ACTA OPHTHALMOLOGICA VOL. 57 1979
Department of 0,bhthalmology (Heads: P. M. Msller, E. Goldschmidt, S. Faurschou), University Hospital, Odense, Denmark
CAPILLARY HAEMANGIOMA OF THE OPTIC DISC A Case Report BY
P. GILBERT NIELSEN
A case of capillary haemangioma of the optic disc is reported. The ophthalmoscopic and fluorescein angiographic characteristics of the different varieties of haemangioma of the optic nerve head are discussed and the problems of treatment by photocoagulation outlined.
Key words: hacmangioma - optic disc - fluorescein angiography - photocoagulation.
Angiomatosis retinae and von Hippel’s disease are terms used synonymously to describe capillary angiomatous hamartomas of the retina and the optic nerve (Gass 1974). T h e development of an angioma in the optic disc or juxtapapillary retina is very uncommon. This report documents, with the help of fluorescein angiography, a case of capillary haemangioma of the optic disc. T h e various types of angiomas of the optic nerve head are tabulated and the problems of management outlined.
Case Report A 24-year-old woman complained of blurred vision in the left eye some two months prior to being referred to the University Eye Clinic, Odense, in November 1976 for examination. No previous eye symptoms. There was nothing unusual in her past medical history. A younger sister suffers from epilepsy of unknown aetiology. Received June 28, 1978.
63
P . Gilbert Nielsen
Fig. I . November 1976. Reddish, slightly elevated turiiour on the left optic disc with cystic detachment and exudation. Visual acuity 6/6.
Figs. 2 and 3. Fig. 2 . Angiogram arterial phase showing tumour-vessels arising from the optic disc. Fig. 3. Late angiograni showing massive dyeleakage.
64
Capillnry Huemangionta o / the Optic Disc
Visual acuity was 6/6 emmetropic in both eyes. Right eye was normal. Funduscopic examination of the left eye revealed a reddish tumour arising from the lower half of the optic disc and adjacent peripapillary retina. A double disc-sized, cystic detachment extended downwards; discrete, yellowish exudates a t the margins of this detachment were noted. Perimetry showed enlargement of the blind spot and a paracentral scotoma; this finding corresponding well to those of the ophthalmoscopy. T h e intraocular tension was normal. Fluorescein angiography demonstrated, in the retinal arterial phase, a wellcircumscribed mass of small calibre vessels, confined to the area around the lower margin of the optic disc. Intraretinal leakage of the dye from the tumour was seen in the intermediate arteriovenous phase, and late angiograms showed massive dye leakage. T h e calibration of the large central vessels was normal and no evidence of pigment epithelial or retinal abnormality outside the im-
Fig. 4.
July 1977. Central exudation and cystic detachment progressing. Note photocoagulationsmarks round the periphery. Applications on tumour-vessels not visible. 65 . k l a ophthal. 5 i . I
,
mediate area of the slightly elevated optic nerve head tumour could be observed. Some progression of the detachment and the exudation were noted during repeated follow-up examinations over the next six months. Visual acuity remained 6 / 6 and the visual field defect was stationary. She was admitted for a general, thorough evaluation in February 1977. No neurologic or other abnormality was found. Visual acuity in the left eye had become reduced to 619 by June 1977. Further progression of the detachment was noted, and the exudation had increased, with central oedema and the development of a macular star figure. She was subjected to three applications of argon laser photocoagulation between June and November 1977. T h e photocoagulation was aimed directly onto the tumour vessels and round the periphery of the lesion. After a temporary improvement in visual acuity to 6/6 and apparently some regression in the affection, the situation again deteriorated, with a gradual reduction in visual
Fig. 5. April 1978. Complete macular star developed. Visual acuity reduced to 6/36,
Capillnry Haemungionzn
of
the Optic Disc
acuity to 6/36 and the development of a complete macular star figure in January 1978. T h e visual acuity was unchanged and the exudation still progressing, when last seen in April 1978.
Discussion Three varieties of haemangioma of the optic nerve head have been noted (Henkind & Benjamin 1976) a) T h e racemose haemangioma is quite rare but may be part of the WyburnMason syndrome with associated midbrain lesions or occurs as an isolated entity. T h e retina usually shows almost no clinical reaction, no haemorrhage, oedema or exudation. T h e bizarre vessel-configurations may approach the macula very closely without interfering with visual acuity. Fluorescein angiography is helpful in demonstrating the abnormal arteriovenous connections. Quiescent cases show no vascular fluorescein leakage (Henkind et al. 1971). b) T h e cavernous haemangioma is often asymptomatic and appears to be identical to intraretinal cavernous haemangiomata. T h e condition is not related to von Hippel-Lindau’s disease. T h e fluorescein angiographic appearance is characteristic with the vascular spaces filling very slowly and often with a fluid level. No dye leakage occurs (MacDonald et al. 1975). c) T h e capillary haemangioma range in appearance from small well-circumscribed, elevated lesions obscuring part of the disc to large epipapillary vascular masses. Bilateral cases of papillary haemangiomatosis have been reported (MacNair et al. 1966), but on the whole the condition seems to be almost invariably unilateral. T h e usual type of retinal haemangiomatosis is bilateral in 50°/o of the cases (Duke-Elder 1967). Unlike retinal haemangiomatosis, dilatation and tortousity of the feeder vessels do not occur or are not visible in those angiomas arising from or immediately adjacent to the optic nerve head and angiographic evidence of arteriovenous shunting is difficult or impossible to demonstrate (Gass 1974). Johnson et al. (1976) found, in an ultrastructural study, the endothelial cells of the capillary haemangioma to be phenestrated, thus providing an explanation of the fluorescein leakage and the extravasated exudate that is characteristic of this tumour. About one quarter of patients with capillary haemangiomas have other clinical evidence of von Hippel-Lindau’s disease (Duke-Elder 1967). Visual loss occurs in more than half of the cases of capillary haemangioma and is generally the results of chronic macular oedema. Vitreous haemorrhage and retinal detachment may also occur (MacDonald et al. 1975). 67
Photocoagulation is the treatment of choice in retinal haemangiomatosis, but is, for obvious reasons, a risky venture when dealing with papillary-juxtapapillary lesions. The risk of destroying useful central vision is obvious, particularly in those located on or surrounding the temporal margin of the optic disc. Photocoagulations of selected portions of the tumour may be successful in temporarily reducing the degree of intraretinal and subretinal exudation. The long-term prognosis for central vision in these papillary and peripapillary tumours is, however, poor and photocoagulation should not be done in patients with normal visual acuity (Gass 1974). Repeated photocoagulations have not been successful in arresting the exudative changes and vision has decreased from 616 to 6/36 over the relatively short period of observation in the reported case.
References Duke-Elder S. (1967) System of o ~ l r f h u l m o l o g y Vol. , X,p. 741-752. Kimpton, London. Gass J. D. M. (1974) Diffcrrntinl rlicignosis of intmortrlrrr trrmors, p. 265-267. Mosby, St. Louis. Henkind P. BC Benjamin I. V. (1976) Vascular anomalies and neoplasms of the optic nerve head. Trans. ophthnl. Soc. l r .K . 96, 418-423. Henkind P. et al. (1971) T h e retinril circulntion, p. 263. Harper and Row, New York. Johnson F. B. et al. (1976) Angiomatosis retinae. Cancer 38, 2042-2056. MacDonald P. R. et al. (197.5) Hemangiomas of the optic disc. Canad. 1. Oplithnl. 10, 305-3 17 . MacNair I. N. et al. (1966) Bilateral peripapillary retinae hemangiomas. Arch. OPltllial. (Chicago) 75, 77-81.
Artlhor’s uddress: P. Gilbert Nielsen, Dept. of Ophthalmology, University Hospital, 5000 Odense. Denmark.
(8i
Department of 0,bhthalmology (Heads: P. M. Msller, E. Goldschmidt, S. Faurschou), University Hospital, Odense, Denmark
CAPILLARY HAEMANGIOMA OF THE OPTIC DISC A Case Report BY
P. GILBERT NIELSEN
A case of capillary haemangioma of the optic disc is reported. The ophthalmoscopic and fluorescein angiographic characteristics of the different varieties of haemangioma of the optic nerve head are discussed and the problems of treatment by photocoagulation outlined.
Key words: hacmangioma - optic disc - fluorescein angiography - photocoagulation.
Angiomatosis retinae and von Hippel’s disease are terms used synonymously to describe capillary angiomatous hamartomas of the retina and the optic nerve (Gass 1974). T h e development of an angioma in the optic disc or juxtapapillary retina is very uncommon. This report documents, with the help of fluorescein angiography, a case of capillary haemangioma of the optic disc. T h e various types of angiomas of the optic nerve head are tabulated and the problems of management outlined.
Case Report A 24-year-old woman complained of blurred vision in the left eye some two months prior to being referred to the University Eye Clinic, Odense, in November 1976 for examination. No previous eye symptoms. There was nothing unusual in her past medical history. A younger sister suffers from epilepsy of unknown aetiology. Received June 28, 1978.
63
P . Gilbert Nielsen
Fig. I . November 1976. Reddish, slightly elevated turiiour on the left optic disc with cystic detachment and exudation. Visual acuity 6/6.
Figs. 2 and 3. Fig. 2 . Angiogram arterial phase showing tumour-vessels arising from the optic disc. Fig. 3. Late angiograni showing massive dyeleakage.
64
Capillnry Huemangionta o / the Optic Disc
Visual acuity was 6/6 emmetropic in both eyes. Right eye was normal. Funduscopic examination of the left eye revealed a reddish tumour arising from the lower half of the optic disc and adjacent peripapillary retina. A double disc-sized, cystic detachment extended downwards; discrete, yellowish exudates a t the margins of this detachment were noted. Perimetry showed enlargement of the blind spot and a paracentral scotoma; this finding corresponding well to those of the ophthalmoscopy. T h e intraocular tension was normal. Fluorescein angiography demonstrated, in the retinal arterial phase, a wellcircumscribed mass of small calibre vessels, confined to the area around the lower margin of the optic disc. Intraretinal leakage of the dye from the tumour was seen in the intermediate arteriovenous phase, and late angiograms showed massive dye leakage. T h e calibration of the large central vessels was normal and no evidence of pigment epithelial or retinal abnormality outside the im-
Fig. 4.
July 1977. Central exudation and cystic detachment progressing. Note photocoagulationsmarks round the periphery. Applications on tumour-vessels not visible. 65 . k l a ophthal. 5 i . I
,
mediate area of the slightly elevated optic nerve head tumour could be observed. Some progression of the detachment and the exudation were noted during repeated follow-up examinations over the next six months. Visual acuity remained 6 / 6 and the visual field defect was stationary. She was admitted for a general, thorough evaluation in February 1977. No neurologic or other abnormality was found. Visual acuity in the left eye had become reduced to 619 by June 1977. Further progression of the detachment was noted, and the exudation had increased, with central oedema and the development of a macular star figure. She was subjected to three applications of argon laser photocoagulation between June and November 1977. T h e photocoagulation was aimed directly onto the tumour vessels and round the periphery of the lesion. After a temporary improvement in visual acuity to 6/6 and apparently some regression in the affection, the situation again deteriorated, with a gradual reduction in visual
Fig. 5. April 1978. Complete macular star developed. Visual acuity reduced to 6/36,
Capillnry Haemungionzn
of
the Optic Disc
acuity to 6/36 and the development of a complete macular star figure in January 1978. T h e visual acuity was unchanged and the exudation still progressing, when last seen in April 1978.
Discussion Three varieties of haemangioma of the optic nerve head have been noted (Henkind & Benjamin 1976) a) T h e racemose haemangioma is quite rare but may be part of the WyburnMason syndrome with associated midbrain lesions or occurs as an isolated entity. T h e retina usually shows almost no clinical reaction, no haemorrhage, oedema or exudation. T h e bizarre vessel-configurations may approach the macula very closely without interfering with visual acuity. Fluorescein angiography is helpful in demonstrating the abnormal arteriovenous connections. Quiescent cases show no vascular fluorescein leakage (Henkind et al. 1971). b) T h e cavernous haemangioma is often asymptomatic and appears to be identical to intraretinal cavernous haemangiomata. T h e condition is not related to von Hippel-Lindau’s disease. T h e fluorescein angiographic appearance is characteristic with the vascular spaces filling very slowly and often with a fluid level. No dye leakage occurs (MacDonald et al. 1975). c) T h e capillary haemangioma range in appearance from small well-circumscribed, elevated lesions obscuring part of the disc to large epipapillary vascular masses. Bilateral cases of papillary haemangiomatosis have been reported (MacNair et al. 1966), but on the whole the condition seems to be almost invariably unilateral. T h e usual type of retinal haemangiomatosis is bilateral in 50°/o of the cases (Duke-Elder 1967). Unlike retinal haemangiomatosis, dilatation and tortousity of the feeder vessels do not occur or are not visible in those angiomas arising from or immediately adjacent to the optic nerve head and angiographic evidence of arteriovenous shunting is difficult or impossible to demonstrate (Gass 1974). Johnson et al. (1976) found, in an ultrastructural study, the endothelial cells of the capillary haemangioma to be phenestrated, thus providing an explanation of the fluorescein leakage and the extravasated exudate that is characteristic of this tumour. About one quarter of patients with capillary haemangiomas have other clinical evidence of von Hippel-Lindau’s disease (Duke-Elder 1967). Visual loss occurs in more than half of the cases of capillary haemangioma and is generally the results of chronic macular oedema. Vitreous haemorrhage and retinal detachment may also occur (MacDonald et al. 1975). 67
Photocoagulation is the treatment of choice in retinal haemangiomatosis, but is, for obvious reasons, a risky venture when dealing with papillary-juxtapapillary lesions. The risk of destroying useful central vision is obvious, particularly in those located on or surrounding the temporal margin of the optic disc. Photocoagulations of selected portions of the tumour may be successful in temporarily reducing the degree of intraretinal and subretinal exudation. The long-term prognosis for central vision in these papillary and peripapillary tumours is, however, poor and photocoagulation should not be done in patients with normal visual acuity (Gass 1974). Repeated photocoagulations have not been successful in arresting the exudative changes and vision has decreased from 616 to 6/36 over the relatively short period of observation in the reported case.
References Duke-Elder S. (1967) System of o ~ l r f h u l m o l o g y Vol. , X,p. 741-752. Kimpton, London. Gass J. D. M. (1974) Diffcrrntinl rlicignosis of intmortrlrrr trrmors, p. 265-267. Mosby, St. Louis. Henkind P. BC Benjamin I. V. (1976) Vascular anomalies and neoplasms of the optic nerve head. Trans. ophthnl. Soc. l r .K . 96, 418-423. Henkind P. et al. (1971) T h e retinril circulntion, p. 263. Harper and Row, New York. Johnson F. B. et al. (1976) Angiomatosis retinae. Cancer 38, 2042-2056. MacDonald P. R. et al. (197.5) Hemangiomas of the optic disc. Canad. 1. Oplithnl. 10, 305-3 17 . MacNair I. N. et al. (1966) Bilateral peripapillary retinae hemangiomas. Arch. OPltllial. (Chicago) 75, 77-81.
Artlhor’s uddress: P. Gilbert Nielsen, Dept. of Ophthalmology, University Hospital, 5000 Odense. Denmark.
(8i
