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Corneal Epitheliopathy in Congenital Methemoglobinemia

Chaurasia, Sunita MD; Ramappa, Muralidhar MD; Bhargava, Archana DNB

doi: 10.1097/ICO.0000000000000076
Case Report

Purpose: The aim of this study was to describe a previously unreported characteristic corneal epitheliopathy in congenital methemoglobinemia (type 1).

Methods: This was an observational case series of ocular findings in congenital methemoglobinemia.

Results: Siblings of 2 different affected families (age range, 12–14 years; 3 girls, 1 boy) affected with congenital methemoglobinemia had similar ocular complaints and clinical presentation. All of them had recurrent episodes of irritation and tearing in both eyes. Slit lamp biomicroscopy examination in all of them revealed the presence of dark colored corkscrew conjunctival vessels (prominently seen on the tarsal and forniceal region) and grayish-white raised corneal epithelial lesions. Diagnostic corneal scrapings in 2 of the patients showed the presence of epithelial cells with nonspecific inflammatory cells, and the results were negative for microbes. None of the patients had any abnormality in the lid adnexa, blink response, corneal sensation, or in the tear film. The corneal lesions resolved in 2 to 3 weeks time while on supportive therapy alone and on vitamin C supplements.

Conclusions: Dark colored conjunctival vessels and recurrent corneal epitheliopathy causing irritation and tearing may be ocular features associated with congenital methemoglobinemia. It is pertinent for ophthalmologists, hematologists, and pediatricians to be aware of this association.

*Cornea and Anterior Segment Services, LV Prasad Eye Institute, Hyderabad, India; and

Internal Medicine, LV Prasad Eye Institute, Hyderabad, India.

Reprints: Muralidhar Ramappa, Cornea and Anterior Segment Services, LV Prasad Eye Institute, Kallam Anji Reddy Campus, Banjara Hills, Hyderabad, India 560 034 (e-mail:

Supported by the Hyderabad Eye Research Foundation, India.

The authors have no funding or conflicts of interest to disclose.

Received August 20, 2013

Accepted December 23, 2013

Copyright © 2014 Wolters Kluwer Health, Inc. All rights reserved.