Hermoso-Fernández et al. present a curious case study of retinal detachment in the rarely-encountered UGH syndrome in a 2021 paper.*
UGH (uveitis-glaucoma-hyphema) syndrome, also known as Ellingson syndrome, is not something vision professionals expect to encounter often in an average day at the office. A subacute or chronic complication of cataract surgery that results from issues with the intraocular lens (IOL), UGH syndrome is often underdiagnosed because of its rarity and its ability to show up weeks or even years after the procedure. Clinicians need to have this disease on their radar, however, as catching it early is key to preventing some of the syndrome’s more dire complications. In a 2021 case study involving UGH syndrome and one such dire complication (retinal detachment), Hermoso-Fernández et al. delve into the details surrounding this elusive and serious affliction.
When Strange Symptoms Appear
At first glance, the particulars of the case were nothing beyond the pale — a 56-year-old man was admitted to the emergency room complaining of blurred vision, redness and pain in his right eye for three days. Eight months prior, the man had cataract surgery involving IOL implantation on the right eye, with the anterior capsulorhexis leak as the only complication. An ophthalmological examination seemed to tell a familiar story: A VA of 6/5 in his right eye, good anterior chamber depth, moderate posterior capsule opacity, ciliary hyperemia, Tyndall (++++) and grade II corneal edema, no posterior synechiae, an IOP of 34 mmHg, and papillary excavation of 0.3 papilla diameters, with the rest of the posterior pole normal. Acute anterior uveitis due to Possner Schlossman syndrome was diagnosed, and dexamethasone, timolol and cyclopentolate eye drops were prescribed.
However, over the next nine years strange symptoms began to pop up. Response to treatment seemed good initially, but there were numerous recurrences with high IOP peaks. Multiple referrals to the hospital’s systemic diseases unit found the development of axonal neuropathy with increased papillary excavation accompanied by a concentric reduction of the visual field (central 10-degree preservation), with complete quadrantanopia being observed at the upper nasal level.
Eventually, a posterior capsulotomy with YAG laser was performed on the right eye. Ten days later, disaster struck — the patient reported vision loss and visual acuity of 6/40 was recorded. Biomicroscopy revealed Tyndall (+++), abundant pigment in the endothelium, a zone of transillumination and atrophy of the temporal sector of the iris, and vitreous at the lower pupillary level. Ultimately, a vitreous hemorrhage was found, which prevented correct retinal visualization; an Ultrasound B was conducted and showed hemovitreous echoes, suggesting retinal elevation at the lower level.
A pars plana vitrectomy with scleral buckle was landed on for surgery. During the procedure, an inferior retinal detachment was discovered with an elevation of the inferior hemimacula to the foveal limit. After nine years of head scratching, UGH syndrome entered the picture as a potential diagnosis due to the evolution of the patient’s condition and atrophic quadrant of the iris at the temporal level. Anterior segment OCT imaging was conducted to confirm, and an area of atrophy in the temporal sector of the iris was spotted — all due to contact with the IOL from the cataract surgery.
In the end, the results of the surgery were as good as could be hoped for with the late UGH syndrome diagnosis and near catastrophic progression of the condition. The patient improved to a visual acuity of 6/10 in the right eye with good IOP control, and the IOL was kept in situ. The visual field did retain an islet of inferior paracentral vision, with the scotoma enlargement coinciding with the area affected by retinal detachment. The study authors also added that the papilla demonstrated an inferior 67-micron, superior 71-micron, temporal 49-micron, and nasal 57-micron disc thickness map.
Lessons Learned: A Cautionary Tale
The case presented by Hermoso-Fernández et al., should read as a cautionary tale for clinicians dealing with cataract surgery patients. UGH syndrome is a vicious snake in the grass; it can masquerade as any one of its constituent ailments, making a premature diagnosis both tempting and all the more disastrous.
Early diagnosis is the name of the game with UGH syndrome. It should not be ruled out, as it can occur with any type of IOL — even those that are intracapsular and well-positioned. The mechanisms by which the disease works are critical knowledge, including mechanical irritation caused by poor IOL placement or subluxation that leads to mechanical and repetitive trauma to the iris.
The study authors also point to critical postoperative signs for early detection, such as transillumination, micro-hyphemas, pigment dispersion, iris neovascularization, macular cystoid edema, and recurrent vitreous hemorrhage. Individual patient characteristics like zonular laxity, a narrow pupil, anticoagulants, and knowledge of the design of the implanted lens also increase the patient’s risk of developing UGH syndrome. For a more complete list of preoperative and postoperative signs and risk factors, see the “Discussion” and “Conclusion” sections of the study.
In such cases that UGH is identified early, the authors suggest alpha2 antagonist treatment to prevent the progression of axonal neuropathy. In general, however, going to the heart of the problem might be best and rotating the IOL, or removing the implant for replacement, and the sooner the problem is recognized, the better the patient outcome will be.
*Hermoso-Fernández FM, Gonzalez-Gallardo C, Cruz-Rojo M. Retinal detachment in UGH Syndrome after cataract surgery. Rom J Ophthalmol. 2021; 65(4): 395–398.
