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

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

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

• J. Martin Heur, MD, PhD, Associate Professor of Clinical Ophthalmology, heur@med.usc.edu
• Christine Greer, MD, PGY-2 Ophthalmology Resident, christine.greer@med.usc.edu

Section Editors

• Vivek Patel, MD, Associate Professor of Clinical Ophthalmology, Program Director, vivek.patel@med.usc.edu
• Jesse Berry, MD, Assistant Professor of Clinical Ophthalmology, Associate Program Director, jesse.berry@med.usc.edu

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