|Year : 2018 | Volume
| Issue : 1 | Page : 83-86
Cavernous hemangioma of the optic disc
Mona M Aly MD 1, Ihab A Mohamed2
1 Department of Ophthalmology, Faculty of Medicine, Al-Azhar University, Cairo, Egypt
2 Vitreoretinal Department, Memorial Institute of Ophthalmology, Giza, Egypt
|Date of Submission||09-Jun-2017|
|Date of Acceptance||31-Aug-2017|
|Date of Web Publication||1-Feb-2018|
Mona M Aly
Department of Ophthalmology, Faculty of Medicine, Al-Azhar University, Nasr City 11754, Cairo
Source of Support: None, Conflict of Interest: None
This case represents an isolated cavernous hemangioma of the optic disc diagnosed by various diagnostic modalities. A 32-year-old woman with no ocular or systemic history was diagnosed with cavernous hemangioma of the optic disc. Fundus examination revealed a cluster of grape-like lesion occupying the lower part of the optic disc and the adjacent peripapillary retina. Fundus fluorescein angiography showed delayed filling of the lesion with late intravascular plasma–erythrocyte separation ‘characteristic fluorescein capping’ with no leakage. Optical coherence tomography revealed dilated saccular clusters occupying the lower optic disc and adjacent retina. Optical coherence tomography angiography showed a hyperintense lesion (denoting vascular flow) involving the lower half of the disc. No associated systemic hemangiomas were found. After a 1-year follow-up period, no changes have been documented.
Keywords: cavernous hemangioma, fundus fluorescein angiography, optic disc, optical coherence tomography angiography
|How to cite this article:|
Aly MM, Mohamed IA. Cavernous hemangioma of the optic disc. Delta J Ophthalmol 2018;19:83-6
| Introduction|| |
Retinal cavernous hemangioma is a rare, benign vascular tumour that has pathognomonic angiographic and optical coherence tomographic (OCT) findings that distinguish it from other retinal vascular entities . The angiomas can usually be found away from the posterior pole, so that location at the optic disc or macula is exceptional . Several systemic abnormalities can occur in association with retinal cavernous hemangioma, including cavernous hemangioma of the central nervous system and several types of cutaneous hemangiomas. Familial pedigrees have been reported, suggesting inherited forms .
| Case report|| |
A 32-year-old asymptomatic female patient presented for a routine eye examination. Visual acuity was 6/6 unaided in both eyes with normal external examination and no evidence of afferent pupillary defect. Anterior segment slit lamp examination revealed no abnormalities and intraocular pressure was within the normal range. Upon slit lamp biomicroscopy fundus examination with +90 diopter noncontact lens, the right eye had normal fundus whereas the left eye fundus examination revealed a vascular malformation at the optic disc in the form of a group of elevated, dark red saccular grape-like aneurysmal dilatations, which were filled with blood and obscured the lower part of the optic disc and adjacent peripapillary retina with whitish glial tissues ([Figure 1]). Fundus fluorescein angiography (FFA) revealed early hypofluorescence corresponding to the area of the lesion in the early-phase frames and slowly progressive filling of the vascular saccules comprising the lesion. The vascular saccules did not leak. In the late frames, the inferior aspect of the saccules appeared hypofluorescent, whereas the overlying parts were hyperfluorescent ‘fluorescence capping’ ([Figure 2]). OCT, B (radial) scans and OCT angiography were performed. OCT-B (radial) scans on the optic disc showed multiple grape-like hyper-reflective lesions involving the lower half of the disc and adjacent peripapillary retina with underlying shadowing ([Figure 3]). No evidence of exudation was seen. OCT angiography on the optic disc showed hyperintense lesion (denoting vascular flow) involving the lower half of the disc with intravitreal extension ([Figure 4]). These findings were consistent with cavernous hemangioma of the optic disc and peripapillary retina. No cutaneous or central nervous system involvement, on systemic evaluation and neuroimaging, could be detected.
|Figure 1: Coloured fundus photography of the left eye showing a cluster of grape-like appearance of optic disc cavernous hemangioma.|
Click here to view
|Figure 2: Fundus fluorescein angiography, early hypofluorescence corresponding to the area of the lesion in the early-phase frames; the late phase showed the classical picture of cavernous hemangioma. The dye has filled the upper aspect of the saccular aneurysms whereas the lower part remains hypofluorescent ‘fluorescein capping’.|
Click here to view
|Figure 3: Optical coherence tomography-B (radial) scans showing multiple grape-like hyper-reflective lesions involving the lower half of the disc and adjacent peripapillary retina with underlying shadowing.|
Click here to view
|Figure 4: Optical coherence tomography angiography scans showing hyperintense lesion (denoting vascular flow) involving the lower half of the disc with intravitreal extension.|
Click here to view
| Discussion|| |
Cavernous hemangioma is a nonprogressive rare vascular hamartoma. Typical fundus findings are clumps of dark saccular aneurysms (cluster of grapes appearance) with fibroglial tissue over the tumour, absence of feeder vessels and absence of exudation . Part or all of the optic nerve head may be invested and the tumour almost always extends into the peripapillary retina . To date, there have been limited reports of optic disc cavernous hemangioma . It can occur as an isolated solitary lesion or as a component of an autosomal dominantly inherited oculoneurocutaneous syndrome that can manifest similar central nervous system vascular anomalies and several types of cutaneous vascular malformations . The average age of presentation is 23 years and about 60% of tumours occurred in women. Less than 10% occur bilaterally and the visual acuity is usually normal unless the fovea is affected. Progression appears to be rare . Histology of cavernous hemangiomas had been reported. The ultrastructurally normal vessel wall maintains the blood retinal barrier, which explains the absence of fluorescein leak . Angiographically, the tumour stains during the mid and late venous phases without evidence of leakage. A characteristic plasma–erythrocyte level occurs related to the sedimentation of erythrocytes in the dependent portions of the aneurysms blocking fluorescence, whereas the plasma in the superior portion of the lesion fluoresces intensely . Rani et al.  have diagnosed a case of cavernous hemangioma of the optic disc based on clinical picture and typical fluorescein cap sign seen on FFA with hyperfluorescence due to staining of supernatant plasma and hypofluorescence at the bottom of the aneurysm.
OCT provides a fast noninvasive method for assessing the progression of these lesions . Robinet et al.  described the spectral domain OCT findings of a retinal cavernous hemangioma of the optic disc which disclosed numerous, interconnected, blood-filled vascular saccules within inner retinal layers, separated by thin septa, in which a plasma–erythrocyte level was observable.
OCT angiography can be used as a noninvasive tool in the diagnosis of cavernous hemangioma of the optic nerve head . OCT angiography image delineates the entire lesion in similar potential as FFA noninvasively, without the use of fluorescein dye and provides dynamic information of the cavernous hemangioma, which is lacking on FFA .
This report describes a cavernous hemangioma of the optic disc in a female patient without skin or neurological findings (isolated cavernous hemangioma of the optic disc). The diagnosis of cavernous hemangioma of the optic disc is based on the typical clinical picture, FFA appearance, OCT and OCT angiography findings which support the current diagnosis. The patient has been followed for 1-year without changes in visual acuity, optic disc appearance on fundus examination, fluorescein angiographic features nor OCT-B scans and OCT-angiographic features.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Kavoussi SC, Brinton GS. Epiretinal membrane demonstrated by optical coherence tomography in a patient with retinal cavernous hemangioma. JAMA Ophthalmol 2015; 133:e151163.
Heimann H, Damato B. Congenital vascular malformations of the retina and choroid. Eye (Lond) 2010; 2:459–467.
Patikulsila D, Visaetsilpanonta S, Sinclair SH, Shields JA. Cavernous hemangioma of the optic disk. Retina 2007; 27:391–392.
Rani PK, Peguda HK, Kaliki S, Chhablani J. Reverse fluorescein cap sign of cavernous haemangioma of optic nerve on OCT angiography. BMJ Case Rep 2016; 2016:1–2.
Brown GC, Shields JA. Tumors of the optic nerve head. Surv Ophthalmol 1985; 29:239–264.
Katta M, Mehta H, Ho I, Garrick R, Chong R. Optical coherence tomography imaging of optic disc cavernous haemangioma. J Clin Neurosci 2016; 33:234–235.
Shields JA, Eagle RC Jr, Ewing MQ, Lally SE, Shields CL. Retinal cavernous hemangioma: fifty-two years of clinical follow-up with clinicopathologic correlation. Retina 2014; 34:1253–1257.
Pringle E, Chen S, Rubinstein A, Patel CK, Downes S. Optical coherence tomography in retinal cavernous haemangioma may explain the mechanism of vitreous haemorrhage. Eye (Lond) 2009; 23:1242–1243.
Bruè C, Vance SK, Yannuzzi LA, Freund KB. Cavernous hemangioma associated with retinal macrovessels. Retin Cases Brief Rep 2011; 5:323–325.
Robinet A, Korobelnik JF, Quentel G, Rougier MB, Gontier B, Delyfer MN. Spectral-domain optical coherence tomography findings in cavernous hemangioma of the optic disk. Retina 2017; 37:e11–e13.
Kalevar A, Patel KH, McDonald HR. Optical coherence tomography angiography of retinal cavernous hemangioma. Retina 2017; 37:e50–e51.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]