Sir,
Posterior scleritis is a potentially blinding but frequently underdiagnosed condition. Serous retinal detachment (SRD) is a common finding,1 whereas vascular occlusions are rare.2, 3, 4 We report simultaneous SRD and retinal arterial occlusion as a presentation of posterior scleritis, successfully treated with systemic corticosteroids.
Case report
A 26-year-old lady presented with painful diminution of vision OD for a week. She had a similarly painful visual loss OS 2 years back, which was permanent in spite of some unspecified treatment taken elsewhere. There was no history of any systemic illness affecting skin, joints, or organ systems accompanying or following the visual loss in the run-up to the current episode. Her best-corrected visual acuity (BCVA) was 2/60 OD and 6/36 OS. Intraocular pressures were normal, and anterior segment was unremarkable OU, except a relative afferent pupillary defect OD. Fundus examination OD revealed optic disc oedema with SRD at the posterior pole, retinal whitening superotemporally (Figure 1a), and peripheral choroidal infiltrates (not shown). Fundus OS revealed extensive chorioretinal scarring over macula (Figure 1b). Neither eye revealed vitreous cells or flare.
Fluorescein angiography OD showed delayed arteriovenous transit in the superotemporal branch retinal artery (Figure 1c). Late phases showed dye leakage from the disc and pooling in detachment spaces. B-scan ultrasonogram revealed grossly thickened sclera with sub-Tenon's fluid (Figure 1d). After obtaining a detailed history related to previous illness, current symptoms, or drug intake, a complete systemic evaluation including mucocutaneous, musculoskeletal, respiratory, cardiovascular, gastrointestinal, and genitourinary systems was performed by the in-house physician. As the patient had an uneventful history for the preceding 2 years and an unremarkable review of systems, general screening investigations were ordered, including erythrocyte sedimentation rate, a complete haemogram (including total and differential leukocyte counts), tuberculin skin test, chest X-ray, urinalysis, and serological tests such as Treponema pallidum haemagglutination antigen, rheumatoid factor, C-reactive protein, and antinuclear antibody. The investigations were not suggestive of any systemic infectious, rheumatic, or vasculitic disease. In the absence of anterior scleritis, episcleritis, and keratitis, systemic infections were a remote possibility in our patient.5 The most common infections—herpes zoster, syphilis, and tuberculosis5—were further ruled out by the absence of typical mucocutaneous findings and negative investigations, as mentioned above. Neuroretinitis—most commonly caused by Bartonella infection—was also a differential diagnosis because of the presence of disc oedema, SRD, and subretinal infiltrates.6 Absence of features such as a history of exposure to cats, systemic flu-like illness, a benign course, granulomatous or ulcerative conjunctivitis, and regional lymphadenopathy ruled against cat-scratch disease.6
After carefully ruling out all the infectious aetiologies, the patient was treated with intravenous methyl prednisolone (15 mg/kg/day) for 3 days, followed by oral corticosteroids (1 mg/kg/day). After 2 weeks, BCVA improved to 6/60 with decreased scleral thickening. At 16 weeks, BCVA was 6/9 OD, with a brisk pupillary reaction. There was complete clinical and sonographic resolution of scleritis (Figure 1e and f), with no residual field defect.
Comment
Posterior scleral inflammation can extend into the optic nerve and retinal vessels, resulting in vascular occlusions:7 cilioretinal artery occlusion, retinochoroidal infarction, and combined retinal vascular occlusion have been reported.2, 3, 4 We are unaware of any report describing simultaneous retinal arterial occlusion with SRD in posterior scleritis. Ocular pain, SRD, disc oedema, and choroidal infiltrates were important clues; ultrasonography clinched the diagnosis. Although macular lesions and visual loss mandate aggressive therapy, a third of the patients suffer further loss of vision.1 We obtained a rapid anatomic and functional resolution of scleritis and its sequelae with early diagnosis and aggressive treatment. This was of critical importance in a patient who had already suffered visual loss probably due to the same cause in the fellow eye, as indicated by similar antecedent symptoms and chorioretinal sequelae. Indeed, nearly half of the patients with scleritis develop bilateral disease, 50% of whom have delayed onset in the fellow eye, mostly of the same type of scleritis.8 A high index of suspicion may uncover this sight-threatening but treatable condition.
References
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Shukla, D., Agrawal, D., Dhawan, A. et al. Posterior scleritis presenting with simultaneous branch retinal artery occlusion and exudative retinal detachment. Eye 23, 1475–1477 (2009). https://doi.org/10.1038/eye.2008.217
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DOI: https://doi.org/10.1038/eye.2008.217
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