Idiopathic uveal effusion syndrome

Idiopathic uveal effusion syndrome (UES) is a rare ocular condition characterized by the spontaneous accumulation of fluid (effusion) beneath the choroid and retina of the eye, without an identifiable underlying cause such as inflammation, infection, tumor, or elevated intraocular pressure. The condition primarily affects the uveal tract — the middle vascular layer of the eye comprising the choroid, ciliary body, and iris. Patients typically present with progressive, painless vision loss, often accompanied by serous retinal detachment and choroidal detachment. The condition may affect one or both eyes and tends to occur more frequently in middle-aged men, particularly those with shorter axial eye length (nanophthalmos or relative nanophthalmos) and abnormally thickened sclera. The pathophysiology of idiopathic uveal effusion syndrome is thought to involve impaired transscleral outflow of fluid from the suprachoroidal space, often related to abnormal scleral composition or thickness. This leads to protein-rich fluid accumulating beneath the choroid and subsequently beneath the retina. Three types have been described: Type 1 associated with nanophthalmos, Type 2 associated with abnormal sclera in eyes of normal size, and Type 3 occurring in eyes with clinically normal sclera and normal axial length. Treatment options are limited and depend on the type. Surgical intervention, particularly partial-thickness sclerectomy or sclerostomy (creating windows in the sclera to improve fluid drainage), is the primary treatment approach, especially for Types 1 and 2. This procedure has shown variable success in reducing effusions and improving or stabilizing vision. Medical treatments including corticosteroids and anti-inflammatory agents have generally been ineffective. The condition may be chronic and recurrent, and visual prognosis varies, with some patients experiencing significant permanent vision loss despite treatment.

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