Subretinal hemorrhages associated with angioid streaks following a mild ocular trauma.

Introduction

Angioid streaks (AS) are a rare ophthalmic finding. They are most frequently associated with pseudoxanthoma elasticum (PXE). Choroidal neovascularization is the most known complication of AS. We report a rare case of a patient having AS who presented with mutiple subretinal hemorrhages following a mild contusive ocular trauma.

Case Report

A young male patient aged of 30 year old presented in the out-patients department with a sudden severe decreasing of visual acuity following a mild ocular trauma in the left eye which occurred one month prior.

Visual acuities at presentation were 20/20 in the right eye (RE) and 20/200 in the left eye (LE). Slit lamp examination found in both eyes clear cornea, anterior chamber normally deep and quiet, clear lens, and a normal intraocular pressure. Fundus examination found angioid streaks (AS) emanating from the optic disc in both eyes with “peau d’orange” aspect [Figures 1 and 2]. Furthermore, we noted submacular hematoma with disciform scaring of the macula and two other subretinal hemorrhages laying nasal and inferior to the optic disc in the left eye [Figure 2]. To this was associated a linear rupture of Bruch's membrane located inside the main submacular hemorrhage. Systemic examination found skin changes in the neck and the axillary space compatible with PXE. This was confirmed later by a skin biopsy. Fluorescein angiography [Figure 3] showed staining along with Bruch's membrane rupture and angioid streaks. No neovascularization was noted in both eyes. The ocular coherence tomography (OCT) of the left eye showed macular detachment by effusion of submacular hemorrhage [Figure 4]. The splitting is located in the space between the neuroepithelium (NE) and the retinal pigment epithelium. Also is noted, hypereflectivity and thickening of the photoreceptors’ layer, compatible with the disciform macular scar aspect. At the level of the Bruch's membrane rupture, the macular OCT showed fusion of the RPE, choroid, and NE [Figure 5].

Figure 1

Angioid streaks and “peau d’orange” aspect in the right eye

Figure 2

Multiples subretinal hemorrhages and Bruch's membrane rupture in the left eye

Figure 3

Fluorescein angiography of the left eye showing staining along with Bruch's membrane rupture and angioid streaks

Figure 4

Left macular OCT showing macular detachment by effusion of submacular hemorrhage

Figure 5

OCT at the level of the Bruch's membrane rupture showing fusion of the RPE, choroid and neuroepithelium

The course was characterized by resolution of hemorrhage and occurrence of a fibrosis scaring of the macula after 4 months with no improvement of visual acuity [Figure 6]. At this stage, macular OCT showed reabsorption of hemorrhages, diffuse hypereflectivity, and thickening of the photoreceptors’ layer [Figure 7].

Figure 6

Fibrosis scaring of the macula in the left eye

Figure 7

Left macular OCT showing reabsorption of hemorrhages, diffuse hypereflectivity, and thickening of the photoreceptors’ layer

Discussion

Angioid streaks are caused by cracks in Bruch's membrane resulting from changes in the elastic fibers. Fragility of Bruch's membrane when associated with AS is confirmed by the occurrence of subretinal hemorrhage after a slight ocular trauma[1-3] or even an indirect trauma 4.

There are some similar cases that have been reported.[1-5] Soutome et al[5] reported a case of subretinal hemorrhage after blunt trauma in a patient with PXE; the fundus examination showed angioid streaks in both eyes, subretinal hemorrhage, and Bruch's membrane ruptures in the traumatised eye. The course was spontaneously favorable with resolution of hemorrhage. Pandoflo et al[4] reported a case of bilateral ocular involvement by subretinal hemorrhage associated with angioid streaks following indirect trauma. The impact of the contusive trauma was in fact located in the temporoparietal region of the head. The course was characterized by the occurrence of choroidal neovascularization and disciform scar in the left eye.

To our knowledge, this is the first case in which macular OCT was performed to assess the level of split within the retina in case of subretinal hemorrhages associated with AS. In fact, as shown in Figure 2, blood lays in the potential space between the pigmentary epithelium and the photoreceptor cell layer as suggested in a previous publication 2. This was obviously allowed by the weak resistance at the level of the Bruch's membrane.

The patient was screened for other potentially associated systemic conditions. There were no cardiovascular, neurologic, or digestive diseases related to PXE.

No ophthalmic treatment was proposed. However, we advised a regular follow up because of the potential risk of choroidal neovascularization.