Chapter 98 My child’s eyes are dry and sore
Introduction
The eye is protected against damage and infection by the lids and by a lubricating tear film. The tear film consists of three layers: mucous (produced by conjunctival goblet cells), aqueous (secreted by the lacrimal glands), and lipid (secreted by the meibomian glands). Dysfunction in any layer of the tear film can result in ocular surface drying and damage. Dry eye disease may be categorized as either aqueous deficient (caused by disorders affecting the lacrimal gland) or evaporative (due to meibomian gland dysfunction or abnormalities of the lid, lid closure and globe leading to exposure).1 Ocular dryness results in increased osmolarity of the tear film and inflammation of the ocular surface. The presence of corneal anesthesia exacerbates the effects of drying and exposure and puts the eye at risk of defective epithelial healing, corneal ulceration, and perforation.
Dry eye in childhood is underdiagnosed and whilst a severely dry and ulcerated eye is easily recognized, milder forms may go unrecognized. Many cases of dry eye have a simple and easily identified explanation, e.g. a lid notch or, most commonly, secondary to allergic eye disease. However, a dry eye in childhood may be the sign of a rare, but serious systemic disorder, and the ophthalmologist should be alert to this possibility.2 Tables 98.1 and 98.2 give details of ocular and systemic diseases/conditions associated with dry eye in children.
Table 98.1 Ocular disease causing dry eye
| Allergic eye disease | Papillae, giant papillae, corneal erosions |
| Anesthetic cornea from corneal disease | Damage to trigeminal nerve, e.g. herpes simplex, herpes zoster |
| Aniridia | Abnormal tear film stability and meibomian gland dysfunction |
| Chemical burns | Conjunctival scarring |
| Congenital alacrima | Primary form limited to lacrimal gland (also occurs as part of syndromes) |
| Contact lens wear | Reduced tear film volume |
| Dacryoadenitis | Secondary to infection, e.g. Epstein-Barr virus |
| Incomplete lid closure, proptosis, facial palsy | Intensive care patients, VII palsy, shallow orbits, orbital tumors |
| Lid margin disease | Telangiectatic lid margin vessels, scaly debris in lashes, blocked meibomian glands |
| Lid notch | Allows evaporation of tear film |
| Ocular surface abnormalities | Conjunctival scarring (including strabismus surgery), dermolipomas, corneal dellen |
| Post-ptosis surgery | Incomplete lid closure, aggravated by reduced Bell’s or reduced upgaze |
| Reduced blink rate | May be associated with prolonged computer or electronic games use |
| Topical drug therapy | Preservatives can cause conjunctival irritation and dryness |
Table 98.2 Systemic disease causing dry eye
| Allgrove’s syndrome | Triple A syndrome: adrenocorticoid deficiency, achalasia of the cardia, alacrima |
| Autoimmune polyendocrinopathy syndrome type 1 | Reduced tear production, hypoparathyroidism, mucocutaneous candidiasis, adrenocortical insufficiency |
| Blepharophimosis syndrome | Absent lacrimal glands giving alacrima |
| Chronic renal failure | Reduced tear secretion and tear film stability |
| CIPA: congenital insensitivity to pain with anhidrosis | Dry eye, reduced corneal sensivity and ulceration, recurrent fever, anhidrosis, delayed healing |
| Complete androgen insensitivity syndrome | Sex hormone related dry eye may occur before sexual maturation |
| Craniofacial syndromes | Proptosis with exposure |
| Cystic fibrosis | May be vitamin A deficiency or a direct manifestation of cystic fibrosis |
| Diabetes | May relate to autonomic dysfunction |
| Down’s syndrome | Incomplete lid closure is common leading to dry eye |
| Ectodermal dysplasia | Anomalies include ectrodactyly, defects of hair, teeth and sweat glands, cleft lip and palate |
| Environmental factors | Heating, low humidity, air-conditioning, extensive computer use |
| Epidermolysis bullosa | Skin and mucous membrane disease with conjunctival scarring |
| Goldenhar’s syndrome | Dermolipomas, epibulbar dermoids |
| Graft-versus-host disease | Common after pediatric bone marrow transplantation |
| HIV | Lacrimal gland infiltration |
| Juvenile dermatomyositis | Dry eye reported in dermatomyositis and secondary Sjögren’s syndrome |
| Juvenile idiopathic arthritis | Reduced basal tear secretion |
| Juvenile localized scleroderma | Particularly form involving face (en coup de sabre) |
| KID syndrome: keratitis-ichthyosis-deafness | Hyperkeratotic skin lesions, sensorineural hearing loss and vascularizing keratitis |
| LOC: laryngo-onycho-cutaneous syndrome | Ocular granulation tissue with progressive scarring of conjunctiva and cornea |
| Medication | Antihistamines, antispasmodics, retinoids, topical drugs containing preservatives |
| Möbius’ syndrome | Reduced blinking with facial weakness |
| Multiple endocrine neoplasia type IIB | Marfanoid appearance, thick lips and eyelid neuromas, may have prominent corneal nerves |
| Neuroparalytic keratitis | Trigeminal nerve damage: acoustic neuroma, pontine tumors, Goldenhar syndrome, leprosy, after trauma |
| Pierre Robin sequence | May be associated with congenital alacrima |
| Post-orbital radiotherapy | Lacrimal gland damage |
| Riley-Day syndrome | Affects autonomic and sensory nervous system with dry, anesthetic eye |
| Sjögren’s syndrome | Lacrimal gland infiltration producing aqueous deficiency |
| Stevens-Johnson syndrome | Blisters or pseudomembrane in the early stages, later scarring and symblepharon |
| Trachoma | Common in developing world, mucopurulent conjunctivitis then cicatrization and corneal scarring |
| Turner’s syndrome | May relate to hormonal effect on meibomian gland function |
| Vitamin A deficiency | After bowel surgery, restricted diet, cystic fibrosis, dry conjunctiva, Bitot’s spots, keratomalacia, night blindness |
| Xeroderma pigmentosum | Dry pigmented skin, photophobia, reduced tearing, ocular surface squamous neoplasia |
History
The parents should be asked if the child produces reflex or emotional tears. It has been said that babies do not produce tears until 6 weeks of age, but tears are produced from the first day of life.3 Congenital alacrima is rare but the eye surface is often moist despite the absence of reflex or emotional tears.4 Ask if the child sleeps with their eyes open. This may occur in normal children, in those with craniofacial disorders, proptosis, and lid abnormalities, or after ptosis surgery.
