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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 33  |  Issue : 2  |  Page : 194-196

Bilateral vitreous hemorrhage with branch retinal vein occlusion secondary to hyperhomocysteinemia: A rare case report


Department of Ophthalmology, JIPMER Hospital, Gorimedu, Puducherry, India

Date of Submission01-Jun-2020
Date of Decision09-Jun-2020
Date of Acceptance18-Jun-2020
Date of Web Publication21-Aug-2021

Correspondence Address:
Dr. Sandip Sarkar
Department of Ophthalmology, JIPMER Hospital, Gorimedu - 605 006, Puducherry
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/kjo.kjo_74_20

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  Abstract 


Retinal vascular occlusions are uncommon in young people and require more in-depth investigation into the cause. A high level of circulating homocysteine carries a risk for retinal vaso-occlusive events. We, hereby, report a rare case of hyperhomocysteinemia-related bilateral branch retinal vein occlusion (BRVO). A 27-year-old male presented with sudden painless gross diminution of vision in the left eye (LE) for 10 days. Further assessment revealed inferotemporal BRVO (ITBRVO) in the right eye (RE) with mild vitreous hemorrhage (VH) and dense VH in the LE. Extensive investigations into the cause revealed an elevated level of serum homocysteine. The patient was started on Vitamin B6 and B12 supplementation. RE sectoral panretinal photocoagulation and LE pars plana vitrectomy were done. LE ITBRVO was noted intraoperatively for which endolaser was done. Hyperhomocysteinemia through its prothrombotic properties is an independent modifiable risk factor for vascular thrombotic events. Hence, it should be considered as a differential diagnosis for retinal vascular occlusions to prevent further recurrences and cerebrovascular events, especially in young adults.

Keywords: Bilateral branch retinal vein occlusion, hyperhomocysteinemia, vitreous hemorrhage


How to cite this article:
Deb AK, Rathinam N, Sarkar S, Suneel S, MalvikaMani, Kaliaperumal S. Bilateral vitreous hemorrhage with branch retinal vein occlusion secondary to hyperhomocysteinemia: A rare case report. Kerala J Ophthalmol 2021;33:194-6

How to cite this URL:
Deb AK, Rathinam N, Sarkar S, Suneel S, MalvikaMani, Kaliaperumal S. Bilateral vitreous hemorrhage with branch retinal vein occlusion secondary to hyperhomocysteinemia: A rare case report. Kerala J Ophthalmol [serial online] 2021 [cited 2021 Nov 30];33:194-6. Available from: http://www.kjophthal.com/text.asp?2021/33/2/194/324201




  Introduction Top


Bilateral retinal vein occlusions (RVO) are very rare with a 0.04% of 15-year cumulative incidence as reported in Beaver Dam study.[1] Bilateral RVO in young adults may be the initial presentation of a severe potentially life-threatening systemic disease. It, therefore, warrants detailed systemic workup to rule out hypercoagulable, myeloproliferative, and inflammatory disorders. Hyperhomocysteinemia is one such common hypercoagulable state causing vaso-occlusive events. Only very few cases of branch retinal vein occlusion (BRVO) secondary to hyperhomocysteinemia have been reported in literature.[2],[3],[4] We, hereby, report a unique case of bilateral inferotemporal branch retinal vein occlusion (ITBRVO) associated with dense visually debilitating vitreous hemorrhage (VH) in one eye and mild inferior VH in the other eye in a young healthy male with hyperhomocysteinemia.


  Case Report Top


A 27-year-old male presented to us with a history of sudden-onset painless gross diminution of vision in the left eye (LE) for 10 days associated with floaters. There was no history of trauma to the eye and no similar history in the past. There were no visual complaints in his right eye (RE). He had no other systemic comorbidities. He was a nonsmoker. On examination, best-corrected visual acuity (BCVA) was 6/6 in the RE and hand movements in the LE. Anterior segment examination was normal in both the eyes. Fundus examination of the RE showed multiple superficial retinal hemorrhages with sclerosed vessels in the IT quadrant suggestive of ITBRVO [Figure 1]a and [Figure 1]b. There was also mild dispersed VH inferiorly. Media was very hazy in the LE due to dense dispersed VH with no view of the retina. Ultrasonography-B scan of the LE confirmed the presence of VH with total posterior vitreous detachment and no evidence of retinal detachment [Figure 2]a. Complete hemogram, blood sugar profile, erythrocyte sedimentation rate, and renal function test were all within the normal limits. C-reactive protein and Mantoux tests were negative. Chest X-ray was normal. Workup for systemic vasculitis including antinuclear antibody and antineutrophil cytoplasmic antibodies was normal. Protein C, Protein S, and antithrombin III levels were normal. Serum homocysteine level was, however, found to be elevated,- 30.12 μmol/L. The patient was started on capsule homocheck (cyanocobalamin 400 μg + pyridoxine 10 mg + folic acid 1 mg) and tablet Vitamin C 500 mg BD. RE sectoral panretinal photocoagulation was done in the IT quadrant. One month later, RE fundus examination showed resolved vitreous and retinal hemorrhages, whereas LE showed nonresolving VH. BCVA in the RE was 6/6 and hand movements in the LE. Pars plana vitrectomy was done subsequently in the LE. Intraoperative fundus examination in the LE showed superficial retinal hemorrhages, new vessels, and sclerosed vessels along the IT quadrant suggestive of LE ITBRVO [Figure 2]b. Endolaser was done along the IT quadrant during vitrectomy. BCVA in the LE improved to 6/6 2 weeks following surgery. Both eyes maintained status quo at 12 months of follow-up till date.
Figure 1: Fundus images of the right eye (a and b) showing retinal hemorrhages in inferotemporal quadrant, sectoral laser marks (arrows), and mild vitreous hemorrhage suggestive of inferotemporal branch retinal vein occlusion

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Figure 2: Ultrasound B scan image of the left eye (a) showing dense vitreous hemorrhage and total posterior vitreous detachment, and fundus image of the left eye in the postoperative period (b) showing sclerosed vessel (arrow) in the inferotemporal quadrant and endolaser marks suggestive of inferotemporal branch retinal vein occlusion

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  Discussion Top


VH is one of the most important causes for spontaneous and sudden-onset gross diminution of vision in an young adult. Spontaneous VH in young adults can be secondary to high-risk proliferative diabetic retinopathy (PDR), ischemic RVO, familial exudative vitreoretinopathy, hematological disorders and blood dyscrasias including proliferative sickle-cell retinopathy and leukemias, and retinal vasculitis including Eales disease, tuberculosis (TB), and systemic lupus erythematosus (SLE). Blood sugar profile, blood pressure, and complete hemogram including peripheral smear evaluation were all normal in our case, thereby ruling out PDR and hematological disorders. Eales' disease is another common differential diagnosis for VH in the young healthy Indians. More than 60% of cases have vitreous hemorrhage at presentation.[5],[6] Chest X-ray and Mantoux test were normal in our case, thereby ruling out TB and Eales' disease. Another important etiology of VH to consider in our case was RVO. The RE had shown BRVO with retinal hemorrhages and mild VH (occlusive vasculopathy) at presentation, whereas the LE also had evidence of occlusive vasculopathy based on intraoperative retinal examination. Therefore, we had a provisional diagnosis of bilateral RVO or occlusive vasculopathy with VH in our case. Bilateral RVOs are very rare as reported in Beaver Dam study.[1] Bilateral RVO warrants more extensive evaluation to rule out all possible systemic diseases – diabetes, elevated blood pressure, hypercholesterolemia, hypercoagulable, myeloproliferative and inflammatory disorders, etc., Hypercoagulable disorders causing retinal vascular occlusive disease include hyperhomocysteinemia, antiphospholipid syndrome, factor V Leiden mutation, protein C and S resistance, and antithrombin deficiency. Myeloproliferative disorders comprise of polycythemia, multiple myeloma, Waldenström macroglobulinemia, etc., whereas inflammatory conditions include occlusive periphlebitis-sarcoidosis, SLE, and Behçet's disease.[7] Systemic workup for all suspected etiologies for bilateral RVO in our case was normal except for elevated serum homocysteine level. Various studies have shown relations of elevated serum levels of homocysteine with occurrence of RVOs, but there is a lack of conclusive evidence for this association.[8],[9] Carral-Santander et al. have reported a case of bilateral retinal occlusive vasculopathy involving both retinal arteries and veins in a 43-year-old male secondary to hyperhomocysteinemia.[3] Gore et al. have reported unilateral extramacular multiple BRVOs in a 45-year-old male with hyperhomocysteinemia.[2] Kumar et al. have reported unilateral central retinal vein occlusion with cilioretinal artery occlusion in a 27-year-old healthy male with hyperhomocysteinemia.[4] To the best of our knowledge, ours is the only case report with bilateral VH secondary to extramacular BRVO in a case of hyperhomocysteinemia.

Hyperhomocysteinemia is one of the most common causes of hypercoagulable disorder. It can be graded as mild (15–30 μmol/L), moderate (30–100 μmol/L), and severe (>100 μmol/L) based on fasting serum concentrations. Mild-to-moderate elevation of plasma homocysteine levels is usually associated with retinal vascular occlusive disease. Our patient also had moderately elevated (30.12 μmol/L) serum homocysteine levels. Hyperhomocysteinemia-related retinal blood vessel damage is multifactorial and includes induced oxidative stress (redox stress), reduced nitric oxide bioavailability, altered expression of various thrombotic factors, increased platelet activation, increased coagulability, and mutagenic effect on the arterial smooth muscle cells.[2],[9],[10]

Treatment of the RE in our case included sectoral laser of IT quadrant. Treatment of the LE included pars plana vitrectomy with endolaser. Frequent follow-up is advised in such cases to check for the adequacy of treatment and also to detect any new episodes of vascular occlusion as they are prone to bilateral and multiple vascular occlusions. It is also essential to detect and treat any underlying cause of secondary hyperhomocysteinemia. Vitamins B6 and B12 and folic acid are important cofactors necessary for transsulfuration and re-methylation of homocysteine. Oral supplementation of these cofactors is, therefore, recommended to improve homocysteine metabolism and potentially reduce the incidence of future vascular occlusive events as well as improve vascular endothelial functions.[3]


  Conclusion Top


Hyperhomocysteinemia should be considered as a differential diagnosis for retinal vascular occlusions with or without VH in young adults. Patients presenting with such a clinical picture should undergo thorough investigations to identify and treat any modifiable risk factor such as hyperhomocysteinemia. This will ensure the prevention of any further fatal thromboembolic or vaso-occlusive events in the future.[2],[6]

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.
Klein R, Moss SE, Meuer SM, Klein BE. The 15-year cumulative incidence of retinal vein occlusion: The Beaver Dam Eye Study. Arch Ophthalmol 2008;126:513-8.  Back to cited text no. 1
    
2.
Gore AD, Rao GS, Gore MA, Desai AR. Multiple extra macular branch retinal vein occlusions in hyperhomocysteinemia. Indian J Ophthalmol 2014;62:489-91.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Carral-Santander IE, Santos-Palacios A, Martínez-Baez BE, Cernichiaro-Espinosa L, Elizondo-Camacho JM, Valdés-Lara CA, et al. Secondary hyperhomocysteinemia-related occlusive retinal vasculopathy: A case report. Am J Ophthalmol Case Rep 2019;13:41-5.  Back to cited text no. 3
    
4.
Kumar V, Chandra P, Kumar A. Central retinal vein occlusion with cilioretinal artery occlusion in hyperhomocysteinemia. BMJ Case Rep 2015;2015:bcr2015212259.  Back to cited text no. 4
    
5.
Saxena S, Jalali S, Verma L, Pathengay A. Management of vitreous haemorrhage. Indian J Ophthalmol 2003;51:189-96.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Sudhalkar A, Chhablani J, Rani PK, Jalali S, Balakrishnan D, Tyagi M. Bilateral vitreous hemorrhage in children: Clinical features and outcomes. J Ophthalmic Vis Res 2015;10:139-43.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
Castro-Navarro V, Odaibo SG, Ghodasra DH, Besirli CG. Bilateral BRVO in a patient with recurrent prostate cancer. BMJ Case Rep 2015;2015:bcr201521246.  Back to cited text no. 7
    
8.
Al Wadani F, Khandekar R, Salim G, Al Ali M, Ramzi S. Hyperhomocysteinia is a risk factor for retinal venous occlusion: A case control study. Indian J Ophthalmol 2014;62:291-4.  Back to cited text no. 8
    
9.
Taubert M, Dowd TC, Wood A. Malnutrition and bilateral central retinal vein occlusion in a young woman: A case report. J Med Case Rep 2008;2:77.  Back to cited text no. 9
    
10.
Shute C. A case report of branch retinal artery occlusion in a teenager due to hyperhomocysteinaemia; the interplay of genetic and nutritional defects. BMC Ophthalmol 2018;18 Suppl 1:220.  Back to cited text no. 10
    


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