First Author: P.Sakemi Fukuhara BRAZIL
Co Author(s): J. Reis Guimaraes T. Aihara R. Pereira de Almeida Manzano
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To report the importance of regular monitoring of a patient with Uveal Effusion Syndrome its clinical evolution for posterior treatment and discuss the best surgical technique in these cases when disease progresses.
The patient followed in Ophthalmology Department of Irmandade Santa Casa de Misericórdia de São Paulo (ISCMSP) – São Paulo, Brazil
Case report of a male, 31 years old, Caucasian, with low visual acuity in right eye (OD) for 3 months. Associated with these symptoms, the patient had nanophthalmos and high hypermetropia. The best correct visual acuity (BCVA) was 20/200 in OD (+17,00) and 20/40 (+15,00) in left eye (OS). At the slit-lamp examination, there was a shallow anterior chamber in both eyes (OU). The fundoscopy showed serous retinal detachment in OU and choroidal detachment in OU, more evident in OD. Intraocular pressure (IOP) was 18mmHg in OD and 12mmHg in OS. Afterwards, retinography (TRC-NW8F, Topcon), B-scan ultrasonography, which demonstrated scleral thickening, and optical coherence tomography (OCT) were performed for the documentation and follow-up of the case. According to clinical characteristics and ophthalmic examination, Uveal Effusion Syndrome was confirmed. The patient was periodically evaluated every fortnight and it was decided to do prednisone 50mg/day (1mg/kg/day) in regression every 10 days. He evolved with slight improvement in OU after 2 months, and then we decided to perform a surgical procedure.
Uveal Effusion Syndrome causes an abnormal accumulation of fluid in the suprachoroidal space and it is a rare condition usually considered a diagnosis of exclusion. In this case,the patient had all the characteristics to confirm the diagnosis according to the pathogenesis of the disease that includes: vortex vein compression, reduced scleral protein permeability, reduced scleral hydraulic conductivity, chronic hypotony and increased choroidal permeability. He had Uveal Effusion Syndrome type 1, found in nanophthalmic patients. Then, subscleral sclerectomy and sclerotomy were performed at the inferonasal and inferotemporal quadrants to relieve the vortex vein area and help the drainage of suprachoroidal fluid. Two-thirds of thickness scleral flap measuring 4mm x 5mm were made. Under the scleral flap, the remaining thickness of the sclera was excised in pieces measuring 3mm x 4mm,and the choroid was exposed. The scleral flap at inferonasal quadrant was loosely sutured, but Tenon’s capsule and the bulbar conjunctiva were closed tightly. And a full-thickness sclerectomy was performed at the inferotemporal quadrant, removing the scleral flap (region that choroidal detachment was more evident), and Tenon’s capsule and the bulbar conjunctiva were closed too. After the surgery, the patient evolved with a slight improvement as seen at the fundoscopy exam, but the visual acuity remained poor. The outpatient follow-up is being done until the present day.
Patients with Uveal Effusion Syndrome usually present low visual acuity, high hypermetropia, functional alterations due to exudation, choroidal detachment and thickening. A regular monitoring to follow them up is required because, in case the disease gets worse, it will be possible to perform a surgical intervention to relieve the areas of the vortex vein and try to improve the clinical condition. In this particular case, we used two different techniques in the same surgery: we maintained the scleral flap of the sclerotomy of the inferonasal quadrant and removed the scleral flap from the inferotemporal quadrant. A large number of surgical techniques have been described, ranging from the decompression of vortex veins to different sclerectomy and sclerotomy techniques, such as the full-thickness sclerotomy. The best technique at the moment has not been proven and the surgeon should carefully evaluate each case to indicate the best procedure. Further research is required to help determine a better treatment option for each patient