A rare case report of unilateral posterior scleritis with neuroretinitis
Prerana Patil1*, Jayashree S Shah2, P Rachana1
1*Junior Resident, 2 Professor and HOD, Department of Ophthalmology, Sri Siddhartha Medical College, SSAHE, Tumkur Karnataka India
* Address for Correspondence:
Dr. PreranaPatil, Department of Ophthalmology, Sri Siddhartha Medical College & Research Centre, Sri Siddhartha Academy of Higher Education, Tumkur, Email: firstname.lastname@example.org
Posterior scleritis is a potentially blinding condition in which diagnosis is commonly delayed, with an adverse prognostic effect. We are reporting a 40-year-old female patient presenting with blurred disc margins, treated as a case of unilateral optic neuropathy progressed to neuroretinitis with associated posterior scleritis of an unknown etiology. This case was a medical challenge in terms of prognosis of the patients and eliciting the underlying etiology, it's a rare presentation of 2 diseases coexisting in the same eye. The mainstay for treatment with oral steroids was aimed towards improving the vision and halting the progress of the disease.
Keywords: posterior scleritis, neuroretinitis, oral steroids
Scleritis can be classified as anterior, posterior scleritis depending on the site of inflammatory process within the sclera. Posterior scleritis is a rare but potentially vision-threatening condition, clinically posterior scleritis presents with varied features and is underdiagnosed. Patients present with mild to moderate pain with degrees of inflammation of sclera posterior to OraSerrata, it seen across all ethnicities, commonly presenting in middle aged women. It accounts for not more than 2% - 12% of all cases of scleritis. In 50% cases it can be bilateral.Neuroretinitis is an inflammation of the neural retina and optic nerve. It was originally described by Leber in 1916 as a "stellate maculopathy," but this definition was challenged by Don Gass in 1977, citing that disc edema precedes macular exudates. It is a rare entity that we see 2 diverse diseases with such varied causes coexisting in the same eye. This case is being presented as a rare presentation of posterior scleritis.
A 40-year-old female presented to Ophthalmology outpatient department with sudden loss of vision in left eye for one week, associated with pain on ocular movements. Patients in not a known case of hypertension diabetes mellites, no history suggestive of rheumatoid arthritis, history was elicited to rule out systemic association. On ocular examination the best corrected visual acuity (BCVA) in the Right eye was 6/6, the near vision was N6 and BCVA in the Left eye was 6/60, near vision was N36.Patient had impaired color vision in the left eye, anterior segment examination showed relative afferent pupillary defect (RAPD) in the LE other parameters were within normal limits. Fundus examination of the left eye showed Blurred disc margins with 2-disc diopter (DD) elevation (Figure 1), Patient was started on oral steroids -T Prednisolone 80mg OD.
Figure 1: Blurred disc margin (LE)
Patient came for a follow up visit after 5 days, she complained of worsening of vision despite initiation of treatment. On dilated fundus evaluation, the patient had Blurred disc margins with 2DD elevation and showed few hard exudates arranged in a radial pattern around macula-appearance of macular star in the left eye (Figure 2). The right eye showed a normal fundus picture. B scan was advised which showed sub retinal fluid with prominent T sign (Figure 3). A final diagnosis of Posterior scleritis was made. MRI was done to rule out a possible compressive mass, after which patient was continued with oral steroids on a tapering dose and was planned for periocular steroid injection post which patient was lost in follow up.
Figure 2: Blurred disc with macular star (LE) Figure 3: T-sign due to fluid collection within
Posterior scleritis presents with severe inflammatory pathology showing microangiopathy suggesting features of autoimmune disorder. Patients with this condition present with periocular pain, red eye, decreased vision & painful ocular movements. Posterior scleritis is one of the most misdiagnosed conditions due to its variable presentations. Ultrasonography (A- and B-scan) is the key investigation necessary to make the diagnosis of posterior scleritis. CT and MRI scans are especially important in differential diagnosis of posterior scleritis or the diagnosis of the underlying disease. In this patients there were features of neuroretinitis in which differential diagnosis for macular star includes: hypertensive retinopathy, papilledema, anterior ischemic optic neuropathy, diabetic papillopathy, posterior vitreous traction, disc and juxta papillary tumors, and toxic etiologies, making it essential to rule out the etiology and a proper systemic work up was prompted, as there is rapid progression of the disease. Patients with loss of vision, involvement of optic nerve, systemic association needs aggressive anti-inflammatory medication with systemic immunosuppressive therapy. Follow up is important as recurrence and bilateral involvement is seen.
It is important to advise B-scan in cases of idiopathic and non-responsive posterior segment pathology to rule out posterior scleritis, in this case B-scan proved to be the key diagnostic tool. Oral corticosteroids used as the first line of treatment in this entity showed promising results, although complete resolution of inflammation often takes several weeks. Timely follow up with such patients is essential to monitor the diseases progression and effects of corticosteroids.
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Conflict of interest: Nil
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