Different scleral decompression surgical procedures have been proposed for the treatment of idiopathic and nanophthalmic uveal effusion syndrome (UES). The aim of this review is to describe the different surgical approaches reported in the literature and compare the outcomes and complications between them. We searched PubMed/MEDLINE, Scopus and Web of Science for all articles that reported scleral decompressive surgical procedures for idiopathic and/or nanophthalmic UES treatment, as well as publications reporting prophylactic surgeries for uveal effusion in nanophthalmic eyes. Risk of bias was assessed using the Cochrane proposed tool for randomized controlled trials- RoB2 and the JBI checklist for case series and for cohort studies. Twenty-eight articles were included and reviewed. Sclerectomies were the most frequently reported procedures, associated or not with sclerostomies or sclerotomies. Following in frequency were sclerostomies and sclerotomies alone, whereas vortex vein decompression (VVD) was the less frequently reported surgery. Overall, the articles demonstrated positive results in the resolution of uveal effusion and retinal/choroidal detachment, as well as in the prevention of uveal effusion in nanophthalmic eyes. Improvement in visual acuity (VA) was reported by most authors, except in cases with long-term retinal detachments (RD), where retinal damage prevented an enhancement of VA even with good anatomical results. Moreover, three studies included the use of adjunctive treatment to the surgeries, particularly mitomycin C (MMC) and intravitreal anti-VEGF injections. Complications of scleral decompression surgeries were reported in only ten articles and the most frequent and serious ones included phthisis bulbi, retinal and suprachoroidal hemorrhage, and vortex vein incision, among others. In general, scleral decompressive surgeries showed efficacy in treating and preventing UES. However, bigger studies would be necessary to minimize possible bias and to draw more solid conclusions regarding the benefit of surgical management of these patients, compared to a conservative one, and to better understand if adjunctive treatment can be, in fact, beneficial or not. What is known The best treatment for idiopathic and nanophthalmic uveal effusion syndrome is not yet clear among ophthalmologists, particularly due to their rare incidence. Many surgical techniques have been proposed for the management of these conditions, but a bigger study on this topic has not yet been made. What is new We performed a systematic review of all published literature on surgical approaches for idiopathic and nanophthalmic uveal effusion syndrome, including its prophylaxis in nanophthalmic eyes. Most studies demonstrated good results with the use of scleral decompressive surgeries, particularly sclerectomies, sclerostomies and sclerotomies. Bigger studies with control groups are, however, necessary to create more robust evidence.

This study aimed to report the diagnostic process, treatment, and follow-up of a patient with bullous exudative retinal detachment (RD) associated with an atypical variant of bilateral central serous chorioretinopathy (CSCR). A 28-year-old woman was referred to our clinic for total bullous RD in the right eye with a vision level of light perception only. She had been previously diagnosed with idiopathic uveal effusion syndrome and treated with systemic corticosteroid therapy with no response, and was referred to us for scleral window surgery. Four-quadrant scleral window surgery with external drainage of the subretinal fluid was performed, resulting in a transient partial attachment of the retina. RD started to progress again within 3 weeks, which prompted comprehensive imaging together with more advanced systemic workup for systemic lupus erythematosus and other rheumatological and immunological diseases. Systemic corticosteroid therapy was initiated during this period but did not stop the progression and was discontinued after a short time. Fluorescein angiography and indocyanine green angiography revealed multifocal choroidal leakage foci and large choroidal vessels without any intraocular inflammation findings and led to the diagnosis of atypical CSCR. Pars plana vitrectomy (PPV), internal drainage of the subretinal fluid, endolaser to the focal leakage areas, and intravitreal aflibercept injection were performed. Visual acuity increased to 0.8 within 8 months after the surgery with no recurrence. Bullous exudative RD is a very rare and atypical form of CSCR, and a favorable outcome can be obtained with PPV and surgical drainage of subretinal fluid followed by laser photocoagulation.

To describe central serous chorioretinopathy cases presenting as uveal effusion syndrome, providing new insights into "pachychoroid spectrum" diseases. Clinical charts, color fundus photographs, fluorescein angiography, indocyanine green angiography, optical coherence tomography, ultrasound imaging, cerebral magnetic resonance imaging, and biometry of four eyes of three patients were assessed. A literature review was conducted. The three patients had peripheral choroidal detachment and inferior bullous retinal detachment associated with central serous chorioretinopathy features detected using multimodal imaging, including fluorescein and indocyanine green angiography. The choroid was thick in the three patients, and uveal effusion occurred after steroid treatment in all cases. Subretinal fluid drainage and deep sclerectomy with flaps of 4 × 4 mm in both inferior quadrants were performed in three eyes of two patients with good outcomes. One patient was treated with photodynamic therapy. All three patients developed a typical leopard-spot pigmentary pattern in the fundus. A severe presentation of highly exudative central serous chorioretinopathy may occur in rare cases with a peripheral choroidal detachment mimicking uveal effusion syndrome. These severe cases highlighted the role of choroidal thickening and hyperpermeability, choroidal vein dilation, and possible scleral thickening in both entities.

To investigate the clinical characteristics of idiopathic uveal effusion syndrome (IUES) and to identify effective surgical modalities for its treatment. This retrospective analysis included clinical data of 33 eyes from 26 patients with IUES at Beijing Tongren Hospital. Records of eye examinations, ocular ultrasound, ocular ultrasound biomicroscopy (UBM), and follow-up surgical treatment were reviewed and analyzed. Of 26 patients, 17 (65.4%) were male and 9 (34.6%) were female. The average age of disease onset was 46.8y (range: 22-64y). Seven patients (26.9%) showed retinal detachment in both eyes at presentation. B-ultrasound showed the presence of retinal detachment in one eye or both eyes. All patients had binocular ciliary leakage and detachment. Eyes with retinal detachment underwent four-quadrantic partial-thickness sclerectomy and sclerostomy. Subretinal fluid resolution was achieved within 6mo. Recurrence was observed in three eyes and was resolved with re-operation. Ophthalmic ultrasound and UBM, among others, can be helpful in the diagnosis of IUES. Sclerectomy and sclerostomy are surgical modalities that can successfully treat the disease. Some patients may experience recurrence after surgery; reoperation remains safe and effective for them. Long-term follow-up is essential in such settings.