|Year : 2020 | Volume
| Issue : 1 | Page : 39-42
Posterior scleritis-induced optic neuropathy and exudative retinal detachment – A challenging diagnostic dilemma
Pritam Bawankar1, Dipankar Das2, Harsha Bhattacharjee1, Ronel Soibam1
1 Department of Vitreo-Retina Surgery, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
2 Department of Ocular Pathology, Uveitis and Neuro-Ophthalmology Services, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
|Date of Submission||05-Mar-2019|
|Date of Decision||10-Oct-2019|
|Date of Acceptance||22-Oct-2019|
|Date of Web Publication||6-Mar-2020|
Department of Vitreo-Retina Surgery, Sri Sankaradeva Nethralaya, Beltola, Guwahati - 781 028, Assam
Source of Support: None, Conflict of Interest: None
We report three cases of posterior scleritis (PS) to analyze the clinical profile and ultrasonographic and fluorescein angiography features of this rare disorder. Fundus findings included serous retinal detachment (RD), disc edema, disc hyperemia, corkscrewed retinal vessel, and retinal folds. Ultrasonography revealed a variable degree of thickening of the posterior eye wall (choroid and sclera). Fluorescein angiography revealed persistent dye leakage from the disc and early pinpoint areas of hyperfluorescence with pooling of dye in late frames of an angiogram. Optical coherence tomography showed serous macular detachment in all cases at the time of presentation. The purpose of this manuscript was to describe three cases of PS associated with optic neuropathy and exudative RD previously misdiagnosed with a range of conditions. This case study also demonstrates the importance of B-scan ultrasonography and fluorescein angiography for the appropriate diagnosis of PS and also the effectiveness of systemic corticosteroid therapy.
Keywords: B-scan ultrasonography, exudative retinal detachment, fundus fluorescein angiography, optic neuropathy, posterior scleritis
|How to cite this article:|
Bawankar P, Das D, Bhattacharjee H, Soibam R. Posterior scleritis-induced optic neuropathy and exudative retinal detachment – A challenging diagnostic dilemma. J Clin Ophthalmol Res 2020;8:39-42
|How to cite this URL:|
Bawankar P, Das D, Bhattacharjee H, Soibam R. Posterior scleritis-induced optic neuropathy and exudative retinal detachment – A challenging diagnostic dilemma. J Clin Ophthalmol Res [serial online] 2020 [cited 2020 Dec 3];8:39-42. Available from: https://www.jcor.in/text.asp?2020/8/1/39/280207
Posterior scleritis (PS) is an uncommon entity, which refers to an inflammation of the sclera posterior to the ora serrata that is severe enough to cause abnormalities in the posterior segment (choroid, retina, and optic nerve) and the anterior segment of the eye., The classic symptoms associated with PS are the periocular pain, pain on movement, decreased vision, and redness of the eye, but it must be stressed that many patients have none or only one of these. PS has been reported to account for only 2%–12% of all cases of scleritis and in up to 50% of cases, it can be bilateral. However, its actual incidence is probably underrecognized due to its clinical features (papillitis, disc edema, retinal folds, choroidal folds, serous retinal detachment [RD], etc.) that may resemble other intra- or extraocular inflammatory and neoplastic conditions. Middle-aged women are more frequently affected than men. Ultrasonography is the key investigation necessary to make a diagnosis of PS, and computed tomography (CT) scan, magnetic resonance imaging (MRI), and fundus fluorescein angiography (FFA) can also be used as ancillary tests.
Herein, we describe three cases of PS associated with optic neuropathy and exudative RD previously misdiagnosed with a range of conditions and the role of ultrasound and FFA findings in the diagnosis and also demonstrate the effectiveness of high-dose methylprednisolone therapy.
| Case Report|| |
We describe three cases which were diagnosed as having PS in our hospital, which is a referral eye center. The information regarding age, sex, laterality, the preliminary diagnosis of the referring ophthalmologist, pre- and posttreatment best-corrected visual acuity, and the treatment advised were noted [Table 1]. Status of retinal vessels, optic nerve head, secondary RD, retinal or choroidal folds, etc., was revealed on fundus examination [Table 2]. B-scan ultrasonography (BSU), optical coherence tomography (OCT), and FFA were done in all cases [Table 2]. The presenting complaint among these patients was a variable decrease of vision associated with mild-to-moderate ocular pain over a period of 1–2 weeks. All the cases were unilateral. Case 3 had a history of fever, cough, and body ache at the time of presentation. Case 1 had lid edema, chemosis, anterior scleritis, and anterior uveitis at the time of presentation. External examination and slit-lamp findings were unremarkable in cases 2 and 3.
|Table 1: Clinical profile, referral diagnosis, and the treatment characteristics of three cases of posterior scleritis|
Click here to view
Ultrasonography was done in all cases which revealed a variable degree of thickening of the posterior eye wall (choroid and sclera). Characteristic “T-” sign due to edema in the Tenon's space was seen in case 2 and a shallow serous RD was seen in case 1. Repeat ultrasonography following clinical improvement showed the total reduction in choroidal and scleral thickening with complete resolution of serous RD. FFA revealed persistent dye leakage from the disc and early pinpoint areas of hyperfluorescence with pooling of dye in late frames of an angiogram. OCT showed serous macular detachment in all cases at the time of presentation. Fundus imaging, ultrasonography, FFA, and OCT findings of cases 1, 2, and 3 are depicted in [Figure 1], [Figure 2], [Figure 3], [Figure 4], respectively. Routine hemogram, erythrocyte sedimentation rate, blood sugar, serum acetylcholinesterase, rheumatoid factor, antinuclear antibodies, and antineutrophil cytoplasmic antibodies were all normal. Chest X-ray and Mantoux test were also within normal limits. A diagnosis of PS was made in all cases, and the patients were commenced on intravenous methylprednisolone (500 mg/day for 3 consecutive days). All patients were started on oral prednisone (1 mg/kg body weight) for the 4th day, and this was weaned off by 5 mg/week. Clinical resolution in all cases occurred with the use of systemic steroids.
|Figure 1: Case 1 – (a) Color fundus image showing hyperemic edematous optic disc, exudative retinal detachment at the posterior pole, retinal folds, and corkscrewed retinal vessels. (b-d) Early, mid, and late phases of the angiogram showing early hyperfluorescence with late pooling of dye in the subretinal space and persistent dye leakage from the disc|
Click here to view
|Figure 2: Case 1 – (a) Color fundus image showing resolution of disc edema, macular detachment, and retinal folds after 2 months of treatment. (b) Pretreatment optical coherence tomography image showing subretinal fluid at the macula. (c) Posttreatment optical coherence tomography image showing resolution of exudation and flatting at the macula. (d and e) B-scan ultrasonography showing choroidal and scleral thickening with exudative detachment at the macula|
Click here to view
|Figure 3: Case 2 – (a) Fundus image showing exudative macular detachment which appears like central serous chorioretinopathy. (b) Optical coherence tomography image showing focal detachment at the macula. (c) B-scan ultrasonography showing classic “T-” sign and marked thickening of the posterior eye wall. (d) Late phase of angiogram showing persistent dye leakage from the disc and in the subretinal space at the macula|
Click here to view
|Figure 4: Case 3 – (a) Color fundus image showing hyperemia with disc edema, subretinal fluid accumulation at the macula. (b) Optical coherence tomography image showing subretinal fluid at the macula. (c) B-scan ultrasonography showing gross choroidal and scleral thickening. Fluorescein angiography showing (d) early pinpoint hyperfluorescence in the early phase and (e) areas of late pooling of dye and diffuse disc leakage in the late phase of angiogram|
Click here to view
| Discussion|| |
PS is often an underrecognized form of inflammation most likely due to its location and associated nonspecific signs and symptoms. A high index of suspicion is usually required for the diagnosis, and it is usually based on a compatible clinical spectrum in addition to indirect signs observed on BSU and other ancillary tests such as FFA, CT, and MRI. Ultrasonography is the most helpful ancillary test in the diagnosis of PS. However, it is unable to detect some early forms of this disease, and a characteristic T-sign may be absent in up to 75% of PS cases. In this sense, PS is often misdiagnosed by the referring ophthalmologist who may refer these patients to other physicians rather than ophthalmologists, particularly if ultrasonography findings are nonspecific or unremarkable.
PS is often a complex and challenging diagnostic dilemma, and it is also important to avoid oversimplifying the diagnosis without adequately considering other possibilities. As PS patients may present with proptosis, lid swelling, limitation of ocular movements, intense periscleral inflammation, disc edema, and choroidal folds, one should consider orbital tumor, inflammatory pseudotumor, or thyroid ophthalmopathy in the differential diagnosis. In the case of subretinal mass findings, choroidal melanoma, metastatic carcinoma to the choroid, or choroidal hemangioma should be excluded. In cases with a serous detachment of the retina, conditions such as uveal effusion syndrome, Vogt–Koyanagi–Harada disease, or central serous retinopathy should be kept in mind.
In case 1, moderate-to-severe pain, pain on eye movement, conjunctival chemosis, proptosis, and disc edema indicated a diagnosis of the pseudotumor. However, associated anterior scleritis and anterior uveitis indicated a suspicion of PS. Pseudotumor can closely mimic an acute PS, particularly when associated with anterior scleritis. Both conditions can cause retino-choroidal striae, scleral thickening, retrobulbar edema, and extraocular muscle enlargement. However, in PS, intraocular findings such as anterior uveitis and retinal and optic nerve involvement are more prominent than extraocular findings. The diagnosis of pseudotumor often depends on mostly demonstration of an orbital mass by ultrasonography or CT scan; therefore, PS was the distinct possibility in this case.
In case 2, a diagnosis of central serous chorioretinopathy (CSR) was made based on the serous RD involving the macular area. CSR does not cause pain and has clear subretinal fluid. FFA and ultrasonography can certainly differentiate PS from CSR. CSR nearly always has only a single leak on FFA and on ultrasonography shows only a serous RD, not the choroidal and scleral thickening, disc edema, retrobulbar edema, and “T-” sign that are seen in PS.
PS may be associated with optic disc swelling in up to 17% of patients. In case 3, mild-to-moderate pain associated with defective vision, disc edema, exudative macular detachment, and characteristic findings on USG and FFA supported the diagnosis of PS.
The management of PS is less challenging in contrast to the diagnosis. It is a treatable disease, as shown by the prompt remission of signs and symptoms in all patients we treated with systemic corticosteroids. To conclude, a high index of suspicion should be kept in any disc edema associated with exudative RD involving the posterior pole if associated with pain. In such a case, a BSU and an FFA can be done to establish the diagnosis. Ophthalmologists must remain aware of the protean manifestations of this unique entity, and early diagnosis of PS is most important due to its excellent response to anti-inflammatory medications, particularly with systemic steroids.
The authors would like to acknowledge Sri Kanchi Sankara Health and Educational Foundation, Guwahati, Assam, India.
Financial support and sponsorship
This study was financially supported by Sri Kanchi Sankara Health and Educational Foundation.
Conflicts of interest
There are no conflicts of interest.
| References|| |
Watson P, Hazleman BL, Pavésio C, Green WR. Sclera and Systemic Disorders. 2nd
ed. Oxford: Butterworth Heinemann; 2004.
Sadiq Z, Shepherd R, Vardy S. Posterior scleritis – An unusual presentation of facial pain. Br J Oral Maxillofac Surg 2003;41:62-3.
McGavin DD, Williamson J, Forrester JV, Foulds WS, Buchanan WW, Dick WC, et al.
Episcleritis and scleritis. A study of their clinical manifestations and association with rheumatoid arthritis. Br J Ophthalmol 1976;60:192-226.
McCluskey PJ, Watson PG, Lightman S, Haybittle J, Restori M, Branley M. Posterior scleritis: Clinical features, systemic associations, and outcome in a large series of patients. Ophthalmology 1999;106:2380-6.
Maggioni F, Ruffatti S, Viaro F, Mainardi F, Lisotto C, Zanchin G. A case of posterior scleritis: Differential diagnosis of ocular pain. J Headache Pain 2007;8:123-6.
Cordero-Coma M, García-Morán A, Yilmaz T, Sánchez-Campos S, Calleja-Antolín S, Martín-Escuer B, et al.
Adjunctive globe magnetic resonance imaging in the diagnosis of posterior scleritis. Can J Ophthalmol 2011;46:329-32.
Benson WE, Shields JA, Tasman W, Crandall AS. Posterior scleritis. A cause of diagnostic confusion. Arch Ophthalmol 1979;97:1482-6.
Akpek EK. Necrotizing Scleritis: Diagnosis and Therapy. Ocular Immunology and Uveitis Foundation. Available from: http://www.uveitis.org/
. [Last visited on 2013 Apr 24].
Biswas J, Mittal S, Ganesh SK, Shetty NS, Gopal L. Posterior scleritis: Clinical profile and imaging characteristics. Indian J Ophthalmol 1998;46:195-202.
] [Full text]
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
[Table 1], [Table 2]