From the Department of Ophthalmology (W-YY, C-KC, P-PH, K-HH), Shin-Kong Wu Ho-Su Memorial Hospital, Taipei; the Department of Ophthalmology (C-KC), College of Medicine, Fu Jen Catholic University, Taipei, Taiwan; and the Department of Ophthalmology (C-KC), Nation Taiwan University Hospital, College of Medicine, Nation Taiwan University, Taipei, Taiwan.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Cheng-Kuo Cheng, Department of Ophthalmology, Shin Kong Wu Ho-Su Memorial Hospital, No.95, Wen-Chang Road, Shih-Lin District, Taipei 11120, Taiwan.
The Foster Kennedy syndrome is an unusual sign noted when a brain tumor, usually a meningioma, directly compresses the atrophic optic nerve and simultaneously produces intracranial hypertension, which can only be appreciated contralaterally.1 It accounts for less than 1% of all patients with symptomatic meningioma.2 This report presents a rare case of concomitant advanced retinitis pigmentosa and the meningioma-related Foster Kennedy syndrome. The association of retinitis pigmentosa and the Foster Kennedy syndrome, as demonstrated in our patient, may be uncommon. To the best of our knowledge, the association of the Foster Kennedy syndrome and retinitis pigmentosa has never been reported before.
A 57-year-old woman who had suffered from long-term visual disturbance due to a dominant type of retinitis pigmentosa noted an even more severe deterioration in the vision of her left eye for an uncertain period of time before first presentation. Several of her family members including her father, brother, niece, and daughter have also been diagnosed with retinitis pigmentosa. Due to the above problem, she had visited the local medical department twice, but only progression of retinitis pigmentosa was diagnosed. Occasional vomiting, unsteady gait, frontal headache, and near blindness of the left eye were also noted afterward. She then came to our clinic for further survey. On ophthalmologic examination, her vision was 1/60 in the right eye and she only had perception of light in the left eye. The eyes were aligned in all positions of gaze and versions were normal. A marked relative afferent pupillary defect was present in her left eye. Examinations of bilateral anterior segment and intraocular pressures were normal. Bilateral mydriatic ophthalmoscopic examination revealed a typical appearance of advanced retinitis pigmentosa including marked vascular attenuation and retinal pigmentary changes. The optic disc of her right eye was markedly swollen and reddish, whereas that of the left eye was distinctly pale (Fig. 1). Under the impression of the Foster Kennedy syndrome, a magnetic resonance imaging (MRI) study of the brain was performed. The images of MRI revealed a well-encapsulated round mass with homogenous signal on T2-weighted images, which was about 4.45 cm in diameter and was located in the left frontal base arising from the medial sphenoidal ridge (Fig. 2A). Vasogenic edema in the left frontal and temporal lobes with falxial herniation and midline shift to the right side were also noted. The root of the left optic nerve was embraced in the lower part of the tumor and was displaced medially through the axial and sagittal view of the MRI (Figs. 2B, C, E, and F). Imaging evidence of increased intracranial pressure by ventriculomegaly was also shown in MRI. No lesions were identified close to the right optic nerve. The left cavernous sinus seemed to be invaded by the tumor. The diagnosis was consistent with sphenoid ridge meningioma (Fig. 2D). She was then transferred to a neurosurgeon and the well-encapsulated tumor was successfully removed by a left craniotomy. The histopathologic study of the excised specimen disclosed a meningiothelial meningioma.
Figure 1. Optic Discs at Presentation Showing Right Optic Papilloedema and Left Optic Atrophy. Bilateral Advanced Retinits Pigmentosa with Posterior Pole Involvement Was also Noted.
Figure 2. (A) Preoperative Axial T2-Weighted Magnetic Resonance Imaging (MRI) Revealed a Well-Encapsulated off Round Mass About 4.45 Cm in Diameter Located in the Left Frontal Base Arising from the Sphenoidal Ridge. Vasogemic Edema and Mass Effect Were also Presented in the Left Frontal and Temporal Lobes. (B)(C)(E)(F) The Root of the Left Optic Nerve Was Embraced in the Lower Part of the Tumor and Was Displaced Medially (white Arrow) on the Axial and Coronal T1- and T2-Weighted MRI. No Lesions Were Identified Close to the Right Optic Nerve. (D) Contrast-Enhanced T1-Weighted Coronal MRI Further Demonstrated a Globose, Homogenous and Enhancing Meningioma Arising from the Medial Sphenoidal Ridge with Suspected Cavernous Sinus Involvement.
In 1911, Foster Kennedy (1884 to 1952) first described the syndrome as primary optic atrophy on the side of the frontal lobe tumor with concomitant papilloedema in the opposite eye.1 It is often associated with meningioma arising in the meninges surrounding the frontal lobe such as sphenoid wing,3 falx4 and olfactory groove.5 It may also occur in association with a wider variety of other intracranial spaces occupying lesions such as pituitary adenoma,2 nasopharyngeal carcinoma,6 juvenile nasopharyngeal angiofibroma,7 metastatic cerebral tumor without frontal lobe or anterior cranial fossa involvement8 and non-tumor-related entities such as microvasculitis.9
The original disease mechanism proposed by Foster Kennedy is a direct tumor compression of the optic nerve with increased intracranial pressure. The compressed eye has an appearance of optic atrophy, whereas the contralateral eye has an appearance of papilloedema because the tumor induces increased cerebrospinal fluid pressure.10 It usually causes marked visual deterioration in the compressed eye with only a mild visual disturbance in the contralateral eye.10
Another alternative mechanism proposed by Watnick and Trobe11 stated bilateral asymmetric compression of both optic nerves either directly by the tumor itself or by the swollen aqueductal system, which could also cause the same phenomenon of papilloedema on one side and optic atrophy on the other side. However, the MRI study of the brain in our patient (Fig. 2) showed an obvious direct tumor embrace of the left optic root without involving the contralateral side. The falxial herniation and midline shift to the right side indicated that our patient was suffering from severe increased intracranial pressure. This evidence revealed that the mechanism causing the disease in our patient belonged to the original mechanism proposed by Foster Kennedy rather than the alternative mechanism.11
This case was of interest in the concurrent association of retinitis pigmentosa and the meningioma that induced the Foster Kennedy syndrome. The family history of dominant hereditary pattern, bilateral involvement, progressive course, and the lack of previous episodes of posterior segment disease suggested that our patient had hereditary retinitis pigmentosa. Although retinitis pigmentosa has been shown to coexist with several kinds of tumors such as acquired vasoproliferative tumors,12–15 colon cancer,16 and carcinoid tumor,17 no association between meningiomas and retinitis pigmentosa has been described so far. Further, the genes known to be associated with meningioma were very different from those associated with retinitis pigmentosa. Therefore, it is most likely that the coexistence of retinitis pigmentosa and meningioma in this patient occurred only incidentally.
Intracranial meningioma is often slow-growing, and the clinical presentation is often insidious in nature. The onset of headache suggesting raised intracranial pressure is commonly not apparent. Patients with medial sphenoid ridge meningiomas often do not have clear symptoms until their tumors are greater than 3 cm in diameter, already surrounding or displacing the optic nerves. This makes early diagnosis difficult. Chamberlain and coworkers reviewed the literature of intracranial meningiomas and found that visual impairment occurs in about 16% in benign meningioma and 29% in malignant meningioma, respectively.18 Early detection of intracranial meningioma is necessary to prevent the development of the Foster Kennedy syndrome.
The diagnosis of the Foster Kennedy syndrome in our patient was further compromised by the fact it was concomitantly associated with severe retinitis pigmentosa. The reduction in visual acuity might not have been easily detected earlier because the patient had already suffered from long-term visual impairment caused by retinitis pigmentosa. Further, the optic atrophy of her left eye might be masqueraded by the “waxy pallor” appearance of optic disc commonly associated with advanced retinitis pigmentosa. However, there were still some clues that could suggest a pathological cause other than retinitis pigmentosa in our patient. First, the visual acuity impairment in retinitis pigmentosa is usually bilaterally symmetrical. Rapid deterioration in only one eye is unusual for retinitis pigmentosa. Second, the patient had a recent development of occasional vomiting, unsteady gait, and frontal headache, possibly suggesting problems in the central nervous system. Third, the papilledema is distinctively not a common feature of advanced retinitis pigmentosa. Early diagnosis requires a high suspicion of all these symptoms and signs.
Surgical resection remains a common first-line treatment for these tumors. The large majority, approximately 80%, of meningiomas can be treated by surgery.19 Early diagnosis may be able to save the vision of the compressed eye. As visual impairment is a common feature in the compressed eye of the Foster Kennedy syndrome, the ophthalmologist may be the most important detector in this disease. This report may help our colleagues to be more aware of this syndrome. Systemic evaluation for central nervous system abnormalities is warranted in such cases.
- Foster K. Retrobulbar neuritis as an exact diagnostic sign of certain tumors and abscesses in the frontal lobes. Am J Med Sci. 1911;142:355–368. doi:10.1097/00000441-191109000-00005 [CrossRef]
- Ruben S, Elston J, Hayward R, Pituitary adenoma presenting as the Foster-Kennedy syndrome. Br J Ophthalmol. 1992;76:117–119. doi:10.1136/bjo.76.2.117 [CrossRef]
- Moss JP, Freemon FR, Hester RW, Toledo JA, The Foster Kennedy sign: a case with papilledema contralateral to a sphenoid wing meningioma but without apparent optic atrophy. Dis Nerv Syst. 1977;38:62–63.
- Neville RG, Greenblatt SH, Kollartis CR, Foster Kennedy syndrome and an optociliary vein in a patient with a falx meningioma. J Clin Neuroophthalmol. 1984;4:97–101.
- Fukuyama J, Hayasaka S, Setogawa T, Ando S, Moritake K, Foster Kennedy syndrome and optociliary shunt vessels in a patient with an olfactory groove meningioma. Ophthalmologica. 1991;202:125–131.
- Zohdy G, Ghabra M, Donogue C, Nasopharyngeal carcinoma: a cause of Foster Kennedy syndrome. Eye. 1994;8:364–367.
- Aga A, Juvenile nasopharyngeal angiofibroma presenting as Foster Kennedy Syndrome. Ethiop Med J. 2001;39:251–260.
- Yildizhan A, A case of Foster Kennedy syndrome without frontal lobe or anterior cranial fossa involvement. Neurosurg Rev. 1992;15:139–142. doi:10.1007/BF00313511 [CrossRef]
- Tamai H, Tamai K, Yuasa H, Pachymeningitis with pseudo-Foster Kennedy syndrome. Am J Ophthalmol. 2000;130:535–537. doi:10.1016/S0002-9394(00)00566-3 [CrossRef]
- Tso MO, Hayreh SS, Optic disc edema in raised intracranial pressure. IV. Axoplasmic transport in experimental papilledema. Arch Ophthalmol, 1977;95:1458–1462.
- Watnick RL, Trobe JD, Bilateral optic nerve compression as a mechanism for the Foster Kennedy syndrome. Ophthalmology. 1989; 96:1793–1798.
- Kollarits CR, Mehelas TJ, Shealy TR, Zahn JR, Von Hippel tumors in siblings with retinitis pigmentosa. Ann Ophthalmol. 1982;14:256–259.
- Bec P, Mathis A, Adam P, Maillard P, Alberge Y, Retinitis pigmentosa associated with astrocytic hamartomas of the optic disc. Ophthalmologica. 1984;189:135–138.
- Medlock RD, Shields JA, Shields CL, Yarian DL, Beyrer CR, Retinal hemangioma-like lesions in eyes with retinitis pigmentosa. Retina, 1990;10:274–277. doi:10.1097/00006982-199010000-00009 [CrossRef]
- Shields CL, Shields JA, Barrett J, De Potter P, Vasoproliferative tumors of the ocular fundus. Classification and clinical manifestations in 103 patients. Arch Ophthalmol. 1995;113:615–623.
- Winzelberg GG, Greenstein R, Ferrucci JT Jr, . Retinitis pigmentosa and colon cancer: a previously unreported association. Cancer. 1980; 45:2897–2898. doi:10.1002/1097-0142(19800601)45:11<2897::AID-CNCR2820451131>3.0.CO;2-V [CrossRef]
- Zusman J, Nesbit ME Jr, . Carcinoid tumor in retinitis pigmentosa. South Med J. 1978;71:1211–1212.
- Chamberlain MC, Blumenthal DT, Intracranial meningiomas: diagnosis and treatment. Expert Rev Neurother, 2004;4:641–648. doi:10.1586/1473722.214.171.1241 [CrossRef]
- Russell SM, Benjamin V, Medial sphenoid ridge meningiomas: classification, microsurgical anatomy, operative nuances, and long-term surgical outcome in 35 consecutive patients. Neurosurgery. 2008;62:1169–1181. doi:10.1227/01.neu.0000317372.92393.e8 [CrossRef]