CHAPTER

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Systemic Ophthalmology
Disorders of skin and mucous membranes Haematological diseases OCULAR ABNORMALITIES IN TRISOMIES ADVERSE OCULAR EFFECTS OF COMMON SYSTEMIC DRUGS

OCULAR MANIFESTATIONS OF SYSTEMIC DISEASES Introduction Nutritional deficiences Xerophthalmia Systemic infections Metabolic disorders

OCULAR MANIFESTATIONS OF SYSTEMIC DISEASES

INTRODUCTION

Ocular involvement in systemic disorders is quite frequent. It is imperative for the ophthalmologists as well as physicians to be well conversant with these. Many a time, the ocular manifestations may be the presenting signs and the ophthalmologist will refer the patient to the concerned specialist for diagnosis and/or management of the systemic disease. While, in other cases the opinion for ocular involvement may be sought for by the physician who knows to look for it. Ocular lesions of the common systemic disorders are enumerated and a few important ones are described here.
OCULAR MANIFESTATIONS OF NUTRITIONAL DEFICIENCES

the eyes due to conjunctival irritation and vascularisation of the cornea. 4. Deficiency of vitamin C. It may be associated with haemorrhages in the conjunctiva, lids, anterior chamber, retina and orbit. It also delays wound healing. 5. Deficiency of vitamin D. It may be associated with zonular cataract, papilloedema and increased lacrimation.
XEROPHTHALMIA

They term xerophthalmia is now reserved (by a joint WHO and USAID Committee, 1976) to cover all the ocular manifestations of vitamin A deficiency, including not only the structural changes affecting the conjunctiva, cornea and occasionally retina, but also the biophysical disorders of retinal rods and cones functions.
Etiology

1. Deficiency of vitamin A. Ocular manifestations of vitamin A deficiency are referred to as xerophthalmia. 2. Deficiency of vitamin B1 (thiamine). It can cause corneal anaesthesia, conjunctival and corneal dystrophy and acute retrobulbar neuritis. 3. Deficiency of vitamin B2 (riboflavin). It can produce photophobia and burning sensation in

It occurs either due to dietary deficiency of vitamin A or its defective absorption from the gut. It has long been recognised that vitamin A deficiency does not occur as an isolated problem but is almost invariably accompanied by protein-energy malnutrition (PEM) and infections.
WHO classification (1982)

The new xerophthalmia classification (modification of original 1976 classification) is as follows:

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XN X1A X1B X2 X3A X3B XS XF

Night blindness Conjunctival xerosis Bitots spots Corneal xerosis Corneal ulceration/keratomalacia affecting less than one-third corneal surface Corneal ulceration/keratomalacia affecting more than one-third corneal surface. Corneal scar due to xerophthalmia Xerophthalmic fundus.

Clinical features

1. X N (night blindness). It is the earliest symptom of xerophthalmia in children. It has to be elicited by taking detailed history from the guardian or relative. 2. X1A (conjunctival xerosis). It consists of one or more patches of dry, lustreless, nonwettable conjunctiva (Fig. 19.1), which has been well described as emerging like sand banks at receding tide when the child ceases to cry. These patches almost always involve the inter-palpebral area of the temporal quadrants and often the nasal quadrants as well. In more advanced cases, the entire bulbar conjunctiva may be affected. Typical xerosis may be associated with conjunctival thickening, wrinkling and pigmentation.

Fig. 19.2. Xerophthalmia, stage XIB: Bitot spots.

4. X2 (corneal xerosis). The earliest change in the cornea is punctate keratopathy which begins in the lower nasal quadrant, followed by haziness and/or granular pebbly dryness (Fig. 19.3). Involved cornea lacks lustre. 5. X3A and X3B (corneal ulceration/keratomalacia), Stromal defects occur in the late stage due to colliquative necrosis and take several forms. Small ulcers (1-3 mm) occur peripherally; they are characteristically circular, with steep margins and are sharply demarcated (Fig. 19.4). Large ulcers and areas of necrosis may extend centrally or involve the entire cornea. If appropriate therapy is instituted immediately, stromal defects involving less than one-third of corneal surface (X3A) usually heal, leaving some useful vision. However, larger stromal defects (X3B) (Fig. 19.5) commonly result in blindness.

Fig. 19.1. Xerophthalmia, stage XIA: Conjunctival xerosis.

3. X1B (Bitots spots). It is an extension of the xerotic process seen in stage X1A. The Bitots spot is a raised, silvery white, foamy, triangular patch of keratinised epithelium, situated on the bulbar conjunctiva in the inter-palpebral area (Fig. 19.2). It is usually bilateral and temporal, and less frequently nasal.

Fig. 19.3. Xerophthalmia, stage X2: Corneal xerosis.

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Fig. 19.4. Xerophthalmia, stage X3A: Keratomalacia involving less than one-third of corneal surface.

Fig. 19.6. Xerophthalmia, stage XS: Corneal scars.

Fig. 19.5. Xerophthalmia, stage X3B: Keratomalacia involving more than one-third of corneal surface.

Fig. 19.7. Xerophthalmia, stage XF: Xerophthalmic fundus.

6. XS (corneal scars). Healing of stromal defects results in corneal scars of different densities and sizes which may or may not cover the pupillary area (Fig. 19.6). A detailed history is required to ascertain the cause of corneal opacity. 7. XFC (Xerophthalmic fundus). It is characterized by typical seed-like, raised, whitish lesions scattered uniformly over the part of the fundus at the level of optic disc (Fig. 19.7).
Treatment

2. Vitamin A therapy. Treatment schedules apply to all stages of active xerophthalmia viz. XN, X1A, X1B, X2, X3A and X3B. Oral administration is the recommended method of treatment. However, in the presence of repeated vomiting and severe diarrhoea, intramuscular injections of water-miscible preparation should be preferred. The WHO recommended schedule is as given below: i. All patients above the age of 1 year (except women of reproductive age): 200,000 IU of vitamin A orally or 100,000 IU by intramuscular injection should be given immediately on diagnosis and repeated the following day and 4 weeks later. ii. Children under the age of 1 year and children of any age who weigh less than 8 kg should be treated with half the doses for patients of more than 1 year of age.

It includes local ocular therapy, vitamin A therapy and treatment of underlying general disease. 1. Local ocular therapy. For conjunctival xerosis artificial tears (0.7 percent hydroxypropyl methyl cellulose or 0.3 percent hypromellose) should be instilled every 3-4 hours. In the stage of keratomalacia, full-fledged treatment of bacterial corneal ulcer should be instituted (see pages 120-123).

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iii. Women of reproductive age, pregnant or not: (a) Those having night blindness (XN), conjunctival xerosis (X1A) and Bitots spots (X1B) should be treated with a daily dose of 10,000 IU of vitamin A orally (1 sugar coated tablet) for 2 weeks. (b) For corneal xerophthalmia, administration of full dosage schedule (described for patients above 1 year of age) is recommended. 3. Treatment of underlying conditions such as PEM and other nutritional disorders, diarrhoea, dehydration and electrolyte imbalance, infections and parasitic conditions should be considered simultaneously.
Prophylaxis against xerophthalmia

2. Medium-term approach. It includes food fortification with vitamin A. 3. Long-term approach. It should be the ultimate aim. It implies promotion of adequate intake of vitamin A rich foods such as green leafy vegetables, papaya and drum- sticks (Fig. 19.8). Nutritional health education should be included in the curriculum of school children.

The three major known intervention strategies for the prevention and control of vitamin A deficiency are: 1. Short-term approach. It comprises periodic administration of vitamin A supplements. WHO recommended, universal distribution schedule of vitamin A for prevention is as follows: i. Infants 6-12 100,000 IU orally every months old and 3-6 months. any older children who weigh less than 8 kg. ii. Children over 200,000 IU orally every 1 year and under 6 months. 6 years of age iii. Lactating 20,000 IU orally once at mothers delivery or during the next 2 months. This will raise the concentration of vitamin A in the breast milk and therefore, help to protect the breastfed infant. iv. Infants less 50,000 IU orally should than 6 months be given before they old, not being attain the age of 6 breastfed. months. A revised schedule of vitamin A supplements being followed in India since August 1992, under the programme named as Child Survival and Safe Motherhood (CSSM) is as follows: First dose (1 lakh I.U.)at 9 months of age along with measles vaccine. Second dose (2 lakh I.U.)at 18 months of age along with booster dose of DPT/OPV. Third dose (2 lakh I.U.)at 2 years of age.

Fig. 19.8. Rich sources of vitamin A.

Note. The short-term approach has been mostly in vogue especially in Asia. The best option perhaps is a combination of all the three methods with a gradual weaning away of the short-term approach. OCULAR MANIFESTATIONS OF SYSTEMIC INFECTIONS
A. VIRAL INFECTIONS Measles. Ocular lesions are: catarrhal conjunctivitis,

Kopliks spots on conjunctiva, corneal ulceration, optic neuritis and retinitis. Mumps. Ocular involvement may occur as conjunctivitis, keratitis, acute dacryoadenitis and uveitis. Rubella. Ocular lesions seen in rubella (German measles) are congenital microphthalmos, cataract, glaucoma, chorioretinitis and optic atrophy. Whooping cough. There may occur subconjunctival haemorrhages and rarely orbital haemorrhage leading to proptosis.
Ocular involvement in AIDS

AIDS (Acquired Immune Deficiency Syndrome) is caused by Human immunodeficiency virus (HIV) which is an RNA retrovirus.