Keratomalacia results from vitamin A deficiency and is characterized by a dry and hazy cornea. It is typically found in patients suffering from protein-calorie malnutrition.
Presentation
Vitamin A is one of the fat-soluble and essential vitamins that is acquired through dietary intake. A normal amount of vitamin A is necessary to maintain a healthy skin, immune system, retina, and ocular surface [1].
Vitamin A deficiency presents with different signs and symptoms. The ocular presentations are variable and potentially affect all epithelial cells of the eyes. These features may range from simple conjunctival and corneal dryness to xerosis, keratomalacia, scarring of the cornea, and perforation. The resulting visual impairment can lead to dysfunctional dark adaptation, night blindness and damage to the pigment epithelial cells of the photoreceptors [2].
The direct cause of xerophthalmia is hypovitaminosis A. Active corneal xerophthalmia may be observed in severe famines, otherwise, it is an extremely rare occurrence in older children or adults. This condition is considered a medical emergency and requires prompt management using high doses of vitamin A supplementation [3] [4].
Keratomalacia is the most severe form of xerophthalmia. More than one-third of the cornea is involved in this case. The cornea can be thickened and edematous and eventually melt away because of necrosis in the structure of collagen in the cornea. This process may only take a few days.
Children affected by keratomalacia are usually malnourished. However, previously healthy children may develop keratomalacia after episodes of diarrhea or measles infection in which vitamin A stores are rapidly depleted as a result of increased metabolism. Generally, these patients were deficient in vitamin A and the infection triggered further depletion of vitamin A stores [4] [5].
An incomplete immunization can predispose children to diseases like measles, thereby increasing the risk of Vitamin A deficiency and subsequent corneal ulceration [6].
Nyctalopia is often the first presenting feature and results from rod dysfunction. Cone dysfunction occurs later and to a lesser extent than rod dysfunction [7].
Workup
In children younger than 12 years of age, a serum retinol of less than 0.7 mg/L is considered low. This test is expensive but a direct measure of serum retinol values. A serum retinol-binding protein (RBP) study is not as costly and easier to perform than the serum retinol test since RBP can be detected by an immunologic assay. RBP is more stable with respect to temperature and light but its levels are less accurate than retinol levels because the RBP types cannot be differentiated and the values are affected by serum protein concentrations [8] [9] [10] [11].
Electroretinography (ERG) may be used to confirm and monitor retinopathy. Dark adaptometry is used to evaluate night blindness. The test is subjective but malingering and poor cooperation can easily be detected. Adaptometry is a complement to ERG since it is a focal test and therefore can be more sensitive than an ERG in some instances. Dark adaptometry is useful in the evaluation of cone dysfunction syndromes by showing degrees of cone adaptation.
Treatment
The primary treatment for keratomalacia is the administration of vitamin A supplements to address the deficiency. This can be done orally or through injections, depending on the severity of the condition. In addition to vitamin supplementation, supportive care for the eyes is essential. This may include the use of lubricating eye drops to relieve dryness and protect the cornea. In cases where corneal ulcers have developed, antibiotic eye drops may be prescribed to prevent infection. Severe cases might require surgical intervention, such as corneal transplantation.
Prognosis
The prognosis for keratomalacia largely depends on the stage at which treatment is initiated. Early intervention with vitamin A supplementation can lead to significant improvement and prevent further damage. However, if the condition is advanced and the cornea has been severely damaged, the prognosis may be poor, with a risk of permanent vision loss. Timely treatment and addressing the underlying vitamin A deficiency are crucial for a favorable outcome.
Etiology
Keratomalacia is primarily caused by a deficiency of vitamin A, an essential nutrient for maintaining healthy vision. Vitamin A is vital for the production of rhodopsin, a pigment in the retina that helps with low-light vision. A deficiency can result from inadequate dietary intake, malabsorption disorders, or increased physiological needs, such as during pregnancy or lactation. Populations with limited access to vitamin A-rich foods, such as fruits, vegetables, and animal products, are at higher risk.
Epidemiology
Keratomalacia is more prevalent in developing countries where malnutrition and vitamin A deficiency are common. It primarily affects children and pregnant women, who have higher nutritional needs. The World Health Organization estimates that vitamin A deficiency affects millions of children worldwide, with a significant number at risk of developing keratomalacia. Efforts to improve nutrition and provide vitamin A supplementation have been crucial in reducing the incidence of this condition.
Pathophysiology
The pathophysiology of keratomalacia involves the depletion of vitamin A, which is essential for maintaining the health of epithelial tissues, including the cornea. Vitamin A deficiency leads to the breakdown of the corneal epithelium, resulting in dryness and keratinization (the formation of a hard, protective layer). This compromises the cornea's integrity, leading to softening and ulceration. The lack of vitamin A also impairs the production of mucin, a component of tears, exacerbating dryness and damage.
Prevention
Preventing keratomalacia involves ensuring adequate intake of vitamin A through a balanced diet rich in fruits, vegetables, and animal products. Public health initiatives, such as vitamin A supplementation programs, have been effective in reducing the incidence of vitamin A deficiency in at-risk populations. Educating communities about the importance of nutrition and providing access to fortified foods can also play a significant role in prevention.
Summary
Keratomalacia is a serious eye condition caused by vitamin A deficiency, leading to corneal softening and potential blindness. Early symptoms include night blindness and dry eyes, progressing to corneal ulcers if untreated. Diagnosis involves clinical evaluation and blood tests to confirm vitamin A deficiency. Treatment focuses on vitamin A supplementation and supportive eye care. Prevention through adequate nutrition and public health measures is crucial in reducing the incidence of this condition.
Patient Information
For patients, understanding keratomalacia is important for recognizing early symptoms and seeking timely treatment. It is a condition caused by a lack of vitamin A, which is essential for eye health. Symptoms like difficulty seeing at night and dry eyes should not be ignored. Ensuring a diet rich in vitamin A, found in foods like carrots, sweet potatoes, and liver, can help prevent this condition. If you experience any symptoms, consult a healthcare provider for evaluation and possible vitamin A supplementation.
References
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- Rubino P, Mora P, Ungaro N, et al. Anterior Segment Findings in Vitamin A Deficiency: A Case Series. Case Rep Ophthalmol Med. 2015;2015.
- Arunachalam C, Hegde V, Jain R, D′Souza N. An unusual ocular presentation of acquired immune deficiency syndrome. Indian J. Ophthalmol. 2008;56(5):434-436.
- Ross DA. Recommendations for vitamin A supplementation. J Nutr. 2002;132:2902S–6S.
- Gilbert C. The eye signs of vitamin A deficiency. Comm Eye Health. 2013;26(84):66-67.
- Vajpayee RB, Ray M, Panda A, et al. Risk factors for pediatric presumed microbial Keratitis a case-control study. Cornea. 1999;18:565–9.
- Roncone DP. Xerophthalmia secondary to alcohol-induced malnutrition. Optometry. 2006;77:124–133.
- Gomes MM, Saunders C, Ramalho A. Placenta: a possible predictor of vitamin A deficiency. Br J Nutr. 2009;15:1-5.
- de Pee S, Dary O. Biochemical indicators of vitamin A deficiency: serum retinol and serum retinol binding protein. J Nutr. 2002;132(9 Suppl):2895S-901S.
- Weinman AR, Jorge SM, Martins AR, et al. Assessment of vitamin A nutritional status in newborn preterm infants. Nutrition. 2007;23(6):454-60.
- Gorstein JL, Dary O, Pongtorn, et al. Feasibility of using retinol-binding protein from capillary blood specimens to estimate serum retinol concentrations and the prevalence of vitamin A deficiency in low-resource settings. Public Health Nutr. 2008;11(5):513-20.