Functional and anatomical investigations in racemose haemangioma

Abstract

ABSTRACT.Purpose: To determine the vascular morphology of racemose haemangioma and related functional alterations in arteriovenous (AV) malformation type 3. Methods: 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). Results: 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. Conclusion: The racemose haemangioma led to a marked visual field defect. Racemose haemangiomas are associated with severe changes in the haemodynamics of the retinal vasculature.

Spontaneous development of macular ischemia in a case of racemose hemangioma

“…The lesions have been reported either to remain static or regress1–2 or to enlarge gradually. Vision may be affected directly due to macular involvement or by producing hemorrhage or exudation 3,4. Based on Archer et al the angioma in our case was grade 3, although there were no systemic findings 5.…”

Temporal Branch Retinal Vein Occlusion Secondary to a Racemose Hemangioma

“…A defect in the differentiation of the primordial cerebral vascular channels into arterial, venous, and capillary components could account for this variety of vascular malformation. 5,7,9 This type of AVM requires periodic ophthalmic examinations and treatment of any such complications. The earliest retinal vasculature forms in the vicinity of the optic nerve at around 14 weeks of gestation; at this stage there is no division between arteries and veins.…”

“…2,3 Complications at the cerebral level depend on the location and extent of the lesions and may be asymptomatic or present subarachnoid or intracerebral hemorrhage due to AVM rupture. 7 This mass of dilated tortuous vessels forms large loops that may extend from the optic nerve head to the periphery. Group 1 is characterized by the inter-position of an arteriolar or abnormal capillary plexus between the major communicating vessels.…”

“…4 Archer et al 5 classified retinal AVMs into three groups, according to structural alteration and retinal hemodynamics. 7 If the posterior pole is affected, there is often associated visual impairment, and ophthalmoscopic examination often shows the presence of rlbrosis or perivascular sheathing, exudation, and associated pigmentary anomalies. Vascularizaion of the optic nerve head is usually unaffected, and the AVMs are usually stable, without visual repercussions or alterations in retinal hemodynamics.…”

Vergleich von Spektral- und Zeit-Domänen-OCT hinsichtlich der automatisierten Erfassung der zentralen Netzhautdicke

“…Zusätzlich wurden bereits Erweiterungen des OCT entwickelt, welche eine Kontrastverstär-kung bewirken und eine funktionellen Bildanalyse der Netzhaut zulassen (z. B. Blutfluss, spektroskopische, polarisationsempfindliche und physiologische Aussagen über die Netzhaut) [10,12]. Dementsprechend kann das funktionelle OCT die Differenzierung retinaler Prozesse auf dem Boden fokussierter retinaler Antworten oder metabolischer Eigenschaften gewährleisten.…”