A prospective, population-based, surveillance (BOSU) study of uveal effusion syndrome in the UK

2021 ◽  
pp. 112067212096936
Author(s):  
Rohit Sharma ◽  
Barny Foot ◽  
Timothy L Jackson

Purpose: To determine the incidence and demographic profile of uveal effusion syndrome (UES), and to describe the visual and anatomic outcome following deep sclerectomy or vortex vein decompression. Methods: The British Ophthalmological Surveillance Unit (BOSU) mails reporting cards monthly to 1149 senior UK ophthalmologists, who are requested to report incident cases of specified rare diseases. UES was included in the reporting system from October 2009 to October 2011. If UES was identified, ophthalmologists were mailed a questionnaire to collect anonymized clinical data at baseline, and 12 months after. Results: Over 2 years, 29 cases were reported. Two cases were duplicates and 12 failed to meet the eligibility criteria. Of the 15 eligible cases, age ranged from 11 to 91 years (mean 62) and nine were males (60%). Ten patients were hypermetropic; three had an axial length of 19.0 mm or less. Estimated annual incidence was 1.2 per 10 million population. Seven cases were managed nonsurgically, including observation (one case), topical steroids (two cases), systemic steroids (three cases), and cyclodiode laser (one case). Eight cases (11 eyes) underwent full-thickness sclerectomy; the elevated flap was retained in four. The sclera was noted to be thick and rigid during surgery in five cases. Median preoperative visual acuity was 6/18, changing slightly to 6/21.5 at final review, with three eyes showing complete anatomic response, five showing some improvement, and three failing to respond. Conclusions: UES is extremely rare. It occurs in a range of ages, but is most common in middle-aged, hypermetropic men. Visual acuity can be materially reduced. The most commonly used surgical treatment in the UK is deep sclerectomy.

Crisis ◽  
2014 ◽  
Vol 35 (4) ◽  
pp. 268-272
Author(s):  
Sean Cross ◽  
Dinesh Bhugra ◽  
Paul I. Dargan ◽  
David M. Wood ◽  
Shaun L. Greene ◽  
...  

Background: Self-poisoning (overdose) is the commonest form of self-harm cases presenting to acute secondary care services in the UK, where there has been limited investigation of self-harm in black and minority ethnic communities. London has the UK’s most ethnically diverse areas but presents challenges in resident-based data collection due to the large number of hospitals. Aims: To investigate the rates and characteristics of self-poisoning presentations in two central London boroughs. Method: All incident cases of self-poisoning presentations of residents of Lambeth and Southwark were identified over a 12-month period through comprehensive acute and mental health trust data collection systems at multiple hospitals. Analysis was done using STATA 12.1. Results: A rate of 121.4/100,000 was recorded across a population of more than half a million residents. Women exceeded men in all measured ethnic groups. Black women presented 1.5 times more than white women. Gender ratios within ethnicities were marked. Among those aged younger than 24 years, black women were almost 7 times more likely to present than black men were. Conclusion: Self-poisoning is the commonest form of self-harm presentation to UK hospitals but population-based rates are rare. These results have implications for formulating and managing risk in clinical services for both minority ethnic women and men.


2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Anadi Khatri ◽  
Sweta Singh ◽  
Kriti Joshi ◽  
Muna Kharel

Abstract Background Uveal effusion syndrome is a rare entity of idiopathic exudative detachments of uveal tissues and retina. Medical treatments with systemic steroids and antimetabolites have been tried but with variable results. Scleral windows or vortex decompressions are usually performed and surgeons usually perform partial sclerectomy in all the quadrants. Case presentation For the first time, we report 2 cases of nanophthalmic uveal effusion syndrome managed with our technique. Conclusion Quadrantic vortex vein decompression with external drainage for nanophthalmic uveal effusion can provide immediate and stable gain in vision.


Medicina ◽  
2021 ◽  
Vol 57 (2) ◽  
pp. 120
Author(s):  
Ivajlo Popov ◽  
Veronika Popova ◽  
Vladimir Krasnik

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.


2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Li Song ◽  
Fangtian Dong ◽  
Changxian Yi

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.


2020 ◽  
Vol 35 (4) ◽  
Author(s):  
Sidrah Riaz ◽  
Muhammad Tariq Khan ◽  
Khalid Mehmood

LONG TERM FOLLOW UP OF CASES OF UVEAL EFFUSION SYNDROME TREATED WITH PARTIAL THICKNESS SCLERECTOMIESPurposeTo report our experience with partial thickness sclerectomy in patients diagnosed as idiopathic uveal effusion syndrome.Study Design: prospective quasi experimentalPlace and duration of study: multicentre study, duration of study is five yearsMaterial and methodsA prospective study conducted in multi centre hospitals including LRBT eye hospital, Medicare hospital and Myo hospital, Lahore, over the period of five years from January 2010 to august 2015.Total six eyes of four patients (two males and two females) with bilateral idiopathic uveal syndrome were included in study. The diagnosis was clinical confirmed on B scan ultrasonography which confirmed relatively short axial length, exudative retinal detachment and scleral thickening. ResultsAll six (6) patients showed improvement clinically in visual acuity and in fundoscopy in term of retinal reattachment on B scan after partial thickness sclerectomies. ConclusionPartial thickness sclerectomy is treatment of choice for patients of uveal effusion syndrome. It didn’t respond to medical treatment. Key wordsUveal effusion syndrome, quadrantic sclerectomy, exudative retinal detachment (RD), intraocular pressure (IOP), visual acuity (VA), light perception (PL). There is no financial interest of authors.


Eye ◽  
2021 ◽  
Author(s):  
Sana Hamid ◽  
Parul Desai ◽  
Pirro Hysi ◽  
Jennifer M. Burr ◽  
Anthony P. Khawaja

AbstractEffective population screening for glaucoma would enable earlier diagnosis and prevention of irreversible vision loss. The UK National Screening Committee (NSC) recently published a review that examined the viability, effectiveness and appropriateness of a population-based screening programme for primary open-angle glaucoma (POAG). In our article, we summarise the results of the review and discuss some future directions that may enable effective population screening for glaucoma in the future. Two key questions were addressed by the UK NSC review; is there a valid, accurate screening test for POAG, and does evidence exist that screening reduces morbidity from POAG compared with standard care. Six new studies were identified since the previous 2015 review. The review concluded that screening for glaucoma in adults is not recommended because there is no clear evidence for a sufficiently accurate screening test or for better outcomes with screening compared to current care. The next UK NSC review is due to be conducted in 2023. One challenge for POAG screening is that the relatively low disease prevalence results in too many false-positive referrals, even with an accurate test. In the future, targeted screening of a population subset with a higher prevalence of glaucoma may be effective. Recent developments in POAG polygenic risk prediction and deep learning image analysis offer potential avenues to identifying glaucoma-enriched sub-populations. Until such time, opportunistic case finding through General Ophthalmic Services remains the primary route for identification of glaucoma in the UK and greater public awareness of the service would be of benefit.


BMJ ◽  
2021 ◽  
pp. n214
Author(s):  
Weedon MN ◽  
Jackson L ◽  
Harrison JW ◽  
Ruth KS ◽  
Tyrrell J ◽  
...  

Abstract Objective To determine whether the sensitivity and specificity of SNP chips are adequate for detecting rare pathogenic variants in a clinically unselected population. Design Retrospective, population based diagnostic evaluation. Participants 49 908 people recruited to the UK Biobank with SNP chip and next generation sequencing data, and an additional 21 people who purchased consumer genetic tests and shared their data online via the Personal Genome Project. Main outcome measures Genotyping (that is, identification of the correct DNA base at a specific genomic location) using SNP chips versus sequencing, with results split by frequency of that genotype in the population. Rare pathogenic variants in the BRCA1 and BRCA2 genes were selected as an exemplar for detailed analysis of clinically actionable variants in the UK Biobank, and BRCA related cancers (breast, ovarian, prostate, and pancreatic) were assessed in participants through use of cancer registry data. Results Overall, genotyping using SNP chips performed well compared with sequencing; sensitivity, specificity, positive predictive value, and negative predictive value were all above 99% for 108 574 common variants directly genotyped on the SNP chips and sequenced in the UK Biobank. However, the likelihood of a true positive result decreased dramatically with decreasing variant frequency; for variants that are very rare in the population, with a frequency below 0.001% in UK Biobank, the positive predictive value was very low and only 16% of 4757 heterozygous genotypes from the SNP chips were confirmed with sequencing data. Results were similar for SNP chip data from the Personal Genome Project, and 20/21 individuals analysed had at least one false positive rare pathogenic variant that had been incorrectly genotyped. For pathogenic variants in the BRCA1 and BRCA2 genes, which are individually very rare, the overall performance metrics for the SNP chips versus sequencing in the UK Biobank were: sensitivity 34.6%, specificity 98.3%, positive predictive value 4.2%, and negative predictive value 99.9%. Rates of BRCA related cancers in UK Biobank participants with a positive SNP chip result were similar to those for age matched controls (odds ratio 1.31, 95% confidence interval 0.99 to 1.71) because the vast majority of variants were false positives, whereas sequence positive participants had a significantly increased risk (odds ratio 4.05, 2.72 to 6.03). Conclusions SNP chips are extremely unreliable for genotyping very rare pathogenic variants and should not be used to guide health decisions without validation.


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