CHAPTER 22: Ophthalmology

The surface of the eye needs to stay well lubricated or it can lead to vision-threatening complications in the ICU

PATHOPHYSIOLOGY

• Normal ocular surface protective mechanisms include tear production, intact corneal sensation, blinking, and complete eyelid closure

• Impaired protective mechanisms result in corneal exposure and drying

• Corneal “dryness” (subclinical epithelial breakdown) may progress to corneal abrasion, ulceration, infection, scarring, thinning, and/or perforation if untreated

CLINICAL MANIFESTATIONS

• Risk factors for corneal exposure include loss of protective mechanisms due to deep sedation, neurologic impairment, or eyelid abnormality; overhead warmers; treatments causing air to blow over the eyes

• Risk increases with poor eyelid closure: Low risk with eyelids that close completely, increasing with white sclera showing, highest with cornea or underlying iris showing

• Eye exam may reveal conjunctival redness or swelling, corneal haze or opacity, blunted red reflex

DIAGNOSTICS

• Slit lamp biomicroscope exam and fluorescein staining may reveal punctate erosions, corneal abrasion, opacity (ulcer), thinning, or perforation

MANAGEMENT

• Prophylaxis for at-risk patients (e.g., intubated and sedated) is critical:

  • ✓ Lubricating eye ointment (Lacri-lube ointment which consists of mineral oil and white petrolatum) with frequency according to eyelid position

  • ✓ Closed lids every 6–8 hours, sclera showing every 4–6 hours, cornea showing every 2–4 hours; the frequency of ointment administration may be reduced if the eye and ointment are then covered with a piece of non-sticky plastic wrap (e.g., saran wrap) to form a “moisture chamber”

  • ✓ Artificial tear drops evaporate quickly and are not useful

• Prompt ophthalmology consultation for red conjunctiva, corneal haze or opacity, or if the cornea is visible due to incomplete eyelid exposure in an at-risk patient

• Antibiotic ophthalmic ointment (erythromycin, polysporin) if there is corneal epithelial staining with fluorescein

• Complicated cases may require tarsorrhaphy (suturing of eyelids), bandage contact lens, corneal gluing, or emergent corneal transplantation

The cornea should always be clear with visible iris details and a bright red reflex, and any opacity, whether diffuse or focal, is a sign of serious eye disease.

Glaucoma is irreversible optic nerve damage due to increased intraocular pressure.

DIFFERENTIAL DIAGNOSIS OF CORNEAL CLOUDING IN AN INFANT

• Trauma: Forceps injury, corneal perforation with amniocentesis

• Infection: Syphilis, rubella, HSV, bacterial ulcer

• Infantile glaucoma: Associated with enlarged eye (buphthalmos)

• Corneal or limbal dermoid, associated with Goldenhar syndrome

• Anterior segment dysgenesis: Peters anomaly (central corneal opacity), sclerocornea

• Corneal dystrophy: Congenital hereditary endothelial dystrophy (CHED), congenital hereditary stromal dystrophy (CHSD)

• Metabolic: Mucopolysaccharidoses (Hurlers, MPS IH; Type IV, mucolipidoses), cystinosis, tyrosinemia

EPIDEMIOLOGY AND ETIOLOGY

Primary Glaucoma

• Primary infantile glaucoma (congenital glaucoma): 1:10,000–1:15,000; 90% sporadic

• Caused by developmental defect in the structure of the anterior chamber

• Associated systemic syndromes include Sturge–Weber, neurofibromatosis type 1, Marfan, Stickler, Lowe, Rubinstein–Taybi, Wolf–Hirschhorn

• Also associated with ocular syndromes such as aniridia, Peters anomaly

Secondary Glaucomas

• Secondary glaucomas of childhood are more common than primary glaucomas

• Traumatic: Acute glaucoma related to a hyphema (see Hyphema) or glaucoma years after trauma secondary to damage of drainage angle in the eye (angle recession)

• Inflammatory: Caused by trabecular meshwork inflammation or clogging with inflammatory debris; for example, uveitis associated with juvenile idiopathic arthritis

• Steroid-induced: Can be caused by topical, systemic, or inhaled forms of glucocorticoids, by decreasing aqueous outflow

• Aphakic: Absence of the natural lens usually due to cataract extraction; 8% to 41% chance of developing glaucoma

• Intraocular neoplasms such as retinoblastoma, juvenile xanthogranuloma

PATHOPHYSIOLOGY

• Intraocular pressure (IOP) is maintained by the balance of aqueous humor production by the ciliary body and drainage by the trabecular meshwork

• When this drainage system is impaired, IOP increases

• Elevated IOP causes loss of the nerve fiber layer of the retina, optic nerve damage, and, if left untreated, irreversible blindness

CLINICAL MANIFESTATIONS

• Children younger than 3 years present with enlarged eye (buphthalmos), photophobia, epiphora (tearing), blepharospasm (repetitive involuntary closure of the lids), and cloudy cornea (corneal edema)

• Children older than 3 years may have completely asymptomatic glaucoma until advanced, irreversible visual loss occurs

• Check ocular size (typically by measuring corneal diameter, see Diagnostics below), corneal clarity, and visual acuity

• Haab’s striae: Breaks in Descemet’s membrane (basement membrane of the cornea) that occur as the cornea enlarges. Typically seen as horizontal lines in the cornea and develop in children younger than 3 years

• Cupping of the optic nerve: Cup to disc ratio greater than 0.4 is suggestive of glaucoma

• Look for evidence of associated syndromes

DIAGNOSTICS

• Tonometry: Pressure greater than 21 mm Hg in a calm infant is abnormal

• Gonioscopy: Visually assess the angle anatomy

• Measure corneal diameter and axial length to follow response to treatment: Normal corneal diameter is 9.5–10.5 mm in newborns and 11–12 mm in adults

MANAGEMENT

• Ophthalmology should be consulted on all patients with a clinical suspicion of glaucoma or with any corneal opacity regardless of concern for glaucoma

Medical

• Topical Therapy:

  • ✓ Beta-blocker (e.g., timolol maleate 2 times per day) is a common first-line agent

  • ✓ Initial IOP >30 mm Hg or insufficient lowering of IOP to <21 mm Hg requires addition of a carbonic anhydrase inhibitor (e.g., dorzolamide or brinzolamide 2 or 3 times daily) and/or prostaglandin (latanoprost once daily)

• Oral therapy: Acetazolamide for highly or persistently elevated IOP

• Duration of therapy is lifelong unless surgical treatment is performed

Surgical

• Primary congenital glaucoma is a surgical disease

• 80% of children can be cured with:

  • ✓ Goniotomy: An incision is made in the trabecular meshwork with a blade inserted into the anterior chamber. Improves trabecular outflow and reduces IOP. Can only be performed if the cornea is clear

  • ✓ Trabeculotomy ab externo: External dissection of Schlemm’s canal to increase exit of aqueous fluid. Can be done even if corneal is cloudy (corneal edema)

  When primary infantile glaucoma fails above surgeries and other types of glaucomas fail medical management then:

  • ✓ Trabeculectomy: Filtration procedure creating a communication between the anterior chamber and subconjunctival space to allow drainage of aqueous

  • ✓ Glaucoma drainage device: Similar purpose to trabeculectomy but uses an implant

  • ✓ Photocyclocoagulation and cyclocryotherapy: Destroys the ciliary body, thereby reducing production of aqueous fluid; usually a last resort

Red reflex testing should be done on all newborns, infants, and young children

“Leukocoria” is from Greek meaning “white pupil”

EPIDEMIOLOGY

• Congenital cataracts are most common cause of leukocoria: 1/2500 live births

• Retinoblastoma in 1/15,000 people; extremely rare after 6 years of age

DIFFERENTIAL DIAGNOSIS

• Cataract (lens opacity) may result in permanent vision loss if not treated early

• Retinoblastoma is life threatening and the most concerning diagnosis

• Advanced retinopathy of prematurity and retinal detachment

• Strabismus will cause asymmetric red reflexes

• High refractive errors cause blunted red reflex

• Other ocular disorders, including retinal dysplasia (developmental anomaly, present at birth), coloboma (missing piece of tissue in the eye, such as a chorioretinal or optic nerve head coloboma), and Coats disease (retinal vascular abnormalities and lipid exudates)

PATHOPHYSIOLOGY

• The vascular choroid behind the retina reflects light, forming the normal “red reflex”

• Light passes through the cornea, aqueous humor, pupil, lens, and vitreous to get to the retina. Interference by any of these structures may lead to a diminished, irregular, absent, white, or asymmetric red reflex

CLINICAL MANIFESTATIONS

• Red reflex exam: Use a direct ophthalmoscope an arm’s length from the infant’s eyes in a dark room

  • ✓ A normal exam has clear, bilaterally symmetric reflexes with equal color, intensity, and clarity

  • ✓ Any white spots, opacities, or asymmetry are abnormal and require evaluation

  • ✓ Darker pigmented children will have reflexes that appear darker

DIAGNOSTICS

• A patient with abnormal or asymmetric red reflexes or leukocoria urgently requires a full eye exam, including a dilated fundus examination by an ophthalmologist

• Ocular ultrasound by an ophthalmologist and MRI of the eye may help to rule out retinoblastoma

MANAGEMENT

• Depends on etiology

• Congenital cataracts are typically surgically removed by 4–6 weeks of age and require ongoing amblyopia treatment

• Retinoblastoma treatment is begun immediately because mortality is tied to spread of disease; untreated children will die from extension to the brain

• Children with bilateral congenital cataracts may require evaluation for congenital infections (TORCH), metabolic diseases, and chromosomal abnormalities

Uveitis is intraocular inflammation of the uveal tract (iris, ciliary body, choroid)

CAUSES OF UVEITIS

• Idiopathic

• Systemic inflammatory disorders: JIA (especially pauciarticular, ANA positive, rheumatoid factor negative), sarcoidosis, Behcet’s, inflammatory bowel disease, HLA-B27 associated disease

• Infection: Lyme, HSV, CMV, HIV, TB, toxoplasmosis, toxocara, syphilis

• Tubular interstitial nephritis and uveitis (TINU)

• Trauma

• Neoplasm (masquerade syndrome)—Retinoblastoma, leukemia, lymphoma

DIFFERENTIAL DIAGNOSIS

• Conjunctivitis or extra-ocular inflammation does not affect corneal or red reflex clarity

• Key warning signs that a red eye is not a simple conjunctivitis: Vision loss, photophobia, eye pain, eye surgery, contact lens use, chronic (>2 weeks), hyper-purulent discharge, corneal opacity, irregular pupil, poor red reflex, vesicles on lids

CLINICAL MANIFESTATIONS

• Symptoms: Photophobia, pain, redness, decrease in vision, floaters

• Children may be completely asymptomatic (no conjunctival redness, no pain), especially with JIA

• Signs: Conjunctival injection, corneal endothelial deposits, anterior chamber cells, hypopyon (grossly visible collection of white cells behind the cornea), irregular pupil from synechiae (scarring between iris and lens behind it), blunted red reflex, inflammatory lesions on fundus exam

DIAGNOSTICS

• Diagnosis is made visually by slit lamp biomicroscopic and fundus examinations

• Laboratory and radiographic workup for recurrent, bilateral, or posterior uveitis or suspicion of systemic disease

MANAGEMENT

• Uveitis is a site-threatening condition requiring diagnosis and management by an ophthalmologist, often jointly with a pediatric rheumatologist

• Aggressive control of intraocular inflammation with topical steroids (e.g., prednisolone acetate 1% every hour until inflammation is controlled)

  • ✓ Systemic steroids if topical steroids do not adequately control the inflammation or if the posterior structures of the eye are involved

  • ✓ Steroid-sparing immunosuppressive agents (e.g., methotrexate, infliximab) if oral steroids cannot be tapered off without recurrence of uveitis

• Cycloplegic eyedrops (e.g., atropine) while active inflammation is present

• Antibiotics as indicated for infectious causes

Blood in the anterior chamber of the eye

EPIDEMIOLOGY

• Annual incidence of 17–20 per 100,000; mostly younger than 20 years old

PATHOPHYSIOLOGY

• Blunt or penetrating trauma damages iris blood vessels, causing blood to leak into the anterior chamber with potential to increase IOP

• Bleeding stops when a clot forms

• Re-bleeding risk is highest during first 5 days post-trauma when the clot weakens, and can result in pressure spike

• Spontaneous (non-traumatic) hyphema may be caused by intraocular tumors

CLINICAL MANIFESTATIONS

• Patients present with a history of eye trauma, eye pain, and vision loss

• Children may be somnolent

• Some patients have history of bleeding disorders, sickle cell disease, or anticoagulation therapy

• Blood in the anterior chamber appears as a red or dark meniscus in the aqueous humor behind the cornea and in front of the iris

DIAGNOSTICS

• Small hyphemas may only be seen with magnification, best provided by a slit lamp biomicroscope

• Assess visual acuity and IOP

• Rule-out an open-globe injury (see below)

• Sickle cell testing for African Americans, because patients with sickle cell trait or disease are at much greater risk for acute glaucoma with hyphema

MANAGEMENT

• Ophthalmology should be consulted on all patients with hyphema

• Prevent re-bleeding with the following measures:

  • ✓ Bed rest

  • ✓ Avoid NSAIDs

  • ✓ Protect the eye with a plastic or metal shield, which covers but does not touch the eye. Do not apply gauze patches, as these touch the eye

• Admission and/or sedation may be required for young, active children

• Topical mydriatics (e.g., atropine 1% twice per day) and topical steroids (prednisolone acetate 1% four times per day)

• Glaucoma medications for elevated IOP (see Glaucoma management above, but note that topical and systemic carbonic anhydrase inhibitors are relatively contraindicated in sickle cell or sickle trait positive patients with hyphema)

• Consider surgical intervention if: Elevated IOP unresponsive to medications, patients with sickle cell disease, corneal blood staining

• Surgical procedures: Anterior chamber washout and clot removal

Fracture of one or more of the orbital bones due to trauma, including from assault, falls, motor vehicle accidents, and sports

DIFFERENTIAL DIAGNOSIS

• Bruised extraocular muscles, cranial nerve palsy, and orbital edema all present with double vision

• Eye trauma may result in open globe injury, hyphema, retinal detachment, vitreous hemorrhage, lens dislocation or cataract, and/or choroidal rupture

PATHOPHYSIOLOGY

• The orbit is composed of seven bones: Maxilla, zygoma, lacrimal, ethmoid, palatine, sphenoid, and frontal. The walls are thin; the rims are thick

• Most fractures occur in the medial wall or posteromedial floor, near the infraorbital groove, which contains the infraorbital nerve (V2), providing sensation to the cheek and upper alveolus/teeth (Figure 22-1)

• Extraocular muscle may become entrapped in a fracture, causing oculocardiac reflex (vagal nerve) induced bradycardia

• Open globe injuries (full thickness hole in the wall of the eye) may result from penetrating trauma (laceration) or blunt trauma (ruptured globe), in which increased IOP causes a sclera break and may not be accompanied by an orbital fracture

• Extensive retrobulbar (orbital) hemorrhage with orbital fracture may cause an orbital compartment syndrome and ischemic injury to the optic nerve and retina

CLINICAL MANIFESTATIONS

• Patients may be asymptomatic (“white-eyed” blow out fracture) or present with periorbital ecchymosis and swelling, pain on vertical gaze, double vision, and/or decreased sensation in the V2 cranial nerve distribution

• Check ocular motility, which may be decreased if there is muscle entrapment, and palpate orbital rims for a step off

• Extraocular muscle entrapment may cause a symptomatic oculo-cardiac reflex with bradycardia, and pre-syncopal symptoms, particularly with eye movement

• Signs of an open globe injury include

  • ✓ Obvious laceration site

  • ✓ Protruding uvea (appears as brown tissue)

  • ✓ Large amount of subconjunctival hemorrhage

  • ✓ Collapsed anterior chamber (cornea and iris come close to one another)

  • ✓ Peaking of the pupil toward wound site

  • ✓ Decreased vision

• Signs of an orbital compartment syndrome include

  • ✓ Decreased vision

  • ✓ Proptosis

  • ✓ Increased eye pressure with an eye that feels hard to palpation

DIAGNOSTICS

• Thin cut CT in both axial and coronal planes is the study of choice

• Plain film x-rays are insufficient for ruling out an orbital fracture; MRI depicts bones poorly

MANAGEMENT

Nonsurgical

• Ice compresses for 48 hours; elevation of head in bed

• Avoid aspirin and NSAID use, minimize coughing and nose blowing

• The following medications have not clearly been shown to improve outcomes, and some ophthalmologists do not use any of these approaches

  • ✓ Some ophthalmologists consider the use of nasal decongestants to prevent or minimize nose blowing

  • ✓ Oral steroids to reduce edema, particularly if ocular motility is decreased

  • ✓ Antibiotics covering sinus flora (e.g., ampicillin-sulbactam), particularly if there is a concomitant sinus infection

Surgical

• Immediate canthotomy and cantholysis for orbital compartment syndrome with decreased vision

• Urgent surgery for entrapped muscles, particularly if a symptomic oculocardiac reflex is present

• Otherwise, fracture repair is recommended within 1–3 weeks if patient has diplopia, enophthalmos, soft tissue herniation into maxillary sinuses, or if greater than 30% of orbital floor is fractured

• Open globe injuries should be shielded (not patched) and the patient made NPO for surgical repair

• Discuss exam location and timing with the ophthalmology team. For some conditions, a slit lamp biomicroscope exam is important and best performed in the ophthalmology clinic. It may be advantageous to wait until the patient is well enough to travel to the office or to arrange an outpatient visit

• Pupillary dilation with mydriatic eye drops will prevent a reliable pupil exam for 4 or more hours but is necessary for a complete exam

Symptoms or signs as an inpatient

• Red eye

• Vision loss or blurry vision

• Eye pain

• Diplopia (double vision)

• Risk for corneal exposure

• Corneal opacity

• Irregular pupil

• Abnormal red reflex

• New onset strabismus

• Nystagmus

Based on suspected or confirmed systemic disease (Finding)

• CARDIOLOGY

  • ✓ Congenital heart disease

  • ✓ CHARGE syndrome

  • ✓ PHACES syndrome

• CHILD ABUSE

  • ✓ Intracranial hemorrhage in an infant: Retinal hemorrhages with abusive head trauma

• CRANIOFACIAL

  • ✓ Pierre Robin sequence: Stickler syndrome

  • ✓ Hydrocephalus: Papilledema

• DERMATOLOGY

  • ✓ Stevens–Johnson syndrome, toxic epidermal necrolysis

  • ✓ Peri-ocular capillary hemangioma

  • ✓ PHACES syndrome

  • ✓ Incontinentia pigmenti

  • ✓ Juvenile xanthogranuloma

  • ✓ Oculocutaneous albinism

• GASTROENTEROLOGY

  • ✓ Wilson’s disease: Kayser–Fleischer ring, cataracts

  • ✓ Alagille syndrome: Posterior embryotoxon

• GENETICS

  • ✓ Marfan syndrome: Lens subluxation

  • ✓ Neurofibromatosis: Iris Lisch nodules

  • ✓ Tuberous sclerosis

  • ✓ Trisomy 21: Nystagmus, strabismus, cataracts, glaucoma, blocked tear ducts, high refractive error

  • ✓ 22q11.2 deletion syndrome

• HEMATOLOGY

  • ✓ Hermansky–Pudlak syndrome: oculocutaneous albinism

• IMMUNOLOGY

  • ✓ Chediak–Higashi syndrome: oculocutaneous albinism

• INFECTIOUS DISEASE

  • ✓ HIV

  • ✓ Preseptal or orbital cellulitis

  • ✓ Neonatal conjunctivitis

  • ✓ Neonatal herpes simplex virus infection

  • ✓ Periocular herpes simplex virus or varicella virus infection

  • ✓ Cat-scratch disease: Parinaud’s oculoglandular syndrome, neuroretinitis, focal chorioretinitis

  • ✓ Lyme: Optic neuritis, cranial nerve palsies, uveitis

  • ✓ Fungemia

  • ✓ Endocarditis: Retinal hemorrhages and septic emboli

  • ✓ TORCH infections: Toxoplasmosis, Rubella, CMV, Herpes, Syphilis

• METABOLISM

  • ✓ Galactosemia: Cataract

  • ✓ Tyrosinemia: Type 2 with corneal deposits causing redness, tearing, photophobia

  • ✓ Cystinosis

• MITOCHONDRIAL DISEASE

  • ✓ MERFF (myoclonic epilepsy with ragged red fibers)

  • ✓ MELAS (mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes)

  • ✓ Kearns–Sayre

  • ✓ NARP: Neuropathy, ataxia, retinitis pigmentosa

• NEONATOLOGY

  • ✓ Neonatal conjunctivitis

  • ✓ Extreme prematurity (birth weight ≤1501 g, gestational age ≤30 weeks; guidelines vary for middle income countries)—Retinopathy of prematurity exams

• NEPHROLOGY

  • ✓ Tubular interstitial nephritis: Uveitis

• NEUROLOGY

  • ✓ Seizures, especially infantile spasms

    • ▪ Tuberous sclerosis: Retinal hamartomas

    • ▪ Aicardi syndrome (X-linked dominant, primarily affecting girls): Retinal lacunae

    • ▪ Use of vigabatrin for seizure control

  • ✓ Myasthenia gravis: Ptosis, strabismus, ocular motility deficits

  • ✓ Cranial nerve palsies: 3rd, 4th, 6th, and 7th nerve palsies

  • ✓ Multiple sclerosis: Optic neuritis, internuclear ophthalmoplegia

• RHEUMATOLOGY

  • ✓ JIA: Uveitis, slit lamp exam essential, patients generally asymptomatic

  • ✓ Kawasaki disease: Conjunctivitis and uveitis