Petroclival Meningioma Presenting With an Ipsilateral Sixth Nerve Palsy and a Contralateral Homonymous Quadrantanopia: A Unique and Topographically Localizing Syndrome

An 84-year-old woman reported onset of headaches, diplopia, and blurred vision. On examination, she was found to have a left sixth nerve palsy and an incongruous right homonymous hemianopia. Brain MRI demonstrated a left petroclival meningioma, causing this unusual combination of clinical findings. The patient was treated with radiation therapy and has remained stable over 4 years of follow-up.

Photo Essay Section Editors: Melissa W. Ko, MD Dean M. Cestari, MD Petroclival Meningioma Presenting With an Ipsilateral Sixth Nerve Palsy and a Contralateral Homonymous Quadrantanopia: A Unique and Topographically Localizing Syndrome Ardalan Sharifi, BS, Lance J. Lyons, MD, Aroucha Vickers, DO, Andrew G. Lee, MD FIG. 1. Automated perimetry (Humphrey 24-2) demonstrates an incongruous right homonymous hemianopia. Abstract: An 84-year-old woman reported onset of headaches, diplopia, and blurred vision. On examination, she Department of Ophthalmology & Visual Sciences (AS, LJL, AGL), University of Texas Medical Branch, Galveston, Texas; Department of Ophthalmology (AV, AGL), Blanton Eye Institute, Houston Methodist Hospital, Houston, Texas; Department of Ophthalmology (AGL), Baylor College of Medicine, Houston, Texas; Departments of Ophthalmology, Neurology, and Neurosurgery (AGL), Weill Cornell Medicine, New York, New York; Section of Ophthalmology (AGL), University of Texas MD Anderson Cancer Center, Houston, Texas; and Department of Ophthalmology (AGL), University of Iowa Hospitals and Clinics, Iowa City, Iowa. The authors report no conflicts of interest. Address correspondence to Andrew G. Lee, MD, Blanton Eye Institute, Houston Methodist Hospital, 6560 Fannin Street Suite 450, Houston, TX 77030; E-mail: [e-mail] Sharifi et al: J Neuro-Ophthalmol 2019; 39: 405-407 was found to have a left sixth nerve palsy and an incongruous right homonymous hemianopia. Brain MRI demonstrated a left petroclival meningioma, causing this unusual combination of clinical findings. The patient was treated with radiation therapy and has remained stable over 4 years of follow-up. Journal of Neuro-Ophthalmology 2019;39:405-407 doi: 10.1097/WNO.0000000000000742 © 2019 by North American Neuro-Ophthalmology Society A n 84-year-old woman complained of headaches, blurred vision, and diplopia for the past 6 years. She had multiple medical problems, including hypertension, 405 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay FIG. 2. Brain MRI. Precontrast axial T1 scan (A) shows a left-sided mass compressing the brainstem and left temporal lobe, whereas the T2 axial image (B) also demonstrates left temporal lobe edema. Postcontrast axial (C) and coronal (D) T1 scans confirm the presence of an extra-axial dural‐based homogeneously enhancing mass. diabetes mellitus, rheumatoid arthritis, cardiac disease, and a previous history of a left cerebellar stroke. On examination, visual acuity was 20/25 in the right eye and 20/30 in the left eye. Pupillary reactions were normal. Extraocular movements demonstrated a partial left sixth nerve palsy with other cranial nerves intact. Fundus examination showed evidence of age-related macular degeneration while automated visual fields (Humphrey 24-2) revealed an incongruous right homonymous hemianopia (Fig. 1). Brain MRI showed an extradural-based enhancing mass in the left parasellar region with mass effect on the pons and left medial temporal lobe (Fig. 2). The mass eroded the medial aspect of the left petrous apex and encased the petrous and cavernous segments of the left internal carotid artery. It also involved the left cavernous sinus and Meckel's cave. These findings were consistent of a petroclival meningioma. Given the tumor location and the multiple medical problems of the patient, she underwent stereotactic, fractionated, intensity-modulated radiation therapy of 50 Gy. Her clinical examination and follow-up MRI scans have remained stable over 4 years of follow-up. Petroclival meningiomas are intracranial masses with dural attachments to the petroclival synchondrosis; these are usually located near the upper two-thirds of the clivus (1). In a study of 98 patients, the most frequent presenting 406 symptoms were headache and gait disturbances with cerebellar signs (2). In addition, patients with petroclival meningiomas frequently develop cranial nerve symptoms including diplopia, trigeminal neuralgia, and disturbances of hearing. We were unable to find previous reports of involvement of the afferent visual pathways (1,3). Petroclival meningiomas remain a surgical challenge because of their capacity to encapsulate cranial nerves, compress surrounding blood vessels, and cause edema and mass effect. With advances in neuroimaging and refinement in surgical approaches, the outcomes from resection of petroclival meningiomas have improved. The surgical goal of gross total removal of tumor must be tempered with optimal patient function after treatment. In addition, radiosurgery has been advocated as an alternative or complementary treatment modality. Multimodal treatment strategies continue to evolve for patients with petroclival meningiomas (4). STATEMENT OF AUTHORSHIP Category 1: a. conception and design: A. Sharifi, L. J. Lyons, A. Vickers, and A. G. Lee; b. acquisition of data: A. Sharifi, L. J. Lyons, A. Vickers, and A. G. Lee; c. analysis and interpretation of data: A. Sharifi, L. J. Lyons, A. Vickers, and A. G. Lee. Category 2: a. drafting the manuscript: A. Sharifi, L. J. Lyons, A. Vickers, and A. G. Lee; b. revising it for intellectual content: A. Sharifi, L. J. Lyons, Sharifi et al: J Neuro-Ophthalmol 2019; 39: 405-407 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay A. Vickers, and A. G. Lee. Category 3: a. final approval of the completed manuscript: A. G. Lee. REFERENCES 1. Hunter JB, Weaver KD, Thompson RC, Wanna GB. Petroclival meningioma. Otolaryngol Clin North Am. 2015;48:477-490. Sharifi et al: J Neuro-Ophthalmol 2019; 39: 405-407 2. Van Havenbergh T, Carvalho G, Tatgiba M, Plets C, Samii M. Natural history of petroclival meningiomas. Neurosurgery. 2003;S2:55-62. Discussion 62-64. 3. Kawase T, Shiobara R, Ohira T, Toya S. Developmental patterns and characteristic symptoms of petroclival meningiomas. Neurol Med Chir (Tokyo). 1996;36:1-6. 4. Maurer AJ, Safavi-Abbasi S, Cheema AA, Glenn CA, Sughrue ME. Management of petroclivial meningiomas: a review of the development of current therapy. J Neurol Surg. 2014;75:358-367. 407 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.