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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 739-741

Retinal racemose hemangioma and macroaneurysm with multilayered hemorrhage: A case report


Department of Retina, Susrut Eye Foundation and Research Centre, Kolkata, West Bengal, India

Date of Submission05-Aug-2020
Date of Acceptance06-Mar-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Madhurima Roy
Fellow Vitreo Retina, Susrut Eye Foundation and Research Centre, Kolkata, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_2496_20

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  Abstract 


A 42-year-old female presented with sudden diminution of vision in the right eye (RE) for 1 day. The vision was counting fingers with Relative afferent pupillary defect (RAPD) (RE) and 20/20 (LE). Fundus revealed dilated tortuous arteries and veins in all quadrants with premacular and submacular hemorrhage. Fundus fluorescein angiography (FFA) showed nonleaking vascular loops with hypofluorescence at the macula. Optical coherence tomography (OCT) revealed localized posterior hyaloid detachment with hemorrhage. We report this unilateral case of retinal racemose hemangioma with multilayered hemorrhage managed with pneumatic displacement and revealed retinal artery macroaneurysm in the background. On follow-up there was development of localized vitreomacular traction. It was treated with Yttrium Aluminium Garnet (YAG) hyaloidotomy, which improved vision to 20/80.

Keywords: Focal VMT, multilayered hemorrhage, retinal arterial macroaneurysm, retinal racemose hemangioma


How to cite this article:
Roy S, Maiti A, Mondal P, Mandal S, Roy M. Retinal racemose hemangioma and macroaneurysm with multilayered hemorrhage: A case report. Indian J Ophthalmol Case Rep 2021;1:739-41

How to cite this URL:
Roy S, Maiti A, Mondal P, Mandal S, Roy M. Retinal racemose hemangioma and macroaneurysm with multilayered hemorrhage: A case report. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 21];1:739-41. Available from: https://www.ijoreports.in/text.asp?2021/1/4/739/327652



Retinal racemose hemangioma (RRH) is a rare congenital phakomatosis present as predominantly unilateral dilated and tortuous vessels extending from disc to retina, and is usually innocuous. Sometimes it presents with exudation, retinal hemorrhage, and central venous occlusion.[1] It is occasionally associated with arteriovenous malformations in the brain, as seen in Wyburn-Mason syndrome.[2]

We report this case of retinal racemose hemangioma presenting with premacular and submacular hemorrhage, which was managed with pneumatic dispersion of the hemorrhage and later revealed retinal artery macroaneurysm beneath the hemorrhage. It was later complicated by a localized vitreous detachment causing vitreomacular traction (VMT), which was managed with YAG hyaloidotomy.


  Case Report Top


A 42-year-old female presented with sudden onset diminution of vision in the right eye (RE) for 1 day. The best-corrected visual acuity (BCVA) was finger counting close to face in RE and 20/20 in the left eye (LE). Anterior segment was within normal limits in both eyes except RAPD in RE. On posterior segment evaluation, RE had dilated and tortuous arteries and veins in all four quadrants, which was more prominent in the inferior quadrant with premacular and submacular hemorrhage. There was no angioma seen. LE fundus was within normal limits. Clinically, the patient was diagnosed with racemose angioma with premacular and submacular hemorrhage [Figure 1]a. Fundus fluorescein angiography (FFA) showed dilated and tortuous vessels in all quadrants with vascular loops which were not leaking, with hypofluorescence at macula due to premacular hemorrhage [Figure 1]b. Optical coherence tomography (OCT) showed localized posterior hyaloid detachment with premacular and submacular hemorrhage [Figure 1]c. Brain magnetic resonance imaging (MRI) and abdominal ultrasound were done to rule out Wyburn-Mason syndrome. Intravitreal C3F8 gas (0.3 mL, 100%) was injected to disperse the submacular hemorrhage after doing paracentesis, followed by strict prone positioning for 7 days. Intravitreal tissue plasminogen was avoided as a precautionary measure to prevent further bleeding from the dilated vessels.
Figure 1: (a) Fundus photograph of the right eye showing dilated and tortuous vessels in all four quadrants most prominently in the inferior part with subretinal and preretinal hemorrhage involving the macula. (b) Fundus fluorescein angiography of the same eye showing filling of dilated vessels without leakage. Hypofluorescence caused by blockage over the posterior pole, darker one suggestive of preretinal hemorrhage obscuring underlying details, and lesser hypofluorescence with overlying retinal blood vessels suggestive of submacular hemorrhage (c) Optical coherence tomography through the fovea showing hyperreflective thin membrane suggesting posterior hyaloid detachment with underlying heterogeneous premacular and subretinal hemorrhage obscuring choroidal details

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At a 7-day follow-up, BCVA improved to 20/200 and the blood was dispersed inferiorly from subfoveal space with a gas bubble visible in the superior quadrant [Figure 2]a. OCT showed posterior hyaloid detachment with minimal blood in the subfoveal region [Figure 2]b. At 2 weeks of follow-up, BCVA had improved to 20/120 with complete dispersion of blood. On careful examination with slit-lamp biomicroscope, a retinal artery macroaneurysm (RAM) was seen in the inferotemporal arcade, which was not visible on presentation due to the hemorrhage [Figure 3]a. Focal Nd YAG laser was done to ablate RAM using low power (50 mW) and increased time duration (400 ms). Follow-up OCT revealed evolving vitreomacular traction (VMT) with foveal detachment [Figure 3]b. The patient was asked to follow-up in anticipation of spontaneous release of VMT with posterior vitreous detachment induction. At 4 weeks, the patient complained of a drop in vision. BCVA dropped to Finger count (FC) at 4 ft. The posterior segment examination did not reveal any rebleed although the localized VMT had become prominent. OCT showed deterioration of the VMT with increased distortion of the fovea [Figure 3]c. Since clinically the VMT was visible as a localized vitreous detachment at the fovea, YAG hyaloidotomy was tried as a bridging procedure. At 2 weeks, BCVA improved to 20/80. On examination, the blood was completely displaced from the macula with the release of VMT. OCT showed complete resolution of the VMT with foveal thinning [Figure 4].
Figure 2: (a) Fundus photo of the same after 1 week of pneumatic displacement where blood is dispersed inferiorly as fovea is visible and resolving gas bubble seen in superior quadrant. (b) Optical coherence tomography after a 1-week postoperative period, posterior hyaloid detachment with a decrease in height of hemorrhage in the foveal region

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Figure 3: (a) Magnified view after 2 weeks of the postoperative period showing displaced hemorrhage from the foveal region and revealing underlying retinal artery macroaneurysm along the inferotemporal arcade. Also, the outline of focal hyaloid detachment is visible, correlating with the OCT image. (b) OCT scan along the region revealed RAM lesion (white arrow) with vitreomacular traction with focal hyaloid detachment. (c) OCT scan after 6 weeks of follow-up showing increased vitreomacular traction with focal posterior hyaloid detachment distorting retinal layers

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Figure 4: (a) Fundus photo showing the complete dissolution of hemorrhage with involuted RAM along with few hard exudates. (b) OCT scan after 2 weeks of laser hyaloidotomy showing the complete release of vitreomacular traction with no distortion of the retina and foveal thinning

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  Discussion Top


Retinal racemose hemangioma (RRH) is a rare unilateral, nonhereditary, sporadic phakomatosis characterized by direct communication between arteries and veins.[3] Near about 30% of RRH patients have a coexisting arteriovenous malformation (AVM) in the brain as seen in Wyburn-Mason syndrome. It can occur as an isolated lesion or as a component of this syndrome. Archer et al. proposed a classification of congenital arteriovenous anastomosis in three groups based on dimensions and presence of an interposed capillary bed.[3] There is a high risk for vision loss due to complications such as retinal vein occlusion (45%), hemorrhage (33%), macular edema, macroaneurysms, and complications associated with intracranial AVM.[4] Various hypotheses postulated that ocular complications in AVMs are caused by hyperdynamic blood flow through small-caliber vessels, and steal phenomenon caused by increased venous pressure with decreased arterial pressure.[5] Our patient had grade 3 AVM with complex overlapping arteries and veins with retinal hemorrhage. MRI brain was performed to rule out related midbrain and mandibular lesion of Wyburn-Mason syndrome.

Retinal arterial macroaneurysm (RAM) is an acquired, isolated dilatation of a retinal arteriole usually associated with hemorrhage, edema, and exudations. The misdiagnosis rate of RAM at first presentation is around 75% when there is preretinal, intraretinal, or vitreous hemorrhage (VH), like in our case.[6] VH is seen in 2%–10% of cases of ruptured RAM and simultaneous subretinal and preretinal hemorrhage have been reported in up to 40% of cases.[7] In this communication, we describe this rare combination of RRH with RAM. Retrospective reports of Sub retinal hemorrhage (SRH) demonstrated that without treatment it leads to poor vision as long-standing blood damages the photoreceptors permanently. This is due to a combined effect of chemical toxins, outer retinal shear forces, and/or a barrier effect limiting diffusion between the photoreceptors and Retinal pigment epithelium (RPE).[8],[9] Therefore, displacement of hemorrhage is recommended as soon as possible. In our case, we demonstrate successful displacement of blood and significant improvement of vision with intravitreal injection of expansile gas performed within 1 day of onset of symptoms. It seems to be a safe and cost-effective procedure with no major complications. After the dissolution of blood, underlying a RAM was found as the causative factor and a focal laser was done over and surrounding the RAM lesion as it was threatening vision. Smaller case series have yielded the role of photocoagulation in RAM, usually when edema or exudation is persisting and causing decreased visual acuity. Laser photocoagulation had a role in reducing leakage in approximately 16%–27% of RAMs. A recent report demonstrated that a focal laser was needed to treat recurrent cystoid macular edema, even after anti-vascular endothelial growth factor (VEGF) injection in cases of hemorrhage-associated RAM.[10] Nd: YAG laser hyaloidotomy is a minimally invasive treatment option and offers numerous advantages. The possibility of a rapid increase in vision (if no other macular pathology present), the low rate of complications, the good availability, and the low costs make hyaloidotomy a good treatment option. The goal in treating posterior vitreous detachment with focal VMT was to perform a bridging procedure before attempting major surgical intervention. Although there is no sufficient evidence for YAG laser in VMT and laser application might have contributed partially in increasing the traction, our patient regained vision of 20/80. This may be an area of further research.


  Conclusion Top


Any case of multilayered retinal hemorrhage should be addressed with caution as RAM can be one of the coassociation with retinal racemose hemangioma. Vision-threatening RAM should be intervened early. Any focal VMT on follow-up can be managed conservatively or by YAG hyaloidotomy before proceeding to major surgical intervention, though more evidence is needed.

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 initials 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 Top

1.
Mansour AM, Wells CG, Jampol LM, Kalina RE. Ocular complications of arteriovenous communications of the retina. Arch Ophthalmol 1989;107:232-6.  Back to cited text no. 1
    
2.
Wyburn-Mason R. Arteriovenous aneurysm of mid-brain and retina, facial naevi and mental changes. Brain 1943;66:163-203.  Back to cited text no. 2
    
3.
Archer DB, Deutman A, Ernest JT, Krill AE. Arteriovenous communications of the retina. J Am Optom Assoc 1973;75:224-41.  Back to cited text no. 3
    
4.
Qin X-J, Huang C, Lai K. Retinal vein occlusion in retinal racemose hemangioma: A case report and literature review of ocular complications in this rare retinal vascular disorder. BMC Ophthalmol 2014;14:101.  Back to cited text no. 4
    
5.
Sharma P, Sridhar J, Rayess N, Maguire JI. Optical coherence tomography angiography (OCT-A) of type 2 retinal arteriovenous malformation. Can J Ophthalmol 2015;50:e93-6.  Back to cited text no. 5
    
6.
Lavin MJ, Marsh RJ, Peart ST, Rehman A. Retinal arterial macroaneurysms: A retrospective study of 40 patients. Br J Ophthalmol 1987;71:817-25.  Back to cited text no. 6
    
7.
Goff MJ, McDonald HR, Johnson RN, Ai E, Jumper JM, Fu AD. Causes and treatment of vitreous hemorrhage. Compr Ophthalmol Update 2006;7:97-111.  Back to cited text no. 7
    
8.
Speilburg AM, Klemencic SA. Ruptured retinal arterial macroaneurysm: Diagnosis and management. J Optom 2014;7:131-7.  Back to cited text no. 8
    
9.
Tonotsuka T, Imai M, Saito K, Iijima H. Visual prognosis for symptomatic retinal arterial macroaneurysm. Jpn J Ophthalmol 2003;47:498-502.  Back to cited text no. 9
    
10.
Leung EH, Reddy AK, Vedula AS, Flynn Jr HW. Serial bevacizumab injections and laser photocoagulation for macular edema associated with a retinal artery macroaneurysm. Clin Ophthalmol 2015;9:601-9.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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