A Cocooned ACIOL Secondary to UGH Syndrome
Title: A Cocooned ACIOL Secondary to UGH Syndrome
Author: Tania Padilla Conde, MSIV, University of South Dakota Sanford School of Medicine
Photographer: Dr. Alan Crandall
Image or video:
Keywords/Main Subjects: UGH syndrome, cocooned IOL, uveitis, glaucoma, hyphema
Section 11 lens and cataractà Complications of cataract surgeryà UGH syndrome
Diagnosis: Inflamed cocoon anterior chamber IOL secondary to UGH syndrome, right eye
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This patient is an 85-year-old, African woman who presented with a chief complaint of worsening severe ocular pain in her right eye over the last several months. Her ocular history is significant for uveitis, elevated intraocular pressure (IOP), and cystoid macular edema (CME) of her right eye. Her ocular surgeries include cataract surgery in her right eye involving an anterior chamber intraocular lens (ACIOL) and a second cataract surgery in her left eye 8 years involving a posterior chamber intraocular lens (PCIOL). At presentation, she was on alphagan three times a day and timolol twice a day for her elevated IOP.
On exam, her visual acuity was OD count fingers at 3ft and OS 20/500. IOP by tonometry was OD 12 and OS 10. Pupils were non-reactive OU. Extraocular movements were full and ortho OU. Slit lamp examination OD showed benign melanosis, peripheral anterior synechiae (PAS) and a dislocated cocooned ACIOL. We were unable to get a clear view of the anterior chamber and the iris. We were also unable to get a clear view of the fundus on dilated exam. OS exam was unremarkable.
The patient was subsequently diagnosed with uveitis-glaucoma-hyphema (UGH) syndrome and underwent a removal of the ACIOL, anterior vitrectomy and synechiaelysis of the right eye. Due to severe inflammation, the decision was made to leave the patient aphakic with the possibility of an IOL implant in the future. The explanted IOL was sent to pathology and was found to be a one-piece with haptic PMMA anterior chamber Kelman multiflex-style IOL, Microscopic examination of the IOL showed several small fine granules of pigment on the haptics.
UGH syndrome is a rare condition that classically presents with uveitis, glaucoma, and hyphema in the setting of an anterior chamber IOL. However, it can be diagnosed when one, two, or all three of these conditions are present in the setting of any IOL causing irritation of the iris or angle structures.1 Although it is traditionally seen as a complication of anterior chamber IOLs, single-piece acrylic IOLs or sulcus IOLs, cases with posterior chamber IOLs have also been reported.2 UGH syndrome is commonly characterized by chronic inflammation, CME, secondary iris neovascularization, recurrent hyphemas, and glaucomatous optic neuropathy leading to a loss of vision.3 The diagnosis is clinical, based on the history and physical exam, and can be supplemented by ultrasound biomicroscopy.4
UGH syndrome results from peripupillary contact of the iris with the lens optic/haptics leading to mechanical irritation and erosion of uveal structures. This chafing leads to a breakdown of the blood-aqueous barrier and subsequent release of pigment, red blood cells, protein, and white blood cells into the anterior chamber.5 The resultant inflammation causes uveitis. The release of the red blood cells can additionally cause a hyphema. The increase in IOP is a result of red and white blood cells in the anterior chamber blocking the trabecular meshwork and/or by the destruction of outflow structures. Electron microscopy of explanted IOLs often reveals coccoid-like structures on the haptic surface and melanosomes, likely from damaged iris pigment epithelial cells.6
Medical management can include topical steroids for uveitis, IOP lowering topical and systemic medications for intraocular hypertension, and topical steroids and cycloplegics for hyphema.6 The use of anti-VEGF therapy has been shown to induce the regression of iris neovascularization and inflammation in uveitic macular edema.7 The definitive treatment is often surgical intervention including IOL repositioning, explantation and/or exchange.
- Cheng L, Fox AR, Kam JP, Alward WLM. Uveitis Glaucoma Hyphema (UGH) Syndrome. EyeRounds.org. posted October 3, 2017; Available from: http://EyeRounds.org/cases/257-UGH-syndrome.htm
- Zhang L, Hood CT, Vrabec JP, Cullen AL, Parrish EA, Moroi SE. Mechanisms for in-the-bag uveitis-glaucoma-hyphema syndrome. J Cataract Refract Surg 2014;40(3):490-492. https://www.ncbi.nlm.nih.gov/pubmed/24417893
- Crowell EL. Uveitis-Glaucoma-Hyphema Syndrome. EyeWiki. http://eyewiki.aao.org/ Uveitis-Glaucoma-Hyphema Syndrome. Accessed August 1, 2018.
- Lima BR, Pichi F, Hayden BC, Lowder CY. Ultrasound biomicroscopy in chronic pseudophakic ocular inflammation associated with misplaced intraocular lens haptics. Am J Ophthalmol. 2014 Apr;157(4):813–817. e1. doi: 10.1016/j.ajo.2013.12.025. https://www.ncbi.nlm.nih.gov/pubmed/24398393
- Chang DF, Masket S, Miller KM, Braga-Mele R, Little BC, Mamalis N, Oetting TA, Packer M. Complications of sulcus placement of single-piece acrylic intraocular lenses. Recommendations for backup IOL implantation following Posterior Capsule Rupture. J Cataract Refract Surg 2009;35:1445-58.
- Asaria RH, Salmon JF, Skinner AR, Ferguson DJ, McDonald B. Electron microscopy findings on an intraocular lens in the uveitis, glaucoma, hyphaema syndrome. Eye.1997;11(Pt 6):827–829. https://www.ncbi.nlm.nih.gov/pubmed/9537139
- Ellingson FT. The uveitis-glaucoma-hyphema syndrome associated with the Mark VIII anterior chamber lens implant. J Am Intraocul Implant Soc 1978;4(2):50-53. https://www.ncbi.nlm.nih.gov/pubmed/701165
- Rech L, Heckler L, Damji KF. Serial intracameral bevacizumab for uveitis glaucoma hyphaema syndrome: a case report. Can J Ophthalmol. 2014;49:e160–e162. https://www.ncbi.nlm.nih.gov/pubmed/25433757
Faculty Approval by: Dr. Alan Crandall and Dr. Griffin Jardine
Copyright statement: Copyright Tania Padilla Conde, ©2018. For further information regarding the rights to this collection, please visit: URL to copyright information page on Moran CORE
Disclosure (Financial or other): None