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Background: Surgical treatment of optic nerve tumors is challenging. The study's objective was to evaluate the efficacy of a combined endoscopic transethmoid and transconjunctival approach in patients without functional vision. Design: A retrospective, noncomparative case series. Methods: Retrospective data were collected from all patients who had undergone tumor resection using this approach at the authors' institution between 2015 and 2021. Preoperative assessments included magnetic resonance imaging and ophthalmological examinations, and re-assessments were conducted three months after surgery and regularly during the follow-up period. Results: Seventeen patients, mean age 35 ± 19.0 years, were enrolled. Of these, 64.7% presented with visual acuity (VA) of light perception or no light perception. Gross total resection was realized in all patients. The average decline in exophthalmos was 3.63 ± 1.93 mm. Tumor histopathological analysis identified 12 optic nerve sheath meningiomas and 5 optic gliomas. The mean follow-up time was 30 months during which there was no local recurrence in any of the patients. Conclusions: The combined endoscopic transethmoid and transconjunctival approach offers an additional choice for accessing optic nerve tumors. The procedure is both safe and effective and provides an alternative transcranial route to the orbit.

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Objective: To evaluate long-term visual function after fractionated stereotactic radiotherapy (FSRT) for primary optic nerve sheath meningioma (PONSM). Methods: This 22-year retrospective study included 34 subjects (34 affected eyes) with PONSM who were treated with FSRT exclusively. Subjects with a history of biopsy/resection were excluded. Visual function, including visual acuity (VA) and visual field mean deviation (VF MD), was evaluated at presentation (pre-radiotherapy; pre-RT) and at the final follow-up (post-radiotherapy; post-RT); treatment complications were also evaluated. Treatment success was defined as either stabilization or improvement of visual function. Results: The median pre-RT VA and pre-RT VF MD were 0.70 logarithm of the minimum angle of resolution (logMAR; range: 0.0-2.9 logMAR) and -15.4 decibels (dB) (range: -31.4 to -3.2 dB), respectively. The median total dose of FSRT was 50 Gy (range: 45-54 Gy) and the median number of fractions was 25 (range: 25-30). The median follow-up interval was 89 months (range: 6-251 months). The median post-RT VA and post-RT VF MD were 0.48 logMAR (range: 0.0-2.9 logMAR) (p = 0.010) and -6.8 dB (range: -20.6 to -1.6 dB) (p = 0.005), respectively. Among the 34 included eyes, VA was successfully treated in 29 eyes (85.3%) and worsened in 5 eyes (14.7%). Of the 14 eyes with both VA and reliable VF MD at pre-RT and post-RT time points, VF MD was successfully treated in 13 eyes (92.8%) and worsened in one (7.2%); overall visual function was successfully treated in 13 eyes (92.8%) and worsened in 1 eye (7.2%). Complications occurred in one subject (2.9%; radiation retinopathy). Conclusion: Approximately 90% of PONSM subjects exhibited long-term treatment success in terms of VA, VF MD, and overall visual function after FSRT. Additionally, the incidence of complications was low. Therefore, FSRT is effective and safe treatment for PONSM.

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Purpose: This work describes a case of Waldenström macroglobulinemia (WM) relapse presenting with unilateral blurred vision. Method: A case report is presented. Results: A 60-year-old woman with a history of WM in remission was referred for suspicious peripheral choroidal lesions and left optic disc swelling. Magnetic resonance imaging revealed optic nerve and cranial nerve infiltration consistent with central nervous system invasion from WM relapse, called Bing-Neel syndrome. Irradiation of the optic nerve and systemic targeted therapy were successful in addressing the ocular features as well as reducing immunoglobulin M paraprotein levels and lymphoproliferative disease burden. Conclusions: We described the first documented case to our knowledge of intraocular involvement as the earliest sign of relapse of WM. Ophthalmology assessment is warranted in patients with a history of WM who present with new ocular symptoms to aid early detection and treatment of this disease.

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Objective This study was aimed to review issues relating to the recognition, radiographic diagnosis, monitoring, and management of primary and secondary optic nerve sheath meningioma (ONSM). Design This study is a review of peer-reviewed literature combined with illustrative case studies. Participants and Methods A literature search was conducted via the PubMed database using pertinent search terms. Selected articles were limited to those written or translated into English. Additional works cited within articles were also included. Individual cases were drawn from the experience of a tertiary academic neuroophthalmic and orbital practice. Tables summarize radiotherapeutic and surgical studies, excluding single case reports and studies focusing on meningioma of intracranial origin. Main Outcome Measurements Review of reported surgical and radiotherapeutic series is the primary measurement. Results The natural history of optic nerve sheath meningiomas is primarily characterized by progressive ipsilateral vision loss. Diagnosis is typically based on radiographic imaging findings, with biopsy remaining indicated in some patients. Management strategies may include observation, radiation, and/or surgical intervention, or a combination of these approaches. The role of surgery, especially with respect to primary ONSM (pONSM), remains controversial. Advancement of radiotherapy techniques has shifted modern treatment paradigms in pONSM toward radiation as primary treatment, as surgical outcomes are inferior in major studies. Although radiation remains the treatment of choice in many cases, selected patients may benefit from surgery, especially in the setting of secondary ONSM (sONSM). Conclusion A wide variety of radiotherapeutic and surgical treatment modalities for ONSM exist. The specific indications for each management strategy continue to be redefined.

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PURPOSE: Primary optic nerve sheath meningiomas (ONSM) are benign lesions that typically present with findings of painless proptosis (59%), optic nerve pallor (55%), and decreased peripheral vision (35%). Herein we share an atypical case of a patient who presented acutely with periorbital pain and optic nerve head edema, and was ultimately determined to have a low-grade optic nerve meningioma. OBSERVATIONS: A 36-year-old healthy woman presented with acute onset of left periorbital discomfort. She was found to have intact visual acuity, full peripheral vision, and ipsilateral optic nerve edema. MRI imaging revealed a large intraconal mass partially encircling the left optic nerve. Incisional biopsy revealed a diagnosis of meningioma, WHO grade 1. CONCLUSIONS: Low-grade optic nerve sheath meningiomas may uncommonly present with acute pain and optic nerve head swelling, and absence of classic disease features.

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PURPOSE: We report an unusual case of a thirty-three-year-old woman who presented with acute unilateral vision loss following pregnancy and was ultimately discovered to have a pilocytic astrocytoma of the optic nerve. OBSERVATIONS: A thirty-three-year-old previously healthy female presented one month following Caesarean section with unilateral vision loss. She was found to have significantly decreased visual acuity, an afferent pupil deficit, proptosis, optic nerve edema and choroidal folds. Imaging revealed a large lesion of the optic nerve. Biopsy was performed and pathologic analysis revealed a pilocytic astrocytoma, WHO grade 1. The patient opted for close observation without further management and demonstrated mild improvement in visual function. CONCLUSIONS/IMPORTANCE: Optic pathway gliomas (OPG) most commonly present in the pediatric patient population with painless proptosis, slowly progressive vision loss, and clinical findings of chronic optic neuropathy.( Farazdaghi et al., 2019 Sep) 1 Acute presentations of this disease in adulthood are rare. This case demonstrates a rare case of acute optic nerve glioma presentation during the post-partum period.

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PURPOSE: To evaluate the clinical course of optic nerve sheath meningioma (ONSM) in Korean patients. METHODS: A retrospective study of 11 eyes of 11 patients with a diagnosis of ONSM between 2002 and 2015 at Severance Hospital. RESULTS: The mean age at symptom onset was 47.6 years. Ten females and 1 male participated in the study and all tumors were unilateral. Patients typically presented with visual loss and proptosis. Three patients complained of limited extraocular movements and seven patients exhibited visual field defects. Three patients who had a greater growth rate with intracranial involvement and two patients who had decreased vision received treatments. Five patients maintained good vision and visual field during the follow-up period. However, one patient who underwent surgical treatment presented significant visual loss and deterioration of visual field defect. One out of two patients who received three-dimensional conformal radiotherapy (3D-CRT) experienced improvement in visual field, and the other showed no change in visual field defect but remained stable with decreased tumor size. One out of two patients who underwent gamma-knife surgery showed aggravated visual field defect and the other presented with visual loss. CONCLUSIONS: ONSM is typically a slow-growing tumor. Deterioration of visual loss and visual field defect can occur after treatment of ONSM. Therefore, management should be considered carefully and should be limited to cases in which progression of the disease is advanced or tumor growth is fast. 3D-CRT can be considered in patients in need of treatment.

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Bilateral optic nerve sheath meningioma is rare. A meningioma is a benign neoplastic lesion from meningothelial cells of the meninges. They usually involve the intracanalicular portion of the optic nerve but may extend into the optic canal and through it to occupy the intracranial space. We present a case of 25-year-old Saudi female with bilateral optic nerve sheath meningioma. The diagnosis was delayed more than six years from initial symptoms.