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  Table of Contents 
CASE REPORT
Year : 2020  |  Volume : 27  |  Issue : 2  |  Page : 134-138  

Multimodal imaging of nodular posterior scleritis: Case report and review of the literature


1 Department of Ophthalmology, College of Medicine, King Saud University, Riyadh, Saudi Arabia
2 Department of Ophthalmology; Dr. Nasser Al Rashid Research Chair in Ophthalmology, College of Medicine, King Saud University, Riyadh, Saudi Arabia

Date of Submission02-Apr-2020
Date of Acceptance21-Jun-2020
Date of Web Publication20-Jul-2020

Correspondence Address:
Prof. Ahmed M Abu El-Asrar
Department of Ophthalmology, King Abdulaziz University Hospital, Old Airport Road, Malaz, P.O. Box 245, Riyadh 11411
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/meajo.MEAJO_115_20

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   Abstract 


Nodular posterior scleritis represents a small percentage of all cases of posterior scleritis. Because of the scarcity of nodular posterior scleritis, it may be confused or even misdiagnosed as an intraocular tumor or posterior uveitis. Here, we are reporting a case of nodular posterior scleritis in a 25-year-old medically free male. Furthermore, we reviewed previously reported cases of nodular posterior scleritis. Our patient presented with a choroidal mass of about one disc diameter in size. In addition, the patient had exudative retinal detachment and chorioretinal folds. B scan ultrasonography showed subretinal fluid, macular nodular thickening and underlying echolucent area along with medium internal reflectivity on A scan. Fluorescein angiography revealed early pinpoint areas of hyperfluorescence and late pooling under the detached retina. Indocyanine green angiography demonstrated early diffuse hypofluorescence corresponding to the area of detachment and late multiple pinpoint spots of hyperfluorescence. After intravenous methylprednisolone 1 g for 3 days followed by a course of oral prednisolone along with mycophenolate mofetil, the patient experienced rapid recovery with improvement in vision and complete resolution of subretinal fluid. On further follow-up, the patient regained 20/20 vision. Nodular posterior scleritis is a rare unilateral disease with strong female predominance. Multimodal imaging should be employed to confirm the diagnosis. The disease must be diagnosed correctly to avoid any unnecessary diagnostic work-up and aggressive management. Most cases carry excellent prognosis with no recurrence.

Keywords: Choroidal mass, exudative retinal detachment, nodular posterior scleritis, posterior scleritis


How to cite this article:
Alsarhani WK, Abu El-Asrar AM. Multimodal imaging of nodular posterior scleritis: Case report and review of the literature. Middle East Afr J Ophthalmol 2020;27:134-8

How to cite this URL:
Alsarhani WK, Abu El-Asrar AM. Multimodal imaging of nodular posterior scleritis: Case report and review of the literature. Middle East Afr J Ophthalmol [serial online] 2020 [cited 2022 May 24];27:134-8. Available from: http://www.meajo.org/text.asp?2020/27/2/134/290185




   Introduction Top


Posterior scleritis is inflammation of the sclera posterior to the equator.[1] Posterior scleritis may be classified into diffuse posterior scleritis or rarely nodular posterior scleritis.[2],[3] The diffuse form causes generalized increased eye wall thickness, while the nodular subtype causes a scleral nodule. The latter usually causes an amelanotic subretinal mass, with adjacent subretinal fluid and chorioretinal folds. Because of the scarcity of nodular posterior scleritis, it may be confused or even misdiagnosed as an intraocular tumor or posterior uveitis. Misdiagnosis may lead to aggressive treatment methods such as with radiotherapy or chemotherapy. One of the cases reported had been misdiagnosed as choroidal melanoma and was referred for enucleation.[4] Shields et al. reported that posterior scleritis represented 1.5% of 400 lesions mimicking choroidal melanoma.[5] Up to 29% of all cases of posterior scleritis are associated with other autoimmune diseases such as rheumatoid arthritis (9%), granulomatosis with polyangiitis (4%), and systemic vasculitis (4%).[3] B-scan ultrasonography may show nodular scleral thickening with fluid in Tenon's space. In this paper, we are reporting a case of nodular posterior scleritis and summarizing previously reported cases.


   Case Report Top


A 25-year-old male, otherwise medically free, presented with pain, redness, and decreased vision in the left eye for 2 days. He was given elsewhere topical prednisolone acetate and diclofenac with no improvement. At presentation, visual acuity was counting fingers at one foot. Extraocular motility was limited in all gazes. He had severe conjunctival chemosis with a deep and quiet anterior chamber. On fundus examination, an amelanotic choroidal mass of about one disc diameter in size located inferotemporal to the disc was found. In addition, there was exudative retinal detachment (ERD) involving the macula [Figure 1]a.
Figure 1: Fundus photograph illustrating a subretinal mass located at the inferior part of the macula. Exudative retinal detachment and chorioretinal folds are seen in the macula (a). Optical coherence tomography showing choroidal thickening with subretinal fluid and hyperreflective fibrinous material (b). B-scan ultrasonography showing surbretinal fluid and nodular thickening at the macula with underlying echolucent area (c). A-scan ultrasonography showing medium internal reflectivity (d)

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Optical coherence tomography (OCT) examination revealed multifocal ERD with increased choroidal thickening [Figure 1]b. B-scan ultrasonography revealed subretinal fluid and hyperechogenic nodular scleral thickening with underlying diffuse edema in Tenon's space [Figure 1]c. Extraocular muscles were normal and not involved on ultrasonography. The lesion showed medium internal reflectivity on A-scan ultrasonography [Figure 1]d. Fundus fluorescein angiography (FFA) showed early pinpoint areas of hyperfluorescence along with late pooling of dye under the detached retina [Figure 2]a, [Figure 2]b, [Figure 2]c. Indocyanine green angiography (ICGA) revealed diffuse hypofluorescence corresponding to the area of ERD in the early phase with multiple pinpoint hyperfluorescence in the late phases [Figure 2]d, [Figure 2]e. Computerized tomography (CT) scan of the orbit with contrast demonstrated scleral thickening [Figure 2]f.
Figure 2: Fluorescein angiography demonstrating multiple areas of pinpoint leakage (a and b) and diffuse pooling at the area of the exudative retinal detachment (c). Indocyanine green angiography showing an area of early hypofluorescence at the location of the detached retina (d) and areas of pinpoint hyperfluorescence during late phases (e). Computerized tomography showing scleral thickening (f)

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Systemic workup including antinuclear antibodies, antidouble-stranded DNA, antineutrophilic cytoplasmic antibodies, rheumatoid factor, IgG4, angiotensin-converting enzyme serum lysozyme, tuberculin skin test, syphilis serology, and CT chest was all unremarkable. The patient had a mildly elevated erythrocyte sedimentation rate and C-reactive protein.

The patient received intravenous methylprednisolone 1 g for 3 days, followed by a combination of a course of oral prednisolone (1 mg/kg/day) along with mycophenolate mofetil 1 g twice a day as a steroid-sparing agent. The patient experienced rapid improvement in vision. One month after starting treatment, visual acuity improved to 20/20, and OCT examination revealed the complete resolution of subretinal fluid [Figure 3]. Moreover, there was no recurrence over 6 months.
Figure 3: Fundus image demonstrating complete resolution of the subretinal mass and exudative retinal detachment (a). Optical coherence tomography image illustrating complete resolution of subretinal fluid after treatment (b)

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   Discussion Top


On review of the literature, the mean age at diagnosis was 52 years. Nodular posterior scleritis is a unilateral disease with strong female predominance. Most cases reported did not have any systemic association. The most common presenting symptom was pain and decreased vision. Presenting visual acuity ranged from 20/20 to 20/200. About half of the cases reported in the literature did not have any anterior segment inflammation. Similarly, McCluskey et al. reported that anterior segment involvement is seen in 60% of posterior scleritis cases.[3] The most common clinical features reported were amelanotic subretinal mass, ERD, and chorioretinal folds. While disc swelling was a common sign of diffuse posterior scleritis, it was only reported in two nodular posterior scleritis cases.[6],[7] The differential diagnosis of amelanotic subretinal mass includes amelanotic melanoma, choroidal granuloma, choroidal hemangioma, and choroidal osteoma.[8] Clinical features used to distinguish nodular posterior scleritis from choroidal melanoma include the presence of pain, conjunctival injection, chemosis, limited extraocular motility, and choroidal folds. Cases with normal anterior segment represent a diagnostic challenge as they closely resemble choroidal melanoma.

FFA is an important assessment tool distinguishing nodular posterior scleritis from choroidal melanoma. FFA findings in our case were consistent with what has been reported in the literature. The most common FFA finding was multiple pinpoint areas of leakage and pooling of dye consistent with subretinal fluid. ICGA in our case confirms that posterior scleritis may cause major alteration in the choroid. It showed early diffuse hypofluorescence corresponding to the ERD, which is possibly due to choroidal perfusion defect. Moreover, there was pinpoint hyperfluorescence seen in intermediate and late stages. This most likely represents choroidal inflammation and leakage from the large choroidal vessels. This pattern is seen in acute uveitis associated with Vogt-Koyanagi-Harada disease.[9] ICGA findings of nodular posterior scleritis were rarely reported in the literature. The ICGA pattern in our case is similar to what has been reported by Liu.[10] OCT typically shows choroidal mass, subretinal fluid, and choroidal folds. The most common finding on B-scan ultrasonography was an acoustically solid scleral mass with adjacent echolucent area representing scleral edema. Most cases showed medium-to-high internal reflectivity.[4],[6],[7],[11],[12],[13]

The prognosis of nodular posterior scleritis is excellent. There was no recurrence in the cases reported in the literature. Most showed complete resolution of the mass and subretinal fluid with improvement in vision to 20/30 or better [Table 1]. Similarly, our patient achieved a visual acuity of 20/20. Most cases responded well to systemic steroids [Table 2]. One case showed no response to steroids.[13] Besides systemic steroids, one case was treated with mycophenolate mofetil.[20] In our case, steroids achieved rapid remission of the disease. We added mycophenolate mofetil to steroids to spare the patient from systemic steroids. The choice of mycophenolate mofetil was based on its safety profile and efficacy in the treatment of noninfectious ocular inflammatory diseases.[23]
Table 1: Literature review of patients with posterior nodular scleritis: Demographics and historical findings

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Table 2: Treatment modalities and outcome of patients with posterior nodular scleritis

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   Conclusion Top


Nodular posterior scleritis is a rare unilateral disease, which may mimic an intraocular tumor. It predominantly affects females. It causes a yellow subretinal mass, ERD, and chorioretinal folds. Multimodal imaging including OCT, FFA, ICGA, ultrasonography, and CT orbit should be employed to confirm the diagnosis. The disease must be diagnosed correctly to avoid any unnecessary diagnostic workup and aggressive management. Most cases carry excellent prognosis with no recurrence.

Acknowledgment

The authors would like to thank Ms. Connie Unisa-Marfil for secretarial assistance. The research is supported by King Saud University through Vice Deanship of Research Chair in Ophthalmology (Dr. Nasser Al-Rashid Research Chair in Ophthalmology (Abu El-Asrar A)).

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
de la Foster CS. The Sclera. New York, NY: Springer-Verlag; 1994. p. 112-5.  Back to cited text no. 1
    
2.
Watson P, Hazleman B, Pavésio C. Clinical presentation of episcleritis and scleritis. In: The Sclera and Systemic Disorders. 3rd ed.. London: JP Medical Pub.; 2012. p. 66.  Back to cited text no. 2
    
3.
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.  Back to cited text no. 3
    
4.
Finger PT, Perry HD, Packer S, Erdey RA, Weisman GD, Sibony PA. Posterior scleritis as an intraocular tumour. Br J Ophthalmol 1990;74:121-2.  Back to cited text no. 4
    
5.
Shields JA, Augsburger JJ, Brown GC, Stephens RF. The differential diagnosis of posterior uveal melanoma. Ophthalmology 1980;87:518-22.  Back to cited text no. 5
    
6.
Wang JK, Lai PC, Yang CH. Subretinal mass as a presenting sign of posterior scleritis: A case report. Kaohsiung J Med Sci 2003;19:522-5.  Back to cited text no. 6
    
7.
Brod RD, Saul RF. Nodular posterior scleritis. Arch Ophthalmol 1990;108:1170-1.  Back to cited text no. 7
    
8.
Welch RJ, Newman JH, Honig SE, Mayro EL, McGarrey M, Graf AE, et al. Choroidal amelanotic tumours: Clinical differentiation of benign from malignant lesions in 5586 cases. Br J Ophthalmol 2020;104:194-201.  Back to cited text no. 8
    
9.
Abouammoh MA, Gupta V, Hemachandran S, Herbort CP, Abu El-Asrar AM. Indocyanine green angiographic findings in initial-onset acute Vogt-Koyanagi-Harada disease. Acta Ophthalmol 2016;94:573-8.  Back to cited text no. 9
    
10.
Liu AT, Luk FO, Chan CK. A case of giant nodular posterior scleritis mimicking choroidal malignancy. Indian J Ophthalmol 2015;63:919-21.  Back to cited text no. 10
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11.
Sridharan S, Juneja R, Hussain A, Biswas J. Giant nodular posterior scleritis mimicking choroidal tumor. Retin Cases Brief Rep 2007;1:65-7.  Back to cited text no. 11
    
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Shukla D, Kim R. Giant nodular posterior scleritis simulating choroidal melanoma. Indian J Ophthalmol 2006;54:120-2.  Back to cited text no. 12
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13.
Arevalo JF, Shields CL, Shields JA. Giant nodular posterior scleritis simulating choroidal melanoma and birdshot retinochoroidopathy. Ophthalmic Surg Lasers Imaging 2003;34:403-5.  Back to cited text no. 13
    
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Shibata Y, Kase S, Namba K, Ishida S. A case of nodular posterior scleritis simulating intraocular tumor. Int J Ophthalmol 2019;12:685-8.  Back to cited text no. 14
    
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Alsharif HM, Al-Dahmash SA. Atypical posterior scleritis mimicking choroidal melanoma. Saudi Med J 2018;39:514-8.  Back to cited text no. 15
    
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Agrawal R, Lavric A, Restori M, Pavesio C, Sagoo MS. NODULAR POSTERIOR SCLERITIS: Clinico-sonographic characteristics and proposed diagnostic criteria. Retina 2016;36:392-401.  Back to cited text no. 16
    
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Sin PY, Liu DT, Young AL. Nodular posterior scleritis mimicking choroidal tumor in a patient with systemic lupus erythematous: A case report and literature review. Asia Pac J Ophthalmol (Phila) 2016;5:324-9.  Back to cited text no. 17
    
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Ozkaya A, Alagoz C, Koc A, Ozkaya HM, Yazıcı AT. A case of nodular posterior scleritis mimicking choroidal mass. Saudi J Ophthalmol 2015;29:165-8.  Back to cited text no. 18
    
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Hage R, Jean-Charles A, Guyomarch J, Rahimian O, Donnio A, Merle H. Nodular posterior scleritis mimicking choroidal metastasis: A report of two cases. Clin Ophthalmol 2011;5:877-80.  Back to cited text no. 19
    
20.
Hatef E, Wang J, Ibrahim M, Turkcuoglu P, Khwaja A, Channa R, et al. Nodular sclerochoroidopathy simulating choroidal malignancy. Ophthalmic Surg Lasers Imaging 2010;41:e1-5.  Back to cited text no. 20
    
21.
Pérez-Campagne E, Guex-Crosier Y, Schalenbourg A, Uffer S, Zografos L. Giant nodular posterior scleritis compatible with ocular sarcoidosis simulating choroidal melanoma. Arch Soc Esp Oftalmol 2007;82:563-6.  Back to cited text no. 21
    
22.
Demirci H, Shields CL, Honavar SG, Shields JA, Bardenstein DS. Long-term follow-up of giant nodular posterior scleritis simulating choroidal melanoma. Arch Ophthalmol 2000;118:1290-2.  Back to cited text no. 22
    
23.
Daniel E, Thorne JE, Newcomb CW, Pujari SS, Kaçmaz RO, Levy-Clarke GA. Mycophenolate mofetil for ocular inflammation. Am J Ophthalmol 2010;149:423-32.  Back to cited text no. 23
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1], [Table 2]


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