Journal of Pediatric Ophthalmology and Strabismus

Short Subjects 

Bilateral Keratomalacia Leading to Blindness Secondary to Diet-Induced Vitamin A Deficiency in Infants

Meenakshi Wadhwani, MS; Rahul Singh, MS

Abstract

Vitamin A is a fat-soluble vitamin that plays an important role in the development of the immune system, and a deficiency can cause blindness associated with xerophthalmia and premature mortality. Blindness from vitamin A deficiency is avoidable if supplements such as breast milk and other food fortification are provided to neonates at the time of weaning. The authors describe two infants who presented with keratomalacia and subsequent blindness. [J Pediatr Ophthalmol Strabismus. 2020;57:e12–e14.]

Abstract

Vitamin A is a fat-soluble vitamin that plays an important role in the development of the immune system, and a deficiency can cause blindness associated with xerophthalmia and premature mortality. Blindness from vitamin A deficiency is avoidable if supplements such as breast milk and other food fortification are provided to neonates at the time of weaning. The authors describe two infants who presented with keratomalacia and subsequent blindness. [J Pediatr Ophthalmol Strabismus. 2020;57:e12–e14.]

Introduction

Vitamin A deficiency occurs in nutritionally deprived children in developing countries despite the international and national programs involved with controlling this public health problem. In 2009, the World Health Organization's global estimate indicated that 5.2 million preschool aged children had night blindness, 190 million preschool aged children had low serum retinol concentrations (< 0.70 µmol/l), and approximately 7 million pregnant women had vitamin A deficiency.1,2 Vitamin A is a fat-soluable vitamin whose function is important to the visual cycle of the retinal pigment epithelium. It also plays a significant role in the growth of the epithelium and differentiation of the limbal cells.3

The first ophthalmic symptom of vitamin A deficiency is nyctalopia (a disorder of vision in twilight) and the second is xerophthalmia (a pronounced drying of the anterior segment). However, the pathology may progress to severe keratomalacia with perforation of the cornea if the patient's diet does not improve.3 Xerophthalmic fundus is a specific and rare finding characterized by yellowish stains on the periphery of the ocular fundus.4

Vitamin A deficiency is associated with an increased risk of illnesses, including diarrheal diseases, measles, and respiratory tract infections, all of which are associated with an increased incidence of mortality for children in low- and middle-income countries.4,5 Malnutrition can decrease the body's absorption and increase the body's loss of vitamin A, thus worsening the existing deficiency and increasing the risk of infection and mortality.6 We describe two infants who presented to our hospital with bilateral keratomalacia leading to blindness secondary to diet-induced vitamin A deficiency.

Case Reports

A limitation of this report is that laboratory facilities for serum retinal level and conjunctival impression cytology were not available.

Case 1

A 9-month-old female infant presented to our outpatient department with a whitish discoloration in both eyes, which had been present for 1 week. There was no history of trauma or surgery. On general examination, the patient was severely malnourished and had dry skin. On ocular examination, she was not fixing and following at torch light and her ocular surface was dry. There were matted cilia, a dry and wrinkled conjunctival surface, and no tear meniscus in both eyes. There was also a bilaterally melted cornea with prolapsed uveal tissue. The right cornea was perforated superiorly with uveal tissue prolapse and edematous inferiorly. The remaining peripheral corneal rim was thin, dry, and lusterless bilaterally. The deep structures were not visible in both eyes.

After a full clinical work-up and investigation, she was diagnosed as having bilateral keratomalacia (stage X3B) secondary to nutritional vitamin A deficiency (Figure 1). Although the child had not been fed breast milk since birth, she had nutritional supplementation in the form of toned milk. Her parents had a low income and belonged to a low socioeconomic class. Her immunization history was not available. Her treatment included oral vitamin A supplements (100,000 IU on the 1st, 2nd, and 14th day) and topical lubricating drops, ointments, and prophylactic antibiotics.

Photograph of case 1 showing keratomalacia in both eyes at (A) 4 and (B) 9 months of age.

Figure 1.

Photograph of case 1 showing keratomalacia in both eyes at (A) 4 and (B) 9 months of age.

Case 2

A 2.5-month-old male infant presented to our hospital's pediatric emergency department with complaints of difficulty breathing for 6 days and whitish discoloration in both eyes. He had no symptoms 2 weeks prior. The patient was diagnosed as having sepsis with pneumonia and keratomalacia in both eyes (Figure 2). The findings from the general and ocular examinations were the same as the findings in case 1. A corneal ulcer was found in both eyes and perforation of the cornea was impending in the left eye. In addition to malnutrition, systemic disease also aggravated his vitamin A deficiency. Similar to case 1, his parents also belonged to a low socioeconomic class. He was severely malnourished and was not fed breast milk because, according to his parents, his mother was also malnourished so breast milk formation was minimal. The patient's treatment started similarly to the treatment in case 1 and included systemic antibiotics and antipyretics.

Photograph of case 2 showing keratomalacia in both eyes, with impending perforation of the cornea in the left eye.

Figure 2.

Photograph of case 2 showing keratomalacia in both eyes, with impending perforation of the cornea in the left eye.

Discussion

The factors responsible for increasing vulnerability to vitamin A deficiency at specific stages of life are characterized by increased growth requirements, including infancy, childhood, and pregnancy. Vitamin A deficiency in neonates is associated with lack of access to breast milk or insufficient levels of vitamin A in breast milk. Following weaning, vitamin A deficiency usually results from a chronic dietary shortage of vitamin A, a reduced intestinal absorption of vitamin A, or an increased use of vitamin A as occurs in various infections, leading to the depletion of vitamin A levels in the liver, tissue, and plasma. In addition to being an immunomodulator, vitamin A is also required for proper functioning of several organ systems (eg, musculoskeletal, eye, gastrointestinal, liver, and kidney).

Vitamin A deficiency is a primary cause of corneal blindness in childhood in nutritionally deprived populations. Blindness from vitamin A deficiency is associated with a high mortality rate in children younger than 5 years and it is underreported. A study on childhood blindness in India reported that vitamin A deficiency accounted for 22% of cases (in addition to measles) that led to severe visual impairment or blindness.7

Infant feeding practices are correlated with the development of vitamin A deficiency. The development of xerophthalmia is inversely proportional to the consumption of diets rich in vitamin A. Breast feeding is an important protective factor because breast milk contains vitamin A and prevents the child from developing infections. Parental awareness and education regarding the importance of breast milk should also be increased.

Because our patients were from extremely poor socioeconomic statuses and had poor nutritional histories, this severe form of xerophthalmia might have developed from a dietary deficiency.8,9 Based on the characteristic conjunctival and corneal findings, malnourished general appearance of the patient, poor nutritional history, higher prevalence of vitamin A deficiency in India, and absence of any other local and systemic conditions to explain the ocular findings, both patients were diagnosed as having xerophthalmia stage X3B (keratomalacia).

Although the incidence of vitamin A deficiency has decreased, strong networking between gynecologists, ophthalmologists, and pediatricians is required to counsel parents about breast feeding, antenatal nutrition of the mother, and proper and timely treatment of this entity leading to blindness in pre-school aged children.

References

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Authors

From the Department of Ophthalmology, Chacha Nehru Bal Chikitsalya, Geeta Colony, New Delhi, India.

The authors have no financial or proprietary interest in the materials presented herein.

Correspondence: Meenakshi Wadhwani, MS, Chacha Nehru Bal Chikitsalaya, Geeta Colony, New Delhi 110031, India. E-mail: mkgang08@gmail.com

Received: June 17, 2019
Accepted: October 03, 2019
Posted Online: March 12, 2020

10.3928/01913913-20191210-01

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