Keratomalacia in a young diabetic: A tracer for a hidden systemic condition

Jyoti Shakrawal; Kavita R Bhatnagar; Nikhil Agarwal; Falguni Roy

doi:10.4103/ijo.IJO_2151_22

CASE REPORT

Year : 2023 | Volume : 3 | Issue : 2 | Page : 302-303

Keratomalacia in a young diabetic: A tracer for a hidden systemic condition

Jyoti Shakrawal, Kavita R Bhatnagar, Nikhil Agarwal, Falguni Roy
Department of Ophthalmology, All India Institute of Medical Sciences (AIIMS), Jodhpur, Rajasthan, India

Date of Submission	27-Aug-2022
Date of Acceptance	08-Feb-2023
Date of Web Publication	28-Apr-2023

Correspondence Address:
Jyoti Shakrawal
Department of Ophthalmology, All India Institute of Medical Sciences, Jodhpur, Rajasthan - 342 005
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2151_22

Abstract

A 38-year-old male presented with complaints of gradual and painless diminution of vision in both the eyes for 1 month. He also had a history of on and off diarrhea for the past 4 months. He was a known case of latent auto-immune diabetes mellitus. Ophthalmic examination showed bilateral conjunctival xerosis and inferior corneal ulceration with stromal edema and Descemet's folds. With the suspicion of vitamin A deficiency-induced keratomalacia, a complete workup was performed. He turned out to be a case of chronic calcific pancreatitis, and stool test was also positive for steatorrhea. The patient was started on oral vitamin A supplementation. Topical lubricants and prophylactic antibiotics were also started in both the eyes with the application of bandage contact lens. Visual acuity improved within a period of 2–3 weeks of treatment and is now on regular follow-up.

Keywords: Keratomalacia, pancreatitis, vitamin A deficiency

How to cite this article:
Shakrawal J, Bhatnagar KR, Agarwal N, Roy F. Keratomalacia in a young diabetic: A tracer for a hidden systemic condition. Indian J Ophthalmol Case Rep 2023;3:302-3

How to cite this URL:
Shakrawal J, Bhatnagar KR, Agarwal N, Roy F. Keratomalacia in a young diabetic: A tracer for a hidden systemic condition. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 5];3:302-3. Available from: https://www.ijoreports.in/text.asp?2023/3/2/302/374941

Vitamin A deficiency (VAD)-associated visual impairment is common among children in developing countries usually associated with a prolonged near-starvation diet. However, keratomlacia in adults is rare and associated to secondary VAD because of chronic malabsorption-related disorders.^[1],[2],[3] We here report a rare case of bilateral keratomalacia in a young patient of latent auto-immune diabetes mellitus, which revealed a hidden chronic pancreatitis. So far, this has not been previously reported.

Case Report

A 38-year-old male presented with a complaint of painless progressive diminution of vision in both the eyes for the past 1 month, which was more pronounced at night time. His best-corrected visual acuity (BCVA) on presentation was 0.1 decimal in both the eyes. The intra-ocular pressures were 12 and 14 mm of Hg with non-contact tonometry in right eye (RE) and left eye (LE), respectively. On slit-lamp examination, the ocular surface was dry in appearance with a tear film break-up time of less than 2 seconds and Schirmer's test less than 5 mm in both the eyes. On further evaluation, bilateral conjunctival xerosis was present. A sterile corneal ulcer of a size measuring 3.5 × 1.5 mm in RE and 2.5 × 1.5 mm in LE was present inferiorly, associated with stromal edema and Descemet's folds [Figure 1]a and [Figure 1]b. However, there were no signs of inflammation in anterior chamber. Bilaterally, the lens was clear and the posterior segment was within normal limits on indirect ophthalmoscopy. The NEI (National Eye Institute) grading was 3/15 for cornea and 2/18 for conjunctiva in both the eyes. No microorganism growth was seen on corneal scrappings. Serum vitamin A levels were reduced with a value of 0.08 μmol/L. A provisional clinical diagnosis of keratomalacia was made.

Figure 1: (a) Right eye showing conjunctival xerosis and a corneal ulcer of 3.5 × 1.5 mm (black arrow) along with stromal edema and Descemet's folds. (b) Left eye showing conjunctival and corneal xerosis and a corneal ulcer of 2.5 × 1.5 mm (white arrow) along with stromal edema and Descemet's folds. (c and d) Right eye and left eye showing corneal keratinization along with reduced epithelial defect, resolved corneal edema, and Descemet's folds

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He was a diagnosed case of insulin-dependent diabetes mellitus and was on insulin for the past 18 years. He also had a history of chronic diarrhea for 4 months. In view of this history, he was systemically evaluated by a gastroenterologist and endocrinologist. HbA1c was raised to 11.6, which suggested poorly controlled sugar levels. Stool test was also positive for steatorrhea. Contrast-enhanced computed tomography abdomen was also performed. He was diagnosed with chronic pancreatic.

Therefore, the characteristic ocular features associated with chronic pancreatic-induced exocrine insufficiency led to the final diagnosis of VAD xerophthalmia.

Bandage contact lenses were applied in both the eyes, and topical lubricants [e/d hydroxypropylmethycellulose (0.3%) 2 hourly, e/oint hydroxypropylmethylcellulose 2% BD+HS] with prophylactic antibiotics to prevent super-added infection were started. He had also received vitamin A supplementation of 200,000 IU for 2 days, which was repeated after 2 weeks. He was also started on multi-vitamin supplementation. Insulin was titrated according to blood sugar levels.

On routine follow-up of every 3 days with a change in bandage contact lens on each visit, the epithelial defect had reduced in size. Over a period of 3 weeks, the BCVA had improved to 0.3 decimals in BE with corneal keratization along with a reduced epithelial defect size, 2 × 1.5 mm in RE and 1.5 × 1 mm in LE [Figure 1]c and [Figure 1]d. The patient was continued with vitamin A supplementation, along with intense lubrication.

Discussion

VAD being the leading cause of preventable blindness in developing countries can be prevented by early diagnosis and proper vitamin A supplementation. The morbidity and mortality of primary VAD are more predominant in children in impoverished regions throughout the world. However, adults may present with ocular manifestation of secondary VAD in cases of chronic indigestion or malabsorption arising because of pancreatic, liver, or intestinal pathology.^[1],[2],[3] This may be further complicated by low vitamin A dietary intake. Severe keratomalacia and corneal ulceration have been documented after acute or chronic pancreatitis, but both patients had a history of chronic alcoholism.^[1],[3] Our case was a non-alcoholic with the only significant history of chronic diarrhea. Cheshire et al.^[2] reported VAD as an ophthalmic manifestation of failed renal transplant, chronic pancreatitis, and diabetes mellitus. VAD has also been previously reported after pancreatic resection or intestinal bypass surgeries.^[4],[5] Our case presented to us with VAD, which revealed a hidden abdominal pathology later, for which systemic management was also started.

VAD starts with night blindness, but as the deficiency progresses and the serum retinol level falls below 10 mg/ml, the sign of xeropthalmia begins to develop.^[6] Xerophthalmia is a spectrum of ocular disorder caused by VAD characterized by a range of ocular manifestations from conjuctival xerosis (dry eye) to corneal ulceration and keratomalacia (necrosis and softening of the cornea, leading to its perforation) and if left untreated leads to blindness. The diagnosis of VAD is mainly clinical; however, serum vitamin A levels can be requested to confirm the diagnosis, but treatment should not be delayed. An oral dose of 200,000 IU of vitamin A for 2 days followed by another dose 1–4 weeks later is recommended by World Health Organization.^[7]

Our patient had latent auto-immune diabetes mellitus associated with undiagnosed chronic calcified pancreatitis, which resulted in exocrine insufficiency and inadequate production of hydrolysis enzymes. This led to chronic malabsorption of fat-soluble vitamins, especially vitamin A. As a result, he developed keratomalacia, which was preceded by a history of night blindness. The patient improved significantly after vitamin A supplementation and oral enzymes.

Conclusion

Our case is unique as it highlights the importance of early recognition of vitamin A deficiency in young patients to save vision with possible suspicion of gastro-intestinal disorders for which a multi-disciplinary approach is mandatary. Early identification and management may preserve the sight of the patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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