Central serous chorioretinopathy associated with nonspecific orbital inflammation: a case report

Abstract

We herein present a rare case of acute central serous chorioretinopathy (CSC) associated with nonspecific orbital inflammation (NSOI). A 38-year-old woman presented with a 3-day history of ocular pain, reduced vision, periorbital swelling, proptosis, conjunctival chemosis, and restricted eye movements. Optical coherence tomography of the affected eye confirmed signs of CSC. Additionally, a computed tomography scan revealed enlargement of intraconal soft tissues and the lacrimal gland. Ocular ultrasonography detected posterior sclera thickening, indicating posterior scleritis. Following the diagnosis of NSOI, the patient received treatment with systemic corticosteroids, resulting in gradual regression of both the orbital inflammation and CSC. This is the first reported case of localized posterior pole CSC documented in a patient with NSOI. Vigilant monitoring for any ocular disorders is important in patients with orbital inflammation.

Introduction

Nonspecific orbital inflammation (NSOI), often referred to as orbital pseudotumor, is an enigmatic and non-granulomatous inflammatory condition occurring within the orbital cavity. This condition can affect different orbital soft tissues, including the extraocular muscles (myositis), the lacrimal gland (dacryoadenitis), and even the posterior sclera (scleritis). Establishing a diagnosis of NSOI is contingent upon a meticulous assessment that excludes other potential causative factors, including neoplastic disorders and systemic inflammation. Thus, comprehensive clinical and paraclinical evaluations are imperative for suspected cases. Such evaluations should entail detailed ocular and adnexal examinations, orbital imaging, and a systemic workup for inflammatory diseases.^1,2

Remarkably, NSOI has been associated with a spectrum of posterior segment eye disorders such as papillitis, choroiditis, choroidal detachment, retinal vascular occlusion, and exudative retinal detachment.^3–6 We herein present an exceedingly rare case of diffuse NSOI marked by inflammation of the intraconal soft tissues, dacryoadenitis, and posterior scleritis associated with acute central serous chorioretinopathy (CSC).

Case report

The reporting of this study conforms to the CARE guidelines. ⁷ This study adhered to the principles outlined in the Declaration of Helsinki, and written informed consent for publication of the report and all related images was obtained from the patient. Furthermore, all of the patient’s details were de-identified. The institutional review board does not require ethical approval for case reports.

The patient was a 38-year-old woman who presented to our clinic with a 3-day history of blurred vision, ocular pain, and progressive protrusion of her left eye. She had a history of stable hypothyroidism for which she was currently receiving medical treatment.

Upon examination, the patient’s best-corrected visual acuity was 20/20 in the right eye and 20/63 in the left eye. Pupillary reactions were normal, and no relative afferent pupillary defect was detected. Intraocular pressures were 14 mmHg in the right eye and 22 mmHg in the left eye, measured using a Goldmann tonometer. The patient exhibited periorbital swelling on the left side along with restricted eye movements (Figure 1(a)). Furthermore, axial proptosis of the left globe was present as evidenced by a Hertel exophthalmometer reading of 18 mm for the right eye and 21 mm for the left eye. Slit-lamp examinations revealed conjunctival chemosis with diffuse ciliary injection in the left eye. No abnormal findings were observed in other anterior segment examinations of both eyes. On dilated fundus examination, the vitreous was clear in both eyes. However, the left eye contained a serous detachment of the macula with no chorioretinal folds or other abnormalities. Optical coherence tomography confirmed serous macular detachment with accumulation of subretinal fluid (SRF) in the left eye. The maximum height of the SRF pocket, defined as the vertical distance from the retinal pigment epithelium to the external limiting membrane, measured 175 µm (Figure 1(b)). Posterior segment examinations of the right eye were unremarkable. B-scan ultrasonography of the left globe revealed a trace amount of sub-Tenon’s fluid with thickening of the posterior sclera (T-sign), consistent with posterior scleritis (Figure 1(c)). A computed tomography scan of the orbits showed proptosis, enlargement of the intraconal soft tissues, and enlargement of the left lacrimal gland compared with the contralateral side (Figure 1(d, e)). Laboratory test results, including a complete blood cell count, erythrocyte sedimentation rate, and antinuclear antibody, were all within normal limits.

Figure 1.

(a) The patient presented with periorbital swelling and conjunctival chemosis in the left eye. (b) Optical coherence tomography of the left eye showed serous macular detachment with the accumulation of subretinal fluid (indicated by blue asterisk). (c) B-scan echography (longitudinal scan with a gain of 80 dB) of the left eye revealed trace fluid in t sub-Tenon’s space (white arrow) and increased thickness (2.4 mm, indicated by the double-headed red arrow) of the posterior sclera (T-sign), which was compatible with posterior scleritis. (d, e) Non-contrast axial and coronal computed tomography scans of the orbit showed axial proptosis of the left globe with homogeneous enlargement of intraconal soft tissues behind the posterior sclera, extending anteriorly (indicated by white asterisk). Additionally, enlargement of the left lacrimal gland was detected (indicated by red asterisk). (f) One month after treatment, there was a significant improvement in periorbital swelling and conjunctival chemosis in the left eye and (g) After 3 months, follow-up optical coherence tomography revealed complete resorption of the subretinal fluid.

Consequently, the patient was diagnosed with NSOI along with CSC in the left eye. Treatment was initiated with intravenous methylprednisolone at a dosage of 500 mg twice a day for 2 days, followed by oral prednisolone at 75 mg per day and eplerenone at 20 mg twice a day. Remarkably, the patient’s signs and symptoms, including the periorbital swelling, proptosis, restricted eye movements, and conjunctival chemosis, showed substantial improvement after 1 month (Figure 1(f)). The best-correct visual acuity in the affected eye improved to 20/20, and complete resorption of the SRF was evident on follow-up optical coherence tomography 1 month after initiating treatment with oral prednisolone (Figure 1(g)). The medication was gradually tapered over this period and subsequently discontinued, with no recurrence of signs or symptoms of the disease.

Discussion

NSOI is characterized by idiopathic inflammation within the orbital structures and can extend to adjacent ocular components, including the choroid and retina.^3–6 In our case, the patient exhibited NSOI with intraconal soft tissue inflammation, dacryoadenitis, and posterior scleritis concomitant with acute CSC. To the best of our knowledge, this is the first reported case of localized posterior pole CSC documented in a patient with NSOI.

CSC manifests as serous retinal detachment that is primarily localized in the posterior pole. Its precise pathogenesis remains unclear, although it is associated with leakage of fluid that originates from the choriocapillaris, passes through the retinal pigment epithelium, and accumulates beneath the neural retina. ⁸ In our patient, the SRF accumulation might have been caused by vascular hyperpermeability triggered by orbital-associated inflammation and choroidal vasculitis, particularly in the context of posterior scleritis. ⁹

Alternatively, during posterior scleritis, the vortex veins (which are responsible for choroidal outflow and traversing the sclera) become congested because of reduced fluid drainage through the thickened sclera. This congestion may cause upstream disruption of choroidal blood flow, increased choriocapillaris permeability, and subsequent SRF accumulation.^6,9 Notably, scleral thickness is significantly greater in CSC-affected eyes than in normal eyes.^10,11 In our patient, ultrasonographic evidence of posterior scleritis with thickening provides a plausible explanation for the potential causes of CSC, as described above.

Although corticosteroid administration is a well-known risk factor for CSC, ⁸ our patient exhibited a remarkable treatment response to systemic steroids, likely because of the underlying inflammatory pathophysiology of NSOI. Similarly, a previous report documented the improvement of retinal vein occlusion secondary to NSOI following the treatment of orbital inflammation with systemic steroids. ⁵ Consequently, when NSOI coexists with ocular disorders, systemic immunosuppressive therapy should be initiated regardless of other associated conditions.^1,2 However, the side effects of systemic steroids should be diligently managed through adjuvant therapies and a tapering regimen.

Conclusion

This case illustrates an uncommon presentation wherein orbital inflammation leads to CSC. Therefore, when managing patients with orbital inflammation, it is imperative to promptly and adequately treat the condition while vigilantly monitoring for any associated ocular disorders.