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Show journal of Neuro- Ophthalmology 14( 3): 157- 259, 1994. © 1994 Raven Press, Ltd., New York Feature Photo Large Optic Nerve with Central Retinal Artery and Vein Occlusions from Optic Neuritis/ Perineuritis Rather Than Tumor Jacqueline M. S. Winterkorn, Ph. D., M. D., Jeffrey G. Odel, M. D., Myles M. Behrens, M. D., and Sadek Hilal, M. D. A swollen optic disc with superimposed occlusion of both the central retinal artery and vein is a rare event, with catastrophic visual consequences. Although it can result from any rapidly enlarging, space- occupying intraconal lesion ( 1), it is feared to herald an aggressive neoplasm ( 2). This paper describes two patients who presented with vascular occlusions and an enlarged optic nerve owing to a form of idiopathic orbital inflammation ( pseudotumor) rather than tumor. CASE REPORTS Case 1 A 5- year- old boy in good general health was seen when he developed pain, right upper lid swelling, and loss of vision in the right eye. Six months earlier, esotropia had been noted by a pediatrician. Three weeks prior to presentation, the boy had been given Gantrisin ointment for mild bilateral conjunctivitis, which resolved completely. In his left eye, visual acuity was 20/ 20 with 6/ 6 AOHRR color plates appreciated and visual field full. In the right eye, there was no light perception, From the Departments of Ophthalmology and Neurology ( J. M. S. W.), Cornell University Medical College, and Division of Neuro- Ophthalmology ( J. M. S. W.) North Shore University Hospital- Cornell University Medical College, E. S. Harkness Eye Institute ( J. G. O., M. M. B.), and Department of Radiology ( S. H.), Columbia- Presbyterian Medical Center, New York, New York, U. S. A. Address correspondence and reprint requests to Dr. J. M. S. Winterkorn, Division of Neuro- ophthalmology, 300 Community Drive, Manhasset, New York 11030, U. S. A. and the pupil was amaurotic. The right upper lid was mildly ptotic, and abduction in the right eye was limited by 2 mm. Axial globe proptosis and resistance to retropulsion were noted. The right optic disc was elevated, edematous, and surrounded by flame hemorrhages. The arterioles were narrowed, the veins dark and distended, and the retina appeared pale behind extensive hemorrhage ( Fig. 1A). Computerized tomography ( CT) scans demonstrated moderate fusiform thickening of the right optic nerve ( Fig. 2A), with minimal enlargement of the right optic canal but no intracranial extension and a normal optic chiasm. The sclera and optic nerve sheath enhanced, and the appearance of the orbital fat was consistent with inflammation, vascular congestion, or edema. The child was treated with high doses of corticosteroid for an inflammatory optic neuritis/ perineuritis and followed without biopsy. There was rapid and complete resolution of the abnormalities on magnetic resonance imaging ( MRI) and CT scans ( Fig. 2B) and of the clinical syndrome except for the visual loss from retinal infarction, which was permanent ( Fig. IB). Case 2 A healthy 53- year- old man experienced tenderness, pain, injection, and limitation of abduction in the right eye. Within a week, he developed a partial central retinal artery occlusion with infarction of the lower retina, a cherry- red spot, and diminished ophthalmodynanometry ( systolic 25 OD and 60 OS). CT scan demonstrated an ill- defined den- 257 158 j . M. S. WINTERKORN ET AL. FIG. 1. Case 1. A: Fundus photograph of the right eye of 5- year- old boy demonstrates swollen optic disc and superimposed central retinal artery and vein occlusions. B After treatment of inflammatory perineuritis with high- dose steroids, clinical signs resolved, but vision did not improve, and the retina showed sclerotic vessels and absence of nerve fiber layer. sity around the right optic nerve ( Fig. 3). The patient was treated with oral prednisone for a presumed inflammatory optic neuritis/ perineuritis. His pain and injection resolved promptly and oph-thalmodynanometry subsequently became normal. DISCUSSION In a child with optic nerve enlargement, visual loss and retinal artery and vein occlusions, diagnostic possibilities include optic nerve neoplasm ( e. g. glioma, meningioma), and optic neuritis/ perineuritis, a form of inflammatory pseudotumor ( 3,4). Recent authors have emphasized the diagnostic criteria used to differentiate among these entities by CT scan ( 3- 5). In this case, while the striking enlargement of the optic nerve was consistent with the appearance of glioma, in which case the fatty changes or scleral enhancement could possibly have been attributable to ischemic necrosis, the history of visual loss was acute, and there was no evidence of neurofibromatosis in the child. The CT picture showing enhancement of ad- FIG. 2. Case 1. A: CT scan with contrast before treatment shows inflammatory perineuritis. B: CT scan with contrast after treatment shows resolution of abnormality. / Neuro- Ophtluilmol, Vol. 14, No. 3, 1994 VASCULAR OCCLUSION FROM PSEUDOTUMOR 159 FIG. 3. Case 2. CT scan of orbit showing enlarged superior ophthalmic vein. ventitia around a swollen optic nerve and areas of enhancement in orbital fat ultimately was interpreted as inflammatory optic neuritis/ perineuritis. The CT scan of the adult also was considered diagnostic of idiopathic orbital inflammation. The accompanying clinical signs in each patient- swelling, proptosis, and motility dysfunction with pain- were supportive of the diagnosis of idiopathic orbital inflammation ( 4,6). A substantially swollen optic nerve with superimposed vascular occlusion prompts investigation for orbital tumor. Central vein and artery occlusion have been associated with malignant optic glioma in the adult ( 7,8), and venous stasis retinopathy has been observed in a child with optic glioma ( 3). In a seeing eye, biopsy of optic nerve usually is not recommended ( 4,7), although in the blind eye of the child described here, biopsy or excision of the nerve might well have done no harm. However, both the child and the adult were presumed to have a form of orbital inflammatory syndrome involving the optic nerve and its sheath. Biopsy was deferred in these patients and treatment with corticosteroids resulted in improvement. Although one case has been reported ( 9) in which a vein occlusion accompanied orbital pseudotumor, the cases presented here are the first reports of retinal artery and vein occlusion owing to optic neuritis/ perineuritis in that entity. Vascular occlusions may result from compression of the vessels within the inflamed optic nerve sheath; a vasculitic basis seems less likely. These cases demonstrate that idiopathic orbital inflammation can produce vein and artery occlusions, and reinforce the need for rapid diagnosis and treatment. REFERENCES 1. Gass, J. Donald M. Stereoscopic atlas of macular diseases, diagnosis and treatment, 3rd Ed. St. Louis, MO: Mosby, 19: 354- 8. 2. Buchanan TAS, Hoyt WF. Optic nerve glioma and neovas-cular glaucoma: a report of a case. Br j Ophthalmol 1982; 66: 96- 8. 3. Trokel SL, Hilal SK. Submillimeter resolution CT scanning of orbital diseases. Ophthalmology 1980; 87: 412- 17. 4. Kennerdell JS, Dresner SC. The nonspecific orbital inflammatory syndromes. Surv Ophthalmol 1984; 29: 93- 103. 5. Rothfus WE, Curtin HD, Slamovits TL, Kennerdell JS. Optic nerve sheath enlargement. Radiology 1984; 150: 409- 15. 6. Mottow LS, Jakobiec FA. Idiopathic inflammatory orbital pseudotumor in childhood. I. Clinical characteristics. Arch Ophthalmol 1978; 96: 1410- 17. 7. Hoyt WF, Meshel LG, Lessel S, Schatz NJ, Suckling RD. Malignant optic nerve glioma of adulthood. Brain 1973; 96: 121- 32. 8. Spoor TC, Kennerdell JS, Martinez J, Zorub D. Malignant gliomas of the optic nerve pathways. Am / Ophthalmol 1980; 89: 282- 92. 9. Coop ME. Pseudotumor of the orbit. Br / Ophthalmol 1961; 45: 513- 42. / Nvuro- Ophtlialmol, Vol. 14. No. .3, 1994 |
