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Year : 2022  |  Volume : 2  |  Issue : 2  |  Page : 552-553

Bilateral isolated superior ophthalmic vein thrombosis: An initial presentation of antiphospholipid syndrome

Oculoplasty and Paediatric Ophthalmology Services, Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India

Date of Submission21-Oct-2021
Date of Acceptance17-Jan-2022
Date of Web Publication13-Apr-2022

Correspondence Address:
Deepsekhar Das
Oculoplasty and Pediatric Ophthalmology Services, Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2666_21

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Bilateral superior ophthalmic vein thrombosis (SOVT) being extremely rare requires diligent evaluation for a definitive diagnosis. A 19-year-old male presented with pain and swelling around both eyes for 5 days. He had bilateral proptosis with gross limitation of movements (extraocular movement) and visual acuity of 6/9 in both eyes. On palpation, no mass was found. Contrast-enhanced magnetic resonance imaging orbit demonstrated bilateral superior ophthalmic vein dilation. Keeping a clinical suspicion of bilateral SOVT, systemic evaluation revealed raised anticardiolipin antibody levels at two occasions 12-weeks apart. A diagnosis of antiphospholipid syndrome was made and anticoagulant therapy was commenced. At 2-week follow-up, there was marked improvement in clinical features.

Keywords: APLA syndrome, ocular anticoagulant therapy, ocular thrombosis, proptosis, superior ophthalmic vein thrombosis

How to cite this article:
Agrawal S, Neupane S, Modaboyina S, Bajaj MS, Das D. Bilateral isolated superior ophthalmic vein thrombosis: An initial presentation of antiphospholipid syndrome. Indian J Ophthalmol Case Rep 2022;2:552-3

How to cite this URL:
Agrawal S, Neupane S, Modaboyina S, Bajaj MS, Das D. Bilateral isolated superior ophthalmic vein thrombosis: An initial presentation of antiphospholipid syndrome. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 May 18];2:552-3. Available from: https://www.ijoreports.in/text.asp?2022/2/2/552/342972

Thrombosis of the superior ophthalmic vein (SOV) is a rare orbital condition that is typically characterized by a sudden development of painful proptosis and conjunctival injection. The condition may lead to diminution of vision if the optic nerve gets involved. The orbit gets congested due to impaired venous outflow. A preceding cavernous sinus pathology is the most common reason for a superior ophthalmic vein thrombosis (SOVT).[1]

The term “Isolated SOVT” is used when SOVT occurs without any systemic association or without evidence of cavernous sinus thrombosis.[1] It is manifested by lid involvement, mucocutaneous involvement, secondary Sjogren's syndrome, choroidopathy, and neuro-ophthalmic disorders.[2]

  Case Report Top

A 19-year-old male presented to our casualty with a history of pain and swelling around the right eye for 3 days. Symptoms were gradually progressive. There was no history of trauma.

On examination, visual acuity in the right eye was 5/60 and that in the left eye was 6/24. Intraocular pressure in both eyes was 18 mmHg. There was a marked restriction of movements in both eyes, more so in the right eye, associated with proptosis in both eyes. Hertel's exophthalmometry was 22 in both eyes. There was upper and lower lid swelling in both eyes, with bilateral superior orbital fullness was noted [Figure 1]a and [Figure 1]b. There was no detectable bruit noted in either orbit. On anterior segment examination, there was congestion and chemosis in both eyes with signs of exposure keratopathy. Fundus examination revealed no abnormality.
Figure 1: (a and b) Clinical photograph of the patient showing upper and lower lid swelling in both eyes with bilateral superior orbital fullness and proptosis. (c and d) Axial and coronal sections of CECT head and orbit showing hypodense, bilateral nonenhancing lobulated lesion in intraconal as well as extraconal compartments, extending into preseptal spaces displacing optic nerve, superior rectus, and superior oblique muscles. (e) Clinical photograph of the patient after 2 weeks treatment with anticoagulant, showing a marked resolution of symptoms and proptosis

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Prothrombin time was found to be 13.600 s, and international normalized ratio (INR) was found to be 1.169. Serum cardiolipin level was raised along with a high IgA level at two occasions 12 weeks apart. Contrast-enhanced computed tomography (CECT) was done, which revealed bilateral hyperdense nonenhancing lobulated lesions in intraconal as well as extraconal compartments, extending into preseptal spaces displacing the optic nerve, superior rectus, and superior oblique [Figure 1]d and [Figure 1]e. Contrast-enhanced magnetic resonance imaging (CEMRI) was done to confirm the diagnosis of bilateral SOVT.

The patient was started on anticoagulant therapy with warfarin given 8 mg once a day and lubricants for exposure keratopathy. INR was maintained between 2.0 and 3.0, while he was on anticoagulant therapy. On 2 weeks follow-up, visual acuity in the right eye was found to be 6/24, improving to 6/12 and that in the left eye was 6/12, improving to 6/9 with a marked resolution of symptoms and proptosis [Figure 1]c.

  Discussion Top

SOVT commonly occurs secondary to orbital inflammations, infections, compression by tumors, and carotid-cavernous fistula. The components of Virchow's triad (vascular damage, stasis, and hypercoagulability) are considered risk factors contributing to the development of SOVT.

Septic causes leading to SOVT are orbital cellulitis, paranasal sinusitis, and septic cavernous sinus thrombosis. Spontaneous thrombosis of the dural cavernous fistula, tumors of the cavernous sinus, facial trauma, and Tolosa–Hunt syndrome are aseptic causes of SOVT.[1]

Systemic factors that can be attributed to the development of SOVT include hypercoagulable states (use of oral contraceptive pills, pregnancy, thrombocytosis, sickle cell trait, and hereditary hemorrhagic telangiectasia), inflammatory diseases like Grave's disease, systemic lupus erythematosus, sarcoidosis, Behcet's syndrome and amyloidosis, systemic malignancies like leukemia, lymphoma, and multiple myeloma.[1] Orbital pathology commonly contribute to unilateral SOVT, whereas systemic conditions can lead to unilateral or bilateral SOVT.

Unilateral SOVT associated with orbital cellulitis has been described by Luxenberg et al.[3]; Berenholz et al.[4] described its association with ethmoidal sinusitis, and Jaais and Habib described its association with oral contraceptive pills.[5] Michaelides et al.[6] reported bilateral SOVT in a young woman with increased factor VIII level.

Bilateral SOVT related to antiphospholipid syndrome has rarely been reported. Idrees Z et al.[7] had reported one such case, but in an elderly (77 years old) female. Another case of SOVT related to antiphospholipid syndrome that has been reported was unilateral and in adult (41 years old) male.[8]

Antiphospholipid syndrome is an acquired autoimmune disorder. It is diagnosed clinical criteria of objectively confirmed recurrent thrombosis (arterial, venous, and/or small vessel) and laboratory criteria of high anticardiolipin antibodies or persistently positive lupus anticoagulant and/or presence of anti-β2-glycoprotein-1 antibody.[9] It encompasses a wide variety of ocular manifestations, the most common being vascular occlusions of the retina.[9]

The diagnosis of SOVT is made based on imaging findings. On CECT, SOVT is characterized by thickened SOV with an indistinct outer border. A filling defect may be seen within the SOV. Contrast-enhanced magnetic resonance imaging shows enhancement of intraorbital fat and swelling and enhancement of the eye musculature. It can also show an intraluminal filling defect in the SOV.[10] Treatment options include antibiotics, steroids, and anticoagulant therapy. As highlighted previously, it is important to exclude an infectious cause and thereafter early use of antibiotics. There are no controlled studies to support the efficacy of the use of steroids and anticoagulant therapy due to the rarity of the condition. Surgery is reserved for cases associated with sinusitis, orbital abscess, or dental infection.[10]

  Conclusion Top

In conclusion, SOV occlusion is usually unilateral and associated with conditions like infections of the midface, Grave's ophthalmopathy, orbital pseudotumor, vascular malformations, and Tolosa–Hunt syndrome. Clinical signs and imaging like ultrasound B-scan, CECT, and CEMRI help the ophthalmologist in making a diagnosis of SOVT. Early initiation of anticoagulant therapy in SOVT helps in preventing complications like cavernous sinus thrombosis and marked symptomatic improvement in visual acuity. Management involves a multidisciplinary approach involving ophthalmologists, hematologists, and rheumatologists.

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


Conflicts of interest

There are no conflicts of interest.

  References Top

Rao R, Ali Y, Nagesh CP, Nair U. Unilateral isolated superior ophthalmic vein thrombosis. Indian J Ophthalmol 2018;66:155-7.  Back to cited text no. 1
[PUBMED]  [Full text]  
Sambhav K, Shakir O, Chalam KV. Bilateral isolated concurrent superior ophthalmic vein thrombosis in systemic lupus erythematosus. Int Med Case Rep J 2015;8:181-3.  Back to cited text no. 2
Luxenberg MN. Colour doppler imaging of superior ophthalmic vein thrombosis. Arch Ophthalmol 1991;109:582-3.  Back to cited text no. 3
Berenholz B, Kessler A, Shlomkovitz N. Superior ophthalmic vein thrombosis – complication of ethmoidal rhinosinusitis. Arch Otolaryngol Head Neck Surg 1998;1:95-7.  Back to cited text no. 4
Jaais F, Habib ZA. Unilateral superior ophthalmic vein thrombosis in a user of oral contraceptives. Med J Malaysia 1994;49:416-8.  Back to cited text no. 5
Michaelides M, Aclimandos W. Bilateral superior ophthalmic vein thrombosis in a young woman. Acta Ophthalmol Scand 2003;81:88-90.  Back to cited text no. 6
Idrees Z, Dooley I, Jackson A, Roche C, Fahy G. Simultaneous isolated bilateral superior orbital vein thrombosis as a presenting feature of antiphospholipid syndrome. Orbit 2014;33:214-6.  Back to cited text no. 7
Dey M, Charles Bates A, McMillan P. Superior ophthalmic vein thrombosis as an initial manifestation of antiphospholipid syndrome. Orbit 2013;32:42-4.  Back to cited text no. 8
Demirci FY, Kucukkaya R, Akarcay K, Kir N, Atamer T, Demirci H, et al. Ocular involvement in primary antiphospholipid syndrome. Ocular involvement in primary APS. Inter Ophthalmol 1998;22:323-9.  Back to cited text no. 9
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. 10


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