A new hypothesis concerning pathogenesis and technique of surgical treatment. Uveal effusion syndrome, survey of ophthalmology 10. Uveal effusion syndrome is a rare disease that largely affects middleaged male. Uveal effusion syndrome ues is an extremely rare but potentially serious condition characterized by choroidal fluid collections, often in association with serous retinal detachment.
Redfree wideangle imaging confirmed choroidal folds for 360 degrees, suggestive of effusion. Choroidal effusions american academy of ophthalmology. Icsc has been reported in the hallermanstreiff syndrome but not in kennycaffey syndrome. The terms uveal effusion, choroidal effusion, ciliochoroidal effusion. Fullthickness sclerotomy without sclerectomy was performed on five eyes of four patients with ues with or without nanophthalmos. This condition frequently presents as central serous retinopathy. Spectrum of angle closure, uveal effusion syndrome, and.
Chest xray is the first test done to confirm the presence of pleural fluid. In this report we present a chronic phase of cml case that was treated with nilotinib due to imatinib gleevec allergy and had pleural effusion with nilotinib at 5th year of treatment. Uveal effusion syndrome and hypotony maculopathy clinical gate. Oct 09, 2015 ubm can detect very small effusions over ciliary body without clinically detectable choroidal detachmentciliary body is detached at scleral spur in uveal effusion syndrome angiography may demonstrate a leopardskin appearance of hyperfluorescence and hypofluorescence oct may show focal thickening of the retinal pigment. This overlap can lead to a uveal melanoma diagnosis as these can be characteristics of both. Further articles were identified from the reference lists of retrieved articles, and from relevant textbooks. Ct images demonstrate a left choroidal effusion this case was donated to by. Both are discussed, as is the rationale behind the current management of this unusual condition. On echography, effusions are notable for their anterior angle and extension to the ora serrata. Primary uveal effusion may also occur with nanophthalmos or scleral abnormalities.
Uveal effusion syndrome uveal effusion syndrome ues may be idiopathic or associated with abnormalities of choroid or sclera. Uveal effusion syndrome is a rare syndrome of idiopathic exudative. To the authors knowledge, this is the first case of isolated posterior choroidal effusion that has been described in the literature. Nanophthalmos, uveal effusion syndrome, and acute angle closure glaucoma acg can present as a continuum in a patient, as is described here. 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. This can include the iris iritis or the iris and the ciliary body iridocyclitis. 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. Uveal effusion syndrome ues is a rare idiopathic cause of choroidal.
Id just feel so alone prior to joining, so opening up to the people in your same shoes and. Wills eye manual pages 301 350 text version fliphtml5. Resistance to steroid treatment25 and surgical intervention28 in a number of. Previously, it was suggested that vortex vein compression by the thick sclera resulted in such detachments in nanophthalmic eyes, which resolved after vortex vein decompression 8. Blunting of the lateral costophrenic angle usually requires about 175 ml but may take as much as 500 ml. Uveal effusion syndrome may be caused by a variety of disease states such as postoperative hypotony, scleral buckling procedures and scleritis.
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 retina is a thin layer of delicate tissue in the back of your eye, which lines. In patients with uveal effusion syndrome or the closely related condition of nanophthalmos. Nanophthalmic eyes are typically deep set, associated with narrow palpebral fissures,3 7 17 and are markedly hypermetropic2 4 68 10 with axial lengths less than 20 mm.
Jan 03, 2015 what is posterior uveal effusion syndrome pues. Uveal effusion syndrome is a rare syndrome of idiopathic exudative detachments of choroid, ciliary body and retina, thought to arise from impaired posterior segment drainage usually associated with scleral thickening. Idiopathic uveal effusion syndrome ues is another important cause of exudative retinal and ciliochoroidal detachment, which may be associated with nanophthalmos. Uveal effusion syndrome ues is a rare syndrome causing idiopathic exudative detachments of the choroid, ciliary body, and retina.
Uveal effusion syndrome usually manifests with striking clinical features of nontraumatic, painless ciliochoroidal elevation in 1 to 4 quadrants simulating an intraocular mass and is often associated with shallow serous retinal detachment. In crystal growth, a knudsen cell is an effusion evaporator source for relatively low partial pressure elementary sources e. A scanning electron microscopic study of corrosion casts. Uveal effusion syndrome is a rare entity involving the idiopathic collection of serous fluid in the suprachoroidal space. The lateral upright chest xray should be examined when a pleural effusion is suspected. Choroidal effusions are diagnosed clinically and usually appear elevated in a fourlobed presentation because of firm attachments of the choroid to the vortex veins. In 1983, jmd gass 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 1. The procedure consists of placing contraction burns long duration, low power, and large spot size in the extreme. It is the most common type of uveitis and the most painful. The surgical management of uveal effusion syndrome eye. Department of ophthalmology loyola university chicago nanophthalmos small organized eye, with essential normal structure bilateral inherited condition, usually autosomal recessive. Choroidal effusion is commonly misdiagnosed as choroidal melanoma. Scleral hydraulic conductivity and macromolecular diffusion in patients with uveal effusion syndrome.
Express shunt implantation without vortex vein decompression or scleral windows is an effective surgical option for the management of significant uveal effusion syndrome. Nanophthalmic uveal effusion managed with scleral windows. Online survey managing secondary angleclosure glaucomas. The margins of the detachment are sloping and merge gradually into the attached retina. Knudsen effusion cells, kcell, effusion cell for mbe riber.
Pleural effusion develops because of excessive filtration or defective absorption of accumulated fluid. Riber provides a wide range of molecular beam epitaxy mbe knudsen cells, also called kcells or effusion cells, including single or dual filament. If pleural effusion develops in patients taking nilotinib and. Retina today differentiation of choroidal effusion from. To establish the diagnostic role of imaging by ultrasound for the diagnosis of uveal effusion syndrome. Deepen your understanding of uveal effusion syndrome. Uveal effusion syndrome is a rare disease which belongs to the differential diagnosis of choroidal tumour with serous retinal detachment.
Uveal effusion syndrome ues is a rare disorder associated with thickening of the choroid and secondary retinal detachment as a possible serious complication. Knudsen cells are designed for an operating temperature up to 1200c, and outgassing sequences at 1400c. Choroidal findings in idiopathic uveal effusion syndrome opth. Uveal effusion syndrome ues is a rare idiopathic cause of choroidal detachment that may be associated with nanophthalmos and increased scleral thickness. Gass and jallow 2 described a similar syndrome in 1982, idiopathic uveal effusion syndrome, in healthy middleaged men with normal eyes 2. The surgery resulted in complete resolution of the choroidal detachment and exudative retinal detachment of the left eye. 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 ues is a rare, distinct primary abnormality of the choroid or sclera.
Uveitis is classified according to the part of the uveal tract that the inflammation affects. In this retrospective case series, yepez and arevalo present 3 eyes of 2 patients with uveal effusion syndrome without coexisting nanophthalmos type 2 who underwent implantation of an express shunt alcon laboratories. 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. Acute uveal effusion during phacoemulsification with. 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. Uveal effusion was first described in a nanophthalmic patient by schepens and brockhurst in 1963 1. Pregnancyinduced hypertensionrelated chorioretinitis. The presence of uveal effusion in hunters syndrome, in which the sclera has been histologically demonstrated to be abnormally thickened, supports the recently proposed pathophysiology of the uveal effusion syndrome. An increased risk of uveal melanoma has been found in those with blue, green or gray iris color. 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.
It is basically a diagnosis of exclusion when all other causes are ruled out. Posterior uveal effusion syndrome after trabeculectomy in. The vortex veins demonstrated a marked constriction before leaving the eye. Pleural effusion, as a side effect of tyrosine kinases, may be seen as most commonly associated with dasatinib and very rarely seen with nilotinib. The most common causes of transudative pleural effusion in the united states are heart failure and cirrhosis. It is hypothesized to be caused by impaired vascular drainage usually associated with scleral thickening 1. Acute uveal effusion during phacoemulsification with preoperative central serous chorioretinopathy. Because it is easy to control the temperature of the evaporating material in knudsen cells, they are commonly used in molecularbeam epitaxy. Bscan echography helps to differentiate choroidal effusions from retinal detachments. 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 four of the eyes, exudative retinal detachment associated with ues resolved after the sclerotomy. Vortex vein decompression for nanophthalmic uveal effusion. Download pdf sir, we read with great interest andrijevic derk et al 1 manuscript on medical therapy for uveal effusion syndrome ues and wish to add our suggestions whether surgical intervention.
To be certain that there was no intraocular tumor, mri scan was obtained and showed 360 degrees effusion with no. Uveal effusion syndrome was described for the first time in 1963 by rj brockhurst. The authors treated a 38yearold man with nanophthalmic uveal effusion with scleral windows and local application of mitomycinc. Although uveal effusion involving the posterior pole has been described in the past,2 it was seen in association with peripheral effusion, presumably as a posterior extension of subretinal fluid. Fluctuation theorem for the effusion of an ideal gas. Pdf spectrum of angle closure, uveal effusion syndrome, and. Many cases have been reported since then, especially in nanophthalmic eyes. Anterior uveitis is the term for inflammation which affects the eyes front anterior part of the uveal tract. In all of the eyes, exudative choroidal retinal detachment associated with uveal effusion syndrome resolved shortly after the device implantation. Spontaneous resolution of uveal effusion simulating.
Two cases of uveal effusion syndrome jong hyun lee, md, jin young choi, md, sung soo kim, md the institute of vision research, department of ophthalmology, yonsei university college of medicine, seoul, korea purpose. Kawai department of physics, university of alabama at birmingham, birmingham, alabama 35294, usa. Pdf the surgical management of uveal effusion syndrome. Histological studies have demonstrated disruption of collagen. The correct diagnosis is important as sclerectomy or sclerotomy may be an effective treatment. Two cases of uveal effusion syndrome semantic scholar.
To report the surgical outcome of fullthickness sclerotomy in five cases of uveal effusion syndrome ues. It leads to an abnormal collection of fluid that expands the suprachoroidal space, resulting in an. In the absence of prior cns disease, biopsy remains the gold standard. In 1986, oum 9 reported subscleral sclerectomy in a case of uveal effusion syndrome in korea. In 1996, it was approved by the food and drug administration for the treatment of. Fluctuation theorem for the effusion of an ideal gas b. Bilateral uveal effusion and angleclosure 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. We report choroidal findings by means of enhanced depth imaging spectraldomain optical coherence tomography edioct in a patient with idiopathic uveal. Axial length measurement by partial coherence interferometry optical low coherence re. Bupropion hydrochloride, an aminoketone antidepressant, is a dopamine reuptake inhibitor with norepinephrine and nicotinic acetylcholine receptor antagonist actions. 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. 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.
The idiopathic uveal effusion syndrome is characterized by a spontaneous serous detachment of the choroid and ciliary body. There were 2 distinct venous drainage systems in the horse choroid. Ophthalmic surgery, lasers and imaging retina abstract. Uveal melanomas are the most common primary intraocular tumor in adults. Nephrotic syndrome, leading to the loss of large amounts of albumin in urine and resultant low albumin levels in the blood and reduced colloid osmotic pressure, is another less common cause of pleural effusion. Effusion cells, valved cracker cells and gas injectors riber. Case report fullthickness sclerotomy for uveal effusion. Idiopathic uveal effusion syndrome iues is a rare disease characterized by.
Pathophysiology is related to a relative scleral impermeability to protein. Two cases of uveal effusion syndrome pubmed central pmc. To report a case of uveal effusion syndrome associated with hypotony and a case of uveal effusion syndrome in nanophthalmos. A medline database search was conducted using the following key words and mesh headings. The surgical management of uveal effusion syndrome article pdf available in eye 1 pt 11. Four quadrant sclerotomies for uveal effusion syndrome eye. Choroidal findings in idiopathic uveal effusion syndrome. This may have implications for treatment, as the normalsized, normal. About 12% of uveal melanoma arise from the ciliary body. In an upright xray, 75 ml of fluid blunts the posterior costophrenic angle. Fullthickness sclerotomy for uveal effusion syndrome. Uveal effusion syndrome ues is a rare idiopathic cause of choroidal detachment that.
The bundles of collagen fibers in the sclera had a markedly irregular arrangement and varied in width, and abnormal deposition of glycosaminoglycan among the collagen bundles was noted. Pdf nanophthalmos, uveal effusion syndrome, and acute angle closure glaucoma acg can present as a continuum in a patient, as is. Serous retinal detachment and suprachoroidal collection of serous fluid may be seen and confirmed with transillumination of the globe or ultrasonography. Uveal effusion syndrome is a rare disease that largely affects middleaged male subjects. A novel technique for choroidal fluid drainage in uveal. Ultrasound showed uveal effusion with a single area of a solid appearing mass. Oct 01, 2010 deepen your understanding of uveal effusion syndrome difficult to diagnose, this sightthreatening condition can be treated with surgery. 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. Apr 30, 2018 pleural effusion, which in pediatric patients most commonly results from an infection, is an abnormal collection of fluid in the pleural space. More than any other component, effusion cells and sources play a key role in the quality of materials grown by mbe i.
Spontaneous resolution of uveal effusion simulating choroidal. The nanophthalmic macula british journal of ophthalmology. Three patients with bilateral ues were treated with the same. Uveal effusion syndrome an overview sciencedirect topics. The vortex vein ampulla was flattened and showed a slitlike lumen at the merge site with the ophthalmic vein. The vascular middle layer of the eye constituting the. Quadrantic vortex vein decompression with subretinal fluid. Vision can be severely reduced, and both eyes are ultimately involved in a majority of patients. Associated with the mucopolysaccharidoses, fetal alcohol syndrome, myotonic dystrophy, and achondroplasia visual.
Express shunt for choroidal fluid drainage in uveal effusion. Mar 01, 2010 uveal effusion syndrome uveal effusion syndrome elagouz, mohammed. 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. Pues is an abnormal leakage of clear fluid under the retina, which causes symptoms of blurred vision, sometimes with distortion of straight lines.
Jackson tl, hussain a, morley ams, sullivan pm, hodgetts a, elosta a, et al. Case report fullthickness sclerotomy for uveal effusion syndrome. Uveal effusion syndrome usually causes peripheral chorioretinal detachment, but posterior. Bilateral, multiple choroidal effusions after vomiting eye. Uveal effusion and cancer drugs american academy of.
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