Keywords

Overview

  • Definition

    • Anterior and intermediate uveitis resulting from a localized inflammatory reaction to crystalline lens proteins

    • Triggering events include

      • Mature or hypermature cataract

      • Retained lens materials after extracapsular cataract surgery

      • Lens capsule rupture following blunt or penetrating trauma

      • Iatrogenic capsular rupture from glaucoma surgery or other intraocular procedures

  • Symptoms

    • Blurry vision

    • Photophobia

    • Pain

    • Floaters

  • Laterality

    • Usually unilateral

  • Course

    • Hours to decades after the causative event

    • Inflammation is persistent and may be severe until lens materials are extracted completely

    • Left untreated, LIU is complicated by severe glaucoma as well as pupillary and cyclitic membrane formation that ultimately results in hypotony, retinal detachment, and phthisis bulbi

  • Age of onset

    • 60–70 years

  • Gender/race

    • No gender or racial predilection

  • Systemic association

    • None

Exam: Ocular

Anterior Segment

  • Non-granulomatous or granulomatous inflammation, with abundant cells and flare

  • Small KPs (early disease) that coalesce into mutton-fat KPs (late, severe disease)

  • Residual lens material in the anterior or posterior chamber (may be visible only on gonioscopy)

  • Mature or hypermature cataract

  • Ragged or ruptured anterior lens capsule

  • Hypopyon or pseudohypopyon (inflammatory cells mixed with lens material)

  • Posterior synechiae

  • Elevated IOP

Posterior Segment

  • Vitritis is always present, though may be obscured by media opacity

  • Retinal detachment may occur in severe, untreated cases as the result of cyclitic membrane formation and contraction

Exam: Systemic

  • Signs of head or facial trauma

Imaging

  • Anterior segment OCT or UBM

    • Useful in detecting retained lens material especially when gonioscopy is obscured by dense AC reaction

  • B-scan

    • May reveal retained lens fragments in the posterior segment

Laboratory and Radiographic Testing

  • AC or vitreous tap

    • Histologically characterized by zonal inflammation in and around the lens, consisting of lymphocytes, neutrophils, macrophages, epithelioid and giant cells

    • Culture and PCR to exclude infectious masqueraders

Differential Diagnosis

  • Sympathetic ophthalmia

    • Bilateral

    • Panuveitis with frequent inflammatory relapses

  • Infectious endophthalmitis (exogenous or postoperative)

    • Exogenous

      • Commonly Staphylococcus epidermidis

      • Open globe or intraocular foreign body may be present

      • Panuveitis

    • Postoperative

      • Acute : S. epidermidis, typically occurring 2–6 weeks postoperatively

      • Chronic: Propionibacterium acnes, typically occurring 3 months postoperatively

      • Vitritis is usually mild with a granulomatous anterior uveitis

      • Retained lens material not typically found

  • Glaucomatocyclitic crisis (Posner-Schlossman syndrome)

    • Recurrent episodes of elevated IOP, mydriasis, corneal edema and low-grade AC reaction

  • IOL-associated uveitis

    • Unlikely with biocompatible, acrylic IOLs

  • Uveitis-glaucoma-hyphema (UGH) syndrome

  • Consider other causes of anterior/intermediate uveitis if removal of lens material does not result in resolution of inflammation

Treatment

  • Steroid, cycloplegic, and glaucoma drops for immediate inflammatory and IOP control

  • Surgical removal of retained lens material, either via limbal incision or pars plana vitrectomy, offers definitive cure

    • If retained lens material is minimal and resorption is likely, observation and treatment with topical steroids until all lens material is resorbed may be sufficient

Referral/Co-management

  • None