Bilateral multiple retinal artery macroaneurysms with coexistent diabetic retinopathy

Roshni Mohan; M Arthi

doi:10.4103/tjosr.tjosr_173_21

CASE REPORT

Year : 2022 | Volume : 60 | Issue : 3 | Page : 266-268

Bilateral multiple retinal artery macroaneurysms with coexistent diabetic retinopathy

Roshni Mohan, M Arthi
Department of Retina and Vitreous, Aravind Eye Hospital, Pondicherry, India

Date of Submission	13-Nov-2021
Date of Decision	01-Jun-2022
Date of Acceptance	06-Jun-2022
Date of Web Publication	26-Sep-2022

Correspondence Address:
Roshni Mohan
Department of Retina and Vitreous, Aravind Eye Hospital, Thavalakuppam, Pondicherry - 605 007
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjosr.tjosr_173_21

Abstract

To discuss the clinical profile of retinal artery macroaneurysms (RAMs), its presentation, rarity, complexity in diagnosis and management modalities. RAMs are seen commonly in elderly females who have associated systemic hypertension. Most of these aneurysms undergo spontaneous involution due to fibrosis while others may result in vascular leakage with macular oedema and haemorrhage into various layers of retina. Long-standing exudation may result in the development of cystoid macular oedema (CME), macular hole, macular exudate, retinal gliosis, and atrophy. Since the arterioles are high-flow vessels, it ruptures under high pressure resulting in haemorrhages at various levels – vitreal, preretinal, intraretinal, and subretinal. Multiple RAMs maybe hereditary, inherited in an autosomal recessive pattern presenting with recurrent vitreous haemorrhage. Treatment is to ensure rapid resolution of haemorrhage and exudation thereby facilitating early visual recovery and avoiding permanent visual loss. Referral to a physician is essential for the management of hypertension and associated systemic illnesses. Here we describe a case of multiple bilateral macroaneurysms with coexistent diabetic retinopathy.

Keywords: Cystoid macular oedema, Hypertension, macular exudate, retinal artery macro aneurysms, retinal haemorrhage

How to cite this article:
Mohan R, Arthi M. Bilateral multiple retinal artery macroaneurysms with coexistent diabetic retinopathy. TNOA J Ophthalmic Sci Res 2022;60:266-8

How to cite this URL:
Mohan R, Arthi M. Bilateral multiple retinal artery macroaneurysms with coexistent diabetic retinopathy. TNOA J Ophthalmic Sci Res [serial online] 2022 [cited 2022 Nov 30];60:266-8. Available from: https://www.tnoajosr.com/text.asp?2022/60/3/266/357109

Case Report

A 67-Year-old female presented to us with complaints of defective vision in both eyes (OU), left eye (OS) more than the right eye (OD). She had defective vision in OU for the past six months. Systemic history was significant for uncontrolled diabetes and hypertension despite treatment for the past six years. Her best-corrected visual acuity (BCVA) was OD 20/40, N10 and OS 20/80, N18. The anterior segment had PCIOL with posterior capsular opacity. Fundus evaluation of OU media was hazy due to PCO. OU showed features of moderate non-proliferative diabetic retinopathy with hard exudates at the macula. OS had circinate retinopathy at the macula.

Optical coherence tomography (OCT) OD showed hyperreflective deposits in outer layers and preserved foveal contour [Figure 1]a. OCT OS showed schitic changes, multiple hyperreflective layers and hyporeflective lumen at the fovea, with multiple hyperreflective clumps in the middle and outer layers [Figure 1]b and [Figure 1]c. She underwent OS intravitreal triamcinolone acetonide injection 2 mg/0.05 ml for the chronic diabetic macular oedema.

Figure 1: Optical coherence tomography (a) OD showing hyperreflective clump hard exudates in the middle and outer retinal layers and preserved foveal contour (b) Line scan showing cystic changes at the fovea (c) Line scan showing schitic changes with hyperreflective layers and hyporeflective lumen at the fovea and subfoveal hard exudates at the fovea (d) Post-injection line scan showing multilayered haem and loss of foveal contour

Click here to view

One week post-injection, she experienced a sudden drop in vision OS. Anterior segment was normal. Her BCVA OS was <20/200. Fundus evaluation OS showed multilayered haemorrhage with circinate retinopathy at the macula [Figure 1]. Careful evaluation OD revealed dark, retinal, and preretinal haemorrhages along first-order vessels with central clearing [Figure 2]. OCT OS showed multilayered bleed at the fovea.

Figure 2: Fundus photograph OD (on the left side) showing dark haemorrhage nasally with central clearing and hard exudates at the macula. Fundus photograph OS (on the right side) showing multilayered haem at the macula with circinate retinopathy and diabetic vascular changes

Click here to view

Blood investigations, including peripheral smear, complete blood count, were normal. Fundus fluorescein angiography (FFA) OD showed focal areas of hyperfluoresence along superonasal, inferonasal, and inferotemporal arteries which were more discrete in later phases [Figure 3]a and [Figure 3]c. There was diffuse leakage at the posterior pole and blocked fluorescence around the lesions. Indocyanide green angiography (ICGA) OD showed these lesions to be discrete and more numerous in the later frames [Figure 3]b and [Figure 3]d. FFA OS showed small saccular outpouchings along the superonasal artery with hyperfluoresence and diffuse leakage at the posterior pole. Late phases showed blocked fluorescence at the macula with faint indistinct hyperfluorescence [Figure 4]a. ICGA OS also showed blocked fluoresence at the macula [Figure 4]b. A diagnosis of OU multiple RAM with moderate non-proliferative diabetic retinopathy with sub-macular haemorrhage OS due to ruptured RAM was made. This patient was advised OS intravitreal bevacizumab (IVB) with pneumatic displacement (C3F8). However, her blood pressure was very high (200/120 mm Hg) and hence the procedure was deferred. She was asked to review back with cardiologist fitness for the procedure.

Figure 3: Fundus fluorescein angiogram (FFA) and indocyanine green angiogram (ICGA) OD (a and b) Early Frames showing focal hyperfluoresence along the superonasal, inferonasal, and inferotemporal arteries (indicated by white circles) suggestive of RAMA – saccular outpouching. OD (c and d) Late frames showing the hyperfluoresence becoming more numerous and discrete in all quadrants (indicated by white circles). The focal hyperfluoresence has surrounding blocked fluoresence due to haem

Click here to view

Figure 4: FFA OS(a) and ICGA OS (b) showing late frames with discrete focal hyperfluoresence in the nasal quadrant suggestive of RAMA and faint indistinct hyperfluoresence at macula (indicated by white circle)- Probable site of RAMA that has ruptured

Click here to view

Discussion

Retinal artery macroaneurysms (RAMs) are acquired dilatations of 100 to 250 μmin size occurring within the first three bifurcations of retinal arterioles. They commonly involve the superotemporal or inferotemporal arteries and occur at arteriolar bifurcations or arteriovenous crossings. The absence of adventitious layer at AV crossings results in a lesser structural support leading to an increased incidence of RAM at these sites. They are more common in females and are seen to be more prevalent in individuals with hypertension and systemic arteriosclerosis. They are termed simple RAM when presenting only with vascular dilatations and complex RAM when accompanied by haemorrhage. They may be bilateral in 10% of cases and multiple RAMs may be present in the same eye or vessel in 20% of cases.^[1] These bilateral multiple RAMs can be familial like familial retinal artery macroaneurysms (FRAM) with peripheral ischaemia where these patients have associated cardiac involvement in the form of supravalvular pulmonic stenosis^[2] or can have systemic associations like cerebral macroaneurysms.^[3]

The effect of ageing and arteriosclerosis cause atrophy of the muscular layer, which leads to thinning and fibrosis of the vascular wall and reduced elasticity which when subjected to hypertension results in dilatation. Fusiform dilatations result from generalized dilatation of vascular wall whereas localized dilatation leads to saccular RAMs.^[4] Loss of endothelial cells causes plasma leakage resulting in exudative maculopathy, and rupture of the vessel wall itself may lead to haemorrhage involving different layers of the retina typically described as 'hourglass haemorrhage'.

They are usually asymptomatic. Reduction in vision may occur due to vitreous haemorrhage or due to haemorrhage or exudation involving the macula. Subretinal haemorrhage can damage photoreceptors due to release of haem, fibrin, hemosiderin, and iron. The shearing effect of clot retraction leads to chronic separation of photoreceptors from RPE resulting in atrophy and scar formation.^[5]

Ancillary testing may help us differentiate RAM from other conditions causing subretinal bleeding like Age Related macular degeneration (AMD) and polypoidal choroidal vasculopathy (PCV). ICGA may be useful in cases where haemorrhage blocks visualization of aneurysm. Differential diagnosis includes trauma, anaemia, leukaemia, Valsalva retinopathy, PCV, and coat's disease depending on the clinical features during presentation.^[6]

Treatment of RAM depends on presenting features, locations, and associated complications. Asymptomatic cases can be observed. Laser to visible leaking macroaneurysms can be attempted in case of exudation causing macular oedema. Laser can be performed directly to the aneurysm itself or to the area surrounding the aneurysm.^[7] Management of ruptured RAM presenting with haemorrhage depends on the location and extent of the haem. Subretinal haemorrhage in the macular area can be managed by pneumatic displacement or vitrectomy, ILM peeling, and subretinal injection of tissue plasminogen activator.^[8] Vitrectomy also plays a role in visual rehabilitation of patients with non-clearing vitreous haemorrhage for more than three months. Pre-retinal haemorrhage can be managed by YAG hyaloidotomy. McCabe et al. observed that RAM with haemorrhage which was observed without intervention, the mean time taken for the bleed to clear was 4.6 months. Poor visual acuity in 34% was due to pigmentary changes.^[9]

Anti-VEGF injections have a role in the management of RAM, though the mechanism of action is still unclear. VEGF causes activation of coagulation cascades and stimulates the endothelial production of nitric oxide. Hence anti-VEGF may cause vasoconstriction and alter the balance between coagulation and fibrinolysis and hence reducing the exudative complications of RAM. In a case series by Cho et al.,^[10] patients treated with anti-VEGF showed faster resolution of retinal haemorrhage and rapid gain in visual acuity compared with the control group.

Conclusion

Our case had bilateral multiple RAMs, risk factors being female gender, age, and uncontrolled hypertension. The presence of round hyperreflective wall with hyporeflective lumen on OCT (ring sign), the presence of saccular outpouchings on angiogram helped us rule out other similar conditions and establish the diagnosis. We considered pneumatic displacement with anti-VEGF for the sub-macular bleed with macular oedema along with control of systemic risk factors. The patient was lost to follow-up during the first wave of the COVID pandemic and is yet to be reviewed.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Speilburg AM, Klemencic SA. Ruptured retinal arterial macroaneurysm: Diagnosis and management. J Optom 2014;7:131-7.
2.	Alotaibi MD, Alsarhani WK, Al-Qahtani BS, AlBloushi AF, Abu El-Asrar AM. Familial retinal arterial macroaneurysms with peripheral retinal ischemia: A characterization on ultra-widefield fluorescein angiography. Middle East Afr J Ophthalmol 2021;27:228-30.
3.	Nalcaci S, Oztas Z, Eraslan C, Akkin C. Are multiple retinal arterial macroaneurysms considered a sign of cerebral aneurysms?; Ophthalmic Surg Lasers Imaging Retina 2017;48:79-82.
4.	Robertson DM. Macroaneurysms of the retinal arteries. Trans Am Acad Ophthalmol Otolaryngol 1973;77:OP55-67.
5.	Adamczyk AT, Olivares GE, Petito GT. Retinal arterial macroaneurysm: A longitudinal case study. J Am Optom Assoc 1989;60:840-5.
6.	Holdeman NR, Le H. Retinal arterial macroaneurysm. Clin Sur Ophthalmol 2010;28:238-42.
7.	Meyer JC, Ahmad BU, Blinder KJ, Shah GK. Laser therapy versus observation for symptomatic retinal artery macroaneurysms. Graefes Arch Clin Exp Ophthalmol 2015;53:537-41.
8.	Humayun M, Lewis H. Management of submacular hemorrhage associated with retinal artery macroaneurysms. Am J Ophthalmol 1998;126:358-61.
9.	McCabe CM, Flynn HW Jr, McLean WC, Brod RD, McDonald HR, Johnson MW, et al. Nonsurgical management of macular hemorrhage secondary to retinal artery macroaneurysm. Arch Ophthalmol 2000;118:780-5.
10.	Cho HJ, Rhee TK, Kim HS, Han JI, Lee DW, Cho SW, et al. Intravitreal bevacizumab for symptomatic retinal arterial macroaneurysm. Am J Ophthalmol 2013;155:5:898-904.