Spontaneous development of macular ischemia in a case of racemose hemangioma

Abstract

Purpose: To report a rare case of racemose hemangioma which developed spontaneous macular ischemia.

Methods: A 32-year-old healthy Caucasian lady presented complaining of recent deterioration of vision in her left eye. At presentation, her best corrected visual acuity (BCVA) was 20/20 in her right eye and counting fingers in her left eye (LE). Fundus examination and fluorescein angiography were performed. The patient had regular follow-up appointments over a period of 8 years.

Results: Fundus examination and fluorescein angiography revealed findings consistent with arteriovenous communications of the retina or racemose hemangioma, in the posterior pole of the LE with the presence of macular ischemia. Complete and systemic examination was unremarkable, excluding the possibility of Wyburn-Mason syndrome. Eight years after presentation, findings and BCVA in the LE have remained stable, with no extension of the retinal ischemia or development of neovascularization.

Conclusion: Although extensive retinal ischemia has been reported to result in complications such as retinal or iris neovascularization, in our case the macular ischemia has not expanded further over a period of 8 years. However, due to this macular ischemia the patient unfortunately lost her central vision.

Keywords: macular ischemia; racemose hemangioma; retinal ischemia.

Figure 1

Color fundus picture of the left eye at presentation showing the retinal racemose angioma. The optic disc is obscured by vessels. The retinal vessels in the macular area are white indicating ischemia. Findings remained stable over the 8 year follow-up period.

Figure 2

Early (A) and late (B) phases of fundus fluorescein angiography of the left eye showing macular ischemia and extensive arteriovenous communications and dilated intertwined vessels. There are small hemorrhages and exudates and white (sheathed) vessels in the macular area.