Rapid resolution of severe exudation in uveal effusion syndrome with anti-vascular endothelial growth factor alone in a case of bilateral nanophthalmos: a case report

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.

Multimodal imaging to aid in diagnosis of uveal effusion syndrome type 3

A 47-year-old man presented to our retina clinic with gradual onset diminution of vision in his right eye for the last 3 months. Anterior segment evaluation was normal in both eyes. Fundus evaluation showed the presence of leopard spot appearance in the right eye with inferior exudative retinal detachment. Multimodal imaging was done to evaluate retinal pigment epithelium function, choroidal thickness, choroidal vascularity and scleral thickness. Multimodal imaging showed increased choroidal thickness, choroidal congestion on indocyanine green angiography and peripheral choroidal detachment with normal sclera thickness in our patient, thus pointing towards a diagnosis of uveal effusion syndrome type 3.

Partial Thickness Sclerectomy for Uveal Effusion Syndrome in nanophthalmic eyes

Introduction: Nanophthalmos characterized by short axial length, high lens-to-eye ratio and thick sclera, is more prone to develop uveal effusion syndrome (UES). This rare entity can result in idiopathic exudative detachment of the choroid, ciliary body and retina. Abnormality in the scleral thickness with resultant obstruction of the vortex veins and reduced trans-scleral drainage of fluid is responsible for exudative retinal detachment (ERD). Methods: A retrospective study of UES in nanophthalmic patients treated with partial thickness sclerectomy in tertiary eye care centre from January 2015 to June 2019. Five eyes of five patients (four males and one female) with a diagnosis of nanophthalmos suffered from angle closure glaucoma associated with ERD. Raised intra-ocular pressure (IOP) not amenable to conservative medical management were subjected to surgery. Lamellar sclerectomy was performed in two or more quadrants without drainage which was judged on the basis of maximum amount of exudative fluid present in the subsequent quadrants. Results: The average age at surgery was 39.2 years and the mean follow-up duration was 9.2 months (6 to 18 months). Revision sclerectomy was required in 2/5 (60%) eyes. The serous fluid gradually resolved and retina remained reattached at the end of final follow up. The useful vision was preserved and IOP was normalized. Conclusion: Nanophthalmic UES remains a challenging clinical entity. Partial thickness sclerectomy may be an effective option in the treatment of nanophthalmic UES, not amenable to the conventional medical management in a low resource setup.

Comparing the Results of Vitrectomy and Sclerectomy in a Patient with Nanophthalmic Uveal Effusion Syndrome

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.

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

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.

Uveal effusion syndrome in combination with mature cataract and secondaryangle closure

Актуальность. Синдром увеальной эффузии(УЭС) - редкое клиническое состояние, включающее в себя спонтанную цилиохориоидальную отслойку и серозную отслойку сетчатки у пациентов с неотягощенным анамнезом. Несмотря на характерный симптомокомплекс, постановка данного диагноза нередко затруднена. Цель. Анализ клинического случая УЭС. Материал и методы. Пациент Г., 46 лет. При обращении: острота зрения OS – 0,01 н/к, ВГД –16 мм рт.ст. ПЗО OS - 21,56 мм. При биомикроскопии – мелкая передняя камера, диффузно мутный хрусталик. По данным офтальмосонографии и УБМ – субтотальная цилиохориоидальная отслойка и закрытие угла передней камеры. Выставлендиагноз: зрелая катаракта, осевая гиперметропия, синдром увеальной эффузии OS. Выполнена факоэмульсификация катаракты с имплантациейзаднекамерной ИОЛ и одномоментной задней трепанацией склеры. Результаты. В послеоперационном периоде: зрение OS – 0,5 c cyl. – 1,5 дптр = 0,9. ВГД – 16 ммрт.ст. Цилиохориоидальная отслойка не определялась. ОКТ макулярной зоны OS: выявлена обширная отслойка нейроэпителия, что подтвердило диагноз УЭС. Выводы. В результате проведенного лечения достигнута высокая острота зрения и восстановление нормальных анатомо – функциональных взаимоотношений между структурами переднего и заднего отрезков глаза. Закрытие УПК может быть объяснено смещением иридохрусталиковой диафрагмы кпереди на фоне скопления жидкости в супрахориоидальном пространстве.