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. 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. 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. We suggest that scleral thickening caused obstruction of vortex veins followed by uveal effusion and compression of posterior ciliary arteries within their intrascleral tract, leading to aion. Clinical data of patients with uveal effusion syndrome ues mri scan. Uveal effusion syndrome is a rare disorder characterized by the accumulation of serous transudate in the suprauveal space resulting in ciliochoroidal thickening, ciliochoroidal detachment, and serous nonrhegmatogenous retinal detachment, especially in middleaged men.
Spectrum of angle closure, uveal effusion syndrome, and. Pues is an abnormal leakage of clear fluid under the retina, which causes symptoms of blurred vision, sometimes with distortion of straight lines. Evidence that abnormal sclera is responsible for the uveal effusion syndrome. Sometimes, its cause is obviously related to an underlying general or cardiac disease, or to a syndrome of inflammatory or infectious acute pericarditis. The history and clinical findings are presented of a patient who suffered from the uveal effusion syndrome over a 10. Bap1 cancer syndrome is a rare inherited condition in which family members are at increased risk for uveal eye melanoma, as well as melanoma of the skin, malignant mesothelioma, kidney cancer and others. Uveal melanoma research and treatment at mayo clinic. C bscan ultrasonography imaging of the bullous serous choroidal. 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.
Pericardial effusion is a common finding in everyday practice. Uveal effusion syndrome ues is a rare condition which usually occurs in hyperopic or nanophthalmic eyes with thicker than normal scleral and choroidal tissue. Uveal effusion syndrome complicated by anterior ischemic. The vascular middle layer of the eye constituting the iris, ciliary body, and choroid. Uveal effusion syndrome ues is a rare disease of idiopathic exudative. The retina is a thin layer of delicate tissue in the back of your eye, which lines. Medical therapy for bilateral uveal effusion syndrome in. Uveal effusion syndrome is a rare disease that largely affects middleaged male subjects. Retina today management of uveal effusion syndrome.
Bmode ocular ultrasound of the posterior sclera is also helpful. Aims to define an unusual macular appearance found in association with nanophthalmos. This condition is caused by an inherited mutation change in the bap1 gene and tends to form aggressive cancers that appear at younger ages. We report choroidal findings by means of enhanced depth imaging spectraldomain optical coherence tomography edioct in a patient with idiopathic uveal. 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. Scleral hydraulic conductivity and macromolecular diffusion in patients with uveal effusion syndrome.
The surgical management of uveal effusion syndrome article pdf available in eye 1 pt 11. A novel technique for choroidal fluid drainage in uveal. 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. Uveal effusion syndrome an overview sciencedirect topics.
Choroidal effusions american academy of ophthalmology. Fullthickness sclerotomy for uveal effusion syndrome. Uveal effusion was first described in a nanophthalmic patient by schepens and brockhurst in 1963 1. Uveal effusion syndrome, survey of ophthalmology 10. Diffuse uveal melanocytic proliferation is a rare paraneoplastic syndrome resulting in rapid bilateral visual loss due to proliferation of benign melanocytes within the choroid and ciliary body. It is speculated that compression of the vortex veins may increase. They all exhibited the same clinical findings of an unusual yellow macula appearance with retinal folds and crowded optic discs. Many cases have been reported since then, especially in nanophthalmic eyes.
Uveal effusion syndrome ues is a rare disease manifested by suprachoroidal fluid accumulation. Nanophthalmos, sclerotomy, uveal effusion syndrome. Serous retinal detachment and suprachoroidal collection of serous fluid may be seen and confirmed with transillumination of the globe or ultrasonography. Pdf medical therapy for uveal effusion syndrome researchgate. Pregnancyinduced hypertensionrelated chorioretinitis resem. The patient refused surgical treatment because he already had terminal cancer. When choroidal metastasis extends anteriorly, there may be an associated episcleral sentinel vessel yeo et al. Posterior uveal effusion syndrome following ectopia lentis surgery in a case with marfans syndrome pakistan journal of ophthalmology vol. It consists of the iris, ciliary body, and choroid, and forms the pigmented layer. Uveal effusion syndrome is primarily a clinical diagnosis, but it is important to measure refraction and axial length.
On other occasions, pericardial effusion is an unexpected finding that requires specific evaluation. Uveal effusion after immune checkpoint inhibitor therapy. The funduscopic appearance is illustrated both at the time of initial presentation and 36 years later. Pdf the surgical management of uveal effusion syndrome. Uveal effusion syndrome stelmach 1994 australian and. Most of the previously reported cases have been seen with bilateral involvement and typical ocular features. Posterior uveal effusion syndrome following ectopia lentis. It is known that idiopathic uveal effusion has a poorer prognosis than other types, and that surgery or systemic steroid therapy is not effective. Of 104 eyes, treatment included ocs n 27, pcs n 12. Uveal melanoma research and treatment at mayo clinic mayo clinic in rochester, minnesota, has a long history in the treatment of uveal melanomas. These detachments are accompanied by shifting patterns of subretinal.
In 1986, oum9 reported subscleral sclerectomy in a case of uveal effusion syndrome in korea. In these cases, the main issues are aetiology, the clinical. All patients were free from recurrence during the followup period. Uveal effusion syndrome ues is a rare, distinct primary abnormality of the choroid or sclera. The pathogenetic mechanism behind the development of the uveal effusion syndrome is believed to be a thickened sclera resulting from the accumulation of. The nanophthalmic macula british journal of ophthalmology. Deepen your understanding of uveal effusion syndrome. 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. It leads to an abnormal collection of fluid that expands the suprachoroidal space, resulting in an. Nevertheless it cant be excluded that aion was the. Posterior uveal effusion syndrome after trabeculectomy in an eye with ocular venous congestion. To our knowledge such an association hasnt been previously described.
Uveal effusion syndrome ues was reported by schepens and brockhurst in 1963, 1 and later described as a nanophthalmic disorder with a scleral abnormality. A new hypothesis concerning pathogenesis and technique of surgical treatment. Uveal effusion syndrome is a rare entity involving the idiopathic collection of serous fluid in the suprachoroidal space. Choroidal findings in idiopathic uveal effusion syndrome opth. To report a case series of three patients with bilateral uveal effusion syndrome ues, treated conservatively with oral carbonic anhydrase inhibitors and topical prostaglandin analogues pas. Choroidal retinopathy is rare but extremely destructive to visual function. Abnormal scleral findings in uveal effusion syndrome. Jan 03, 2015 what is posterior uveal effusion syndrome pues. Uveal effusion syndrome is a rare syndrome of idiopathic exudative.
The thickened sclera impairs diffusion of fluid from the suprachoroidal space. A case of atypical idiopathic choroidal effusion syndrome. In the early 1980s, dennis robertson, md, was one of the first to use iodine125 plaques, the form of brachytherapy most commonly used for treatment of uveal melanomas in the united states today. Mar 01, 2010 uveal effusion syndrome uveal effusion syndrome elagouz, mohammed. To establish the diagnostic role of imaging by ultrasound for the diagnosis of uveal effusion syndrome.
Jackson tl, hussain a, morley ams, sullivan pm, hodgetts a, elosta a, et al. Exudative retinal detachments may be associated with metastasis shields et al. This patients angle closure was thought to be caused by idiopathic uveal effusion syndrome, and while there. Scleritis with uveal effusion from alirocumab annals of. Uveal effusion syndrome uveal effusion syndrome elagouz, mohammed. Almost two decades later, gass and jallow in 1982 coined the term idiopathic uveal effusion syndrome to describe idiopathic serous detachment of the. 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. Case report fullthickness sclerotomy for uveal effusion syndrome. Effective treatment often involves sclerotomy, quadrantic sclerectomy, andor decompression of vortex veins. 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 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 and hypotony maculopathy clinical gate. Nanophthalmic uveal effusion managed with scleral windows and. However, this may arise from other intraocular surgeries and a number of conditions, including inflammatory and infectious diseases, trauma, neoplasms, drug reactions, and venous congestion. Resistance to steroid treatment25 and surgical intervention28 in a number of. Spontaneous resolution of uveal effusion simulating choroidal. For the treatment of ues, brockhurst described good surgical results.
Fullthickness sclerotomy for uveal effusion syndrome ncbi. Express shunt for choroidal fluid drainage in uveal effusion. Uveal effusion syndrome is a rare disease which belongs to the differential diagnosis of choroidal tumour with serous retinal detachment. 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 was characterized by the accumulation of fluid escaped from the choriocapillaries into nearby free space, resulting from. 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. Risk factors for eye cancer american cancer society. Choroidal effusionan abnormal accumulation of fluid in the suprachoroidal spaceis a common complication of glaucoma surgery. We report on a case of idiopathic uveal effusion syndrome complicated by aion. Nanophthalmic uveal effusion syndrome can be associated with reduced scleral permeability to albumin, and a very high concentration of retained suprachoroidal albumin. 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. Nanophthalmos, uveal effusion syndrome, and acute angle closure glaucoma acg can present as a continuum in a patient, as is described here. Uveal effusion may be idiopathic or might develop from trauma, drugs, or inflammatory conditions. 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.
Nanophthalmos is a cause of refractory uveal effusions. It can be thought that decreasing intraocular pressure and preventing inflammation may decrease the risk of ue. It is used as a secondline treatment for high cholesterol levels in adults whose. Jun 06, 2014 recognition of this syndrome is important because. Circumscribed ciliochoroidal effusion presenting as an acute. Comment on idiopathic uveal effusion syndrome causing unilateral acute angle closure in a pseudophakic patient h r atta 1 eye volume 26, page 750 2012 cite this article.
It was characterized by the accumulation of fluid escaped from the choriocapillaries into nearby free. We reported uveal effusion and transient myopia as the initial presentation of systemic lupus erythematosus with pulmonary arterial hypertension. Case report fullthickness sclerotomy for uveal effusion. Uveal effusion syndrome ues was first reported by schepens and. Spontaneous exudative detachment of the choroid and ciliary body was first reported by schepens and brockhurst in 1963 1. Uveal effusion and transient myopia as the initial. Scleral thickening is well described, especially in nanophthalmic ues, but it can also occur in idiopathic ues. Uveal effusion syndrome is a rare disease that largely affects middleaged male. Due to the idiopathic nature, the diagnosis is made after excluding other causes of effusion such as inflammation or infection, neoplasm, or trauma. Almost two decades later, gass and jallow in 1982 coined the term idiopathic uveal effusion syndrome to describe idiopathic serous detachment of the choroid, ciliary body, and retina. In summary, ciliochoroidal effusion is a rare occurrence, and its presentation as an acute angle closure attack is even less uncommon. Posterior uveal effusion syndrome after trabeculectomy in.
Express shunt for choroidal fluid drainage in uveal. Uveal effusion syndrome has been recognized to be an abnormal condition, pooling fluid in the suprachoroidal space. Condition exudative or transudative clinical clues. Pregnancyinduced hypertensionrelated chorioretinitis. A case of hunter syndrome with bilateral retinal detachment eye.
Posterior uveal effusion syndrome after trabeculectomy in an. Uveal effusion syndrome usually causes peripheral chorioretinal detachment, but posterior. A fundus photograph shows peripheral choroidal detachment and macular folds. Two cases of uveal effusion syndrome semantic scholar. An effusion is exudative if it meets any of the following three criteria. Figure 5 redfree fundus photograph of the left eye showing. It is by definition idiopathic but can be associated with nanophthalmia or hypermetropia in some cases. Results seven children aged 8 months to 17 years with nanophthalmos were examined. Visual electrophysiology performed in four cases was normal. In 2001, banta and colleagues reported a case of uveal effusion and acute bilateral secondary angleclosure glaucoma associated with topiramate use that has been well documented in many cases thereafter. The correct diagnosis is important as sclerectomy or sclerotomy may be an effective treatment. 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.1039 179 673 200 314 337 898 243 935 758 1236 120 1274 576 707 169 321 1101 1457 907 1008 411 624 532 678 735 959 1038 1065 1407 1179 1171 1254 395 778 1320 125 894 1131 1119 1174 1409 1420 1274 163 1447 1124 472 814