Choroidal Effusion Mimicking Uveal Melanoma: A Novel Presentation of Idiopathic Systemic Capillary Leak Syndrome

Abstract

Background: Idiopathic systemic capillary leak syndrome (ISCLS, also known as Clarkson's disease) is a rare medical condition characterized by episodes of capillary endothelial cell dysfunction with leakage of fluid into the interstitial space resulting in severe hypotension, hemoconcentration, hypoalbuminemia, and generalized edema. Each episode can result in multiorgan failure due to systemic hypoperfusion.

Case presentation: We report a case of uveal effusion, mimicking uveal melanoma, associated with ISCLS following viral infection. A 74-year-old white male was evaluated in our ocular tumor clinic for a large intraocular mass in the right eye concerning for choroidal melanoma. We completed a review of the literature and list clinical recommendations for these cases. ISCLS, although rare, was a significant diagnostic consideration in this patient. Due to the high mortality rate of this condition, accurate diagnosis and prompt treatment was critical. We hypothesize that the mechanism of choroidal effusion development was due to reduced oncotic pressure from rapid decrease in serum albumin. Increased permeability of choroidal capillaries may be an additional mechanism leading to uveal effusion.

Conclusion: With treatment, the patient had complete resolution of his choroidal effusion with no recurrence of his ISCLS. Further research should be considered on the role of viral infections in the pathogenesis of ISCLS.

Keywords: Choroidal effusion; Idiopathic systemic capillary leak syndrome; Pseudomelanoma; Uveal effusion; Uveal melanoma.

Fig. 1

Photos and ultrasound of the right eye upon presentation. A color fundus photograph (a) and wide-field imaging (b) of the ocular fundus of the right eye demonstrate a large pigmented elevated choroidal lesion located nasally. In the fluorescein angiogram mid phase (c) and wide-field late phase (d), the vasculature appears elevated, and capillaries are prominent with dye. No secondary circulation was noted. The B-scan ultrasound of the right eye (e) demonstrates a large echolucent dome-shaped serous effusion superiorly. The A-scan (f) shows no internal echoes in the lesion.

Fig. 2

B-scan ultrasound and color fundus photograph post-treatment. The B-scan ultrasound (a), color fundus photograph (b), and wide-field image of the ocular fundus (c) of the right eye show resolving choroidal effusion 2 months following treatment.