Case of the Month

Edited by Robert N. Johnson, MD

Dec, 2016

A 77 year-old woman presents with blurred vision in the right eye.

Presented by Ananda Kalevar, MD

Figure 1A and Inset: Wide field and detailed photograph of the right eye. Note the discrete yellowish-green area of subretinal blood with overlying preretinal and intraretinal hemorrhage. A thin layer of subretinal hemorrhage extends towards macula.

Figure 2: Wide field photograph of the right eye. Note the vitreous hemorrhage settled inferiorly

Case History

A 77 year-old woman presents with blurred vision in her right eye for two weeks.  Her past medical history was relevant for hypertension, asthma and remote cervical and breast cancer (both in remission).  Her past ocular history, family history, social history and medications were non-contributory.

On examination, best-corrected visual acuity was 20/25 and 20/20 in her right and left eyes, respectively.  Intraocular pressure was normal in both eyes.  The anterior segment examination was remarkable for mild nuclear sclerosis.  The posterior segment exam of the right eye showed a large area of dehemoglobinized blood superiorly with subretinal, intraretinal and preretinal blood (Figure 1A, and inset).  Mild hypertensive retinopathy was present.  There was also mild vitreous hemorrhage, settled mostly inferiorly (Figure 1B).  Fluorescein angiography (FA) of the right eye revealed significant blockage from the blood and late focal leakage along the superior arcade (Figure 2).  Examination of the left eye was remarkable for mild hypertensive retinopathy.

Spectral domain OCT (SD-OCT) of the right eye through the fovea was unremarkable and a normal retinal contour was present (Figure 3).  SD-OCT along the superior arcade showed retinal thickening with hyperreflective material consistent with blood that appeared to be under the ILM, and intraretinal and subretinal (Figure 4). SD-OCT of the left eye was unremarkable (Images not shown).  Fundus autofluorescence (FAF) of the right eye showed well delineated areas of hypo-autofluorescence where hemorrhage was on noted on clinical exam (Figure 5).  FAF of the left eye was unremarkable.

Figure 2: Wide-field fluorescein angiogram of the right eye. Note the large area of blockage superiorly due to subretinal, intraretinal and preretinal blood. A focal area of hyperflourescence is noted with some mild leakage within the area of blocked fluorescence.

Figure 3: SD-OCT of the macula and nerve is unremarkable

Figure 4: SD-OCT through the area of hemorrhage. Note the hyperreflective areas superficially in the retina (with deeper blockage) as well as hyperreflective material under the retina (with deeper blockage) consistent with hemorrhage.

Figure 5: Wide-field fundus autofluorescence. Note the discrete areas of reduced autofluorescence corresponding to the areas of blood.

What is your Diagnosis?

Differential diagnosis

Branch retinal vein occlusion, diabetic retinopathy, choroidal neovascularization, traumatic hemorrhage, capillary hemangioma, cavernous hemangioma, retinal telangiectasis, and malignant melanoma

 

Discussion

Retinal arterial macroaneurysms (RAM) are acquired saccular or fusiform dilations of the retinal arterioles that usually occur within the first three orders of bifurcation.  The superotemporal artery is the most commonly reported location. This most commonly occurs in women in their sixth or seventh decade of life and is usually unilateral.  General atherosclerotic cardiovascular disease such as hypertension, dyslipidemia and diabetes mellitus are often associated.1-3

Fluorescein angiography and OCT are particularly helpful to diagnose and manage RAMs.  Angiographic findings include an early ‘light-bulb’ like area of hyperfluorescence in many cases. Hemorrhage may obscure this finding and ICG angiography may be helpful in these cases. In the late phases of FA, staining of the macroaneurysm may be seen as well as marked leakage in other cases.

RAMs may be asymptomatic or cause a number of complications such as macular edema, serous macular detachment, hard exudates, branch retinal artery occlusion and hemorrhage in the subretinal or intraretinal or preretinal or sub-ILM or subhyaloidal or vitreous space.  This predilection hemorrhage to occur in front of and under the retina has been described by Schatz et al4 as an “hour-glass hemorrhage.”  OCT through a RAM of another patient clearly demonstrates the extent of the aneurysm and in this case as it spans most of the retinal layers, offers insight into how hemorrhage can occur at these different levels (Figure 6). The diagnosis is usually not challenging but occasionally the appearance of a discrete area of older greenish appearing blood under the retina can even mimic the appearance of a choroidal melanoma.

Overall, the natural history and prognosis can be quite favorable. There is no consensus regarding management.  Most of the RAMs spontaneously thrombose and involute.  Subsequently, edema and exudates are absorbed.   If there is significant exudation of lipid, and fluid, then treatment is warranted as subfoveal lipid usually leads to permanent retinal damage. The principle method of treatment has been laser photocoagulation directly over the lesion.5  Potential complications from laser include rupture with hemorrhage, and possible occlusion of the distal portion of the retinal arteriole.  Recently, anti-VEGF agents have been shown to be beneficial in treating RAMs.6   Less commonly, vitrectomy surgery with intravireal tissue plasminogen activator with or without gas injection has been done. Results with this approach in cases of thick submacular hemorrhages have been reported to be more favorable.7 The exact role of this approach and the benefits, however, are uncertain, but ideally if it were to be undertaken, it would be in a situation of a very recent onset hemorrhage.

Figure 6: SD-OCT through a RAM in another patient. Note the almost full-thickness involvement of the retina. It is easy in this situation to explain bleeding into the preretinal, intraretinal and subretinal space in such a case.

Take Home Points

  • Retinal arterial macroaneursyms typically occur in older women with hypertension and other risk factors for atherosclerosis.
  • Visual loss can occur from subretinal hemorrhage, fluid and lipid though the prognosis is usually good.
  • Management ranges from observation to thermal laser photocoagulation in most cases.

References

  1. Ryan S.  Retina, 5th ed.  2013:  1026-1028.
  2. Pitkänen L, Tommila P, Kaarniranta K, Jääskeläinen JE, Kinnunen K. Retinal arterial macroaneurysms. Acta ophthalmologica. 2014 Mar 1;92(2):101-4.
  3. Robertson D.  Macroaneursym of the retinal arteries.  Trans Am Acad Ophtal Otolaryngol.  1973; 77:  55-67.
  4. Schatz H, Gitter K, Yannuzzi L, Irvine A. Retinal arterial macroaneurysms: A large collaborative study. Presented at the American Academy of Ophthalmology Annual Meeting, Chicago, November, 1980.
  5. Meyer JC, Ahmad BU, Blinder KJ, Shah GK. Laser therapy versus observation for symptomatic retinal artery macroaneurysms. Graefe's Archive for Clinical and Experimental Ophthalmology. 2015 Apr 1;253(4):537-41.
  6. Pichi F, Morara M, Torrazza C, Manzi G, Alkabes M, Balducci N, Vitale L, Lembo A, Ciardella AP, Nucci P. Intravitreal bevacizumab for macular complications from retinal arterial macroaneurysms. American journal of Ophthalmology. 2013 Feb 28;155(2):287-94.
  7. Nakamura H, Hayakawa K, Sawaguchi S, Gaja T et al. Visual outcome after vitreous, sub-internal limiting membrane, and/or submacular hemorrhage removal associated with ruptured retinal arterial macroaneurysms. Graefes Arch Clin Experimental Ophthalmology. 2008 246:661-669.

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