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Show ]. Clin. Neuro-ophthalmol. 4: 97-101,1984. Foster Kennedy Syndrome and an Optociliary Vein in a Patient with a Falx Meningioma ROBERT G. NEVILLE, M.D. SAMUEL H. GREENBLATT, M.D. CAROL R. KOLLARITS, M.D. Abstract We describe a 51-year-old woman with a falx meningioma causing Foster Kennedy syndrome accompanied by optociliary shunt vessels on the atrophic optic nerve. The tumor did not directly involve optic nerves or chiasm. The optociliary ves· sels disappeared after successful tumor removal. The patient's arteriograms, surgical findings, and postoperative course support the theory that optic atrophy in Foster Kennedy syndrome may be caused by compression of the optic nerve by adjacent brain tissue displaced by a distant intracranial mass lesion. Introduction In 1911, Foster Kennedy presented six cases of unilateral papilledema with contralateral optic atrophy. He considered these signs, often accompanied by loss of smell on the atrophiC side, as pathognomonic of a space-occupying basofrontal lesion on the side of the optic atrophy. I Since this report, multiple additional cases have been described, the majority associated with subfrontal tumors, usually meningiomas. 2 Optociliary veins have rarely been reported. When these vessels are seen in an eye with optic atrophy and progressive visual loss, a clinical triad strongly suggestive of spheno-orbital meningioma is present.3 - 6 Such vessels are also seen in old central retinal vein occlusions7 . 8 and less commonly in other conditions.9 - 14 We have recently documented the presence of an optociliary shunt vessel of the atrophic optic disc in a patient with Foster Kennedy syndrome and a unilateral falx meningioma. To our knowledge, this combination of findings has not been reported previously. From the Divisiun uf Ophthalmology, Department of Surgery (RGN, CRK), and the Division of Neurosurgery, Department of Neurosciences (SHG), Medical College of Ohio, Toledo, Ohio. June 1984 Case Report A 50-year-old woman had noted the sudden loss of light perception in her left eye 3 weeks prior to admission. She admitted that her vision had been blurred in that eye for an uncertain interval previously. There had been a past history of headaches, but none in recent weeks. The patient denied other neurologic or ocular symptoms. Her daughter had noted no changes in alertness or personality. Physical examination revealed a well-nourished white woman in no acute distress, oriented, but somewhat childlike and euphoric in her responses. Visual acuity was 20/25 in the right eye, and no light perception in the left eye. There was an afferent pupillary defect on the left. Ocular motility and slit lamp examination were normal. There was no proptosis. Goldmann perimetry revealed an enlarged blind spot and inferior nasal step in the field of the right eye. The right optic disc was elevated and hyperemic. The left optic disc was pale temporally, with elevation and blurring of the nasal margin. There was an optociliary shunt vessel arising at the inferior disc margin and joining the inferior branch of the central retinal vein (Fig. lA). Moderate venous engorgement was present bilaterally, with absence of spontaneous venous pulsations. There were no hemorrhages or exudates. Neurologic examination revealed only left-sided anosmia. A CT scan of the head revealed a large mass within the left frontal lobe (Fig. 2). The mass appeared to spare the frontal floor and perichiasmal region. Skull films showed erosion of the dorsum sellae. Carotid arteriography demonstrated a marked frontal shift of the midline structures to the right, secondary to a relatively hypovascular left frontal mass. Posterior displacement of the left frontal lobe substance was evidenced by a downward shift of the left internal carotid bifurcation (Figs. 3/7 and 3/7). CT scans of the orbit and optic canal revealed no evidence of optic nerve sheath meningioma. 97 Falx Meningioma Figure 1. (A) Preoperative photograph of left optic disc. The venous nature of the aberrant vessel in this photograph was confirmed by fluoroscein angiography, showing it to fill with the retinal veins in the early arteriovenous phase. (8) Postoperative photograph of left optic disc. The optociliary vessel has disappeared 4 months after removal of the falx meningioma. Journal of Clinical Neuro-ophthalmology Figure 2. CT scan showing mass arising from the falx cerebri and displacing left frontal lobe. A diagnosis of probable falx meningioma was made, and a bifrontal craniotomy was carried out. In the left frontal region, there was a large parasagittal meningioma attached to the sagittal sinus and the falx cerebri. Its anterior attachment was 4-6 cm along the falx from the attachment of the latter to the frontal floor. The tumor extended for another 5-6 cm posteriorly, and burrowed very deeply into the medial aspect of the left frontal lobe. As predicted from the CT scan, the medial aspect of the right frontal lobe was indented by the bulging falx, but there was no tumor actually on the right side of the falx. The external aspect of the tumor was tough, but its center was soft. After the center of the tumor was gutted, its capsule was gently pulled away from adjacent br.ain, allowing complete removal of the tumor With attached falx. A thin layer of brain remained at the base of the residual defect, so that the frontal floor was never actually observed after tum~r ~emoval. The histologic diagnosis was menIngioma. Postoperatively, the patient's vision ~as unchanged. Four months later, the opticocilI~ ry shunt vessels on the left optic nerve had disappeared (Fig. 18), and the right papilledema had resolved completely. Comment . The complete Foster Kennedy syndrome of opti~ a~ophy, contralateral papilledema, and anosnua IS classically associated with basofrontal tu- June 1984 Neville, Greenblatt, Kollarits mors. The optic atrophy is commonly felt to result from optic nerve compression, and the contralateral papilledema from increased intracranial pressure. 15 Foster Kennedy syndrome associated with intracranial mass lesions is relatively rare; Walsh15 noted "a very few cases· among his patients. There are multiple case reports in the literature, many of which have been criticized as incompletely documented. The majority of these cases have involved frontal floor tumors, such as olfactory groove and sphenoid ridge meningiomas, with gliomas occasionally reported. 2 "PseudoFoster Kennedy syndrome· produced by ischemic optic neuropathy is more commonly seen than true Foster Kennedy syndrome. 1b Optociliary veins are felt to result from dilation of preexisting capillary vessels connecting the central retinal vein and the peripapillary choroid. 3 • 4 These venous shunt vessels, commonly seen after central retinal vein occlusion, have been associated with chronic optic nerve compressio~ d':le to s~heno-orbital or optic ner:re sheath meningiomas. -7 Less commonly, optooliary veins have been reported in association with papilledema,9 optic nerve glioma, 10 optic disc drusen, II congenital disc anomalies, 12 arachnoid cysts of the optic nerve,13 and in association with phakomatoses. 14 Discussion In this patient, the tumor was situated in the frontal lobe, not directly related to optic nerves or chiasm. Similar cases of nonbasal tumors associated with Foster Kennedy syndrome have been reported in the European literature. Von Wowernl7 found four cases of Foster Kennedy syndrome in a series of 2,688 patients with intracranial tumors. Two of these patients had tumors that were nonbasal in location; one had a frontal lobe glioma, the other a temporal lobe glioblastoma. Tonnis found 28 cases of Foster Kennedy syndrome among 3,033 patients with histologically verified intracranial tumors. Of these cases, 12 involved intracerebral tumors remote from optic nerves and chiasm. 17 The ability of such nonbasal tumors to produce Foster Kennedy syndrome has not been well-explained. David and Hartmannl8 and Garcia-Miranda l " postulated compression of the optic nerve by adjacent brain tissue displaced by a distant mass. The arteriographic findings support such a mechanism in our case. The internal carotid bifurcation on the side of the tumor was shifted downward and posteriorly, to a position behind the optic foramen. Clearly, this shift was due to displacement of brain tissue by the tumor; the frontal lobe had been ~erniateddown over the left sphenoid ridge and lImbus sphenoidalis. The intracranial optic 99 Falx Meningioma \1') Figures 3<1 and 3b. La tt'ra I views uf bilateral carotid ,uteriugrams. A large frontal mass is seen un the Idt (a), causing d"wnward displacement uf the bifurcation of the internal cawtid artery (arrow) as compared tu the normal position uf the right carotid artery (I.). Journal of Clinical Neuro-ophthalmology nerve lies immediately posterior and inferior to the latter structure. The postoperative disappearance of the optociliary shunt vessels from our patient's left optic disc strikingly confirmed that optic nerve decompression had occurred. While the rare syndrome of Foster Kennedy is most commonly produced by anterobasal meningiomas, the clinician should be aware that tumors in more remote locations are capable of producing this syndrome, as in this case. References 1. Kennedy, F.: Retrobulbar neuritis as an exact diagnostic sign of certain tumors or abscesses in the frontal lobes. Am. f. Med. Sci. 142-355,1911. 2. Jarus, G.D., and Feldon, S.E.: Clinical and computed tomographic findings in the Foster Kennedy syndrome. Am. f. Ophthalmol. 93: 317-322, 1982. 3. Frisen, L.: Hoy:t, W.F., and Tengroth, B.M.: OptoCIliary vems, dISC pallor and visual loss. A triad of signs indicating spheno-orbital meningioma. Acta Ophthalmol. 51: 241-249,1973. 4. Rodrigues, M., Savino, P.j., and Schatz, N.j.: Spheno-orbital meningioma with opto-ciliary veins. Am. f. Ophthalmol. 81(5): 666-670, 1976. 5. Zakka, K.A, Summerer, RW., Vee, RD., Voss, RY., and Kim, J.: Opto-ciliary veins in a primary optIc nerve sheath meningioma. Am. f. Ophthalmol. 87: 92-95,1979. 6. Ellenberger, c.: Perioptic meningiomas: Syndrome of long-standing visual loss, pale disk edema and optociliary veins. Arch. Neural. 33: 671-674, 1976. 7. Sanders, M.D.: A classification of papilledema based on a f1uoroscein angiographic study of 69 cases. Trans. Ophthalmol. Soc. U.K. 89: 117-192 1969. ' 8. Baschetti, N.V., Smith, J.L., Osher, R.H., Gass, J.D., June 1984 Neville, Greenblatt, Kollarits and Norton, E.W.D.: Fluorescein angiography of optociliary shunt vessels. f. Clin. Neuro-ophthalmol. 1: 9-30,1981. 9. Eggers, H.M., and Sanders, M.D.: Acquired optoCIliary shunt vessels in papilledema. Br. f. Ophthalmol. 64: 267-271, 1980. 10. Hoyt, W.F., and Beeston, D.: The Ocular Fundus in Neurologic Disease. C.V. Mosby, St. Louis, 1966, p. 42. 11. Karel, J., Ostradovek, J., and Peleska, M.: fluorescein angiography in circulatory disturbance in drusen of the optic disk. Ophthalmologica 164: 449462, 1972. 12. Irvine, AR, Shorb, S.R, and Morris, B.W.: Optociliary veins. Trans. Am. Acad. Ophthalmol. Otolaryngol. 83: OP-541-546, 1977. 13. Miller, R., and Green, W.R: Arachnoid cysts involvmg a portion of the intraorbital optic nerve. Arch. Ophthalmol. 93: 1117-1121, 1975. 14. Zaret, C.R, Chromokos, E.A, and Meisler, D.M.: Cilio-optic vein associated with phacomatosis. Ophthalmology 87(4): 330-336, 1980. 15. Walsh, F.B.: Clinical Neura-ophthalmology, Vol. 3 (3rd ed.). Williams & Wilkins, Baltimore, 1969, pp. 68,2171. 16. Schatz, N.J., and Smith, J.L.: Non-tumor causes of the Foster Kennedy syndrome. f. Neurasurg. 27: 37-44,1967. 17. VonWowern, F.: Foster Kennedy syndrome; an evaluation of its diagnostic value. Acta. Neural. Scan~ 4~ 205-214, 1967. 18. David, M., and Hartmann, E.: Les symptoms oculalres dans les meningiomes de la petite aile du sphenoide. Ann. d'ocul. 172: 177-212, 1935. 19. Garcia-miranda, A: "Umgekehrtes" Foster-Kennedy syndrom. Ophthalmologica 112: 72-7, 1946. 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