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Show Hemorrhage Within the Optic Nerve From a Cavernous Hemangioma of the Optic Disc Seanna R. Grob, MD, MAS, Ashley A. Campbell, MD, Andrew Gross, MD, Dean M. Cestari, MD Abstract: A 49-year-old woman with a known right optic disc cavernous hemangioma experienced pain with eye move-ments and worsening of a superior visual field defect. While she retained 20/20 visual acuity in each eye, findings on magnetic resonance imaging were consistent with a hemor-rhage in the anterior portion of the right intraorbital optic nerve. Her visual function stabilized spontaneously. We are unaware of previous reports of hemorrhage into the optic nerve from a cavernous hemangioma of the optic disc. Journal of Neuro-Ophthalmology 2015;35:277-279 doi: 10.1097/WNO.0000000000000274 © 2015 by North American Neuro-Ophthalmology Society A49-year-old woman with a known right optic disc cav-ernous hemangioma diagnosed incidentally at age 10 years was hospitalized for severe gastroenteritis 2 weeks before presentation. Five days before presentation, she awoke with pain behind the right eye but did not seek care due to her systemic illness. The night before, she had several episodes of forceful vomiting. On our initial evaluation, the patient reported right retro-orbital pain that increased with eye movements and worsening of a known superior visual field defect in the right eye. Visual acuity was 20/20 bilaterally. While color vision was intact in each eye, the Ishihara color plates were read more slowly with the right eye and there was a right relative afferent pupillary defect. There was no proptosis and extraocular movements were intact. Slit lamp examination showed normal anterior segments with normal intraocular pressures. Ophthalmoscopy revealed a vascular anomaly on the right optic disc that had the appearance of a cluster of grapes consistent with FIG. 1. Cavernous hemangioma of the optic disc. A. Vascular tumor is present on the surface of the right optic disc with engorgement of the retinal venous system. B. Automated right visual field shows a superior altitudinal defect. C. On fluo-rescein angiography, there is pooling of dye in a cluster-like formation without leakage. D. Optical coherence tomography reveals an elevated mass on the optic disc with oval hyporeflective areas. Neuro-Ophthalmology (AG, DMC), Massachusetts Eye and Ear Infirmary, Boston, Massachusetts; and Department of Ophthal-mology (SRG, AC, AG, DMC), Harvard Medical School, Boston, Massachusetts. The authors report no conflicts of interest. Address correspondence to Dean M. Cestari, MD, Neuro- Ophthalmology, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114; E-mail: dean_cestari@meei.harvard.edu Grob et al: J Neuro-Ophthalmol 2015; 35: 277-279 277 Photo Essay Section Editor: Timothy J. McCulley, MD Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. a cavernous hemangioma (Fig. 1A) and a normal left optic disc. The maculae and peripheral retina were nor-mal in both eyes. Automated visual field testing demon-strated a superior altitudinal defect in the right eye (Fig. 1B) while the left visual field was normal. Fluorescein angiography showed plasma-erythrocyte separation within the hemangioma with late pooling in a cluster-like formation on the optic disc (Fig. 1C). Optical coherence tomography revealed an elevated lesion of the right optic disc containing hyporeflective oval spaces (Fig. 1D). Orbital magnetic resonance imaging (MRI) showed changes in the anterior and midorbital portions of the right optic nerve (Fig. 2). On the T2 axial images, 2 loculated foci with fluid-fluid levels were apparent. Posterior to these foci was an area of T2 signal dropout. These findings were consistent with hemorrhage of different ages within the optic nerve with various stages of blood breakdown products. Over the next 2 months, the patient's vision remained stable. At her 5-month follow-up visit, she reported wors-ening vision in the right eye. Visual field testing showed progression of her right superior altitudinal defect, but her orbital MRI was unchanged. In 1971, Gass (1) first described cavernous hemangi-oma of the optic disc. This is a rare vascular hamartoma consisting of numerous, clustered, thin-walled aneur-ysms, appearing like a "bunch of grapes." On fluorescein angiography, there is a slow rate of dye perfusion within the lesion and plasma-erythrocyte separation showing a fluid level within the aneurysms (2). Plasma-erythrocyte separation occurs during the late venous phase when the fluorescein pools superiorly in the plasma resulting in hyperfluorescence while erythrocytes collect inferiorly. Greater than 90% of hemorrhages due to hemangioma of the optic disc are unilateral and approximately one quarter are detected within the first 3 decades of life (2). They are more prevalent in females and can be associated with other skin and central nervous system hamartomas (2,3). Fortunately, cavernous hemangiomas of the optic disc often are asymptomatic with preserved visual acuity and a stable clinical course (1,3-5). Visual field defects com-monly reveal an enlargement of the blind spot and/or nerve fiber bundle defects (2,6). Usually they do not enlarge (2); however, there are cases of documented growth over 5-10 years (7). Cavernous hemangiomas of the optic disc are not without complications. Cases of vitreous and retinal hemorrhage from these lesions previously have been described (2,3,7,8). In a report by Kuschner et al (7), one patient had a vitreous hemorrhage that was signifi-cant enough to require vitrectomy. In cases requiring surgery, removal of portions of the tumor has been per-formed to reduce the risk for significant hemorrhage (3). More typically, the vitreous hemorrhages are small but have been known to recur (2,3). In these cases, observa-tion usually is sufficient. There are also reports of hemorrhage from optic disc cavernous hemangiomas during pregnancy (6,9). In some instances, the cause of the hemorrhage may be from Valsalva maneuvers during labor, increasing venous conges-tion and enlargement of the thin-walled aneurysms in these lesions. Our patient was recovering from gastroenteritis, during which she described multiple episodes of forceful emesis shortly before the development of eye pain. With the previous reports of Valsalva-induced hemor-rhages, it seems likely that our patient's vomiting episodes provided a similar mechanism for the develop-ment of hemorrhage, which extended into the orbital portion of the optic nerve. FIG. 2. Axial (A) and coronal (B) T2 magnetic resonance imaging shows areas of variable density (arrows) in the anterior portion of the right optic nerve. 278 Grob et al: J Neuro-Ophthalmol 2015; 35: 277-279 Photo Essay Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. REFERENCES 1. Gass JD. Cavernous hemangioma of the retina. A neuro-oculo-cutaneous syndrome. Am J Ophthalmol. 1971;71:799-814. 2. Lewis RA, Cohen MH, Wise GN. Cavernous haemangioma of the retina and optic disc. A report of three cases and a review of the literature. Br J Ophthalmol. 1975;59:422-434. 3. Haller JA, Knox DL. Vitrectomy for persistent vitreous hemorrhage from a cavernous hemangioma of the optic disk. Am J Ophthalmol. 1993;116:106-107. 4. Mansour AM, Jampol LM, Hrisomalos NF, Greenwald M. Case report. Cavernous hemangioma of the optic disc. Arch Ophthalmol. 1988;106:22. 5. Patikulsila D, Visaetsilpanonta S, Sinclair SH, Shields JA. Cavernous hemangioma of the optic disk. Retina. 2007;27:391-392. 6. Vela JI, Garcia-Vilaro M, Buil JA. Haemorrhage of a cavernous haemangioma of the optic disc in pregnancy. BMJ Case Rep. 2010;doi: 10.1136/bcr.03.2010.2806. 7. Kushner MS, Jampol LM, Haller JA. Cavernous hemangioma of the optic nerve. Retina. 1994;14:359-361. 8. Spencer WH. Ophthalmic Pathology: An Atlas and Textbook, 3rd edition. Philadelphia, PA: W.B. Saunders, 1985. 9. Smith BT, Joseph DP. Labor-induced hemorrhage of a retinal cavernous hemangioma. Ophthalmic Surg Lasers Imaging. 2009;40:419-420. Grob et al: J Neuro-Ophthalmol 2015; 35: 277-279 279 Photo Essay Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. |
