...
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.
PubMed: 33829072
DOI: 10.14338/IJPT-20-00025.1 -
Neurosurgery Jan 2023Compression of the anterior visual pathways by sellar and parasellar masses can produce irreversible and devastating visual loss. Optical coherence tomography (OCT) is a... (Review)
Review
Compression of the anterior visual pathways by sellar and parasellar masses can produce irreversible and devastating visual loss. Optical coherence tomography (OCT) is a noninvasive high-resolution ocular imaging modality routinely used in ophthalmology clinics for qualitative and quantitative analysis of optic nerve and retinal structures, including the retinal ganglion cells. By demonstrating structural loss of the retinal ganglion cells whose axons form the optic nerve before decussating in the optic chiasm, OCT imaging of the optic nerve and retina provides an excellent tool for detection and monitoring of compressive optic neuropathies and chiasmopathies due to sellar and parasellar masses. Recent studies have highlighted the role of OCT imaging in the diagnosis, follow-up, and prognostication of the visual outcomes in patients with chiasmal compression. OCT parameters of optic nerve and macular scans such as peripapillary retinal nerve fiber layer thickness and macular ganglion cell thickness are correlated with the degree of visual loss; additionally, OCT can detect clinically significant optic nerve and chiasmal compression before visual field loss is revealed on automated perimetry. Preoperative values of OCT optic nerve and macular parameters represent a prognostic tool for postoperative visual outcome. This review provides a qualitative analysis of the current applications of OCT imaging of the retina and optic nerve in patients with anterior visual pathway compression from sellar and parasellar masses. We also review the role of new technologies such as OCT-angiography, which could improve the prognostic ability of OCT to predict postoperative visual function.
Topics: Humans; Tomography, Optical Coherence; Optic Nerve Diseases; Retinal Ganglion Cells; Optic Chiasm; Optic Nerve; Vision Disorders
PubMed: 36519859
DOI: 10.1227/neu.0000000000002186 -
Cureus Mar 2023Craniopharyngiomas are rare, solid or mixed solid, cystic tumors that generally occur in the sellar and suprasellar regions of the central nervous system along the...
Craniopharyngiomas are rare, solid or mixed solid, cystic tumors that generally occur in the sellar and suprasellar regions of the central nervous system along the pituitary stalk near the optic chiasm. Although benign, patients clinically present with symptoms from mass effect or the resultant increase in intracranial pressures. The recurrence rate of craniopharyngiomas is high. When present, recurrence is typically at the site of the primary tumor bed. Here, we present a case of a 27-year-old male who was found to have a suprasellar mass on enhanced CT imaging. The mass was resected and a ventriculoperitoneal shunt was placed. The lesion was identified as a craniopharyngioma, and the patient completed treatment and was discharged appropriately. Ten years later, the patient presented with headaches and on enhanced CT imaging was found to have a radiopaque mass originating from the right lateral ventricle and extending into the splenius of the corpus callosum. This new lesion was identified as a craniopharyngioma. Ectopic recurrence of a craniopharyngioma is a rare phenomenon and is thought to be the result of direct seeding along the site of surgical resection or via cerebrospinal fluid circulation.
PubMed: 37041897
DOI: 10.7759/cureus.35988