Sunir Garg
BASICS
DESCRIPTION
• Lens-induced uveitis results from an autoimmune reaction to crystalline lens protein released from surgical or accidental trauma (phacoantigenic endophthalmitis) or leaked from a hypermature cataract (phacolytic glaucoma). Phacoantigenic endophthalmitis was formerly known as phacoanaphylactic endophthalmitis (1)[C], (2)[C].
• Uveitis-glaucoma-hyphema (UGH) syndrome is a complication of intraocular lens (IOL) implantation.
EPIDEMIOLOGY
Rare cause of uveitis, exact incidence and prevalence unknown.
RISK FACTORS
• Lens-induced uveitis occurs with cataract extraction surgery, ocular trauma with disruption of the anterior lens capsule, or a hypermature cataract.
• UGH syndrome is more commonly seen with iris-fixated, anterior chamber IOLs, and sulcus placed posterior chamber IOLs.
PATHOPHYSIOLOGY
• Immune reaction to lens protein in the anterior chamber causes lens-induced uveitis. Inflammation can result from surgery or trauma that disrupts the anterior lens capsule. This inflammation can be granulomatous and severe (phacoantigenic endophthalmitis).
• Leakage of hypermature lens protein through an intact capsule can stimulate a nongranulomatous anterior uveitis with elevated intraocular pressure (phacolytic glaucoma).
• UGH syndrome is caused by contact between the uveal tissue and the IOL haptic or optic.
ETIOLOGY
• In lens-induced uveitis, lens proteins are exposed through trauma (either surgical or accidental) or leakage from a hypermature cataract through an intact lens capsule.
• Iris chafe in UGH syndrome is caused by iris-clipped IOLs, inappropriately sized IOLs, malpositioned IOLs, or rigid closed-loop haptics. It may also occur with poorly manufactured IOL edges and decentered posterior chamber IOLs.
DIAGNOSIS
HISTORY
• Recent history of surgery or eye trauma for lens-induced uveitis
• UGH syndrome more often seen with history of IOL implantation one or more years prior to presentation
• Symptoms include pain, redness, photophobia, and blurred vision.
PHYSICAL EXAM
• Lens-induced uveitis may cause an anterior uveitis than can be mild or severe and granulomatous or nongranulomatous. Posterior synechiae and elevated intraocular pressure are common. Cells may be present in anterior vitreous. Integrity of anterior lens capsule should be assessed.
• The uveitis in UGH syndrome is nongranulomatous and may be accompanied by transillumination defects in the iris, pigment dispersion, hyphema, and elevated intraocular pressure.
DIAGNOSTIC TESTS & INTERPRETATION
Imaging
Ultrasound biomicroscopy (UBM) of the anterior segment may demonstrate IOL contact with uvea for UGH syndrome (3)[C].
Diagnostic Procedures/Other
• Diagnosis in lens-induced uveitis can be confirmed by cytologic analysis of cells obtained via anterior chamber paracentesis (4)[C].
– Infectious endophthalmitis can be ruled out by analysis of vitreous or aqueous cultures.
Pathological Findings
Lens protein can be observed with anterior chamber paracentesis.
DIFFERENTIAL DIAGNOSIS
• Infectious endophthalmitis, especially following surgery
• Other causes of acute anterior uveitis (e.g. HSV, VZV, idiopathic, sarcoidosis, HLA-B27, and others)
TREATMENT
MEDICATION
First Line
• Topical corticosteroids, with systemic corticosteroids if needed
• Topical antiglaucoma medications for elevated intraocular pressure
SURGERY/OTHER PROCEDURES
• UGH syndrome patients may require IOL explantation for persistent inflammation, glaucomatous nerve damage, or recurrent hyphemas (6)[C].
• Prompt removal of all lens remnants can be curative for lens-induced uveitis:
– Optimal treatment involves controlling intraocular inflammation and pressure prior to surgery.
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
Close follow-up to ensure that intraocular inflammation and pressure are controlled
COMPLICATIONS
Phthisis, glaucomatous visual field loss, central retinal artery occlusion (secondary to elevated intraocular pressure)
REFERENCES
1. Apple DJ, Mamalis M, Steinmetz RL, et al. Phacoanaphylactic endophthalmitis associated with extracapsular cataract extraction and posterior chamber intraocular lens. Arch Ophthalmol 1984;102:1528–1532.
2. Marak GE Jr. Phacoanaphylactic endophthalmitis. Surv Ophthalmol 1992;36(5):325–329.
3. Piette S, Canlas OA, Tran HV, et al. Ultrasound biomicroscopy in uveitis-glaucoma-hyphema syndrome. Am J Ophthalmol 2002;133(6):839–841.
4. Kalogeropoulos CD, Malamou-Mitsi VD, Asproudis I, et al. The contribution of aqueous humor cytology in the differential diagnosis of anterior uvea inflammations. Ocul Immunol Inflamm 2004;12(3):215–225.
5. Abrahams IW. Diagnosis and surgical management of phacoanaphylactic uveitis following extracapsular cataract extraction with intraocular lens implantation. J Am Intraocul Implant Soc 1985;11(5):444–447.
6. McMahon MS, Weiss JS, Riedel KG, Albert DM. Clinically unsuspected phacoanaphylaxis after extracapsular cataract extraction with intraocular lens implantation. Br J Ophthalmol 1985;69(11):836–840.
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