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. Uveal effusion syndrome ues is a rare disease manifested by suprachoroidal fluid accumulation with consecutive choroidal and retinal detachment. Pregnancyinduced hypertensionrelated chorioretinitis resem. On echography, effusions are notable for their anterior angle and extension to the ora serrata. We report two cases of topiramate induced uveal effusions. 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.
Jan 03, 2015 what is posterior uveal effusion syndrome pues. 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. Both are discussed, as is the rationale behind the current management of this unusual condition. Uveal effusion syndrome is a rare disease that largely affects middleaged male subjects. Choroidal findings in idiopathic uveal effusion syndrome opth. The onset is spontaneous and the progression of the disease is slow. The association of nanophathalmos and spontaneous or. 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. The thickened sclera impairs diffusion of fluid from the suprachoroidal space. Visual electrophysiology performed in four cases was normal. Here we highlight the meticulous planning that is imperative to an uncomplicated outcome.
Spectrum of angle closure, uveal effusion syndrome, and. A novel technique for choroidal fluid drainage in uveal. Uveal effusion after immune checkpoint inhibitor therapy. Uveal effusion syndrome ues was first reported by schepens and brockhurst in a seminal paper in 1963 in which they described the clinical. Uveal effusion may be idiopathic or might develop from trauma, drugs, or inflammatory conditions. 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. In one case, prompt discontinuation of topiramate prevented bilateral angle closure glaucoma. 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 usually causes peripheral chorioretinal. Uveal effusion syndrome is a rare entity involving the idiopathic collection of serous fluid in the suprachoroidal space. After subscleral sclerectomy, intraocular pressure remained stable and the patient recovered premorbid vision. We report choroidal findings by means of enhanced depth imaging spectraldomain optical coherence tomography edioct in a patient with idiopathic 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. If your access is via an institutional subscription, please contact your librarian to request reinstatement. The patient returned 2 years postoperatively with the same presentation in the contralateral eye. Bscan echography helps to differentiate choroidal effusions from retinal detachments. Partial thickness scleral windows and subscleral sclerectomy were performed. To our knowledge, case 1 is the first case in the ophthalmic literature describing myopic shift without glaucoma. Uveal effusion syndrome caused by choroidal invasion of. Results seven children aged 8 months to 17 years with nanophthalmos were examined.
Choroidal effusions are diagnosed clinically and usually appear elevated in a fourlobed presentation because of firm attachments of the choroid to the vortex veins. There are many causes of uveal effusion, often occurring in association with hypotony or inflam mation. 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. Ultrasound examination confirmed thickened sclera, choroidal effusion, and nanophthalmos. This patients angle closure was thought to be caused by idiopathic uveal effusion syndrome, and while there. It is used as a secondline treatment for high cholesterol levels in adults whose. These detachments are accompanied by shifting patterns of subretinal. Uveal effusion was first described in a nanophthalmic patient by schepens and brockhurst in 1963 1. Uveal effusion syndrome ues was reported by schepens and brockhurst in 1963, 1 and later described as a nanophthalmic disorder with a scleral abnormality.
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. Vision can be severely reduced, and both eyes are ultimately involved in a majority of patients. Nanophthalmic uveal effusion syndrome after prophylactic. May 15, 2018 uveal effusion syndrome ues is a rare clinical entity with the potential to cause devastating visual consequences. In this setting, patients are thought to have a distinct, primary abnormality of the choroid or sclera, called uveal effusion syndrome ues. Deepen your understanding of uveal effusion syndrome. Nanophthalmic uveal effusion syndrome can be associated with reduced scleral permeability to albumin, and a very high concentration of retained suprachoroidal albumin.
Controlled and repeated sessions of transscleral cyclophotocoagulation may be considered as a viable management option in these cases. Uveal effusion syndrome is a rare syndrome of idiopathic exudative detachments of choroid, ciliary body and retina, thought to arise from. Uveal effusion syndrome ues is an extremely rare but potentially serious condition characterized by choroidal fluid collections, often in association with serous retinal detachment. Uveal effusion syndrome uveal effusion syndrome elagouz, mohammed. 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. Retina today management of uveal effusion syndrome. Uveal effusion syndrome an overview sciencedirect topics. Posterior uveal effusion syndrome after trabeculectomy in an eye with ocular venous congestion. Express shunt for choroidal fluid drainage in uveal. It is by definition idiopathic but can be associated with nanophthalmia or hypermetropia in some cases. 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. Cataract surgery in a nanophthalmic eye is challenging. The history and clinical findings are presented of a patient who suffered from the uveal effusion syndrome over a 10year period from 1956.
Choroidal effusions american academy of ophthalmology. Mar 10, 2020 access to paid content on this site is currently suspended due to excessive activity being detected from your ip address 207. Uveal effusion syndrome ues is a rare idiopathic cause of choroidal detachment that may be associated with nanophthalmos and increased scleral thickness. Case report fullthickness sclerotomy for uveal effusion syndrome. Transscleral cyclophotocoagulation as primary management. Posterior uveal effusion syndrome following ectopia lentis. 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. Uveal effusion syndrome has been recognized to be an abnormal condition, pooling fluid in the suprachoroidal space. 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, distinct primary abnormality of the choroid or sclera. Nanophthalmos, uveal effusion syndrome, and acute angle closure glaucoma acg can present as a continuum in a patient, as is described here. Posterior uveal effusion syndrome after trabeculectomy in. It is speculated that compression of the vortex veins may increase.
The surgical management of uveal effusion syndrome eye. Uveal effusion syndrome ues is a rare clinical entity with the potential to cause devastating visual consequences. 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. After subscleral sclerectomy, intraocular pressure remained stable and the patient. Topiramate induced myopic shift and angle closure glaucoma. Choroidal findings in idiopathic uveal effusion syndrome. A fundus photograph shows peripheral choroidal detachment and macular folds. Aims to define an unusual macular appearance found in association with nanophthalmos. Uveal effusion syndrome, survey of ophthalmology 10. Many cases have been reported since then, especially in nanophthalmic eyes.
Exudative retinal detachments may be associated with metastasis shields et al. Posterior uveal effusion syndrome after trabeculectomy in an. Pdf posterior uveal effusion syndrome after trabeculectomy. They all exhibited the same clinical findings of an unusual yellow macula appearance with retinal folds and crowded optic discs.
Case report castlemans disease presenting with uveal. The funduscopic appearance is illustrated both at the time of initial presentation and 36 years later. Gass and jallow 2 described a similar syndrome in 1982, idiopathic uveal effusion syndrome, in healthy middleaged men with normal eyes 2. 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. C bscan ultrasonography imaging of the bullous serous choroidal. Uveal effusion syndrome and hypotony maculopathy clinical gate. Including the three other cases in the ophthalmic literature which reported angle closure glaucoma, 3, 4 all were women between ages 34 and 53. The management of nanophthalmic eyes can be quite challenging due to the risk of inciting uveal effusion syndrome with any form of intraocular surgery. It can be thought that decreasing intraocular pressure and preventing inflammation may decrease the risk of ue. Uveal effusions with ciliary body swelling cause forward rotation of the lensiris diaphragm, causing myopia and angle closure glaucoma.
Nanophthalmos preparing for the challenge sujatha v. Examples include trauma, scleritis, pars planitis, and. Kadambi and sripriya krishnamoorthy nanophthalmos, a rare condition, is an important cause of secondary angle closure, especially in young adults. The retina is a thin layer of delicate tissue in the back of your eye, which lines. 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. 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.
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. Pues is an abnormal leakage of clear fluid under the retina, which causes symptoms of blurred vision, sometimes with distortion of straight lines. Uveal effusion and cancer drugs american academy of. Uveal effusion syndrome ues is a rare condition which usually occurs in hyperopic or nanophthalmic eyes with thicker than normal scleral and choroidal tissue. Almost two decades later, gass and jallow in 1982 coined the term idiopathic uveal effusion syndrome to describe idiopathic serous detachment of the. In summary, ciliochoroidal effusion is a rare occurrence, and its presentation as an acute angle closure attack is even less uncommon. 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. Serous retinal detachment and suprachoroidal collection of serous fluid may be seen and confirmed with transillumination of the globe or ultrasonography. Uveal effusion syndrome department of ophthalmology. Circumscribed ciliochoroidal effusion presenting as an.
Uveal effusion itself refers to transudation from the choriocapillaris into the suprachoroidal space, which causes subsequent choroidal thickening and detachment. When choroidal metastasis extends anteriorly, there may be an associated episcleral sentinel vessel yeo et al. It leads to an abnormal collection of fluid that expands the suprachoroidal space, resulting in an. Pdf purposeto report a case series of three patients with bilateral uveal effusion syndrome ues, treated conservatively with oral carbonic anhydrase. Pregnancyinduced hypertensionrelated chorioretinitis. 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. Uveal effusion syndrome is a rare disease which belongs to the differential diagnosis of choroidal tumour with serous retinal detachment. 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. Scleritis with uveal effusion from alirocumab annals of. It is hypothesized that the primary underlying cause of the idiopathic uveal effusion syndrome is a congenital anomaly of the sclera, and in some cases, the vortex veins.
The correct diagnosis is important as sclerectomy or sclerotomy may be an effective treatment. Diagnosis of ues is often delayed due to an overlap in clinical findings with posterior uveitis and intraocular lymphoma. 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. Uveal effusion syndrome is a rare condition characterized by idiopathic spontaneous serous detachment of the retina and peripheral choroid, suggesting a primary scleral abnormality. 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 fullthickness sclerotomy for uveal effusion. This condition frequently presents as central serous retinopathy.
739 1551 706 666 1495 705 149 220 1555 1341 1249 228 118 123 1611 1255 1571 309 412 1504 20 90 1333 530 186 863 1029 543 1355 1343 1308 966 759 1377 513 1498 209 1043 425 508 622 521 651 1107 268 1413