ADULT OPTIC NERVE GLIOMA PRESENTING AS CENTRAL RETINAL VEIN OCCLUSION Yossi Yatziv, MD, Felix Bukstein, MD, Yoram Segev, MD, Anat Kesler, MD
A 70-year-old man developed central retinal artery occlusion (CRAO). The laboratory workup and imaging results were unremarkable. Six months later, he developed ipsilateral third cranial nerve palsy with visual deterioration and a contralateral temporal visual field defect. Brain imaging and optic nerve biopsy revealed an optic nerve glioma involving the chiasm. Response to chemotherapy of this potentially lethal lesion was remarkable. The authors stress comprehensive and careful monitoring of atypical CRAO cases.
From the Department of Ophthalmology, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. Case Report A 70-year-old man presented with decreasing vision in his right eye over the past 1 month and further deterioration during the last 10 days. Medical history was remarkable for hypertension, heavy smoking, and high bilateral myopia. Visual acuity was light perception in the right eye and 20/30 in the left eye. There was a right afferent pupillary defect of ⫹3 to 4, with normal anterior segments except for mild bilateral nuclear sclerosis. Right funduscopic examination (Fig. 1) revealed a swollen disk with splinter and flameshaped hemorrhages in four quadrants, consistent with central retinal vein occlusion. The left fundus was normal apart from retinal arterial narrowing. Brain and orbit computed tomography disclosed an enlarged right optic nerve with no optic canal involvement. Brain magnetic resonance imaging (MRI) demonstrated right optic nerve and nerve sheath thickening with enhancement; there was no evidence of involvement of the optic canal (Fig. 2). The differential diagnosis at this point, based on imaging findings, was optic neuritis, optic nerve meningioma, or glioma. Because of the severe visual loss and atypical course, the patient was hospitalized for further workup. Vision in the right eye deteriorated to no light perception 1 day after admission. Results of comprehensive laboratory workups and lumbar puncture were normal. Fluorescein angiography demonstrated blocked fluorescence from intraretinal hemorrhages and late leakage from the optic disk. The presumptive diagnosis based on the severe progressive visual loss was combined central retinal vein and artery occlusion. Therapy with oral prednisone (1 mg/kg) was initiated, and the dose was soon tapered and stopped because of no clinical response. He was discharged 7 days later with no change in vision. The patient was lost to follow-up; he returned only 6 months later complaining
Fig. 1. Right funduscopic examination showed a swollen disk with intraretinal hemorrhages in four quadrants.
of visual loss in his left eye. Vision was no light perception in the right eye and 20/400 in the left eye. Ocular motility showed limited elevation, depression, and adduction of the right eye, consistent with third nerve palsy. Funduscopy revealed a swollen disk with intraretinal hemorrhages in the right eye and temporal pallor of the left optic disk. Goldmann visual field perimetry demonstrated a deep temporal defect in the left eye. Brain and orbit computed tomography showed further thickening of the right optic nerve with widening of the optic canal. Brain MRI demonstrated a large lesion involving the optic chiasm and the proximal part of the left optic nerve (Fig. 3). Open biopsy of the right optic nerve disclosed high-grade optic nerve glioma. The patient received conformal three-dimensional radiation treatments (total dose, 6,500 cGy), which resulted in significant reduction of the pathologic enhancement of the visual pathway
None of the authors have any proprietary interest in this report. Reprint requests: Anat Kesler, MD, Department of Ophthalmology, Tel Aviv Sourasky Medical Center, 6 Weizman Street, Tel Aviv 64239, Israel; e-mail: [email protected]
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RETINAL CASES & BRIEF REPORTSℜ
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Fig. 2. Postcontrast axial T1-weighted magnetic resonance imaging with fat suppression showed thickening and enhancement of the intraorbital right optic nerve.
as evidenced by MRI 1 month after radiotherapy. Chemotherapy (temozolomide [200 mg/m2/d] for 5 days every 28 days) was started. At the time of this writing, after 18 cycles of chemotherapy, he was completely blind, his general condition was good, and he was asymptomatic neurologically. His latest fundus examination demonstrated bilateral optic atrophy. MRI performed every four chemotherapy cycles demonstrated an almost complete disappearance of pathologic enhancement of the visual pathways.
Discussion Optic nerve gliomas in adults are rare and, unlike childhood low-grade tumors, are high-grade astrocytomas. The adult form usually involves middle-aged males and begins with signs and symptoms mimicking
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2007
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optic neuritis, progressing within 5 weeks to 6 weeks to total blindness and ending fatally several months later. The initial symptom is monocular loss of vision, often associated with retroorbital pain. The frequently present optic nerve edema can mimic optic neuritis when combined with (brief) visual improvement after steroid therapy. Vision usually continues to deteriorate with progressive swelling of the optic disk due to superimposed occlusion, first venous then arterial. Temporal hemianopia in the contralateral eye usually develops 5 weeks to 6 weeks after first symptom onset. Hoyt et al1 reported 30 cases of adult glioma in 1973; most of these patients were blind within 2 months to 4 months and died after 6 months to 9 months. Since then, ⬇44 cases have been reported.2 Pathologic features usually have an aggressive histologic appearance, and initially nonspecific MRI signs evolve into an aggressive process extensively involving the optic nerve, sheath, chiasm, and contralateral optic nerve. After conventional radiation, our patient received temozolomide, an alkylating agent recently introduced for treating malignant glial tumors and found to be effective in both recurrent and newly diagnosed high-grade gliomas:3 several cycles led to complete disappearance of the pathologic enhancement. At the 2-year follow-up, our patient was blind bilaterally but otherwise was in good health. His was a unique and remarkable response to therapy with unprecedented survival from a potentially lethal tumor. Our patient presented with a clinical picture of central retinal vein occlusion. New surgical modalities have been reported for treatment of this condition over the past few years, such as radial optic neurotomy4 and injection of tissue plasminogen activator into the retinal venous circulation.5 At some initial point of management, a surgical option had been considered
Fig. 3. Axial (A) and coronal (B) postcontrast T1weighted magnetic resonance imaging showed marked thickening and enhancement of the chiasm bilaterally with involvement of the proximal left optic nerve (arrow in A).
ADULT OPTIC NERVE GLIOMA PRESENTING AS CENTRAL RETINAL VEIN OCCLUSION
for our patient, but it was soon abandoned because of the atypical clinical course. Surgical treatment of central retinal vein occlusion secondary to a malignant process can have serious clinical and legal implications. We therefore recommend that all patients with central retinal vein occlusion who present atypically— especially those with profound vision loss and evidence of vascular occlusion— undergo thorough investigation, including serial MRI of the orbit and central nervous system. Key words: central retinal vein occlusion, optic nerve glioma.
35
References 1. 2.
3.
4.
5.
Hoyt WF, Meshel LG, Lessel S, et al. Malignant glioma of adulthood. Brain 1973;96:121–132. Wabbels B, Demmler A, Seitz J, et al. Unilateral adult malignant optic nerve glioma. Graefes Arch Clin Exp Ophthalmol 2004;242:741–748. Yung WK, Albright RE, Olson J, et al. A phase II study of temozolomide vs. procarbazine in patients with glioblastoma multiforme at first relapse. Br J Cancer 2000;83:588–593. Opremcak EM, Bruce RA, Lomeo MD, et al. Radial optic neurotomy for central retinal vein occlusion: a retrospective pilot study of 11 consecutive cases. Retina 2001;21:408–415. Weiss JN, Bynoe LA. Injection of tissue plasminogen activator into a branch retinal vein in eyes with central retinal vein occlusion. Ophthalmology 2001;108:2249–2257.
A 70-year-old man developed central retinal artery occlusion (CRAO). The laboratory workup and imaging results were unremarkable. Six months later, he developed ipsilateral third cranial nerve palsy with visual deterioration and a contralateral temporal visual field defect. Brain imaging and optic nerve biopsy revealed an optic nerve glioma involving the chiasm. Response to chemotherapy of this potentially lethal lesion was remarkable. The authors stress comprehensive and careful monitoring of atypical CRAO cases.
From the Department of Ophthalmology, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. Case Report A 70-year-old man presented with decreasing vision in his right eye over the past 1 month and further deterioration during the last 10 days. Medical history was remarkable for hypertension, heavy smoking, and high bilateral myopia. Visual acuity was light perception in the right eye and 20/30 in the left eye. There was a right afferent pupillary defect of ⫹3 to 4, with normal anterior segments except for mild bilateral nuclear sclerosis. Right funduscopic examination (Fig. 1) revealed a swollen disk with splinter and flameshaped hemorrhages in four quadrants, consistent with central retinal vein occlusion. The left fundus was normal apart from retinal arterial narrowing. Brain and orbit computed tomography disclosed an enlarged right optic nerve with no optic canal involvement. Brain magnetic resonance imaging (MRI) demonstrated right optic nerve and nerve sheath thickening with enhancement; there was no evidence of involvement of the optic canal (Fig. 2). The differential diagnosis at this point, based on imaging findings, was optic neuritis, optic nerve meningioma, or glioma. Because of the severe visual loss and atypical course, the patient was hospitalized for further workup. Vision in the right eye deteriorated to no light perception 1 day after admission. Results of comprehensive laboratory workups and lumbar puncture were normal. Fluorescein angiography demonstrated blocked fluorescence from intraretinal hemorrhages and late leakage from the optic disk. The presumptive diagnosis based on the severe progressive visual loss was combined central retinal vein and artery occlusion. Therapy with oral prednisone (1 mg/kg) was initiated, and the dose was soon tapered and stopped because of no clinical response. He was discharged 7 days later with no change in vision. The patient was lost to follow-up; he returned only 6 months later complaining
Fig. 1. Right funduscopic examination showed a swollen disk with intraretinal hemorrhages in four quadrants.
of visual loss in his left eye. Vision was no light perception in the right eye and 20/400 in the left eye. Ocular motility showed limited elevation, depression, and adduction of the right eye, consistent with third nerve palsy. Funduscopy revealed a swollen disk with intraretinal hemorrhages in the right eye and temporal pallor of the left optic disk. Goldmann visual field perimetry demonstrated a deep temporal defect in the left eye. Brain and orbit computed tomography showed further thickening of the right optic nerve with widening of the optic canal. Brain MRI demonstrated a large lesion involving the optic chiasm and the proximal part of the left optic nerve (Fig. 3). Open biopsy of the right optic nerve disclosed high-grade optic nerve glioma. The patient received conformal three-dimensional radiation treatments (total dose, 6,500 cGy), which resulted in significant reduction of the pathologic enhancement of the visual pathway
None of the authors have any proprietary interest in this report. Reprint requests: Anat Kesler, MD, Department of Ophthalmology, Tel Aviv Sourasky Medical Center, 6 Weizman Street, Tel Aviv 64239, Israel; e-mail: [email protected]
33
RETINAL CASES & BRIEF REPORTSℜ
34
Fig. 2. Postcontrast axial T1-weighted magnetic resonance imaging with fat suppression showed thickening and enhancement of the intraorbital right optic nerve.
as evidenced by MRI 1 month after radiotherapy. Chemotherapy (temozolomide [200 mg/m2/d] for 5 days every 28 days) was started. At the time of this writing, after 18 cycles of chemotherapy, he was completely blind, his general condition was good, and he was asymptomatic neurologically. His latest fundus examination demonstrated bilateral optic atrophy. MRI performed every four chemotherapy cycles demonstrated an almost complete disappearance of pathologic enhancement of the visual pathways.
Discussion Optic nerve gliomas in adults are rare and, unlike childhood low-grade tumors, are high-grade astrocytomas. The adult form usually involves middle-aged males and begins with signs and symptoms mimicking
●
2007
●
VOLUME 1
●
NUMBER 1
optic neuritis, progressing within 5 weeks to 6 weeks to total blindness and ending fatally several months later. The initial symptom is monocular loss of vision, often associated with retroorbital pain. The frequently present optic nerve edema can mimic optic neuritis when combined with (brief) visual improvement after steroid therapy. Vision usually continues to deteriorate with progressive swelling of the optic disk due to superimposed occlusion, first venous then arterial. Temporal hemianopia in the contralateral eye usually develops 5 weeks to 6 weeks after first symptom onset. Hoyt et al1 reported 30 cases of adult glioma in 1973; most of these patients were blind within 2 months to 4 months and died after 6 months to 9 months. Since then, ⬇44 cases have been reported.2 Pathologic features usually have an aggressive histologic appearance, and initially nonspecific MRI signs evolve into an aggressive process extensively involving the optic nerve, sheath, chiasm, and contralateral optic nerve. After conventional radiation, our patient received temozolomide, an alkylating agent recently introduced for treating malignant glial tumors and found to be effective in both recurrent and newly diagnosed high-grade gliomas:3 several cycles led to complete disappearance of the pathologic enhancement. At the 2-year follow-up, our patient was blind bilaterally but otherwise was in good health. His was a unique and remarkable response to therapy with unprecedented survival from a potentially lethal tumor. Our patient presented with a clinical picture of central retinal vein occlusion. New surgical modalities have been reported for treatment of this condition over the past few years, such as radial optic neurotomy4 and injection of tissue plasminogen activator into the retinal venous circulation.5 At some initial point of management, a surgical option had been considered
Fig. 3. Axial (A) and coronal (B) postcontrast T1weighted magnetic resonance imaging showed marked thickening and enhancement of the chiasm bilaterally with involvement of the proximal left optic nerve (arrow in A).
ADULT OPTIC NERVE GLIOMA PRESENTING AS CENTRAL RETINAL VEIN OCCLUSION
for our patient, but it was soon abandoned because of the atypical clinical course. Surgical treatment of central retinal vein occlusion secondary to a malignant process can have serious clinical and legal implications. We therefore recommend that all patients with central retinal vein occlusion who present atypically— especially those with profound vision loss and evidence of vascular occlusion— undergo thorough investigation, including serial MRI of the orbit and central nervous system. Key words: central retinal vein occlusion, optic nerve glioma.
35
References 1. 2.
3.
4.
5.
Hoyt WF, Meshel LG, Lessel S, et al. Malignant glioma of adulthood. Brain 1973;96:121–132. Wabbels B, Demmler A, Seitz J, et al. Unilateral adult malignant optic nerve glioma. Graefes Arch Clin Exp Ophthalmol 2004;242:741–748. Yung WK, Albright RE, Olson J, et al. A phase II study of temozolomide vs. procarbazine in patients with glioblastoma multiforme at first relapse. Br J Cancer 2000;83:588–593. Opremcak EM, Bruce RA, Lomeo MD, et al. Radial optic neurotomy for central retinal vein occlusion: a retrospective pilot study of 11 consecutive cases. Retina 2001;21:408–415. Weiss JN, Bynoe LA. Injection of tissue plasminogen activator into a branch retinal vein in eyes with central retinal vein occlusion. Ophthalmology 2001;108:2249–2257.
