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Aphakic Bullous Keratopathy/Pseudophakic Bullous Keratopathy

ROUTINE — Cornea

Symptoms

  • Decreased visual acuity.
  • Ocular pain, tearing, and foreign body sensation.
  • Photophobia and redness.
  • History of prior cataract surgery in the affected eye.

Clinical Findings

Corneal signs

  • Corneal edema in an aphakic or pseudophakic eye.
  • Presence of epithelial bullae and Descemet folds.
  • Subepithelial haze, scarring, or neovascularization (chronic disease).

Associated signs

  • Cystoid macular edema (CME) frequently coexists.
  • Pre-existing endothelial guttae (Fuchs dystrophy) may be present in either eye.
  • Retained lens fragments or malpositioned intraocular lens (IOL).

Etiology

Pathophysiology

  • Endothelial cell density falls below critical threshold (~500 cells/mm²).
  • Failure of pump-leak equilibrium leads to stromal and epithelial edema.

Surgical trauma

  • Prolonged phacoemulsification time or mechanical trauma (iris prolapse, vitreous loss).
  • Complicated ACIOL placement or retained lens fragments.

Mechanical contact

  • ACIOL risk: Rigid PMMA haptics chronically abrading endothelium.
  • Vitreous-cornea touch or subluxed IOL contacting the endothelium.

Pre-existing & chronic factors

  • Endothelial compromise (Fuchs guttae, prior surgery, uveitis, glaucoma).
  • UGH syndrome: Chronic inflammation from IOL malposition accelerates cell loss.

Red Flags

  • Painful bullae: Requires bandage contact lens and hypertonic saline.
  • Vitreous-cornea touch: Mandates anterior vitrectomy plus keratoplasty.
  • Recurrent infection: Risk with bandage lenses; requires prophylactic antibiotics.
  • Concurrent CME: Must be addressed to optimize visual potential after surgery.

Investigations

Slit lamp exam

  • Fluorescein staining: To detect ruptured bullae or denuded epithelium.
  • IOL/Vitreous assessment: Look for malposition or vitreous-cornea touch.
  • Fellow eye: Screen for Fuchs endothelial dystrophy.

Ancillary testing

  • IOP measurement: Manage associated glaucoma.
  • Macular OCT: To exclude or document concurrent CME (may be limited by haze).

Treatment

Medical management

  • Sodium chloride 5% drops QID and ointment QHS.
  • Lower IOP if elevated.
  • Avoid prostaglandins and epinephrine derivatives (CME risk).

Management of ruptured bullae

  • Topical antibiotics (erythromycin/bacitracin q2h) and cycloplegics (cyclopentolate 1% TID).
  • Bandage soft contact lens for comfort.
  • Chronic pain: Anterior stromal micropuncture or excimer PTK.

Surgical intervention

  • Endothelial keratoplasty (DMEK/DSAEK): First-line definitive surgery.
  • Penetrating keratoplasty (PK): Reserved for cases with significant stromal scarring.
  • Combined procedures: IOL repositioning/exchange or vitrectomy as required.

Clinical Pearl

A hazy cornea in the setting of an IOL is pseudophakic bullous keratopathy. Endothelial keratoplasty (DMEK/DSAEK) is the definitive treatment and restores vision more effectively than full-thickness PK.

Differential Diagnosis

  • Fuchs endothelial dystrophy
  • ICE syndrome
  • Resolving postsurgical corneal edema
  • Infectious keratitis

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