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A Man With a Cloudy Cornea

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 77-year-old man was referred to the cornea service for painless, progressive blurring of vision in his left eye. His best-corrected visual acuity was 20/40 OS, and the patient reported that his vision had waxed and waned over several months. His ocular history included cataract surgery in the left eye, followed by yttrium-aluminum-garnet capsulotomy 2 months before his referral. Of note, he had recently undergone Mohs surgery and subsequent reconstruction for squamous cell carcinoma in situ of the lateral canthus in the fellow eye; he also had a medical history of multiple skin malignant neoplasms.

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Corneal intraepithelial neoplasia

A. Discuss superficial keratectomy to confirm the diagnosis by histopathologic examination

Calcium chelation with EDTA therapy (choice B) is not recommended because the appearance and location of the lesion are atypical for band keratopathy. Performing a penetrating keratoplasty (choice C) would not be indicated because the lesion is superficial and does not affect the deeper cornea stromal layers. Finally, observation (choice D) would not be preferred because the patient is experiencing visual issues due to the lesion, and other conditions, such as a malignant neoplasm, should be excluded.

Corneal intraepithelial neoplasia (CIN), also known as corneal epithelial dysplasia, is usually grouped with conjunctival intraepithelial neoplasia under the broader classification of ocular surface squamous neoplasia (OSSN). Ocular surface squamous neoplasia is one of the most common ocular tumors in the US and is generally found in males of advanced age.1 Risk factors include prolonged exposure to UV light, infection with human papillomavirus or human immunodeficiency virus, smoking, and immunosuppression.2 Patients customarily present with nonspecific progressive symptoms of painless vision loss, which may be associated with ocular foreign body sensation or redness.3 Corneal intraepithelial neoplasia onset is typically in the limbal area, with a gradual extension toward the central visual axis. Lesions may or may not be accompanied by neovascularization.

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Article Information

Corresponding Author: Christine Shieh, MD, Department of Ophthalmology and Visual Sciences, Vanderbilt Eye Institute, 2311 Pierce Ave, Nashville, TN 37203 (christine.shieh@vumc.org).

Published Online: December 23, 2020. doi:10.1001/jamaophthalmol.2020.4646

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Clinical approach to neoplastic disorders of the conjunctiva and cornea. In: Weisenthal RW, ed.  2019-2020 BCSC: Basic and Clinical Science Course: External Disease and Cornea. American Academy of Ophthalmology; 2019:327-350.
2.
Chin  EK , Cortés  DE , Lam  A , Mannis  MJ .  Anterior segment OCT and confocal microscopy findings in atypical corneal intraepithelial neoplasia.   Cornea. 2013;32(6):875-879. doi:10.1097/ICO.0b013e318285cab9 PubMedGoogle ScholarCrossref
3.
Alomar  TS , Nubile  M , Lowe  J , Dua  HS .  Corneal intraepithelial neoplasia: in vivo confocal microscopic study with histopathologic correlation.   Am J Ophthalmol. 2011;151(2):238-247. doi:10.1016/j.ajo.2010.08.035 PubMedGoogle ScholarCrossref
4.
Wessel  MM , Sarkar  JS , Jakobiec  FA ,  et al.  Treatment of Lisch corneal dystrophy with photorefractive keratectomy and mitomycin C.   Cornea. 2011;30(4):481-485. doi:10.1097/ICO.0b013e3181ec8e26 PubMedGoogle ScholarCrossref
5.
Barros  JN , Lowen  MS , Ballalai  PL , Mascaro  VLDM , Gomes  JAP , Martins  MC .  Predictive index to differentiate invasive squamous cell carcinoma from preinvasive ocular surface lesions by impression cytology.   Br J Ophthalmol. 2009;93(2):209-214. doi:10.1136/bjo.2008.147710 PubMedGoogle ScholarCrossref
6.
Carreira  H , Coutinho  F , Carrilho  C , Lunet  N .  HIV and HPV infections and ocular surface squamous neoplasia: systematic review and meta-analysis.   Br J Cancer. 2013;109(7):1981-1988. doi:10.1038/bjc.2013.539 PubMedGoogle ScholarCrossref
7.
Sayed-Ahmed  IO , Palioura  S , Galor  A , Karp  CL .  Diagnosis and medical management of ocular surface squamous neoplasia.   Expert Rev Ophthalmol. 2017;12(1):11-19. doi:10.1080/17469899.2017.1263567 PubMedGoogle ScholarCrossref
8.
Shields  CL , Kaliki  S , Kim  HJ ,  et al.  Interferon for ocular surface squamous neoplasia in 81 cases: outcomes based on the American Joint Committee on Cancer classification.   Cornea. 2013;32(3):248-256. doi:10.1097/ICO.0b013e3182523f61 PubMedGoogle ScholarCrossref
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