Uveal Effusion Syndrome as the Initial Manifestation of Primary Sjögren's Syndrome and Review of the Literature.

Purpose: The authors report a38-year-old woman with primary Sjögren's syndrome who initially showed recurrent blurred vision caused by uveal effusion syndrome and later developed dry mouth, dry eyes, and arthralgia. During the 5-year-course of disease, the patient's 3-time-onset was all manifested as blurred vision after decreased immunity. Despite the initial absence of sufficient immunological evidence, the final presence of positive serum anti-SS-A, rheumatoid factors, ANA, and inflammatory findings in minor salivary gland biopsy indicated primary Sjögren's syndrome.Methods: Retrospective review of a case note.Conclusions: The manifestation of UES requires further exploration of its real pathogenesis, and the possibility of systemic disease should never be excluded.

A Retrospective Case Series of Uveal Effusion Syndrome.

PURPOSE: This study aimed to describe the clinical findings and management of eyes affected by uveal effusion syndrome. METHODS: We retrospectively evaluated the charts of 13 eyes of 8 consecutive patients diagnosed with uveal effusion syndrome attending the Ophthalmology Unit of the University Hospitals Leuven, Belgium, between 2007 and 2018. The presenting features, investigations, management, and outcomes were analyzed for each case. RESULTS: Cataract surgery was the predisposing factor for uveal effusion in 6 eyes, 2 bilateral uveal effusions (4 eyes) were considered to be medication-induced, and in 3 eyes, the uveal effusion was described as idiopathic. Fundus examination of 5 of 13 eyes showed bullous choroidal detachment, treated with pars plana vitrectomy with superotemporal sclerectomy or transscleral punction. Fundoscopy showed uveal effusion without serous retinal detachment in 3 eyes. Serous retinal detachment accompanied by uveal swelling was observed in 3 eyes and the 2 remaining eyes presented with uveal swelling only. The 8 nonbullous choroidal detachments were treated in a conservative way. A rapid resolution of subretinal fluid and uveal effusion was observed in all cases. CONCLUSIONS: A conservative approach with acetazolamide treatment or just observation was used in our case series in choroidal detachment without substantial visual loss if, over time, slow improvement was documented. However, further studies are needed to verify the effectiveness of the reported therapy.

Type III uveal effusion syndrome suspected to be related to pachychoroid spectrum disease: A case report.

INTRODUCTION: We report a case of type III uveal effusion syndrome (UES) suspected to be related to pachychoroid spectrum disease. PATIENT CONCERNS: A 42-year-old man became aware of visual field constriction and deterioration of visual acuity in his right eye. DIAGNOSIS: Upon examination, a bullous non-rhegmatogenous retinal detachment was observed in the inferior 2 quadrants of the right eye fundus, and the subretinal fluid moved with postural changes. The axial length in that eye was 22.36 mm, thus indicating no nanophthalmia. Preoperative indocyanine green angiography revealed dilated choroidal vessels in the posterior pole of the right eye and mild leakage in the late phase. Optical coherence tomography examination revealed choroidal thickening in both eyes. INTERVENTIONS: For treatment, we first performed sclerotomy, and the intraoperative findings showed no thickening of the sclera. Following surgery, reattachment of the retina was not achieved. OUTCOMES: Thus, we next performed vitrectomy, which led to successful reattachment of the retina. LESSONS: In this case, we theorize that pachychoroid spectrum disease might have been involved in the pathogenesis of type III UES.

Familial Nanophthalmos Presenting with Spontaneous Uveal Effusion Syndrome.

Purpose: To report two unique cases of spontaneous uveal effusion syndrome (UES) from the same family.Methods: The patients included father (54 years old) and a daughter (23 years old). The father presented 7 years ago with bilateral exudative retinal detachment, nanophthalmic eyes (axial length 16.11 mm and 16.43 mm in right and left eyes, respectively) with hypermetropia, and a thickened sclera on MRI. The daughter presented with left eye peripheral exudative annular retinal detachment with bilateral nanophthalmos (axial length 20.7 mm and 20.33 mm, in right and left eyes, respectively), with MRI showing ciliochoroidal detachment, suprachoroidal effusion and mild thickening of the sclera in left eye.Results: The father underwent subscleral sclerectomy in the left eye that led to resolution of submacular fluid. Scleral biopsy confirmed the deposition of glycosaminoglycans on Alcian blue stain. The daughter was kept under observation, because of absence of any macular subretinal fluid, and counselled about the familial nature of the disease and possible need for future intervention.Conclusion: This is the first report of familial nanophthalmos presenting with spontaneous UES.