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PurposeObservationsConclusionsUveal effusion syndrome (UES) is an exudative detachment of ciliary body, retina and choroid. Various underlying causes leads to UES-like drugs (topiramate), inflammation and hypotony. Wnt gene involvement has never been associated with UES. We report a case of bilateral UES being misdiagnosed as Vogt–Koyanagi–Harada syndrome (VKH), with Wnt gene dysfunction as the underlying trigger.A 32-year-old male presented with diminution of vision in his left eye. He was found to have choroidal detachment with retinal detachment in his left eye. Choroidal detachment was noted in the right eye. Various ocular imaging including fundus fluorescein angiography and ocular coherence tomography was done. He was misdiagnosed as a case of VKH syndrome, for which he was treated with systemic immunomodulatory therapy. However, a subclinical response was made to revisit the diagnosis. Ultrasound biomicroscopy showed supraciliary effusion. Systemic and genetic evaluation led to the detection of Wnt10A pathway mutation.UES is an entity of diagnostic challenge. Careful and thorough systemic evaluation is required to clinch the diagnosis. We reported the first case of bilateral UES recalcitrant to corticosteroids, with Wnt10A gene mutation. [ABSTRACT FROM AUTHOR]

Central serous chorioretinopathy (CSC) is a common disorder characterized by serous retinal detachment. Several studies using indocyanine green angiography (ICGA) have revealed that choroidal filling delay, choroidal vascular dilation, and choroidal vascular hyperpermeability are the characteristic findings of CSC. These ICGA findings confirm that choroidal circulatory disturbances are the primary factors in the pathogenesis of CSC. With advancements in optical coherence tomography (OCT), choroidal thickness has been found to be significantly greater in eyes with CSC than in normal eyes. Dilated large choroidal vessels reportedly account for the thickened choroid in eyes with CSC. Although many possible mechanisms and risk factors have been suggested, the pathophysiologic features of choroidal circulatory disturbances and choroidal thickening in eyes with CSC have not yet been fully elucidated. Recently, using anterior segment OCT, we proposed that the sclera may induce choroidal circulatory disturbances since CSC eyes have significantly thicker sclera than do normal eyes. This review summarizes updated information on the close relationship between CSC pathogenesis and the sclera. [ABSTRACT FROM AUTHOR]

Abstract Background This case report is applicable to the field of ophthalmology because there is a paucity of medical literature related to the clinical presentation, diagnosis, and management of uveal effusion syndrome. This is an urgent concern because there are severe complications associated with this disease, including non-rhegmatogenous retinal detachment, angle closure glaucoma, and possible blindness. This report will fill clinical knowledge gaps using a patient example. Case presentation A 68-year-old white male with multiple cardiovascular risk factors initially presented to the Eye Institute Urgent Care Clinic with new onset visual symptoms, including eye pain, eye lid swelling, redness, and tearing of his left eye. He had experienced a foreign body sensation in the left eye and bilateral floaters weeks prior to his presentation. The patient was examined, and vision was 20/30 in both eyes, and intraocular pressure was 46 in the right eye and 36 in the left eye. After initial assessment, including compression gonioscopy, intermittent angle closure glaucoma was suspected. He received oral diamox 500 mg, one drop of alphagan in both eyes, one drop of latanoprost in both eyes, one drop of dorzolamide in both eyes, and one drop of 2% pilocarpine in both eyes. There was only slight response in intraocular pressure. Owing to the bilateral angle closure, he underwent laser peripheral iridotomy to decrease intraocular pressure and open the angle that was found closed on gonioscopy. The patient was discharged on oral and topical glaucoma drops and scheduled for the glaucoma clinic. When he presented for follow-up in the glaucoma clinic, he was evaluated and noted to have bilateral narrow angles and intraocular pressure in the mid-twenties. A brightness scan (B-scan) was performed and was noted to have bilateral choroidal effusions, confirmed by Optos fundus photos. He was started on prednisone at 60 mg once per day (QD) with taper, continuation of oral and topical glaucoma medications, and a retina evaluation. Evaluation with a retina specialist showed resolving choroidal effusion in the left eye. He continued the prednisone taper as well as glaucoma drops as prescribed. Follow-up in the glaucoma clinic revealed a grade 3 open angle. He continued the prednisone taper, cosopt twice per day in both eyes, and discontinued brimonidine. The magnetic resonance imaging (MRI) that was performed showed results that were remarkable. No hemorrhage or mass was present. Follow-up with the retina specialist found that the choroidal effusions had resolved completely. Conclusion This case report emphasizes the value in early detection, keen diagnostic evaluation, and cross-collaboration between multiple ophthalmology specialists to optimize healthcare outcomes for patients with uveal effusion syndrome.

Case 1: A 72-year-old male presented with a complaint of "decline in visual acuity in both eyes for 3 years, with the right eye being more severe". The patient has had poor vision in both eyes since childhood and underwent bilateral surgical treatment for glaucoma 10 years ago, with no details on the specific surgical procedure. The uncorrected distance visual acuity (UDVA) was hand motion (HM) in the right eye (OD) and finger counting (FC) at 20 cm in the left eye (OS). Best corrected visual acuity (BCVA) was 0.02 (+18.0 DS) OD and 0.12 (+18.0 DS) OS. Slit-lamp examination showed extremely shallow anterior chambers and nuclear cataracts in both eyes. B-scan ultrasonography suggested the possibility of microphthalmos in both eyes and peripheral retinal detachment in the inferotemporal quadrant of both eyes. Axial lengths(AL) were 16.21 mm OD and 16.31 mm OS. The diagnosis was bilateral nanophthalmos with cataract. The patient underwent general anesthesia for right eye phacoemulsification with intraocular lens (IOL) implantation and sclerostomy. A 40.0 D intraocular IOL was implanted. The surgery went smoothly without complications. Case 2: A 59-year-old male sought treatment for "a 2-year decline in vision in the right eye". The patient had poor vision in both eyes since childhood and had undergone sclerectomy for uveal effusion syndrome. The UDVA was hand motion (HM) OD and finger counting (FC) at 20 cm OS. BCVA was 0.01 (+18.5 DS) OD and 0.2 (+17.00 DS/-0.75 DC×85°) OS. Slit-lamp examination showed extremely shallow anterior chambers and nuclear cataracts bilaterally. The retina appeared flat at the posterior pole in both eyes. B-scan ultrasonography suggested microphthalmos in both eyes with thickened sclera. AL were 15.40 mm OD and 15.51 mm OS. The diagnosis was bilateral nanophthalmos with cataract. The patient underwent general anesthesia for right eye phacoemulsification with a 40.0 D IOL cataract surgery on patients with nanophthalmos and a history of eye surgery, it is necessary to design a customized surgical plan. If necessary, prophylactic sclerectomy was recommended to prevent the occurrence of choroidal effusion. At the same time, regarding the selection of IOLs, the choice of Piggyback IOL should be made with caution, as the safe restoration of visual function is more important. [ABSTRACT FROM AUTHOR]

Background: This case report is applicable to the field of ophthalmology because there is a paucity of medical literature related to the clinical presentation, diagnosis, and management of uveal effusion syndrome. This is an urgent concern because there are severe complications associated with this disease, including non-rhegmatogenous retinal detachment, angle closure glaucoma, and possible blindness. This report will fill clinical knowledge gaps using a patient example. Case presentation: A 68-year-old white male with multiple cardiovascular risk factors initially presented to the Eye Institute Urgent Care Clinic with new onset visual symptoms, including eye pain, eye lid swelling, redness, and tearing of his left eye. He had experienced a foreign body sensation in the left eye and bilateral floaters weeks prior to his presentation. The patient was examined, and vision was 20/30 in both eyes, and intraocular pressure was 46 in the right eye and 36 in the left eye. After initial assessment, including compression gonioscopy, intermittent angle closure glaucoma was suspected. He received oral diamox 500 mg, one drop of alphagan in both eyes, one drop of latanoprost in both eyes, one drop of dorzolamide in both eyes, and one drop of 2% pilocarpine in both eyes. There was only slight response in intraocular pressure. Owing to the bilateral angle closure, he underwent laser peripheral iridotomy to decrease intraocular pressure and open the angle that was found closed on gonioscopy. The patient was discharged on oral and topical glaucoma drops and scheduled for the glaucoma clinic. When he presented for follow-up in the glaucoma clinic, he was evaluated and noted to have bilateral narrow angles and intraocular pressure in the mid-twenties. A brightness scan (B-scan) was performed and was noted to have bilateral choroidal effusions, confirmed by Optos fundus photos. He was started on prednisone at 60 mg once per day (QD) with taper, continuation of oral and topical glaucoma medications, and a retina evaluation. Evaluation with a retina specialist showed resolving choroidal effusion in the left eye. He continued the prednisone taper as well as glaucoma drops as prescribed. Follow-up in the glaucoma clinic revealed a grade 3 open angle. He continued the prednisone taper, cosopt twice per day in both eyes, and discontinued brimonidine. The magnetic resonance imaging (MRI) that was performed showed results that were remarkable. No hemorrhage or mass was present. Follow-up with the retina specialist found that the choroidal effusions had resolved completely. Conclusion: This case report emphasizes the value in early detection, keen diagnostic evaluation, and cross-collaboration between multiple ophthalmology specialists to optimize healthcare outcomes for patients with uveal effusion syndrome. [ABSTRACT FROM AUTHOR]

Purpose: To report a rare case of Harada disease that initially presented as a unilateral ring of choroidal effusions. Methods: Retrospective chart review. Results: A 42-year-old Trinidadian woman presented with a serous retinal detachment overlying a unilateral ring of choroidal effusions in the absence of any systemic symptoms. Visual acuity was hand motion with a relative afferent pupillary defect. Biometry revealed an axial length of 21.12 mm and a thickened sclerochoroidal complex. Fluorescein angiography did not demonstrate pinpoint leakage or subretinal pooling classically seen in Harada disease. Oral steroids improved the visual acuity to 20/50, and laboratory testing and head imaging returned nonrevealing. After 2 months of incomplete steroidal treatment due to nonadherence, the patient represented with bilateral decreased visual acuity, anterior uveitis, serous detachments, and diffuse pinpoint leakage on fluorescein angiography consistent with the early stage of Harada disease. Conclusion: This is a case of a unilateral presentation of Harada disease with an impressive ring of choroidal effusions before developing a classic bilateral appearance. Possible contributions to the initial presentation include choroidal venous congestion in the setting of a borderline short eye and thickened sclerochoroidal complex. [ABSTRACT FROM AUTHOR]

Purpose: To report a rare case of uveal effusion syndrome following COVID-19 vaccination. Observation: A 71-year-old Asian man presented to his ophthalmologist with blurred vision and noticing distorted lines in his left eye two weeks after the first dose of COVID-19 vaccination. Examination revealed choroidal detachment and he was advised systemic corticosteroids. The symptoms were ignored and the second vaccine dose was taken. After five months, he presented to our clinic with persistent visual complaints. He also had a history of COVID-19 infection three months prior to vaccination. Ocular examination revealed a quiet anterior chamber with annular choroidal detachment consistent with the diagnosis of Type 3 uveal effusion syndrome. B-scan ultrasonography revealed increased choroidal thickness with detachment. Optical coherence tomography showed subretinal fluid with retinal pigment epithelium and choroidal folds. Ultrasound biomicroscopy revealed all around supraciliary effusion in the left eye. The patient was treated with oral prednisolone and mycophenolate mofetil which resulted in complete resolution of uveal effusion and improvement in visual acuity. Conclusions and importance: Uveal effusion syndrome is a rare ocular disease, however it may manifest following COVID-19 vaccination. Our case highlights the importance of a complete ophthalmic examination in patients with ocular symptoms after vaccination.

Supplemental Digital Content is Available in the Text. Four eyes (three patients) presented with an acute inferior exudative retinal detachment associated with an annular choroidal detachment mimicking uveal effusion syndrome. They had a history of central serous chorioretinopathy in the same eye or in the fellow eye. The role of choroidal venous congestion and scleral thickening is discussed. Purpose: To describe central serous chorioretinopathy cases presenting as uveal effusion syndrome, providing new insights into "pachychoroid spectrum" diseases. Methods: 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. Results: 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. Conclusion: 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. [ABSTRACT FROM AUTHOR]

Introduction: Uveal effusion syndrome (UES) is a rare ocular disease causing idiopathic uveal effusion, often with associated ciliochoroidal and retinal detachment. UES diagnosis is challenging because of overlapping features with other ocular inflammatory, neoplastic, iatrogenic, and drug-induced causes of uveal effusion. While several successful surgical treatments have been described, such as full-thickness or partial-thickness sclerectomy, medical therapies may also have a therapeutic role. Objective: To provide an updated review of the published literature on the course of the disease, medical and surgical management strategies, as well as newer treatment modalities. [ABSTRACT FROM AUTHOR]

Purpose: To describe the clinical features and treatment outcomes in spontaneous uveal effusion syndrome (UES).Methods: A 10-year retrospective chart review of UES patients from a tertiary eye center was carried out. Optical coherence tomography (OCT), fundus fluorescein angiography, and ultrasound biomicroscopy (UBM) scans were performed. UES was managed based on presenting best-corrected visual acuity (BCVA), symptoms, and fundus findings. Patients with secondary causes of uveal effusion were excluded.Results: Twenty-five eyes of 16 patients were included. Of the 16 patients, 14 (88%) were male and 9 (56%) had bilateral disease. Fifteen of 25 affected eyes had nanophthalmos (axial length (AL) <20.5 mm) and 6 had hyperopia with AL >20.5 mm. The presenting mean distance BCVA was 0.74 ± 0.64 logMAR (mean Snellen: 20/100). Eleven eyes had exudative retinal detachment, and 4 also had exudative choroidal detachment (CD). Choroidal thickness (CT) was increased in 11 eyes on B-scan ultrasonography, and the mean CT was 1.74 ± 0.38 mm. Sub-retinal fluid (SRF) and retinal folds were the most common OCT findings. UBM findings included shallow angles, peripheral CD, and supra-ciliary effusion. A combination of local and systemic corticosteroids was used to successfully treat 12 eyes, 6 needed surgery, and 7 were observed. Partial sclerectomy with anterior chamber maintainer-assisted SRF drainage was the favored surgery. The median period of follow-up was 6.5 months (0.1-76 months), and the mean distance BCVA at the last follow-up was 0.58 ± 0.42 logMAR (mean Snellen: 20/80).Conclusion: UES can be suitably managed both medically and surgically based on clinical presentation. [ABSTRACT FROM AUTHOR]

Abstract Background Uveal effusion syndrome is a rare disease characterized by exudative detachments of the choroid, ciliary body, and retina. Various surgical procedures and nonsurgical strategies have been described to treat uveal effusion syndrome with limited success. The treatment for uveal effusion syndrome remains a serious challenge for clinicians. To the best of our knowledge, no previous report has described a severe uveal effusion syndrome patient with nanophthalmos treated by using an anti-vascular endothelial growth factor agent alone. We report here one such case with unexpected positive results. Case presentation A 30-year-old Chinese male patient presented with painless vision loss in both eyes that had persisted for 2 months. Examination of the right eye revealed a best corrected visual acuity of 0.03; the best corrected visual acuity of the left eye was finger count/20 cm. The intraocular pressure was normal on both eyes. A-scan revealed an right eye axial length of 15.88 mm and a left eye axial length of 16.21 mm. In the right eye, half of the peripheral choroid and nearly three-fourths of the retina were detached. The left fundus was not visible because of the total retinal detachment located just behind the lens, which could be clearly observed directly with a slit lamp. Considering all the possibilities and available treatments as well as the patient’s intentions after discussion, we first administered an intravitreal injection of ranibizumab 0.5 ml into both eyes. The patient’s visual perception improved 3 days after the injection. One month later, most of the effusion under the choroid and retina was absorbed. Visual acuity improved from finger count to 0.05 in both eyes, and vision quality was remarkably improved. Encouraged by this good result, the patient opted to undergo a second injection 1 month later. Choroidal and retinal detachment completely vanished 30 days after the second injection. Conclusions Using an anti-vascular endothelial growth factor agent alone may be a potentially effective and safe method for managing some types of uveal effusion syndrome, such as in nanophthalmos. The injection may be administered before considering more aggressive procedures in some uveal effusion syndrome patients.

Purpose: To report a case of uveal effusion syndrome in association with primary COVID-19 infection to share our experience and insight in diagnosing and managing this unique case., Case Presentation: A 56-year-old woman presented with angle closure glaucoma of both eyes. Further examination and imaging revealed the etiology to be related to bilateral uveal effusions and choroidal thickening in the setting of recent COVID-19 infection. The patient's glaucoma was managed with bilateral iridotomies and medical therapy, while the precipitating uveal effusions resolved with treatment on oral steroids., Conclusion: While uveal effusion syndrome has been associated with COVID-19 vaccination, it has not yet been reported after primary infection. Recognition of this rare phenomenon will allow for better diagnosis and treatment in future cases.

PurposeIn this study, we aimed to investigate clinical characteristics and histopathology and evaluate surgical outcomes of quadrantic lamellar-sclerectomy with sclerostomy for uveal effusion syndrome (UES).DesignRetrospective, cohort study.ParticipantsOverall, 106 eyes of 66 patients diagnosed with UES were treated at the Beijing Tongren Hospital between January 1, 2001 and June 26, 2021.MethodsPatients were examined by routine ophthalmologic examinations, fluorescein and indocyanine green angiography (FFA/ICGA); axial length determination; color Doppler ultrasound (CDU); ultrasound biomicroscopy (UBM), optical coherence tomography (OCT), and optical coherence tomographic angiography (SD/SS-OCTA). Quadrantic lamellar-sclerectomy with sclerostomy was performed at the equator in all patients and histopathological examination of the excised sclera was analyzed in all samples.Main Outcome MeasuresThe reattachment of the choroid and retina with resolution of the serous fluid, best corrected visual acuity (BCVA), choroidal thickness, and recurrence of ciliochoroidal detachment were the main outcome measures.ResultsTwo subgroups were identified: (1) type 1 (nanophthalmic eye), wherein the eyeball was small (average axial length 15.83 ± 1.45 mm) with high hypermetropia (average 12.6 diopters) and (2) type 2 (non-nanophthalmic eye), wherein the eyeball size was normal (average axial length 23.45 ± 1.68 mm) with or without refractive error, combined with or without systemic symptoms. Histopathologically, types 1 and 2 demonstrated similarly abnormal sclera with the disorganization of collagen fiber bundles and deposits of proteoglycans in the matrix. Quadrantic lamellar-sclerectomy with sclerostomy was effective in both types 1 and 2, inducing post-operative resolution of the subretinal fluid accumulation and increasing the useful BCVA. The choroidal thickness was significantly different before and after surgery (P < 0.05). Approximately 98.1% of cases attained permanent reattachment within 6 months after one operation through this procedure. The single operation success rate was 96.2%, and success with one or two operations was 100%.ConclusionsUES is caused by abnormalities of the sclera and increased resistance to transscleral fluid outflow, combined with increased choroidal thickness. Quadrantic lamellar-sclerectomy with sclerostomy is an effective treatment for UES that can rescue correct the useful visual acuity.

Abstract Background To report an unusual case of non-nanophthalmic uveal effusion syndrome (UES) with histologically normal sclera but responsive to scleral resection. Case presentation A73-year-old man presented with a bullous retinal detachment without ciliochoroidal detachment on funduscopic examination of the right eye. The axial length of both eyes was normal. Extensive investigations for possible causes of exudative retinal detachment were performed with unremarkable results except for choroidal hyperpermeability on indocyanine green angiography (ICGA). Ultrasound biomicroscopy (UBM) revealed scleral thickening with peripheral choroidal elevation leading to the diagnosis of UES. Partial thickness sclerectomy and sclerotomy was performed resulting in complete retinal reattachment, reduction of choroidal hyperpermeability on ICGA and improvement of visual acuity. However, histological studies of the excised sclera revealed no scleral architectural changes or abnormal deposits. Conclusions The diagnosis of UES in non-nanophthalmic eyes is challenging. Thorough systemic and ocular investigations are critical to rule out other etiologies. UBM can be helpful to evaluate scleral thickness and anterior choroid in equivocal cases. Our case was unique in that, although the sclera was thick, no abnormal microscopic scleral architecture could be identified. Misdiagnosis may lead to different surgical procedures such as vitrectomy resulting in unfavorable outcomes.

Uveal effusion is a rare disease that is characterized by exudative detachment of the ciliary body and choroid. Herein, we report a rare case of uveal effusion associated with viral encephalitis, which resolved following the treatment of the viral encephalitis and administration of corticosteroids. A 67-year-old man who was hospitalized for viral encephalitis was referred to our clinic. He had been treated for herpes zoster ophthalmicus in his left eye 3 weeks previously. Choroidal detachment and uveal effusion between the ciliary body and sclera were observed. He was prescribed oral and topical steroids and cycloplegics to treat uveal effusion, and an antiviral agent (Acyclovir) to treat viral encephalitis. After 4 weeks, the choroidal detachment resolved completely. Uveal effusion syndrome can develop in association with viral encephalitis and be treated successfully with oral and topical steroids; we suggest that medical treatment should be attempted prior to surgery. ADEM: Acute disseminated encephalomyelitis; BCVA: Best corrected visual acuity; CSF: Cerebrospinal fluid; CT: Computed tomography; MRI: Magnetic resonance imaging; WBC: White blood cell [ABSTRACT FROM AUTHOR]

Objectives: To report visual and anatomical outcomes following two- or four-quadrant partial-thickness sclerectomy and sclerotomy surgery to treat nanophthalmos (NO)-related uveal effusion (UE). Materials and Methods: Consecutive patients with NO-related UE were treated with four-quadrant or two-quadrant (for those with associated glaucoma) partial-thickness sclerectomy and sclerotomy surgery. Axial length, extent of UE, preoperative, postoperative, and final best corrected visual acuity (BCVA), time to retinal reattachment, and rates of retinal reattachment and recurrence were noted. Results: Fourteen eyes of 10 patients with NO-related UE were operated. Retinal detachment (RD) involved mainly the peripheral retina in 7 (50%) eyes, macula in 2 eyes (14.2%), both macula and peripheral retina in 4 eyes (28.6%), and the whole retina in 1 eye. Eleven eyes had four-quadrant surgery, and 3 eyes with associated glaucoma had two-quadrant surgery. External subretinal drainage was performed in one patient who had total RD. The mean preoperative logMAR BCVA of 1.50±0.53 increased significantly to 0.92±0.49 after surgery (p=0.002). Resolution of RD could be achieved with two-quadrant surgery in only 1 of 3 eyes. In the other 2 eyes, retinal reattachment was achieved after a secondary surgery for the remaining two quadrants to complete four-quadrant sclerectomy. Final outcome was total reattachment of the retina in 11 eyes (78.6%), partial reattachment in 1 eye (7.1%), and recurrence of macular detachment in 2 (14.3%) eyes. Conclusion: Quadrantic partial-thickness sclerectomy and sclerotomy surgery seems effective for treating UE in eyes with NO. Twoquadrant surgery may be tried for mild UE associated with glaucoma to preserve the superior quadrants for future possible glaucoma surgeries, but secondary surgery for the superior quadrants may be needed. External drainage of subretinal fluid may be an option in severe cases to achieve quicker resolution. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: Uveal effusion syndrome is a rare disease characterized by exudative detachments of the choroid, ciliary body, and retina. Various surgical procedures and nonsurgical strategies have been described to treat uveal effusion syndrome with limited success. The treatment for uveal effusion syndrome remains a serious challenge for clinicians. To the best of our knowledge, no previous report has described a severe uveal effusion syndrome patient with nanophthalmos treated by using an anti-vascular endothelial growth factor agent alone. We report here one such case with unexpected positive results.Case Presentation: A 30-year-old Chinese male patient presented with painless vision loss in both eyes that had persisted for 2 months. Examination of the right eye revealed a best corrected visual acuity of 0.03; the best corrected visual acuity of the left eye was finger count/20 cm. The intraocular pressure was normal on both eyes. A-scan revealed an right eye axial length of 15.88 mm and a left eye axial length of 16.21 mm. In the right eye, half of the peripheral choroid and nearly three-fourths of the retina were detached. The left fundus was not visible because of the total retinal detachment located just behind the lens, which could be clearly observed directly with a slit lamp. Considering all the possibilities and available treatments as well as the patient's intentions after discussion, we first administered an intravitreal injection of ranibizumab 0.5 ml into both eyes. The patient's visual perception improved 3 days after the injection. One month later, most of the effusion under the choroid and retina was absorbed. Visual acuity improved from finger count to 0.05 in both eyes, and vision quality was remarkably improved. Encouraged by this good result, the patient opted to undergo a second injection 1 month later. Choroidal and retinal detachment completely vanished 30 days after the second injection.Conclusions: Using an anti-vascular endothelial growth factor agent alone may be a potentially effective and safe method for managing some types of uveal effusion syndrome, such as in nanophthalmos. The injection may be administered before considering more aggressive procedures in some uveal effusion syndrome patients. [ABSTRACT FROM AUTHOR]

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Background/Purpose: To describe the use of topical mitomycin-C in sclerostomy revision for recalcitrant idiopathic uveal effusion syndrome. Methods: A 50-year-old healthy man presented with painless, gradual vision loss in the right eye. He underwent multimodal retinal imaging with wide-field fundus photography, spectral domain optical coherence tomography, and B-scan and A-scan ultrasonography. He was found to have idiopathic (non-nanophthalmic) uveal effusion syndrome with choroidal and serous retinal detachments in the right eye and a peripheral choroidal detachment in the left eye. Central vision became threatened in the right eye. Medical treatment with oral corticosteroids and surgical treatment with choroidal drainage through sclerostomies and sclerostomy revision were administered. Results: Initial treatment with systemic corticosteroids was ineffective. Subsequent choroidal drainage through sclerostomies only partially resolved the effusion. Later sclerostomy revision with application of topical mitomycin-C led to complete resolution with anatomical stability maintained after at least 42 months of follow-up. Conclusion: Successful use of topical mitomycin-C in sclerostomy revision has not previously been reported in idiopathic (non-nanophthalmic) uveal effusion syndrome. We propose that topical mitomycin-C may be considered as a potential therapeutic adjunct in the treatment of refractory idiopathic uveal effusion syndrome before further sclerostomy procedures are attempted in additional quadrants of the eye. [ABSTRACT FROM AUTHOR]

Aims: The aim of the study is to describe and evaluate the clinical features, multimodal imaging, differential diagnoses, treatment options, and visual outcomes in patients with diagnosis of uveal effusion syndrome (UES). Materials and Methods: This was a retrospective, observational case series of patients with diagnosis of UES. Variables analyzed included age, gender, medical history, initial and final best-corrected visual acuity (BCVA), clinical features, type of treatment, and follow-up. Results: We report three cases, consisting of 2 males and 1 female, aged 37, 58, and 72 years with the diagnosis of UES. One patient was treated with medical therapy (acetazolamide) and the other two were submited to surgical treatment including one case of two-thirds thickness sclerotomy and one case with subscleral sclerectomy. All cases had complete resolution of subretinal fluid (SRF). BCVA improved in two cases, while there was no improvement of BCVA in one case due to outer retina atrophy as a consequence of chronic SRF. Conclusions: The diagnosis of UES still remains a challenge for a clinician. It is essential to know the clinical features differential diagnosis and be familiarized with the different therapeutic approaches to establish accurate diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Background: To report an unusual case of non-nanophthalmic uveal effusion syndrome (UES) with histologically normal sclera but responsive to scleral resection.Case Presentation: A73-year-old man presented with a bullous retinal detachment without ciliochoroidal detachment on funduscopic examination of the right eye. The axial length of both eyes was normal. Extensive investigations for possible causes of exudative retinal detachment were performed with unremarkable results except for choroidal hyperpermeability on indocyanine green angiography (ICGA). Ultrasound biomicroscopy (UBM) revealed scleral thickening with peripheral choroidal elevation leading to the diagnosis of UES. Partial thickness sclerectomy and sclerotomy was performed resulting in complete retinal reattachment, reduction of choroidal hyperpermeability on ICGA and improvement of visual acuity. However, histological studies of the excised sclera revealed no scleral architectural changes or abnormal deposits.Conclusions: The diagnosis of UES in non-nanophthalmic eyes is challenging. Thorough systemic and ocular investigations are critical to rule out other etiologies. UBM can be helpful to evaluate scleral thickness and anterior choroid in equivocal cases. Our case was unique in that, although the sclera was thick, no abnormal microscopic scleral architecture could be identified. Misdiagnosis may lead to different surgical procedures such as vitrectomy resulting in unfavorable outcomes. [ABSTRACT FROM AUTHOR]

Microphthalmos is a type of developmental disorder ophthalmopathy, which can occur isolated or combined with other ocular malformations and can occur secondary to a systemic syndrome. Nanophthalmos is one of the clinical phenotypes of microphthalmos. Due to the special and complex structure of nanophthalmic eyes, the disorder is often associated with many complications, including high hyperopia, angle-closure glaucoma, and uveal effusion syndrome. The management of these complications is challenging, and conventional therapeutic methods are often ineffective in treating them. The purpose of this paper was to review the concept of nanophthalmos and present the latest progress in the study of the pathogenesis and treatment of its complications. As it is considerably challenging for ophthalmologists to prevent or treat these nanophthalmos complications, timely diagnosis and a suitable clinical treatment plan are vital to ensure that nanophthalmos patients are treated and managed effectively. [ABSTRACT FROM AUTHOR]

Purpose: Uveal effusion syndrome (UES) is a rare eye condition characterized by fluid accumulation in the uveal layer. We investigated its clinical characteristics and treatment modalities and their association with long-term visual outcomes., Methods: This retrospective cohort study included patients with UES treated at two tertiary hospitals between November 2005 and June 2023. Clinical characteristics and treatment outcomes by modality were compared between nanophthalmic Type 1 UES (UES-1) and non-nanophthalmic Type 2 UES (UES-2), and between initial and final visits. Logistic regression analysis was used to identify factors associated with vision loss., Results: Twenty-three eyes were included (UES-1, n = 10; UES-2, n = 13). Retinal pigment epithelium mottling was significantly more common in UES-1 than in UES-2 ( P = 0.043); no other between-group differences were observed. Post-treatment, in UES-1, best-corrected visual acuity ( P = 0.028) and central macular thickness ( P = 0.046) significantly decreased; in UES-2, best-corrected visual acuity significantly improved ( P = 0.021), and subfoveal choroidal thickness ( P = 0.048), central subretinal fluid height ( P = 0.011), and central macular thickness ( P = 0.010) significantly decreased. UES-2 was associated with a lower risk of vision loss (odds ratio, 0.024; P = 0.044). No other associated factors were identified., Conclusion: The UES type was the sole independent prognostic factor for vision loss, whereas treatment modalities had no significant impact on visual outcomes., Competing Interests: None of the authors has any commercial associations that might pose a conflict of interest in connection with the submitted article.

Effective therapeutic options are limited to the management of exudative retinal detachments (RDs). The authors describe the resolution of the case following partial-thickness scleral resection. This 31-year-old male presented with a bilateral exudative RD. There was no history of steroid use either locally or systemically. The fundus eye showed typical of chronic exudative RDs. The axial length was 16.08 mm in the right eye and 15.17 mm in the left eye. Thickening of the sclera was evident in ocular ultrasound. Considering an axial length (nanophthalmic eye), four partial-thickness scleral resections were performed. The exudative RD resolved at 4 months. Partial-thickness scleral resections may be considered as an option for treating chronic exudative RDs patients with nanophthalmic eye. [ABSTRACT FROM AUTHOR]

Purpose: To report the effect of partial thickness sclerectomy in patients with idiopathic uveal effusion syndrome. Study Design: Interventional case series. Place and Duration of Study: Multi-center study at LRBT eye hospital, Medicare hospital and Mayo hospital, Lahore from January 2010 to August2015. Material and Methods: Total six eyes of four patients (two males and two females) with bilateral idiopathic uveal syndrome were included in the study. The diagnosis was clinically confirmed on B scan ultrasonography, which confirmed relatively short axial length, exudative retinal detachment and scleral thickening. The surgical procedure included 360 degrees peritomy, followed by partial thickness sclerotomies measuring 4 x 6 mm placed in between the recti muscles. Results: All six (6) patients showed clinical improvement in visual acuity. Preoperative visual acuity was perception of light (PL) in all cases which improved to 6/36-6/24 at 12 weeks follow up. On fundoscopy and B scan ultrasound there was resolution of exudative retinal detachment after partial thickness sclerectomies in all cases. The intraocular pressure was high (26 mm Hg) in only one case which improved to 18 mm Hg at 12 weeks follow-up. All other cases had IOP within normal limits pre and post operatively. Conclusion: Partial thickness sclerectomy is an effective treatment option for patients of uveal effusion syndrome not responding to medical treatment. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of the study was to investigate the corticosteroids for uveal effusion syndrome (UES). Methods: Retrospective series of 104 eyes with UES treated with oral corticosteroids (OCS), periocular corticosteroids (PCS), topical corticosteroids (TCS), or observation (OBS). Main outcome measure was UES resolution. Results: Of 104 eyes, treatment included OCS (n = 27), PCS (n = 12), TCS (n = 11), and OBS (n = 54). A comparison of the four groups (OCS vs. PCS vs. TCS vs. OBS) revealed differences in those managed with OCS versus OBS as younger (66 vs. 72 years, P = 0.049), PCS versus OBS as male (100% vs. 54%, P = 0.002), PCS versus OBS with decreased visual acuity (VA)/visual field (91% vs. 51%, P = 0.018), and OBS versus OCS as asymptomatic (28% vs. 0%, P = 0.001). Of the 59 with follow-up information, management included OCS (n = 21), PCS (n = 12), TCS (n = 6), and OBS (n = 20). There were differences in initial VA

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Nanophthalmic uveal effusion syndrome (UES) is an extremely rare idiopathic disease characterized by a short axial length of the eye, extremely thick sclera and choroid. These structural changes can lead to spontaneous serous detachment of the retina and peripheral choroid. There are many other causes of UES such as trauma, inflammation, cataract surgery, glaucoma, or retinal detachment. UES is classified into three types. All are characterized by a relapsing-remitting clinical course. The loss of visual acuity ranges from mild to very severe, depending on macular involvement. Changes of the retinal pigment epithelium develop secondary after long-standing choroidal effusion and retinal detachment. Subretinal exudates could be seen and mistakenly diagnosed as chorioretinitis. UES can be very difficult to treat. The most commonly used treatment is surgery involving the creation of surgical sclerostomies (scleral window surgery) or partial thickness sclerectomies to support transscleral drainage. In our case, we present a bilateral nanophthalmic UES, which was misdiagnosed as bilateral ocular Vogt−Koyanagi−Harada disease. We documented the course of the disease and the results of the different surgical approaches in both eyes. A pars plana vitrectomy was performed in the right eye and a sclerectomy with sclerostomies in the left eye. In the left eye, even long lasting loss of visual acuity due to a serous retinal detachment was partially reversed.

The purpose — to evaluate the effectiveness of the posterior sclerectomy with the corneal trepan in a uveal effusion syndrome.Patients and methods. Patient 1. The man, 61 years, complained about a blindness of the right eye and the progressing decrease in vision of the left eye. According to data of examinations the diagnosis was: uveal effusion syndrome, detachment of the choroid, exudative retinal detachment, the complicated cataract of both eyes. Patient 2. The man, 62 years, with complaints to low vision of the right eye and a blindness of the left-hand eye. Diagnosis: uveal effusion syndrome, detachment of the choroid, exudative retinal detachment, the initial complicated cataract of the right eye. Subatrophy operated retinal detachment, complicated cataract, silicone into the vitreal cavity of the left eye. Both patients underwent trepanation posterior sclera. Results. The patient 1noted significant improvement of vision in both eyes at the last examination. Мisual acuity with correction was OD — 0,2, OS — 0.3. Intraocular pressure was normal, improvement of electrical sensitivity and lability was diagnosed. On ultrasound examination of the retina belonged, moderate swelling of the choroid remained. Patient 2. Visual acuity was 0,1 + 3,0 D at the time of the last inspection. According to the ultrasonic B-scan of the right eye retinal detachment decreased to 3,8 mm, moderate swelling of the choroid remained. The OСT has showed detachment of the neuroepithelium in the macula to 60 μm. In ultrasonic biomicroscopy circular, ciliochoroidal detachment to 0,15 mm was diagnised.Conclusion. Described clinical cases confirmed the almost complete recovery of patients with the uveal effusion syndrome. Sclerectomy with the use of corneal trepan is a technically simple procedure and helps to define the path of the outflow suprachoroidal fluid subtenon space.

Nanophthalmos is a clinical spectrum of disorders with a phenotypically small but structurally normal eye. These disorders present significant clinical challenges to ophthalmologists due to a high rate of secondary angle-closure glaucoma, spontaneous choroidal effusions, and perioperative complications with cataract and retinal surgeries. Nanophthalmos may present as a sporadic or familial disorder, with autosomal-dominant or recessive inheritance. To date, five genes (i.e., MFRP, TMEM98, PRSS56, BEST1, and CRB1) and two loci have been implicated in familial forms of nanophthalmos. Here, we review the definition of nanophthalmos, the clinical and pathogenic features of the condition, and the genetics of this disorder. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of the study was to investigate the corticosteroids for uveal effusion syndrome (UES).Methods: Retrospective series of 104 eyes with UES treated with oral corticosteroids (OCS), periocular corticosteroids (PCS), topical corticosteroids (TCS), or observation (OBS). Main outcome measure was UES resolution.Results: Of 104 eyes, treatment included OCS (n = 27), PCS (n = 12), TCS (n = 11), and OBS (n = 54). A comparison of the four groups (OCS vs. PCS vs. TCS vs. OBS) revealed differences in those managed with OCS versus OBS as younger (66 vs. 72 years, P = 0.049), PCS versus OBS as male (100% vs. 54%, P = 0.002), PCS versus OBS with decreased visual acuity (VA)/visual field (91% vs. 51%, P = 0.018), and OBS versus OCS as asymptomatic (28% vs. 0%, P = 0.001). Of the 59 with follow-up information, management included OCS (n = 21), PCS (n = 12), TCS (n = 6), and OBS (n = 20). There were differences in initial VA <20/400 in PCS versus OBS (42% vs. 5%, P = 0.018), effusion thickness in TCS versus OCS (7 vs. 3 mm, P = 0.004), and serous retinal detachment in PCS versus OBS (100% vs. 30%, P < 0.001) and PCS versus OCS (100% vs. 57%, P = 0.012). Regarding outcomes, VA showed less worsening in OCS versus OBS (0% vs. 30%, P = 0.008) and OCS versus PCS (0% vs. 33%, P = 0.012). There was no difference in rate of effusion resolution or effusion recurrence. Overall, using combination of corticosteroid therapies, effusion resolution was achieved in 56/59 (95%) cases and the need for surgical management with scleral windows was necessary in only 3/59 (5%) cases. Complications included cataract (n = 9) and no instance of steroid-induced glaucoma.Conclusion: Management of UES is complex and depends on disease severity. Using various corticosteroid delivery routes, UES control was achieved in 95%, and scleral window surgery was required in only 5%. A trial of corticosteroids can benefit patients with UES. [ABSTRACT FROM AUTHOR]

Background: We report a case of acute uveal effusion during phacoemulsification in an eye with preoperative chronic central serous chorioretinopathy (CSC).Case Presentation: A 55-year-old man with a history of chronic CSC for >18 months presented with bilateral opaque lenses. A preoperative ophthalmic examination showed suspected lenticonus and risky anatomical features, including a thick ciliary body, and anterior rotation of the ciliary process and iris root in both eyes. Optical coherence tomography (OCT) detected CSC in the left eye, but the results of fundus photography and B-scan ultrasonography were unremarkable. The anterior chamber flattened during phacoemulsification. Anterior vitrectomy was quickly performed for suspected infusion misdirection syndrome, and was followed by uneventful surgery. On postoperative day 1, fundus photography, type B ultrasound, and OCT revealed uveal exudation in the macula of the left eye. On postoperative day 50, the patient's visual acuity recovered to 20/32, and fundus photography, ultrasonography, and OCT revealed complete resolution of the uveal effusion.Conclusions: This case suggests an association between preoperative CSC and uveal effusion during surgery, because choroidal hyperperfusion and hyperpermeability were present in the patient's CSC-affected eyes. [ABSTRACT FROM AUTHOR]

A 20-year-old, female Catalina macaw ( Ara ararauna × Ara macao) was presented with bilateral uveitis and hyphema. The hyphema initially improved with 0.12% prednisolone acetate ophthalmic drops (1 drop OU q4h for 7 days), but the hyphema recurred after the drops were tapered. The bird subsequently developed inappetance, weight loss, regurgitation, and lethargy and was euthanatized 24 days after initial presentation. Necropsy revealed marked splenomegaly and hepatomegaly, with significant mucosal ulcerations of the proventriculus and petechiation associated with both kidneys. Histopathologic examination revealed multicentric lymphoma, with neoplastic cells observed in ocular, splenic, hepatic, renal, proventricular, intestinal, pancreatic, and choanal tissue. Neoplastic lymphocytes effaced the iris, ciliary body, and the choroid of the eyes, and neoplastic lymphocytes were attached to the corneal endothelium and infiltrated the sclera, episclera, and conjunctivae. Immunohistochemical results indicated that the neoplastic lymphocytes were CD3+ and CD79a−, which is consistent with T-cell lymphoma. [ABSTRACT FROM AUTHOR]

Purpose: To present challenging cases of vitreoretinal lymphoma (VRL) that was misdiagnosed as uveitis because of the apparent intraocular inflammation. At the light of the new classification of intraocular lymphomas, we detail the characteristics that masqueraded the tumors and the clinical aspects that guided us to the correct diagnosis.Materials and Methods: We retrospectively reviewed the patients referred to our uveitis service between January 2006 and December 2014.Results: Seven patients referred with a presumptive diagnosis of idiopathic uveitis received a final diagnosis of VRL. The median time between the onset of symptoms and definitive diagnosis was 25 months for these complex cases. The median time from presentation at our clinic to final diagnosis was 1 month. The described clinical features including dense vitreous cells and subretinal infiltrates were characteristic and tend to be present in all these chronically ill patients. Vitreous samples were collected, and all demonstrated the pathognomonic tumor cells, the specific immunoglobulin heavy chain gene rearrangements, and an interleukin (IL)-10 to IL-6 ratio >1.Conclusion: VRLs are severe diseases with a poor prognosis that may be misdiagnosed as idiopathic inflammatory conditions of the eye. Treatment with steroids may occult the tumors and delay the correct diagnosis. Appropriate evaluation may prompt to a timely vitreous sampling and therefore to a faster diagnosis in these peculiar cases where the correct diagnosis was delayed by several months. [ABSTRACT FROM AUTHOR]

Purpose: To describe the clinical findings and management of eyes affected by uveal effusion syndrome (UES) presenting with clinical features mimicking inflammatory ocular diseases, treated using individualized surgical approaches. Methods: We report a consecutive interventional case series of seven eyes of four patients affected by UES. On presentation in our clinic, all patients showed signs of steroid effects as a consequence of a presumptive diagnosis; one eye had undergone vitrectomy for retinal detachment (RD), without benefit. Diagnosis of UES was based on ophthalmic examination, ultrasonography, fluorescein angiography, biometry and magnetic resonance imaging. Five eyes with active disease were treated using scleral thinning surgical procedures based on the extent and characteristics of the disease: sclerectomy sites were ultrasound-guided to the area of maximal choroidal swelling, associated with evacuative puncture in the case of bilateral funnel-shaped RD. Results: One patient was diagnosed with type 1 UES, two with type 2, and one with type 3. Mean postoperative follow-up was 26 months. In all eyes, surgery resolved the ciliochoroidal and retinal detachment and improved visual acuity. In two eyes, visual restoration was limited by a prolonged disease course. Conclusion: UES may be mistaken for other sources of ciliochoroidal effusion. Early diagnosis and treatment is critical to avoid unnecessary procedures and to prevent severe visual loss as a result of neuroretinal damage. Surgical treatment based on the extent and characteristics of the disease may be effective for the resolution of ciliochoroidal effusion, even in type 3 UES, where conventional surgery has proved unsuccessful. [ABSTRACT FROM AUTHOR]

Bilateral uveal effusion syndrome associated with bilateral acute retinal necrosis is a diagnostic and therapeutic challenge. A 52 year old man presented with bilateral angle closure with choroidal detachment. With restricted fundus view, parenteral steroid was started. During close follow up bilateral discrete areas of peripheral retinitis were noted. Parenteral steroids were promptly stopped and parenteral antivirals with oral steroids were continued. It showed healing response with nil recurrences till last follow up. Aggressive treatment of bilateral uveal effusion with parenteral steroids can cause progression of bilateral acute retinal necrosis leading to phthisis bulbi. However early diagnosis, prompt intervention and close follow up are the key elements to therapeutic success even during diagnostic surprises and avoid costly mistakes. [ABSTRACT FROM AUTHOR]

Background: The purpose of this study was to report the demographics, disease characteristics, treatments, and visual outcomes of pediatric uveitis patients without juvenile idiopathic arthritis managed in a tertiary medical center. Methods: A retrospective, observational study was performed in pediatric uveitis patients without juvenile idiopathic arthritis and aged 0-18 years, who were seen at the University of Virginia from 1984 to 2014. Results: Thirty-nine pediatric uveitis patients (57 eyes) were identified. The patient population was 51.28% female, 51.28% Caucasian, and 33.33% African American. The mean age at diagnosis was 11.9 years. The mean duration of follow-up was 3.11 years. The mean number of visits to the clinic was 10.41. Of 57 eyes, 31 (54.39%) had anterior uveitis, 12 (21.05%) had intermediate uveitis, nine (15.79%) had posterior uveitis, and five (8.77%) had panuveitis. The leading diagnoses were traumatic uveitis (25.64%), undifferentiated anterior uveitis (17.95%), undifferentiated intermediate uveitis (15.38%), HLA-B27-associated anterior uveitis (7.69%), and herpetic anterior uveitis (7.69%). Systemic associations included sarcoidosis, ulcerative colitis, and psoriatic arthritis (n=3). The most common treatment modalities included local steroids (66.67%), systemic steroids (23.08%), and antimetabolites (20.51%). Ocular hypertension was found in five (12.82%) patients. Ocular surgery was performed in six (15.38%) patients. Mean best-corrected visual acuity (BCVA) at baseline across all anatomical locations was 0.458 logMAR, and was 0.411 logMAR at final follow-up. Mean BCVA improved during follow-up in all but the anterior uveitis group. The mean baseline intraocular pressure was 14.27 mmHg, and was 14.22 mmHg at final follow-up. Conclusion: Uveitis in childhood is a vision-threatening group of inflammatory disorders arising from numerous etiologies that vary geographically and historically. Because of the high burden of disease, the difficulty of making precise etiologic diagnoses, and the complicated management, it is imperative that affected children be referred to and closely monitored by uveitis specialists to prevent devastating ocular damage. This study found that BCVA and intraocular pressure did not vary significantly during follow-up, suggesting that close management by an ophthalmologist may prevent adverse visual outcomes, and highlighted the high prevalence of traumatic uveitis in children, which tends to have good visual outcomes. [ABSTRACT FROM AUTHOR]