Cataracts

Congenital cataracts occur in two in 10,000 births (Figure 32-1). Of these, 20% to 25% of cases occur secondary to a congenital infection (rubella, cytomegalovirus, or toxoplasmosis) or as a component of a genetic or metabolic condition, such as Turner syndrome, Down syndrome, trisomy 13 and 18, galactosemia, and peroxisomal disorders. Children exposed to high-dose long-term corticosteroid therapy are also at risk, as are children with uveitis or who sustain ocular trauma.

Figure 32-1 Cataract.

Retinoblastoma

Retinoblastoma occurs in one in 15,000 live births, and 250 to 300 new cases are diagnosed annually in the United States. The hereditary form is caused by a mutation in the retinoblastoma gene, a tumor suppressor gene. A second mutation is then necessary for tumor growth. In the hereditary form, approximately 60% of cases are bilateral and are associated with other cancers, notably osteosarcoma. Retinoblastoma can present with leukocoria and strabismus, and in more advanced cases, proptosis, eye pain, or hyphema. The extent of the disease should be evaluated by computed tomography (CT) or magnetic resonance imaging and orbital ultrasonography. The average age at diagnosis is 1 year for bilateral cases and 2 years for unilateral cases. Urgent referral to an ophthalmologist for potentially vision-sparing and lifesaving treatment is imperative.

Strabismus

Misalignment of the eyes affects approximately 4% of children younger than 6 years of age (Figure 32-2). Heterophoria is the intermittent tendency for eyes to deviate, and heterotropia is a constant misalignment. The prefixes eso- (inward), exo- (outward), hyper- (upward), and hypo- (downward) indicate the direction of the misaligned eye. Other causes of eye deviations are cranial nerve palsies, intracranial or intraorbital mass, increased intracranial pressure (ICP), and myasthenia gravis.

Figure 32-2 Strabismus.

Heterophorias are usually not apparent; however, under certain conditions such as stress, fatigue, or illness, this latent deviation can be detected. If the deviation is large, patients may experience double vision (diplopia), headache, or eye strain. Heterotropias are present at all times.

Tropias can be tested using the corneal light reflex. The examiner shines a light onto both cornea and notes the placement of the light reflex. If strabismus is present, the reflected light is asymmetric on the cornea. To further test for strabismus, the examiner can perform a cover test. The child should look at an object in the distance. The examiner covers one eye and watches for movement in the uncovered eye. If movement occurs in the uncovered eye, then a misalignment exists in that eye. Phorias can be detected by covering the affected eye; when the eye is uncovered, the practitioner will note the eye moving back into alignment.

Early detection of strabismus is essential because amblyopia can develop if misalignment persists, resulting in permanent visual impairment. Strabismus that is constant or intermittent strabismus that does not correct by age 3 months should prompt ophthalmology referral to begin treatment. The unaffected eye is patched or blurred (with glasses or drops), thereby forcing the strabismic eye to provide a retinal image to the brain and stimulate the proper visual development. In some cases, surgery on the extraocular muscles is necessary to achieve proper alignment.

Amblyopia

Amblyopia is vision impairment caused by an interference with a clear retinal image in one or both eyes during the development of visual acuity in infancy and early childhood. Amblyopia occurs during the critical period of development before the cortex has become visually mature, mainly within the first decade of life. It can be caused by a deviated eye (strabismus), refractive error, or opacity within the visual axis. Treatment is specific to the cause such as strabismus correction, corrective glasses, or removal of the opacity.

Viral Conjunctivitis

Viral conjunctivitis presents with watery or mucopurulent discharge, eye irritation, and scleral injection (Figure 32-3). Both eyes are usually affected simultaneously or in sequence. More serious infection causes pseudomembranes (inflammatory debris and fibrin) or true conjunctival membranes. Punctate keratitis and subepithelial opacities may also occur, causing decreased vision, photosensitivity, or glare and haloes around bright lights.

Figure 32-3 Red eye.

Adenovirus is the most common pathogen causing viral conjunctivitis. Other symptoms include fever, pharyngitis, rhinitis, cough, and preauricular lymphadenopathy. This infection is highly contagious, and those who are infected should avoid sharing towels and touching their eyes and should wash their hands frequently. Treatment is symptomatic, including cool compresses or antiallergy drops for itchiness. Topical antibiotics should be used if large epithelial defects are present.

Other agents such as measles (rare because of widespread immunization), influenza, enterovirus, and herpes simplex virus (HSV) can cause conjunctivitis. Primary or recurrent HSV can also cause keratitis (corneal inflammation) with a dendrite pattern seen on fluorescein staining (see Figure 32-3). Treatment of ocular HSV includes topical antivirals (trifluridine, vidarabine, or iododeoxyuridine) and, depending on the extent of infection, oral or intravenous acyclovir. Consultation with an ophthalmologist is recommended.

Bacterial Conjunctivitis

Bacterial conjunctivitis presents with hyperemia, edema, mucopurulent discharge, and ocular pain. Common organisms involved are nontypeable Haemophilus influenzae (often associated with ipsilateral otitis media), staphylococcus and streptococci species, and Moraxella catarrhalis. Gram stain and culture can be performed to identify the organism. Treatment involves topical antibiotic drops or ointment such as polymyxin–trimethoprim, erythromycin, or bacitracin, and patients who wear contact lens should be treated for Pseudomonas aeruginosa with fluoroquinolone drops.

Practitioners should suspect Neisseria gonorrhoeae or Chlamydia trachomatis infection when symptoms of conjunctivitis are present in an infant in the first 2 weeks of life, termed ophthalmia neonatorum (see Chapter 105 for a detailed discussion).

Allergic Conjunctivitis

The hallmark of allergic conjunctivitis is itching along with clear tearing, injected conjunctivae, and conjunctival edema (chemosis) in both eyes. In more severe cases, cobblestoning of the tarsal conjunctivae is present (see Figure 32-3). Allergic conjunctivitis occurs as a seasonal disorder accompanied by allergic rhinitis or can occur perennially if associated with allergens such as cat dander, dust mites, mold spores, and other environmental allergens. Elimination of the offending agent and symptomatic treatment with cold compresses is recommended. Topical therapy with mast cell stabilizers or antihistamines can be used, as can oral antihistamines. More severe cases may require referral to an allergist, who may prescribe topical corticosteroids or immunotherapy.

Chemical Conjunctivitis

Various substances can cause a chemical conjunctivitis. Alkali exposure lingers in the conjunctival tissues and continues to cause damage for hours or days. Acids precipitate proteins and produce their effect immediately. In every patient with a chemical exposure, the eyes should be irrigated thoroughly with normal saline to remove the noxious substance. Irrigation should continue until a neutral pH (≈7.4) of the eye is achieved.

Uveitis

Uveitis is inflammation of the inner vascular coat of the eye (iris, ciliary body, and choroids). Posterior uveitis involves the choroid and presents with visual changes such as floaters and decreased vision. Anterior uveitis is synonymous with iritis and involves inflammation of the iris and ciliary body. Patients may have circumlimbal redness, eye pain, headache, photophobia, decreased vision, miosis, hypopyon (layering of white blood cells in the anterior chamber), and keratic precipitates. Uveitis can be seen in systemic immune-mediated diseases such as juvenile idiopathic arthritis, inflammatory bowel disease, and sarcoid and certain infections such as toxoplasmosis, tuberculosis, cytomegalovirus, and syphilis. Patients should be referred to an ophthalmologist for management.

Nasolacrimal Duct Obstruction

Nasolacrimal duct obstruction occurs in 2% to 4% of full-term babies and is even more common in premature babies. Congenital obstruction is usually caused by an imperforate membrane at the distal end of the nasolacrimal duct (Figure 32-4). Patients present with tears overflowing the eyelid (epiphora) and mucoid matter crusting the eyelashes.

Figure 32-4 Disorders of the adnexal structures.

If the patient has excessive tearing associated with photophobia, blepharospasm, or corneal and ocular enlargement, the physician should consider glaucoma as a cause of the symptoms and refer the patient to an ophthalmologist immediately. Glaucoma is a rare entity in infancy but can be vision threatening. Other causes of excessive tearing are corneal abrasion, intraocular inflammation, and foreign body.

Treatment of nasolacrimal duct obstruction involves applying digital pressure with downward strokes several times a day. Approximately 90% of cases resolve before 1 year of age. Patients can be referred to an ophthalmologist if symptoms do not resolve, in which case probing of the duct may be necessary. If mucopurulent discharge is expressible with palpation of the lacrimal sac and the sac is swollen, red, and tender, the patient may have an associated dacryocystitis, or inflammation of the lacrimal sac, which would require treatment with topical and often oral or intravenous antibiotics.

Hordeolum and Chalazion

A hordeolum is inflammation or impaction of a sebaceous gland of the eyelid. Whereas an internal hordeolum involves the meibomian glands and occurs on the conjunctival surface, infection of the glands of Zeiss or Moll causes an abscess on the eyelid margin known as an external hordeolum (stye). A chalazion is a chronic granulomatous inflammation of a meibomian gland, which is noted as a small, rubbery nodule located more centrally on the eyelid (see Figure 32-4).

Treatment of an external hordeolum involves warm compresses to encourage drainage and rarely incision and drainage. Topical antibiotics can be used for lesions that appear infected or are accompanied by discharge. Resolution may take several weeks. If infection spreads further, causing preseptal cellulitis, oral or intravenous antibiotics are required. A chalazion, if persistent, can be removed by an ophthalmologist by incision and curettage or steroid injection.

Preseptal (Periorbital) Cellulitis

Preseptal cellulitis is inflammation of the eyelids and periorbital tissues anterior to the orbital septum (see Figure 32-4), usually caused by contiguous infection of the periorbital soft tissues of the face. The most common pathogens are Staphylococcus aureus, group A β-hemolytic streptococcus, and Streptococcus pneumoniae. Patients present with erythema and swelling of the eyelids and conjunctival injection but will not have proptosis or limited ocular movements as in orbital cellulitis (see below).

Mild cases can be managed on an outpatient basis with broad spectrum antibiotics such as amoxicillin-clavulanate or clindamycin while more severe cases require intravenous antibiotics. If there is no clinical response within 24 hours or any suspicion for orbital cellulitis, the patient should have a CT scan of the sinuses and orbits and receive parenteral antibiotics.

Orbital Cellulitis

Orbital cellulitis presents with the previously described symptoms of periorbital cellulitis, but also with proptosis, limitation of eye movement, and sometimes decreased visual acuity, which distinguish it from a preseptal infection. Paranasal (ethmoid) sinusitis is the most common cause, but may also result from seeding from bacteremia as well as direct extension or venous spread of infection from contiguous sites such as the eyelids, conjunctiva, globe, lacrimal gland, and nasolacrimal sac. S. aureus, group A streptococcus, S. pneumoniae, Haemophilus influenzae, and anaerobes are commonly implicated as pathogens. Fungal infection should be considered in immunocompromised hosts. More serious cases may involve the optic nerve and cause loss of vision or extend intracranially and result in complications such as cavernous sinus thrombosis, meningitis, epidural and subdural empyema, or brain abscesses.

Treatment with broad-spectrum parenteral antibiotics such as high-dose ampicillin–sulbactam is indicated. Clindamycin or vancomycin may be used as well to ensure coverage of methicillin-resistant S. aureus (MRSA), and metronidazole can be added for additional anaerobe coverage. A CT of the sinuses and orbits with contrast should be performed to confirm infection within the orbit and to detect subperiosteal abscess or intracranial extension. Neurosurgical intervention may be necessary in cases with abscess or empyema formation.

Corneal Abrasion

Corneal abrasion presents with the sensation of a foreign body in the eye, pain, scleral injection, tearing, and photophobia. Pediatricians can detect corneal epithelial defects with fluorescein dye (see Figure 32-3). The eyelid should be everted to check for retained foreign body. Vision testing should also be performed, and if vision changes are present, the patient should be referred to an ophthalmologist. Topical antibiotic ointment is prescribed for infection prophylaxis and lubrication of the eye. Patients who wear contact lenses should be treated with topical fluoroquinolones for Pseudomonas spp. Without treatment, an infection could progress to a corneal ulcer. Oral analgesics can be used for pain. Topical anesthetics are no longer recommended because they slow epithelial healing and decrease the protective blinking reflex.

Hyphema

Hyphema is blood in the anterior chamber that is usually caused by blunt injury. Layering of blood cells can be seen by penlight or slit lamp (Figure 32-5). Increased ocular pressure may develop if the trabecular meshwork is affected either by the original injury or by red blood cells causing a blockage. Patients are at risk for rebleeding 3 to 5 days later. Patients with sickle cell disease or trait are at higher risk for complications and require more aggressive intervention.

Figure 32-5 Ocular trauma.

An ophthalmology evaluation is required urgently. Treatment involves wearing an eye shield and maintaining a 30-degree angle of the head to promote drainage and prevent secondary bleeding. The ophthalmologist may prescribe topical mydriatics, topical or oral corticosteroids, or aminocaproic acid to prevent rebleeding. Nonsteroidal antiinflammatory drugs and aspirin should be avoided.

Retinal Hemorrhage

Retinal hemorrhages can be caused by increased intrathoracic pressure or by acceleration–deceleration injuries. They are common in newborns delivered vaginally and resolve within 2 to 6 weeks without sequelae. They can also occur during trauma and are present in 85% of cases of shaken babies. The hemorrhages seen in non-accidental trauma have a distinctive pattern and are often bilateral, flame-shaped, and include preretinal structures and the macula (see Figure 32-5).

Subconjunctival Hemorrhage

Subconjunctival hemorrhage is the presence of blood between the conjunctiva and sclera (see Figure 32-5), is extremely common in newborns after vaginal birth, and resolves spontaneously within 2 weeks. In older children and adults, these hemorrhages are usually caused by increased intraocular pressure from coughing or sneezing or result from infection such as adenoviral conjunctivitis. Patients should be carefully examined to rule out a perforating injury.