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Case Reports and Small Case Series
February 2000

Capillary Hemangioma of the Optic Nerve Head and Juxtapapillary Retina

Arch Ophthalmol. 2000;118(2):289-291. doi:

A 24-year-old man was noted to have decreased vision in the left eye on a routine eye examination. Funduscopy revealed a mass overlying the optic nerve head and adjacent superotemporal retina in the left eye, as well as a large amount of exudate in the macula. Fluorescein angiography demonstrated early hyperfluorescence of the lesion and late pooling of the dye, outlining the retinal detachment. A diagnosis of capillary hemangioma of the optic nerve head and juxtapapillary retina was made. The lesion was treated with argon laser photocoagulation. Subsequent progression of the tumor led to a total retinal detachment and rubeosis iridis and resulted in enucleation. Histopathologic examination of the lesion revealed a mass composed of fine capillaries, lined with endothelium, and filled with red blood cells. Capillary hemangioma of the optic nerve head and juxtapapillary retina is rare, and only a few reports of its histopathologic characteristics exist. The association of the tumor with von Hippel-Lindau syndrome should be considered by ophthalmologists.

Retinal capillary hemangioma is a rare, benign vascular tumor of the retina. Two variants of the tumor are recognized: peripheral retinal capillary hemangioma and the less frequent capillary hemangioma of the optic nerve head and/or juxtapapillary retina. Either variant can be associated with von Hippel-Lindau syndrome. Retinal capillary hemangioma usually occurs in the second or third decade of life with equal frequency in both sexes and no racial predilection. Hereditary and nonhereditary forms exist. Initial symptoms include decreased visual acuity and visual field loss. The lesion may be asymptomatic and discovered on a routine ophthalmic examination. An untreated tumor frequently enlarges, and, over time, leads to progressive subretinal and intraretinal exudation and retinal detachment. We present a case of a capillary hemangioma of the optic nerve head and juxtapapillary retina in a 24-year-old man.

Report of a Case

A 24-year-old white man was noted to have decreased visual acuity in the left eye on a routine eye examination. He had not been aware of the visual loss. His medical history was unremarkable. Visual acuity was 20/20 OD and counting fingers at 1.2 m (4 ft) in the left eye. On dilation of the pupil, a discrete, elevated, reddish mass obscuring the optic nerve head was noted in the left eye (Figure 1). The elevated portion of the mass was approximately 4.5 mm in diameter. Contact B-scan ultrasonography demonstrated the mass measured 5.4 mm thick and 11.5 mm × 9.4 mm in diameter at the base. Dilated, tortuous feeder vessels, arising from the optic disc, were seen on the surface of the lesion. Subretinal lipid exudate surrounded the lesion, and an exudative retinal detachment involving the macula was present. No other hemangiomatous lesions were present in the left eye. Results of the fundus examination of the right eye were normal. Fluorescein angiography demonstrated early hyperfluorescence of the vascular lesion and late pooling of the dye, outlining the retinal detachment (Figure 2).

Figure 1.
Fundus photograph of the left eye showing a discrete, elevated, reddish lesion obscuring the optic nerve head. Note the surrounding subretinal lipid exudate.

Fundus photograph of the left eye showing a discrete, elevated, reddish lesion obscuring the optic nerve head. Note the surrounding subretinal lipid exudate.

Figure 2.
Fluorescein angiogram demonstrating hyperfluorescence of the lesion in the late venous stage.

Fluorescein angiogram demonstrating hyperfluorescence of the lesion in the late venous stage.

Based on the clinical appearance of the mass and the supporting diagnostic studies, a diagnosis of capillary hemangioma of the optic nerve head and juxtapapillary retina was made. The tumor was treated with argon laser photocoagulation on 2 occasions. Following this treatment, the patient was lost to follow-up for 8 months. Upon his return to care, he had developed a total retinal detachment and rubeosis iridis, and the eye was enucleated. Although the patient had no family history or symptoms suggesting von Hippel-Lindau syndrome, he was advised to undergo testing to rule out cerebellar hemangioblastoma or other visceral tumors, but he failed to comply with that recommendation.

The gross section of the enucleated eye revealed a funnel-shaped retinal detachment with xanthochromic subretinal fluid containing refractile particles and a white mass lesion (Figure 3). The mass, measuring 4.0 mm wide and 3.0 mm thick, arose mainly from the optic nerve head and involved the inner retina superotemporally. Histological examination demonstrated a total retinal detachment with a massive amount of subretinal fluid with numerous cholesterol clefts, corresponding to the refractile particles seen on a gross section (Figure 4). A lobulated mass composed of fine capillaries lined with normal endothelium and filled with red blood cells was seen. The vascular channels were separated by vacuolated interstitial cells (Figure 5). In the anterior segment of the eye, rubeosis iridis and anterior displacement of the iris and lens were apparent.

Figure 3.
Photograph of gross section of the enucleated eye showing a funnel-shaped retinal detachment with xanthochromic subretinal fluid and mass overlying the optic nerve head.

Photograph of gross section of the enucleated eye showing a funnel-shaped retinal detachment with xanthochromic subretinal fluid and mass overlying the optic nerve head.

Figure 4.
Low-magnification photomicrograph demonstrating retinal detachment with a massive amount of subretinal fluid with numerous cholesterol clefts (hematoxylin-eosin, ×2). Note the elevated lesion overlying the optic nerve head.

Low-magnification photomicrograph demonstrating retinal detachment with a massive amount of subretinal fluid with numerous cholesterol clefts (hematoxylin-eosin, ×2). Note the elevated lesion overlying the optic nerve head.

Figure 5.
Photomicrograph showing fine capillaries lined with normal endothelium and filled with red blood cells (hematolyxin-eosin, ×400).

Photomicrograph showing fine capillaries lined with normal endothelium and filled with red blood cells (hematolyxin-eosin, ×400).

Comment

Capillary hemangioma of the retina most likely represents a vascular hamartoma and was first described by von Hippel in 1904. Lindau reported the association between retinal capillary hemangiomas and central nervous system tumors. The term "von Hippel-Lindau syndrome" is applied to an autosomal dominant disorder, recently linked to abnormalities in the short arm of chromosome 3 (locus 3p25),1 characterized by cerebellar and spinal cord hemangioblastomas, renal cell carcinoma, pheochromocytoma, and retinal angiomas.

Peripheral capillary hemangioma of the retina is often multifocal and bilateral and has the characteristic appearance of a reddish or gray nodule in the peripheral retina supplied by enlarged, tortuous feeder vessels. The well-circumscribed endophytic capillary hemangioma arising from the optic nerve head can also be recognized clinically without difficulty. When sessile or exophytic, capillary hemangioma of the juxtapapillary retina and optic nerve head may be misdiagnosed as unilateral papilledema, papillitis, choroiditis, choroidal neovascularization, or choroidal hemangioma.2

Stereoscopic fluorescein angiography is often helpful in establishing the diagnosis of retinal angioma.2 Fluorescein angiography shows hyperfluorescence of the vascular lesion in the middle and later retinal stages, often followed by continuous leakage from the lesion in the later stages of the study. Dilated feeder vessels can be readily visualized with angiography.

Histologically, the tumor is composed of numerous capillaries lined by normal endothelium, separated by plump, vacuolated interstitial cells, containing lipidlike material.3 Juxtapapillary capillary angioma has been shown histologically to have a choroidal as well as a retinal blood supply.3 With time, the capillaries within the hemangioma become incompetent, which presumably leads to progressive subretinal and intraretinal exudation in the macula, often resulting in a total retinal detachment. Spontaneous regression of a retinal hemangioma has been described, but this is a rare occurrence.4

Eyes with untreated retinal angiomas tend to have a poor prognosis because of the associated macular exudate and exudative retinal detachment. Early treatment of peripheral capillary hemangioma lesions with argon and xenon photocoagulation is advocated and tends to be more successful with tumor size of 1 disc diameter or smaller.5 Multiple treatment sessions with moderate-intensity, prolonged duration photocoagulation are recommended. Other treatment modalities such as cryotherapy, diathermy, and proton beam irradiation have been used as well. Photocoagulation of juxtapapillary lesions is associated with poor outcomes,6 since destruction of nerve fiber layer in the macula during photocoagulation may contribute to the visual loss. Therefore, treatment of juxtapapillary angiomas is generally not recommended until macular exudation threatens the central vision.2 It is also unlikely for photocoagulation to penetrate a large endophytic lesion, such as the one described in this case report.

Retinal capillary hemangioma is often associated with von Hippel-Lindau syndrome, but the actual incidence is not precisely known and may have been underestimated in previous studies. Schindler et al6 found a 24% incidence of von Hippel-Lindau syndrome or a positive family history in 55 patients with capillary angioma of the optic disc.6 Thus, it is important to obtain a family history and refer the affected individual for the appropriate systemic studies, which include magnetic resonance imaging of the brain and spinal cord to rule out hemangioblastomas, and abdominal computed tomography to rule out renal and other visceral cysts. Urinary studies for pheochromocytoma have also been advocated. The patient's relatives should undergo a dilated fundus examination to exclude retinal angiomas.

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Article Information

This study was supported in part by grants from the St Giles Foundation, New York, NY, and by Research to Prevent Blindness, New York, NY.

Corresponding author: Barrett G. Haik, MD, Department of Ophthalmology, University of Tennessee, 956 Court Ave, Suite D228, Memphis, TN 38163.

References
1.
Seizinger  BRRouleau  GAOzelius  LJ von Hippel-Lindau disease maps to the region of chromosome 3 associated with renal cell carcinoma. Nature. 1988;332268- 269Article
2.
Gass  JDMBraunstein  R Sessile and exophytic capillary angiomas of the juxtapapillary retina and optic nerve head. Arch Ophthalmol. 1980;981790- 1797Article
3.
Nicholson  DHGreen  WRKenyon  KR Light and electron microscopic study of early lesions in angiomatosis retinae. Am J Ophthalmol. 1976;82193- 204
4.
Whitson  JTWelch  RBGreen  WR von Hippel Lindau disease: case report of a patient with spontaneous regression of a retinal angioma. Retina. 1986;6253- 259Article
5.
Gass  JDM Treatment of retinal vascular anomalies. Trans Am Acad Ophthalmol Otolaryngol. 1977;83432- 442
6.
Schindler  RFSarin  LKMacDonald  PR Hemangiomas of the optic disc. Can J Ophthalmol. 1975;10305- 318
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