Compressive Optic Disc Edema and Contralateral Papilledema: Type 2 Foster Kennedy Variant Syndrome

A 43-year-old woman presented with progressive anosmia, self-neglect, and lethargy for 1 year. Brain MRI demonstrated a mass that was compressing the optic nerve in the right eye and had a significant mass effect and cerebral edema producing increased intracranial pressure. Examination revealed the patient to have a variant of Foster Kennedy syndrome involving ipsilateral compressive optic neuropathy with superimposed optic disc edema and contralateral papilledema.

Photo and Video Essay Section Editors: Melissa W. Ko, MD Dean M. Cestari, MD Peter Quiros, MD Compressive Optic Disc Edema and Contralateral Papilledema: Type 2 Foster Kennedy Variant Syndrome Jason Zehden, Shruthi Harish Bindiganavile, MD, Nita Bhat, MD, Andrew G. Lee, MD FIG. 1. A. Postcontrast T1 axial and (B) coronal imaging study showing a large midline anterior skull base meningioma measuring 6.4 · 6 · 5 cm. There is a prominent mass effect on the adjacent frontal lobes with prominent adjacent parenchymal vasogenic edema, with extension into the suprasellar cistern and displaces the optic chiasm and infundibulum posteriorly and inferiorly. The intracranial optic nerves are slightly displaced laterally. Abstract: A 43-year-old woman presented with progressive anosmia, self-neglect, and lethargy for 1 year. Brain MRI demonstrated a mass that was compressing the optic nerve in the right eye and had a significant mass effect and cerebral edema producing increased intracranial pressure. Examination revealed the patient to have a variant of Foster Kennedy syndrome involving ipsilateral compressive optic Baylor College of Medicine (JZ, AGL), Houston, Texas; Department of Ophthalmology (SHB, NB, AGL), Blanton Eye Institute, Houston Methodist Hospital, Houston, Texas; The Houston Methodist Research Institute (AGL), Houston Methodist Hospital, Houston, Texas; Departments of Ophthalmology (AGL), Neurology, and Neurosurgery, Weill Cornell Medicine, New York, New York; Department of Ophthalmology (AGL), University of Texas Medical Branch, Galveston, Texas; University of Texas MD Anderson Cancer Center (AGL), Houston, Texas; Texas A and M College of Medicine (AGL), Bryan, Texas; and Department of Ophthalmology (AGL), The 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] Zehden et al: J Neuro-Ophthalmol 2021; 41: e217-e219 neuropathy with superimposed optic disc edema and contralateral papilledema. Journal of Neuro-Ophthalmology 2021;41:e217–219 doi: 10.1097/WNO.0000000000001091 © 2020 by North American Neuro-Ophthalmology Society A previously healthy 43-year-old woman presented with progressive anosmia for 1 year. She developed progressive apathy and increasing self-neglect. She was laid off from work and her family attributed her worsening symptoms to “depression.” She became progressively more lethargic, was unkempt (e.g., not maintaining self-hygiene and not combing her hair), and was unable to do her normal activities or keep up with her usual routines. She was referred for allergy testing by her primary physician due to the loss of smell and was told it was “postviral.” The patient presented to neuro-ophthalmology with painless progressive loss of vision in the right eye. Past e217 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo and Video Essay medical history was negative. She was taking no medications. She had no past ocular history. MRI of the brain showed a large anterior skull-based extra-axial enhancing mass lesion that filled the anterior cranial fossa and was centered on the planum sphenoidale (Fig. 1). The mass was compressing the optic nerve in the right eye and had a significant mass effect and cerebral edema producing increased intracranial pressure. Neuro-ophthalmic examination showed a visual acuity of light perception in the right eye and 20/25 in the left eye. The pupils measured 4 mm in the dark and 2 mm in light, and there was a right relative afferent pupillary defect. Automated perimetry (automated 24-2 visual field) testing could not be performed in the right eye and showed enlargement of the blind spot and an inferior arcuate defect in the left eye (Fig. 2). Intraocular pressure measured 17 mm Hg in the right eye and 19 mm Hg in the left eye. The slit lamp, motility, and external examinations were normal in both eyes. Fundus examination showed bilateral but markedly asymmetric optic disc edema. The optic nerve showed Frisen Grade 1 optic disc edema with superimposed pallor in the right eye and marked Frisen Grade 4 optic disc edema in the left eye (Fig. 3). Optical coherence tomography showed a retinal nerve fiber layer measurement of 121 mm in the right eye and 135 mm in the left eye. The patient underwent subtotal resection of WHO Grade 1 meningioma. Postoperative MRI showed residual meningioma measures up to 6.4 · 6.4 · 4.8 cm (anteroposterior · transverse · Craniocaudal). Postoperative stereotactic radiotherapy was planned, but the patient’s postoperative course was complicated by a pulmonary embolism needing an inferior vena cava filter. Slow-growing intracranial lesions of the anterior cranial fossa (e.g., frontal, olfactory, or sphenoid wing meningioma) can produce a unique combination of ocular findings known as the Foster Kennedy syndrome (FKS) (1). Foster Kennedy syndrome classically presents as optic atrophy in one eye from a compressive optic neuropathy from the tumor and disc FIG. 3. Fundus photograph showing mild (Grade 1) disc edema with sector pallor in the right eye and marked Grade 4 disc edema in the left eye. edema in the contralateral eye due to elevated intracranial pressure–related papilledema (Type 1 FKS) (1). Foster Kennedy syndrome is a well-known but uncommon neuroophthalmic syndrome but may be the presentation in 0.9%– 2.5% of intracranial masses (2). Papilledema is optic disc edema due to increased intracranial pressure and is typically bilateral. Although the classic Type 1 FKS is well known, Types 2 and 3 FKS are less commonly recognized. Bilateral but asymmetric optic disc edema with an ipsilateral compressive optic neuropathy, optic atrophy, disc edema, and markedly asymmetric contralateral papilledema is likely a variant of Type 2 FKS. We describe such a case in this photo essay. The Foster Kennedy syndrome originally described 3 neurologic/neuro-ophthalmic signs of a frontal lesion (3). Type 1 is the classic FKS with optic atrophy in one eye and disc edema in the contralateral eye (1,3). Type 2 is bilateral papilledema developing unilateral optic atrophy (3). Type 3 is bilateral papilledema developing bilateral optic atrophy (3). Our patient presented with a Type 2 FKS who presented with an ipsilateral compressive optic neuropathy with superimposed optic disc edema and contralateral papilledema due to a giant planum sphenoidale meningioma. Although most cases of FKS are caused by meningiomas of the olfactory groove, falx cerebri, sphenoidal wing, or subfrontal regions, other lesions can produce FKS (4). Anosmia is a common feature in FKS as in our patient (5,6). FIG. 2. Automated perimetry 24-2 testing showing enlarged blind spot in the left eye. e218 Zehden et al: J Neuro-Ophthalmol 2021; 41: e217-e219 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo and Video Essay It is important to recognize a constellation of symptoms, including anosmia, apathy and self-neglect, which localizes to the frontal lobe. In any such patient, vision symptoms must be specifically enquired into and an ophthalmology referral may be indicated because the patient may not complain about vision loss, given their cognitive disability and subjective indifference to their symptoms. Our patient specifically mentioned that she did not recall noticing her vision loss. Neuroimaging, preferably contrast MRI is recommended for FKS (4). Surgical removal of the mass is the primary treatment for FKS. In summary, although the typical FKS (Type 1) is well known, the less common forms of bilateral optic disc edema with an associated unilateral (Type 2) or bilateral compressive optic neuropathy and secondary optic atrophy (Type 3) are also forms of FKS. We believe that our case of optic atrophy and optic disc edema in the right eye with markedly asymmetric contralateral papilledema in the left eye is a Type 2 FKS variant. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: J. Zehden, S. H. Bindiganavile, and A. G. Lee; b. Acquisition of data: J. Zehden, S. H. Bindiganavile, N. Bhat, and A. G. Lee; c. Analysis and interpretation Zehden et al: J Neuro-Ophthalmol 2021; 41: e217-e219 of data: J. Zehden, S. H. Bindiganavile, and A. G. Lee. Category 2: a. Drafting the manuscript: J. Zehden, S. H. Bindiganavile, N. Bhat, and A. G.Lee; b. Revising it for intellectual content: J. Zehden, S. H. Bindiganavile, and A. G. Lee. Category 3: a. Final approval of the completed manuscript: J. Zehden, S. H. Bindiganavile, N. Bhat, and A. G. Lee. REFERENCES 1. Sadun A, Agarwal M. Topical diagnosis of acquired optic nerve disorders. In: Miller N, Biousse V, Newman N, Kerrison J, eds. Walsh and Hoyt’s Clinical Neuro-Ophthalmology. Vol 1. 6th edition. Philadelphia, PA: Lippincott Williams & Wilkins; 2005:228–229. 2. von Wowern F. The Foster Kennedy syndrome: an evaluation of its diagnostic value. Acta Neurol Scand. 1967;43:205– 214. 3. Lai A, Chiu S, Lin I, Sanders M. Foster Kennedy syndrome: now and then. J Neuroophthalmol. 2014;34:92–94. 4. Lotfipour S, Chiles K, Kahn J, Bey T, Rudkin S. An unusual presentation of subfrontal meningioma: a case report and literature review for Foster Kennedy syndrome. Intern Emerg Med. 2011;6:267–269. 5. Stone J, Vilensky J, McCauley T. Neurosurgery 100 years ago: The Queen Square letters of Foster Kennedy. Neurosurgery. 2005;57:797–808. 6. Micieli J, Al-Obthani M, Sundaram A. Pseudo-Foster Kennedy syndrome due to idiopathic intracranial hypertension. Can J Ophthalmol. 2014;49:e99–e102. e219 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.