Visual Outcomes Following Surgical Debulking in Exophytic Optic Nerve Sheath Meningiomas.

PURPOSE: To report a case of a globular primary optic nerve sheath meningioma managed surgically with improvement in vision and review the literature for outcomes of purely intraorbital exophytic-globular primary optic nerve sheath meningiomas managed surgically. METHODS: A literature review was conducted using Google Scholar and PubMed with the search terms "primary optic nerve sheath meningioma," "surgery," "exophytic," and "globular." Articles were included if they were available in English. Individual cases from the reviewed articles were included if the tumor was purely intraorbital with a globular or exophytic morphology, was managed with total or subtotal surgical excision, and visual outcomes were reported. Cases were excluded if the tumor extended intracanalicularly or intracranially, tumor morphology was unknown, or surgical management consisted of biopsy, optic nerve sheath decompression, or optic canal decompression rather than tumor debulking. RESULTS: A total of 28 patients with intraorbital globular-exophytic primary optic nerve sheath meningiomas managed surgically have been reported in the literature. Vision improved in 29% (n = 8/28) and remained stable in 43% (n = 12/28) of patients. Furthermore, patients with good (Snellen notation ≥ 0.5) vision (n = 10) typically retained good vision postoperatively and at follow-up, with 1 patient experiencing a decline to poor (Snellen ≤0.1) vision at the last follow-up (92 months postoperatively). Similarly, patients with fair (Snellen notation >0.1 and <0.5) vision (n = 5) often improved to good vision (n = 3) or stayed at fair vision (n = 1), with 1 declining to poor vision at postoperative hospital discharge. CONCLUSIONS: Surgical management of exophytic or globular optic nerve meningiomas does not universally lead to vision loss and may be appropriate in select patients.

A Patient With Exophytic Primary Optic Nerve Sheath Meningioma: Stratified Risk of Surgery Based on the Morphology.

Primary optic nerve sheath meningioma (pONSM) is one of the most challenging tumors to manage. Although surgical excision may be considered a treatment option to maintain visual function, the safety of surgery remains debatable due to the non-negligible risk of optic nerve injury. pONSM often grows concentrically around the optic nerve; however, it can also demonstrate an exophytic growth from the optic nerve. The risk of surgical excision of pONSM may vary based on the tumor's growth pattern and area of contact with the optic nerve; however, there has been no detailed report on risk stratification to date. The authors present an illustrative case of an exophytic pONSM which was removed surgically without complications, suggesting that tumor morphology may also influence the surgical risk. In this report, the imaging and intraoperative features of exophytic pONSM are presented in detail, and the risk factors for complications are discussed.

Radiation optic neuropathy and retinopathy in patients with presumed benign intraorbital tumours treated with fractionated stereotactic radiotherapy.

PURPOSE: To assess the long-term effects of fractionated stereotactic radiotherapy (fSRT) for the treatment of (presumed) benign intraorbital tumours on visual acuity, visual fields, globe and eyelid position, and complications including radiation retinopathy and deviations of retinal nerve fibre layer (RNFL) on OCT. METHODS: Multi-centre retrospective follow-up study of a consecutive series of 25 patients treated in the Rotterdam Orbital Center (collaboration between Erasmus Medical Center and Rotterdam Eye Hospital) between 2002 and 2018. Data on the dose of fSRT, visual acuity, Humphrey field analyser (HFA) perimetry, globe and eyelid position were obtained from the medical records. RESULTS: In this retrospective consecutive series of 25 patients with a median follow-up of 104 months (range 48-215 months), 80.0% of the patients had presumed optic nerve sheath meningioma and 20.0 % presumed cavernous haemangioma with signs suggestive of recent growth. In most patients, a better visual acuity and RNFL thickness were observed after stereotactic radiotherapy. Improvement of the visual field defects was observed after treatment, with a mean deviation of -14.98 dB (12.9 SD) before treatment versus -4.56 dB (10.8 SD) after treatment, respectively. Significant, but small changes in exophthalmometry values were observed with a mean of 14.92 mm (7.9 SD) versus 13.79 mm (7.3 SD) after treatment, respectively. Only 3 patients (15.0%) developed radiation retinopathy after stereotactic radiotherapy. All patients with radiation retinopathy had presumed optic nerve sheath meningioma. CONCLUSIONS: Based on our results, fSRT is an effective treatment modality for this subset of orbital tumours with few complications and good long-term visual and cosmetic outcomes. FSRT is non-invasive and safer than surgery for lesions in the posterior orbit or around the optic nerve.

Radiotherapy and Radiosurgery in the Management of Optic Nerve Sheath Meningiomas: An International Systematic Review and Meta-Analysis of Twenty Studies.

BACKGROUND: Optic nerve sheath meningiomas (ONMs) are often managed with radiotherapy (RT) with the goal of achieving radiographic local control (LC) and preventing deterioration of visual acuity (VA). We aimed to perform a systematic review and meta-analysis of outcomes for patients with ONM treated with RT. METHODS: The PICOS/PRISMA/MOOSE selection criteria were used to identify studies. Primary outcomes were stable or improved VA and radiographic LC at last follow-up. The secondary outcomes were incidences of radiation-induced retinopathy and xerophthalmia and stable or improved visual fields (VFs). Weighted random-effects meta-analyses using the DerSimonian and Laird methods were conducted to characterize effect sizes. Mixed-effects regression models were used to examine potential correlations between gross tumor volume (GTV) and outcomes. RESULTS: In total, 444 patients with ONM across 20 published studies were included. The estimated LC rate was 99.8% (95% confidence interval [CI], 98.3%-100%), and the estimated proportion of patients with stable or improved VA or VF was 89.7% (95% CI, 86.2%-92.4%) and 93.3% (95% CI, 89.5%-95.8%), respectively. Estimated incidences of radiation-induced retinopathy and xerophthalmia were 7.2% and 10.1%, respectively. GTV was significantly associated with VA (P = 0.014) with estimated VA rates of 96.4%, 91.4%, and 80.5% for GTVs of 2.0, 3.0, and 4.0 cm3, respectively. CONCLUSIONS: RT was well tolerated, with excellent LC achieved. Nearly 90% of patients noted either stability or improvement in VA and VF. Larger ONMs were associated with poorer VA.

Awake Microsurgical Resection for Optochiasmatic Cavernous Malformation.

Cavernous malformations (CM) affect approximately 0.5% of the population, with only a limited portion being located in the optic nerve and chiasma. The clinical presentation is determined by their locations. In the optochiasmatic CM, the acute visual disturbance is the most common presentation. Chronically, many show a progressive visual loss, chronic headache, and pituitary disturbances. The differential diagnosis includes optic glioma, arteriovenous malformations, aneurysm, craniopharyngioma, pituitary apoplexy, and inflammatory conditions. In Video 1, we present the case of a 39-year-old woman with a history of a hemorrhagic optochiasmatic cavernoma in 2016, who started using propranolol to reduce the lesion and symptoms of visual loss. Moreover, the first microsurgical resection of the cavernoma and evacuation of the hematoma were performed in the same year. Owing to evolvement from a partial to a total vision loss in the left eye and presentation of new symptoms in the right eye, the patient underwent microsurgical resection. The surgery was performed sequentially. An awake craniotomy was performed to monitor the chiasma and right optic nerve. The postoperative magnetic resonance imaging showed complete resection of the CM, and the patient fully recovered. The patient signed the institutional consent form, stating that he or she accepts the procedure and allows the use of his or her images and videos for any type of medical publications in conferences and/or scientific articles.

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

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

Sarcoidosis Mimicking Skull Base Meningioma

Sarcoidosis is a systemic disease characterized by granulomatous inflammation. Pulmonary and lymphatic granulomatous involvement are common.We present a rare case report of involvement of the central nervous system affecting the ocular region and mimicking optic nerve sheath meningioma. We report the case of a 79-year-old female patient with progressive visual impairment with an evolution of 4 years. Amagnetic resonance imaging scan of the cranium with gadolinium and intense homogeneous contrast enhancement revealed an expansive lesion in the right optic nerve, at the height of the optic canal. The patient was submitted to the neurosurgical approach with lesion biopsy, which showed sarcoidosis of the central nervous system. Due to the rarity of central nervous system involvement, the diagnosis of this pathologymay unfortunately be postponed. The present article aims to elucidate this pathology as a differential diagnosis of retro-orbital tumors.

Demographic and prognostic factors of optic nerve astrocytoma: a retrospective study of surveillance, epidemiology, and end results (SEER).

BACKGROUND: Optic nerve astrocytomas (ONAs) are neurological neoplasms in the central nervous system (CNS), and they have the highest incidence rate among all the tumor types in the visual pathway. In this study, we conducted a Surveillance, Epidemiology, and End Results (SEER) -based research to explore the demographic, survival, and prognostic factors of patients diagnosed with ONAs. METHODS: Utilizing the SEER database, we retrospectively evaluated data of patients diagnosed with ONAs of all ages from 1984 to 2016. We used the Student's t distribution to test variables of patients and various characteristics, and Kaplan-Meier curve to illustrate overall survival (OS) with 95.0% confidence intervals (CIs). We also performed univariate and multivariate analyses to evaluate various variables' validity on overall survival. RESULTS: A total of 1004 cases were analyzed, and revealed that age (P<0.001, hazard ratio (HR) = 8.830, 95% CI: 4.088-19.073), tumor grade (P<0.001, HR = 1.927, 95% CI: 1.516-2.450), diagnostic confirmation (P<0.001, HR = 2.444, 95% CI: 1.632-3.660), and histology type (P = 0.046, HR = 1.563, 95% CI: 1.008-2.424) of the tumor were associated with decreased survival. CONCLUSIONS: From this large, comparative study of ONAs, we found that younger age may be considered as a protective indicator, while high-grade astrocytic tumors have a worse prognosis. We also found that diagnostic confirmation and tumor grade were independent prognostic factors in this patient population.

Surgical Treatment of Bilateral Optic Nerve Sheath Meningioma: 2-Dimensional Operative Video.

Even though intracranial meningiomas commonly invade the optic canals, true optic nerve sheath meningiomas are extremely rare. They are insidious lesions that frequently grow in 4 stages, leading to progressive visual loss.1-4 Frequently, management includes observation for asymptomatic patients, and fractioned stereotactic radiotherapy with progressive visual loss.1,3 However, surgery is avoided due to the risk of perioperative visual loss.2-4 However, many of these cases present in the early stage of tumor growth, where an arachnoidal plane is still present, and vision can be saved by microsurgical tumor resection.2 Here we present the case of a 33-yr-old female presenting with a 3-wk history of blurred vision. Neuroimaging depicted an enhancing lesion along both optic nerves consistent with bilateral optic nerve sheath meningioma. Neuroophthalmological examination showed inferior cut of visual field bilaterally, more extensive on the left eye. Acuity was 20/20 on both eyes. Microsurgical resection of the tumor was performed through left supraorbital craniotomy, with the goal of eliminating intracranial extension and stabilizing visual function.5 The optic canal was unroofed with diamond bit drill under copious irrigation. A remarkable improvement of her visual field was observed with maintenance of acuity at 20/20 on follow-up. As the patient is attempting pregnancy, she is closely monitored without radiation. Surgery can be offered as a primary treatment of optic nerve sheath meningiomas, especially in early stages, with likely preservation and, in some cases, improvement of visual function.6 The patient consented to the procedure and the use of image. Images at 1:46 and 3:48 reprinted from Al-Mefty O, Operative Atlas of Meningiomas. 1998: Raven Press; Philadelphia, PA, with permission from LWW. Image at 2:57 reprinted from Rassi et al2; © Anil Can, 2018, used with permission. Image at 9:27 in public domain/age.

Minipterional Transsylvian Approach for Resection of a Cavernous Malformation in the Optic Chiasm.

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

Opto-chiasmatic apoplexy as a compartment syndrome? Anatomical and surgical considerations on two bleeding cavernous malformations.

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

OPTIC DISK ASTROCYTOMA UNASSOCIATED WITH TUBEROUS SCLEROSIS COMPLEX MANAGED WITH SURGICAL EXCISION AND A 7-YEAR FOLLOW-UP.

PURPOSE: Optic disk astrocytoma is a rare variant of congenital hamartoma, which is usually associated with tuberous sclerosis complex. Generally, it is stationary but in rare cases, it can show progressive growth and cause severe local complications. METHODS: Clinical-pathologic correlation. RESULTS: A 10-year-old boy presented a unilateral optic disk mass with vitreous hemorrhage that eventually necessitated vitrectomy of the affected eye. Visual acuity was hand movements at presentation. Pars plana vitrectomy was performed because of sudden loss of vision. At the time of vitrectomy, a yellow tumor that measured 1.5 mm in basal diameter and 6 mm in thickness was surgically removed. Cytological analysis revealed benign giant cells. The cells were negative for HMB-45 but stained positively for glial fibrillary acidic protein and neuron-specific enolase compatible with an astrocytic lesion. At the follow-up of 7 years, his vision was 20/80 and there has been no recurrence of the tumor. CONCLUSION: Optic disk astrocytoma can be managed with local surgical excision and vision can be preserved if treated timely. Histopathologic features can help evaluate the degree of nuclear pleomorphism and cell morphology. Special immunohistochemical studies of the tumor's coexpressed neuronal markers will help differentiate astrocytoma from other neoplasms.

Optic pathway tumor in children: Toward a new classification for neurosurgical use.

OBJECT: Optic pathway tumors (OPT) represent a challenge for pediatric neurosurgeons. Role of surgery is debated due to the high risk of iatrogenic damage, and in lasts decades it lost its importance in favor of chemotherapy. However, in some cases surgery is necessary to make biomolecular and histological diagnosis, to manage intracranial hypertension (IH) and to cooperate with medical therapies in controlling tumor relapse. With the aim to standardize selection of surgical OPT cases, we propose a simple, practical and reproducible classification. METHODS: We retrospectively analyzed data of 38 patients with OPT treated at our institution (1990-2018). After careful analysis of MRI images, we describe a new classification system. Group 1: lesion limited to one or both optic nerve(s). Group 2: chiasmatic lesions extending minimally to hypothalamus. Group 3: hypothalamo-chiasmatic exophitic lesions invading the third ventricle; they can be further divided on the base of concomitant hydrocephalus. Group 4: hypothalamo-chiasmatic lesions extending widely in lateral direction, toward the temporal or the frontal lobes. Patients' data and adopted treatment are reported and analyzed, also depending on this classification. RESULTS: Twenty children were operated on for treatment of OPT during the study period. Permanent clinical impairment was noted in 5 (25%) of operated patients, while visual improvement was noted in 1 patient. OS rate was 100% at 5 years, with a median follow up of 9 years (ranging from 2 to 23). Prevalence of intracranial hypertension and proportion of first-line surgical treatment decision were significantly higher in groups 3-4 compared to groups 1-2 (P<0.001 for both tests). CONCLUSION: Surgery can offer a valuable therapeutic complement for OPT without major risk of iatrogenic damage. Surgery is indispensable in cases presenting with IH, as in groups 3 and 4 lesions. Eligibility of patients to surgery can be based on this new classification system.

Cavernous malformation of the intracranial optic nerve with operative video and review of the literature.

Optic pathway cavernous malformations represent less than 1% of all central nervous system cavernomas. They can lead to visual loss with indeterminate speed, and therefore, the timing of intervention is controversial. We present a patient with an optic nerve cavernoma, which was discovered incidentally 3 years before the onset of visual symptoms. The evolution of her symptoms, visual function and radiographic findings are reported in detail. The cavernoma was eventually removed via a transciliary orbitocranial keyhole approach with the goal to protect the optic chiasm from progressive involvement. The function in the affected optic nerve was not salvageable. This is the second reported case of a cavernoma selectively involving the intracranial portion of the optic nerve. The debate on the timing of intervention is highlighted with reference to the natural history of these rare lesions.

Microneurosurgical treatment under the guidance of neuroendoscopy for an intracranial optic nerve Schwannoma: A case report.

RATIONALE: Optic Schwannoma is rarely observed clinically as optic nerve had anatomically impossibility for the location of Schwannoma. However, several reports described the established cases of optic Schwannoma, of which the locations were in orbit or within optic canal. The occurrence of optic Schwannoma intracranially has been not reported. PATIENT CONCERNS: A 60-year-old female complained of visual impairment in the right eye and the frequent headache and the dizziness over 2 years with unknown reasons. The result of magnetic resonance imaging (MRI) showed a round, well-circumscribed, heterogeneously enhancing signal with cystic change displayed on the right suprasellar cistern. DIAGNOSES: Intracranial optic Schwannomas. INTERVENTIONS: The patient accepted microneurosurgery assisted by endoscopy. We observed a gray and yellow lesion located near the right anterior clinoid process with a mid-sized cyst. And there was a vague boundary between the tumor and the right optic nerve which was compressed by the tumor. Optic chiasm and left optic nerve were also compressed. Meanwhile, the tumor had also adhesion to the right anterior cerebral artery (ACA). OUTCOMES: After the tumor was totally resected, the patient had satisfactory recovery. LESSONS: We reported an intracranial optic Schwannoma removal with the lateral supraorbital keyhole approach assisted by neuroendoscopy. Intracranial optic Schwannoma was rarely seen clinically. Neuroendoscopy imaging suggested the close relationship between the tumor and ACA, supporting vasculature-origin hypothesis for the optic Schwannoma.

Surgical treatment of the cancer of the optic nerve and orbit. An historical overview.

Introduction: Aim of the present study is to present an historical overview of the surgical techniques introduced for the treatment of the cancer of the optic nerve and orbit.Methods: From the late 19th and early twentieth century there were introduced many novel surgical techniques for the treatment of the cancer of the optic nerve and orbit. The majority of these techniques concerned for the cure of orbital malignance, while optic nerve cancer many times was treated by enucleation of the eyeball.Results: Except of the extraction of the malignant tumors the surgeons of the time tried to present the best aesthetic result, fact which points the progress of the surgery those years.Conclusions: Many of the techniques introduced those years are still in use.

Accuracy of preoperative imaging in predicting optic nerve invasion in retinoblastoma: A retrospective study.

Purpose: Optic nerve invasion is an important cause of mortality in retinoblastoma. We aimed correlate preoperative imaging and surgical histopathology findings in enucleated eyes with retinoblastoma to determine the efficacy of preoperative imaging in predicting optic nerve invasion in retinoblastoma. Methods: A retrospective review of records of all patients undergoing primary enucleation for retinoblastoma at a tertiary eyecare system between March 2013 and December 2017 with all patients having undergone preoperative imaging, either CT scan or MRI. Data was analyzed statistically to determine the correlation between preoperative CT scan/MRI and histopathology. Results: Totally, 97 eyes of 97 patients were included in the study who underwent primary enucleation for unilateral retinoblastoma. The average age at presentation was 27.8 months with the chief complaint being leukocoria in all the cases. 14 patients (14.43%) had evidence of optic nerve involvement in preoperative imaging. 30 patients had optic nerve invasion on histopathology (laminar and retrolaminar). Spearman's rank correlation test revealed a significant correlation between MRI findings and HP and an insignficant correlation between CT findings and HP. The CT scan had a sensitivity of 20%, specificity of 88.89%, 50% positive predictive value and 66.67% negative predictive value. MRI had a sensitivity of 40%, specificity of 93.55%, positive predictive value of 66.67% and a negative predictive value of 82.86%. Conclusion: MRI showed significant moderate correlation with surgical histopathology for predicting optic nerve invasion in retinoblastoma whereas CT shows insignificant correlation with HPE. Therefore, we recommend MRI for predicting optic nerve invasion in cases of retinoblastoma.