Functional and anatomical investigations in racemose haemangioma

To determine the vascular morphology of racemose haemangioma and related functional alterations in arteriovenous (AV) malformation type 3.

A 17-year-old patient with unilateral racemose haemangioma received a full ophthalmic examination including Snellen visual acuity (VA) and Goldmann visual field. The central vision was investigated by scanning laser ophthalmoscope (SLO) and multifocal electroretinogram (mfERG). The ocular haemodynamics were examined by fluorescence angiography and Doppler ultrasound. The tomographic contour of the vascular architecture was visualized using B-scan ultrasound, Stratus optical coherence tomography (OCT) and three-dimensional Heidelberg retina tomograph (3D-HRT II).

The VA of the patient's right eye was reduced to 20/400 and her visual field was constricted concentrically. Microperimetry revealed a small central field with good central fixation. The mfERG demonstrated reduced amplitudes of the central retina. On fluorescein angiography, there was a fast filling of the retinal branches related to the racemose vessels. Doppler ultrasound confirmed a significantly changed haemodynamic flow in the racemose vessels. Ultrasound, OCT and HRT demonstrated a prominent optic nerve head.

The racemose haemangioma led to a marked visual field defect. Racemose haemangiomas are associated with severe changes in the haemodynamics of the retinal vasculature.