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LARGEST STUDY ON CHINESE AMERICANS PUBLISHED

LARGEST STUDY ON CHINESE
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USC Ophthalmology Researchers Find More
Effective Treatments For Blinding Eye Diseases

EDUCATION

Case Study: A Whirlwind Diagnosis

Greer Heur
Presenter: Christine Greer, MD Discussant: J. Martin Heur, MD, PhD
 

History

  • 50-year-old Caucasian man from Wyoming presents with blurry vision in the left eye
    • Patient reports fuzzy and monocular double vision in the left eye for prior two to three months
    • Patient states he feels growth on the cornea OS
  • No previous ophthalmologic problems within his immediate family
  • Past medical history: hypertension, hyperlipidemia, under good control
  • Social history: professional photographer, non-smoker
  • Previous surgical history of superficial keratectomy in both eyes

Exam Findings

  • VA 20/20 || 20/60 PH 20/40
  • Pupils RR OU, no RAPD
  • IOP (Tp) 12, 13
  • EOMI
Figure 1
Figure 1: External photograph of patient’s right eye depicting large, paracentral corneal opacification.
Figure 2
Figure 2: Slit lamp photograph of patient’s right eye. Note that the layer of involvement of the corneal opacity is within the epithelium.
Figure 3
Figure 3: Sclerotic scatter demonstrating white-gray paracentral opacification with feathery borders and densely crowded microcysts without clear, intervening zones.
Figure 4
Figure 4: Slit lamp photograph of patient’s left eye. Corneal opacity within the epithelium.
Figure 5
Figure 5: Sclerotic scatter demonstrating white-gray paracentral opacification with feathery borders and densely crowded microcysts without clear, intervening zones.
Figure 6
Figure 6: Anterior segment OCT with sharp demarcation of the epithelium, from hyperreflective to normal cornea.
Figure 7
Figure 7: A and B show no astigmatism OU.

 

Differential Diagnosis

  • Lisch corneal dystrophy
  • Meesmann corneal dystrophy
  • Epithelial basement membrane dystrophy
  • Thiel-Behnke corneal dystrophy
  • Reis-Bücklers corneal dystrophy
  • Systemic: verticillata
  • Limbal stem cell deficiency
  • Ocular surface squamous neoplasia

Diagnosis

  • Lisch corneal dystrophy

Pathophysiology

  • Corneal dystrophies
    • Bilateral, symmetric inherited conditions
    • Slowly progressive and more pronounced over age
    • Genetics: TGFB1, protein keratoepithelin
    • corneadystrophy.org for classifications
  • Lisch corneal dystrophy
    • X-linked dominant; unknown gene
    • Slit lamp: discrete sectoral, gray, band-shaped and feathery lesions that appear in whorled pattern
    • Retroillumination: intraepithelial, densely crowded microcysts with surrounding clear cornea
    • No pain (recurrent erosions)
      • Differentiates from other anterior corneal dystrophies
    • Pathology
      • Light microscopy: diffuse cytoplasmic vacuolization
      • Sharp delineation from normal areas
      • Scattered staining with Ki-67 without evidence of increased mitotic activity
Figure 8
Figure 8: A. shows high-power field cornea epithelial with intracytoplasmic vacuoles; B. shows this finding in low-power field.

 

Treatment

  • Epithelial debridement (superficial keratectomy)
    • Typically recurs

References

  • Middle East Afr J Ophthalmol. 2013 Jan-Mar;20(1):5-10.
  • Lisch WA, A new, band-shaped and whorled microcystic dystrophy of the corneal epithelium. Amer J Ophthal. 1992 July;114:35-44.
  • Cirino AC, Color Atlas and Synopsis of Clinical Ophthalmology-Wills Eye Hospital. 2012. 49(4):109.

Contact

Section Editors

 

Produced by: Monica Chavez, John Daniel, Joseph Yim and Dr. Vivek Patel
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