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Advanced Keratomalacia With Descemetocele in an Infant With Cystic Fibrosis
Arch Ophthalmol. 2005;123:1012-1016.
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Xerophthalmia refers to the spectrum of ocular manifestations of vitamin A deficiency. It represents the leading cause of childhood blindness worldwide but is uncommon in industrialized countries,1 where xerophthalmia is more often the result of malabsorption than malnutrition due to poverty. Cystic fibrosis (CF) is an autosomal recessive disease with hyperviscosity of mucus secretions causing chronic pulmonary changes and pancreatic insufficiency. Anderson2 was the first to note the association between xerophthalmia and CF, now thought to be due to fat malabsorption resulting in fat-soluble vitamin deficiency. Advanced xerophthalmia has been reported as an initial sign of CF.3-4 A recent review article5 summarized the ocular findings of CF to include xerophthalmia, tear film abnormalities, papilledema, and nyctalopia. To our knowledge, this is the first clinicopathologic report of keratomalacia with a descemetocele requiring keratoplasty as the initial manifestation of CF.
Report of a Case
A 5-month-old girl from Juarez, Mexico, was admitted to a hospital in . . . [Full Text of this Article]
Comment
AUTHOR INFORMATION
Sonja Wamsley, MD, MPH;
Sarit M. Patel, MD;
Mark G. Wood, MD;
Robert Villalobos, MD;
Daniel M. Albert, MD, MS;
V. Vinod Mootha, MD
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