Long Way To Success: A Case Report Of Uveitis Glaucoma Hyphema Plus Syndrome In A Young Female Patient

Uveitis Glaucoma Hyphema Plus (UGH+) Syndrome is a rare and complex condition that involves the coexistence of uveitis, glaucoma, hyphema and additionally in this particular variant vitreous hemorrhage often leading to challenging diagnostic and management scenarios. The fundamental step in the pathogenesis of UGH syndrome appears to arise from repetitive mechanical iris trauma by malpositioned or subluxed IOL. Most commonly associated with anterior chamber intraocular lenses or sulcus implanted posterior chamber intraocular lenses. Herein we would like to underline how challenging could be the diagnosis and management of aforementioned condition

This case was managed at a tertiary referral ophthalmology hospital SPKSO in Warsaw. The clinic is equipped with advanced diagnostic tools, such as ultrasound biomicroscopy and offers a range of surgical and laser treatments. The patient’s care involved a collaborative approach between specialists in glaucoma, uveitis, and vitreoretinal diseases. The management of this case involved a combination of medical therapy, surgical interventions, and constant patient's monitoring. 

A 42-year-old female presented with left ocular pain and decreased vision. On examination, scattered blood in the anterior chamber, small nongranulomatous keratoprecipitates, iridodonesis and ectopic pupils were observed. Markedly elevated intraocular pressure (IOP) (~60 mmHg) in the left eye(OS) was measured, despite a full medical regimen (topical latanoprost, timolol, dorzolamide, and brimonidine, and oral dorzolamide 2x/day).The patient’s history reported cataract surgery with PCIOL implantation OU in 2015, history of recurrent anterior uveitis of unknown etiology in OS since 2022 and a vitreous hemorrhage in OS (08/2024). An emergency trabeculectomy was performed (09/2024). One month following the surgery(10/2024), a fresh hemorrhage into both vitreous cavity and anterior chamber was noticed. Various additional procedures (YAG suturolisis, needling) were unsuccessful to maintain stable IOP. Due to persistent hipertonia and recurrent hemorrhages, decided to proceed with a revision of the trabeculectomy(12/2024), with no clinical success. Pars plana vitrectomy (PPV) was performed(01/2025), preceded by intravitreal bevacizumab injection. Ultrasound biomicroscopy (UBM) revealedevidence of peripheral iridocorneal contact (chafing). Diagnosis of Uveitis Glaucoma Hyphema (UGH) syndrome was established. The malpositioned PCIOL was removed (01/2025). One month after surgery, IOP was normalized, and no further hemorrhages were observed. The patient remains under ongoing care.

This case highlights the challenges in diagnosing and managing Uveitis Glaucoma Hyphema (UGH) syndrome. Despite initial treatment attempts, the presence of a malpositioned PCIOL contributed to persistent intraocular pressure elevation and recurrent hemorrhages. Surgical intervention, including trabeculectomy, vitrectomy, and PCIOL removal, ultimately led to the normalization of intraocular pressure and resolution of recurrent hemorrhages. This case underscores the importance of considering UGH syndrome in pseudophakic patients with elevated IOP, uveitis, recurrent hyphema and vitreous hemorrhages, and the need for a multidisciplinary approach to achieve the best possible outcomes.