Large Planum Sphenoidale Meningioma in a Patient with Bilateral Optic Disc Pallor

Abstract

Introduction

Differentiating non-glaucomatous and glaucomatous etiologies of optic neuropathy clinically can be challenging. We describe a patient with glaucoma and a concurrent planum sphenoidale meningioma to highlight the importance of fundoscopic examination and ancillary diagnostic tests. Despite thinning of the retinal nerve fiber layers (RNFL), tumor resection led to encouraging improvement in postoperative visual field (VF) testing. This suggests that the presence of reasonably preserved nerve fiber layers is a prognostic factor for visual field recovery following neurosurgical intervention in cases involving the chiasmal region.

Case Presentation

A 73-year-old female presented with a 1-year history of headaches, intermittent ocular pain, blurred vision, and gradual loss of peripheral vision in her left eye. Initial evaluation revealed normal intraocular pressure and asymmetric cupping of 0.6 and 0.4 in the right and left eyes, respectively. While normal tension glaucoma was a possible diagnosis, her bilateral optic nerve head pallor, thinning of the RNFL, and VF defects that did not match the cupping raise suspicion of an intracranial lesion. Magnetic resonance imaging (MRI) and histopathology confirmed a large planum sphenoidale meningioma, which was surgically resected. Postoperative recovery showed drastically improved VF and resolution of symptoms, though visual acuity remained suboptimal.

Conclusion

Surgical resection of a large tumor size, as seen in our patient, can still result in visual field improvement despite suboptimal recovery of visual acuity. This case highlights the importance of considering intracranial tumors in the differential diagnosis to prevent long-term visual impairment.

Introduction

Planum sphenoidale meningiomas are rare, slow-growing tumors that arise from the meninges covering the sphenoid bone, particularly at the base of the anterior cranial fossa. While meningiomas account for around 30% of primary intracranial tumors, planum sphenoidale meningiomas located in the suprasellar region make up 5–10% of all meningiomas [1, 2]. These tumors can compress critical neurovascular structures, such as the optic nerves, optic chiasm, and cerebral arteries [3]. Consequently, patients often present with progressive visual impairment, including vision loss, visual field deficits, and optic disc pallor, which results from optic nerve atrophy due to chronic compression [4].

Prompt diagnosis and intervention are crucial to avoid serious complications. Ophthalmologic examination, including fundoscopy, is vital for detecting optic disc abnormalities [5]. A comprehensive cranial nerve assessment is critical in the initial evaluation of any patient presenting with undiagnosed optic disc abnormalities. Although magnetic resonance imaging (MRI) with contrast remains the gold standard for diagnosing brain tumors [6], routine neuroimaging is not typically performed in all patients with optic disc changes [7, 8]. Hence, visual field evaluation is crucial for suspecting undetected optic nerve and/or brain lesions, particularly in the presence of concomitant glaucoma-related visual field (VF) defects [9]. Studies have reported that baseline findings such as preserved retinal nerve fiber layer (RNFL) thickness, tumor anteriorly located in the visual pathway, and small tumor size are associated with better clinical outcomes following tumor resection [10, 11]. Herein, this report describes a patient with a large planum sphenoidale meningioma who presented with progressive vision loss and bilateral optic disc pallor, emphasizing the diagnostic complexities and therapeutic strategies used to diagnose and manage the tumor while aiming to preserve visual function. Most notably, even with thin RNFL at baseline, improvement in VF defects can still occur following the resection of a large planum sphenoidale meningioma as demonstrated in this patient. Institutional approval was waived as our single case report involves retrospective medical record review of 1 patient, and the only interaction with the patient was for treatment purposes, which does not meet the Common Rule definition of research (45 CFR 164.501). Although institutional approval was not required to publish the case details, we obtained written informed consent from the patient for the publication of her case and any accompanying images. The CARE Checklist has been completed by the authors for this case report, attached as online supplementary material (for all online suppl. material, see https://doi.org/10.1159/000547058).

Case Report

A 73-year-old female with a medical history of diabetes, hyperlipidemia, and hypertension presented with a 1-year history of headaches, intermittent ocular pain, blurred vision and gradual loss of peripheral vision in her left eye. The headache was described as a dull, bilateral pressure-like sensation, occurring daily and not associated with nausea, photophobia, or phonophobia. The intermittent ocular pain and blurred vision in the left eye occurred independently of headache episodes, typically lasting a few seconds to a minute, with no associated photopsia or visual aura. There were no accompanying systemic symptoms such as motor or sensory deficits, and the neurological review of systems was unremarkable. Upon initial examination, her best-corrected visual acuity (BCVA) was 20/30 in the right eye (OD), but 20/80 in the left eye (OS). Intraocular pressure (IOP) was measured at 15 mm Hg in the right eye and 17 mm Hg in the left eye. Ocular motility testing revealed a slight limitation in abduction of the left eye, while the patient reported no diplopia. Slit-lamp examination showed round, reactive pupils without a relatively afferent pupillary defect. The right eye had a posterior chamber intraocular lens, while the left eye displayed a dense 3+ nuclear sclerotic and 2+ cortical cataract. Cranial nerve I olfactory and V1 sensory function were intact bilaterally.

Fundus examination revealed optic nerve head pallor in both eyes, with an asymmetric optic nerve head cup-to-disc ratio and height of 0.6 in the right eye and 0.4 in the left eye. There was no presence of optic notching or hemorrhages. Gonioscopy showed open angles with visualization of the ciliary body in all four quadrants in both eyes. Corneal pachymetry measurements were 524 μm and 531 μm in the right and left eye, respectively. The patient had no prior history of glaucoma or family history of the condition, and she had never used topical glaucoma medications. Optical coherence tomography (OCT) of the RNFL showed superior and inferior thinning in the right eye (average thickness 68 μm), as well as superior, inferior, and temporal thinning in the left eye (average thickness 60 μm). Humphrey visual field 24-2 testing revealed general depression in the right eye and a global defect in the left eye. One month later, the patient underwent cataract surgery on her left eye, resulting in an improved BCVA to 20/50. Bimatoprost (AbbVie Inc., North Chicago, IL, USA) was initiated in both eyes due to the asymmetric enlarged cupping, RNFL thinning, and VF defect. The relatively poor BCVA in both eyes following cataract surgeries suggested that an unknown disorder existed; however, the patient was lost to follow-up for 10 months. Upon returning, her BCVA had worsened to 20/50 in the right eye and 20/80 in the left eye, with an IOP of 17 mm Hg in both eyes. Persistent optic atrophy and ancillary diagnostic tests such as bilateral progressive RNFL thinning, and cupping non-matching VF general depression, raise suspicion of an intracranial lesion.

MRI of the brain with contrast revealed a large, enhancing lesion measuring 4.7 × 4.6 × 4.7 cm along the planum sphenoidale, exerting a mass effect on the base of the anterior cranial fossa (Fig. 1a). The patient underwent tumor resection. Histopathological analysis of the resected tissue confirmed the diagnosis of meningioma, showing the typical whorled architecture of spindle-shaped cells and psammoma bodies (Fig. 1b). Postoperatively, the patient had an uncomplicated course of recovery and experienced resolved headaches and improvement in vision. Her BCVA remained 20/50 in the right eye and 20/200 in the left eye. IOP was 15 mm Hg in both eyes, with persistent optic nerve pallor and unchanged cup-to-disc ratios of 0.6 and 0.4 in the right and left eyes, respectively. Follow-up VF tests showed significant improvement in both eyes compared to before surgery (Fig. 2a, b).

Fig. 1.

Gadolinium-enhanced MRI and pathology confirmed planum sphenoidale meningioma. a Gadolinium-enhanced MRI (left panel: axial and right panel: coronal view) shows a poorly circumscribed, avidly enhancing 4.7 × 4.6 × 4.7 cm mass originating from the floor of the anterior cranial fossa. b Hematoxylin and eosin (H&E) staining show oval to elongated tumor cell nuclei with eosinophilic cytoplasm with characteristic whorled arrangement of spindle-shaped cells (asterisk), scattered psammoma bodies (arrow), and vessels with hyalinized walls (arrowhead).

Fig. 2.

Improvement of visual field after tumor removal. a Pre-operative automated perimetry revealed and a global defect in the left eye (left panel) and a general depression in the visual field of the right eye (right panel). b Postoperative automated perimetry demonstrated improvement in both eyes.

Discussion

Our study describes a case of a large planum sphenoidale meningioma that highlights the importance of identifying non-glaucomatous conditions causing cupping non-matching visual field defects and optic disc pallor in the setting of normal IOP. It is not uncommon to misinterpret these results as glaucomatous changes as opposed to intracranial optic lesions. Large perisellar tumors, especially suprasellar tumors, such as in our patient, have been reported to associate with a glaucoma-like appearance of the optic nerve head [10]. In addition, routine neuroimaging is not recommended for all patients with questionable non-glaucomatous optic neuropathy because of the low yield of intracranial pathological detection [8]. Importantly, optic nerve pallor in the absence of cupping greater than 0.85–0.95 is not found in glaucoma [12]. Optic nerve pallor, especially with modest optic nerve cupping in our case, mandates that the clinician seeks another non-glaucomatous cause. In addition, progressive visual failure following cataract surgery, such as in our case, in the absence of any other cause, mandates a thorough exam. Hence, optic disc pallor, cranial nerve exams, and ancillary diagnostic tests such as RNFL and VF testing [13, 14] are critical to increase the index of suspicion for non-glaucomatous disease.

Imaging modalities, such as OCT RNFL, are an important diagnostic or potential prognostic tool for patients with primary or secondary optic nerve problems such as anterior visual pathway meningiomas. In one retrospective case series of meningiomas, eyes with normal pre-surgical RNFL thickness showed more improvements in post-operative visual acuity and perimetric mean deviation compared to the eyes that had thin pre-surgical RNFL thickness [11]. In contrast, our case demonstrated that thin RNFL at baseline (68 μm OD; 60 μm OS) can result in significant improvement of VF after tumor removal. The discrepancy may be due to a multitude of factors, including the selection of patients in the subgroup-specific in our patient with planum sphenoidale meningiomas versus different locations [11, 15], tumor size, vascularity of meningioma, timing of surgical intervention, and pre- and postsurgical residual functional axons [16–19].

The defective and suboptimal postoperative BCVA in the left eye of our patient is pre-existing optic nerve compromise due to the planum sphenoidale meningioma, despite successful removal of the cataract or tumor removal. While a relatively preserved nerve fiber layer on OCT analysis can be a positive prognostic indicator for the recovery of visual acuity and visual fields after neurosurgical procedures involving the chiasmal region [19], the improvement in VF in our case did not lead to visual improvement. The visual function might have been restricted due to a delay in surgical resection as our patient had been lost to follow-up for over 10 months. In addition, literature studies suggested a large tumor size of planum sphenoidale meningioma may result in poor visual outcomes, increased morbidity, and mortality. A review study reported that visual outcomes were superior in patients who underwent meningiomas removal surgery with tumors smaller than 3 cm in diameter compared to those larger than 3 cm in diameter. Among the 47 patients studied, 80% of those with tumor size <3 cm had improved VA, with all achieving a final VA of 20/50 or better; whereas 42.6% of patients with tumor size >3 cm showed improved VA, with only 48.9% of eyes having a final VA of 20/50 or better [20]. Although our patient showed improvement in the VF following resection, visual outcome was still suboptimal as the lesion was around 5 cm.

There have been reports in the literature of CNS tumors presenting with optic nerve cupping. One such example is a large pituitary macroadenoma, which may mimic normal tension glaucoma by causing non-glaucomatous optic disc cupping [13]. In more detail, one retrospective study identified 336 pituitary gland tumors, 32 meningiomas, 89 craniopharyngiomas, 9 chiasmal gliomas, and 35 other types of tumors that presented with abnormal optic head rim appearance and visual field defects [10]. This case report further emphasizes the importance of evaluating patients with optic nerve cupping and pallor for potential intracranial causes. Ancillary testing, like RNFL analysis and VF testing, can aid in identifying non-glaucomatous origins. Our case presentation utilizes data from ancillary testing to inform and guide management decisions, resulting in significant improvements in visual field outcomes.

Conclusion

This case of a large planum sphenoidale meningioma demonstrated the importance of early identification and intervention in the presence of optic disc pallor seen on a fundoscopic exam. Although there was RNFL thinning at baseline, significant VF improvement was achieved post-resection. This case emphasizes the necessity for timely neuroimaging and intervention in patients presenting with non-glaucomatous optic nerve abnormalities to optimize visual outcomes.

Statement of Ethics

This retrospective review of patient data did not require ethical approval in accordance with local and national guidelines. Ethical approval was waived as our single case report involves retrospective medical record review of 1 patient and the only interaction with the patient has been for purposes of treating the patient and does not meet the Common Rule definition of research (45 CFR 164.501). Although institutional approval was not required to publish the case details, written informed consent was obtained from the patient for publication of the details of their medical case and any accompanying images.

Conflict of Interest Statement

None of the authors have conflicts of interest.

Funding Sources

This study was not supported by any grant.

Author Contributions

Veshesh Patel contributed with manuscript writing, editing, and conceptualization. Anny M.S. Cheng and Joby Tsai contributed with manuscript writing, editing, and data collection. Woon Nam Chow contributed with conceptualization, manuscript editing, and data interpretation. Ricardo J. Komotar contributed with manuscript editing, investigation, and data interpretation. Arash Maleki contributed with idea formation, manuscript editing, and manuscript reviewing. Scott Schecter contributed with idea formation, conceptualization, manuscript editing, and supervision.

Funding Statement

This study was not supported by any grant.

Data Availability Statement

The data that support the findings of this study are not publicly available due to Health Insurance Portability and Accountability Act (HIPAA) but are available from the corresponding author upon reasonable request.