Seema S. Mishra
Katherine M. Konzen
HIGH-YIELD FACTS
• Normal visual acuity is 20/40 in a 3-year-old, 20/30 in a 4-year-old, and 20/20 in a 5- to 6-year-old child.
• Steroids should not be used for patients with iritis or keratitis until herpes simplex is excluded.
• Glaucoma should be suspected in patients who have eye pain and nausea and vomiting.
• Neonates with suspected gonococcal conjunctivitis should undergo a complete sepsis work-up, including a lumbar puncture. These patients should be admitted for intravenous antibiotics.
• Chemical alkali burns to the eye can result in liquefactive necrosis and should be irrigated until the eye pH is between 6 and 8.
Children with eye disorders often come to the emergency department (ED). It is imperative that the emergency physician performs a complete eye examination in order to avoid overlooking potentially debilitating ophthalmologic conditions. The physician must remember certain important guidelines when facing patients with ocular disease.
A thorough and complete history must be taken. Have there been previous eye problems or surgeries? Are there underlying health problems? Does the patient wear glasses or contact lenses? What was done for the patient prior to arrival in the ED? In the absence of trauma, is eye pain present? Has there been eye discharge or exposure to others with similar conditions? Has there been use of systemic or topical medications?
The visual acuity in both eyes must always be checked. Information about the unaffected eye can help guide one in the assessment of the affected eye. The eye examination should be performed in a logical, methodical manner. Patients should be observed for any facial asymmetry. Toys or other objects that hold the interest of the child and allow proper evaluation of the visual fields should be used. The eye should be touched and dilated only after a thorough systemic examination, and only if indicated.
PHYSICAL EXAMINATION OF THE EYE AND DIFFERENTIAL CONSIDERATIONS
A thorough and systematic eye examination is divided into six major categories: vision, lids and orbit, anterior segment, pupils and extraocular movements, posterior segment, and intraocular pressure.
VISION
For very young children, the ability to focus on an object such as a toy may give a rough assessment of visual acuity. A newborn can fixate on a close object and a 1-month-old infant should be able to follow a moving object. For older children, Snellen letters or Allen figures are useful to check visual acuity in both eyes. Normal visual acuity is 20/40 in a 3-year-old, 20/30 in a 4-year-old, and 20/20 in a 5- to 6-year-old child. Vision can be impaired from any obstruction of the visual pathway.
LIDS AND ORBIT
The lids must be examined by testing the ability to raise and lower the eyes and noting any erythema, edema, lacerations, or ecchymosis. Children with periorbital cellulitis will often have significant edema and erythema of both the upper and lower eyelids. The upper lid must be everted to rule out the presence of a foreign body by firmly grasping the lashes at the lid margin and everting the lid against countertraction at the superior tarsal margin using a cotton-tip applicator.
Examination of the orbit includes palpation for defects in the orbital bony structure or for subcutaneous emphysema. Orbital fractures are often accompanied by ecchymosis, lid swelling, proptosis, and limitation in extraocular movements. Herniation and entrapment of the inferior rectus muscle within the orbital floor fracture results in paralysis of upward gaze. Sinus fractures may be associated with subcutaneous emphysema. Physicians should note the presence of exophthalmos or enophthalmos.
ANTERIOR SEGMENT
Inspect the sclera and conjunctiva for swelling, erythema, foreign bodies, hemorrhage, or discharge. Diseases of the cornea and conjunctiva are divided into two main categories of infection or trauma. The history should lead one to the most likely problem. Infections are of bacterial, viral, or fungal etiologies. Conjunctival infections often begin unilaterally but may spread to the other eye within a few days. Crusting and exudate are usually present. In North America, the most common corneal infection causing permanent visual impairment is herpes simplex. Throughout the rest of the world, the most common agent is trachoma. Traumatic injuries to the cornea should be considered in even the youngest of children and can be the cause of a crying infant. Evaluate the corneal light reflex for both briskness and adequacy of response. Self-inflicted thermal wounds from curling irons, microwave popcorn bags, or other mechanism should be thoroughly evaluated.
The anterior chamber comprises the aqueous humor, iris, and lens. Acute iritis (anterior uveitis) is rare in children and may be associated with juvenile rheumatoid arthritis or sarcoidosis. One should consider the possibility of iritis in children who have unilateral, sudden onset of pain, photophobia, and redness. Physical examination reveals a miotic pupil and perilimbal injection. Aqueous flare and cells are seen on slit-lamp examination. Treatment includes early ophthalmologic consultation, cycloplegics, and steroid drops if recommended by the specialist. Infections of the uvea can be caused by bacteria, fungi, viruses, or helminths. Measles, mumps, and pertussis may be associated with a uveitis; however, this is not due to direct invasion.
Trauma can cause damage to the anterior chamber. The iris should be evaluated for shape and contour. Under penlight or direct ophthalmoscopic examination, the lens should be clear. If opacification is present, cataracts should be considered. (See Chapter 97, Traumatic Eye Emergencies).
Aniridia presents as an apparent absence of the iris but has many variations. The pupil appears as large as the cornea while the iris remains as a small residual structure. The visual acuity for patients with aniridia is extremely poor due to macular hypoplasia; nystagmus and photophobia are often present. Two-thirds of patients have a hereditary autosomal dominant condition, whereas one-third are sporadic cases. Approximately 20% of infants with sporadic aniridia develop a Wilms tumor, other genitourinary defects, or mental retardation. Other ocular defects associated with aniridia include a displaced lens, cataracts, corneal epithelial dystrophy, and glaucoma.
PUPILS AND EXTRAOCULAR MOVEMENTS
Pupils should be black, round, symmetric, and equally reactive to light. Changes in the anterior chamber, lens, or vitreous may result in a pupil that is not black. A ruptured globe or intracranial process can lead to pupillary asymmetry. Assess extraocular movements in all visual fields and clearly document all deficits Pupillary assessment includes evaluation for an afferent pupillary defect known as a Marcus Gunn pupil in which pupillary constriction is delayed and diminished in both eyes when light is shone in the affected eye as compared to the normal eye. A Marcus Gunn pupil is the evidence of injury to the anterior visual system and is a poor prognostic sign.
A white spot on the pupil, leukocoria, can be due to congenital cataract, coloboma, retinopathy of prematurity, retinal dysplasia, congenital toxoplasmosis, old vitreous hemorrhage, retinoblastoma, or retinal detachment, in addition to a wide variety of other hereditary, developmental, inflammatory, and miscellaneous conditions. It is essential for patients with leukocoria to have a thorough fundoscopic examination by an ophthalmologist.
POSTERIOR SEGMENT
The posterior segment comprises the vitreous, retina, and optic nerve. Direct ophthalmoscope can be used to examine for papilledema, hemorrhages, retinal detachment, and intraocular foreign bodies. Chronic conditions including uveitis can cause deposits in the vitreous. Endophthalmitis (infections inside the eye) may result from penetrating injury, worsening superficial infection, or surgery. Children will have unilateral severe pain in or around the eye and compromised vision. Purulent exudate in the vitreous will show up as a greenish color on the ophthalmoscopic examination. Often, a hypopyon (pus in the anterior chamber) is seen.
Blunt or penetrating trauma to the eye can lead to a vitreous hemorrhage. Other causes of hemorrhage include diabetes mellitus, hypertension, sickle cell disease, leukemia, retinal tears, central retinal vein occlusion, and tumor. These patients typically present with sudden loss of vision.
Retinal artery and retinal vein obstruction are relatively uncommon in the pediatric population and etiologies include trauma and other systemic entities. Retinal artery occlusion is a true ocular emergency and can be due to emboli in patients with endocarditis and systemic lupus erythematosus or result from hypercoagulability in patients with sickle cell disease. When central retinal artery occlusion occurs, there is sudden, painless loss of vision in one eye. Ophthalmoscopic examination reveals the cherry-red spot of the fovea, a pale optic nerve, and markedly narrowed arteries. A Marcus Gunn pupil may be present. Ophthalmology consultation should be immediately obtained for possible paracentesis of the anterior chamber to decompress the globe. Temporizing measures include ocular digital massage, topical beta-blocker (Timoptic 0.5% one drop, twice a day), Diamox (500 mg IV or PO), and CO2rebreathing by having patients blow into a paper bag for 5 to 10 minutes.
Retinal vein obstruction also leads to sudden painless loss of vision that varies depending on the extent of the obstruction. Retinal hemorrhages and a blurred, reddened optic disk may be seen. These findings are often described as a “blood and thunder” fundus. Arteries are often narrowed, veins are distended, and there may be white exudates. Retinal vein obstruction can occur in trauma as well as leukemia, cystic fibrosis, and retinal phlebitis. An ophthalmologist should be consulted. Aspirin therapy may be initiated to inhibit further thrombosis.
Retinal tears can lead to vitreous hemorrhage causing diminished vision in the affected eye. Retinal detachment may take years to develop after a tear. As the detachment progresses, patients may have a visual field deficit or may complain of flashing lights or a “curtain” draping over the affected eye. Ophthalmoscopic examination will reveal a light-appearing retina in the area of detachment.
The optic nerve is responsible for the transmission of visual information to the cortex. Disruptions in this transmission can lead to visual loss. Optic neuritis is usually due to inflammation or demyelination. It is characterized by an abrupt, rapid, unilateral loss of vision while pain is variable. Rarely does optic neuritis present as a separate entity in children. Most often it is caused by meningitis, viral infections, encephalomyelitis, and demyelinating diseases. Lead poisoning and long-term chloramphenicol therapy are other known culprits.
Various toxins have been associated with impaired vision. Most act on the ganglion cells of the retina or optic nerve causing visual defects. Methyl alcohol can cause sudden, permanent blindness. Other recognized toxins include sulfanilamide, quinine, quinidine, and halogenated hydrocarbons.
Finally, one must consider that visual loss can result from impedance within the visual cortex of the brain. Head trauma, hypoglycemia, leukemia, cerebrovascular accidents, and anesthetic accidents can all be associated with cortical blindness.
INTRAOCULAR PRESSURE
Either infection or injury to the anterior chamber can lead to increased intraocular pressure. Glaucoma can manifest any time after an insult to the eye. Pain and blurred vision should alert one to the possibility of glaucoma. The pupil is nonreactive and dilated, and patients may complain of seeing halos around objects. Accurate measurement is accomplished by slit-lamp tonometry or with a handheld tonometer. This should not be undertaken, however, if the possibility of a ruptured globe exists. In patients with acute glaucoma, rough tactile measurement of intraocular pressure can be made by gentle palpation of the globes with the fingers through the eyelids. An extremely firm eye can easily be detected. Normal eye pressure in children ranges from 10 to 22 mm Hg. An ophthalmologist must be immediately involved in the care and treatment of children with suspected glaucoma. Immediate medical management includes a combination of topical pilocarpine 1% to 2% once the intraocular pressure is below 40 mm Hg, adrenergic agents, mannitol 1 to 2 g/kg IV, and carbonic anhydrase agents. Diamox is most often used as an oral dose of 15 mg/kg/d.
NONTRAUMATIC EYE DISORDERS
EYELID INFECTIONS
Eyelid infections are frequent throughout childhood. The glands of Zeis are sebaceous glands attached directly to the hair follicles; the Meibomian glands are sebaceous glands that extend through the tarsal plate. Eyelid infections (blepharitis) often involve one of these glands. The most common infections of the eyelid include chalazion, hordeolum, impetigo contagiosa, and herpes simplex.
Clinical Findings An external hordeolum or stye is a suppurative infection of the glands of Zeis, whereas an internal hordeolum or chalazion is an acute infection of a Meibomian gland. A chalazion presents as a painless, hard nodule and is often located in the mid-portion of the tarsus, away from the lid border caused from obstruction of the gland duct. Chalazions are uncommon during infancy but frequently occur during childhood. Initially, the swelling may be diffuse but usually becomes localized to the lid margin. Impetigo contagiosa is a pyoderma usually presenting with vesicles; it then develops a yellowish crust, which occurs due to local invasion by staphylococci or streptococci. In patients with impetigo, there can often be an underlying seborrheic dermatitis. Herpes simplex can present on the eyelids of children and can lead to latent infection, which may persist throughout life and be reactivated. Recurrent infection often involves the cornea.
Differential Diagnosis The differential diagnosis of an external hordeolum includes contact dermatitis and allergic conjunctivitis. Itching is a more prominent feature in the latter two entities and is not usually associated with a hordeolum. The differential diagnosis of a chalazion includes rhabdomyosarcoma, capillary hemangiomas, dermoids, orbital cysts, molluscum contagiosum, sarcoidosis, fungal infections, foreign bodies, and juvenile xanthogranuloma. Differentiation is made by lack of response to local therapy and/or biopsy. Impetigo contagiosa and herpes simplex can be easily confused. Cultures should be obtained to ascertain etiology.
Management The management of hordeolum includes warm compresses and eyelid hygiene using baby shampoo or eyelid washes on a washcloth. Twice-daily application of an antistaphylococcal antibiotic ointment (erythromycin ophthalmic ointment or polymyxin B sulfate) or ophthalmic drops should also be initiated. A chalazion is initially managed in the same manner, and antibiotic treatment should be continued for several days after rupture of the chalazion to prevent recurrence. If there is a lack of response to medical treatment, surgical incision and drainage under general anesthesia is recommended for young children. Recurrence commonly results from autoinoculation and inattention to good hygienic care.
Impetigo contagiosa should be treated by removing crusts and with topical antistaphylococcal and streptococcal antibiotics. A cotton-tip applicator soaked in baby shampoo can be used to clean the lid margins. Bacitracin ophthalmic ointment is often effective; however, topical erythromycin and gentamicin can be used. If systemic impetigo is present, oral antibiotics should be initiated.
Herpes simplex blepharitis should be treated with trifluorothymidine topical drops. Oral acyclovir should be considered but may be of limited value. Treatment of herpes simplex will be discussed in further detail in the following sections.
CELLULITIS OF THE PERIORBITAL AND ORBITAL REGION
Periorbital infections are common in childhood and usually resolve with appropriate therapy and without sequelae. The anatomic development of the sinuses in children is thought to play a major role in the development of orbital and periorbital infections.
Periorbital infections, particularly sinusitis, may cause infection or severe inflammation in the orbital tissues thus leading to a preseptal or orbital cellulitis. The proximity of the paranasal sinuses to the orbital walls and the interconnection between the venous system of the orbit and the face allow infection to spread from the sinuses to the orbit either directly or via the bloodstream. Orbital and facial infections can lead to cavernous venous thrombosis.
The orbital periosteum and septum are important anatomic structures, which help to limit direct spread of infection. The orbital periosteum acts as a barrier to the spread of infection from the sinuses; however, it may become eroded if a periorbital abscess develops. The orbital septum may also limit the spread of infection from the preseptal space to the orbit. The following classification has been described for orbital infections:
• Class I: Periorbital or preseptal cellulitis (Fig. 98-1)—Cellulitis is confined to the anterior lamella tissue. Lid edema and erythema may be mild or severe. The globe ordinarily is not involved and so vision and function remain normal.
FIGURE 98-1. A patient with an insect bite and associated preseptal cellulitis. (Used with permission from Dr. Katherine Konzen, Rady Children’s Hospital, San Diego.)
• Class II: Orbital cellulitis—Orbital tissue is infiltrated with bacteria and cells, which extend through the septum into the orbital fat and other tissues. Manifestations usually include proptosis, impaired or painful movement, and periocular pain. Visual acuity may be impaired and septicemia may be present.
• Class III: Subperiosteal abscess (Fig. 98-2)—Purulent material collects between the periosteum and the orbital wall. Medial wall involvement causes the globe to be displaced inferiorly or laterally. Symptoms include edema, chemosis, and tenderness with ocular movement, while vision loss and proptosis vary in severity.
FIGURE 98-2. CT scan demonstrating sinus disease with inflammation of preseptal (lid) structures as well as purulent material in the periosteal space, causing lateral displacement of the globe. (Used with permission from Dr. Melvin Senac, Rady Children’s Hospital, San Diego.)
• Class IV: Orbital abscess—When pus accumulates within the orbital fat inside or outside the muscle cone, an orbital abscess has developed. The infectious process becomes localized and encapsulated, unlike orbital cellulitis, which tends to be more diffuse. Exophthalmos, chemosis, ophthalmoplegia, and visual impairment are generally severe; systemic toxicity may be impressive.
• Class V: Cavernous sinus thrombosis—Thrombosis results from extension of an orbital infection into the cavernous sinus. Nausea, vomiting, headache, fever, pupillary dilation, and other systemic signs may be present. There is marked lid edema and early onset of third, fourth, and sixth cranial nerve palsies.
The bacteriology involved in orbital infections depends on the age of the patient and the underlying problem. In newborn infants and in those up to the age of 5 years, Haemophilus influenzae, Streptococcus pneumoniae, and Moraxella catarrhalis are predominant, particularly in children with upper respiratory tract infections, conjunctivitis, sinusitis, or otitis media. The incidence of infections due to H. influenzaetype B has dropped since the advent of routine vaccination against this pathogen.1 In patients with a history of skin infections or trauma, Staphylococcus aureus and streptococcal species are the main offending agents. The incidence of infections with community-acquired Methicillin-resistant S. aureus continues to increase.2 Polymicrobial and anaerobic infections are more common in older children. Children with cystic fibrosis are susceptible to infections with Pseudomonas aeruginosa.
Fungal orbital cellulitis is uncommon in children. A slowly progressive course of orbital swelling in children with vomiting or dehydration may indicate an underlying fungal infection; immunocompromised and diabetic children may be at greatest risk. Rhizopus and Mucor are the most common fungi causing infections.
Clinical Findings Most of the clinical findings are described with each class of infection. Preseptal or periorbital cellulitis is marked by periorbital edema, erythema, and tenderness but is not accompanied by proptosis, ophthalmoplegia, or loss of visual acuity. Chemosis and conjunctivitis may be present, as well as fever and leukocytosis. Patients with orbital cellulitis present similarly but have further development of ophthalmoplegia, proptosis, pain on eye movement, worsening chemosis, and changes in vision. Fever and leukocytosis are often seen. Blood cultures are positive in up to 25% of patients. If the orbital cellulitis is secondary to sinusitis, headache, rhinorrhea, and swelling of the nasal mucosa may also be present. At times, swelling of the eyelid may be so severe that further evaluation is necessary. Computed tomography (CT) has been useful in the delineation of periorbital cellulitis from orbital cellulitis.
Management In patients with mild periorbital cellulitis and no history of fever or other systemic illness, a thorough physical examination is recommended but laboratory investigation may be unnecessary. Mild cases of preseptal cellulitis due to local trauma or conjunctivitis can be treated with oral antibiotics such as amoxicillin-clavulanate (20–40 mg/kg/d) to cover against S. aureus. Cellulitis associated with an upper respiratory infection may also be treated with amoxicillin-clavulanate or with cefuroxime. For cellulitis secondary to bug bites, oral anti-histamines and warm compresses may also be helpful. Close follow-up is mandatory.
For patients requiring hospitalization, a complete blood cell count, lumbar puncture, cultures of the blood, nasal mucosa, throat and conjunctiva, and CT of the head may be warranted. A lumbar puncture should be considered to rule out meningitis if patients appear toxic and are younger than 2 years or if infection with H. influenzae is suspected. The following management scheme has been recommended by several authors:
• All patients hospitalized for orbital inflammation should receive ophthalmologic and otolaryngology consultation.
• Broad-spectrum antimicrobial therapy should be instituted at once while awaiting blood or intraoperative culture results. Children younger than 5 years of age, without a history of trauma, should be placed on appropriate coverage against H. influenzae type B, S. pneumoniae, and Group A streptococcus. A suggested initial regimen consists of ceftriaxone, 100 mg/kg/d, with the addition of vancomycin, 40 mg/kg/d in severe cases. Children older than 5 years, or those fully immunized with the Haemophilus vaccine, do not generally require coverage for Haemophilus; appropriate antimicrobials are similar to those used for treatment of severe sinusitis.
• Attempts must be made to delineate if the cellulitis is of preseptal or postseptal origin. CT of the head is a helpful diagnostic aid but may not differentiate between subperiosteal abscess and reactive periosteal edema.
• Surgical indications include diminishing visual acuity, lack of improvement despite adequate antibiotics, or spiking fevers suggesting possible development of orbital abscess or cavernous venous thrombosis.
Orbital cellulitis secondary to sinusitis should be managed with the consultation of ophthalmology and otolaryngology. Intravenous antibiotics should consist of a third-generation cephalosporin and a penicillinase-resistant penicillin. If the potential for an anaerobic infection exists, clindamycin may be substituted for the penicillinase-resistant penicillin. Frequent ophthalmologic examination with thorough clinical reassessment is warranted to determine response to treatment and need for surgical intervention (Fig. 98-3).
FIGURE 98-3. Algorithm delineating the inpatient and outpatient diagnosis and management of preseptal and orbital cellulitis. (Used with permission from Dr. Lauren P. Ortega, Medical City Hospital, Dallas, TX.)
Complications In addition to the previously mentioned complications of cavernous venous thrombosis and meningitis, blindness has been associated with postseptal cellulitis. In the pre-antibiotic era, up to 20% of patients with postseptal inflammation developed blindness. Alarmingly, recent studies report a 10% incidence of blindness resulting from orbital complications of sinusitis. Clearly, broad-spectrum antibiotics and modern surgical techniques have not totally alleviated this devastating complication. In fact, negative or equivocal CT findings often contributed to an inappropriate delay in surgical intervention. CT of the head cannot solely determine patient management; clinical judgment must prevail.
SCLERITIS AND EPISCLERITIS
The sclera, made up mainly of collagen and connective tissue, is the thick vascular covering of the eye. Scleritis is uncommon but can be associated with juvenile rheumatoid arthritis or various infectious processes including herpes simplex, varicella zoster, mumps, syphilis, and tuberculosis.
The thin vascular membrane between the sclera and conjunctiva is called the episclera. Inflammation of this area produces some irritation but not the severe pain associated with scleritis. Episcleritis is also associated with a variety of diseases including varicella zoster, syphilis, Henoch–Schonlein purpura, erythema multiforme, and penicillin sensitivity. Episcleritis often presents as a distinct area of injected conjunctiva with dilated vessels in the involved layer of tissue. Differentiation may be helpful with the administration of topical phenylephrine, which constricts vessels dilated by conjunctivitis but not those vessels involved in scleritis or episcleritis. Management consists of treating the underlying disease and some combination of oral nonsteroidal anti-inflammatory agents, topical corticosteroids, and cycloplegics.
CONJUNCTIVITIS
OPHTHALMIA NEONATORUM
Conjunctivitis in newborn infants (first 28 days of life) is not uncommon (Table 98-1). Because of the potential complications from ocular infections in infancy, neonates with symptoms mandate a thorough evaluation. Important guidelines for evaluation include the following:
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TABLE 98-1 |
Ophthalmia Neonatorum |
• Obtaining a detailed maternal history including prenatal care, history of or exposure to venereal disease, duration of rupture of membranes, type of delivery, agent used for ocular prophylaxis at birth, recent exposure to conjunctivitis, and timing of onset of symptoms. History should also include a description of excessive tearing, type and amount of exudate, and elucidation of systemic signs of illness in the baby, such as fever, vomiting, irritability, or lethargy.
• Physical examination must be thorough, including a comprehensive eye examination searching for evidence of eyelid erythema, edema, discharge, corneal ulceration, globe perforation, or foreign body. In addition, general physical examination must be complete; special attention must focus on the skin, respiratory, and genitourinary system for evidence of concomitant systemic involvement.
• Conjunctival scrapings should be obtained for Gram stain, Giemsa stain, and viral and bacterial cultures including Neisseria. A rapid antigen test is sensitive and specific for Chlamydia and can be obtained easily from the conjunctiva. Culture is usually not necessary. Multiplex PCR is now available at many centers to test for viruses such as Herpes Simplex Virus (HSV) 1 and 2, Human herpes virus 6 (HHV-6), cytomegalovirus (CMV) Ebstein–Barr Virus (EBV), and Varicella zoster virus (VZV), all of which can cause a conjunctivitis. Studies have shown that use of fluorescein and topical anesthetics may reduce the accuracy of PCR tests. The eye should be rinsed with saline after the use of these agents and before PCR samples are obtained.3
Differential Diagnosis
Chemical Conjunctivitis Chemical conjunctivitis caused by silver nitrate drops in the immediate newborn period occurs in almost 10% of newborns. Signs of this type of conjunctivitis include bilateral conjunctival hyperemia and mild discharge that begin in the first 24 hours of life and usually subside within 48 hours. Gram stain reveals no organisms and only a few white blood cells. The inflammation is typically quite mild and does not require intervention.
Chlamydia Trachomatis Chlamydia infections have a typical incubation period of 1 to 2 weeks, but can occur earlier if there was premature rupture of membranes. Onset of symptoms may be delayed in babies who received prophylaxis at birth. Typically, the conjunctiva becomes hyperemic and edematous with the palpebral conjunctiva more involved than is the bulbar conjunctiva. Unilateral, purulent involvement is characteristic. Neonates may also have evidence of a concomitant otitis media or afebrile pneumonia. Samples are obtained by scraping the palpebral conjunctiva of the lower lid. The diagnosis is confirmed by identification of chlamydial antigen, detection of intracellular inclusions from Giemsa stain, or isolation of the organism. Antigen detection tests are rapid, sensitive, and specific and are the most efficient means of confirming the diagnosis. Gram stain is not helpful in confirming the diagnosis.
Management Systemic therapy is absolutely essential in the treatment of this condition. The treatment of choice is oral erythromycin (40–50 mg/kg/d) for a 2- to 3-week course to eliminate both conjunctival and nasopharyngeal colonization. Administration of a topical agent is unnecessary. Since chlamydia is the most frequent sexually transmitted disease, prevention by detection of infection in the mother prior to delivery is essential. Treatment of the affected infant’s mother and her partner(s) is also recommended. Early studies suggested that the administration of erythromycin ointment prophylaxis in newborns was effective in preventing chlamydia conjunctivitis but not in altering the rate of development of pneumonia or nasopharyngeal infection. Subsequent studies have revealed that neonatal ocular prophylaxis with erythromycin does not reduce the incidence of chlamydial conjunctivitis. Chlamydial conjunctivitis can lead to chronic changes such as conjunctival scarring and micropannus formation. Fortunately, these long-term ocular sequelae are quite rare. In some cases, chlamydial conjunctivitis may resolve spontaneously without treatment; however, these infants are at risk for reoccurrence of infection involving not only the eyes, but the pharynx, rectum, and lungs as well.
Bacterial Conjunctivitis The role of other bacteria in the newborn period is not quite as clear and can be caused by S. aureus, Haemophilus spp., S. pneumoniae, and Enterococci. Many studies have also shown that these bacteria, in addition to Corynebacterium, Propionibacterium, Lactobacillus, and Bacteroides can be normal flora. Typically, the conjunctiva is red and edematous with some amount of exudate. Diagnosis is made by Gram stain and culture. Broad-spectrum topical antimicrobial therapy is initiated. Untreated cases of bacterial conjunctivitis could potentially progress to corneal ulceration, perforation, endophthalmitis, and septicemia.
Neisseria Gonorrhoeae Historically, gonococcal ophthalmia neonatorum has been of greatest concern because of its serious complications. In the early 1900s, approximately 25% of children admitted to American schools for the blind acquired their disability from N. gonorrhoeae. By the late 1950s, 0.5% were blind as a result of Neisseria. With the onset of antibiotics and postnatal prophylaxis, the current incidence in the United States is thought to be 2 to 3 cases per 10,000 live births. The mean incubation period is 6.5 days with a range of 1 to 31 days. Gonococcal ophthalmia neonatorum classically presents as a purulent, bilateral conjunctivitis. Conjunctival hyperemia, chemosis, eyelid edema, and erythema may also be seen. This entity is diagnosed by Gram stain, revealing gram-negative intracellular diplococci. Cultures should be sent immediately on blood and chocolate agar, because the organisms die rapidly at room temperature. Cultural growth usually occurs within 2 days. Infants with conjunctivitis may have other manifestations of localized disease including rhinitis, anorectal infection, arthritis, and meningitis. Neonates with suspected gonococcal conjunctivitis or any neonate with fever and conjunctivitis should have a sepsis evaluation, including a lumbar puncture. Gonococcal conjunctivitis is considered an ophthalmologic emergency.
Management Treatment must be systemic. Neonates without meningitis should be treated for 7 days with either ceftriaxone or cefotaxime. If meningitis is present, treatment continues for 10 to 14 days. If the organism is sensitive to penicillin, penicillin G can be substituted. Treatment must also include frequent saline irrigation of the eyes. Parents should be screened for gonococcal disease. An infant born to a mother with known active gonococcal infection should receive one dose of ceftriaxone immediately after delivery.
Herpes Simplex Most neonates with herpes simplex become colonized during the birth process. Neonatal herpes simplex may occasionally present first as conjunctivitis. The onset is generally 2 to 14 days after birth. Characteristics are not clinically distinctive; however, unilateral or bilateral epithelial dendrites are virtually diagnostic. Fluorescein staining reveals these defects. Parental history of herpes is important to obtain. Often conjunctivitis leads to further disseminated infections that carry a high morbidity and mortality rate. The conjunctivitis can be diagnosed with conjunctival scrapings looking for multinucleated giant cells and intranuclear inclusions. A fluorescent antibody test should be obtained followed by a viral culture.
Management Treatment should consist of intravenous acyclovir (30 mg/kg/day divided q8h IV for14 to 21 days for neonates, 3 months–12 years: 20 mg/kg/dose IV q8h for10 days) and topical trifluorothymidine (≥6 years: 1 drop onto cornea q2h) while awake until re-epithelialization (not to exceed 9 gtt/d), then 1 drop q4h (minimum 5 drops/d) for 7–21 days. Parents must be aware of the high risk of recurrence of keratitis later in life; an ophthalmologist should follow these children closely. Recurrences are treated with topical therapy alone. Neonatal herpes simplex can lead to the development of keratitis, cataracts, chorioretinitis, and optic neuritis in addition to numerous other ocular problems.
Viral Etiologies (Nonherpetic) Other viral causes of conjunctivitis in neonates are infrequent. Conjunctivitis in a sibling or parent is the most likely source of infection. Hands or fomites are the modes of transmission. Diagnosis is made by history of recent exposure and clinical findings. Usually infections are self-limited. Education regarding hand-washing and not sharing of washcloths and towels is necessary.
Obstructed Nasolacrimal Duct Congenital nasolacrimal duct obstruction, or dacryostenosis, is often only recognized when infants have a history of recurrent ocular infections. The blockage is frequently caused by failure to canalize a membrane called the valve of Hasner, which is located at the lower end of the nasolacrimal duct. Affected infants often have pooling of tears onto the lower lid and cheeks and maceration of the eyelids. Upon crying, tears fail to arrive at the external nares. It is important to differentiate nasolacrimal duct obstruction from congenital glaucoma. Congenital glaucoma presents with tearing, photophobia, and a cloudy, enlarged cornea. Redness is not a major feature of nasolacrimal duct obstruction. Conservative treatment consists of massaging the lacrimal sac, suppressive topical antimicrobials, and warm compresses. Probing of the nasolacrimal system is not recommended until after 1 year of age because 95% of children younger than 13 months will experience spontaneous opening of the lacrimal duct (Fig. 98-4).
FIGURE 98-4. A newborn infant with congenital lacrimal duct stenosis causing dacryocystitis. (Used with permission of Shira L. Robbins MD, FAAP, FAAO; American Academy of Pediatrics; Challenging Cases in Pediatric Ophthalmology, 2012)
Noninfectious Etiologies The differential diagnosis of the red eye in neonates should also include noninfectious etiologies. Corneal abrasions can be detected in infants and may often be secondary to a scratch from their fingernail. Conjunctival hyperemia may be present; fluorescein staining is diagnostic. Linear abrasions on the superior aspect of the cornea should alert the physician to an upper eyelid foreign body. Trauma to the eye during delivery can also cause a corneal abrasion or laceration.
CONJUNCTIVITIS BEYOND THE NEONATAL PERIOD
Conjunctivitis is a frequently encountered entity in children (Table 98-2).
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TABLE 98-2 |
Conjunctivitis in Childhood |
It is crucial to identify conjunctivitis from more serious conditions. Conjunctivitis in older children is characterized by normal vision, a gritty sensation in the eye, diffuse injection, and exudate. Photophobia and lacrimation are not usually associated with conjunctivitis. Keratitis and iritis typically are associated with impaired vision, true pain, photophobia, and lacrimation.
Clinically, viral conjunctivitis is difficult to distinguish from bacterial conjunctivitis. Marked exudate, severe injection, and lid matting is more typical of bacterial or chlamydial infections. Preauricular adenopathy is often associated with viral infections. Follicles on the palpebral conjunctivae are more indicative of viral or chlamydial infections.
Bacterial Conjunctivitis The bacteriology of conjunctivitis in children is expansive and includes Haemophilus aegyptius, S. pneumoniae, S. aureus, N. gonorrhoeae and meningitides, Escherichia coli, P. aeruginosa, Proteus spp., viridans streptococci, S. pyogenes, Corynebacterium diphtheria, Chlamydia trachomatis, and M. catarrhalis. Outbreaks of acute catarrhal conjunctivitis, also known as “pink eye,” may occur in day care or among school-age children. The offending organisms are most frequently S. pneumoniae or H. aegyptius. Outbreaks are more often seen in the winter months. The role of S. aureus in nontraumatic conjunctivitis is difficult to determine because it occurs frequently in asymptomatic patients. In addition, Haemophilus has been associated with concomitant otitis media, and subsequent studies have coined the term conjunctivitis-otitis syndrome when the two occur together. The otitis associated with this syndrome may be asymptomatic, but when detected should be treated with oral antibiotics. The addition of topical antibiotics may not increase efficacy of treatment.
Pseudomonas is an infrequent pathogen that can cause an acutely advancing necrotizing picture, so it must be recognized and treated aggressively. Children with cystic fibrosis and those who wear contact lenses are at highest risk for infection with Pseudomonas. These patients should be evaluated by an ophthalmologist to assess for the presence of corneal ulceration. Although most types of acute bacterial conjunctivitis are self-limited, the use of topical antibiotic therapy is thought to more quickly eradicate the organism, thereby decreasing the amount of time patients are contagious. Routine Gram stain and culture usually is unnecessary unless there is a history of copious mucopurulent exudate (Neisseria) or a chronic history of conjunctivitis.4
Treatment is empiric with topical antimicrobial ointments or ophthalmic drops. Specific drugs include tobramycin drops, ciprofloxacin drops or ointment, ofloxacin drops, polymyxin B sulfate drops, erythromycin ointment, azithromycin, trimethoprim-polymyxin drops, or gentamicin drops. Typical treatment is for 7 days, three to four times a day.
Contact lenses can cause conjunctivitis and corneal abrasions. Lens wear should be discontinued; storage and cleaning solutions must be replaced to prevent further contamination. Topical antibiotics to avoid Pseudomonas or secondary bacterial infection may be initiated. An aminoglycoside such as tobramycin (Tobrex) drops or a fluoroquinolone such as ciprofloxacin (Ciloxan) or ofloxacin (Ocuflox) should be administered four times a day for 5 to 7 days. Corneal ulcers should always be considered in patients who wear contact lenses and have a red eye. These patients should be referred to an ophthalmologist.
Viral Conjunctivitis Adenoviruses are the most common cause of viral conjunctivitis in children. There are a few clinical syndromes associated with this group of viruses including pharyngoconjunctival fever, epidemic keratoconjunctivitis, and nonspecific follicular conjunctivitis. Pharyngoconjunctival fever is most common in children and is associated with an upper respiratory tract infection, regional lymphadenopathy, and fever. The illness is usually self-limited, lasting 1 to 2 weeks. Enteroviral infections from particular coxsackieviruses and echoviruses may cause conjunctivitis but are often associated with other clinical signs including rash or aseptic meningitis. Acute hemorrhagic conjunctivitis is caused by enterovirus 70 or coxsackie A24 and also occurs in epidemics. Transmission is by direct contact with an incubation of less than 2 days. Clinically, patients present with sudden onset of unilateral ocular redness, excessive tearing (epiphora), photophobia, pain, purulent discharge, and eyelid swelling, which develop in a span of 6 to 12 hours. Patients may complain of a burning pain often described as a foreign body. In 80% of the cases, the other eye becomes involved within 24 hours. Some patients develop subconjunctival hemorrhages, which are usually located beneath the superior bulbar conjunctiva. Malaise, myalgias, fever, headache, and upper respiratory tract symptoms may also accompany conjunctivitis. Conjunctival scrapings for a viral culture yield identification of the virus. Ophthalmologic sequelae are rare: <5% of cases develop a secondary bacterial conjunctivitis.
Management Treatment is symptomatic with cool compresses. Many physicians prescribe a topical antimicrobial to prevent secondary bacterial infection, but this practice has not been proven to be effective. Patients with epidemic keratoconjunctivitis should be kept out of school for 2 weeks to prevent an outbreak.
Herpes Simplex and Varicella-Zoster Vesicular lesions on the eyelid can be due to herpes simplex, varicella-zoster, impetigo, or contact dermatitis. Herpes simplex infections are characterized by unilateral, follicular conjunctivitis with vesicles localized to the eyelids. Preauricular lymphadenopathy is commonly present. Fifty percent of the patients develop keratitis within 2 weeks. The virus remains latent in the sensory ganglion and lacrimal glands. Approximately 25% of all children will have recurrences; these usually begin with corneal involvement. Long-term complications include necrotizing stromal disease, diffuse retinitis, and scarring. Herpes simplex is the most common cause of severe corneal ulceration in children and is second only to trauma as a cause of corneal blindness in children. Trifluorothymidine (≥6 years: 1 drop onto cornea every 2 hour) while awake until reepithelialization (not to exceed 9 gtt/d), then 1 drop q4h (minimum 5 drops/d) γ—7 to 21 days is the preferred agent for the treatment of herpes simplex because of its increased solubility, diminished toxicity, and lack of viral resistance.5Approximately 95% of the corneal ulcers treated with it are cured within 2 weeks; however, treatment should be extended for 1 additional week after resolution of the lesions. Rarely corneal debridement is required. For herpetic eye lesions, systemic acyclovir is not recommended because the drug does not penetrate the avascular cornea (Figs. 98-5 and 98-6).6
FIGURE 98-5. Ocular herpes simplex. This 10-year-old has had recurrent ocular herpes simplex since the age of 6 years. Vesicles should not be mistaken for hordeola. (Reproduced with permission from Knoop, et al. Atlas of Emergency Medicine. 3rd ed. New York, NY: McGraw-Hill; 2010. (Photo contributed by Lawrence B. Stack, MD.)
FIGURE 98-6. This microscopic view of a patient with herpes keratitis demonstrates the stereotypical conjunctival dendrite (branching ulceration) seen with this infection. (Used with permission of Shira L. Robbins MD, FAAP, FAAO; American Academy of Pediatrics; Challenging Cases in Pediatric Ophthalmology, 2012.)
Ocular involvement with varicella is relatively uncommon, occurring in <5% of cases. In chicken-pox, the conjunctiva can become involved through two mechanisms: eyelid vesicles can slough virus into the conjunctival cul-de-sac or vesicle formation can take place on the conjunctival surface. Occasionally, the cornea is involved. Fluorescein staining of the cornea and conjunctiva is necessary.
Zoster is uncommon in children, with only 5% of all cases occurring in children younger than 5 years of age. Zoster infections of the eye notably follow the distribution of the first division of the trigeminal nerve. Lesions are usually located on the forehead and upper eyelid and can be located on the tip of the nose. Varicella and zoster conjunctivitis may be treated with oral acyclovir.
Neisseria Gonorrhoeae Gonococcal eye infections can occur in prepubertal children. A nonvenereal mode of transmission has been suggested. Intravenous antibiotics are still recommended for this age group.
Gonococcal conjunctivitis can occur in sexually active children and adolescents; the mode of transmission is similar to that of adults. Treatment may consist of ceftriaxone, 1 g IM plus saline irrigation. Alternatively, ceftriaxone 1 g IM or IV may be administered for 5 days along with saline irrigation.
Occasionally a Neisseria conjunctivitis is attributable to N. meningitides. The eye may act as a portal and patients may develop meningococcemia and/or meningitis. These patients should be treated with ceftriaxone and their contacts given prophylaxis with Rifampin.
Chlamydia Trachomatis Chlamydial eye infections can occur outside of the newborn period. Infection of children and adolescents may warrant an investigation for child sexual abuse and/or other concomitant sexually transmitted infections. Treatment consists of systemic oral erythromycin for 2 to 3 weeks. Children aged 1 and older may be treated with a single oral dose of azithromycin (20 mg/kg with a maximum dose of 1 g).7
Allergic Conjunctivitis
Seasonal and Perennial Allergic Conjunctivitis Itching is frequently the hallmark of allergic conjunctivitis. Seasonal allergic conjunctivitis has its onset of symptoms in either the fall or spring. Patients with sensitivity to grass have more symptoms in the spring, whereas individuals sensitive to ragweed have more symptoms in the fall. Patients often complain of bilateral itchy, watery eyes with a burning sensation. The conjunctiva is mildly inflamed with varying degrees of edema. Perennial allergic conjunctivitis is a variant with symptoms on a year-round basis and often allergens such as dust, mites, animal dander, and feathers are responsible for it. This conjunctivitis represents a type I hypersensitivity reaction.
Management Treatment consists of a combination of topical vasoconstrictors (naphazoline-antazoline and naphazoline pheniramine), antihistamines (levocabastine 0.05%, emedastine difumarate 0.05%, epinastine, azelastine; usually used at one drop twice a day), topical steroids (rimexolone 1% or loteprednol etabonate 0.2%; one drop four times a day), and anti-inflammatory agents (ketorolac 0.5% or diclofenac 0.1%, one drop four times a day). Systemic anti-histamines such as loratadine may be of some benefit. Mast cell stabilizers (Cromolyn sodium 4%, nedocromil 2%, pemirolast potassium 0.1%, and lodoxamide tromethamine 0.1%) eye drops have also been shown to be effective when used as a prophylactic agent (Fig. 98-7). They should be used in conjunction with antihistamines as they do not relieve existing symptoms. Olopatadine and ketotifen (both one drop, twice a day) are both anti-histamines and mast cell stabilizers.
FIGURE 98-7. This patient is suffering from chemosis associated with allergic conjunctivitis. (Reproduced with permission from Knoop K: The Atlas of Emergency Medicine, 3rd edition. McGraw-Hill Professional; 2009.)
Vernal Conjunctivitis Vernal keratoconjunctivitis is a rare condition mainly affecting children under the age of 10. It is common in warm, dry climates, and occurs most commonly in males (male-to-female ratio of 2:1). Often there is a significant history of atopy. Peak incidence is between April and August. Patients usually have a history of bilateral itching, foreign-body sensation, clear mucoid discharge, photophobia, and injection. The giant papillae involve the upper tarsal conjunctiva and consist of large “cobblestone” papillae. The patho-physiology is not entirely clear; IgE and IgG are thought to play a role. The mainstay of treatment consists of mast cell stabilizers and topical antihistamines. Systemic antihistamines, anti-inflammatory drops, and steroid drops may also be added. Cyclosporine drops are reserved for steroid-resistant disease.8
Special Forms of Conjunctivitis Patients with Stevens–Johnson syndrome may have severe conjunctival involvement. In the acute phase of the disease, the palpebral and ocular conjunctiva can scar together. Often goblet cells are lost in the conjunctival epithelium and the mucous layer of tear film is lost. As mucus allows tear film to stick to the surface of the eye, the dry-eye state of Stevens–Johnson syndrome is characterized by abundant tears that do not cover the surface of the eye because they are unable to adhere to it. Treatment consists of a combination of topical lubricants and antibiotics.
Kawasaki disease is associated with a bilateral bulbar, nonexudative conjunctivitis. This diagnosis should be suspected in patients who have fever for more than 5 days and have conjunctivitis, strawberry tongue, cervical adenopathy, fissuring of the lips, diffuse oral injection, erythema and induration of the hands and feet, rash, and desquamation of the fingers and toes. Not all symptoms are required to make the diagnosis.
A chronic blepharoconjunctivitis can be caused by Phthirus pubis when the eyelashes are infected by nits or by the bug itself. The only recognized lice to infect the eyelashes are pubic lice. The type of conjunctivitis seen with lice results from a hypersensitivity reaction. Systemic treatment of the organism is necessary for successful eradication. Eye ointments have been used for treatment because they are thought to paralyze and smother the lice. A cotton-tip applicator should be used for debridement prior to the placement of the ointment.
Molluscum contagiosum can cause conjunctivitis when the virus is shed into the eye. Typically, it causes a chronic conjunctivitis that does not respond to topical antimicrobials. The problem results from the virus protein, which is toxic to the eye. One may see lesions on the eyelids that are often buried between the eyelashes. Eradication of the virus requires that the lesions be opened with a needle and the central core of the umbilicated region be removed. Bleeding into the core is considered definitive treatment.
Other viral syndromes can be associated with nonspecific conjunctivitis. These include rubella, influenza, mumps, measles, infectious mononucleosis, and cytomegalovirus. Papillomavirus can cause eyelid warts, which shed on the conjunctiva, causing a type of conjunctivitis similar to that described for molluscum contagiosum.
Parinaud oculoglandular syndrome is a rare manifestation of cat scratch disease that occurs when the conjunctiva is directly inoculated with Bartonella henselae or Afipia felis. The condition manifests as a unilateral granulomatous or follicular conjunctivitis. Lymphadenopathy in the preauricular, cervical, and/or submandibular regions is common. Treatment consists of oral doxycycline, erythromycin, or ciprofloxacin. A similar syndrome can be caused by infections with Francisella tularensis, Sporothrix schenckii, Mycobacterium tuberculosis, or Treponema pallidum.
Other systemic diseases presenting with eye findings mimicking conjunctivitis include ataxia-telangiectasia, where large tortuous vessels are noted on the bulbar conjunctiva. Patients with Lyme disease may develop nonspecific conjunctivitis with or without eye pain. There are many different causes of a red eye in children including conjunctivitis in its many forms as well as other local and systemic entities (Table 98-3).
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TABLE 98-3 |
Differential Diagnosis of the Red Eye |
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