2657: Quiz: Why did this happen? Help us!

We need your help. With the collective minds and experience of thousands of ophthalmologists, I trust that we can solve this mystery case. This patient presents to our guest surgeon with acute bilateral white cataracts, bilateral corneal edema, and bilateral hypotony. Here is a summary. Please read carefully and leave your comments below so we can solve this mystery together!

video link here

https://youtu.be/0xUbMicNy-w

Summary of Key Clinical Features

Demographics/History:

• 49-year-old female
• History of keratoconus
• Systemic illness: compensated Primary Biliary Cirrhosis (on Urosfalk + carvedilol)
• History of unsupervised/off-label IV treatments via alternative medicine

Sequence of Events:

1. Prescribed dex/levo eye drops for 4 weeks pre-ICRS
2. Sudden bilateral vision loss, pain, and photophobia
3. Exam: bilateral acute intumescent cataracts, shallow AC, hypotony, corneal edema
4. B-scan: clear vitreous; UBM: phacomorphic-like, no CB rotation
5. Intense fibrinous reaction → resolved with oral (not topical) steroids
6. Cataract surgery performed with difficult view, rhexis extension, but normal IOL position
7. Persistent hypotony, corneal edema, photophobia postop

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Differential Diagnosis & Pathophysiological Considerations

1. Idiosyncratic reaction to topical steroids

• The rapid and severe fibrin reaction to topical steroids (yet resolution with systemic steroids) is highly unusual.
• Idiosyncratic steroid reactions usually manifest as:
  • IOP elevation
  • Rarely as inflammatory reactions due to vehicle/preservative (e.g., BAK, phosphate buffer)
• The lack of inflammation postoperatively on oral steroids argues against classic autoimmune uveitis, but supports a toxic or hypersensitivity etiology.

2. Toxic anterior segment syndrome (TASS)-like phenomenon

• The bilateral, synchronous, rapid course, and significant fibrin, corneal edema, hypotony, and pain raise suspicion for a TASS-like event.
• However, this predated surgery and was not associated with intraocular procedure.
• Possibly triggered by:
  • Prolonged use of topical dex/levo – especially if containing phosphate-buffered dexamethasone (phosphate may chelate calcium and cause calcific deposits/toxicity)
  • Unregulated IV medications from homeopathy clinics – unknown toxicity or immune-mediated response

3. Bilateral phacomorphic response without uveal effusion or CB rotation

• Rare, but the intumescent lens picture suggests osmotic swelling of the lens, possibly due to:
  • Metabolic derangement (PBC-related, or from toxic exposure)
  • Steroid-induced metabolic lens changes (unusual, but theoretically plausible)
  • Subacute lens protein alteration → secondary inflammation/hypotony

4. Ciliary body shutdown / toxic cyclitis

• Explains persistent hypotony even after IOL placement and deep AC
• Supported by:
  • Poor IOP recovery postop
  • No apparent leakage or overt inflammation
  • Possibly sterile inflammation of CB due to systemic or topical insult

5. Corneal decompensation – endothelial insult

• Given pre-existing keratoconus, the endothelium may be functionally marginal.
• Toxic injury to endothelium from:
  • Topical medications
  • Surgical trauma unlikely (no phaco, minimal power)
  • Inflammatory milieu / cytokine storm effect
• Persistent edema one week post-op, with Descemet’s folds, is concerning for irreversible damage

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Investigative Pathways

1. Specular Microscopy (if possible) – assess endothelial cell count and morphology
2. Anterior Segment OCT – to evaluate Descemet’s, corneal thickness, iris/CB configuration
3. Repeat UBM – to assess ciliary body function/atrophy or detachment
4. Aqueous Tap for Cytology/Culture – especially if any suspicion of infection or retained toxin
5. Toxicology / Immunology Workup – for unusual serum metabolites or circulating toxins

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Working Hypothesis

This appears to be a bilateral toxic anterior segment insult, likely multifactorial, with key contributors being:

1. Prolonged topical dexamethasone-phosphate (±preservatives) causing:
   • Endothelial toxicity
   • Possible sterile anterior uveitis-like reaction
2. Idiosyncratic or hypersensitivity reaction to topical medications (or even vehicle components)
3. Unregulated systemic therapies (IV substances from homeopathic clinic) leading to:
   • Lens osmotic changes (intumescence)
   • Possible immune dysregulation
   • Ciliary body suppression

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Management Thoughts & Prognosis

Short-term:

• Continue systemic corticosteroids – appears more effective than topical
• Carefully titrate topical steroids – right eye trial reasonable
• Aggressive hypertonic saline, Muro 128, and aqueous suppressants if IOP begins to rise
• Consider temporary intracameral viscoelastic if hypotony severe/persistent

Long-term:

• If endothelial function does not recover → DMEK or DSAEK consideration
• Monitor closely for chronic hypotony maculopathy, optic nerve damage
• Evaluate ocular surface and lacrimal function – chronic inflammation may unmask severe dry eye

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Final Thoughts

This is a uniquely complex case with overlapping iatrogenic, inflammatory, and possibly toxic-metabolic components. A central mechanism seems to be bilateral anterior segment toxicity—possibly drug-induced—with associated ciliary body dysfunction and endothelial compromise. The precise trigger may never be identified, but the interplay between unregulated systemic treatments, topical steroids, and pre-existing ocular pathology (keratoconus) likely set the stage.