POSTERIOR SCLERITIS RESEMBLING ACUTE RETINAL NECROSIS SYNDROME Josep Badal, MD,* Carlos Lobato, MD,† Alex Fonollosa, MD,* Antonio Segura, MD,* Jose´ Garcı´a-Arumı´, MD, PhD*‡
Purpose: To report a challenging diagnosis of posterior scleritis in a patient with features of retinal necrosis. Methods: Single observational case report. Results: A 74-year-old man presented with atypical anterior scleritis in the left eye. Funduscopy showed subretinal inflammatory infiltration resembling acute retinal necrosis. The patient was treated with indomethacin, with resolution of the process. Conclusion: Typical signs of posterior scleritis include serous retinal detachment and choroidal folds in the posterior pole. Nonetheless, an atypical presentation can also occur, with peripheral whitish or subretinal inflammatory infiltrate, serous retinal detachment, and hemorrhages, resembling an acute retinal necrosis syndrome. RETINAL CASES & BRIEF REPORTS 6:102–104, 2012
noncontributory. Visual acuity was 0.8 in the right eye and 0.5 in the left eye (Snellen charts). On slit-lamp examination, anterior segment biomicroscopy of the left eye showed anterior sectorial scleritis (Figure 1A). The anterior chamber showed +1 cells. Intraocular pressure was 18 mmHg in both eyes. Funduscopy of the right eye showed no abnormalities. In the left eye, mild vitritis and peripheral serous retinal detachment were observed (Figure 1B) with a subretinal whitish inflammatory infiltration in the superotemporal aspect and adjacent retinal hemorrhages (Figure 1C) just beneath the area of scleritis seen on biomicroscopy. Based on the clinical features, a differential diagnosis was set, including posterior scleritis, acute retinal necrosis, and Bechet disease. Although the patient had an ocular history of scleritis, other diagnoses had to be ruled out. The results of aqueous humor polymerase chain reaction performed to investigate herpesvirus were negative. Antinuclear antibody, antineutrophilic cytoplasmic antibody, and rheumatoid factor analyses were negative, the pathergy test was also negative, and there were no clinical findings suggestive of Bechet disease. B-mode ultrasonography showed thickening of the sclera at the affected area (Figure 1D). The patient was started on oral indomethacin, a nonsteroidal antiinflammatory drug, at 75 mg/ day and was monitored closely with continuous follow-up visits. The response was satisfactory and inflammatory signs resolved after 1 week of treatment (Figure 2). Indomethacin was slowly tapered for 5 weeks. There were no recurrences. The diagnosis of posterior scleritis was established clinically and supported by ultrasound findings and the rapid response to nonsteroidal antiinflammatory drug therapy. The history of recurrent scleritis also provided a clue. Scleritis often accompanies systemic vasculitis, but an association with retinal hemorrhages and exudates simulating acute retinal necrosis is uncommon and poorly documented. Frost et al5 reported posterior scleritis with retinal vasculitis and choroidal and retinal infarction. The diagnosis was also established clinically and by B-mode ultrasound study.
From the *Department of Ophthalmology, Hospital Vall d’Hebron, Barcelona, Spain; †Department of Ophthalmology, Hospital de Leo´n, Leo´n, Spain; and ‡Instituto de Microcirugı´a Ocular (IMO), Barcelona, Spain.
S
cleritis is an inflammation of the sclera that can also extend to the cornea, adjacent episclera, and underlying uvea. The reported incidence of systemic disease in patients with scleritis is 39% to 50%.1 Although many systemic disorders are associated with scleral disease, rheumatoid arthritis and Wegener granulomatosis are most commonly linked to scleritis.2,3 The typical signs of posterior scleritis include serous retinal detachment and choroidal folds in the posterior pole.4 We report the case of a patient who presented with clinical features of anterior and posterior scleritis accompanied by a peripheral whitish inflammatory choroidal infiltrate with serous retinal detachment, resembling an acute retinal necrosis syndrome. Case Report A 74-year-old white man with a history of pseudophakia in his right eye and recurrent idiopathic anterior diffuse scleritis in both eyes consulted for pain and floaters of 5 days duration in his left eye. The medical history was unremarkable and the family history The authors have no conflicts of interest to disclose. Reprint requests: Josep Badal, MD, Department of Ophthalmology, Hospital Vall d’Hebron, Barcelona, Spain; e-mail: [email protected]
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POSTERIOR SCLERITIS OR RETINAL NECROSIS?
Fig. 1. A. Sectorial anterior scleritis. B. Retinal infiltration with serous retinal detachment (arrows). C. Yellow-colored subretinal infiltrates with retinal hemorrhages (arrow) and retinal edema. D. Scleral thickening on B-mode ultrasound.
Fig. 2. A. Anterior scleritis resolved after treatment. B. Funduscopy shows absence of retinal infiltrates and hemorrhages. C. Resolution of serous retinal detachment. D. Resolution of posterior scleral swelling.
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RETINAL CASES & BRIEF REPORTS´ 2012 VOLUME 6 NUMBER 1
In summary, posterior scleritis rarely presents with clinical features resembling acute retinal necrosis and requires ruling out several conditions in a challenging differential diagnosis.
Key words: posterior scleritis, retinal necrosis, uveitis. References 1. Calthorpe CM, Watson PG, McCartney ACE. Posterior scleritis: a clinical and histological survey. Eye 1988;2:267–277.
2. Wilhelmus KR, Grierson I, Watson PG. Histopathologic and clinical associations of scleritis and glaucoma. Am J Ophthalmol 1981;91:697–705. 3. Machado Dde O, Curi AL, Bessa TF, Campos WR, Ore´fice F. Scleritis: clinical characteristics, systemic associations, treatment and outcome in 100 patients. Arq Bras Oftalmol 2009;72: 231–235. 4. Okhravi N, Odufuwa B, McCluskey P, Lightman S. Scleritis. Major review. Survey Ophthalmol 2005;4:351–363. 5. Frost NA, Sparrow JM, Rosenthal AR. Posterior scleritis with retinal vasculitis and choroidal and retinal infarction. Br J Ophthalmol 1994;78:410–412.
Purpose: To report a challenging diagnosis of posterior scleritis in a patient with features of retinal necrosis. Methods: Single observational case report. Results: A 74-year-old man presented with atypical anterior scleritis in the left eye. Funduscopy showed subretinal inflammatory infiltration resembling acute retinal necrosis. The patient was treated with indomethacin, with resolution of the process. Conclusion: Typical signs of posterior scleritis include serous retinal detachment and choroidal folds in the posterior pole. Nonetheless, an atypical presentation can also occur, with peripheral whitish or subretinal inflammatory infiltrate, serous retinal detachment, and hemorrhages, resembling an acute retinal necrosis syndrome. RETINAL CASES & BRIEF REPORTS 6:102–104, 2012
noncontributory. Visual acuity was 0.8 in the right eye and 0.5 in the left eye (Snellen charts). On slit-lamp examination, anterior segment biomicroscopy of the left eye showed anterior sectorial scleritis (Figure 1A). The anterior chamber showed +1 cells. Intraocular pressure was 18 mmHg in both eyes. Funduscopy of the right eye showed no abnormalities. In the left eye, mild vitritis and peripheral serous retinal detachment were observed (Figure 1B) with a subretinal whitish inflammatory infiltration in the superotemporal aspect and adjacent retinal hemorrhages (Figure 1C) just beneath the area of scleritis seen on biomicroscopy. Based on the clinical features, a differential diagnosis was set, including posterior scleritis, acute retinal necrosis, and Bechet disease. Although the patient had an ocular history of scleritis, other diagnoses had to be ruled out. The results of aqueous humor polymerase chain reaction performed to investigate herpesvirus were negative. Antinuclear antibody, antineutrophilic cytoplasmic antibody, and rheumatoid factor analyses were negative, the pathergy test was also negative, and there were no clinical findings suggestive of Bechet disease. B-mode ultrasonography showed thickening of the sclera at the affected area (Figure 1D). The patient was started on oral indomethacin, a nonsteroidal antiinflammatory drug, at 75 mg/ day and was monitored closely with continuous follow-up visits. The response was satisfactory and inflammatory signs resolved after 1 week of treatment (Figure 2). Indomethacin was slowly tapered for 5 weeks. There were no recurrences. The diagnosis of posterior scleritis was established clinically and supported by ultrasound findings and the rapid response to nonsteroidal antiinflammatory drug therapy. The history of recurrent scleritis also provided a clue. Scleritis often accompanies systemic vasculitis, but an association with retinal hemorrhages and exudates simulating acute retinal necrosis is uncommon and poorly documented. Frost et al5 reported posterior scleritis with retinal vasculitis and choroidal and retinal infarction. The diagnosis was also established clinically and by B-mode ultrasound study.
From the *Department of Ophthalmology, Hospital Vall d’Hebron, Barcelona, Spain; †Department of Ophthalmology, Hospital de Leo´n, Leo´n, Spain; and ‡Instituto de Microcirugı´a Ocular (IMO), Barcelona, Spain.
S
cleritis is an inflammation of the sclera that can also extend to the cornea, adjacent episclera, and underlying uvea. The reported incidence of systemic disease in patients with scleritis is 39% to 50%.1 Although many systemic disorders are associated with scleral disease, rheumatoid arthritis and Wegener granulomatosis are most commonly linked to scleritis.2,3 The typical signs of posterior scleritis include serous retinal detachment and choroidal folds in the posterior pole.4 We report the case of a patient who presented with clinical features of anterior and posterior scleritis accompanied by a peripheral whitish inflammatory choroidal infiltrate with serous retinal detachment, resembling an acute retinal necrosis syndrome. Case Report A 74-year-old white man with a history of pseudophakia in his right eye and recurrent idiopathic anterior diffuse scleritis in both eyes consulted for pain and floaters of 5 days duration in his left eye. The medical history was unremarkable and the family history The authors have no conflicts of interest to disclose. Reprint requests: Josep Badal, MD, Department of Ophthalmology, Hospital Vall d’Hebron, Barcelona, Spain; e-mail: [email protected]
102
POSTERIOR SCLERITIS OR RETINAL NECROSIS?
Fig. 1. A. Sectorial anterior scleritis. B. Retinal infiltration with serous retinal detachment (arrows). C. Yellow-colored subretinal infiltrates with retinal hemorrhages (arrow) and retinal edema. D. Scleral thickening on B-mode ultrasound.
Fig. 2. A. Anterior scleritis resolved after treatment. B. Funduscopy shows absence of retinal infiltrates and hemorrhages. C. Resolution of serous retinal detachment. D. Resolution of posterior scleral swelling.
103
104
RETINAL CASES & BRIEF REPORTS´ 2012 VOLUME 6 NUMBER 1
In summary, posterior scleritis rarely presents with clinical features resembling acute retinal necrosis and requires ruling out several conditions in a challenging differential diagnosis.
Key words: posterior scleritis, retinal necrosis, uveitis. References 1. Calthorpe CM, Watson PG, McCartney ACE. Posterior scleritis: a clinical and histological survey. Eye 1988;2:267–277.
2. Wilhelmus KR, Grierson I, Watson PG. Histopathologic and clinical associations of scleritis and glaucoma. Am J Ophthalmol 1981;91:697–705. 3. Machado Dde O, Curi AL, Bessa TF, Campos WR, Ore´fice F. Scleritis: clinical characteristics, systemic associations, treatment and outcome in 100 patients. Arq Bras Oftalmol 2009;72: 231–235. 4. Okhravi N, Odufuwa B, McCluskey P, Lightman S. Scleritis. Major review. Survey Ophthalmol 2005;4:351–363. 5. Frost NA, Sparrow JM, Rosenthal AR. Posterior scleritis with retinal vasculitis and choroidal and retinal infarction. Br J Ophthalmol 1994;78:410–412.
