Mohib Naseer
Introduction
Uveitis glaucoma hyphema syndrome also known as Ellingson Syndrome is a complication that occurs due to intraocular chafing and rubbing of intraocular lens against uveal tissue in the eye that leads to iris trans illumination defect, micro hyphema (red blood cells in anterior chamber), gross hyphema, pigment dispersion, raised intraocular pressure (glaucoma) and intraocular inflammation of uveal tissue (uveitis) (1). It can present with symptoms such as decrease in vision, redness, pain, and sensitivity to light. Clinical signs include raised intraocular pressure, anterior chamber inflammatory activity (red blood cell and inflammatory cells), cystoid macular oedema and optic nerve cupping if persistent for long term. Symptoms can be controlled with topical medication like steroids and pressure lowering drops but surgical intervention like repositioning, removal and exchange of intraocular lens is definitive surgical treatment.
History
The term UGH syndrome was first used by Ellingson in 1978(2) when he observed that intraocular lenses in anterior chamber rub against uveal tissue causing mechanical irritation of adjacent tissue in the angle of anterior chamber thus causing pigment dispersion, inflammation, and glaucoma. Since then, the incidents of UGH syndrome significant decreased due to improvement in lens design, intraocular lens material, surgical techniques, and use of posterior chamber intraocular lenses instead of anterior chamber intraocular lens. Studies suggest that incidence of UGH has decreased from 2.2-3% to 0.4-1.2% depending on the IOL type over 1 year.
Epidemiology
UGH syndrome can affect any age group with intraocular lenses or iris prosthesis, but it most commonly affects elderly adults, there are few cases in paediatric population as well. The incidence of UGH syndrome in first six-months of IOL implantation is higher with anterior chamber intraocular lenses (AC IOL) compared to iris plane or posterior chamber intraocular lenses.
Aetiology
Evidence suggests that the disease results from mechanical irritation of anterior segment structure due to intraocular lens causing erosion of uveal structure. This chafing can break down blood-aqueous barrier, releasing pigment, red blood cells, proteins, and white blood cells into the anterior chamber. Red blood cells release causes microhyphema or hyphema. These cells can block trabecular meshwork, increasing intraocular pressure, similarly white blood cells released can cause intraocular inflammation.
Prevention
Prevention of UGH syndrome is crucial and can decrease the incidence. During routine cataract surgery(3), a single piece IOL should be placed in the capsular bag with both leading and trailing haptic inside the bag. If this cannot be achieved due to a surgical complication, then an alternate type of lens should be used with efficient surgical technique to prevent uveal disruption. A single piece IOL should not be placed in the sulcus (space between iris and lens capsule), instead a three-piece IOL should be placed in the sulcus if there is enough capsular support, if there is inadequate capsular or zonular support then a three-piece IOL can be fixated via scleral fixation technique such as Yamane. If an anterior chamber intraocular lens is required, then a modern iris claw lens is preferred over a large AC IOL with haptics which were common in the past. It is also worth mentioning that the AC IOL must be oriented correctly to avoid upside down lens syndrome which might cause chronic iris irritation, iris adhesion and corneal decompensation that might need further surgery to correct.
Diagnosis
Diagnosis of UGH syndrome is clinical, based on history, physical examination, and imaging. History would usually suggest a complicated cataract surgery with incorrect orientation of intraocular lens, slit-lamp examination would reveal corneal oedema (microcystic, due to raised intraocular pressure or corneal decompensation), anterior chamber inflammatory cell or flare (uveitis) and red blood cells in anterior chamber (hyphema). Sometimes red blood cells migrate into vitreous chamber causing vitreous haemorrhage hence causing UGH plus syndrome. Imaging modalities like macular optical coherence tomography (OCT) would suggest cystoid macular oedema.
Symptoms
A patient may present with:
- Intermittent decreased or blurred vision
- Intermittent white out of vision
- Photophobia
- Redness
- Ocular pain
Signs
- Raised intraocular pressure
- Micro hyphema or gross hyphema
- Anterior chamber cells and flare or hypopyon
- Iris neovascularisation
- Iris lens contact
- Iris transillumination defect
- Dislocated or malpositioned IOL
- Misplaced haptic
- Vitreous haemorrhage
- Cystoid macular oedema
Management
There are different treatment modalities initially starting with medical treatment to control intraocular pressure and inflammation to relieve symptoms of UGH syndrome followed by surgical intervention.
Medical Treatment
o Topical corticosteroids to control intraocular inflammation
o Use of IOP lowering medication such as prostaglandin analogues, beta adrenergic antagonists, alpha adrenergic agonists, and carbonic anhydrase inhibitor to control intraocular pressure.
Surgical Treatment
o IOL repositioning, removal and exchange with advanced surgical techniques can be achieved to stop the recurrence of UGH syndrome.
References
- Uveitis-Glaucoma-Hyphema Syndrome – EyeWiki [Internet]. 2024. Available from: https://eyewiki.org/Uveitis-Glaucoma-Hyphema_Syndrome
- Ellingson FT. The Uveitis-glaucoma-hyphema syndrome associated with the Mark VIII anterior chamber lens implant. American Intra-Ocular Implant Society Journal [Internet]. 1978 Apr;Volume 4(Issue 2):50–3. Available from: https://www.sciencedirect.com/science/article/abs/pii/S0146277678800548
- Cataract – EyeWiki [Internet]. 2024. Available from: https://eyewiki.org/Cataract