Orbit, 2014; 33(3): 214–216 ! Informa Healthcare USA, Inc. ISSN: 0167-6830 print / 1744-5108 online DOI: 10.3109/01676830.2014.881398
C ASE REPORT
Simultaneous Isolated Bilateral Superior Orbital Vein Thrombosis as a Presenting Feature of Antiphospholipid Syndrome Zubair Idrees, Ian Dooley, Aoife Jackson, Claire Roche, and Gerry Fahy Department of Ophthalmology, University College Hospital Galway, Ophthalmology, Galway, Galway, Ireland
ABSTRACT Purpose: To report a rare case of isolated bilateral simulataneous superior ophthalmic vein occlusions secondary to antiphospholipid syndrome. Case Report: We report the case of a 77-year-old female who presented with a one week history of right relative proptosis, conjunctival injection and optic disc swelling. Magnetic resonance imaging revealed bilateral orbital masses which were diagnosed as superior ophthalmic vein occlusion. The patient was anticoagulated and the lesions resolved completely. System cardiovascular and haematological work-up revealed the patient was suffering from anti-phospholipid syndrome. Conclusion: Although superior ophthalmic vein occlusion is rare, it is an important differential as orbital biopsy is to be avoided. This is only the second reported case secondary to antiphospholipid syndrome and the first such case with bilateral occlusions. Keywords: Anti-phospholipid syndrome, lupus antibody, lupus anticoagulant, orbital mass, superior ophthalmic vein occlusion
CASE REPORT
of a compressive orbital mass was made. A computed tomography (CT) scan (Figure 1) showed bilateral intraconal, non-specific superior intraorbital masses. The right-sided mass was almost spherical, while the left-sided mass was more fusiform and neither were distinguishable from the superior recti. Some areas of calcification were visible within the right-sided orbital mass. The rest of the scan was unremarkable. Following discussion with the Radiology Department, a magnetic resonance scan (MR) of orbits (Figure 2) was performed. This revealed hetereogenous orbital masses, which lay posterior to globe and adjacent to the superior recti, with no evidence of intracranial extension. The findings were consistent with a diagnosis of bilateral superior ophthalmic venous thrombosis (SOVT). Futher investigations for underlying associated comorbid disorders, revealed the patient had elevated
We report a case of a 77-year-old lady who presented to our Emergency Eye Clinic via her Primary Care Physician, complaining of blurred vision and right proptosis of one week’s duration. Her best-corrected visual acuity (BCVA) was 6/9 in both eyes and her colour vision was normal. She had a 3-mm relative right proptosis, with associated dilated right episcleral vessels and mild right optic disc swelling. The patient still retained full range of ocular movements, had no relative afferent pupillary defect, and the retinal vasculature was normal. Upon further questioning, the patient reported intermittent frontal headache for the preceding one month. She was otherwise well, and had a history of well-controlled hypertension and osteoarthritis. Given the proptosis, optic disc swelling and episcleral vessel dilation a provisional diagnosis of
Received 10 June 2013; Accepted 6 January 2014; Published online 24 February 2014 Correspondence: Gerry Fahy, Galway University Hospital, Galway, Ireland. Tel: +353 91 544733; E-mail: [email protected]
214
Unique Imaging and Management Considerations
215
DISCUSSION
FIGURE 1. An axial-view computed tomograph through the patient’s superior orbit. The scan was taken at presentation and shows a large spherical lesion in the right orbit and an elongated lesion in the left orbit. The lesions were not distinguishable from the superior recti. The right mass demonstrates some areas of increased attenuation, which represents calcification.
FIGURE 2. A coronal-view of a T1 weighted magnetic resonance scan image, through the posterior orbits, showing heterogenous bilateral intraorbital masses. These masses represent the thrombosed superior ophthalmic veins. The globes are not visible in the plan of this scan.
lupus inhibitor (antiphospholipid antibody), which remained positive on serial testing, indicating a diagnosis of Antiphospholipid Syndrome (APS). Warfarin anticoagulation therapy was commenced, with the role of reducing the risk of further thrombotic events. Her symptoms and signs resolved over 4 weeks. Repeat MR scan showed reduction in size of the intraconal masses. !
2014 Informa Healthcare USA, Inc.
Bilateral spontaneous isolated SOVT is very rare and there are few reports in literature. One case of SOVT related to APS has been reported; however, it was unilateral and in a much younger (41 years old) male.1 It is interesting to note that CT angiogram was the diagnostic modality in this report. Signs and symptoms resolved with systemic steroids in this case and again anticoagulation was initiated. Unilateral SOVT has been widely reported, and has been described in association with orbital cellulitis2 and ethmoidal rhinosinusitis.3 Bilateral SOVT has been reported in association thrombosis of the cavernous sinus and carotico-cavernous fistula.4,5 Bilateral isolated SOVT is very rare, although has previously been reported in association with oral contraceptive use.6 This represents the first report of bilateral isolated SOVT related to APS. Our patient, however, had no clinical or radiological manifestation to support a carotid or dural cavernous fistula. Lymphoma and orbital metastases are the most common causes of bilateral orbital masses in an elderly patient. Orbital biopsy is essential to establish the diagnosis in these situations. However the management of isolated superior ophthalmic vein thrombosis is observation. A CT scan of the orbits in our patient was not diagnostic; however, an MR scan of orbits revealed appearances compatible with bilateral SOVT. APS is an acquired autoimmune disorder, characterised by recurrent arterial and/or venous thrombosis, and associated with persistently positive lupus anticoagulant and/or anticardiolipin antibodies.7–9 The ocular manifestations and associations of APS are diverse, but retinal vascular occlusions are the most commonly encountered.7–9 Many ophthalmologists and radiologists would not routinely consider SOVT in the differential diagnosis of a bilateral superior orbital mass. Given the spontaneous resolution, it is possible that this condition is more common but underdiagnosed.
CONCLUSION Isolated bilateral SOVT in elderly patients is very rare and not commonly considered in the context of an orbital mass. Lymphoma and orbital metastases are the most common causes of bilateral orbital masses in elderly patients. Unlike most orbital masses in this age group, management of SOVT is by observation. Orbital CT scan is useful but not necessarily diagnostic, critical evaluation of the MR scan may establish the diagnosis. Orbital biopsy is considered the diagnostic investigation of choice for many orbital masses, but in this case MR scanning was diagnostic and
216 Z. Idrees et al. saved the patient exposure to an unnecessary surgical procedure.
4.
DECLARATION OF INTEREST 5.
The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
6.
REFERENCES 7. 1. Dey M, Charles Bates A, McMillan P. Superior ophthalmic vein thrombosis as an initial manifestation of antiphospholipid syndrome. Orbit 2013;32(1):42–44. 2. Flaharty PM, Phillips W, Sergott RC, et al. Color Doppler imaging of superior ophthalmic vein thrombosis. Arch Ophthalmol 1991;109(4):582–583. 3. Berenholz L, Kessler A, Shlomkovitz N, et al. Superior ophthalmic vein thrombosis: complication of ethmoidal
8.
9.
rhinosinusitis. Arch Otolaryngol Head Neck Surgery 1998; 124(1):95–97. Pendharkar HS, Gupta AK, Bodhey N, Nair M. Diffusion restriction in thrombosed superior ophthalmic veins: two cases of diverse etiology and literature review. J Radiol Case Rep 2011;5(3):8–16. Schuknecht B, Simmen D, Yuksel C, Valavanis A. Tributary venosinus occlusion and septic cavernous sinus thrombosis: CT and MR findings. AJNR. Amer J Neuroradiol Apr 1998;19(4):617–626. Michaelides M, Aclimandos W. Bilateral superior ophthalmic vein thrombosis in a young woman. Acta Ophthalmol Scand 81(1):88–90. Matthews BN, Mirza SA, Durrani OM. A blinding combination. Lancet 2002;360(9341):1220. Demirci FY, Kucukkaya R, Akarcay K, et al. Ocular involvement in primary antiphospholipid syndrome. Ocular involvement in primary APS. Inter Ophthalmol 1998;22(6):323–329. Suvajac G, Stojanovich L, Milenkovich S. Ocular manifestations in antiphospholipid syndrome. Autoimmun Rev 2007;6(6):409–414.
Orbit
Copyright of Orbit is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
C ASE REPORT
Simultaneous Isolated Bilateral Superior Orbital Vein Thrombosis as a Presenting Feature of Antiphospholipid Syndrome Zubair Idrees, Ian Dooley, Aoife Jackson, Claire Roche, and Gerry Fahy Department of Ophthalmology, University College Hospital Galway, Ophthalmology, Galway, Galway, Ireland
ABSTRACT Purpose: To report a rare case of isolated bilateral simulataneous superior ophthalmic vein occlusions secondary to antiphospholipid syndrome. Case Report: We report the case of a 77-year-old female who presented with a one week history of right relative proptosis, conjunctival injection and optic disc swelling. Magnetic resonance imaging revealed bilateral orbital masses which were diagnosed as superior ophthalmic vein occlusion. The patient was anticoagulated and the lesions resolved completely. System cardiovascular and haematological work-up revealed the patient was suffering from anti-phospholipid syndrome. Conclusion: Although superior ophthalmic vein occlusion is rare, it is an important differential as orbital biopsy is to be avoided. This is only the second reported case secondary to antiphospholipid syndrome and the first such case with bilateral occlusions. Keywords: Anti-phospholipid syndrome, lupus antibody, lupus anticoagulant, orbital mass, superior ophthalmic vein occlusion
CASE REPORT
of a compressive orbital mass was made. A computed tomography (CT) scan (Figure 1) showed bilateral intraconal, non-specific superior intraorbital masses. The right-sided mass was almost spherical, while the left-sided mass was more fusiform and neither were distinguishable from the superior recti. Some areas of calcification were visible within the right-sided orbital mass. The rest of the scan was unremarkable. Following discussion with the Radiology Department, a magnetic resonance scan (MR) of orbits (Figure 2) was performed. This revealed hetereogenous orbital masses, which lay posterior to globe and adjacent to the superior recti, with no evidence of intracranial extension. The findings were consistent with a diagnosis of bilateral superior ophthalmic venous thrombosis (SOVT). Futher investigations for underlying associated comorbid disorders, revealed the patient had elevated
We report a case of a 77-year-old lady who presented to our Emergency Eye Clinic via her Primary Care Physician, complaining of blurred vision and right proptosis of one week’s duration. Her best-corrected visual acuity (BCVA) was 6/9 in both eyes and her colour vision was normal. She had a 3-mm relative right proptosis, with associated dilated right episcleral vessels and mild right optic disc swelling. The patient still retained full range of ocular movements, had no relative afferent pupillary defect, and the retinal vasculature was normal. Upon further questioning, the patient reported intermittent frontal headache for the preceding one month. She was otherwise well, and had a history of well-controlled hypertension and osteoarthritis. Given the proptosis, optic disc swelling and episcleral vessel dilation a provisional diagnosis of
Received 10 June 2013; Accepted 6 January 2014; Published online 24 February 2014 Correspondence: Gerry Fahy, Galway University Hospital, Galway, Ireland. Tel: +353 91 544733; E-mail: [email protected]
214
Unique Imaging and Management Considerations
215
DISCUSSION
FIGURE 1. An axial-view computed tomograph through the patient’s superior orbit. The scan was taken at presentation and shows a large spherical lesion in the right orbit and an elongated lesion in the left orbit. The lesions were not distinguishable from the superior recti. The right mass demonstrates some areas of increased attenuation, which represents calcification.
FIGURE 2. A coronal-view of a T1 weighted magnetic resonance scan image, through the posterior orbits, showing heterogenous bilateral intraorbital masses. These masses represent the thrombosed superior ophthalmic veins. The globes are not visible in the plan of this scan.
lupus inhibitor (antiphospholipid antibody), which remained positive on serial testing, indicating a diagnosis of Antiphospholipid Syndrome (APS). Warfarin anticoagulation therapy was commenced, with the role of reducing the risk of further thrombotic events. Her symptoms and signs resolved over 4 weeks. Repeat MR scan showed reduction in size of the intraconal masses. !
2014 Informa Healthcare USA, Inc.
Bilateral spontaneous isolated SOVT is very rare and there are few reports in literature. One case of SOVT related to APS has been reported; however, it was unilateral and in a much younger (41 years old) male.1 It is interesting to note that CT angiogram was the diagnostic modality in this report. Signs and symptoms resolved with systemic steroids in this case and again anticoagulation was initiated. Unilateral SOVT has been widely reported, and has been described in association with orbital cellulitis2 and ethmoidal rhinosinusitis.3 Bilateral SOVT has been reported in association thrombosis of the cavernous sinus and carotico-cavernous fistula.4,5 Bilateral isolated SOVT is very rare, although has previously been reported in association with oral contraceptive use.6 This represents the first report of bilateral isolated SOVT related to APS. Our patient, however, had no clinical or radiological manifestation to support a carotid or dural cavernous fistula. Lymphoma and orbital metastases are the most common causes of bilateral orbital masses in an elderly patient. Orbital biopsy is essential to establish the diagnosis in these situations. However the management of isolated superior ophthalmic vein thrombosis is observation. A CT scan of the orbits in our patient was not diagnostic; however, an MR scan of orbits revealed appearances compatible with bilateral SOVT. APS is an acquired autoimmune disorder, characterised by recurrent arterial and/or venous thrombosis, and associated with persistently positive lupus anticoagulant and/or anticardiolipin antibodies.7–9 The ocular manifestations and associations of APS are diverse, but retinal vascular occlusions are the most commonly encountered.7–9 Many ophthalmologists and radiologists would not routinely consider SOVT in the differential diagnosis of a bilateral superior orbital mass. Given the spontaneous resolution, it is possible that this condition is more common but underdiagnosed.
CONCLUSION Isolated bilateral SOVT in elderly patients is very rare and not commonly considered in the context of an orbital mass. Lymphoma and orbital metastases are the most common causes of bilateral orbital masses in elderly patients. Unlike most orbital masses in this age group, management of SOVT is by observation. Orbital CT scan is useful but not necessarily diagnostic, critical evaluation of the MR scan may establish the diagnosis. Orbital biopsy is considered the diagnostic investigation of choice for many orbital masses, but in this case MR scanning was diagnostic and
216 Z. Idrees et al. saved the patient exposure to an unnecessary surgical procedure.
4.
DECLARATION OF INTEREST 5.
The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
6.
REFERENCES 7. 1. Dey M, Charles Bates A, McMillan P. Superior ophthalmic vein thrombosis as an initial manifestation of antiphospholipid syndrome. Orbit 2013;32(1):42–44. 2. Flaharty PM, Phillips W, Sergott RC, et al. Color Doppler imaging of superior ophthalmic vein thrombosis. Arch Ophthalmol 1991;109(4):582–583. 3. Berenholz L, Kessler A, Shlomkovitz N, et al. Superior ophthalmic vein thrombosis: complication of ethmoidal
8.
9.
rhinosinusitis. Arch Otolaryngol Head Neck Surgery 1998; 124(1):95–97. Pendharkar HS, Gupta AK, Bodhey N, Nair M. Diffusion restriction in thrombosed superior ophthalmic veins: two cases of diverse etiology and literature review. J Radiol Case Rep 2011;5(3):8–16. Schuknecht B, Simmen D, Yuksel C, Valavanis A. Tributary venosinus occlusion and septic cavernous sinus thrombosis: CT and MR findings. AJNR. Amer J Neuroradiol Apr 1998;19(4):617–626. Michaelides M, Aclimandos W. Bilateral superior ophthalmic vein thrombosis in a young woman. Acta Ophthalmol Scand 81(1):88–90. Matthews BN, Mirza SA, Durrani OM. A blinding combination. Lancet 2002;360(9341):1220. Demirci FY, Kucukkaya R, Akarcay K, et al. Ocular involvement in primary antiphospholipid syndrome. Ocular involvement in primary APS. Inter Ophthalmol 1998;22(6):323–329. Suvajac G, Stojanovich L, Milenkovich S. Ocular manifestations in antiphospholipid syndrome. Autoimmun Rev 2007;6(6):409–414.
Orbit
Copyright of Orbit is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
