Ultrasound examination confirmed thickened sclera, choroidal effusion, and nanophthalmos. Uveal effusion syndrome may be caused by a variety of disease states such as postoperative hypotony, scleral buckling procedures and scleritis. Uveal effusion syndrome ues is a rare clinical entity with the potential to cause devastating visual consequences. Immune checkpoint inhibitors, including antiprogrammed cell death protein1 antipd1 and antiprogrammed cell death ligand1 antipdl1 monoclonal antibodies have emerged as promising immunotherapy for solid cancers. It leads to an abnormal collection of fluid that expands the suprachoroidal space, resulting in an. Uveal effusion syndrome is a rare entity involving the idiopathic collection of serous fluid in the suprachoroidal space. Pregnancyinduced hypertensionrelated chorioretinitis resem. Uveal effusion syndrome usually causes peripheral chorioretinal. Pues is an abnormal leakage of clear fluid under the retina, which causes symptoms of blurred vision, sometimes with distortion of straight lines. Vision can be severely reduced, and both eyes are ultimately involved in a majority of patients. The association of nanophathalmos and spontaneous or. Superimposed aging and hormonal changes in the sclera and its emissary channels impair its permeability to protein and predispose the eye to vortex vein obstruction. The nanophthalmic macula british journal of ophthalmology. Pdf posterior uveal effusion syndrome after trabeculectomy.
Schepens and brockhurst,1 in 1963, used the term uveal effusion syndrome to describe spontaneous bilateral serous detachments of the choroid and ciliary body with exudative retinal detachment, primarily occurring in middle aged healthy males. When choroidal metastasis extends anteriorly, there may be an associated episcleral sentinel vessel yeo et al. After subscleral sclerectomy, intraocular pressure remained stable and the patient. It can be thought that decreasing intraocular pressure and preventing inflammation may decrease the risk of ue. Uveal effusion itself refers to transudation from the choriocapillaris into the suprachoroidal space, which causes subsequent choroidal thickening and detachment. Oct 01, 2010 uveal effusion syndrome ues is an extremely rare but potentially serious condition characterized by choroidal fluid collections, often in association with serous retinal detachment. Both are discussed, as is the rationale behind the current management of this unusual condition. Uveal effusion syndrome ues was first reported by schepens and brockhurst in a seminal paper in 1963 in which they described the clinical. Aims to define an unusual macular appearance found in association with nanophthalmos. Many cases have been reported since then, especially in nanophthalmic eyes. Uveal effusion syndrome ues is an extremely rare but potentially serious condition characterized by choroidal fluid collections, often in association with serous retinal detachment. Examples include trauma, scleritis, pars planitis, and. Serous retinal detachment and suprachoroidal collection of serous fluid may be seen and confirmed with transillumination of the globe or ultrasonography.
It is speculated that compression of the vortex veins may increase. Uveal effusion syndrome ues is a rare condition which usually occurs in hyperopic or nanophthalmic eyes with thicker than normal scleral and choroidal tissue. The surgical management of uveal effusion syndrome eye. Nanophthalmos, uveal effusion syndrome, and acute angle closure glaucoma acg can present as a continuum in a patient, as is described here. The correct diagnosis is important as sclerectomy or sclerotomy may be an effective treatment. They all exhibited the same clinical findings of an unusual yellow macula appearance with retinal folds and crowded optic discs. Nanophthalmic uveal effusion syndrome after prophylactic. Spontaneous exudative detachment of the choroid and ciliary body was first reported by schepens and brockhurst in 1963 1. Visual electrophysiology performed in four cases was normal.
Partial thickness scleral windows and subscleral sclerectomy were performed. Uveal effusion and cancer drugs american academy of. Kadambi and sripriya krishnamoorthy nanophthalmos, a rare condition, is an important cause of secondary angle closure, especially in young adults. Scleritis with uveal effusion from alirocumab annals of. Doctors at the university of michigan kellogg eye center have reported a series of 3 patients who developed uveal effusion syndrome following treatment with immune checkpoint inhibitors. It is by definition idiopathic but can be associated with nanophthalmia or hypermetropia in some cases. Uveal effusion after immune checkpoint inhibitor therapy. Bscan echography helps to differentiate choroidal effusions from retinal detachments.
Mar 10, 2020 access to paid content on this site is currently suspended due to excessive activity being detected from your ip address 207. The onset is spontaneous and the progression of the disease is slow. Jun 06, 2014 nanophthalmic uveal effusion syndrome can be associated with reduced scleral permeability to albumin, and a very high concentration of retained suprachoroidal albumin. On echography, effusions are notable for their anterior angle and extension to the ora serrata. Nanophthalmos, uveal effusion syndrome, diffuse retinal pigment epitheliopathy, sclerectomy background exudative retinal detachment rd is a rare complication of chronic central serous chorioretinopathy cscr, or diffuse retinal pigment epitheliopathy, drpe 1, 2 and is difficult to treat. Posterior uveal effusion syndrome following ectopia lentis. The patient returned 2 years postoperatively with the same presentation in the contralateral eye. In patients with uveal effusion syndrome or the closely related condition of nanophthalmos, abnormal sclera, referred to here as scleropathy, is the most likely primary ocular anomaly affecting choroidal fluid dynamics. To our knowledge, case 1 is the first case in the ophthalmic literature describing myopic shift without glaucoma. Uveal effusion syndrome an overview sciencedirect topics. Uveal effusion syndrome ues is a rare disease manifested by suprachoroidal fluid accumulation with consecutive choroidal and retinal detachment. Deepen your understanding of uveal effusion syndrome.
Uveal effusion syndrome department of ophthalmology. The retina is a thin layer of delicate tissue in the back of your eye, which lines. Spectrum of angle closure, uveal effusion syndrome, and. The thickened sclera impairs diffusion of fluid from the suprachoroidal space. Case report fullthickness sclerotomy for uveal effusion. Exudative retinal detachments may be associated with metastasis shields et al. Posterior uveal effusion syndrome following ectopia lentis surgery in a case with marfans syndrome pakistan journal of ophthalmology vol. The response to surgery in these eyes suggests that the approach is justified and provides further evidence that a scleral abnormality is the underlying cause of uveal effusion syndrome. This patients angle closure was thought to be caused by idiopathic uveal effusion syndrome, and while there. Here we highlight the meticulous planning that is imperative to an uncomplicated outcome. Uveal effusion syndrome ues is a rare idiopathic cause of choroidal detachment that may be associated with nanophthalmos and increased scleral thickness. Retina today management of uveal effusion syndrome. Diagnosis of ues is often delayed due to an overlap in clinical findings with posterior uveitis and intraocular lymphoma. Controlled and repeated sessions of transscleral cyclophotocoagulation may be considered as a viable management option in these cases.
The history and clinical findings are presented of a patient who suffered from the uveal effusion syndrome over a 10year period from 1956. The funduscopic appearance is illustrated both at the time of initial presentation and 36 years later. Choroidal effusions are diagnosed clinically and usually appear elevated in a fourlobed presentation because of firm attachments of the choroid to the vortex veins. Uveal effusion syndrome is a rare disease that largely affects middleaged male subjects.
Uveal effusion may be idiopathic or might develop from trauma, drugs, or inflammatory conditions. Posterior uveal effusion syndrome after trabeculectomy in an. Uveal effusion syndrome and hypotony maculopathy clinical gate. Transscleral cyclophotocoagulation as primary management. Results seven children aged 8 months to 17 years with nanophthalmos were examined. Jan 03, 2015 what is posterior uveal effusion syndrome pues. In one case, prompt discontinuation of topiramate prevented bilateral angle closure glaucoma. Uveal effusion syndrome is a rare condition characterized by idiopathic spontaneous serous detachment of the retina and peripheral choroid, suggesting a primary scleral abnormality. Uveal effusion syndrome ues is a rare, distinct primary abnormality of the choroid or sclera. This patients angle closure was thought to be caused by idiopathic uveal effusion syndrome, and while there are no generally accepted diagnosis criteria for nanophthalmos, our patient fulfilled the criteria as defined by wu. Nov 09, 2011 choroidal findings in idiopathic uveal effusion syndrome tomomi harada, shigeki machida, takamistu fujiwara, yasunori nishida, dajiro kurosakadepartment of ophthalmology, iwate medical university school of medicine, iwate, japanpurpose. Case report castlemans disease presenting with uveal.
In this setting, patients are thought to have a distinct, primary abnormality of the choroid or sclera, called uveal effusion syndrome ues. Case report castlemans disease presenting with uveal effusion syndrome sang hoon park, su jeong song department of ophthalmology, kangbuk samsung hospital, s ungkyunkwan university school of medicine, seoul, korea we report a rare case of multicentric castlemans disease that presented with ophthalmic involvement, along with a. In summary, ciliochoroidal effusion is a rare occurrence, and its presentation as an acute angle closure attack is even less uncommon. Express shunt for choroidal fluid drainage in uveal effusion. We report choroidal findings by means of enhanced depth imaging spectraldomain optical coherence tomography edioct in a patient with idiopathic uveal. In patients with uveal effusion syndrome or the closely related condition of nanophthalmos, abnormal sclera, referred to here as scleropathy, is the most likely primary ocular anomaly affecting choroidal fluid dynamics 16. Circumscribed ciliochoroidal effusion presenting as an. Uveal effusion syndrome uveal effusion syndrome elagouz, mohammed. Choroidal findings in idiopathic uveal effusion syndrome. We report two cases of topiramate induced uveal effusions. A novel technique for choroidal fluid drainage in uveal. It was characterized by the accumulation of fluid escaped from the choriocapillaries into nearby free space, resulting from compressed vortex veins due to a thickened sclera. The management of nanophthalmic eyes can be quite challenging due to the risk of inciting uveal effusion syndrome with any form of intraocular surgery.
Nanophthalmic uveal effusion syndrome after prophylactic laser treatment introduction nanophthalmos is a rare bilateral condition characterized by short axial length, diffuse choroidal thickening, severe hyperopia and a tendency towards angleclosure glaucoma and uveal effusion. Nanophthalmos preparing for the challenge sujatha v. Uveal effusion syndrome is a rare disease which belongs to the differential diagnosis of choroidal tumour with serous retinal detachment. Uveal effusion was first described in a nanophthalmic patient by schepens and brockhurst in 1963 1. A case of atypical idiopathic choroidal effusion syndrome. A fundus photograph shows peripheral choroidal detachment and macular folds. Uveal effusion syndrome caused by choroidal invasion of. Pdf purposeto report a case series of three patients with bilateral uveal effusion syndrome ues, treated conservatively with oral carbonic anhydrase. The onset is spontaneous and the progression of the disease is slow, often leading to bilateral serous choroidal detachment and concomitant retinal detachment, shifting subretinal fluid and, in severe cases, total loss of visual acuity. Uveal effusion syndrome is a rare syndrome of idiopathic exudative detachments of choroid, ciliary body and retina, thought to arise from. Nanophthalmic uveal effusion syndrome can be associated with reduced scleral permeability to albumin, and a very high concentration of retained suprachoroidal albumin.
Uveal effusion syndrome, survey of ophthalmology 10. This clinical case is the third case reported on choroidal effusion presenting as an acute angle closure attack and the second one associated with an idiosyncratic reaction to arb losartan. These detachments are accompanied by shifting patterns of subretinal. Express shunt for choroidal fluid drainage in uveal. Posterior uveal effusion syndrome after trabeculectomy in. Uveal effusion, also referred to as choroidal detachment, can occur secondary to a number of ocular conditions that result in inflammatory or hydrostatic changes, including scleritis, posterior uveitis, and hypotony after glaucoma surgery. It is used as a secondline treatment for high cholesterol levels in adults whose. Alirocumab is a human monoclonal antibody that belongs to a novel class of drugs known as proprotein convertase subtilisinkexin type 9 inhibitors that increase the expression of lowdensity lipoprotein receptor and decrease levels of lowdensity lipoprotein cholesterol 1. Including the three other cases in the ophthalmic literature which reported angle closure glaucoma, 3, 4 all were women between ages 34 and 53. Choroidal findings in idiopathic uveal effusion syndrome opth. Uveal effusions with ciliary body swelling cause forward rotation of the lensiris diaphragm, causing myopia and angle closure glaucoma.
Case report fullthickness sclerotomy for uveal effusion syndrome. The pathogenetic mechanism behind the development of the uveal effusion syndrome is believed to be a thickened sclera resulting from the accumulation of. Uveal effusion syndrome has been recognized to be an abnormal condition, pooling fluid in the suprachoroidal space. Uveal effusion syndrome ues was reported by schepens and brockhurst in 1963, 1 and later described as a nanophthalmic disorder with a scleral abnormality. Topiramate induced myopic shift and angle closure glaucoma. Posterior uveal effusion syndrome after trabeculectomy in an eye with ocular venous congestion. If your access is via an institutional subscription, please contact your librarian to request reinstatement. Gass and jallow 2 described a similar syndrome in 1982, idiopathic uveal effusion syndrome, in healthy middleaged men with normal eyes 2. This condition frequently presents as central serous retinopathy. After subscleral sclerectomy, intraocular pressure remained stable and the patient recovered premorbid vision. There are many causes of uveal effusion, often occurring in association with hypotony or inflam mation.
Choroidal effusions american academy of ophthalmology. Pregnancyinduced hypertensionrelated chorioretinitis. Two separate hypotheses have been postulated to explain the pathogenesis of the uveal effusion syndrome, one relating to abnormally thickened sclera, the other to chronic bulbar hypotony. Uveal effusion syndrome is a rare, ocular disorder that typically affects middleage men, and is characterized by idiopathic serous detachment of choroid, ciliary body, and retina. Almost two decades later, gass and jallow in 1982 coined the term idiopathic uveal effusion syndrome to describe idiopathic serous detachment of the. Cataract surgery in a nanophthalmic eye is challenging.
407 1189 1397 335 556 986 1297 1270 303 632 1440 362 19 1016 1194 621 1554 137 1440 500 1076 301 340 1204 199 17 1103 1251 1024 548 1035 1361