Racemose Haemangioma

Overview

Racemose haemangioma is a rare, typically unilateral, non-progressive retinal vascular tumor. It is a malformation of the retinal vessels allowing direct connection between the arteries and veins.

Clinically it presents as dilated and tortuous retinal vessels extending from the optic disc to the retinal periphery. The lesions, if very large, may occasionally result in intraretinal, vitreous or macular haemorrhage however fluorescein angiography usually shows stable, non-leaking lesions. Vaso-occlusive disease (most commonly retinal vein occlusion) and retinal haemorrhage may also be associated with racemose haemangioma.

Excluding these potential complications, there is typically no visual deterioration with this condition.

Racemose Haemangioma can be associated with Wyburn-Mason Syndrome. This syndrome is a vascular condition typified by arteriovenous malformations of the face, orbit, retina and central nervous system. In this syndrome, a retinal racemose haemangioma is often associated with a lesion in the ipsilateral midbrain and there is typically an increase in early mortality due to the tendency of the AV malformations to bleed causing stroke, neurological deficits and death.

Case Examples

  • Case 1

    A 22-year-old male with best corrected visual acuity of 6/6 (20/20) in each eye. He has been diagnosed with Wyburn-Mason syndrome. His left eye is unremarkable so this case will focus on the right eye only.

    Fundus photograph and red-free image (right eye)

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    Fundus photograph and red-free image (right eye)

    Fundus photos show the right optic nerve head to be partially obscured by dilated and tortuous arterioles and venules. Some areas of vascular sheathing are present.

    Stereoscopic photo (right optic disc)

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    Stereoscopic photo (right optic disc)

    Please use a stereoviewer or 15 prism dioptres base out to view the image.

    Stereoscopic viewing shows the vascular lesion to be elevated.

    Optomap widefield images (temporal and superior retina)

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    Optomap widefield images (temporal and superior retina)

    Widefield imaging shows the extent of the lesion which extends both superiorly and inferiorly from the optic nerve head. Tortuosity of the other retinal vessels can also be appreciated. Ghost vessels, sheathing and blot haemorrhages are evident in the temporal periphery.

    Spectralis OCT volume and line scans (right eye)

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    Spectralis OCT volume and line scans (right eye)

    OCT scans through the anomalous vasculature show the dilated vessels to span the full retinal thickness. There is associated inner retinal layer disruption including intra-retinal schisis and cystoid spaces.

  • Case 2

    51-year-old Caucasian male, diagnosed with racemose haemangioma at the age of 30. He is asymptomatic with best corrected visual acuity of 6/7.5 (20/25) in both eyes. The left eye is unremarkable, so this case will focus on the right eye.

    Fundus photograph (right eye)

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    Fundus photograph (right eye)

    The right optic nerve head is indistinguishable due to the presence of dilated and tortuous arterioles and venules covering most of its surface. Vascular sheathing and scattered paravascular pigmentary hypertrophy are present.

    The dilated and tortuous blood vessels follow the inferior-temporal vascular arcade, although other vasculature in the right eye was also tortuous and mildly dilated.

    Stereoscopic photo of lesion (right optic disc)

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    Stereoscopic photo of lesion (right optic disc)

    Please use a stereoviewer or 15 prism dioptres (base out) to view the image.

    A stereoscopic view of the vascular lesion show it to be elevated.

    Optomap widefield and green separation images (right eye)

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    Optomap widefield and green separation images (right eye)

    Widefield imaging illustrates the extent of the racemose haemangioma as it extends to the infero-temporal peripheral retina. The tortuosity of other retinal vessels can also be appreciated in these images.

Differential Diagnosis

Neovasculariation (proliferative diabetic reitnopathy)

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Collateral vessels

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References

Pangtey, B., Kohli, P., & Ramasamy, K. (2018). Wyburn-Mason syndrome presenting with bilateral retinal racemose hemangioma with unilateral serous retinal detachment. Indian journal of ophthalmology, 66(12), 1869–1871.

Qin, Xj., Huang, C. & Lai, K. (2014) Retinal vein occlusion in retinal racemose hemangioma: a case report and literature review of ocular complications in this rare retinal vascular disorder. BMC Ophthalmol 14, 101.

Vishal, R., Avadesh, O., Srinivas, R., & Taraprasad, D. (2018). Retinal racemose hemangioma with retinal artery macroaneurysm: Optical coherence tomography angiography (OCTA) findings. American journal of ophthalmology case reports, 11, 98–100.