통증을 동반한 안근마비와 안구돌출로 발현한 위 눈정맥혈전증

(1)

황윤수¹·송현석²·이학승¹·박현영¹·장 혁¹ 원광의과학연구소¹, 경북대학교 의과대학 신경과학교실²

Superior Ophthalmic Vein Thrombosis Presented with Painful Ophthalmoplegia and Proptosis

Yun Su Hwang, MD¹, Hyun Seok Song, MD², Hak Seung Lee, MD¹, Hyun Young Park, MD¹, Hyuk Chang, MD¹

1Department of Neurology, Wonkwang University School of Medicine, Institute of Wonkwang Medical Science, Iksan; ²Department of Neurology, Kyungpook National University School of Medicine, Daegu, Korea

Superior ophthalmic vein (SOV) thrombosis is a rare entity, which may present with dramatic clinical signs. A 68-year-old woman presented with a 1-week history of painful and progressive right sided proptosis with accompanying blurring of vision. Magnetic resonance imaging (MRI) showed dilated right SOV in T2 weighted MRI and increased signal intensity in right SOV in T1 weighted MRI. We report an unusual case of isolated SOV thrombosis with overlapping features of idiopathic orbital inflammatory disease (IOID). We postulate that the same mechanism, with a predominantly inflammatory component, may account for SOV thrombosis in IOID.

Keywords: Superior ophthalmic vein (SOV) thrombosis; Ophthalmoplegia; Proptosis; Idiopathic orbital inflammatory disease (IOID)

INTRODUCTION

Usually common causes of superior orbital vein (SOV) thrombosis are periorbital or orbital infection including paranasal sinusitis and orbital cellulitis.¹ Other associated entities include vascular malformation, thyroid-associated orbitopathy, sarcoidosis, and Wegener granulomatosis.¹ However isolated SOV thrombosis is a rare entity, which may present with variable clinical signs including painful ophthalmoplegia. We report an unusual case of isolated SOV thrombosis with overlapping features of idiopathic orbital inflammatory disease (IOID).

CASE REPORT

A 68-year-old woman presented with a 1-week history of painful and progressive right sided proptosis with accompanying blurring of vision.

She also gave a history of intermittent retro-orbital pain and diplopia over a 2-week period. Her general health was otherwise good. She had no specific medical history, no regular medications, and no head trauma history.

Ocular examination revealed right periorbital swelling with mild ery- thema. The affected globe was non-pulsatile but proptosed by 6 mm and

Correspondence to: Hak Seung Lee, MD

Department of Neurology, Wonkwang University School of Medicine, 501 Iksan-daero, Iksan 570-711, Korea Tel: +82-63-859-1410; Fax: +82-63-842-7379; E-mail: [email protected]

Received: Aug. 27, 2013 / Accepted: Sep. 22, 2013

CASE REPORT

ISSN: 2234-0971 대한안신경의학회지: 제3권 제2호

Clin Neuroophthalmol 3(2):119-121, December 2013

(2)

Hwang YS, et al. • Superior Ophthalmic Vein Thrombosis Presented with Painful Ophthalmoplegia and Proptosis

Clin Neuroophthalmol 3(2):119-121, December 2013 120 http://neuro-ophthalmology.co.kr

also displaced inferiorly by 2 mm. Visual acuity was light perception on the right (vs 6/9 on the left) and a brisk relative afferent pupillary defect was present. Significant conjunctival chemosis with dilated episcleral vessels was noted. The cornea was clear but the anterior chamber was shallow with evidence of mild activity. The intraocular pressure was slightly elevated at 24 mm Hg. The pupil was fixed with posterior syn- aechia. Undilated fundoscopy showed generalized attenuation of the ret- inal vasculature with marked, apparently solid, elevation of the nasal and inferior retina. Ocular motility was restricted in all positions but more significantly in abduction and elevation.

An orbital computed tomography (CT) scan demonstrated a dilated right SOV combined with diffuse thickening and enhancement of right periorbital soft tissue and mild thickening of right rectus muscles (Fig. 1). Magnetic resonance imaging (MRI) showed dilated right SOV in T2 weighted MRI (Fig. 2A) and increased signal intensity in right SOV in T1 weighted MRI (Fig. 2B). Also T1 coronal image revealed right periorbital soft tissue thickening and diffuse rectus muscle enlargement. Laboratory investigations (serum angiotensin con- verting enzyme, antineutrophilic cytoplasmic antibody, rheumatoid factor, antinuclear antibody, antimicrosomal antibody, antithyro-

globulin, thyroid function tests, and inflammatory markers) for other inflammatory, granulomatosis, and vasculitic disorders potentially causing this presentation were either negative or within normal range.

Chest and abdominal X-ray were also normal.

A diagnosis of SOV thrombosis with possible IOID, by exclusion, was made. The patient was treated with high-dose steroids and anticoagu- lants. Until now, swelling around the right orbit and retro-orbital pain were improved but ophthalmoplegia and proptosis were remained. Eight weeks later, Follow-up CT showed improved status of right SOV and soft tissue thickening of right periorbital tissue (Fig. 3).

DISCUSSION

Common ophthalmic signs of SOV thrombosis include lid swelling, chemosis, conjunctival vascular tortuosity, ophthalmoplegia, and ex- ophthalmos from blockade of venous drainage from the orbit.² The pres- ence of SOV enlargement on CT and MRI scans was concerning for SOV thrombosis.

IOID is a non-infective clinical syndrome with highly variable clinical features ranging from a diffuse to very focal inflammatory process tar- geting orbital tissue.¹ Clinical features are very similar with SOV thrombosis. Proptosis, chemosis, optic neuropathy, and ophthalmoplegia are non-specific signs of orbital congestion also common to IOID. In con- trast, anterior chamber shallowing and choroidal effusion have been shown to be strongly associated with SOV thrombosis.³ SOV thrombosis has been reported as a complication of sepsis and in association with dural-cavernous sinus fisturalae.² Neither of these were present in our patient. To our Knowledge, there is little established association between IOID and SOV thrombosis. However, color doppler imaging studies in patients with active thyroid eye disease have shown significantly reduced Fig. 1. Orbit CT showed a dilated right superior orbital vein (SOV) combined

with diffuse thickening and enhancement of right periorbital soft tissue and mild thickening of right rectus muscles.

A B C

Fig. 2. (A) T2 weighted MRI showed dilated right superior orbital vein (SOV) and (B) T1 weighted MRI showed increased signal intensity in right SOV sug- gesting thrombosis. (C) T1 weighted coronal image revealed right periorbital soft tissue thickening and diffuse rectus muscle enlargement.

Fig. 3. Follow-up CT showed improved status of right SOV and soft tissue thick- ening of right periorbital tissue.

(3)

통증을 동반한 안근마비와 안구돌출로 발현한 위 눈정맥혈전증 • ^{황윤수 외}

Clin Neuroophthalmol 3(2):119-121, December 2013 http://neuro-ophthalmology.co.kr 121

blood flow within the SOV.⁴ This has been attributed to compression of the SOV or its involvement in the inflammatory process. We postulate that the same mechanism, with a predominantly inflammatory component, may account for SOV thrombosis in IOID.

The importance of recognizing SOV thrombosis in association with IOID has implications for management and prognosis. IOID is known to be steroid-responsive and, although relapses and persistent inflammation can complicate the clinical course, most patients experience com- plete resolution over several seeks.¹ In our patient, a tapering regimen of steroids was used over 8 weeks and anticoagulation continued for 3 months to prevent extension of thrombus into the cavernous sinus.

REFERENCES

1. Ahn Yuen SJ, Rubin PA. Idiopathic orbital inflammation: distribution, clinical features and treatment outcome. Arch Ophthalmol 2003;121:491- 2. Grassi MA, Lee AG, Kardon R, Nerad JA. A lot of clot. Surv Ophthalmol 499.

2003;48:555-561.

3. Stiebel-Kalish H, Setton A, Nimii Y, Kalish Y, Hartman J, Huna Bar-on R, et al. Cavernous sinus dural arteriovenous malformations: patterns of ve- nous drainage are related to clinical signs and symptoms. Ophthalmology 2002;109:1685-1691.

4. Somer D, Ozkan SB, Ozdemir H, Atilla S, Soylev MF, Duman S. Colour Doppler imaging of superior ophthalmic vein in thyroid-associated eye disease. Jpn J Ophthalmol 2002;46:341-345.