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Show J. elin. Nturo-ophthalmol. 4: 159-162, 1984. Capillary Hemangioma of the Optic Disc TOSHIHIRO TAKAHASHI, M.D. HAJIME WADA, M.D. EMIKO TANI, M.D. AKIRA NAKAMURA, M.D. KUNIO HIRAMATSU, M.D. Abstract Two cases of capillary hemangioma of the optic disc (one endophytic and the other exophytic) unassociated with central nervous system involvement are reported. Ophthalmoscopic and fluorescein angiographic findings of cap.ilIary h~m~ngi?mas of. the optic disc are summanzed to aId In differenhating them from cavernous and racemose hemangiomas. Therapeutic problems are also discussed. Introduction Capillary hemangioma, also called. von HippelLindau disease of the optic disc, is uncommon. A survey of the literature according to Darr et al. I in 1966 revealed. only 14 cases of capillary hemangioma of the optic disc. Schnidler et al.2 in 1975 collected 48 cases with this condition from the literature and added their seven cases. Capillary hemangioma of the optic disc may be divided into two types, i.e., endophytic and exophytic types.) Ophthalmoscopically, the endophytic capillary hemangioma presents with characteristic features, while the exophytic capillary hemangioma is more subtle and may often be difficult to differentiate from unilateral papilledema, papillitis, or juxtapapillary retinochoroiditis. We report one case each of endophytic and exophytic capillary hemangioma of the optic disc and discuss the diagnostic and therapeutic problems. Cue Reports CaSt 1 A 54-year-old man consulted. an ophthalmologist for examination. The patient was referred to From the lHp.artment of Ophthalmology (IT, HW, ET), Sch~ of M~kine, Kobe University, Kobe, Japan; Takilsago Mum· riple Hospital (AN), Hyogo, Japan; and Hyogo-Pll'fectural Kaibara Hospital (KH), Hyogo, Japan. September 1984 us on August 10, 1983, because of a lesion of the optic disc. . . Vision was 20/20 in both eyes. Exanunahon of the right eye revealed no remarkable change. The anterior segment and media were intact. Ophthalmoscopy disclosed. a well-circumscribed, reddish, elevated mass on the optic disc (Fig. 1). Fluorescein angiography revealed. numerous capillaries in the lesion and dilated. peripapillary vessels along the inferotemporal portion in the art.eri,aJ phase (Fig. 2), and slow leakage of nuorescem m the venous phase (Fig. 3). Goldmann visual fields were normal. CT scans and carotid as well as vertebral angiograms showed. no abnormalities. The diagnosis of endophytic hemangioma was made. Case 2 An 18-year-old woman was referred. to us on August 19, 1983, complaining of blurred vision in the right eye for 3 weeks. Corrected vision was 20/20 in both eyes. Abnormal ocular findings were limited to the right eye. The anterior segment and media were normal. Fundus examination revealed a blurred, slightly elevated disc margin of the superotemporal portion, small superficial hemorrhage in the peripapillary retina of the inferonasal portion, and hard exudates in the macula (Fig. 4). fluorescein angiography demonstrated numerous fine capillaries of the lesion in the arterial phase (Fig. 5) and tumor stain due to leakage of dye in the venous phase (Fig. 6). Examination of the visual field on the tangent screen showed. a paracentral scotoma and enlargement of the blind spot in the right eye. Laboratory and physical examination involving CT scans and carotid as well as vertebral angiograms disclosed no remarkable change. The diagnosis of exophytic capillary hemangioma was established.. Discussion Vascular abnormalities of the optic disc may be classified into three types, i.e., capillary, cavern- 159 160 Figure 1. Well-circumscribed, reddish mass of optic disc. Figure 2. Arlerial angiogram showing numerous capillaries of mass and dilated peripapillary vessels. Figure 3. Venous angiogram demonstrating hyperOuor!"SCence of mass and leakage of dye. Journal of Clinical NeuTo-ophthalmology Figllr~ 4. Blurnd, ~ightly ~Ifovated disc margin of SI.Ip«Ol~mpor.a1 por. tion. Figllr~ 5. Arterial angiogram showing numerolls fine capillaries of lesion. Figur~ 6. Venous angiogram d~tTating tumor sl,in du~ 10 I~ak.lg~ of dy~. ~Iember 1984 161 Capillary Hemangioma ous, and racemose hemangiomas. The capillary hemangioma is a benign proliferation of endothelial cells and pericytes and often may be associated. with von Hippel-Lindau disease.4 This hemangioma appears as a dislocation.3 The endophytic capillary hemangioma appears as a distinctly encapsulated, reddish mass easily seen on the surface of the disc by ophthalmoscopy and rarely as intraretinal exudates and retinal detachment.~' 3. 5 Conversely, the exophytic hemangioma occurs with a blurred, slightly elevated disc margin and appears frequently with yellow intraretinal exudates and retinal serous detachment in the posterior segment. J. 6-9 Fluorescein angiography in both types indicates numerous fine capillaries of the lesion in the arterial phase and marked. hlperfluorescence in the venous phase.~·s.7- The cavernous hemangioma consists of thin-walled cavities lined with a single layer of endothelial cells. This tumor presents with a cluster of dark, saccular venous aneurysms. Fluorescein angiograrchy shows slow transit of dye into the lesion.Y. 4. 0 The racemose hemangioma is a peculiar arteriovenous malformation and may be occasionally associated with Wyburn-Mason syndrome. This malformation appears as abnormal, bizarre, vascular communications involving the optic disc. Fluorescein angiography demonstrates arteriovenous communications and no leakage from the abnormal vessels." 11 Although capillary hemangioma in the retina usually shows a progressive course, the natural course of capillary hemangioma of the optic disc has not yet been fully delineated.. In the followup study of this condition, Oosterhuis and Rubinstein7 and Landbo~ found fair visual prognosis and little evidence of the tumor increasing in size. Conversely, Schindler et al.2 described variable results of the visual prognosis, involving visual loss due to total detachment in nine out of 55 cases. Furthermore, treatment of the capillary hemangioma of the optic disc remains to be elucidated. Darr el al.I and RoselZ found no effect on the tumor after radiation therapy. Schindler et al.l and Nielseng reported cases with deteriorated vision following photocoagulation. Gass and 162 Braunstein9 obtained successful eHect in two out of five cases treated with photocoagulation. In the present two cases, nontreatment was recommended because of normal visual acuity. However, long-term follow-up study needs to be continued. References 1. DafT, J.L.. Hughes, R.P.. Jr., and Me Nair, IN.: Bilateral peripapillary retinal hemangiomas. Arch. aphthalmol. 75: 77-81, 1966. 2. Schindler, R.F., Sarin, L.K., and Mac Donald, P.R.: Hemangiomas of the optic disc. Can. J. aphthalmol. 10: 305-317, 1975. 3. Nicholson, D.H.: Tumors of the optic nerve. In: Neuro-ophthalmology Update, J.L. Smith, Ed. Masson, New York, 1977, chap. 16, pp. 137-140. 4. Schields, J.A.: Diagnosis and Management of Intraocular Tumors. C.V. Mosby, 51. Louis, 1983, chap 18, pp. 534-568. 5. Landbo, K.: A case of optic disc angioma with a fluorescein angiography. Acta Ophthalmol. SO: 431-435,1972. 6. Macmichael, M.: Von Hippel Lindau's disease of the optic disc. Trans. Ophthalmol. Soc. U.K. 90: 877885,1970. 7. Oosterhuis, J.A., and Rubinstein, K.: Haemangioma at the optic disc. Ophthalmologica 164: 362374, 1972. 8. Nielsen, P.G.: Capillary hemangioma of the optic disc. A case report. Acta Ophthalmol. 57: 63-68, 1979. 9. Gass, J.D., and Braunstein, R.: Sessile and exophytic capillary angiomas of the juxtapapillary retina and optic nerve head. Arch. Ophtha/mol. 98: 17901797,1980. 10. Davies, W.s., and Thumin, M.: Cavernous hemangioma of the optic disc and retina. Trans. Am. aphthalmol. Otolaryllgol. 60: 217-218, 1956. 11. Krug, E.F., and Samuels, B.: Venous angioma of the retina, optic nerve, chiasm and brain. Arch. aphthalmol. 8: 871-879, 1932. 12. Rosen, E.: Vascular malformation in the human retina. Am. J. Ophtha/mol. 67: 501-511,1969. Write for reprillts to: Toshihiro Takahashi, M.D., De· partment of Ophthalmology, School of Medicine, Kobe University, Kusunoki-ho, 7-chome, Chuo-ku, Kobe (650), Japan. Journal of Clinical Neuro-ophthalmology [VBwyburnmason] |
