CURRENT Diagnosis and Treatment Pediatrics, (Current Pediatric Diagnosis & Treatment) 22nd Edition

16. Eye

Rebecca Sands Braverman, MD

Normal vision is a sense that develops during infancy and childhood. Pediatric ophthalmology emphasizes early diagnosis and treatment of pediatric eye diseases in order to obtain the best possible visual outcome. Eye disease in children is not always limited to the ocular system and may be a sign of systemic disease.

COMMON NONSPECIFIC SIGNS & SYMPTOMS

Nonspecific signs and symptoms commonly occur as the chief complaint or as an element of the history of a child with eye disease. Five of these findings are described here, along with a sixth—leukocoria—which is less common, but often has serious implications. Do not hesitate to seek the help of a pediatric ophthalmologist when you believe the diagnosis and treatment of these signs and symptoms requires in-depth clinical experience.

RED EYE

Redness (injection) of the bulbar conjunctiva or deeper vessels is a common presenting complaint. It may be mild and localized or diffuse and bilateral. Causes include superficial or penetrating foreign bodies, trauma, infection, allergy, and conjunctivitis associated with systemic entities such as Stevens-Johnson syndrome, uveitis, or Kawasaki disease. Irritating noxious agents also cause injection. Subconjunctival hemorrhage may be traumatic, spontaneous, or may be associated with hematopoietic disease, vascular anomalies, or inflammatory processes. Uncommonly, an injected eye can be due to an intraocular or orbital tumor.

TEARING

Tearing in infants is usually due to nasolacrimal obstruction but may also be associated with congenital glaucoma, in which case photophobia and blepharospasm may also be present. Inflammation, allergic and viral diseases, or conjunctival and corneal irritation can also cause tearing.

DISCHARGE

Purulent discharge is usually associated with bacterial conjunctivitis. In infants and toddlers with nasolacrimal obstruction, a mucopurulent discharge may be present with low-grade, chronic dacryocystitis. Watery discharge occurs with viral infection, iritis, superficial foreign bodies, and nasolacrimal obstruction. Mucoid discharge may be a sign of allergic conjunctivitis or nasolacrimal obstruction. A mucoid discharge due to allergy typically contains eosinophils, whereas a purulent bacterial discharge contains polymorphonuclear leukocytes.

PAIN & FOREIGN BODY SENSATION

Pain in or around the eye may be due to foreign bodies, corneal abrasions, lacerations, acute infections of the globe or ocular adnexa, iritis, and angle-closure glaucoma. Large refractive errors or poor accommodative ability may manifest as headaches. Trichiasis (inturned lashes) and contact lens problems also cause ocular discomfort.

PHOTOPHOBIA

Acute aversion to light may occur with corneal abrasions, foreign bodies, and iritis. Squinting of one eye in bright light is a common sign of intermittent exotropia. Photophobia is present in infants with glaucoma, albinism, aniridia, and retinal dystrophies such as achromatopsia. Photophobia is common after ocular surgery and after dilation of the pupil with mydriatic and cycloplegic agents. Photophobia in individuals with no ocular pathology may be due to migraine headache, meningitis, and retrobulbar optic neuritis.

LEUKOCORIA

Although not a common sign or complaint, leukocoria (a white pupil) is associated with serious diseases and requires prompt ophthalmologic consultation. Causes of leukocoria include retinoblastoma, retinopathy of prematurity (ROP), pupillary membrane, cataract, vitreous opacities, retinal detachment, Toxocara infection, and retinal dysplasia (Figure 16–1).

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image Figure 16–1. Leukocoria of the left eye caused by retrolental membrane (persistent hyperplastic primary vitreous or persistent fetal vasculature).

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Significant refractive errors (myopia, hyperopia, astigmatism, or anisometropia) may cause decreased visual acuity, amblyopia, and strabismus.

image Symptoms and signs of uncorrected refractive error include blurred vision, squinting, headaches, fatigue with visual tasks, and failure of visual acuity screening.

REFRACTIVE ERRORS

image Pathogenesis

Refractive error refers to the optical state of the eye (Figure 16–2). The shape of the cornea and, to a lesser extent, the shape of the lens and length of the eye play a role in the refractive state of the eye. Children at particular risk for refractive errors requiring correction with glasses include those who were born prematurely; have Down syndrome; have parents with refractive errors; or have certain systemic conditions such as Stickler, Marfan, or Ehlers-Danlos syndrome.

Image

image Figure 16–2. Different refractive states of the eye. A: Emmetropia. Image plane from parallel rays of light falls on retina. B: Myopia. Image plane focuses anterior to retina. C: Hyperopia. Image plane focuses posterior to retina. D: Astigmatism, myopic type. Images in horizontal and vertical planes focus anterior to retina. E: Astigmatism, hyperopic type. Images in horizontal and vertical planes focus posterior to retina. F: Astigmatism, mixed type. Images in horizontal and vertical planes focus on either side of retina.

image Diagnosis

There are three common refractive errors: myopia, hyperopia, and astigmatism. Inequality of the refractive state between the two eyes (anisometropia) can cause amblyopia. The refractive state is determined by an eye care professional via a procedure called refraction. The determination of the refractive state in a preverbal child often proves challenging. If there is concern that there may be a significant problem with a preverbal child’s vision, they should be referred to an eye care professional specially trained for treating children.

image Treatment

Not all refractive errors require correction, but severe errors can cause amblyopia (reduced vision with or without an organic lesion). Refractive errors in children are most commonly treated with glasses. Less often, contact lenses are required, usually for very high or asymmetrical refractive errors, or for adolescents who do not want to wear glasses. Laser refractive surgery is not indicated for most children.

MYOPIA (NEARSIGHTEDNESS)

For the myopic or nearsighted individual, objects nearby are in focus; those at a distance are blurred. This is because the plane of focus is anterior to the retina. The onset is typically at about age 8 years and may progress throughout adolescence and young adulthood. A myopic person may squint to produce a pinhole effect, which improves distance vision. Divergent lenses provide clear distance vision. Many studies have attempted to slow or stop myopic progression. Atropine eye drops have shown some effect, but produce many side effects. A newer drug, pirenzepine, has shown promise in animal studies, but human studies have not shown a significant decrease in myopic progression.

HYPEROPIA (FARSIGHTEDNESS)

Saying that the hyperopic child is sighted for far (not near) vision is somewhat misleading, because the child can focus on near objects if the hyperopia is not excessive. Large amounts of uncorrected hyperopia can cause esotropia (inward deviation, or crossing, of the eyes) and amblyopia (see later sections Amblyopia and Strabismus). Most infants have a hyperopic refraction that begins to diminish during the toddler years and does not require correction.

ASTIGMATISM

When either the cornea or the crystalline lens is not perfectly spherical, an image will not be sharply focused in one plane. Schematically, there will be two planes of focus. Both of the planes can be either in front of or behind the retina, or one of the planes can be in front of the retina and the other behind it. This refractive state is described as astigmatism. Large amounts of astigmatism not corrected at an early age can cause decreased vision from amblyopia, but proper refractive correction can prevent this.

OPHTHALMIC EXAMINATION

A history suggesting poor vision or misalignment of the eyes, visual acuity that falls outside the expected level for a specific age, eyelid malposition, abnormal pupil reactivity or shape, and presence of an asymmetric/abnormal red reflex requires referral to an ophthalmologist.

The American Academy of Pediatrics (AAP), American Association for Pediatric Ophthalmology and Strabismus (AAPOS), and the American Academy of Ophthalmology (AAO) policy statements for red reflex testing can be accessed at http://aappolicy.aappublications.org/.

Prompt detection and treatment of ocular conditions can prevent a lifetime of visual disability. The ophthalmic examination should be a part of every well-child assessment.

It is crucial to check newborn infants for vision or life-threatening conditions that present with an abnormal red reflex that may be caused by cataracts or intraocular tumors. Eyelid ptosis (droopy eyelid) that obstructs the visual axis can cause permanent visual acuity loss from deprivation amblyopia and/or induced astigmatism, and requires urgent consultation with an ophthalmologist.

From birth to 3 years of age, the ophthalmic examination should include taking a history for ocular problems, vision assessment, inspection of the eyelids and eyes, pupil examination, ocular motility assessment, and red reflex check.

The ophthalmic examination of children older than 3 years should include taking a history for ocular problems, inspection of the eyelids and eyes, pupil examination, ocular motility assessment, and red reflex check and visual acuity testing with Allen, Lea, HOTV, Tumbling E, or Snellen symbols. Direct ophthalmoscopy should be attempted. Testing of binocular status can be accomplished by various tests, including the Random Dot E stereoacuity test.

HISTORY

Evaluation begins with the chief complaint and history of the present illness. Elements of the ocular history include onset of the complaint, its duration, whether it is monocular or binocular, treatment received thus far, and associated systemic symptoms. If an infectious disease is suspected, ask about possible contact with others having similar findings. The history should include prior ocular disease, perinatal and developmental history, history of allergy, and history of familial ocular disorders.

VISUAL ACUITY

Visual acuity testing is the most important test of visual function and should be part of every general physical examination (http://one.aao.org/Flash/VisionScreening/PediatricVisionScreening.html). Acuity should be tested in each eye individually, using an adhesive eye patch to prevent peeking. Glasses that were previously prescribed should be worn during vision screening. In older children who can cooperate, use of a pinhole will improve vision in children not wearing the appropriate spectacle prescription.

Vision Screening

Vision screening in the pediatric age group is a challenge, especially in younger and developmentally delayed children. Accuracy of the screening test being administered to a particular population and expense in terms of time, equipment, and personnel are some factors that must be considered in choosing a screening test. Vision screening should be done at well child visits. Further information on vision screening can be found at the AAP website (http://www.aap.org). Risk factors that should be screened for because they interfere with normal visual development and are amblyogenic include media opacities (such as cataracts), strabismus (misalignment of the visual axes of the eyes), and refractive errors that are different in the two eyes (anisometropia) or of large magnitude in both eyes.

In the sleeping newborn, the presence of a blepharospastic response to bright light is an adequate response. At age 6 weeks, eye-to-eye contact with slow, following movements is usually present. By age 3 months, the infant should demonstrate fixing and following ocular movements for objects at a distance of 2–3 ft. At age 6 months, interest in movement across the room is the norm. Vision can be recorded for the presence or absence of fixing and following behavior, and whether vision is steady (unsteady when nystagmus is present) and maintained.

In the verbal child, the use of familiar icons will allow for a quantitative test. Allen or Lea symbols with familiar pictures can be used to test children 2–3 years of age. When it is not possible to measure visual acuity or assess alignment in the preschool-aged child, random dot stereopsis testing (for depth perception) is effective in screening for manifest strabismus and amblyopia, but this test may miss some cases of anisometropic (unequal refractive error) amblyopia and small-angle strabismus, and is not designed to detect refractive errors.

Four-year-old children are often ready to play the tumbling E game (in which the child identifies the orientation of the letter E, which is turned in one of four directions) or the HOTV letters game (in which these four letters are shown individually at a distance and matched on a board that the child is holding). Literate children are tested with Snellen letters. Typical acuity levels in developmentally appropriate children are approximately 20/60 or better in children younger than 2–3 years, 20/40–20/30 in 3-year-old children, 20/30–20/25 in 4-year-old children, and 20/20 in literate children 5–6 years old. Referral criteria for children 3–5 years of age include visual acuity of less than 20/40 or 10/20 in either eye or a two-line difference between the eyes. Children 6 years or older should be referred if their visual acuity is less than 20/30 or 20/15 in either eye or a two-line difference is noted between the eyes.

The practitioner should be aware of two situations in which vision screening is complicated by nystagmus. Children who require a face turn or torticollis (in which the head is tilted to the right or left) to quiet the nystagmus will have poor visual acuity results when tested in the absence of the compensatory head posture. When latent nystagmus is present, acuity testing is particularly challenging (see later section Nystagmus). Nystagmus appears or worsens when an eye is occluded, degrading central vision. To minimize the nystagmus, the occluder should be held about 12 in in front of the eye not being tested. Testing both eyes simultaneously without occlusion often gives a better visual acuity measurement than when either eye is tested individually.

Traditional vision screening methods using eye charts in children aged 3–5 years require the child’s cooperation as well as proficiency in testing by the examiner. Additional screening modalities including photoscreening and autorefractors have been used by various volunteer programs in schools, day care facilities, and physician offices. Photoscreening does not screen directly for amblyopia but for amblyogenic factors, which include strabismus, media opacities, eyelid ptosis, and refractive errors. Autorefractors can determine if there is a significant refractive error present in either eye or if there is a significant difference between the two eyes. Some autorefractors can also detect strabismus and eyelid ptosis. If the screening results suggest an amblyogenic factor, children are referred to an eye care professional for a complete eye examination. Problems exist with sensitivity and specificity of the instruments and poor follow-up for referrals to eye care professionals. The cost-effectiveness of various vision screening modalities remains an area of continued research.

Chou R, Dana T, Bougatsos C: Screening for visual impairment in children ages 1-5 years: update for the USPSTF. Pediatrics 2011;127(2):e442–e479 [PMID: 21282269].

Davidson S, Quinn GE: The impact of pediatric vision disorders in adulthood. Pediatrics 2011;127(2):334–339 [PMID: 21199855].

Miller JM, Lessin HR: American Academy of Pediatrics Section on Ophthalmology; Committee on Practice and Ambulatory Medicine; American Academy of Ophthalmology; American Association for Pediatric Ophthalmology and Strabismus; American Association of Certified Orthoptists. Instrument-based pediatric vision screening policy statement. Pediatrics 2012;130(5):983–986 [PMID: 23109679].

EXTERNAL EXAMINATION

Inspection of the anterior segment of the globe and its adnexa requires adequate illumination and often magnification. A penlight provides good illumination and should be used in both straight-ahead and oblique illumination. A Wood lamp or a blue filter cap placed over a penlight is needed for evaluation after applying fluorescein. Immobilization of the child may be necessary. A drop of topical anesthetic may facilitate the examination.

In cases of suspected foreign body, pulling down on the lower lid provides excellent visualization of the inferior cul-de-sac (palpebral conjunctiva). Visualizing the upper cul-de-sac and superior bulbar conjunctiva is possible by having the patient look inferiorly while the upper lid is pulled away from the globe and the examiner peers into the upper recess. Illumination with a penlight is necessary. The upper lid should be everted to evaluate the superior tarsal conjunctiva (Figure 16–3).

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image Figure 16–3. Eversion of the upper lid. A: The patient looks downward. B: The fingers pull the lid down, and an index finger or cotton tip is placed on the upper tarsal border. C: The lid is pulled up over the finger. D: The lid is everted.

When indicated for further evaluation of the cornea, a small amount of fluorescein solution should be instilled into the lower cul-de-sac. Blue light will stain defects yellow-green. Disease-specific staining patterns may be observed. For example, herpes simplex lesions of the corneal epithelium produce a dendrite or branchlike pattern. A foreign body lodged beneath the upper lid shows one or more vertical lines of stain on the cornea due to the constant movement of the foreign body over the cornea. Contact lens overwear produces a central staining pattern. A fine, scattered punctate pattern may be a sign of viral keratitis or medication toxicity. Punctate erosions of the inferior third of the cornea can be seen with staphylococcal blepharitis or exposure keratitis secondary to incomplete lid closure.

PUPILS

The pupils should be evaluated for reaction to light, regularity of shape, and equality of size as well as for the presence of afferent pupillary defect. This defect, which occurs in optic nerve disease, is evaluated by the swinging flashlight test (see later section Diseases of the Optic Nerve). Irregular pupils are associated with iritis, trauma, pupillary membranes, and structural defects such as iris coloboma (see later section on Iris Coloboma).

Pupils vary in size due to lighting conditions and age. In general, infants have miotic (constricted) pupils. Children have larger pupils than either infants or adults, whereas the elderly have miotic pupils.

Anisocoria, a size difference between the two pupils, may be physiologic if the size difference is within 1 mm and is the same in light and dark. Anisocoria occurs with Horner syndrome, third nerve palsy, Adie tonic pupil, iritis, and trauma. Medication could also cause abnormal pupil size or reactivity. For example, contact with atropine-like substances (belladonna alkaloids) will cause pupillary dilation and limit pupillary reaction. Systemic antihistamines and scopolamine patches, among other medicines, can dilate the pupils and interfere with accommodation (focusing).

ALIGNMENT & MOTILITY EVALUATION

Alignment and motility should be tested because amblyopia is associated with strabismus. Besides alignment, ocular rotations should be evaluated in the six cardinal positions of gaze (Table 16–1Figure 16–4). A small toy is an interesting target for testing ocular rotations in infants; a penlight works well in older children.

Table 16–1. Function and innervation of each of the extraocular muscles.

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image Figure 16–4. Cardinal positions of gaze and muscles primarily tested in those fields of gaze. Arrow indicates position in which each muscle is tested.

Alignment can be assessed in several ways. In order of increasing accuracy, these methods are observation, the corneal light reflex test, and cover testing. Observation is an educated guess about whether the eyes are properly aligned. Corneal light reflex evaluation (Hirschberg test) is performed by shining a light beam at the patient’s eyes, observing the reflections off each cornea, and estimating whether these “reflexes” appear to be positioned properly. If the reflection of light is noted temporally on the cornea, esotropia is suspected (Figure 16–5). Nasal reflection of the light suggests exotropia (outward deviation). Accuracy of these tests increases with increasing angles of misalignment.

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image Figure 16–5. Temporal displacement of light reflection showing esotropia (inward deviation) of the right eye. Nasal displacement of the reflection would show exotropia (outward deviation) of the left eye.

Another way of evaluating alignment is with the cover test, in which the patient fixes on a target while one eye is covered. If an esotropia or an exotropia is present, the deviated eye will make a corrective movement to fixate on the target when the previously fixating eye is occluded. The other eye is tested similarly. When the occluder is removed from the eye just uncovered, a refixation movement of that eye indicates a phoria, or latent deviation, if alignment is reestablished (Figure 16–6). If the uncovered eye picks up fixation and strabismus is still present, that eye can be presumed to be dominant and the non-preferred eye possibly amblyopic. If the eye remains deviated after the occluder is removed, a tropia is noted to be present. A deviated eye that is blind or has very poor vision will not fixate on a target. Consequently, spurious results to cover testing may occur, which can happen with disinterest on the part of the patient, small-angle strabismus, and inexperience in administering cover tests.

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image Figure 16–6. Cover testing. The patient is instructed to look at a target at eye level 20 ft away. Note that in the presence of constant strabismus (ie, a tropia rather than a phoria), the deviation will remain when the cover is removed. (Reprinted, with permission, from Riordan-Eva P, Whitcher J: Vaughan & Asbury’s General Ophthalmology, 17th ed. McGraw-Hill; 2008.)

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image The handheld ophthalmoscope is the tool necessary to check the red reflex of the eye.

image Simultaneous examination of both pupils at the same time is called the Brückner test.

Red Reflex Test

image Clinical Findings

The examiner should see a round, red light in both eyes. The red reflex of each eye can be compared simultaneously when the observer is approximately 4 ft away from the patient. The largest diameter of light is shown through the ophthalmoscope, and no correction (zero setting) is dialed in the ophthalmoscope unless it is to compensate for the examiner’s uncorrected refractive error. A red reflex chart is available through the AAP policy statement on red reflex testing on pediatric patients at http://aappolicy.aappublications.org/.

image Differential Diagnosis

The red reflex test (Brückner test) is useful for identifying disorders such as media opacities (eg, cataracts), large refractive errors, tumors such as retinoblastoma, and strabismus.

image Treatment

A difference in quality of the red reflexes between the two eyes constitutes a positive Brückner test and requires referral to an ophthalmologist.

American Academy of Pediatrics; Section on Ophthalmology; American Association for Pediatric Ophthalmology and Strabismus; American Academy of Ophthalmology; American Association of Certified Orthoptists: Red reflex examination in neonates, infants, and children. Pediatrics 2008;122(6):1401–1404 [PMID: 19047263].

OPHTHALMOSCOPIC EXAMINATION

A handheld direct ophthalmoscope allows visualization of the ocular fundus. As the patient’s pupil becomes more constricted, viewing the fundus becomes more difficult. Although pupillary dilation can precipitate an attack of closed-angle glaucoma in the predisposed adult, children are very rarely predisposed to angle closure. Exceptions include those with a dislocated lens, past surgery, or an eye previously compromised by a retrolental membrane, such as in ROP. Therefore, if an adequate view of the fundus is precluded by a miotic pupil, use of a dilating agent (eg, one drop in each eye of 2.5% phenylephrine or 0.5% or 1% tropicamide) should provide adequate mydriasis (dilation). In infants, one drop of a combination of 1% phenylephrine with 0.2% cyclopentolate (Cyclomydril) is safer. Structures to be observed during ophthalmoscopy include the optic disc, blood vessels, the macular reflex, and retina, as well as the clarity of the vitreous media. By increasing the amount of plus lens dialed into the instrument, the point of focus moves anteriorly from the retina to the lens and finally to the cornea.

OCULAR TRAUMA

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image A careful history of the events that lead to the ocular injury is crucial in the diagnosis and treatment of ocular trauma.

image The examination of the traumatized eye may be difficult in the child due to poor cooperation and significant discomfort.

image If the extent of the eye injury is difficult to determine or if it is sight threatening, urgent referral to an ophthalmologist is necessary.

OCULAR FOREIGN BODIES

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image A foreign body of the eye/globe or adnexa may be difficult to visualize due to its small size or location.

image The clinician should always maintain a high index of suspicion for an occult or intraocular foreign body. If the history suggests this, an ophthalmologic referral should be considered.

image Prevention

Protective goggles or prescription glasses can help prevent ocular injuries and should be encouraged while participating in activities at risk for eye injuries, such as grinding metal, hammering, or sawing wood.

image Clinical Findings

Foreign bodies on the globe and palpebral conjunctiva usually cause discomfort and red eye. The history may suggest the origin of the foreign body, such as being around a metal grinder or being outside on a windy day when a sudden foreign body sensation was encountered associated with tearing, redness, and pain. Pain with blinking suggests that the foreign body may be trapped between the eyelid and the eye.

Magnification with a slit lamp may be needed for inspection. Foreign bodies that lodge on the upper palpebral conjunctiva are best viewed by everting the lid on itself and removing the foreign body with a cotton applicator. The conjunctival surface (palpebral conjunctiva) of the lower lid presents no problem with visualization. After simple removal of a foreign body that is thought not to be contaminated, no other treatment is needed if no corneal abrasion has occurred.

image Differential Diagnosis

Corneal abrasion, corneal ulcer, globe rupture/laceration.

image Complications

Pain, infection, and potential vision loss from scarring.

image Treatment

When foreign bodies are noted on the bulbar conjunctiva or cornea (Figure 16–7), removal is facilitated by using a topical anesthetic. If the foreign body is not too adherent, it can be dislodged with a stream of irrigating solution (Dacriose or saline) or with a cotton applicator after instillation of a topical anesthetic. Otherwise, a foreign body spud or needle is used to undermine the foreign body. This must be done with adequate magnification and illumination. An antibiotic ointment is then instilled. Ferrous corneal bodies often have an associated rust ring, which may be removed under slit-lamp visualization in cooperative children or under anesthesia if necessary.

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image Figure 16–7. A: Corneal foreign body at the nasal edge of the cornea. B: Subconjunctival foreign body of graphite.

image Prognosis

Usually excellent if treatment is obtained shortly after injury.

CORNEAL ABRASION

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image A corneal abrasion results in loss of the most superficial layer of corneal cells and causes severe ocular pain, tearing, and blepharospasm.

image An inciting event is usually identifiable as the cause of a corneal abrasion.

image Pathogenesis

Children often suffer corneal abrasions accidentally while playing with siblings or pets as well as participating in sports. Contact lens users frequently develop abrasions due to poorly fitting lenses, overnight wear, and use of torn or damaged lenses.

image Prevention

Proper contact lens care and parental supervision can prevent activities that can lead to a corneal abrasion.

image Clinical Findings

Symptom of a corneal abrasion is sudden and severe eye pain, usually after an inciting event such as an accidental finger poke to the eye. Decreased vision secondary to pain and tearing are common complaints. Eyelid edema, tearing, injection of the conjunctiva, and poor cooperation with the ocular examination due to pain are common signs of a corneal abrasion. Fluorescein dye is instilled into the eye and a cobalt blue or Wood lamp is used to illuminate the affected eye. The area with the abrasion will stain bright yellow.

image Differential Diagnosis

Ocular or adnexal foreign bodies, corneal ulcer, corneal laceration.

image Complications

Possible vision loss from corneal infection and scarring.

image Treatment

Ophthalmic ointment, such as erythromycin ointment, lubricates the surface of the cornea and also helps prevent infections. Patching the affected eye when a large abrasion is present may provide comfort, but it is not advised for corneal abrasions caused by contact lens wear or other potentially contaminated sources. Large corneal abrasions result in referred pain to the ipsilateral brow. If a brow ache is present, it may be treated by the use of a topical cycloplegic agent such as 1% cyclopentolate. Daily follow-up is required until healing is complete.

image Prognosis

Excellent if corneal infection and scarring do not occur.

INTRAOCULAR FOREIGN BODIES & PERFORATING OCULAR INJURIES

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Severe trauma may result in penetration of the eye by foreign bodies or retained foreign bodies, and is an ocular emergency.

image Pathogenesis

Intraocular foreign bodies and penetrating injuries are most often caused by being in close proximity to high-velocity projectiles such as windshield glass broken during a motor vehicle accident, metal ground without use of protective safety goggles, and improperly detonated fireworks.

image Prevention

Use protective eyewear when engaging in activities at risk for ocular injury.

image Clinical Findings

Sudden ocular pain occurs; vision loss, as well as multiple organ trauma, may be present.

Intraocular foreign bodies and corneal and scleral lacerations (ruptured globe) require emergency referral to an ophthalmologist. The diagnosis may be difficult if the obvious signs of corneal perforation (shallow anterior chamber with hyphema, traumatic cataract, and irregular pupil) are not present (Figure 16–8). Furthermore, nonradiopaque materials such as glass will not be seen on x-ray film.

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image Figure 16–8. Corneal laceration with irregular pupil and vitreous loss.

Computed tomographic (CT) scan may be useful in evaluating ocular trauma, including bony injury and intraocular foreign bodies. Magnetic resonance imaging (MRI) must be avoided if a magnetic foreign body is suspected.

image Differential Diagnosis

Corneal abrasion, superficial foreign body of the eye or eyelids.

image Complications

Vision loss, intraocular infection, loss of the eye.

image Treatment

In cases of suspected intraocular foreign body or perforation of the globe, it may be best to keep the child at rest, gently shield the eye with a metal shield or cut-down paper cup, and keep the extent of examination to a minimum to prevent expulsion of intraocular contents. In this setting, the child should be given nothing by mouth in case eye examination under anesthesia or surgical repair is required.

image Prognosis

Prognosis depends on the extent of the trauma.

BLUNT ORBITAL TRAUMA

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Blunt orbital and soft tissue trauma may produce “black eye,” which is ecchymosis (blue or purplish hemorrhagic areas) of the eyelids.

image Pathogenesis

Trauma to the orbit from a closed fist, collision with another player during team sports, and falls are common causes of blunt orbital injuries. Orbital compartment syndrome, which may result from severe orbital trauma, is caused by hemorrhaging within the orbit or severe orbital edema (or both). This is an emergency which may lead to permanent vision loss if not treated urgently.

image Prevention

Protective eyewear during athletic activities and adequate supervision of children at home and school.

image Clinical Findings

Blunt trauma to the orbit may result in orbit fractures. The orbital floor is a common location for a fracture (called a blowout fracture). A specific fracture that occurs mainly in children after blunt orbit trauma is called the white-eyed blowout fracture. This results from a greenstick fracture of the orbit with entrapment of extraocular muscles. It is called “white-eyed” because the external orbital soft tissue injury may appear to be minimal, but the patient will have severe pain with eye movement, as well as nausea, vomiting, and restriction of eye movements.

A blowout fracture must be suspected in a patient with symptoms of double vision, pain with eye movements, and restriction of extraocular muscle movements after blunt orbital trauma. CT images of an orbital floor fracture often reveal herniation of orbital fat or the inferior rectus muscle into the maxillary sinus. Assessment of ocular motility, globe integrity, and intraocular pressure will determine the extent of the blunt orbital injury. Consultation with an ophthalmologist is necessary to determine the full spectrum of the injuries.

Orbital compartment syndrome is an emergency requiring immediate treatment. Patients present with severe edema or ecchymosis of the eyelids (which makes it very difficult to open the eyelids), proptosis, and possibly an acute traumatic optic neuropathy, resulting in decreased vision or an afferent pupillary defect. Neuroimaging may reveal a retrobulbar hemorrhage and proptosis.

image Differential Diagnosis

Orbit fracture with or without muscle entrapment, globe rupture, orbital compartment syndrome, and traumatic or ischemic optic neuropathy.

image Treatment

Orbital compartment syndrome requires emergent lateral eyelid canthotomy and cantholysis to decompress the orbit. Treatment should not be delayed in order to image the orbits. Prompt treatment can prevent permanent vision loss.

Patients with clinical signs of muscle entrapment require urgent surgical repair to avoid permanent ischemic injury to the involved extraocular muscle. Large fractures may need repair to prevent enophthalmos, a sunken appearance to the orbit. This can usually be performed on a nonemergent basis.

Cold compresses or ice packs for brief periods (eg, 10 minutes at a time) are recommended in older children in the first 24 hours after injury to reduce hemorrhage and swelling.

image Prognosis

The prognosis depends on the severity of the blunt trauma, associated ocular injuries, and extent of the orbit fractures.

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Inspection of the eyelids reveals the extent and severity of the traumatic laceration.

image Lacerations of the nasal third of the eyelid and involving the eyelid margin are at risk for lacrimal system injury and subsequent chronic tearing.

LACERATIONS

image Pathogenesis

Lacerations of the eyelids and lacrimal system often result from dog bites, car accidents, falls, and fights.

image Prevention

Supervision of children at home and at school.

image Clinical Findings

Eyelid lacerations may be partial or full thickness in depth depending on the extent of the injury. Foreign bodies, such as glass or gravel, may be present depending on the mechanism of the injury.

image Differential Diagnosis

Lacerations involving the eyelid may or may not involve the nasolacrimal duct system or eyelid margin. Globe injury may be associated with eyelid lacerations as well.

image Complications

Poor surgical repair of lacerations of the eyelid margin can result in eyelid malposition, which causes chronic ocular surface irritation and possible corneal scarring.

image Treatment

Except for superficial lacerations away from the globe, repair in children is best performed in the operating room under general anesthesia. Special consideration must be given to lacerations involving the lid margin, significant tissue loss, full-thickness lacerations, lacerations that may involve the levator muscle in the upper lid, and to those that may involve the canaliculus (Figure 16–9). These injuries are best repaired by an ophthalmologist and may require intubation of the nasolacrimal system with silicone tubes.

Image

image Figure 16–9. Laceration involving right lower lid and canaliculus.

image Prognosis

Prognosis depends on severity of the injury, tissue loss, and adequacy of surgical repair.

BURNS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Severe thermal or chemical burns can result in permanent vision loss, ectropion or entropion of the lid, and scarring of the conjunctiva and cul-de-sac.

image Pathogenesis

Chemical burns with strong acidic and alkaline agents can be blinding and constitute a true ocular emergency. Examples are burns caused by exploding batteries, spilled drain cleaner, and bleach. Eyelid burns can occur in toddlers from contact with a lighted cigarette. The cornea is often involved as well. Curling irons can cause similar burns. Burns of the conjunctiva and cornea may be thermal, radiant, or chemical. Radiant energy causes ultraviolet keratitis. Typical examples are welder’s burn and burns associated with skiing without goggles in bright sunlight.

image Prevention

Protective eyewear when engaging in activities that pose a potential risk for exposure to hazardous chemicals, radiant energy, or when explosive conditions are possible.

image Clinical Findings

Superficial thermal burns cause pain, tearing, and injection. Corneal epithelial defects can be diagnosed using fluorescein dye, which will stain areas of the cornea bright yellow where the epithelium is absent. The fluorescein dye pattern will show a uniformly stippled appearance of the corneal epithelium in ultraviolet keratitis.

image Differential Diagnosis

Corneal abrasion, iritis.

image Complications

Significant corneal injury, especially if associated with an alkali burn, may lead to scarring and vision loss. Eyelid scarring can result in chronic exposure, dry eye, irritation, and entropion or ectropion.

image Treatment

Alkalis tend to penetrate deeper than acids into ocular tissue and often causes severe injury. Damage to the conjunctival vessels gives the eye a white or blanched appearance, resulting in ocular ischemia. Immediate treatment consists of copious irrigation and removal of precipitates as soon as possible after the injury. Initial stabilization of the injury is initiated by using topical antibiotics and patching the injured eye closed. A cycloplegic agent such as cyclopentolate 1% may be added if corneal involvement is present. This reduces the pain from ciliary spasm and iritis that may accompany the injury. The patient should be referred to an ophthalmologist after immediate first aid has been given.

image Prognosis

Prognosis depends on the severity of the injury.

HYPHEMA

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Slit-lamp examination or penlight examination may reveal a layer of blood within the anterior chamber. Other injuries to the globe and orbit are often present.

image A hyphema may be microscopic or may fill the entire anterior chamber (Figure 16–10).

Image

image Figure 16–10. Hyphema filling approximately 20% of the anterior chamber.

image Pathogenesis

Blunt trauma to the globe may cause a hyphema, or bleeding within the anterior chamber, from a ruptured vessel located near the root of the iris or in the anterior chamber angle (Figure 16–10).

image Prevention

Protective eyewear and appropriate supervision at home and at school.

image Clinical Findings

Blunt trauma severe enough to cause a hyphema may be associated with additional ocular injury, including iritis, lens subluxation, retinal edema or detachment, and glaucoma. In patients with sickle cell anemia or trait, even moderate elevations of intraocular pressure may quickly lead to optic atrophy and permanent vision loss. Therefore, all African Americans whose sickle cell status is unknown should be tested if hyphema is observed. These patients require extra vigilance in diagnosing and treating hyphema.

image Differential Diagnosis

Nontraumatic causes of hyphema include juvenile xanthogranuloma and blood dyscrasias. Rarely, hyphema is noted in the newborn after a stressful birth.

image Complications

Increased intraocular pressure, glaucoma, permanent corneal staining, and vision loss.

image Treatment

A shield should be placed over the eye, the head elevated, and arrangements made for ophthalmologic referral.

image Prognosis

The prognosis is worse if intraocular pressure is elevated, the patient has sickle cell disease, or if other associated ocular injuries are present.

ABUSIVE HEAD TRAUMA & NONACCIDENTAL TRAUMA

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Traumatic head trauma, commonly known as shaken baby syndrome, is a form of nonaccidental trauma characterized by a constellation of examination findings, including traumatic brain injury, retinal hemorrhages, and fractures of long bones or ribs.

image The history leading to the diagnosis of shaken baby syndrome is often vague and poorly correlated with the extent of injury.

image Pathogenesis

The mechanism of injury has been ascribed to a rapid back and forth shaking of young children that results in brain and ocular injuries. It is now believed that injuries may also be the result of blunt impact. Additional injury may result from spinal cord injuries and hypoxia.

image Clinical Findings

Victims often have multiple organ system involvement that includes, but is not limited to, traumatic brain injury, bone fractures, and retinal hemorrhages. The presentation can vary from irritability to emesis, change in mental status, or cardiopulmonary arrest.

Neuroimaging of the brain and a skeletal survey are used to diagnose shaken baby syndrome. Ophthalmic consultation and a dilated retinal examination are necessary to document retinal hemorrhages. Hemorrhages may be unilateral or bilateral and may be located in the posterior pole or periphery. Whereas retinal hemorrhages tend to resolve fairly quickly, those in the vitreous do not. If a blood clot lies over the macula, deprivation amblyopia may occur and may require intraocular surgery by a retinal specialist. Other ocular findings associated with nonaccidental trauma include lid ecchymosis, subconjunctival hemorrhage, hyphema, retinal folds, retinoschisis (traumatic separation of the retinal layers), and optic nerve edema. Acute-onset esotropia can also occur.

image Differential Diagnosis

The differential diagnosis of retinal hemorrhages includes those secondary to a fall, seizures, chest compressions during cardiopulmonary resuscitation, blood dyscrasias, and Terson syndrome, among others. A team effort between the primary treating physician, neurosurgery, orthopedics, ophthalmology, and social services is often needed to determine the true cause of a patient’s injuries.

image Complications

The severity of injuries dictates the long-term outcome. Severely diffuse retinal hemorrhages, associated traumatic optic neuropathy, and cortical injury adversely affect the potential for normal vision.

image Treatment

Management of any systemic injuries is required. Observation by an ophthalmologist for resolution of retinal hemorrhages is the usual management. Vitreous hemorrhages or large preretinal hemorrhages that do not resolve within several weeks may need surgical treatment by a retinal specialist.

image Prognosis

Prognosis depends on the severity of ocular and brain injuries.

Levin AV: Retinal hemorrhage in abusive head trauma. Pediatrics 2010;126(5):961–970 [PMID: 20921069].

PREVENTION OF OCULAR INJURIES

Air rifles, paintballs, and fireworks are responsible for many serious eye injuries in children. Golf injuries can be very severe. Bungee cords have been associated with multiple types of severe ocular trauma, including corneal abrasion, iris tears, hyphema, vitreous hemorrhage, retinal detachment, and blindness. Use of these items and associated activities should be avoided or very closely supervised. Safety goggles should be used in laboratories and industrial arts classes, and when operating snow blowers, power lawn mowers, and power tools, or when using hammers and nails.

Sports-related eye injuries can be prevented with protective eyewear. Sports goggles and visors of polycarbonate plastic will prevent injuries in games using fast projectiles such as tennis or racquet balls, or where opponents may swing elbows or poke at the eye.

The one-eyed individual should be specifically advised to always wear polycarbonate eyeglasses and goggles for all sports. High-risk activities such as boxing and the martial arts should be avoided by one-eyed children.

DISORDERS OF THE OCULAR STRUCTURES

DISEASES OF THE EYELIDS

The eyelids can be affected by various dermatologic and infectious conditions.

Blepharitis

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Blepharitis is inflammation of the lid margin characterized by crusty debris at the base of the lashes, erythema of eyelid margins, and ocular irritation.

image Pathogenesis

Blepharitis is caused by inflammation of the eyelid margin, meibomian gland obstruction, bacterial overgrowth, and tear film imbalance.

image Prevention

Eyelid hygiene is essential to prevent or control blepharitis. Eyelid scrubs help decrease the bacterial load on the eyelid margins and lashes. Warm compresses help loosen the secretions of the meibomian glands.

image Clinical Findings

Patients may present with dry eye symptoms, red and irritated eyelid margins, conjunctivitis, and decreased vision from corneal erosions or vascularization. When conjunctival injection accompanies blepharitis, the condition is known as blepharoconjunctivitisStaphylococcus is the most common bacterial cause.

image Differential Diagnosis

Chalazion, hordeolum, and rosacea blepharitis.

image Complications

Permanent corneal and eyelid margin scarring in severe cases.

image Treatment

Treatment includes lid scrubs with baby shampoo several times a week, warm compresses to the eyelids, and application of a topical antibiotic ointment such as erythromycin or bacitracin at bedtime.

image Prognosis

Generally good.

Chalazion

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image A chalazion is an inflammation of the meibomian glands, which may produce a tender nodule over the tarsus of the upper or lower lid.

image Chalazion tends to be recurrent if eyelid hygiene is poor.

image Pathogenesis

Obstruction of the eyelid margin meibomian glands with resultant inflammation, fibrosis, and granuloma formation.

image Prevention

See earlier section Blepharitis.

image Clinical Findings

Eyelid nodule of variable size and localized erythema of the corresponding palpebral conjunctiva that may be associated with a yellow lipogranuloma (Figure 16–11).

Image

image Figure 16–11. Chalazion. A: Right lower lid, external view. B: Right lower lid conjunctival surface.

image Differential Diagnosis

Hordeolum, blepharitis.

image Treatment

See earlier section Blepharitis. Oral flax seed oil may also decrease the risk of recurrent chalazion. If incision and curettage are needed because the lesion is slow to resolve, the child will require a general anesthetic. Topical azithromycin (ophthalmic solution 1%) may also help decrease recurrence of chalazion, but it is still under investigation.

image Prognosis

Generally good.

VIRAL EYELID DISEASE

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Viral infections of the eyelids can result in eyelid vesicles or papules.

image Pathogenesis

Herpes simplex virus (HSV) may involve the conjunctiva and lids at the time of primary herpes simplex infection resulting in blepharoconjunctivitis. Vesicular lesions with an erythematous base occur. Herpes zoster causes vesicular disease in association with a skin eruption in the dermatome of the ophthalmic branch of the trigeminal nerve. Molluscum contagiosum lesions are typically umbilicated papules. If near the lid margin, the lesions may cause conjunctivitis.

image Prevention

Avoid contact with individuals with active viral infections.

image Clinical Findings

A vesicular rash is the most common sign of herpes viral eyelid infection. Fluorescein dye should be administered topically to the effected eye followed by examination with a cobalt blue light to determine if corneal or conjunctival involvement is present. Herpes simplex or herpes zoster can be diagnosed by rapid viral culture (24–48 hours) or detection of antigen in skin lesions (3 hours).

image Differential Diagnosis

Impetigo.

image Complications

Conjunctivitis, keratitis (corneal infection).

image Treatment

Primary herpes simplex blepharoconjunctivitis should be treated with systemic acyclovir (a liquid formulation is available), valacyclovir, or famciclovir. When either the conjunctiva or the cornea is involved, treatment should include topical 1% trifluridine or 3% vidarabine.

Treatment of ophthalmic herpes zoster with nucleoside analogues within 5 days after onset may reduce the morbidity. When vesicles are present on the tip of the nose with herpes zoster (Hutchinson sign), ocular involvement, including iritis, is more likely.

Molluscum contagiosum lesions may be treated with cautery or excision.

image Prognosis

Generally good unless corneal involvement is present.

MISCELLANEOUS EYELID INFECTIONS

Pediculosis

Pediculosis of the lids (phthiriasis palpebrarum) is caused by Phthirus pubis. Nits and adult lice can be seen on the eyelashes when viewed with appropriate magnification. Mechanical removal and application to the lid margins of Phospholine iodide or 1% mercuric oxide ointment can be effective. Other bodily areas of involvement must also be treated if involved. Family members and contacts may also be infected (see Chapter 15).

Papillomavirus

Papillomavirus may infect the lid and conjunctiva. Warts may be recurrent, multiple, and difficult to treat. Treatment modalities include cryotherapy, cautery, carbon dioxide laser, and surgery.

Staphylococcal Infection

Localized staphylococcal infections of the glands of Zeis within the lid cause a sty (hordeolum) (Figure 16–12). When the infection coalesces and points internally or externally, it may discharge itself or require incision. The lesion is tender and red. Warm compresses help to shorten the acute process. Some practitioners prescribe a topical antibiotic ointment. Any coexisting blepharitis should be treated.

Image

image Figure 16–12. Hordeolum and blepharitis, left upper lid.

EYELID PTOSIS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Eyelid ptosis results in a droopy eyelid that may be unilateral or bilateral. If the pupil is obstructed, deprivation amblyopia may result.

image Pathogenesis

Ptosis—a droopy upper lid (Figure 16–13)—may be congenital or acquired but is usually congenital in children owing to a defective levator muscle. Other causes of ptosis are myasthenia gravis, lid injuries, third nerve palsy, and Horner syndrome (see next section). Ptosis may be associated with astigmatism and amblyopia.

Image

image Figure 16–13. Congenital ptosis of severe degree, left upper lid.

image Prevention

Injury prevention.

image Clinical Findings

An association sometimes seen with congenital ptosis is the Marcus Gunn jaw-winking phenomenon. Intermittent reduction of the ptosis occurs during mastication or sucking, due to a synkinesis or simultaneous firing of the external or internal pterygoid muscle (innervated by the trigeminal nerve) and the levator muscle (innervated by the oculomotor nerve).

image Differential Diagnosis

Congenital ptosis, traumatic ptosis, neurogenic ptosis (oculomotor nerve palsy), Horner syndrome.

image Complications

Deprivation amblyopia and induced astigmatism.

image Treatment

Surgical correction is indicated for moderate to severe ptosis. Mild cases less often require operative management. Cosmesis may be better if surgery is delayed until most of the facial growth has occurred, usually around age 5 years.

image Prognosis

The prognosis depends on the presence of amblyopia and whether it is adequately treated.

HORNER SYNDROME

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Horner syndrome, which may be congenital or acquired, presents with signs of unequal pupils (anisocoria), eyelid ptosis, iris heterochromia, and anhidrosis.

image Pathogenesis

The syndrome is caused by an abnormality or lesion to the sympathetic chain. The congenital variety is most commonly the result of birth trauma. Acquired cases may occur in children who have had cardiothoracic surgery, trauma, or brainstem vascular malformation. Most worrisome is a Horner syndrome caused by neuroblastoma of the sympathetic chain in the apical lung region.

image Prevention

Sympathetic chain injury avoidance during cardiothoracic surgery and delivery.

image Clinical Findings

Parents may notice unequal pupils or different colored eyes. Penlight examination of the eyes may reveal unequal pupils (anisocoria), iris heterochromia, and eyelid ptosis of the affected eye.

The ptosis is usually mild with a well-defined upper lid crease. This differentiates it from congenital ptosis, which typically has a poorly defined lid crease. Another key finding of congenital Horner syndrome is heterochromia of the two irides, with the lighter colored iris occurring on the same side as the lesion (Figure 16–14). Anhidrosis can occur in congenital and acquired cases. Of note, not all of the three signs must be present to make the diagnosis.

Image

image Figure 16–14. Congenital Horner syndrome. Ptosis, miosis, and heterochromia. Lighter colored iris is on the affected left side.

Pharmacologic assessment of the pupils with topical cocaine and hydroxyamphetamine or epinephrine will help determine whether the Horner syndrome is due to a preganglionic or postganglionic lesion of the sympathetic chain. Preliminary studies suggest that topical apraclonidine may be useful in the diagnosis of Horner syndrome. Physical examination, including palpation of the neck and abdomen for masses, and MRI of structures in the head, neck, chest, and abdomen should be considered. An excellent screening test for neuroblastoma is the spot urine vanillylmandelic acid/creatinine ratio.

image Differential Diagnosis

Congenital or neurogenic ptosis, physiologic anisocoria.

image Complications

Prognosis depends on the etiology. Ptosis associated with Horner syndrome is usually mild and rarely results in amblyopia.

image Treatment

Management of any underlying disease is required. The ptosis and vision should be monitored by an ophthalmologist.

image Prognosis

Prognosis depends on the etiology. The vision is usually normal.

EYELID TICS

Eyelid tics may occur as a transient phenomenon lasting several days to months. Although a tic may be an isolated finding in an otherwise healthy child, it may also occur in children with multiple tics, attention-deficit/hyperactivity disorder, or Tourette syndrome. Caffeine consumption may cause or exacerbate eyelid tics. If the disorder is a short-lived annoyance, no treatment is needed.

DISORDERS OF THE NASOLACRIMAL SYSTEM

NASOLACRIMAL DUCT OBSTRUCTION

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Nasolacrimal obstruction occurs in up to 6% of infants.

image Most cases clear spontaneously during the first year.

image Pathogenesis

Obstruction in any part of the drainage system may result from either incomplete canalization of the duct or membranous obstructions. Nasolacrimal obstruction may also occur in individuals with craniofacial abnormalities or Down syndrome.

image Prevention

Not applicable.

image Clinical Findings

Nasolacrimal duct obstruction presents with tearing and mucoid discharge from the affected eye. Signs and symptoms include tearing (epiphora), mucoid discharge especially in the morning, erythema of one or both lids, and conjunctivitis (Figure 16–15). Light sensitivity and blepharospasm suggest possible congenital glaucoma and warrant an urgent ophthalmic referral.

Image

image Figure 16–15. Nasolacrimal obstruction, right eye. Mattering on upper and lower lids.

image Differential Diagnosis

The differential diagnosis of tearing includes nasolacrimal duct obstruction, congenital glaucoma, foreign bodies, nasal disorders, and, in older children, allergies.

image Complications

Dacryocystitis, orbital cellulitis, sepsis, respiratory distress.

image Treatment

Massage over the nasolacrimal sac may empty debris from the nasolacrimal sac and clear the obstruction, although the efficacy of massage in clearing nasolacrimal obstruction is debated. Superinfection may occur, and treatment with topical antibiotics may help decrease the discharge.

The mainstay of surgical treatment is probing, which is successful 80% or more of the time, but the success rate may decrease after the infant reaches 1 year of age. Other surgical procedures, including infraction of the inferior nasal turbinate, balloon dilation, and silicone tube intubation, may be necessary if probing fails. Much less often, dacryocystorhinostomy is required.

image Prognosis

Generally good with surgical treatment.

CONGENITAL DACRYOCYSTOCELE

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Presence of a nodular enlargement which may be blue in color, occurring shortly after birth over the nasolacrimal sac/medial eyelids.

image Dacryocystitis often occurs due to bacterial superinfection.

image Pathogenesis

Congenital dacryocystocele is thought to result from obstructions proximal and distal to the nasolacrimal sac.

image Prevention

Early intervention with nasolacrimal duct probing may prevent dacryocystitis.

image Clinical Findings

At birth, the nasolacrimal sac is distended and has a bluish hue that often leads to an erroneous diagnosis of hemangioma. The tense and swollen sac displaces the medial canthus superiorly (Figure 16–16). Digital pressure over the nodule may result in reflux of tears, mucus, or purulent discharge from the inferior eyelid punctum. An intranasal duct cyst may be present beneath the inferior turbinate at the valve of Hasner. These cysts may be associated with respiratory distress.

Image

image Figure 16–16. Congenital dacryocystocele on the left side. Raised, bluish discolored mass of enlarged nasolacrimal sac. Note superiorly displaced medial canthus.

image Differential Diagnosis

Eyelid hemangioma, basal encephalocele.

image Complications

Dacryocystitis, orbital cellulitis, sepsis, respiratory distress.

image Treatment

Massage and warm compresses are rarely effective. Nasolacrimal duct probing and endoscopic marsupialization of the intranasal cyst under general anesthesia may be required. Hospital admission and systemic antibiotics are indicated if dacryocystitis is present. Consultation with an ear, nose, and throat specialist is recommended to aid in the diagnosis and treatment of an associated intranasal cyst.

image Prognosis

Generally good.

DACRYOCYSTITIS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Dacryocystitis is an infection of the nasolacrimal sac that causes erythema and edema over the nasolacrimal sac.

image Pathogenesis

Acute and chronic dacryocystitis are typically caused by bacteria that colonize the upper respiratory tract, such as Stap-hylococcus aureusStreptococcus pneumoniaeStreptococcus pyogenesStreptococcus viridansMoraxella catarrhalis, and Haemophilus species.

image Prevention

Treatment of nasolacrimal duct obstruction.

image Clinical Findings

Acute dacryocystitis presents with inflammation, swelling, tenderness, and pain over the lacrimal sac (located inferior to the medial canthal tendon). Fever may be present. The infection may point externally (Figure 16–17). A purulent discharge and tearing can be expected, because the cause of infection is almost always nasolacrimal obstruction.

Image

image Figure 16–17. Acute dacryocystitis in a 11-week-old infant.

Signs of chronic dacryocystitis are mucopurulent debris on the lids and lashes, tearing, injection of the palpebral conjunctiva, and reflux of pus at the puncta when pressure is applied over the sac. Chronic dacryocystitis and recurrent episodes of low-grade dacryocystitis are caused by nasolacrimal obstruction.

image Differential Diagnosis

Dacryocystocele, preseptal cellulitis.

image Complications

Preseptal cellulitis, orbital cellulitis, sepsis.

image Treatment

Severe acute dacryocystitis is treated with intravenous antibiotics after attempts at identifying the offending organism by culture and staining. Oral antibiotics can be tried in milder cases. Topical antibiotic administration is adjunctive and is also used with recurrent chronic infections. Warm compresses are beneficial. After the acute episode subsides—and in chronic cases—the nasolacrimal obstruction must be relieved surgically. If it cannot be drained via the intranasal portion of the nasolacrimal duct, external drainage may be necessary. This should be done as a last resort since a fistula may develop.

image Prognosis

Generally good.

DISEASES OF THE CONJUNCTIVA

Conjunctivitis may be infectious, allergic, or associated with systemic disease. Trauma, irritation of the conjunctiva, and intraocular inflammation can cause injection of conjunctival vessels that can be confused with conjunctivitis.

OPHTHALMIA NEONATORUM

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Ophthalmia neonatorum (conjunctivitis in the newborn) occurs during the first month of life.

image Pathogenesis

Pathogenesis may be due to inflammation resulting from silver nitrate prophylaxis given at birth, bacterial infection (gonococcal, staphylococcal, pneumococcal, or chlamydial), or viral infection. In developed countries, Chlamydiais the most common cause. Neonatal conjunctivitis may threaten vision if caused by Neisseria gonorrhoeae. Herpes simplex is a rare but serious cause of neonatal conjunctivitis, since it may indicate systemic herpes simplex infection.

image Prevention

Treatment of maternal infections prior to delivery can prevent ophthalmia neonatorum. Although no single prophylactic medication can eliminate all cases of neonatal conjunctivitis, povidone-iodine may provide broader coverage against the organisms causing this disease than silver nitrate or erythromycin ointment. Silver nitrate is not effective against Chlamydia. The choice of prophylactic agent is often dictated by local epidemiology and cost considerations, but erythromycin ophthalmic ointment is most often routinely administered immediately after birth to help prevent ophthalmia neonatorum.

image Clinical Findings

Ophthalmia neonatorum is characterized by redness and swelling of the lids and conjunctiva and by discharge (Figure 16–18). Gram staining, polymerase chain reaction amplification for Chlamydia and HSV, and bacterial and viral cultures aid in making an etiologic diagnosis.

Image

image Figure 16–18. Ophthalmia neonatorum due to Chlamydia trachomatis infection in a 2-week-old infant. Note marked lid and conjunctival inflammation.

image Differential Diagnosis

Chemical/toxic conjunctivitis, viral conjunctivitis, bacterial conjunctivitis, chlamydial conjunctivitis.

image Complications

Chlamydia can cause a delayed-onset pneumonitis. Gonococcal infections can cause blindness through endophthalmitis as well as sepsis.

image Treatment

Treatment of these infections requires specific systemic antibiotics because they can cause serious infections in other organs. Parents should be examined and receive treatment when a sexually associated pathogen is present.

image Prognosis

Prognosis depends on the infectious agent as well as the rapidity of treatment.

BACTERIAL CONJUNCTIVITIS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image In general, bacterial conjunctivitis is accompanied by a purulent discharge.

image Pathogenesis

Common bacterial causes of conjunctivitis in older children include Haemophilus species, S pneumoniaeM catarrhalis, and S aureus.

image Prevention

Hand-washing and contact precautions.

image Clinical findings

Purulent discharge and conjunctival injection of one or both eyes. These symptoms may be associated with an upper respiratory infection. Regional lymphadenopathy is not a common finding in bacterial conjunctivitis except in cases of oculoglandular syndrome due to S aureus, group A β-hemolytic streptococci, Mycobacterium tuberculosis or atypical mycobacteria, Francisella tularensis (the agent of tularemia), and Bartonella henselae (the agent of cat-scratch disease).

image Differential Diagnosis

Viral, allergic, traumatic, or chemical/toxic conjunctivitis.

image Complications

Bacterial conjunctivitis is usually self-limited unless caused by Chlamydia trachomatisN gonorrhoeae, and Neisseria meningitidis which may have systemic manifestations.

image Treatment

If conjunctivitis is not associated with systemic illness, topical antibiotics such as erythromycin, polymyxin-bacitracin, sulfacetamide, tobramycin, and fluoroquinolones are adequate. Systemic therapy is recommended for conjunctivitis associated with C trachomatisN gonorrhoeae, and N meningitidis.

image Prognosis

Generally good.

Gold RS: Treatment of bacterial conjunctivitis in children. Pediatr Ann 2011;40(2):95–105 [PMID: 21323206].

VIRAL CONJUNCTIVITIS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Children with viral conjunctivitis usually present with injection of the conjunctiva of one or both eyes and watery ocular discharge.

image Pathogenesis

Adenovirus infection is often associated with pharyngitis, a follicular reaction and injection of the palpebral conjunctiva, and preauricular adenopathy (pharyngoconjunctival fever). Epidemics of adenoviral keratoconjunctivitis occur. Conjunctivitis may also be due to enterovirus and can occur as part of an acute measles illness. HSV may cause conjunctivitis or blepharoconjunctivitis.

image Prevention

Hand-washing and contact precautions.

image Clinical Findings

Watery discharge associated with conjunctival injection of one or both eyes. A vesicular rash involving the eyelids or face suggests HSV.

image Differential Diagnosis

Bacterial, allergic, traumatic, or chemical/toxic conjunctivitis.

image Complications

Generally, viral conjunctivitis is self-limited. Herpes conjunctivitis may result in keratitis, which can affect visual acuity. This should be treated with antiviral therapy and the patient should be referred to an ophthalmologist.

image Treatment

Treatment of adenovirus conjunctivitis is supportive. Children with presumed adenoviral keratoconjunctivitis are considered contagious 10–14 days from the day of onset. They should stay out of school and group activities as long as their eyes are red and tearing. Strict hand-washing precautions are recommended.

Herpes conjunctivitis can be treated with topical trifluridine 1% drops or 3% vidarabine ointment. Oral acyclovir may be used for treatment of the primary infection to decrease the duration and severity of the infection and as prophylaxis to reduce recurrence of herpes simplex ocular disease.

image Prognosis

Generally good.

ALLERGIC CONJUNCTIVITIS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image A history of itchy, watery, red eyes, often associated with other allergy symptoms such as sneezing and rhinitis.

image Prevention

Decrease exposure to allergens. This may be done by hand washing after handling dogs/cats, washing clothing, and bathing after being outdoors when pollen counts are elevated.

image Clinical Findings

The history of itchy, watery, and red eyes is essential in making the diagnosis of allergic conjunctivitis. Redness of the conjunctiva, tearing, and discharge may be part of the history but need not be present on examination to make the diagnosis. Vernal conjunctivitis is a more severe form of allergic conjunctivitis occurring mostly in the spring and summer that is associated with intense tearing, itching, and a stringy discharge. Vernal allergic conjunctivitis is more common in males. Vernal conjunctivitis may present with giant cobblestone papillae (Figure 16–19) on the eyelid conjunctiva, nodules around the corneal limbus, and even sterile corneal ulcers. Contact lens wear may induce a conjunctivitis that appears similar to the palpebral form of vernal conjunctivitis.

Image

image Figure 16–19. Vernal conjunctivitis. Cobblestone papillae in superior tarsal conjunctiva.

image Treatment

Topical ophthalmic solutions that combine both an antihistamine and mast cell stabilizers, including olopatadine 0.1%, epinastine HCl 0.05%, and ketotifen fumarate 0.025%, are very effective at treating allergic conjunctivitis. Other agents available include a combination topical vasoconstrictor plus an antihistamine (naphazoline antazoline), a nonsteroidal anti-inflammatory drug (NSAID) such as ketorolac tromethamine 0.5%, a mast cell stabilizer such as lodoxamide tromethamine 0.1%, or a corticosteroid such as prednisolone 0.125% (Table 16–2). Corticosteroids should be used with caution because their extended use causes glaucoma or cataracts in some patients. Systemic antihistamines and limitation of exposure to allergens may help reduce symptoms as well.

Table 16–2. Common ocular allergy medications.

Image

image Prognosis

Generally good.

MUCOCUTANEOUS DISEASES

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis are systemic conditions that often affect the eyes, as well as the skin, and oral and genitourinary mucosa.

image Ocular involvement may result in permanent conjunctival scarring, eyelid malposition, severe dry eye syndrome, and permanent vision loss.

image Clinical Findings

Erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis may be caused by medications including: phenytoin, sulfonamides, NSAIDs, and barbiturates or infections such as HSV or Mycoplasma pneumoniae. With Stevens-Johnson syndrome, conjunctival changes include erythema and vesicular lesions that frequently rupture. Staining of the conjunctiva and/or cornea with fluorescein suggests severe ocular involvement and high risk for permanent ocular sequela, including symblepharon (adhesions) between the raw edges of the bulbar (eye) and palpebral (lid) conjunctivae.

image Differential Diagnosis

Viral or bacterial conjunctivitis if the patient presents prior to cutaneous or mucosal eruptions.

image Complications

Severe ocular involvement can result in permanent scarring of the conjunctiva leading to eyelid malposition, trichiasis (eyelashes touching the surface of the eye), and vision loss from chronic ocular irritation and extreme tear film deficiency.

image Treatment

Treatment of the underlying disease, including discontinuation of offending medications and use of appropriate antimicrobials is necessary. Management of conjunctivitis associated with mucocutaneous disease depends on its severity. Artificial tears and ointment provide comfort and a topical corticosteroid may help prevent adhesions and dry eye in mild to moderate cases. Lysis of adhesions or use of a scleral ring by an ophthalmologist may be required. Surgical treatment of severe cases with amniotic membrane grafts may prevent visual disability by decreasing the risk of dry eye from tear-producing glands/goblet cell destruction, symblepharon, and trichiasis. Topical cyclosporine may help decrease the inflammatory reaction that leads to the destruction of tear-producing glands/goblet cells and subsequent dry eye syndrome.

image Prognosis

Prognosis depends on the severity of the underlying condition. Guarded visual prognosis is made in severe cases.

Gregory DG: Treatment of acute Stevens-Johnson syndrome and toxic epidermal necrolysis using amniotic membrane: a review of 10 consecutive cases. Ophthalmology 2011;118(5):908–914 [PMID: 21440941].

DISORDERS OF THE IRIS

IRIS COLOBOMA

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Iris coloboma is a developmental defect due to incomplete closure of the anterior embryonal fissure.

image Iris coloboma may occur as an isolated defect or in association with various chromosomal abnormalities and syndromes.

image Clinical Findings

Penlight examination of the pupils reveals a keyhole shape to the pupil rather than the normal round configuration (Figure 16–20). A dilated examination by an ophthalmologist is necessary to determine if the coloboma involves additional structures of the eye including the retina. If the retina is involved, vision may be poor. A genetic evaluation is usually recommended due to the high rate of associated genetic syndromes.

Image

image Figure 16–20. Iris coloboma located inferiorly.

image Differential Diagnosis

Microphthalmia, aniridia, previous iris trauma.

image Complications

Low vision and rarely a secondary retinal detachment may need surgical intervention.

image Treatment

Patients with coloboma should be monitored by an ophthalmologist for signs of amblyopia, significant refractive errors, and strabismus.

image Prognosis

The prognosis depends on whether there are other ocular structures involved. Visual acuity is guarded if a large retinal coloboma is present.

ANIRIDIA

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Aniridia is a bilateral disorder that results in the absence of the majority of the iris (Figure 16–21).

Image

image Figure 16–21. Bilateral aniridia. Iris remnants present temporally in each eye.

image Pathogenesis

Aniridia may occur as an autosomal dominant disease or in a sporadic form associated with Wilms tumor. The aniridia gene is located within the 11p13 chromosome region. Aniridia, genitourinary abnormalities, and developmental delay have been linked to an 11p deletion.

image Clinical Findings

Slit-lamp or penlight examinations reveal little to no visible iris (see Figure 16–21). Photophobia, nystagmus, and poor vision are present in aniridia. Abdominal ultrasonography is indicated in the sporadic form of aniridia to diagnose Wilms tumor. Genetic evaluation is indicated as well. Cataract, corneal changes, macular hypoplasia, and glaucoma are often seen.

image Differential Diagnosis

Microphthalmia, iris coloboma, previous iris trauma.

image Complications

Low vision, cataracts, and glaucoma.

image Treatment

An ophthalmologist should determine if cataracts or glaucoma are present in patients with aniridia. Surgical treatment of cataracts and glaucoma is often indicated.

image Prognosis

Patients tend to have low vision.

ALBINISM

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Albinism is caused by defective melanogenesis, usually as an autosomal recessive disease, but an X-linked form does occur.

image Pathogenesis

Tyrosinase is an essential enzyme in the production of melanin. Many cases of complete albinism are caused by mutations of the tyrosinase gene. Albinism with some pigment production, especially in people of African descent, is more commonly caused by mutations of the p gene.

image Clinical Findings

Affected individuals are usually legally blind and have nystagmus (see later section Nystagmus). Iris, skin, and hair color vary with the type and severity of albinism as well as with race. Iris transillumination is abnormal transmission of light through an iris with decreased pigment which can aid in the diagnosis of albinism. This may be obvious or may require slit-lamp examination with retroillumination to detect focal areas of transillumination. Other ocular abnormalities include foveal hypoplasia, abnormal optic pathway projections, strabismus, and poor stereoacuity.

image Differential Diagnosis

Albinism may be associated with other systemic manifestations. Bleeding problems occur in individuals with Hermansky-Pudlak syndrome (chromosome 10q23 or 5q13), in which oculocutaneous albinism is associated with a platelet abnormality. Chédiak-Higashi syndrome (chromosome 1q42–44) is characterized by neutrophil defects, recurrent infections, and oculocutaneous albinism. Other conditions associated with albinism are Waardenburg, Prader-Willi, and Angelman syndromes.

image Complications

Low vision, strabismus, high refractive errors, and visual field abnormalities.

image Treatment

Children with albinism should be evaluated by a pediatric ophthalmologist in order to optimize their visual function. Low-vision aids such as telescopes, stand magnifiers, and large-print books are often required. Vision teachers in schools and ophthalmic specialists trained in treating low-vision patients can improve the patient’s ability to perform activities of daily living and function within society. Affected individuals should use sunscreen and protective clothing to prevent skin cancer.

image Prognosis

Vision is subnormal in most individuals.

MISCELLANEOUS IRIS CONDITIONS

Heterochromia, or a difference in iris color, can occur in congenital Horner syndrome, after iritis, or with tumors and nevi of the iris and use of topical prostaglandins. Malignant melanoma of the iris may also cause iris heterochromia. Acquired iris nodules (Lisch nodules), which occur in type 1 neurofibromatosis, usually become apparent by age 8 years. When seen on slit-lamp examination, Lisch nodules are 1–2 mm in diameter. They are often beige in color, although their appearance can vary. Iris xanthogranuloma occurring with juvenile xanthogranuloma can cause hyphema and glaucoma. Patients with juvenile xanthogranuloma should be evaluated by an ophthalmologist for ocular involvement.

GLAUCOMA

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Glaucoma is caused by increased intraocular pressure and results in vision loss due to optic nerve injury, corneal scarring, and amblyopia.

image Pediatric glaucoma can be congenital or acquired, pupillary block or angle closure, and unilateral or bilateral.

image Glaucoma can be classified on an anatomic basis into two types: open-angle and closed-angle.

image Clinical Findings

Signs of glaucoma presenting within the first year of life include enlargement of the globe due to low scleral rigidity in the infant eye (buphthalmos), as well as tearing, photophobia, blepharospasm, corneal clouding due to edema, and optic nerve cupping. After age 3 years, usually only optic nerve changes occur. Findings may be unilateral or bilateral. In general, a red, inflamed eye is not typical of congenital or infantile glaucoma.

Sudden eye pain, redness, corneal clouding, and vision loss suggests possible pupillary block or angle-closure glaucoma. Urgent referral to an ophthalmologist is indicated. Genetic evaluation should be completed if other systemic abnormalities are noted.

Glaucoma also occurs with ocular and systemic syndromes such as aniridia, anterior segment dysgenesis, Sturge-Weber syndrome, the oculocerebrorenal syndrome of Lowe, and the Pierre Robin syndrome. Glaucoma can also occur with a traumatic hyphema, iritis, lens dislocation, intraocular tumor, and ROP.

image Differential Diagnosis

Buphthalmos is glaucoma until proven otherwise. The signs and symptoms of glaucoma are quite variable and should be urgently evaluated by an ophthalmologist.

image Treatment

Treatment depends on the cause, but surgery is often indicated. Topical medications, which are available to decrease the intraocular pressure, have limited success in pediatric glaucoma.

image Prognosis

In general, the prognosis is guarded but is often poor for glaucoma associated with congenital buphthalmos.

Aponte EP, Diehl N, Mohney BG: Medical and surgical outcomes in childhood glaucoma: a population-based study. J AAPOS 2011;15(3):263–267 [PMID: 21652244].

Mandal AK, Chakrabarti D: Update on congenital glaucoma. Indian J Ophthalmol 2011;59 [PMID: 21150027].

UVEITIS

Inflammation of the uveal tract can be subdivided according to the uveal tissue primarily involved (iris, choroid, or retina) or by location (anterior, intermediate, or posterior uveitis). Perhaps the most commonly diagnosed form of uveitis in childhood is traumatic iridocyclitis or iritis.

ANTERIOR UVEITIS/IRIDOCYCLITIS/IRITIS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Iridocyclitis associated with juvenile idiopathic arthritis may be asymptomatic despite severe ocular inflammation.

image Pathogenesis

Iridocyclitis associated with juvenile idiopathic arthritis occurs most often in girls with oligoarticular arthritis and a positive antinuclear antibody test. Inflammatory bowel disease is also associated with iritis—perhaps more commonly with Crohn disease than with ulcerative colitis. Other causes of anterior uveitis in children include syphilis, tuberculosis, sarcoidosis, relapsing fever (borreliosis), and Lyme disease, all but the last also causing posterior uveitis. Juvenile spondyloarthropathies, including ankylosing spondylitis, Reiter syndrome, and psoriatic arthritis, are associated with anterior uveitis. A substantial percentage of cases are of unknown origin.

image Clinical Findings

Injection, photophobia, pain, and blurred vision usually accompany iritis (anterior uveitis or iridocyclitis). An exception to this is iritis associated with juvenile idiopathic arthritis (see Chapter 29). The eye in such cases is quiet and asymptomatic, but slit-lamp examination reveals anterior chamber inflammation with inflammatory cells and protein flare. Children with juvenile idiopathic arthritis should be screened according to a schedule recommended by the AAP (http://www.aap.org). Children with Crohn disease or ulcerative colitis should have routine periodic ophthalmologic examinations to detect ocular inflammation, which may be asymptomatic, and to detect cataracts if they have been receiving long-term systemic corticosteroids.

Other ocular findings of the anterior segment include conjunctivitis, episcleritis, and sterile corneal infiltrates. Posterior segment findings may include central serous retinochoroidopathy, panuveitis (inflammation of all uveal tissue), choroiditis, ischemic optic neuropathy, retinal vasculitis, neuroretinitis, and intermediate uveitis (see later section Intermediate Uveitis).

Posterior subcapsular cataracts can develop in patients with or without ocular inflammation. Most, if not all, of these patients have been taking corticosteroids as part of the long-term treatment of their autoimmune disease.

image Differential Diagnosis

Iridocyclitis due to autoimmune disorder, trauma, infection, malignancy, or idiopathic etiology.

image Complications

Permanent decreased vision due to cataracts, secondary glaucoma, and band keratopathy.

image Treatment

Treatment with a topical corticosteroid and a cycloplegic agent is aimed at quieting the inflammation and preventing or delaying the onset of cataract and glaucoma. Methotrexate and other systemic immunosuppressive agents can be used in refractory cases. Systemic antitumor necrosis factor agents such as etanercept, infliximab, and adalimumab show promise in treating refractory cases of uveitis.

image Prognosis

Prognosis depends on the severity of ocular inflammation, development of cataracts, and secondary glaucoma.

Qian Y, Acharya NR: Juvenile idiopathic arthritis-associated uveitis. Curr Opin Ophthalmol 2010;21(6):468–472 [PMID: 20729734].

Tugal-Tutkun I: Pediatric uveitis. J Ophthalmic Vis Res 2011; 6(4):259–269 [PMID: 22454749].

POSTERIOR UVEITIS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image The terms choroiditisretinitis, and retinochoroiditis denote the tissue layers primarily involved in posterior uveitis. Infectious agents are the most common cause of posterior uveitis in the pediatric population.

image Clinical Findings

Children with posterior uveitis often present with systemic manifestations of a congenital infection. Examples include deafness, developmental delay, cataracts, “salt and pepper” retinopathy, and hearing and cardiac disorders seen in congenital rubella.

Serologic analysis and retinal examination by an ophthalmologist are used to identify the cause of posterior uveitis. Active toxoplasmosis (see Chapter 43) produces a white lesion appearing as a “headlight in the fog” owing to the overlying vitreitis. Inactive lesions have a hyperpigmented border. Contiguous white satellite lesions suggest reactivation of disease.

A granular “salt and pepper” retinopathy is characteristic of congenital rubella. In infants, the TORCH complex (toxoplasmosis, other infections, rubella, cytomegalovirus [CMV], and HSV) and syphilis are congenital infections that cause chorioretinitis.

Congenital chorioretinitis caused by lymphocytic choriomeningitis is diagnosed by immunofluorescent antibody or enzyme-linked immunosorbent assay (ELISA) serologic testing. The virus is transmitted to humans by consumption of food contaminated with rodent urine or feces. It most closely resembles congenital toxoplasmosis in presentation. Ocular candidiasis occurs typically in an immune compromised host or a premature infant in the intensive care nursery receiving hyperalimentation. Candidal chorioretinitis, which is evidence of candidemia, appears as multifocal, whitish yellow, fluffy retinal lesions that may spread into the vitreous and produce a so-called cotton or fungus ball vitritis.

Acute retinal necrosis syndrome is caused most often by varicella-zoster virus and occasionally by HSV. Patients may present with vision loss and a red and painful eye. Ophthalmoscopy may show unilateral or bilateral patchy white areas of retina, arterial sheathing, vitreous haze, atrophic retinal scars, retinal detachment, and optic nerve involvement.

CMV infection is the most common cause of retinitis in immune compromised children, especially those with human immunodeficiency virus (HIV) infection. CMV retinitis appears as a white retinal lesion, typically but not always associated with hemorrhage, or as a granular, indolent-appearing lesion with hemorrhage and a white periphery. Cotton-wool spots (nerve fiber layer infarcts) also commonly occur in HIV-positive patients.

In toddlers and young children, Toxocara canis or Toxocara cati infections (ocular larva migrans; see Chapter 43) occur from ingesting soil contaminated with parasite eggs. The disease is usually unilateral. Common signs and symptoms include a red injected eye, leukocoria, and decreased vision. Funduscopic examination may show endophthalmitis (vitreous abscess) or localized granuloma. Diagnosis is based on the appearance of the lesion and serologic testing using ELISA for T canis and T cati.

image Differential Diagnosis

Posterior uveitis due to autoimmune disorder, trauma, infection, malignancy, or idiopathic etiology.

image Complications

Permanent vision loss due to retinal scarring and detachment.

image Treatment

Congenital toxoplasmosis infections must be treated with systemic antimicrobials (see Chapter 43). Studies have shown improved ophthalmic and neurologic outcomes with prolonged treatment. Other infectious agents such as Candida, varicella, and CMV require systemic and/or intraocular injections of antimicrobial agents, and may require retinal surgery. Treatment of toxocariasis includes periocular corticosteroid injections and vitrectomy.

image Prognosis

The prognosis for vision depends on the severity of retinal and systemic involvement.

INTERMEDIATE UVEITIS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Intermediate uveitis or pars planitis is described as inflammation located in the far anterior periphery of the retina and vitreous base.

image Clinical Findings

Patients with pars planitis, inflammation of the anterior edge of the retina, often complain of decreased vision and floaters. They may also have a history of a red eye and ocular discomfort. Patients with intermediate uveitis often have decreased vision. A prolonged duration of the disease can lead to deprivation amblyopia and strabismus.

A dilated examination is required for observation of inflammation of the pars plana and vitritis. Slit-lamp and dilated funduscopic examinations by an ophthalmologist often reveal chronic signs of inflammation associated with intermediate uveitis, including macular edema, cataracts, increased intraocular pressure, irregular pupil, iris adhesion to the lens, and band keratopathy.

image Differential Diagnosis

Intermediate uveitis is often idiopathic although there are several known etiologies. Toxocara infections with peripheral granuloma can be associated with intermediate uveitis, as can inflammatory bowel disease, multiple sclerosis, and sarcoidosis. Retinoblastoma and other neoplasms can imitate uveitis.

image Complications

Decreased vision due to macular edema, vitreous floaters, cataracts, and glaucoma.

image Treatment

The most common treatment regimen for intermediate uveitis includes subtenon steroid injections, vitrectomy by a retinal surgeon, and systemic immunosuppression. Secondary glaucoma often requires tube shunt surgery.

image Prognosis

The prognosis depends on the severity of the disease and associated secondary complications such as glaucoma and cataracts.

OCULAR MANIFESTATIONS OF AIDS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image The advent of highly active antiretroviral therapy (HAART) has significantly reduced the incidence of CMV retinitis.

image Ocular infections are important manifestations of acquired immunodeficiency syndrome (AIDS) (see Chapter 41).

image Opportunistic ocular infections increase in HIV-infected children as CD4 T-lymphocyte counts fall below 200/μL.

image Pathogenesis

Pathogens commonly causing eye infection include CMV and varicella-zoster virus. Acute retinal necrosis syndrome (see earlier section Posterior Uveitis) is a severe necrotizing retinitis that often results in blindness in patients with AIDS. Most cases are thought to be caused by varicella-zoster virus. Other implicated agents are herpes simplex types 1 and 2.

Patients with CD4 counts below 50/μL are at high risk for CMV retinitis and should have a complete ocular evaluation by an ophthalmologist. Various retinal abnormalities may be present which include cotton-wool spots, retinal hemorrhages, microaneurysms, perivasculitis, and decreased visual acuity from ischemic maculopathy.

Immune recovery uveitis associated with antiretroviral therapy may result in decreased vision and require treatment.

image Differential Diagnosis

Decreased vision due to immune recovery uveitis, CMV retinitis, or acute retinal necrosis syndrome.

image Complications

Retinal scarring, retinal detachment, and blindness.

image Treatment

If immune recovery is sufficient with antiretroviral therapy for an extended period, the patient may be able to discontinue anti-CMV therapy. However, active viral retinitis must be treated with antiviral agents. CMV retinitis is treated with intravenous ganciclovir but foscarnet may be required if resistance develops. Intravitreal ganciclovir or ganciclovir implants in conjunction with oral valganciclovir may be required in severe cases or in individuals intolerant to intravenous therapy. Acute retinal necrosis due to HSV or VZV must be treated with intravenous acyclovir, systemic steroids, and intraocular surgery.

image Prognosis

Viral retinitis generally has a poor prognosis.

Jabs DA: Cytomegalovirus retinitis and the acquired immunodeficiency syndrome—bench to bedside: LXVII Edward Jackson Memorial Lecture. Am J Ophthalmol 2011;151(2):198–216 [PMID: 21168815].

Jabs DA et al: Studies of the Ocular Complications of AIDS Research Group. Course of cytomegalovirus retinitis in the era of highly active antiretroviral therapy: five-year outcomes. Ophthalmology 2010;117(11):2152–2161 [PMID: 20673591].

DISORDERS OF THE CORNEA

CLOUDY CORNEA

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Corneal clouding can be caused by developmental abnormalities, metabolic disorders, trauma, and infection.

image Clinical Findings

The cornea may have a white, hazy appearance on penlight examination. The red reflex may be decreased or absent.

image Differential Diagnosis

Corneal clouding, tearing, blepharospasm, and photophobia in a newborn are signs of congenital glaucoma until proven otherwise. Peter anomaly and sclerocornea are congenital malformations of the anterior segment of the eye that are the most common causes of a cloudy cornea at birth. Direct trauma to the cornea during a forceps delivery can result in corneal haze and significant amblyopia. Systemic abnormalities such as developmental delay and liver or kidney failure suggest metabolic disorders such as mucopolysaccharidoses, Wilson disease, and cystinosis. Corneal infiltrates occur with viral infections, staphylococcal lid disease, corneal dystrophies, and interstitial keratitis due to congenital syphilis.

A complete ocular evaluation by an ophthalmologist is required and should be completed urgently when congenital glaucoma is suspected.

image Complications

Vision loss is likely due to deprivation amblyopia.

image Treatment

Treatment depends on the underlying condition. Surgical treatment of glaucoma and possible corneal transplantation or keratoprosthesis may be required.

image Prognosis

Prognosis depends on the amount of corneal involvement and response to surgical treatment. Corneal transplants have a very high frequency of rejection and subsequently a poor prognosis in children.

Lowe MT, Keane MC, Coster DJ, Williams KA: The outcome of corneal transplantation in infants, children, and adolescents. Ophthalmology 2011;118(3):492–497 [PMID: 20932584].

Nallasamy S, Colby K: Keratoprosthesis: procedure of choice for corneal opacities in children? Semin Ophthalmol 2010;25 (5-6):244–248 [PMID: 21091007].

VIRAL KERATITIS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Herpes simplex, herpes zoster, and adenovirus can infect the cornea.

image When the corneal epithelium is involved, a dendritic or amoeboid pattern can be seen with fluorescein staining.

image Clinical Findings

Patients commonly present with a painful, red eye. Photophobia and decreased vision are also common complaints. Fever, malaise, and symptoms of upper respiratory tract infection may be present.

Fluorescein administration to the involved cornea will reveal areas of staining when viewed with a blue light if the corneal epithelium is involved. The pattern of epithelium staining may be dendritic (branch-like) or irregular and round if a geographic ulcer is present. Slit-lamp examination may reveal white infiltrates beneath the corneal epithelium as a result of corneal stromal scarring or edema from viral infection of the corneal stromal tissue. Decreased visual acuity, photophobia, and conjunctivitis may also be noted.

image Differential Diagnosis

Corneal abrasion, bacterial corneal ulcer, iritis.

image Treatment

Topical antivirals such as trifluridine and vidarabine are indicated when herpes simplex infection is limited to the corneal epithelium, although additional systemic therapy is required in newborns. Topical corticosteroids may be a useful addition to antiviral therapy when stromal disease is present. The use of corticosteroids in the presence of herpetic disease should be undertaken only by an ophthalmologist because of the danger of worsening the disease. Oral acyclovir started in the early phase (first 5 days) may be helpful in treating herpes zoster eye disease. Acyclovir prophylaxis is helpful in preventing recurrent herpetic epithelial keratitis (see earlier section Viral Conjunctivitis) and stromal keratitis caused by herpes simplex.

Adenovirus conjunctivitis may progress to keratitis 1–2 weeks after onset. Vision may be decreased. In most cases, no treatment is necessary because adenovirus keratitis is most often self-limiting. However, adenovirus is highly contagious and easily spread (see section Viral Conjunctivitis).

image Prognosis

Corneal involvement with herpes simplex can be recurrent and lead to blindness.

CORNEAL ULCERS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Decreased vision, pain, injection, a white corneal infiltrate or ulcer (Figure 16–22), and hypopyon (pus in the anterior chamber) may all be present.

image Bacterial corneal ulcers in healthy children who are not contact lens wearers are usually secondary to corneal trauma from corneal abrasion or a penetrating foreign body.

Image

image Figure 16–22. Corneal ulcer. Note white infiltrate located on inferior cornea.

image Clinical findings

A corneal ulcer appears as a white spot on the surface of the cornea that stains with fluorescein (see Figure 16–22). Associated symptoms include pain and decreased vision. Signs often include conjunctival injection, photophobia, tearing, and purulent discharge.

image Differential Diagnosis

Viral keratitis, corneal abrasion, penetrating foreign body.

image Complications

Permanent vision loss may result from corneal scarring. Corneal transplantation may be required.

image Treatment

Prompt referral to an ophthalmologist is necessary for evaluation and management.

image Prognosis

Prognosis depends on how large the ulcer is and whether the central cornea is involved.

DISORDERS OF THE LENS

Lens disorders involve abnormality of clarity or position. Lens opacification (Figure 16–23) can affect vision depending on its density, size, and position. Visual potential is also influenced by age at onset and the success of amblyopia treatment.

Image

image Figure 16–23. Cataract causing leukocoria.

CATARACTS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Cataracts in children may be unilateral or bilateral, may exist as isolated defects, or may be accompanied by other ocular disorders or systemic disease (see Figure 16–23).

image Clinical Findings

Leukocoria, poor fixation, and strabismus or nystagmus (or both) may be the presenting complaints. Absence of a red reflex in the newborn may be due to a cataract which requires an urgent referral to an ophthalmologist.

Laboratory investigation for infectious and metabolic causes of congenital cataracts is often indicated. Such investigation would include cultures or serologic tests for toxoplasmosis, rubella, CMV, HSV, and syphilis, as well as evaluation for inborn metabolic errors, such as galactosemia or Lowe syndrome.

image Differential Diagnosis

Cloudy cornea, intraocular tumor, retinal detachment.

image Complications

Pediatric cataracts are frequently associated with severe deprivation amblyopia.

image Treatment

Early diagnosis and treatment are necessary to prevent deprivation amblyopia in children younger than 9 years, because they are visually immature. Cataracts that are visually significant require removal. Visually significant cataracts in infants are usually removed prior to 6 weeks of age to prevent deprivation amblyopia. Rehabilitation of the vision will require the correction of refractive errors and amblyopia treatment. Contact lenses, glasses, and artificial intraocular lenses are used to correct refractive errors after cataract extraction. Treatment of underlying concomitant congenital infections or systemic diseases must be instituted as appropriate.

image Prognosis

The ultimate visual acuity depends on when the cataract was diagnosed and treated as well as compliance with amblyopia treatment. Glaucoma is often associated with pediatric cataracts and may result in a poor prognosis if not controlled.

DISLOCATED LENSES/ECTOPIA LENTIS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Nontraumatic lens dislocation is usually bilateral.

image Subluxation causes refractive errors of large magnitude that are difficult to correct.

image Pathogenesis

Systemic diseases, including Marfan syndrome, homocystinuria, Weill-Marchesani syndrome, sulfite oxidase deficiency, hyperlysinemia, syphilis, and Ehlers-Danlos syndrome, are often associated with dislocated lenses.

image Clinical Findings

Slit-lamp examination reveals malposition of the intraocular lens. Refraction often reveals significant astigmatism. A complete ophthalmic evaluation, as well as genetic and metabolic evaluation, may be warranted.

image Differential Diagnosis

Ectopia lentis due to systemic disease versus trauma.

image Complications

Ectopia lentis can cause decreased vision and amblyopia due to induced refractive errors. Another ophthalmologic concern is pupillary block glaucoma, in which a malpositioned unstable lens interferes with the normal flow of aqueous humor from the ciliary body (posterior to the pupil), where it is produced, into the trabecular meshwork (anterior to the pupillary plane).

image Treatment

Surgical lensectomy may be required if the visual acuity is not improved significantly with glasses or contact lenses. Underlying metabolic and/or genetic disorders require a multidisciplinary approach.

image Prognosis

Prognosis depends on the severity of the lens dislocation and need for lensectomy.

DISORDERS OF THE RETINA

RETINAL HEMORRHAGES IN THE NEWBORN

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Retinal hemorrhages are commonly seen in the otherwise healthy newborn.

image Retinal hemorrhages occur most often after vaginal delivery but can also be present after suction delivery or cesarean section.

image Clinical Findings

A dilated retinal examination reveals unilateral or bilateral hemorrhages that can be located anywhere in the retina. They may appear as dot, blot, subretinal, or preretinal hemorrhages. They may also break into the vitreous. Examination of the retina of an otherwise healthy newborn infant is not indicated.

image Differential Diagnosis

See earlier section Abusive Head Trauma & Nonaccidental Trauma.

image Treatment

Observation is indicated since retinal hemorrhages of the newborn usually disappear within the first month of life.

image Prognosis

Excellent.

RETINOPATHY OF PREMATURITY

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Screening examinations are recommended for infants with a birth weight of 1500 g or less or gestational age of 30 weeks or less and selected infants with a birth weight between 1500 and 2000 g or gestational age of greater than 30 weeks with an unstable clinical course.

image Cost analysis studies have determined that screening and laser photoablation of retinopathy of prematurity (ROP) are cost-effective medical interventions.

image The joint policy statement from the AAP, AAO, and AAPOS on screening examinations of premature infants for ROP is available at http://www.pediatrics.org.

image Pathogenesis

Premature infants with incomplete retinal vascularization are at risk for developing abnormal peripheral retinal vascularization, which may lead to retinal detachment and blindness. The cause of this disorder—including the role of supplemental oxygen in the neonatal period—is still not fully understood. Recent studies suggest that vascular endothelial growth factor (VEGF) may play a key role in ROP development. Other risk factors for severe ROP are bronchopulmonary dysplasia, intraventricular hemorrhage, sepsis, apnea and bradycardia, and mutations of the Norrie disease gene. White males, infants with zone 1 disease, and infants with very low birth weight and gestational age have a higher risk of developing severe ROP that requires treatment.

image Prevention

The risk of vision loss from ROP can be reduced by timely screening of premature infants by an ophthalmologist.

image Clinical Findings

The Cryotherapy for Retinopathy of Prematurity (CRYOROP) study outlined a standard nomenclature to describe the progression and severity of ROP (Table 16–3). Since retinal blood vessels emanate from the optic nerve and do not fully cover the developing retina until term, the optic nerve is used as the central landmark. The most immature zone of the retina, zone 1, is the most posterior concentric imaginary circle around the optic nerve. The next peripheral area is zone 2, and peripheral to that is zone 3. Zone 1 disease by definition is more high-risk than disease in more anterior/peripheral zones. Similarly, the stages of the abnormal vessels are numbered from zero (simply incomplete vascularization) through stages I–V.

Table 16–3. Stages of retinopathy of prematurity.

Image

Recommendations for initiating eye examination are outlined in the joint policy statement issued by the AAP, AAO, and AAPOS and are based on the gestational age and birth weight. Screening examinations are recommended for infants with a birth weight of 1500 g or less or gestational age of 30 weeks or less and selected infants with a birth weight between 1500 and 2000 g or gestational age of greater than 30 weeks with an unstable clinical course. The frequency of follow-up examinations depends on the findings and the risk factors for developing the disease. Most infants are evaluated every 1–2 weeks. ROP often resolves when the infant reaches 40 weeks estimated gestational age. Examinations can be discontinued when the retinas are fully vascularized, or when the infant is 45 weeks’ gestational age and has never had prethreshold disease or worse, or is vascularized out to zone 3 and never had zone 1 or 2 disease.

image Complications

Low vision, retinal detachment.

image Treatment

Surgical treatment of ROP is indicated when there is zone 1 ROP with any stage and plus disease, zone 1 ROP stage III without plus disease, zone 2 ROP with stage II or III and plus disease. The treatment of ROP within 72 hours can reduce the occurrence of bad visual outcomes by 50%. Some patients still progress to a retinal detachment, which can have a very poor prognosis for vision. Surgical treatment for a retinal detachment involves scleral buckling or a lens-sparing vitrectomy by a vitreoretinal specialist. Intraocular injections of anti-VEGF agents such as bevacizumab and ranibizumab have been used in certain severe cases of ROP but long-term effects are still under investigation.

image Prognosis

Most cases of ROP do not progress to retinal detachment and require no treatment. However, ROP remains a leading cause of blindness in children. Those with a history of ROP are at a much higher risk of developing strabismus, amblyopia, myopia, and glaucoma than the average child.

American Academy of Pediatrics Section on Ophthalmology; American Academy of Ophthalmology; American Association for Pediatric Ophthalmology and Strabismus; American Association of Certified Orthoptists: Screening examination of premature infants for retinopathy of prematurity. Pediatrics 2013; 131(1):189–195 [PMID: 23277315].

Faia LJ, Trese MT: Retinopathy of prematurity care: screening to vitrectomy. Int Ophthalmol Clin 2011;51(1):1–16 [PMID: 21139474].

Mintz-Hittner HA, Kennedy KA, Chuang AZ: BEAT-ROP Cooperative Group. Efficacy of intravitreal bevacizumab for stage 3+ retinopathy of prematurity. N Engl J Med 2011;364(7): 603–615 [PMID: 21323540].

Moshfeghi DM, Berrocal AM: Retinopathy of prematurity in the time of bevacizumab: incorporating the BEAT-ROP results into clinical practice. Ophthalmology 2011;118(7):1227–1228 [PMID: 21724044].

RETINOBLASTOMA

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Retinoblastoma is the most common primary intraocular malignancy of childhood, with an incidence estimated between 1:17,000 and 1:34,000 live births (see Chapter 31).

image Most patients present before age 3 years.

image Pathogenesis

Inherited forms of retinoblastoma are autosomal dominant with high penetrance. The disease may consist of a solitary mass or multiple tumors in one or both eyes. All bilateral cases and some unilateral cases are caused by germinal mutations; however, most unilateral cases are caused by a somatic retinal mutation. In both situations, the mutation occurs in the retinoblastoma gene (Rb) at chromosome 13q14. This is a tumor suppressor gene. One mutated copy may be inherited in an autosomal dominant fashion (germline mutation). If a second mutation spontaneously occurs in any cell, tumorigenesis is likely. Individuals with a germinal mutation are at risk for the development of tumors other than retinoblastoma (pineal tumors, osteosarcoma, and other soft tissue sarcomas). All children with unilateral or bilateral retinoblastoma must be presumed to have the germline form, and followed expectantly for other tumors in the remaining eye and at extraocular sites. Approximately 15% of patients with unilateral disease have germline mutations.

image Clinical Findings

The most common presenting sign in a child with previously undiagnosed retinoblastoma is leukocoria (see Figure 16–1). Evaluation of the pupillary red reflex is important, although a normal red reflex does not rule out retinoblastoma. Examination requires indirect ophthalmoscopy with scleral depression and pupillary dilation, performed by an ophthalmologist. Other presentations include strabismus, red eye, glaucoma, or pseudo-hypopyon (appearance of pus-like material in the anterior chamber).

Genetic testing is available for patients with retinoblastoma. Once the causative mutation is found in an affected individual, unaffected members of the family should be tested to determine their personal and reproductive risk.

image Differential Diagnosis

Retinal vasculature abnormalities seen in diseases such as Coats disease, uveitis, and endophthalmitis.

image Complications

Death if not adequately treated.

image Treatment

The goal of treatment is to preserve the eye and as much useful vision as possible. Chemoreduction is used to reduce initial tumor volume. Local treatment with laser photocoagulation, cryotherapy, plaque radiotherapy, or thermotherapy can often preserve vision and spare the patient enucleation and radiation. Ophthalmic artery chemotherapy can be successful in certain cases.

image Prognosis

Generally good except in developing countries where children often succumb to their disease due to lack of treatment. Patients with germline mutations need lifelong monitoring for secondary neoplasms such as sarcomas.

Palioura S, Gobin YP, Brodie SE, Marr BP, Dunkel IJ, Abramson DH: Ophthalmic artery chemosurgery for the management of retinoblastoma in eyes with extensive (>50%) retinal detachment. Pediatr Blood Cancer 2012;59(5):859–864 [PMID: 22492689].

Shields CL, Shields JA: Retinoblastoma management: advances in enucleation, intravenous chemoreduction, and intra-arterial chemotherapy. Curr Opin Ophthalmol 2010;21(3):203–212 [PMID: 20224400].

RETINAL DETACHMENT

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image A retinal detachment may present as an abnormal or absent red reflex.

image Older children may complain of decreased vision, flashes, floaters, or visual field defects.

image Pathogenesis

Common causes are trauma and high myopia. Other causes are ROP, Marfan syndrome, and Stickler syndrome.

image Clinical Findings

Symptoms of detachment are floaters, flashing lights, and loss of visual field; however, children often cannot appreciate or verbalize their symptoms. A detachment may not be discovered until the child is referred after failing a vision screening examination, strabismus supervenes, or leukocoria is noted.

image Differential Diagnosis

Intraocular tumor.

image Complications

Vision loss, glaucoma, strabismus.

image Treatment

Treatment of retinal detachment is surgical. For children with conditions predisposing to retinal detachment, or a strong family history, examinations under anesthesia by an ophthalmologist, with prophylactic laser treatment, may be recommended.

image Prognosis

Prognosis depends on the location and duration of the detachment.

DIABETIC RETINOPATHY

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Diabetic retinopathy is a specific vascular complication of diabetes mellitus. Patients with type 1, or insulin-dependent, diabetes are at higher risk of developing severe proliferative retinopathy leading to visual loss than are those with type 2, or non–insulin-dependent, diabetes.

image The cost-effectiveness of screening examinations for diabetic retinopathy in children requires further evaluation.

image Prevention

Control of diabetes is the best way to prevent ocular complications.

image Clinical Findings

Acute onset of diabetes may be accompanied by sudden blurred vision due to myopia and cataracts.

In children older than 9 years, referral to an ophthalmologist for screening of retinopathy should occur within 3–5 years after the onset of diabetes. Both conditions may be reversible with good glucose control. Young children with type 1 diabetes should be followed for the Wolfram, or DIDMOD, syndrome, in which diabetes mellitus occurs in conjunction with diabetes insipidus, optic atrophy, and deafness.

image Complications

Vision loss due to vitreous hemorrhage, macular edema, neovascular glaucoma, cataracts, or retinal detachment.

image Treatment

Severe proliferative diabetic retinopathy requires pan-retinal laser photocoagulation or vitreoretinal surgery (or both). Cataracts often require surgical removal and intraocular lens placement. Intraocular steroid injections have been used to treat macular edema in adults, but their role in children is not well established.

image Prognosis

Prognosis depends on the severity of the retinopathy and associated complications.

DISEASES OF THE OPTIC NERVE

OPTIC NEUROPATHY

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Optic nerve function is evaluated by checking visual acuity, color vision, pupillary response, and visual fields.

image Clinical Findings

Poor optic nerve function results in decreased central or peripheral vision, decreased color vision, strabismus, and nystagmus. Optic nerve disorders can be due to congenital malformation, malignancy, inflammation, infection, metabolic disorders, and trauma.

The swinging flashlight test is used to assess function of each optic nerve. It is performed by shining a light alternately in front of each pupil to check for an afferent pupillary defect or Marcus Gunn pupillary defect. An abnormal response in the affected eye is pupillary dilation when the light is directed into that eye after having been shown in the other eye with its healthy optic nerve. This results from poorer conduction along the optic nerve of the affected eye, which in turn results in less pupillary constriction of both eyes than occurs when the light is shined into the noninvolved eye. Hippus—rhythmic dilating and constricting movements of the pupil—can be confused with an afferent pupillary defect.

The optic nerve is evaluated as to size, shape, color, and vascularity. Occasionally, myelinization past the entrance of the optic nerve head occurs. It appears white, with a feathered edge (Figure 16–24). Myelinization onto the retina can be associated with myopia and amblyopia. Anatomic defects of the optic nerve include colobomatous defects and pits.

Image

image Figure 16–24. Myelinization of the optic nerve.

image Treatment

Management of the underlying condition resulting in the optic neuropathy is necessary.

image Prognosis

Prognosis depends on the severity of optic neuropathy and the underlying disease.

OPTIC NERVE HYPOPLASIA

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Optic nerve hypoplasia may be associated with absence of the septum pellucidum and hypothalamic-pituitary dysfunction, which is known as septo-optic dysplasia, or de Morsier syndrome.

image Children with septo-optic dysplasia and hypocortisolism are at risk for sudden death during febrile illness from thermoregulatory disturbance and dehydration from diabetes insipidus.

image Pathogenesis

Optic nerve hypoplasia may occur in infants of diabetic mothers and has also been associated with alcohol use or ingestion of quinine or phenytoin during pregnancy. Anatomically, the optic nerve may range from absent (aplasia) to almost full size, with a segmental defect.

image Clinical Findings

Visual function with optic nerve hypoplasia ranges from mildly decreased to absent light perception. If only one eye is involved, the child usually presents with strabismus. If both eyes are affected, nystagmus is usually the presenting sign. Ophthalmoscopy is performed to directly visualize the optic nerves and to determine the severity of the hypoplasia. Neuroimaging of the brain and endocrine consultation should be performed in all patients with bilateral optic nerve hypoplasia.

image Treatment

Sensory amblyopia and significant refractive errors should be treated by an ophthalmologist. Strabismus surgery may be necessary in certain patients. Endocrine abnormalities should be managed as necessary.

image Prognosis

Severe bilateral optic nerve hypoplasia results in blindness.

PAPILLEDEMA

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Papilledema (optic nerve edema) is associated with increased intracranial pressure due to any cause, such as tumor, craniosynostosis, or intracranial infection.

image Pathogenesis

Papilledema is optic nerve head or optic disc edema associated with increased intracranial pressure. Hydrocephalus and intracranial tumors are common causes of papilledema. In idiopathic intracranial hypertension (pseudotumor cerebri), neuroimaging is normal but papilledema, headaches, and pulsating tinnitus may be present. Papilledema occurs almost equally in boys and girls and sometimes is associated with obesity or upper respiratory tract infection. Other associated causes are viral and other infections, corticosteroid use or withdrawal, sinus infection, trauma, tetracycline use, growth hormone supplementation, and venous sinus thrombosis (see Chapter 25). Early in the illness the patient may not notice a change in vision, although the blind spot may be enlarged. Transient obscuration of vision (amaurosis fugax) may occur as the process becomes more long-standing. Further effects on vision will occur as the papilledema becomes chronic and ultimately leads to optic atrophy. Diplopia (double vision) may occur if increased intracranial pressure results in cranial nerve VI palsy. Workup and treatment are directed toward finding the underlying systemic or central nervous system cause.

image Clinical Findings

Direct visualization of the optic nerve by ophthalmoscopy reveals an elevated disc with indistinct margins, increased vessel diameter, vessel tortuosity, and hyperemia. Hemorrhages and exudates are present in more severe cases. Observed changes may be subtle to striking. Optic nerve head changes are usually bilateral and generally symmetrical. Strabismus may occur if a sixth nerve palsy is associated with the underlying condition.

Pseudopapilledema is a normal variant of the optic disc in which the disc appears elevated, with indistinct margins and a normal vascular pattern. Pseudopapilledema sometimes occurs in hyperopic individuals. It is not associated with vision loss, but true papilledema must be ruled out prior to making the diagnosis.

image Differential diagnosis

Pseudopapilledema, optic neuritis.

image Treatment

Treatment of idiopathic intracranial hypertension may be pharmacologic—for example, using acetazolamide, a carbonic anhydrase inhibitor, or a corticosteroid. Discontinue medications that are suspected of causing papilledema or utilize anticoagulation if venous sinus thrombosis is present. Diagnostic lumbar puncture may also be curative. Optic nerve sheath fenestration and ventriculoperitoneal shunt are surgical interventions used when conservative measures fail. Strabismus surgery, Botox (botulinum toxin type A) injection of extraocular muscles, and amblyopia treatment may be necessary in cases of associated cranial neuropathies resulting in strabismus.

image Complications

Optic atrophy and vision loss.

image Prognosis

Prognosis depends on the underlying etiology, duration, and control of the increased intracranial pressure.

Honorat R, Marchandot J, Tison C, Cances C, Chaix Y: Treatment and prognosis of idiopathic intracranial hypertension in children. Retrospective study (1995–2009) and literature review. Arch Pediatr 2011;18(11):1139–1147 [PMID: 21992894].

OPTIC NEURITIS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Papillitis is a form of optic neuritis seen on ophthalmoscopic examination as an inflamed optic nerve head.

image Optic neuritis in children may be idiopathic or associated with multiple sclerosis, acute disseminated encephalomyelitis, Devic disease, or cat-scratch disease.

image Clinical Findings

Optic neuritis is inflammation of the optic nerve and may have the same appearance as papilledema. Papillitis (inflammation of the optic disc) results in a swollen/elevated nerve head, blurred optic disc margins, hyperemia of the nerve, optic disc hemorrhages, and dilated retinal veins. Retrobulbar optic neuritis (inflammation of the optic nerve posterior to the optic disc) has a normal appearing optic disc on examination by ophthalmoscopy. Optic neuritis may be unilateral, whereas papilledema is almost always bilateral. Optic neuritis is associated with an afferent pupillary defect (Marcus Gunn pupil), decreased visual acuity, decreased color vision, and it may also have pain with eye movements. Other central nervous system (CNS) signs or symptoms may be present so a complete review of systems and neurologic examination are important to complete.

Optic neuritis can be associated with viral and other infections, vaccinations, and CNS inflammatory demyelination diseases such as acute disseminated encephalomyelitis, multiple sclerosis, and neuromyelitis optica (Devic Disease). Neoplasms infiltrating the nerve or orbital infections that compress the optic nerve can also result in optic neuritis.

Workup of the patient with optic neuritis includes lumbar puncture and cerebrospinal fluid analysis. Serology should target infectious and inflammatory markers. Neuromyelitis optica can be diagnosed by detecting neuromyelitis optica immunoglobulin G (IgG). Neuroimaging of the brain and orbits is useful adjunct to the workup.

image Differential Diagnosis

Papilledema, pseudopapilledema, systemic immune disorder, infection, or neoplasm.

image Complications

Decreased visual acuity, color vision, peripheral vision, and contrast sensitivity. CNS involvement can result in a variety of complications based on the underlying etiology.

image Treatment

Treatment of the underlying disease.

image Prognosis

Prognosis depends on the underlying disease process.

OPTIC ATROPHY

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Optic atrophy is pallor of the optic nerve noted on ophthalmoscopy.

image Clinical Findings

Optic atrophy is found in children most frequently after neurologic compromise during the perinatal period. An example would be a premature infant who develops an intraventricular hemorrhage. Hydrocephalus, glioma of the optic nerve, craniosynostosis, certain neurologic diseases, and toxins such as methyl alcohol can cause optic atrophy, as can certain inborn errors of metabolism, long-standing papilledema, or papillitis.

Direct examination of the optic nerve by ophthalmoscopy reveals an optic nerve head with a cream or white color and possibly cupping. Neuroimaging is necessary to delineate CNS abnormalities.

image Complications

Vision loss, decreased peripheral vision, and contrast sensitivity.

image Treatment

Treatment of the underlying condition is indicated.

image Prognosis

Prognosis depends on the severity of the optic nerve atrophy and associated neurologic deficits.

DISEASES OF THE ORBIT

PERIORBITAL & ORBITAL CELLULITIS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image The fascia of the eyelids joins with the fibrous orbital septum to isolate the orbit from the lids.

image The orbital septum helps decrease the risk of an eyelid infection extending into the orbit.

image Infections arising anterior to the orbital septum are termed preseptal.

image Orbital cellulitis denotes infection posterior to the orbital septum and may cause serious complications, such as an acute ischemic optic neuropathy or cerebral abscess.

image Pathogenesis

Preseptal (periorbital) cellulitis usually arises from a local exogenous source such as an abrasion of the eyelid, from other infections (hordeolum, dacryocystitis, or chalazion), or from infected varicella or insect bite lesions. S aureusand S pyogenes are the most common pathogens cultured from these sources. Preseptal infections in children younger than 3 years also occur from bacteremia, although this is rare since Haemophilus influenzae and S pneumoniaevaccines are routinely administered. Bacteremia is still an occasional cause of this infection. Children with periorbital cellulitis from presumed bacteremia must be examined for additional foci of infection.

Orbital cellulitis almost always arises from contiguous sinus infection, because the walls of three sinuses make up portions of the orbital walls and infection can breach these walls or extend by way of a richly anastomosing venous system. The orbital contents can develop a phlegmon (orbital cellulitis), or frank pus can develop in the orbit (orbital abscess). The pathogenic agents are those of acute or chronic sinusitis—respiratory flora and anaerobes. S aureus is also frequently implicated.

The frequency of methicillin-resistant S aureus preseptal and orbital cellulitis has increased over the past several years.

image Clinical Findings

Children with preseptal cellulitis present with erythematous and edematous eyelids, pain, and mild fever. The vision, eye movements, and eye itself are normal. Decreased vision, restricted eye movements, and an afferent pupillary defect suggest orbital cellulitis.

Orbital cellulitis presents with signs of periorbital disease as well as proptosis (a protruding eye), restricted eye movement, and pain with eye movement. Fever is usually high. CT scanning or MRI is required to establish the extent of the infection within the orbit and sinuses.

image Differential Diagnosis

Primary or metastatic neoplasm of the orbit, orbital pseudotumor (idiopathic orbital inflammation), and orbital foreign body with secondary infection.

image Complications

Preseptal cellulitis can progress to orbital cellulitis. Orbital cellulitis can result in permanent vision loss due to compressive optic neuropathy. Proptosis can cause corneal exposure, dryness, and scarring. Cavernous sinus thrombosis, intracranial extension, blindness, and death can result from severe orbital cellulitis.

image Treatment

Therapy for preseptal and orbital cellulitis infection is with systemic antibiotics. Treatment of orbital infections may require surgical drainage for subperiosteal abscess in conjunction with intravenous antibiotics. Drainage of infected sinuses is often part of the therapy.

image Prognosis

Most patients do well with timely treatment.

Seltz LB, Smith J, Durairaj VD, Enzenauer R, Todd J: Microbiology and antibiotic management of orbital cellulitis. Pediatrics 2011; 127(3):e566–e572 [PMID: 21321025].

CRANIOFACIAL ANOMALIES

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Craniofacial anomalies can affect the orbit and visual system.

image Craniofacial anomalies occur with craniosynostoses and midface syndromes such as Treacher Collins and Pierre Robin syndromes.

image Fetal alcohol syndrome is associated with similar changes of the ocular adnexa.

image Clinical Findings

Ocular abnormalities associated with craniofacial abnormalities involving the orbits include visual impairment, proptosis, corneal exposure, hypertelorism (widely spaced orbits), strabismus, amblyopia, lid coloboma, papilledema, refractive errors, and optic atrophy.

image Treatment

Orbital and ocular abnormalities associated with craniofacial anomalies often require a multispecialty approach. Management may require orbital and strabismus surgery. Ophthalmologists also treat amblyopia, refractive errors, and corneal exposure if present.

Taub PJ, Lampert JA: Pediatric craniofacial surgery: a review for the multidisciplinary team. Cleft Palate Craniofac J 2011;48(6): 670–683 [PMID: 21740182].

ORBITAL TUMORS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Both benign and malignant orbital lesions occur in children.

image The most common benign tumor is capillary hemangioma (Figure 16–25).

image The most common primary malignant tumor of the orbit is rhabdomyosarcoma.

Image

image Figure 16–25. Right upper lid hemangioma causing ptosis.

image Clinical Findings

Capillary hemangiomas may be located superficially in the lid or deep in the orbit and can cause ptosis (see Figure 16–25), refractive errors, and amblyopia. Deeper lesions may cause proptosis. Capillary hemangiomas in infants initially increase in size before involuting at about age 2–4 years.

Orbital dermoid cysts vary in size and are usually found temporally at the brow and orbital rim or supranasally. These lesions are firm, well encapsulated, and mobile. Rupture of the cyst causes a severe inflammatory reaction.

Lymphangioma occurring in the orbit is typically poorly encapsulated, increases in size with upper respiratory infection, and is susceptible to hemorrhage. Other benign tumors of the orbit are orbital pseudotumor, neurofibroma, teratoma, and tumors arising from bone, connective tissue, and neural tissue.

Orbital rhabdomyosarcoma (see Chapter 31) grows rapidly and displaces the globe. The average age at onset is 6–7 years. The tumor is often initially mistaken for orbital swelling due to insignificant trauma.

Tumors metastatic to the orbit also occur; neuroblastoma is most common. The patient may exhibit proptosis, orbital ecchymosis (raccoon eyes), Horner syndrome, or opsoclonus (dancing eyes). Ewing sarcoma, leukemia, Burkitt lymphoma, and Langerhans cell histiocytosis may involve the orbit.

Examination of vision, eye movements, eyelids, and orbits often reveals amblyopia, eyelid malposition, strabismus, and proptosis. Neuroimaging with CT or MRI is required to delineate the location and size of orbital tumors.

image Differential Diagnosis

Orbital pseudotumor (idiopathic orbital inflammation), orbital cellulitis.

image Treatment

Therapy for capillary hemangiomas includes observation and intralesional or systemic corticosteroids. Topical and systemic β-blockers have shown success in treating capillary hemangiomas but optimal dosages and duration of treatment are still under investigation. Treatment is indicated if the lesion is large enough to cause amblyopia. Induced astigmatism or amblyopia (or both) are treated with glasses and patching, respectively. Treatment of orbital dermoids is by excision.

Rhabdomyosarcoma is treated with radiation and chemotherapy after biopsy confirms the diagnosis. With expeditious diagnosis and proper treatment, the survival rate of patients with orbital rhabdomyosarcoma confined to the orbit approaches 90%.

Treatment of metastatic disease requires management by an oncologist and may require chemotherapy and radiation therapy.

image Prognosis

Prognosis depends on the underlying disease.

Balma-Mena A et al: Propranolol in the management of infantile hemangiomas: clinical response and predictors. J Cutan Med Surg 2012;16(3):169–173 [PMID: 22713439].

Chakkittakandiyil A et al: Timolol maleate 0.5% or 0.1% gelforming solution for infantile hemangiomas: a retrospective, multicenter, cohort study. Pediatr Dermatol 2012;29(1):28–31 [PMID: 22150436].

NYSTAGMUS

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Nystagmus is a rhythmic oscillation or jiggling of the eyes. It may be unilateral or bilateral, more pronounced in one eye, or gaze-dependent.

image Pathogenesis

Nystagmus may be associated with esotropia or may occur with ocular lesions that cause deprivation amblyopia (eg, cataract and eyelid ptosis) or conditions in which the visual pathways are hypoplastic, sometimes referred to as “sensory nystagmus.” Nystagmus is seen with optic nerve hypoplasia, macular hypoplasia, aniridia, and albinism. Nystagmus can also occur with normal ocular structures and seemingly normal CNS development, sometimes referred to as “motor nystagmus.” In the latter instance, the nystagmus may be blocked in certain positions of gaze, in which case a face turn or torticollis may develop. Latent nystagmus occurs when one eye is occluded. This type of nystagmus occurs in patients with congenital esotropia. An associated amblyopia may be present.

Most nystagmus occurring in childhood is of ocular origin, but CNS disease and, less frequently, inner ear disease are other causes. A CNS cause is likely when the nystagmus is acquired. Patients should be referred to an ophthalmologist for evaluation.

image Clinical Findings

Evaluation for iris transillumination defects caused by albinism should be performed since albinism is a common cause of nystagmus.

Spasmus nutans, in which a rapid, shimmering, disconjugate nystagmus occurs with head bobbing and torticollis. Glioma of the hypothalamus can mimic spasmus nutans. Neuroimaging may be necessary to determine if the cause of the nystagmus is due to a CNS disease. An electroretinogram may be required to rule out retinal pathology as the cause of nystagmus if neuroimaging is normal.

image Differential Diagnosis

Opsoclonus.

image Treatment

Therapy is directed at managing the underlying ocular or CNS disease. An ophthalmologist can optimize vision by correcting significant refractive errors and strabismus. The range of vision varies depending on the cause of the nystagmus. Some patients may benefit from extraocular muscle surgery and contact lenses.

image Prognosis

Most affected individuals have subnormal vision but spasmus nutans usually improves with time.

AMBLYOPIA

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Amblyopia is a unilateral or bilateral reduction in vision due to strabismus, refractive errors, and/or visual deprivation.

image Amblyopia can occur only during the critical period of visual development in the first decade of life when the visual nervous system is plastic.

image Approximately 3% of the population is amblyopic.

image Pathogenesis

Amblyopia is classified according to its cause. Strabismic amblyopia can occur in the nondominant eye of a strabismic child. Refractive amblyopia can occur in both eyes if significant refractive errors are untreated (ametropic or refractive amblyopia). Another type of refractive amblyopia can occur in the eye with the worse refractive error when imbalance is present between the eyes (anisometropic amblyopia). Deprivation amblyopia occurs when dense cataracts or complete ptosis prevents formation of a formed retinal image. Of the three types of amblyopia, the deprivation form of amblyopia results in the worst vision.

image Prevention

Vision screening and referral to an eye care professional if amblyopia is suspected.

image Clinical Findings

Screening for amblyopia should be a component of periodic well-child examinations. The single best screening technique to discover amblyopia is obtaining visual acuity in each eye. In preverbal children unable to respond to visual acuity assessment, amblyogenic factors are sought, including strabismus, media opacities, unequal Brückner reflexes (pupillary red reflexes), and a family history suggestive of strabismus, amblyopia, or ocular disease occurring in childhood (see earlier section Ophthalmic Examination).

image Treatment

The earlier treatment is begun, the better will be the chance of improving visual acuity. Treatment is usually discontinued after age 9 years. Amblyogenic factors such as refractive errors are addressed. Because of the extreme sensitivity of the visual nervous system in infants, congenital cataracts and media opacities must be diagnosed and treated within the first few weeks of life. Visual rehabilitation and amblyopia treatment must then be started to foster visual development.

After eradicating amblyogenic factors, the mainstay of treatment is patching the sound eye, which causes the visual nervous system to process input from the amblyopic eye and in that way permits the development of useful vision. Other treatment modalities include “fogging” the sound eye with cycloplegic drops (atropine), lenses, and filters.

image Prognosis

Prognosis depends on the compliance with treatment but usually good.

STRABISMUS

image DIAGNOSIS & TYPICAL FEATURES

image Strabismus is misalignment of the eyes.

image Its prevalence in childhood is about 2%–3%.

image Strabismus is categorized by the direction of the deviation (esotropia, exotropia, hypertropia, hypotropia) and its frequency (constant or intermittent).

image Strabismus may cause or be due to amblyopia.

Esotropia (Crossed Eyes)

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Pseudoesotropia can result from prominent epicanthal folds that give the appearance of crossed eyes when they are actually straight.

image Esotropia is deviation of the eyes toward the nose and may involve one or both eyes.

image Pathogenesis

Congenital esotropia (infantile esotropia) has its onset in the first year of life in healthy infants. The deviation of the eyes toward the nose is large and obvious. Esotropia beginning in the first year also occurs in premature infants or children with a complicated perinatal history associated with CNS problems such as intracranial hemorrhage and periventricular leukomalacia. The most frequent type of acquired esotropia is the accommodative type (Figure 16–26). Onset is usually between ages 2 and 5 years. The deviation is variable in magnitude and constancy and is often accompanied by amblyopia. One type of accommodative esotropia is associated with a high hyperopic refraction. In another type, the deviation is worse with near than with distant vision. This type of esodeviation is usually associated with lower refractive errors.

Image

image Figure 16–26. Accommodative esotropia. A: Without glasses, esotropic; B: with glasses, well aligned at distance; C: at near with bifocal correction.

Esotropia is associated with certain syndromes. In Möbius syndrome (congenital facial diplegia), a sixth nerve palsy causing esotropia is associated with palsies of the 7th and 12th cranial nerves and limb deformities. Duane syndrome can affect the medial or lateral rectus muscles (or both). It may be an isolated defect or may be associated with a multitude of systemic defects (eg, Goldenhar syndrome). Duane syndrome is often misdiagnosed as a sixth (abducens) nerve palsy. The left eye is involved more commonly, but both eyes can be involved. Girls are affected more frequently. Children with unilateral paretic or restrictive causes of esotropia may develop face turns toward the affected eye to maintain binocularity.

After age 5 years, any esotropia of recent onset should arouse suspicion of CNS disease. Infratentorial masses, hydrocephalus, demyelinating diseases, and idiopathic intracranial hypertension are causes of abducens palsy, which appears as an esotropia, lateral rectus paralysis, and face turn. The face turn is an attempt to maintain binocularity away from the field of action of the paretic muscle. Papilledema is often, but not invariably, present with increased intracranial pressure.

Besides the vulnerability of the abducens nerve to increased intracranial pressure, it is susceptible to infection and inflammation. Otitis media and Gradenigo syndrome (inflammatory disease of the petrous bone) can cause sixth nerve palsy. Less commonly, migraine and diabetes mellitus are considerations in children with sixth nerve palsy.

image Clinical Findings

Observation of the reflection of a penlight on the cornea, the corneal light reflex, is an accurate means of determining if the eyes are straight. If strabismus is present, the corneal light reflex will not be centered in both eyes. Observation of eye movements may reveal restriction of eye movements in certain positions of gaze. Alternate cover testing of the eyes while the child is fixating on a near and/or distant target will reveal refixation movements if the eyes are crossed. Motility, cycloplegic refraction, and a dilated funduscopic examination by an ophthalmologist are necessary to determine the etiology of esotropia. Some children require imaging studies and neurologic consultation.

image Complications

Amblyopia and poor stereoacuity/depth perception.

image Treatment

Surgery is the mainstay of treatment for congenital esotropia. Surgery is typically performed between 6 months and 2 years of age in order to obtain optimal results.

Management of accommodative esotropia includes glasses with or without bifocals, amblyopia treatment, and, in some cases, surgery.

Underlying neurologic disease should be referred to the appropriate specialists for further management.

image Prognosis

Usually good.

Exotropia (Wall-Eyed)

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Exotropia is a type of strabismus in which the eyes are divergent/wall-eyed (Figure 16–27).

image Exotropia may be intermittent or constant and involve one or both eyes.

Image

image Figure 16–27. Exotropia. A: Fixation with left eye. B: Fixation with right eye.

image Clinical Findings

The deviation of the eyes toward the ears most often begins intermittently and occurs after age 2 years (see Figure 16–27). Congenital (infantile) exotropia is extremely rare in an otherwise healthy infant. Early-onset exotropia may occur in infants and children with severe neurologic problems.

Evaluation of the corneal light reflex reveals the penlight’s reflection in the deviated eye is displaced nasally. All children with constant, congenital exotropia require CNS neuroimaging. Referral to an ophthalmologist is indicated.

image Complications

Amblyopia and poor stereoacuity/depth perception.

image Treatment

Treatment of exotropia is with surgery, orthoptic exercises, patching, and occasionally glasses.

image Prognosis

Generally good.

Granet DB, Khayali S: Amblyopia and strabismus. Pediatr Ann 201; 40(2):89–94 [PMID: 21323205].

UNEXPLAINED DECREASED VISION IN INFANTS & CHILDREN

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Blindness in infants and children is commonly caused by retinal dystrophies, optic nerve disorders, or cortical visual impairment due to significant CNS disease.

image Pathogenesis

Occult causes of poor vision and blindness in children may be due to hereditary retinal dystrophies such as Leber congenital amaurosis and optic nerve abnormalities, including optic nerve hypoplasia and atrophy.

Cerebral visual impairment, also known as cortical blindness, is manifested as decreased visual attentiveness of varying degree. Cerebral visual impairment can be congenital or acquired. Insults to the optic pathways and higher cortical visual centers are responsible. Asphyxia, trauma, intracranial hemorrhage, and periventricular leukomalacia are some of the causes of cortical visual impairment.

image Clinical Findings

Affected infants will have poor eye contact, fail to fixate and follow a visual target, and be unresponsive to visual threat. Wandering or roving eye movements and nystagmus are common. Eye poking is seen in some infants with low vision.

Referral to an ophthalmologist is indicated to determine the etiology of the low vision. Diagnostic tests such as an electroretinogram and visual evoked response may be required. Imaging studies of the brain, genetics, and neurology consultations may be useful.

image Differential Diagnosis

Delayed visual maturation, vision loss due to an ocular versus neurologic disease.

image Treatment

Low-vision aids enhance remaining vision. Devices used include magnifiers for both distance and near vision, closed-circuit television, and large-print reading materials. Vision rehabilitation specialists and support groups can help teach the affected child and their family how to best use these devices. Clinical trials are underway for treatment of Leber congenital amaurosis with gene therapy.

image Prognosis

Generally poor for vision.

Hufnagel RB, Ahmed ZM, Corrêa ZM, Sisk RA: Gene therapy for Leber congenital amaurosis: advances and future directions. Graefes Arch Clin Exp Ophthalmol 2012;250(8):1117–1128 [PMID: 22644094].

LEARNING DISABILITIES & DYSLEXIA

image ESSENTIALS OF DIAGNOSIS & TYPICAL FEATURES

image Learning disabilities and dyslexia result in poor reading comprehension and writing.

image Children often have vague complaints of ocular fatigue, headaches, and difficulty reading.

image Clinical Findings

Evaluation of the child with learning disabilities and dyslexia should include ophthalmologic examination to identify any ocular disorders that could cause or contribute to poor school performance. Most children with learning difficulties have no demonstrable problems on ophthalmic examination.

image Treatment

A multidisciplinary approach is recommended by the AAP, the AAPOS, and the AAO for evaluating and treating children with learning disabilities. There is no scientific evidence to support the use of vision therapy, eye exercises, prisms, colored lenses, or filters to treat learning disabilities or dyslexia.

image Prognosis

Generally good.

Handler SM, Fierson WM, Section on Ophthalmology; Council on Children With Disabilities; American Academy of Ophthalmology; American Association for Pediatric Ophthalmology and Strabismus; American Association of Certified Orthoptists: Learning disabilities, dyslexia, and vision. Pediatrics 2011;127(3):e818–e856 [PMID: 21357342].