Combined anterior and posterior scleritis associated with central retinal vein occlusion: a case report
© The Author(s) 2012
Received: 19 December 2011
Accepted: 22 February 2012
Published: 13 March 2012
The purpose of this study is to report an uncommon presentation of anterior and posterior scleritis with central retinal vein occlusion
We report a 30-year-old female presenting with unilateral anterior and posterior scleritis with concurrent central retinal vein occlusion, the subsequent work-up, and the management. The patient presented with decreased vision and extraocular and intraocular inflammatory signs in the left eye.
At presentation, the best corrected visual acuity in the right eye (OD) was 20/20 and left eye (OS) was perception of light, with inaccurate projection of rays in all quadrants. Intraocular pressure was 12 mmHg in both eyes. OS showed mild proptosis with lid edema. Ocular movements were free and full in both eyes. The bulbar conjunctiva showed nodular anterior scleritis. OS showed mild vitreous haze with an exudative detachment at the posterior pole, disc edema with dilated, congested and tortuous veins and multiple dot blot hemorrhages, flame-shaped hemorrhages, and soft exudates throughout the posterior pole and mid-periphery An ultrasound B scan showed a large hypoechoic area in the sub-Tenon’s space (T-sign) suggestive of periocular fluid collection and thickened sclero–choroidal complex. Orbital ultrasound did not show evidence of any orbital mass or any increase in extraocular muscle thickness. Fundus fluorescein angiography showed few areas of pinpoint hyperfluorescence in the early phase with leakage in the late phase, leakage from the optic disc and vascular staining and pooling of dye in areas of exudative detachment in the late phases in the left eye. Systemic work-up was within normal limits. The patient responded well over the next month with systemic and topical steroids showing complete resolution of the scleritis and exudative retinal detachment.
Simultaneous anterior and posterior scleritis with concurrent central retinal vein occlusion is a rare entity requiring prompt diagnosis and systemic work-up for efficient management
Posterior scleritis is seen commonly in middle-aged females, associated with mild ocular congestion, globe tenderness, and varying amount of vision loss. Overall the age distribution is wide with the youngest case reported at 8 years of age and the oldest at 87 years . Posterior scleritis can present in various ways mimicking orbital tumors, orbital inflammation, optic neuritis, and vasculitis [1–3].Due to its protean manifestations, it is among the most underdiagnosed conditions in ophthalmology.
In this case, the patient presented with anterior scleritis, anterior chamber, and vitreous cells, exudative retinal detachment over the posterior pole with multiple peripapillary and retinal dot blot and flame-shaped hemorrhages. The findings were consistent with combined anterior and posterior scleritis with an associated central retinal vein occlusion. Accordingly, we looked for evidence of underlying collagen vascular disorders and systemic vasculitides. The absence of joint pains, fever, skin rashes, evidence of chronic uveitis, and a negative serology ruled out collagen vascular disorders and systemic vasculitides. A normal systemic examination, normal peripheral blood smear, and coagulation profile made leukemia unlikely.
Differentiating orbital inflammatory syndrome from posterior scleritis is difficult as both can have orbital signs and both are exquisitely sensitive to systemic steroids. Intraocular inflammation, though, is not common in orbital inflammatory syndrome . It has been hypothesized on the basis of histopathologic studies that the inflammation in posterior scleritis can contiguously spread to the retinal artery and vein and cause vasculitis with subsequent occlusion . Shukla et al. have described a case of combined central retinal artery and vein occlusion in a case of posterior scleritis . In their case, the anterior segment examination revealed no evidence of anterior scleritis and the systemic work-up was within normal limits. Similar to their case, our case too was treated with intravenous pulse methyl prednisolone followed by tapering doses of oral prednisolone with resolution of the exudative detachment and residual disc pallor with attenuated vasculature. Our case shows that involvement of a particular structure in the eye can sometimes involve an adjacent structure due to contiguous spread of pathology. This can give a mixed clinical picture with overlapping features of multiple conditions. A detailed clinical examination with pathology, physiology, and local anatomy kept in mind can help arrive at the right diagnosis and administer timely treatment. Visual improvement may however be limited depending on the extent of disease involvement.
Though vascular occlusion due to posterior scleritis is mentioned in literature [5–8] it is relatively rare and to the best of our knowledge this is the first report of combined anterior and posterior scleritis with associated central retinal vein occlusion.
None of the authors has any proprietary interest in the above case report or any conflicts of interest. No organizational funding received.
- Cleary PE, Watson PG, McGill JI, Hamilton AM (1975) Visual loss due to posterior segment disease in scleritis. Trans Ophthalmol Soc UK 95:297–299PubMedGoogle Scholar
- Watson PG, Hayreh SS (1976) Scleritis and episcleritis. Br J Ophthalmol 60:163–191PubMedPubMed CentralView ArticleGoogle Scholar
- Benson WE, Shields JA, Tasman W, Crandall AS (1979) Posterior scleritis a cause of diagnostic confusion. Arch Ophthalmol 97:1482–1486PubMedView ArticleGoogle Scholar
- Espinoza G (2010) Orbital inflammatory pseudotumors: etiology, differential diagnosis, and management. Curr Rheumatol Rep 12:443–447PubMedView ArticleGoogle Scholar
- Wilhelmus KR, Grierson I, Watson PG (1981) Histopathologic and clinical associations of scleritis and glaucoma. Am J Ophthalmol 91:697–705PubMedView ArticleGoogle Scholar
- Shukla D, Chandra Mohan K, Rao N, Kim R, Namperumalsamy P, Cunningham E (2004) Posterior scleritis causing combined central retinal artery and vein occlusion. Retina 24:467–468PubMedView ArticleGoogle Scholar
- Sahu DK, Rawoof AB (2000) Cilioretinal artery occlusion in posterior scleritis. Retina 20:303–305PubMedView ArticleGoogle Scholar
- Frost AN, Sparrow JM, Rosenthal AR (1974) Posterior scleritis with retinal vasculitis and choroidal and retinal infarction. Br J Ophthalmol 78:410–412View ArticleGoogle Scholar
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