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Show Journal ofNeuro- Ophthalmology 21( 2): 103- 105, 2001. © 2001 Lippincott Williams & Wilkins, Inc., Philadelphia Optic Neuropathy and Central Retinal Artery Occlusion in Non- Hodgkin Lymphoma Anna B. Fierz, MD, Sabine Sartoretti, MD, and Adelheid M. Thoelen, MD We report a patient with systemic large cell non- Hodgkin lymphoma in remission who presented with the rare combination of optic neuropathy and central retinal artery occlusion. Another unusual feature of this case is the lack of enhancement in the affected region on magnetic resonance imaging only hours after the first dose of steroids. Despite prompt treatment with steroids and radiotherapy, lymphomatous infiltration of the meninges developed 2 months later and was ultimately fatal. Key Words: Central retinal artery occlusion- Magnetic resonance imaging- Non- Hodgkin lymphoma- Optic neuropathy- Infiltrative. Optic nerve infiltration is a rare but well- recognized complication of non- Hodgkin lymphoma ( NHL). Rarely, retinal vascular occlusions ( arterial or venous or both) may also occur in NHL. We report a case of combined optic neuropathy and central retinal artery occlusion ( CRAO) in a patient in apparent NHL remission. CASE REPORT In March 1999, a 75- year- old woman was investigated for hyperparathyroidism and diagnosed with an aggressive form of NHL ( IWF group H) with a focus in the parathyroid gland. Staging revealed additional lesions in the spine and supraclavicular lymph nodes. After four cycles of chemotherapy, she was considered to be in remission. On the evening of July 31, 1999, she suffered Manuscript received November 7, 2000; accepted April 24, 2001. This case was presented as a poster at the meeting of the North American Neuro- Ophthalmology Society in Mont Tremblant, Canada, on March 27, 2000. From the Departments of Ophthalmology ( ABF, AMT) and Neuroradiology ( SS), University Hospital, Zurich, Switzerland. Address correspondence and reprint requests to A. B. Fierz, MD, Augenklinik, Triemlispital, Birmensdorferstrasse 497, 8063 Zurich, Switzerland. visual loss OD, progressing to blindness overnight. On August 1,1999, visual acuity OD was no light perception and there was marked disc edema with some hemorrhages. There were no vitreous cells. Systemic workup did not suggest giant cell arteritis, and erythrocyte sedimentation rate was 18 mm/ h. Computed tomograph ( CT) scan showed slight thickening of the retrobulbar nerve without enhancement. A presumptive diagnosis of non-arteritic anterior ischemic optic neuropathy was made, and aspirin was started. The next day, disc edema had markedly progressed. There was now also conjunctival chemosis OD, lid swelling, proptosis of 2 mm, reduced supraduction, retinal ischemia with a preserved cilioreti-nal vessel, and venous engorgement and hemorrhages temporal to the fovea, suggesting CRAO and accompanying venostasis retinopathy ( Fig. 1). Fluorescein angiography confirmed CRAO OD with a spared cilioretinal artery ( Fig. 2). Oral prednisone, 100 mg daily, was started on August 2, 1999. Magnetic resonance imaging ( MRI) done on August 3, 1999, revealed thickening and hyperintensity of the right retrobulbar optic nerve reaching almost to the chiasm, consistent with ischemia, but no clear enhancement ( Fig. 3). Despite this finding, lymphomatous infiltration was suspected. The patient refused a lumbar puncture. During treatment with prednisone and irradiation of the right orbit with 7x3 Gy, swelling and proptosis resolved, and motility returned to normal, but the eye remained blind. Follow-up MRI 2 weeks later was essentially unchanged. Marked optic atrophy OD developed. In October 1999, lymphomatous infiltration of the meninges was diagnosed, and the patient died in January 2000. No autopsy was performed. DISCUSSION Large cell non- Hodgkin lymphoma is the most common type of lymphoma involving the eye and the central nervous system ( 1). The primary oculocerebral forms of NHL were formerly known as reticulum cell sarcoma, but histologically similar types of NHL can originate in other locations ( commonly lymph nodes or the intestinal 103 104 A. B. FIERZETAL. FIG. 1. Fundus OD on August 2, 1999. Disc edema and central retinal artery occlusion with a preserved cilioretinal vessel and a few hemorrhages, despite retinal ischemia, are visible. tract) and spread to the orbit. There have been several reports of optic neuropathy in NHL, including in patients in remission ( 2). As in our patient, the optic neuropathy may be accompanied by signs of a retrobulbar mass, such as proptosis and impaired motility ( 3). Central retinal vessel occlusion is distinctly unusual in this setting but can occur with or without optic nerve involvement. Saatci et al. ( 4) reported a combined CRAO and vein occlusion with disc edema in a 14- year- old boy. Guyer et al. ( 5) described the combination of CRAO and optic neuropathy in a terminally ill NHL patient with sepsis; the CRAO was presumed to be caused by septic emboli. In our patient, it remains unclear whether the optic nerve itself was infiltrated or whether infiltration of the surrounding tissue led to an ischemic neuropathy. The lesion could not be localized even on MRI, which showed a hyperdense optic nerve on T2- weighted images but no enhancement- neither in the nerve nor in the orbit- such as that usually found with lymphoma. This finding may be the result of the first dose of steroids given 15 hours before imaging, but nonenhancement ( in FIG. 2. Fluorescein angiography OD on August 2, 1999, after 41 seconds. immunocompetent patients) has been reported in primary central nervous system non- Hodgkin lymphoma ( 6). Clinically, visual loss is usually less rapid and profound with infiltration of the nerve only, and some cases have been reported to improve with steroids or irradiation ( 2,3). We therefore believe that the central retinal artery was occluded early, possibly because of direct infiltration at the level of the disc. The development of concomitant venostasis retinopathy is evidence of a comparatively slow occlusion of the artery that permitted a residual perfusion. Because we have no pathology, we cannot rule out that visual loss was caused by another process, e. g., ischemia alone. However, ischemia would fail to explain the signs of a retrobulbar mass, which accompanied the CRAO. The clinical course during the next few months is also classic for lym-phomatous infiltration of the orbit and optic nerve, which frequently heralds spread to the central nervous system. This case demonstrates that a high index of suspicion is required when managing high- grade NHL. Despite the sophisticated imaging techniques available today, clinical judgment remains the key to the correct diagnosis. FIG. 3. T2- weighted MRI of the brain and orbits on August 3, 1999, showing thickening and hy-perintensity of the right optic nerve up to the orbital apex. / Neuro- Ophthalmol, Vol. 21, No. 2, 2001 OPTIC NEUROPATHY AND CRAO IN NON- HODGKIN LYMPHOMA 105 REFERENCES 1. Char DH, Ljung BM, Miller T, et al. Primary intraocular lymphoma ( ocular reticulum cell sarcoma): diagnosis and management. Ophthalmology 1988; 95: 625- 30. 2. Kay MC. Optic neuropathy secondary to lymphoma. J Clin Neuro-ophthalmol 1986; 6: 31- 4. 3. Takahashi T, Kondo T, Isayama Y. Lymphomatose neuropathia nervi optici. Klin Monatsbl Augenheilkd 1981; 178: 360- 5. 4. Saatci AO, Diizovali 6, Ozbek Z, et al. Combined central retinal artery and vein occlusion in a child with systemic non- Hodgkin's lymphoma. Int Ophthalmol 1999; 22: 125- 7. 5. Guyer DR, Green WR, Schachat AP, et al. Bilateral ischemic optic neuropathy and retinal vascular occlusions associated with lymphoma and sepsis. Ophthalmology 1990; 97: 882- 8. 6. Terae S, Ogata A. Nonenhancing primary central nervous system lymphoma. Neuroradiology 1996; 38: 34- 7. J Neuro- Ophthalmol, Vol. 21, No. 2, 2001 |
