Circumferential Peripheral Retinal Cavernous Hemangioma

Cavernous hemangioma of the retina (CHR) is a rare vascular tumor firstdescribed in 1934 by Niccol and Moore,¹ whotermed this condition angiomatosis retinae. Gass² later recognized CHR as a distinct clinical entity.

Cavernous hemangioma of the retina appears most commonly as a solitaryvascular lesion of limited size (1 or 2 disc diameters) in the midperipheralor peripheral retina, although occasionally the lesion can be found in theposterior pole or optic nerve head.³

In this report, 2 unusual cases of CHR extending 360° in the midperipheralfundus with no other associated vascular anomalies are described. To our knowledge,no other cases of such characteristics have been reported in the literature.

Patient 1. A 21-year-old white man was referred to the Medical Retina Service,Aberdeen Royal Infirmary, Aberdeen, Scotland, in November 2000 with the diagnosisof diffuse retinal hemorrhages in the right eye. He was asymptomatic. Hisocular and medical history was unremarkable. He was born at full term anddid not receive supplementary oxygen. His mother had experienced no problemsduring pregnancy. At birth he was noted to have a strawberry birthmark onthe back of his neck, which resolved spontaneously. His family medical historyincluded glaucoma in his maternal grandfather and a peripheral retinal tearin his maternal grandmother. There was no history of fits in the family.

On examination, best-corrected visual acuity was measured at 20/20 OU.Intraocular pressures and results of the slitlamp examination were normalin both eyes. Results of the fundus examination of the left eye were normal.In the right eye, dilated retinal veins with clusters of saccular dilationsinvolving 360° of the midperipheral retina were seen (Figure 1A). A few retinal hemorrhages adjacent to these vascularanomalies were present, but no hard exudates were seen. The optic nerve andmacula disclosed no abnormalities. No cutaneous angiomatous lesions were present.Results of fundus and cutaneous examinations of his mother, father, and sistershowed no abnormalities.

Fluorescein angiography (FFA) demonstrated delayed perfusion of thelesion with a typical blood-fluorescein interface occurring within the sacculardilations (<Figure 1 B and C).Anterior to this venous malformation, the retina appeared avascular. Therewas no leakage of fluorescein present in the late frames of the angiogram.

At the last follow-up, 28 months after our initial examination, hisvisual acuity remains 20/20 OU, and the lesion remains unchanged.

Patient 2. A 25-year-old white woman was found to have a vascular abnormality inher right eye in February 2000 and was referred to the Medical Retina Service,Aberdeen Royal Infirmary, for evaluation. She was asymptomatic. Her ocularand medical history was unremarkable. She had been born at full term and hadreceived no supplementary oxygen therapy. Her mother had experienced no problemsduring pregnancy.

On examination, her best corrected visual acuity was 20/20 OU. Anteriorsegments and intraocular pressures were normal in both eyes. Results of thefundus examination of the left eye were normal. In the right eye, dilatedveins involved 360° of the midperipheral retina, with clusters of sacculardilations (Figure 2A). There wereno hemorrhages or hard exudates associated with this lesion. Results of ageneral examination disclosed no abnormal neurological signs and no cutaneousangiomas. Results of fundus and cutaneous examinations of her father, mother,and brother showed no abnormalities.

Fluorescein angiography showed delayed perfusion of the involved areas.Slow filling of the saccular aneurysms and typical blood-fluorescein interfaceswithin these saccular dilations were observed. Anterior to the venous malformation,the retina appeared avascular. No fluorescein leakage was seen in the lateframes of the angiogram.

At the last follow-up, 3 years after our initial examination, her visualacuity remains at 20/20 OU, and the CHR remains unchanged.

Cavernous hemangioma of the retina is a rare, benign, often unilateralvascular tumor, usually recognized in young individuals. Female subjects aremore commonly affected. Although most patients with CHR are asymptomatic,neurological symptoms and signs or decreased vision can occur. There is anassociation between CHR and cavernous hemangiomas involving the central nervoussystem. In some of these cases, an autosomal dominant inheritance has beennoted.

Typically, ophthalmoscopy reveals clusters of saccular aneurysms filledwith dark venous blood. Superficial retinal hemorrhages are occasionally seen,but hard exudation has not been observed. In some cases, a gray-white preretinalmembrane can partly obscure the surface of the tumor. Although the retinalvasculature elsewhere is usually normal, other associated vascular anomaliescan occur. In early frames of the FFA, the vascular channels within the CHRremain hypofluorescent. As the angiogram progresses, these vascular channelsfill slowly with the dye. In late frames, a blood-fluorescein interface ischaracteristically observed in the saccular dilations within the tumor. Thisphenomenon seems to be related to sedimentation of erythrocytes in the inferioraspect of the venous aneurysm, which appears hypofluorescent. The presenceof plasma in the superior aspect of this vascular space, which stains withfluorescein, appears hyperfluorescent. There is no extravascular leakage ofdye in CHR.

The patients described herein had unusual CHR. The vascular tumors inthese cases extended circumferentially throughout the 360° of the midperipheralretina. The retina anterior to the cavernous hemangioma seemed to be avascular.To our knowledge, only 1 similar case, published by Messmer and coworkers⁴ in 1983, has been reported in the literature.In that case, however, a large peripheral shunt vessel extended temporallyfrom the superonasal to the inferonasal quadrants. Unlike the cases reportedherein, there was a slight reduction in visual acuity in the affected eye(20/50) and some pigment epithelial mottling at the fovea.

The visual prognosis of patients with extensive CHR remains uncertain.Most patients with localized CHR retain good vision, and visual problems relatedto contraction of the preretinal membrane overlying the tumor or vitreoushemorrhage develop in only a few. However, the risk for development of thesecomplications in extensive lesions such as those reported herein may be higher.Similarly, given that the most anterior retina seemed to be avascular, inthese cases there may be also an increased risk for neovascularization ofthe disc, retina, and/or iris.

The differential diagnosis of CHR should include retinal capillary hemangioma;racemose hemangioma; retinal telangiectasis, including Coats disease; andfamilial exudative vitreoretinopathy.

Unlike CHR, retinal capillary hemangioma appears characteristicallyas a discrete red or pink nodular mass with a feeder artery and a drainingvein.³ Retinal capillary hemangioma representsa large arteriovenous shunt and, thus, it fills early on FFA.³ Thisdiffers from the saccular venous dilations of CHR, which fill slowly and progressivelywith fluorescein. Unlike CHR, hard exudates and retinal detachment are frequentlyobserved associated with retinal capillary hemangioma.

Racemose hemangioma is characterized by tortuous dilated retinal vessels,which represent direct arteriovenous communications.³ LikeCHR, racemose hemangioma does not produce exudation or retinal detachment.However, the saccular venous dilations and the characteristic blood-fluoresceininterfaces observed on FFA in CHR are not a feature of racemose hemangioma.

Retinal telangiectasis, including Coats disease, is a nonfamilial vascularabnormality characterized by irregular dilation of the retinal capillariesor arteries. Unlike CHR, retinal telangiectasis is associated with intraretinaland subretinal exudation and an abnormal increased permeability on FFA.^3,5 Exudative retinal detachmentand cystoid macular edema may develop in patients with retinal telangiectasis.

Familial exudative vitreoretinopathy is inherited as an autosomal dominantor X-linked recessive trait. Like peripheral and extensive CHR, peripheralischemia is also a feature of familial exudative vitreoretinopathy. However,unlike CHR, familial exudative vitreoretinopathy is characterized by the presenceof vitreous bands, neovascularization of the disc and peripheral retina, retinaland subretinal exudation, fibrous proliferation with traction and draggingof the major retinal vessels and macula temporally, and retinal detachment.⁵

Given that in a small proportion of patients with CHR the conditionis transmitted in an autosomal dominant fashion, it is important to examineother members of the family to rule out the presence of similar retinal lesions,as there may be an increased chance of cavernous hemangiomas being presentin the central nervous system in familial cases.⁶ Magneticresonance imaging of the brain may be recommended in these latter cases.

Correspondence: Dr Lois, Retina Service, Department of Ophthalmology,Aberdeen Royal Infirmary, Foresterhill, Aberdeen AB25 2NZ, Scotland (noemilois@aol.com).

The authors have no relevant financial interest in this article.

We thank Jerry Shields, MD, and Carol Shields, MD, for their help inthe diagnosis of one of these cases (patient 1), and Alison Farrow, ABIPP,for her technical assistance.