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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 1  |  Page : 229-231

A rare case of bilateral superior ophthalmic vein thrombosis presenting simultaneously with central retinal vein occlusion and cerebral venous sinus thrombosis


1 Department of Ophthalmology, Institute of Medical Sciences and SUM Hospital, Sikshya O Anusandhan (Deemed to be) University, Bhubaneswar, Odisha, India
2 Department of Medicine, Institute of Medical Sciences and SUM Hospital, Sikshya O Anusandhan (Deemed to be) University, Bhubaneswar, Odisha, India

Date of Submission03-Jul-2021
Date of Acceptance17-Aug-2021
Date of Web Publication07-Jan-2022

Correspondence Address:
Dr. Pradeep K Panigrahi
Department of Ophthalmology, Institute of Medical Sciences and SUM Hospital, Sikshya O Anusandhan (Deemed to be) University, 8-Kalinga Nagar, Bhubaneswar - - 751 003, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_1780_21

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  Abstract 


A 57-year-old female presented with redness in both eyes and headache for 1-month duration. The clinical examination showed bilateral episcleral vessels congestion and central retinal vein occlusion. Optical coherence tomography scans were normal in both eyes. Radiological examination revealed bilateral superior ophthalmic vein thrombosis and venous sinus thrombosis involving the left terminal sigmoid sinus. The serum homocysteine level was elevated. The patient was treated with anticoagulants and vitamin B12 supplements. Three months following the initial presentation, there was a reduction in the episcleral vessel congestion and retinal hemorrhages. The magnetic resonance venography at the end of 3 months showed a partially recanalized left jugular bulb with chronic thrombosis of the left terminal sigmoid sinus.

Keywords: Central retinal vein occlusion, cerebral venous thrombosis, homocysteine, superior ophthalmic vein thrombosis


How to cite this article:
Panigrahi PK, Samant S, Dhar SK. A rare case of bilateral superior ophthalmic vein thrombosis presenting simultaneously with central retinal vein occlusion and cerebral venous sinus thrombosis. Indian J Ophthalmol Case Rep 2022;2:229-31

How to cite this URL:
Panigrahi PK, Samant S, Dhar SK. A rare case of bilateral superior ophthalmic vein thrombosis presenting simultaneously with central retinal vein occlusion and cerebral venous sinus thrombosis. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 May 24];2:229-31. Available from: https://www.ijoreports.in/text.asp?2022/2/1/229/334907



Superior ophthalmic vein thrombosis (SOVT) is an extremely rare clinical entity which can lead to devastating complications. It can happen secondary to both infective and non-infective causes.[1] There are very few reports of SOVT associated with retinal vein occlusion in the literature.[2],[3] We report a very rare case of bilateral SOVT with bilateral central retinal vein occlusion and cerebral venous sinus thrombosis with associated elevated homocysteine levels.


  Case Report Top


A 57-year-old non-diabetic and non-hypertensive female presented with redness in both eyes for 1-month duration. She also had complaints of headache and facial pain for 1-month duration. The redness of the eyes was not associated with watering, discharge, or loss of vision. She had consulted elsewhere and had been diagnosed with allergic conjunctivitis and prescribed topical anti-allergic and lubricating eye drops. The patient did not give any previous history of ocular or systemic diseases. She was not using any systemic medications at the time of presentation. There was no history of oral contraceptive use in the past.

On examination, the best-corrected visual acuity was 20/20, N6 in both eyes. The slit-lamp examination of the anterior segment showed bilateral dilated and tortuous episcleral blood vessels [Figure 1]a and [Figure 1]b. The rest of the anterior segment examination was within normal limits. The pupillary reflexes were brisk in both eyes. There were no signs of intraocular inflammation. The intraocular pressure measured using Goldmann's applanation tonometer was 15 and 18 mmHg in the right eye (RE) and left eye (LE), respectively. Gonioscopy examination showed blood in Schlemm's canal bilaterally. The fundus examination of the RE showed clear media, normal optic disk, dilated and tortuous retinal veins, cotton wool spots, and hemorrhages in all four quadrants [Figure 1]c. The fundus examination of the LE showed clear media, normal optic disk, dilated retinal veins, and a few retinal hemorrhages [Figure 1]d. The retinal hemorrhages were more numerous in the RE as compared to the LE. We diagnosed it as a case of central retinal vein occlusion (CRVO) in RE and impending CRVO in LE with prominent episcleral vessels bilaterally.
Figure 1: (a and b) Clinical slit-lamp photographs showing congested episcleral blood vessels in the right and left eyes. (c and d) Color fundus photographs of the right and left eyes showing tortuous, congested retinal veins with hemorrhages in all four quadrants suggestive of central retinal vein occlusion. (e and f) Optical coherence tomography scans of the right and left eyes showing normal foveal contour

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The patient was advised fundus fluorescein angiography which she refused. The optical coherence tomography (OCT) of the RE showed mild disruption of all retinal layers with intact foveal dip and absence of significant macular edema [Figure 1]e. The OCT of the LE showed intact foveal dip with normal foveal architecture [Figure 1]f. The magnetic resonance imaging (MRI) of the brain and orbits showed focal enlargement of the bilateral superior ophthalmic veins with a loss of flow signal which was suggestive of thrombosis [Figure 2]a. A magnetic resonance venogram (MRV) study was planned. The MRV study showed focal intraluminal venous sinus thrombosis involving the left terminal sigmoid sinus and left jugular bulb region [Figure 2]b. Focal thrombus was also noted in the bilateral superior ophthalmic veins. The left transverse and proximal part of the sigmoid sinus were found to be mildly thin but patent. The superior sagittal sinus, straight sinus, and bilateral transverse sinuses showed normal flow signal intensity.
Figure 2: (a) Magnetic resonance imaging of the brain and orbits showing focal loss of flow signal in the bilateral superior ophthalmic veins (black arrows) suggestive of superior ophthalmic vein thrombosis. (b) Magnetic resonance venogram showing focal intraluminal thrombosis involving the left terminal sigmoid sinus and left jugular bulb region (white arrow)

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The patient was now referred to a neuro physician for further investigations. Routine investigations including random blood sugar, complete blood count, C reactive protein, erythrocyte sedimentation rate, thyroid profile, etc., were within normal limits. The tests done for coagulation profile including prothrombin time, activated partial thromboplastin time, protein C, protein S, Factor V Leiden mutation were within normal limits. Other tests including anti-nuclear antibody, anti-phospholipid antibody, and anti-cardiolipin antibody were found to be normal. Her serum homocysteine level was 45.4 μmol/L (normal range 5–15 μmol/L).

The patient was treated with anticoagulants keeping a close watch on her coagulation parameters. She received subcutaneous injections of low-molecular-weight heparin (0.6 U) for 1 week. Along with this, she was started on oral acitrom (2 mg once daily) and vitamin B 12 supplements. On follow-up examination, 1 month after the initial presentation, the best-corrected visual acuity in both eyes was 20/20, N6. The patient acknowledged the reduction in the intensity of headaches. On examination, there was a mild reduction in the episcleral vessel congestion and retinal hemorrhages. Three months following the presentation, the best-corrected visual acuity was 20/20, N6. The episcleral congestion had decreased. There was a reduction of retinal hemorrhages in both eyes [Figure 3]a and [Figure 3]b. A repeat MRV showed a partially recanalized left jugular bulb with chronic thrombosis of the left terminal sigmoid sinus [Figure 3]c. Persistent thrombosis was noted in the bilateral superior ophthalmic veins. A repeat serum homocysteine test done showed that the serum homocysteine levels had reduced to 23 μmol/L but were still above normal limits. The patient was asked to continue medications as advised by the neuro physician and to come for regular ophthalmic consultations.
Figure 3: (a and b) Color fundus photographs of the right and left eyes 3 months following the initial presentation showing decreased retinal hemorrhages. (c) Magnetic resonance venogram image 3 months following the initial presentation showing partially recanalized left jugular bulb (white arrow) and chronic thrombosis of the left terminal sigmoid sinus with reduced caliber of the left transverse and sigmoid sinus

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  Discussion Top


SOVT can be caused by altered venous blood flow which can be secondary to the stasis of blood flow, trauma to the vessel wall, or hypercoagulability.[4] The patients can present with pain, chemosis, lid edema, proptosis, restricted ocular motility, with or without retinal findings, and impaired visual acuity. The diagnosis of SOVT is ascertained by radiological imaging, preferably contrast-enhanced computed tomography or MRI. Raised serum homocysteine level has been reported to be an independent risk factor associated with SOVT, cerebral venous thrombosis, and retinal vein occlusions.[5],[6],[7] The probable causes of thrombosis induced by hyperhomocysteinemia include endothelial toxicity, promotion of smooth muscle proliferation, intimal thickening, increased platelet adhesion, activation of Factor V, and decreased production of nitric oxide and prostacyclin.[8]

In the present case, serum homocysteine levels were found to be increased way beyond the normal limits. The elevated serum homocysteine levels might have led to thrombosis at multiple levels producing this clinical scenario. The central retinal vein drains into the cavernous sinus either directly or through the superior ophthalmic vein.[2] There is a possibility that in this patient, the central retinal veins of both sides emptied into the superior ophthalmic veins before finally draining into the cavernous sinus. Thrombosis of the superior ophthalmic vein proximal to its entry into the cavernous sinus could have led to CRVO. The patients with cerebral venous sinus thrombosis present with features of raised intracranial pressure like headache and clinical examination in most of the cases reveals papilledema. Following thrombus formation in the cerebral venous sinuses, there is a secondary increase in the cerebrospinal fluid pressure due to decreased absorption through the arachnoid granules. Thrombi most commonly affect the superior sagittal and lateral sinuses leading to raised intracranial tension.[9] The increased incidence of raised intracranial tension following thrombus formation in the superior sagittal sinus is because it is responsible for a majority of cerebral venous drainage.[9] Our patient had features of raised intracranial tension like headache but papilledema was absent. This might be due to the non-involvement of the superior sagittal sinus which showed normal flow signal intensity on MRV.


  Conclusion Top


Cerebral venous sinus thrombosis can present with symptoms of raised intracranial tension but with the absence of signs like papilledema. The presence of clinical signs like episcleral venous congestion, blood in Schlemm's canal, and retinal venous congestion are pointers to the possible intracranial etiology warranting neuroimaging studies. Prompt diagnosis and early intervention can help in preventing serious ocular and systemic complications.

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.
Sotoudeh H, Shafaat O, Aboueldahab N, Vaphiades M, Sotoudeh E, Bernstock J. Superior ophthalmic vein thrombosis: What radiologist and clinician must know? Eur J Radiol Open 2019;6:258-64.  Back to cited text no. 1
    
2.
Hsu J, Ibarra MS, Jacobs D, Galetta SL, Brucker AJ. Combined central retinal vein and artery occlusion associated with an isolated superior ophthalmic vein thrombosis. Retina 2004;24:452-4.  Back to cited text no. 2
    
3.
Park HS, Gye HJ, Kim JM, Lee YJ. A patient with branch retinal vein occlusion accompanied by superior ophthalmic vein thrombosis due to severe superior ophthalmic vein enlargement in a patient with graves ophthalmopathy. J Craniofac Surg 2014;25:e322-4. doi: 10.1097/SCS.0000000000000586.  Back to cited text no. 3
    
4.
van der Poel NA, de Witt KD, van den Berg R, de Win MM, Mourits MP. Impact of superior ophthalmic vein thrombosis: A case series and literature review. Orbit 2019;38:226-32.  Back to cited text no. 4
    
5.
Schaffer LR, Wells JR, Francis CE. Superior ophthalmic vein thrombosis secondary to hyperhomocysteinemia. J Neuroophthalmol. 2020. doi: 10.1097/WNO.0000000000001063.  Back to cited text no. 5
    
6.
Shang K, Li H, Luo X. Cerebral venous sinus thrombosis due to hyperhomocysteinemia with cystathionine-β-synthase (CBS) gene mutation: A case report. Medicine (Baltimore) 2019;98:e14349. doi: 10.1097/MD.0000000000014349.  Back to cited text no. 6
    
7.
Vine AK. Hyperhomocysteinemia: A risk factor for central retinal vein occlusion. Am J Ophthalmol 2000;129:640-4.  Back to cited text no. 7
    
8.
Martinelli I, Passamonti SM, Bucciarelli P. Thrombophilic states. Handb Clin Neurol 2014;120:1061-71.  Back to cited text no. 8
    
9.
O'Rourke TL, Slagle WS, Elkins M, Eckermann D, Musick A. Papilloedema associated with dural venous sinus thrombosis. Clin Exp Optom 2014;97:133-9.  Back to cited text no. 9
    


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