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Year : 2020  |  Volume : 34  |  Issue : 1  |  Page : 62-65

Smoldering multiple myeloma revealed by superior ophthalmic vein thrombosis

Department of Neurology's, Habib Bourguiba Hospital, Sfax, Tunisia

Date of Submission30-Apr-2018
Date of Decision27-Dec-2018
Date of Acceptance15-May-2019
Date of Web Publication26-Nov-2020

Correspondence Address:
Nadia Bouattour
Department of Neurology's, Habib Bourguiba Hospital, Ferdaous Street, Sfax 3029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-4534.301163

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Superior ophthalmic vein thrombosis is a rare entity. It is associated with significant morbidities. It may present with dramatic clinical signs. It is frequently secondary to cavernous sinus pathology and it can be a harbinger of cavernous sinus thrombosis. We report an unusual case of superior ophthalmic vein thrombosis, as the first manifestation of multiple myeloma. As far as we know, this is the first case described in the literature. Here we describe a patient presented with a painful, visual blur and a right-sided proptosis due to superior ophthalmic vein thrombosis. Appropriate medical workup was conducted, and smoldering multiple myeloma was diagnosed as the underlying cause. We further discuss the possible involved mechanisms.

Keywords: Hyperviscosity syndrome, multiple myeloma, ophthalmoplegia, proptosis, superior ophthalmic vein

How to cite this article:
Bouattour N, Farhat N, Hamza N, Hadjkacem H, Hdiji O, Sakka S, Dammak M, Mhiri C. Smoldering multiple myeloma revealed by superior ophthalmic vein thrombosis. Saudi J Ophthalmol 2020;34:62-5

How to cite this URL:
Bouattour N, Farhat N, Hamza N, Hadjkacem H, Hdiji O, Sakka S, Dammak M, Mhiri C. Smoldering multiple myeloma revealed by superior ophthalmic vein thrombosis. Saudi J Ophthalmol [serial online] 2020 [cited 2021 Nov 30];34:62-5. Available from: http://www.sjo.org/text.asp?2020/34/1/62/301163

  Introduction Top

Superior ophthalmic vein thrombosis (SOVT) is an uncommon orbital pathology that can present with dramatic clinical signs: a sudden onset proptosis, a conjunctival injection and a loss of visual function. SOVT can have various causes such as an orbital infection, tumors, traumatic or spontaneous carotid cavernous fistulas or vascular and coagulation anomalies.[1],[2],[3],[4] We report an unusual case of SOVT due to hyperviscosity syndrome, as the first manifestation of multiple myeloma. SOVT can be detected with contrast-enhanced computed tomography (CT) or a magnetic resonance imaging (MRI). Appropriate intervention and management strategies based on the underlying disease and the patient's clinical symptoms are required, otherwise severe complications can occur.[3]

  Case Report Top

A 70-year-old man with a history of surgery of right-sided cataract, 2 years ago, presented to the emergency department with an ongoing headache for 2 weeks with a painful and progressive right-sided proptosis, associated to a visual blur, redness, and periorbital edema. He had neither a medical history of previous venous thromboembolism nor a known malignancy or a coagulopathy.

Ocular examination revealed right periorbital swelling with mild erythema. There was no palpable thrill or bruit heard in the affected globe, but it was proptosed by 6 mm. A significant conjunctival chemosis with dilated episcleral vessels was noted. The ocular motility examination showed a total right ophthalmoplegia [Figure 1].
Figure 1: A photograph showing: (a) Right periorbital swelling with mild erythema, ecchymosis, subconjunctival hemorrhage, dilated and tortuous episcleral veins; (b and c) showed a restricted ocular motility

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The left eye examination was normal except for senile immature cataract. The Oropharynx examination was normal and especially nasal swabs and culture ruled out bacterial and fungal sinusitis. Suspecting an intraocular malignancy with orbital involvement, an urgent CT scan of the orbits was performed [Figure 2]. This showed a dilated and thrombosed superior ophthalmic vein with an associated swelling of the extraocular muscles. CT and MRI angiograms excluded a dural cavernous sinus fistula, a sino-orbital infection, but showed a partial extension of thrombus into the ipsilateral cavernous sinus [Figure 3]. Plasma protein electrophoresis showed paraprotein and increased gamma-globulins, whereas Bence-Jones proteins were not detected on urinary protein electrophoresis. Blood counts and investigations were as follows: Hemoglobin 14.5 g/dl, white blood cells 10000/μL, platelets 355000/μL, random sugar levels, renal function, and phosphocalcic balance were normal. Radiographs of the flat bones were normal. Bone marrow biopsy showed atypical plasma cells infiltration equal to 11%. On the basis of these findings, CRAB's criteria were absent and the diagnosis of smoldering (asymptomatic) IgG multiple myeloma was made.[5] As previously mentioned, smoldering multiple myeloma does not require treatment, so the patient, in this case, was not treated by chemotherapy.[5] He underwent IV heparin and bridged to the oral anticoagulant. Evolution was marked ultimately by the gradual disappearance of symptoms 1 week after starting anticoagulation therapy [Figure 4]. Close monitoring was indicated every 4 months including renal function, serum calcium, total blood count, serum protein electrophoresis with immunofixation, 24-hour urine protein electrophoresis with immunofixation and skeletal X-ray survey.
Figure 2: Computed Tomography Scan of the orbits: (a) axial section, (b) coronal reconstruction of the venous phase of computed tomography angiography: The arrow is pointing to dilated right superior ophthalmic vein thrombosis and enlarged extraocular muscles

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Figure 3: Brain MRI (T1 Gadolinium-enhanced): (a) axial section, (b and d), coronal section, (c) sagittal section: the arrow is pointing to right SOVT, (b) T1 coronal section: the arrow is pointing to the partial extension of thrombus into the right cavernous sinus

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Figure 4: External photographs at 1-week follow-up; (b and c): no limitation of ocular motility

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  Discussion Top

SOVT is an extremely rare entity resulting from orbital congestion, such as that caused by infectious diseases, cavernous sinus thrombosis, skull base tumors, arteriovenous malformations, after facial trauma and in a patient with hypercoagulability.[1],[2],[3],[6],[7] It has been reported to occur in a wide range of diseases, as summarized in [Table 1].
Table 1: Review of the literature

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Multiple myeloma is a frequent cause of the hyperviscosity syndrome.[8] Venous thromboembolism is highest during the first four months following the initial diagnosis.[9] SOVT occurred in our patient as a result of hyperviscosity because of an increased amount of circulating IgG paraprotein and cellular blood components.[10],[11],[12] Ocular manifestations can be the first signs of a patient developing multiple myeloma,[13] but SOVT wasn't reported in the literature. Multiple myeloma can be active (symptomatic) or smoldering (asymptomatic). Therapy should be started immediately for symptomatic disease, whereas asymptomatic disease requires only close monitoring.[14],[15] As in the case of our patient.

Patients with SOVT may present with an orbital pain, diplopia or a decreased vision. Clinical findings may include proptosis, chemosis, ophthalmoplegia, and ptosis. The optic nerve may be affected by compression.[16]

SOVT is usually identified by contrast-enhanced CT or MRI followed by an assessment of the patient's clinical symptoms.[17] In our patient, the brain scanner showed engorgement and thrombosis of the SOV. The normal maximal diameter of the SOV was reported to be 3.5 mm.[2] In view of the possible systemic associations of orbital inflammation, inflammatory and autoimmune workup may be indicated. A chest x-ray is indicated to evaluate for sarcoidosis.[18] The optimal treatment for SOVT depends on the etiology and clinical symptoms of the condition [Table 1]. We treated our patient with heparin followed by anticoagulant therapy. Regardless of etiology of SOVT, if there is no contraindication, anticoagulant therapy should be initiated by the clinician. According to the literature evolution under treatment was generally favorable [Table 1].

In conclusion, we described the first case report of SOVT as the first manifestation of multiple myeloma. Cases with SOVT require interdisciplinary collaboration and more detailed and extensive investigations to determine the underlying pathology and to achieve the best clinical outcome. Our case was very unusual in that, the etiology was unusual, hyperviscosity syndrome due to smoldering multiple myeloma.[27]

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Conflicts of interest

There are no conflicts of interest.

  References Top

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Akiyama K, et al. Blindness caused by septic superior ophthalmic vein thrombosis in a Lemierre Syndrome variant. Auris Nasus Larynx 2013;40(5):493-6.  Back to cited text no. 2
Chaudhry IA, et al. Carotid cavernous fistula: Ophthalmological implications. Middle East Afr J Ophthalmol 2009;16(2):57.  Back to cited text no. 3
Kubal WS. Imaging of orbital trauma. Radiographics 2008;28(6):1729-39.  Back to cited text no. 4
Kyle R, et al. Monoclonal gammopathy of undetermined significance (MGUS) and smoldering (asymptomatic) multiple myeloma: IMWG consensus perspectives risk factors for progression and guidelines for monitoring and management. Leukemia 2010;24(6):1121.  Back to cited text no. 5
Bullock JD, Goldberg SH, Connelly PJ. Orbital varix thrombosis. Ophthalmology 1990;97(2):251-6.  Back to cited text no. 6
Mishima M, et al. Superior ophthalmic vein thrombosis associated with severe facial trauma: A case report. J Med Case Rep 2015;9(1):244.  Back to cited text no. 7
Dequeker J, et al. Arteritis associated with hyper viscosity-like syndrome in rheumatoid arthritis, treated by intermittent plasmaexchange for 2.5 years. Rheumatology 1981;20(4):203-7.  Back to cited text no. 8
Cömert M, et al. Quality of life and supportive care in multiple myeloma. Turkish J Hematol 2013;30(3):234.  Back to cited text no. 9
Fahey JL, Barth WF, Solomon A. Serum hyperviscosity syndrome. JAMA 1965;192(6):120-3.  Back to cited text no. 10
Godal H, Borchgrevink C. The effect of plasmapheresis on the hemostatic function in patients with macroglobulinemia Waldenström and multiple myeloma. Scand J Clina Lab Invest 1965;17(Suppl 84):133.  Back to cited text no. 11
Kyle RA. Multiple myeloma: Review of 869 cases. Mayo. Clin Proc 1975.  Back to cited text no. 12
Ahmed YAAR, Eltayeb A. Clinical challenges: Myeloma and concomitant type 2 diabetes. Int J Hematology-oncology Stem Cell Res 2013;7(1):34.  Back to cited text no. 13
Durie BG, et al. Myeloma management guidelines: A consensus report from the scientific advisors of the international myeloma foundation. Hematol J 2003;4(6):379-98.  Back to cited text no. 14
Durie BG, et al. International uniform response criteria for multiple myeloma. Leukemia 2006;20(9):1467.  Back to cited text no. 15
Berenholz L, et al. Superior ophthalmic vein thrombosis: Complication of ethmoidal rhinosinusitis. Arch Otolaryngology-Head Neck Surg 1998;124(1):95-7.  Back to cited text no. 16
Pendharkar HS, et al. Diffusion restriction in thrombosed superior ophthalmic veins: Two cases of diverse etiology and literature review. J Radiol Case Rep 2011;5(3):8.  Back to cited text no. 17
Lim L, et al. Spontaneous superior ophthalmic vein thrombosis: A rare e. ntity with potentially devastating consequences. Eye 2014;28(3):348  Back to cited text no. 18
Schmitt NJ, Beatty RL, Kennerdell JS. Superior ophthalmic vein thrombosis in a patient with dacryocystitis-induced orbital cellulitis. Ophthalmic Plast Reconstr Surg 2005;21(5):387-9.  Back to cited text no. 19
Carrim Z, Ahmed T, Wykes W. Isolated superior ophthalmic vein thrombosis with orbital congestion: A variant of idiopathic orbital inflammatory disease? Eye 2007;21(5):665.  Back to cited text no. 20
Iseki S, et al. Proptosis caused by partially thrombosed orbital varix of the superior orbital vein associated with traumatic carotid-cavernous sinus fistula. Neurol Med Chir 2010;50(1):33-6.  Back to cited text no. 21
Shinder R, et al. Superior ophthalmic vein thrombosis in a patient with chronic myeloid leukemia receiving antifibrinolytic and thrombopoietin receptor agonist therapy. J Ocul Pharmacol Ther 2010;26(3):293-6.  Back to cited text no. 22
Wade RG, Maddock TB, Ananth S. Orbital varix thrombosis: A rare cause of unilateral proptosis. BMJ Case Reports 2013;2013, bcr2012007935.  Back to cited text no. 23
Baidoun F, et al. Acute unilateral blindness from superior ophthalmic vein thrombosis: A rare presentation of nephrotic syndrome from class IV lupus nephritis in the absence of antiphospholipid or anticardiolipin syndrome. Case Rep Hematol 2015;2015.  Back to cited text no. 24
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.  Back to cited text no. 25
Pawar B, Matthews A. Superior ophthalmic vein thrombosis associated with orbital inflammation. Adv Ophthalmol Vis Syst 2017;7(2):00216.  Back to cited text no. 26
Rao R, et al. Unilateral isolated superior ophthalmic vein thrombosis. Indian J Ophthalmol 2018;66(1):155.  Back to cited text no. 27


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

  [Table 1]


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