$Unique_ID{BRK03899} $Pretitle{} $Title{Keratomalacia} $Subject{Keratomalacia Xerotic Keratitis Xerophthalmia Night Blindness Vitamin A Deficiency Retinol Deficiency Sjogren's Syndrome Keratoconus Interstitial Keratitis Bullous Keratopathy} $Volume{} $Log{} Copyright (C) 1990 National Organization for Rare Disorders, Inc. 762: Keratomalacia ** IMPORTANT ** It is possible that the main title of the article (Keratomalacia) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Xerotic Keratitis Xerophthalmia Night Blindness Vitamin A Deficiency Retinol Deficiency Information on the following diseases can be found in the Related Disorders section of this report: Sjogren's Syndrome Keratoconus Interstitial Keratitis Bullous Keratopathy General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Keratomalacia is an eye disease caused by a deficiency of vitamin A. Vitamin A (retinol) is found mainly in fish liver oils, liver, egg yolk, cream and butter. The human body stores vitamin A mainly in the liver. Once it is released by the liver, vitamin A is converted to light sensitive pigments in the retina that are involved in night, day and color vision. Vitamin A also helps maintain healthy body tissue. Keratomalacia is very rare in North America and Western Europe, but is a leading cause of blindness in Southeast Asia, parts of Africa and Central and South America. In these areas vitamin A deficiency is usually caused by general malnutrition in infants and young children. Symptoms A deficiency of Vitamin A may interfere with the eye's ability to adapt to the dark, resulting in night blindness. It may also cause a lack of tears leading to abnormal dryness (xerosis) of the inner surface of the eyelid (conjunctiva) and the transparent covering (cornea) of the eyes. This dryness may result in a sensation of grittiness in the eyes and a painful sensitivity to light (photophobia). Shiny, pearled spots of triangular shaped tissue may occur on the inner surface of the eyelids (Bitot's spots). The cornea may become hazy and gradually dissolve, leading to rupture of the eyeball with extrusion of the eye's contents. In vitamin A deficiency, cells in the lung, gastrointestinal tract and urinary tract may be keratonized. The senses of taste and smell may be defective, and there may be a thickening of the skin. Individuals with Keratomalacia may develop anemia and an increased susceptibility to infection. Growth retardation is a common symptom in children. Causes Keratomalacia is most commonly caused by prolonged dietary deprivation of sources of vitamin A. It occurs most frequently in areas such as southern and eastern Asia where rice is the staple (rice does not contain the necessary dietary pigment, carotene, which the body converts to vitamin A). A secondary cause of Keratomalacia may be due to the body's inadequate conversion of carotene to vitamin A. Keratomalacia may also be caused by an interference with absorption, storage or transport of vitamin A. Interference with absorption or storage of vitamin A may be associated with digestive diseases (sprue), cystic fibrosis, surgery of the pancreas or the small intestine (duodenal bypass), obstruction of the small intestine, obstruction of the bile ducts, chronic diarrhea due to giardiasis and cirrhosis of the liver. In the United States vitamin A deficiency in adults is most likely to be secondary to severe malabsorption of the vitamin, or to eating disorders such as Anorexia Nervosa. Affected Population Keratomalacia affects males and females in equal numbers. It occurs most commonly in developing countries in the Far East and India, affecting poorly nourished infants and adults. Related Disorders Symptoms of the following disorders can be similar to those of Keratomalacia. A comparison may be useful for a differential diagnosis: Sjogren's Syndrome is an autoimmune disorder characterized by abnormally dry eyes and mouth. Membrane dryness may be widespread involving the nose, esophagus, trachea, upper gastrointestinal tract and the vagina. (For more information on this disorder, choose "Sjogren's Syndrome" as your search term in the Rare Disease Database). Keratoconus is a disorder characterized by slow and progressive expansion of the cornea of the eye. The abnormal cone shape that the cornea develops causes major changes in vision, and may require frequent changes in eyeglasses or contact lenses. It may progress to blindness. However, a cornea transplant can cure this eye disease. (For more information on this disorder, choose "Keratoconus" as your search term in the Rare Disease Database). Interstitial Keratitis is a disorder characterized by a chronic inflammation of the deep layers of the cornea. It rarely occurs in the United States. Symptoms may include painful sensitivity to light (photophobia), pain in the eyes, an excess secretion of tears (lacrimation) and a gradual loss of vision. Interstitial Keratitis most commonly affects children and is a late complication of congenital syphilis. Bullous Keratopathy is a disorder characterized by excessive fluid accumulation in the cornea. Symptoms may include pain in the eyes and decreased vision. Bullous Keratopathy most frequently affects the elderly and occasionally occurs after surgery for some other eye problem such as cataracts. Therapies: Standard In treating individuals with Keratomalacia, the cause must be corrected. When dietary deficiency of vitamin A is the cause, Vitamin A supplements must be administered. However, vitamin A can cause birth defects if given in high doses to pregnant women, so care must be taken in treating women of child bearing years. In general, individuals with Keratomalacia respond rapidly to vitamin A replacement therapy if the case is treated early. Dry eyes may be treated with artificial tears. If the eye complications have progressed to the most severe stage (blindness), little can be done to restore vision. Antibiotic sulfonamide ointments may be used for secondary infections. In mild cases of night blindness improvement is often shown following the administration of vitamin A or as a fish liver oil. More advanced cases may require higher doses daily for several months. Large doses of vitamin A should be monitored by a physician to avoid possible overdose. If Keratomalacia is caused by an interference of absorption, storage or transport of vitamin A, the underlying disorder must be treated. Therapies: Investigational This disease entry is based upon medical information available through July 1990. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Keratomalacia, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Eye Institute 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5248 Eye Research Institute of Retina Foundation 20 Staniford St. Boston, MA 02114 (617) 742-3140 National Society to Prevent Blindness 79 Madison Avenue New York, NY 10016 (212) 684-3505 Vision Foundation, Inc. 818 Mt. Auburn Street Watertown, MA 02172 (617) 926-4232 1-800-852-3029 References INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and Co., 1987. Pp. 282, 1264. THE MERCK MANUAL, Volume 1, 14th Ed.: Robert Berkow, M.D., ed.-in-chief; Merck Sharp & Dohme Laboratories, 1982. Pp. 922-923. IMPRESSION CYTOLOGY: A PRACTICAL INDEX OF VITAMIN A STATUS. G. Natadisastra et al.; AM J CLIN NUTR (September, 1988: issue 48(5)). Pp. 426- 429. VITAMIN A FORTIFIED MONOSODIUM GLUTAMATE AND HEALTH, GROWTH, AND SURVIVAL OF CHILDREN: A CONTROLLED FIELD TRIAL. Muhilal et al.; AM J CLIN NUTR (November, 1988: issue 48(5)). Pp. 1271-1276.