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2002 Self-Assessment Exercise — XXII. Disorders of the eye [Return to Category List] Questions Question 14. Answer. You are evaluating a 4-week-old boy for tearing of the right eye that has worsened over the past week. Physical examination reveals slight tearing but no evidence of purulent exudate or conjunctival erythema. All other findings are normal. Of the following, the MOST appropriate initial management is: A. administration of amoxicillin B. endoscopic dacrocystorhinostomy C. instillation of silver nitrate into the eyes D. observation with intermittent massage of the duct E. surgical dilation of the nasolacrimal duct Question 63. Answer. A mother brings her 4-month-old infant to the emergency department because the infant has been excessively irritable for 10 hours. She has been feeding normally. The mother reports that the infant seems to be rubbing her right eye. On physical examination, the child is afebrile, interactive, and soothes temporarily in her mother's arms, but she has frequent outbursts of crying. There is tearing in the right eye, with scant conjunctival injection. Of the following, the evaluation that is MOST likely to aid in the diagnosis is: A. conjunctival swab for Chlamydia trachomatis B. conjunctival swab for Gram stain C. fluorescein staining of both eyes D. funduscopic examination
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Disorders of the Eye

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Page 1: Disorders of the Eye

2002 Self-Assessment Exercise —

XXII. Disorders of the eye

[Return to Category List]

Questions

Question 14. Answer.

You are evaluating a 4-week-old boy for tearing of the right eye that has worsened over the past week. Physical examination reveals slight tearing but no evidence of purulent exudate or conjunctival erythema. All other findings are normal.

Of the following, the MOST appropriate initial management is:

A. administration of amoxicillin

B. endoscopic dacrocystorhinostomy

C. instillation of silver nitrate into the eyes

D. observation with intermittent massage of the duct

E. surgical dilation of the nasolacrimal duct

Question 63. Answer.

A mother brings her 4-month-old infant to the emergency department because the infant has been excessively irritable for 10 hours. She has been feeding normally. The mother reports that the infant seems to be rubbing her right eye. On physical examination, the child is afebrile, interactive, and soothes temporarily in her mother's arms, but she has frequent outbursts of crying. There is tearing in the right eye, with scant conjunctival injection.

Of the following, the evaluation that is MOST likely to aid in the diagnosis is:

A. conjunctival swab for Chlamydia trachomatis

B. conjunctival swab for Gram stain

C. fluorescein staining of both eyes

D. funduscopic examination

E. measurement of intraocular pressure

Question 116. Answer.

You are seeing a 2-week-old infant for the first time in your office. On physical examination, you note ptosis of the right upper eyelid. Findings on the remainder of the physical examination are normal.

Of the following, the MOST likely cause for the ptosis in this patient is:

Page 2: Disorders of the Eye

A. botulism

B. brainstem glioma

C. Horner syndrome

D. malformed levator muscle

E. neonatal myasthenia gravis

Question 150. Answer.

You are discussing with a medical student the criteria for screening infants in the neonatal intensive care unit for retinopathy of prematurity.

Of the following, the MOST accurate statement is that:

A. all infants exposed to prolonged supplemental oxygen should be screened independent of gestational age

B. all infants whose gestational age is less than 29 weeks should be screened independent of oxygen exposure

C. optimal frequency for serial examination is at 2-week intervals for zone I or stage 3 disease

D. optimal time for final retinal examination is at 40 weeks’ postmenstrual age

E. optimal time for initial retinal examination is within 2 weeks of birth

Question 204. Answer.

A 12-year-old child has been struck in the right eye by a baseball traveling at a high rate of speed. On physical examination, there is marked bruising and swelling of the periorbital region. The anterior chamber is clear, and pupillary reflexes are intact. Extraocular movement testing reveals dysconjugate gaze when looking to the left.

Of the following, the MOST likely diagnosis is:

A. blow-out fracture of the orbit

B. hyphema

C. lens disruption

D. retinal detachment

E. ruptured globe

Question 247. Answer.

At a 4-month health supervision visit, a mother expresses concern that her infant son does not see well. Birth and postnatal history are unremarkable. On physical examination, you

Page 3: Disorders of the Eye

note horizontal nystagmus with jerking eye movement when the child looks to the left. Occipitofrontal circumference is 43.0 cm (75th percentile). Red reflex is normal, as are findings on brain magnetic resonance imaging.

Of the following, the MOST likely diagnosis is:

A. congenital nystagmus

B. neuroblastoma

C. retinoblastoma

D. seizures

E. spasmus nutans

Answers

Critique 14. Preferred Response: D

[View Question]

Congenital nasolacrimal duct obstruction is the most common abnormality of the infant’s lacrimal system. Almost 5% of infants manifest nasolacrimal duct obstruction, and 30% of affected infants have bilateral obstruction. The obstruction is usually at the distal end of the nasolacrimal system. Most typically, infants present with tearing and crusting of the eyelashes. Massage of the duct will reveal clear-to-mucopurulent material. Conjunctival inflammation, photophobia, blepharospasm, corneal clouding, or other eye abnormalities are notably absent.

Natural resolution rates as high as 95% have been reported by 13 months of age. Complications are rare and include development of nasolacrimal duct cysts (Figure 14A) with nasal obstruction or dacryocystitis.

Because of the favorable natural history, recommended treatment is conservative. Digital massage of the duct two to three times a day may help to open the occluded system.

Topical antibiotics are indicated only for mucopurulent conjunctivitis. Silver nitrate is indicated for the prevention of gonococcal ophthalmia, but may cause chemical conjunctivitis. Systemic antibiotics may be necessary for treatment of dacryocystitis, a bacterial infection of the lacrimal sac manifested by edema, erythema, and tenderness over the sac.

Nasolacrimal duct probing is appropriate if symptoms persist beyond 13 months of age. Probing usually is performed under general anesthesia and generally is curative. Tube placement or dacrocystorhinostomy (surgical opening of the lacrimal system (Figure 14B) into the lateral nasal wall) are indicated for persistent duct obstruction that does not respond to conservative medical and surgical management.

References:

Page 4: Disorders of the Eye

Korn E. Oculoplastic update. Pediatr Ann. 1990;19:316-322

Lavrich JB, Nelson LB. Disorders of the lacrimal system apparatus. Pediatr Clin North Am. 1993;40:767-776

Ogawa GS, Gonnering RS. Congenital nasolacrimal duct obstruction. J Pediatr. 1991;119:12-17

Olitsky SE, Nelson LB. Disorders of the lacrimal system. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 16th ed. Philadelphia, Pa: WB Saunders Co; 2000:1911

Poole SR. Corneal abrasion in infants. Pediatr Emerg Care. 1995; 11:25-26

Trobe JD, American Academy of Family Physicians. Dacryocystitis. In: The Physician’s Guide to Eye Care. San Francisco, Calif: American Academy of Ophthalmology; 1993:45-46

Critique 63. Preferred Response: C

[View Question]

There are many causes of increased irritability in an infant, including sepsis, meningitis, urinary tract infection, intussusception, supraventricular tachycardia, otitis media, hair tourniquet on a digit, drug reactions, or a corneal abrasion (Figure 63A). At 3 to 4 months of age, infants develop the ability to bring their hands to midline, and as they experiment with this new skill, they may scratch their eye, resulting in a corneal abrasion. The child described in this vignette has signs (unilateral tearing and conjunctival injection) and symptoms (irritability and inconsolable crying) suggestive of a corneal abrasion (or foreign body). Following application of fluorescein dye, damaged areas of the cornea, which take up the dye, fluoresce green under blue light. The cobalt blue light on most modern ophthalmoscopes provides acceptable visualization.

The lack of discharge and only scant injection of the right eye described for the infant in the vignette argue against chlamydial or bacterial conjunctivitis, obviating the need for examination of a conjunctival swab. Funduscopic examination of the eye could be helpful, particularly to search for the retinal hemorrhages (Figure 63B) expected in the shaken baby syndrome, but this diagnosis is not associated with excessive tearing or conjunctival injection. Although glaucoma is rare in infants, it can cause excessive tearing and irritability due to eye pain. Measurement of intraocular pressure in this age group usually requires consultation with an ophthalmologist.

The differential diagnosis for “red eye” in this age group primarily includes conjunctivitis, trauma, abnormalities of the lids or lashes, and systemic diseases. Conjunctivitis can be caused by chemical irritation, allergy, or infection. Posttraumatic iritis can cause scleral erythema, as can corneal abrasion or foreign bodies. Blepharitis (Figure 63C), stye (Figure 63D), chalazion (Figure 63E), or molluscum of the lid margin all can cause conjunctival injection.

Page 5: Disorders of the Eye

In most cases of red eye, the diagnosis becomes apparent after a thorough history and physical examination. If a diagnosis is not apparent and the child has a painful red eye, urgent referral to an ophthalmologist is indicated.

References:

Levin AV. Eye—red. In: Fleisher GR, Ludwig S, eds. Textbook of Pediatric Emergency Medicine. 4th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2000:231-235

Pawel BB, Henretig FM. Crying and colic in early infancy. In: Fleisher GR, Ludwig S, eds. Textbook of Pediatric Emergency Medicine. 4th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2000:193-195

Tingley DH. Consultation with the specialist. Eye trauma: corneal abrasions. Pediatr Rev. 1999;20:320-322

Critique 116. Preferred Response: D

[View Question]

Congenital ptosis (Figure 116A) is usually due to malformation of the levator muscle, which is the muscle that primarily elevates the eyelid. The levator muscle is innervated by the third cranial nerve. If the ptosis is severe, the infant’s vision will be greatly diminished. The infant often keeps the chin up in order to see. Absence of the faint lid crease will be present on the affected side. One or both eyebrows are often markedly elevated.

The treatment for this condition is surgical correction. If untreated, amblyopia and loss of vision occur in many cases. Ophthamologic referral as soon as possible after birth is indicated.

Myasthenia gravis is due to gradual destruction of the motor endplates at the neuromuscular junction. Circulating antibodies to the acetylcholine receptor bind to the receptor on the motor endplate, blocking its function. Ptosis is usually bilateral, and older patients also may complain of diplopia. Infants born to mothers who have myasthenia gravis may manifest neonatal myasthenia gravis, characterized by respiratory insufficiency, inability to suck or swallow, and generalized weakness and hypotonia. After the transplacentally acquired antibodies disappear, the infants have normal strength and are not at increased risk for myasthenia gravis. Congenital myasthenia gravis is a rare, often hereditary disorder that differs from transient neonatal myasthenia gravis. Congenital myasthenia gravis is not due to maternal antibodies and is almost always a permanent disorder without spontaneous remission.

Botulism caused by preformed botulinum toxin is a type of food poisoning. It presents as a rapid onset of cranial nerve weakness; a descending paralysis of limb and respiratory muscles develops later. Botulism evolves rapidly over hours to days and has a progressive course. Infants who have botulism usually present with constipation, poor feeding, lethargy, and weak cry.

Page 6: Disorders of the Eye

Brainstem gliomas are uncommon in infants. They should be considered in any child who has cranial nerve abnormalities. Characteristic long tract findings include spasticity of limbs, exaggerated reflexes, positive Babinski sign, and ataxia.

Mild ptosis with a lid crease, a small pupil (miosis), a difference in iris color (heterochromia) (Figure 116B), and an inability to sweat (anhidrosis) on one side of the face are signs of Horner syndrome caused by sympathetic nerve interruption. In congenital cases, a history of brachial plexus injury resulting from a difficult delivery may help determine the etiology.

References:

Olitsky SE, Nelson LB. Abnormalities of the lids. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 16h ed. Philadelphia, Pa: WB Saunders Co; 2000:1909-1910

Palay DA, Krachmer JH. Ophthalmology for the Primary Care Physician. St Louis, Mo: Mosby-Year Book, Inc; 1997:184-185

Critique 150. Preferred Response: B

[View Question]

All infants whose gestational age is less than 29 weeks should be screened for retinopathy of prematurity (ROP) independent of oxygen exposure. Prematurity is the principal predisposing factor for the occurrence of ROP; the risk of ROP varies inversely with gestational age. In infants whose gestational age is less than 29 weeks, the incidence of any ROP is 66%, moderate ROP is 18%, and severe ROP is 6%.

In the preterm infant, supplemental oxygen therapy is a major risk factor for the occurrence of ROP. However, ROP is uncommon in infants whose gestational age is 32 weeks or older or whose birthweight is more than 1,500 g, even when the infants are exposed to supplemental oxygen.

ROP is classified according to location and extent of disease, severity of abnormal vascularization, and the presence or absence of dilatation of the posterior retinal vessels (referred to as plus disease). The location of the disease is described by zones I to III and the extent by comparing the retina with a clock face marked off by hours 1 to 12. The severity of abnormal vascularization is described by stages 1 to 3. Prethreshold ROP is defined as zone I, any stage; zone II, stage 2 with plus disease; or zone II, stage 3. Threshold ROP is defined as stage 3 disease extending to five contiguous or eight cumulative hours in zone I or II with plus disease. Prethreshold ROP carries a poor prognosis for visual function and warrants weekly monitoring by eye examination for progression. Threshold ROP is an indication for cryotherapy or laser photocoagulation.

The optimal time for initial retinal examination is between 4 and 6 weeks’ chronologic age or 31 and 33 weeks’ postmenstrual age, whichever occurs earlier. Retinal vascularization typically starts near the optic nerve at approximately 20 weeks’ gestational

Page 7: Disorders of the Eye

age. Progression of the retinal vessels toward the periphery of the retina occurs steadily thereafter. Abnormalities of retinal vessels become evident at approximately 4 weeks’ chronologic age or 31 weeks’ postmenstrual age. Earlier eye examinations, therefore, will have a minimal yield.

The optimal time for final retinal examination in ROP is at 44 weeks’ postmenstrual age. Normally, the retina is fully vascularized at that age. Any progression or new occurrence of ROP is extremely unlikely thereafter.

References:

Fierson WM, Palmer EA, Biglan AW, Flynn JT, Petersen RA, Phelps DL. Screening examination of premature infants for retinopathy of prematurity. A joint statement of the American Academy of Pediatrics, the American Association for Pediatric Ophthalmology and Strabismus, and the American Academy of Ophthalmology. Pediatrics. 1997;100:273-274

Palmer EA, Flynn JT, Hardy RJ, et al. Incidence and early course of retinopathy of prematurity. The Cryotherapy for Retinopathy of Prematurity Cooperative Group. Ophthalmology. 1991;98:168-1640

The Committee for the Classification of Retinopathy of Prematurity. The international classification of retinopathy of prematurity. Arch Ophthalmol. 1984;102:1130-1134

Wright K, Anderson ME, Walker E, Lorch V. Should fewer premature infants be screened for retinopathy of prematurity in the managed care era? Pediatrics. 1998;102:31-34

Critique 204. Preferred Response: A

[View Question]

The “orbit” refers to the bony structure surrounding the eye. When a blunt object larger than the orbit makes forceful contact with the orbital rim, rapid compression of the tissues within the bony, confined space causes a large pressure increase within the orbit. This increased pressure can generate sufficient force to fracture the wall of the orbit, causing herniation of orbital contents through the blow-out fracture. The floor and medial walls are the weakest portions of the orbit and are most commonly involved.

The boy described in this vignette has entrapment of the medial rectus muscle, producing left dysconjugate gaze. Other physical findings may include proptosis from hemorrhage within the orbit or a “sunken eye” appearance from herniation of orbital contents through the fracture. Tenderness along the orbital rim or a fracture step-off also may be present. Hypesthesia of the ipsilateral cheek may occur due to damage to the infraorbital nerve, which travels along the floor of the orbit. Approximately 20% of orbital fractures are associated with injury to the globe, requiring dilated ophthalmoscopy and computed tomography of the orbit for evaluation.

Hyphema (Figure 204A) is a collection of blood in the anterior chamber of the eye that is visualized easily with a hand-held ophthalmoscope or even with the naked eye. Lens disruption and retinal detachment (Figure 204B) are possible consequences of severe

Page 8: Disorders of the Eye

trauma to the eye, but visual changes are not dependent on eye position, and neither condition in isolation would cause dysconjugate gaze. A patient who has a ruptured globe has marked visual impairment and a soft, flattened eye and anterior chamber due to decreased intraocular pressure.

References:

American College of Surgeons Committee on Trauma. Ocular trauma. In: Advanced Trauma Life Support for Doctors: Student Course Manual. 6th ed. Chicago, Ill: American College of Surgeons; 1997:411

Klein BR, Sears ML. Eye injury. Pediatr Rev. 1992;13:127-129

Levin AV. Eye trauma. In: Fleisher GR, Ludwig S, eds. Textbook of Pediatric Emergency Medicine. 4th ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 2000:1397-1407

Critique 247. Preferred Response: A

[View Question]

Nystagmus is a rapid, rhythmic oscillation of the eyes that usually is bilateral. It can be a worrisome sign of serious eye or central nervous system pathology and may warrant referral to a neurologist. Congenital nystagmus is characterized by pendular nystagmus in primary gaze that becomes jerkier with lateral gaze, as described for the infant in the vignette. Frequently, there is a null point at which nystagmus is least and vision is best. Visual acuity often is diminished. Typically the child’s distance vision is worse than near vision because nystagmus is dampened with convergence. Congenital nystagmus may be associated with ocular albinism and head titubation (bobbing). Onset of the disorder is typically between 2 and 6 months of age. Many of these children will have lifelong visual impairment.

Spasmus nutans is a triad of nystagmus, head nodding, and head tilt. The nystagmus may be quick and pendular (shimmering) and asymmetric. Onset is typically at 4 to 12 months, and it resolves by 4 years. Rarely, patients have an associated optic pathway pilocytic astrocytoma.

Nystagmus can be seen during a seizure, but it is absent interictally. Downbeating vertical nystagmus is pathognomonic of disease at the cervicomedullary junction, particularly a Chiari malformation. Upbeat nystagmus can be seen with lesions in the low brainstem or cerebellum.

Opsoclonus is distinct from nystagmus, lacking the latter’s rhythmicity and regularity. Rather, it is characterized by irregular, chaotic, conjugate rapid eye movements in all planes. It can be associated with encephalitis, ingestion of toxins, or hydrocephalus, but the finding should trigger a search for occult neuroblastoma. Retinoblastoma is characterized by leukocoria from the tumor producing loss of the red reflex.

References:

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Calhoun JH. Consultation with the specialist. Eye examinations in infants and children. Pediatr Rev. 1997;18:28-31

Olitsky SE, Nelson LB. Disorders of eye movement and alignment. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 16th ed. Philadelphia, Pa: WB Saunders Co; 2000:1904-1908

2001 Self-Assessment Exercise —

XXII. Disorders of the eye

[Return to Category List]

Questions [Print Directions]

Question 46. Answer.

A mother contacts you while she is on vacation. Her 8-year-old son, who has allergies, awoke with bilateral conjunctival injection, mucus-filled eye discharge, and rhinorrhea. He complains of ocular itching, but his eyelids were not stuck together.

Your BEST advice is to recommend

A. an antibiotic eye drop

B. an evaluation for environmental triggers

C. an over-the-counter allergy preparation

D. no therapy

E. ocular antihistamines

Question 85. Answer.

A 2-year-old girl presents with a 2-day history of cough, congestion, and bilateral red eyes with an associated watery discharge. She denies any photophobia or pain, and there is no edema of the eyelid.

The MOST likely diagnosis is

A. blepharitis

B. conjunctivitis

C. corneal abrasion

D. sclerouveitis

E. uveitis

Question 134. Answer.

Page 10: Disorders of the Eye

A 4-year-old girl presents with a fever of 39°C (102.2°F), left periorbital edema, and mild proptosis. Findings on physical examination include restricted lateral and upward gaze. Visual acuity is normal.

Of the following, the MOST likely diagnosis is

A. conjunctivitis

B. orbital cellulitis

C. orbital pseudotumor

D. orbital rhabdomyosarcoma

E. preseptal cellulitis

Question 178. Answer.

A mother is concerned because her 1-month-old son has increased tearing in both of his eyes. He also seems to be bothered by bright light. Findings on physical examination include bilateral bulbar conjunctival injection and large corneas. You have difficulty observing a red reflex bilaterally.

Of the following, the MOST useful investigation to diagnose this infant's condition is

A. chromosomal analysis

B. computed tomography of the head

C. measurement of intraocular pressure

D. rubella titer

E. slitlamp examination of the eyes

Question 214. Answer.

In evaluating a term newborn, you note bilateral leukocoria. There is no evidence of hepatosplenomegaly, microcephaly, intrauterine growth retardation, or heart disease. Audiologic evaluation reveals severe hearing impairment.

Of the following, the congenital infection that is MOST likely responsible for the findings in this infant is

A. cytomegalovirus

B. rubella

C. syphilis

D. toxoplasmosis

E. varicella

Page 11: Disorders of the Eye

Answers

Critique 46. Preferred Response: C

[View Question]

Classic signs of an allergic reaction include conjunctival injection; pruritus; clear, mucus-filled ocular discharge; and rhinorrhea. A common trigger for such an event is the presence of an allergen in the room in which the child is staying. This could be a mold or dust mites, especially in feather bedding.

An ideal choice to treat the allergic reaction is an oral antihistamine, but the family may not be able to obtain that easily on vacation. Accordingly, an over-the-counter allergy preparation would be appropriate, although the mother should be forewarned that it may have some sedative side effects.

The rhinorrhea and clear bilateral ocular discharge experienced by the child suggest that this is not an infectious process, which obviates the need for an antibiotic eyedrop. Another finding arguing against a possible infection is that the child can open his eyes. Patients who have bacterial conjunctivitis commonly report that their eyelids are stuck together.

Ocular antihistamines would not address the nasal symptoms reported for this boy. An evaluation for the environmental trigger may be a reasonable course of action, but if the trigger is outdoor pollen, the child will continue to have significant symptoms without therapy. There is no reason to withhold therapy from a child who suffers with allergic symptoms.

References:

Bielory L. Allergic and immunologic disorders of the eye. In: Middleton E Jr, Ellis EF, Yunginger JW, et al, eds. Allergy Principles & Practice. 5th ed. St Louis, Mo: Mosby-Year Book, Inc; 1998:1148-1161

Gigliotti F. Acute conjunctivitis. Pediatr Rev. 1995;16:203-208

Critique 85. Preferred Response: B

[View Question]

Patients who have viral conjunctivitis (Figure 85A) generally present with red, profusely watery eyes and a preceding history of an upper respiratory tract infection, as described for the girl in the vignette. The conjunctivitis often is contracted at child care facilities or from family members and is a self-limited condition that improves gradually over 1 to 2 weeks.

A corneal abrasion usually presents as a unilateral red eye that frequently is painful. Patients may complain of a foreign body sensation. Fluorescein dye can be used to identify the corneal defect and aid in the diagnosis. Blepharitis is a disease affecting the eyelid margins that is characterized by erythema and crusting of the eyelids, hyperemia, and

Page 12: Disorders of the Eye

itching. Uveitis is a nonspecific term used to describe any inflammatory condition involving the uveal tract, which is composed of the iris, ciliary body, and choroid. Patients who have uveitis often complain of pain and photophobia. Their vision may be normal or decreased, and the eye is red. Various systemic disorders are associated with uveitis, such as juvenile rheumatoid arthritis, Kawasaki disease, Behcet disease, Reiter syndrome, and inflammatory bowel disease. Sclerouveitis is a uveitis due to inflammation of the sclera.

References:

Olitsky SE, Nelson LB. Disorders of the conjunctiva. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 16th ed. Philadelphia, Pa: WB Saunders Co; 2000:1911-1914

Palay DA. Conjunctival abnormalities. In: Palay DA, Krachmer JH, eds. Ophthalmology for the Primary Care Physician. St Louis, Mo: Mosby-Year Book, Inc; 1997:57-67

Critique 134. Preferred Response: B

[View Question]

Orbital cellulitis involves inflammation of the tissues of the orbit, which produces edema of the conjunctiva, swelling of the eyelids, proptosis, and restricted movement of the eye, signs and symptoms described for the child in the vignette. In children, the common bacterial pathogens causing orbital cellulitis include Streptococcus pneumoniae, Haemophilus influenzae, Staphylococcus aureus, and group A beta-hemolytic streptococci. The infection frequently begins in the paranasal sinuses, spreads to the orbit, and can progress rapidly to produce subperiosteal abscess, cavernous sinus thrombosis, meningitis, or brain abscess. It is imperative that orbital cellulitis is recognized and treated with intravenous antibiotics promptly. Surgical intervention is required to drain a large abscess or a small abscess that does not resolve in 2 to 3 days with an intravenous antibiotic regimen or is associated with decreased visual acuity.

Conjunctivitis is an infection of the conjunctiva characterized by tearing, erythema, and watery discharge. High fever, proptosis, and restricted eye movement are not seen with this condition. Common findings in patients who have preseptal cellulitis include fever, redness, swelling, and tenderness of the affected eyelid. The absence of proptosis and restricted eye movements helps to distinguish preseptal cellulitis from orbital cellulitis. Although both orbital pseudotumor and orbital rhabdomyosarcoma can present with proptosis, neither is associated with high fever.

References:

Olitsky SE, Nelson LB. Orbital abnormalities. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 16th ed. Philadelphia, Pa: WB Saunders Co; 2000:1934-1935

Powell KR. Orbital and periorbital cellulitis. Pediatr Rev. 1995;16:163-167

Wojno TH. Orbital disease. In: Palay DA, Krachmer JH, eds. Ophthalmology for the Primary Care Physician. St Louis, Mo: Mosby-Year Book, Inc; 1997:208-224

Page 13: Disorders of the Eye

Critique 178. Preferred Response: C

[View Question]

Congenital or infantile glaucoma is a rare disorder (1 in 10,000 births) that can have devastating consequences unless recognized and treated promptly. Approximately 80% of cases will present within the first 6 months of life. Clinical signs and symptoms of the elevated intraocular pressure include photophobia, increased tearing, blepharospasm, and corneal enlargement, as described for the infant in the vignette. Because of the distensibility of the young infant’s eye, enlargement of the globe (buphthalmos [Figure 178A]) eventually can occur if the condition remains untreated. Corneal clouding can cause leukocoria, suggesting a congenital or acquired cataract. Pediatricians who suspect infantile glaucoma should refer the patient immediately to an ophthalmologist. Measurement of intraocular pressure confirms the diagnosis.

Once glaucoma is confirmed, a cause should be sought. Secondary glaucoma can occur with phacomatoses such as Sturge-Weber syndrome (Figure 178B) or neurofibromatosis or with tumors such as retinoblastoma. If these conditions are suspected, computed tomography of the head is useful to confirm the etiology of the glaucoma. Chromosomal disorders such as trisomy 21 sometimes are associated with glaucoma and can be confirmed by a chromosomal analysis. Congenital rubella syndrome commonly causes cataracts and microphthalmia, but it also can be associated with glaucoma. Inflammatory ocular conditions such as the iridocyclitis of juvenile rheumatoid arthritis, which can be detected by slitlamp examination of the eye, also can cause glaucoma. Finally, prolonged use of topical or systemic steroids can cause glaucoma and cataracts, and children who have conditions necessitating the use of these medications should have regular eye examinations to measure intraocular pressure.

References:

Apt L, Miller KM. Glaucoma. In: Rudolph AM, ed. Rudolph's Pediatrics. 20th ed. Stamford, Conn: Appleton & Lange; 1996:2109-2110

Traboulsi EI, Maumenee IH. Eye problems. In: McMillan JA, DeAngelis CD, Feigin RD, Warshaw JB, eds. Oski's Pediatrics: Principles and Practice. 3rd ed. Philadelphia, Pa: Lippincott Williams & Wilkins; 1999:660-681

Critique 214. Preferred Response: B

[View Question]

Leukocoria (white pupil) in the neonate can be associated with a variety of conditions affecting the retina, vitreous, cornea, and lens of the eye. Cataract is one of the major causes. From 30% to 50% of cataracts have no discernible cause; approximately 30% are hereditary; up to 10% are related to maternal intrauterine infections; and the remainder are associated with a variety of metabolic, chromosomal, or other systemic conditions.

Page 14: Disorders of the Eye

Although many intrauterine infections are associated with ocular abnormalities, congenital rubella syndrome (Figure 214A) is most likely to be associated with congenital cataracts. Congenital varicella-zoster virus infection rarely produce cataracts. Maternal rubella infection causes congenital defects in up to 80% of infants when mothers are infected in the first trimester, but virtually no defects are seen when infection occurs after 16 weeks of gestation. Deafness, heart disease (most commonly patent ductus arteriosus, pulmonary artery stenosis, pulmonary valvular stenosis), intrauterine growth retardation, thrombocytopenic purpura, and hepatosplenomegaly also are associated with congenital rubella syndrome.

Intrauterine cytomegalovirus infection and toxoplasmosis can cause hearing impairment, but they are associated with chorioretinitis, not cataracts. Congenital syphilis rarely produces ocular or hearing impairments in the newborn.

References:

Magramm I. Amblyopia: etiology, detection, and treatment. Pediatr Rev. 1992;13:7-14

Maldonado YA. Rubella virus. In: Long SS, Pickering LK, Prober CG, eds. Principles and Practice of Pediatric Infectious Diseases. New York, NY: Churchill Livingstone; 1997:1228-1237

2000 Self-Assessment Exercise —

XXII. Disorders of the eye

[Return to Category List]

Questions [Print Directions]

Question 91. Answer.

During a health supervision visit, the mother of a 2-year-old child who has Down syndrome expresses concern that her daughter's eyes are turning inward. A corneal light reflex test demonstrates symmetric pupillary light reflexes. A cover test reveals no refixation for either eye.

Of the following, the MOST likely explanation for these findings is

A. accommodative esotropia

B. alternating esotropia

C. amblyopia

D. hyperopia

E. pseudostrabismus

Question 183. Answer.

Page 15: Disorders of the Eye

A 6-year-old boy is hit in the left eye by a rock. He immediately complains of pain and an inability to see out of the injured eye. Upon arrival at the emergency department, examination of the injured eye reveals swollen eyelid, tearing, and a small laceration at the outer edge of the upper eyelid.

Of the following, the most appropriate INITIAL management of this child's injury is

A. assessment of visual acuity

B. examination of the eyeball using a lid retractor

C. instillation of antibiotic ointment

D. instillation of fluorescein drops

E. placement of a plastic shield over the injured eye

Answers

Critique 91 Preferred Response: E

[View Question]

Because prominent epicanthal folds are frequent in children who have Down syndrome, it is not uncommon for parents to be concerned about the possibility of an inward turning eye. The presence of symmetric corneal reflexes in the child described in the vignette indicates that the eyes are aligned correctly, and the absence of refixation during the cover test confirms that finding. Thus, the most likely explanation for these findings is pseudostrabismus, a benign finding that requires no intervention.

Alternating esotropia can be detected using the cover-uncover test. As the child fixates on an object with both eyes, one eye is covered. In patients who have alternating esotropia, the affected eye moves outward to fixate on the object after the unaffected eye is covered; both eyes are affected at various times. Accommodative esotropia occurs when the refractive error in one eye is so great that the focus can be improved only through accommodation. Over time, the inward deviation becomes fixed, and amblyopia, or loss of visual acuity, occurs because the brain will suppress the images from the affected eye, which do not coincide with what is seen by the normal eye. Hyperopia refers to farsightedness and can lead to amblyopia if the refractive error is great. Amblyopia can be detected by performing tests of visual acuity, the corneal light reflex test, and the cover-uncover test. An eye that has developed amblyopia generally cannot fixate well on objects and should swing outward to focus on the object during the cover-uncover test.

Early detection of strabismus allows correction through surgical or nonsurgical means. If performed early enough, patching the stronger eye in a patient who has strabismus, correcting a refractive error by glasses, or occasionally, performing extraocular muscle surgery can preserve vision and prevent amblyopia in patients who have strabismus.

References:

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Apt L, Miller KM. Extraocular muscles and ocular motility. In: Rudolph AM, ed. Rudolph's Pediatrics. 20th ed. Stamford, Conn: Appleton & Lange; 1996: 2111-2113

Magramm I. Amblyopia: etiology, detection and treatment. Pediatr Rev. 1992;13:7-14

Nelson LB. Disorders of eye movement and alignment. In: Behrman RE, Kliegman RM, Nelson WE, eds. Nelson Textbook of Pediatrics. 15th ed. Philadelphia, Pa: WB Saunders Co; 1996:1772-1776

Critique 183 Preferred Response: A

[View Question]

Approximately 2.4 million eye injuries occur in the United States each year, and tens of thousands of these result in some loss of vision. To achieve the best outcome, the examining clinician needs to obtain a detailed, accurate history, perform a systematic examination to determine the extent and severity of the injuries, and institute appropriate initial management.

The history should include any change in visual acuity, what the visual acuity was prior to the injury, and any past ocular history. The nature of the injury should be determined precisely because certain types of trauma are associated with intraocular damage, including significant blunt impact directly to the eyeball (eg, a fist or a ball), injuries caused by projectiles or sharp implements, and injuries that occur while hammering (metal splinter). History of other symptoms, such as photophobia or visual field defects, also should be sought.

Assessment of visual acuity using age-appropriate means always is the first step in the examination of a child who has sustained an eye injury. External examination should include careful evaluation of the periorbital tissues, eyelids, and the bony orbital rim. Infraorbital hypoesthesia may be indicative of an orbital fracture. Small, penetrating eyelid lacerations suggest an intraorbital injury. A laceration along the nasal portion of the lid raises suspicion for an injury to the lacrimal drainage system and should be referred to an ophthalmologist for definitive repair.

The conjunctiva should be inspected for lacerations, which may be the only indication of globe penetration. Eversion of the eyelid to inspect the tarsal conjunctivae is imperative because many foreign bodies become lodged here. The pupils should be assessed for equality in size and reaction, and extraocular muscle movement also should be evaluated. Lack of an upward gaze often is associated with an orbital fracture. The anterior chamber should be examined and evaluated for abrasions, lacerations, corneal foreign bodies, hyphema, or asymmetry of the iris or lens.

After visual acuity is assessed, the external examination should be performed. The eyeball should be examined using a lid retractor only if the patient is unable to open the eye, all attempts at using the examiner’s fingers have failed, and there is no suspicion of a ruptured globe. A hyphema (blood in the anterior chamber) or ruptured globe should be excluded before ointment or drops, including fluorescein, are instilled into the eye. A plastic

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shield that rests on the bony prominences of the orbit should be placed over the injured eye in all cases of suspected ruptured globe or ocular foreign body to prevent further injury. No attempts should be made to remove intraocular foreign bodies. All cases of hyphema, significant chemical injury, and penetrating globe trauma should be referred for immediate ophthalmologic evaluation.

References:

Hoffman RO. Evaluating and treating eye injuries. Contemporary Pediatrics. 1997;14:74-98

Levin AV. Eye trauma. In: Fleisher GR, Ludwig S, eds. Textbook of Pediatric Emergency Medicine. 3rd ed. Baltimore, Md: Williams & Wilkins; 1993:1200-1209

1999 Self-Assessment Exercise —

XXII. Disorders of the eye

[Return to Category List]

Questions [Print Directions]

Question 33. Answer.

A 15-year-old girl complains that her left eye has been painful and teary for the past 4 hours. Physical examination is notable for profuse tearing and redness of the left eye with normal pupil size. Fluorescein staining reveals vertical linear corneal abrasions.

Of the following, the most appropriate INITIAL management of the affected eye is to

A. apply bacitracin ophthalmic ointment

B. apply neomycin ophthalmic drops

C. apply steroid ointment

D. close the eye and cover with a pressure patch

E. evert the eyelid, looking for a foreign body

Question 98. Answer.

You are discussing the risks of retinopathy of prematurity (ROP) with the parents of an infant who was born at 30 weeks’ gestation. The child had moderately severe respiratory distress syndrome that required surfactant therapy, mechanical ventilation for 5 days, and supplemental oxygen for 2 weeks.

Of the following, the statement that you are MOST likely to include in your discussion of ROP is

A. an ophthalmologic evaluation for ROP should be performed when the child is 4 to 6 weeks old

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B. birthweight is not a predictor of ROP

C. boys are more likely than girls to develop ROP

D. children who have ROP that regresses are unlikely to have visual impairment

E. the incidence of ROP is higher in African-American infants

Question 159. Answer.

On occasion, the mother of a 14-month-old girl has noted a whitish area in her daughter’s pupil. It is difficult to obtain an adequate examination of the girl’s eyes because of a lack of cooperation, and you have arranged for evaluation by an ophthalmologist.

Of the following, the MOST likely explanation for this finding is

A. cataract

B. persistent hyperplastic primary vitreous

C. retinoblastoma

D. retinopathy of prematurity

E. uveitis

Question 232. Answer.

A 5-month-old infant has been nursing poorly for the past week. White patches are present on the buccal mucosa. Last month he was treated with amoxicillin for otitis media and developed thrush, which was treated with nystatin.

In addition to initiating oral antifungal therapy for the infant, the BEST method to prevent a recurrence of thrush is to

A. administer oral nystatin prophylaxis daily to the infant

B. administer oral nystatin prophylaxis daily to the mother

C. apply topical nystatin cream to the mother’s nipples

D. rinse the infant’s mouth with water following breastfeeding

E. switch to formula feeding

Answers

Critique 33 Preferred Response: E

[View Question]

Symptoms of a corneal abrasion include exquisite eye pain, a sensation of a foreign body, and tearing. Diagnosis is made by fluorescein staining. When the surface epithelium of the

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cornea is scratched or abraded, the areas of denuded cornea will fluoresce yellow to green due to stain uptake, which is seen best with a Wood lamp. Intact corneal epithelium does not take up the stain.

The possibility of a foreign body should be considered in all cases of corneal abrasions, particularly when the abrasions are vertical or linear, as in the girl in the vignette. The upper lid should be everted upon itself and carefully inspected. If a foreign body is found, it may be removed gently with a cotton–tipped swab or by irrigation. Instillation of a topical anesthetic drop, such as 0.5% proparacaine, will relieve the pain of a corneal abrasion and make examination much easier, but the child should not be sent home with a topical anesthetic in the eye because of the risk of additional injury and delayed healing.

Following complete evaluation of the eye, treatment of a corneal abrasion includes instillation of an ophthalmic antibiotic, usually a lubricating ointment, such as bacitracin. Preparations containing neomycin should not be used because hypersensitivity reactions are common. Topical ophthalmic steroids will delay healing and should be used only in consultation with an ophthalmologist. In patients who are experiencing significant pain, a cycloplegic can be instilled to relieve ciliary muscle spasm. If the corneal abrasion is due to a contact lens or if the patient has a history of ocular herpes, prompt referral to an ophthalmologist is necessary. Although pressure patching long has been recommended, recent studies have demonstrated no benefit in pain control or healing. A patch should not be applied before everting the eyelid to exclude a foreign body.

References:

Eisenbaum AM. Eye. In: Hay WW Jr, Groothuis JR, Hayward AR, Levin MJ, eds. Current Pediatric Diagnosis & Treatment. 13th ed. Stamford, Conn: Appleton & Lange; 1997:375-392

Kaiser P. A comparison of pressure patching versus no patching for corneal abrasions due to trauma or foreign body removal. Corneal Abrasion Patching Study Group. Ophthalmology. 1995;102:1936-1942

Klein BR, Sears ML. Eye injury. Pediatrics in Review. 1992;13:127-129

Klein BR, Sears ML. Pediatric ocular injuries. Pediatrics in Review. 1992;13:422-428

Levin AV. Eye trauma. In: Fleisher GR, Ludwig S, eds. Textbook of Pediatric Emergency Medicine. 3rd ed. Baltimore, Md: Williams & Wilkins; 1993:1200-1209

Critique 98 Preferred Response: A

[View Question]

Retinopathy of prematurity (ROP) is a disorder of developing retinal blood vessels in preterm infants. The retinal blood vessels grow outward from the optic disc to the ora serrata during normal development. This centrifugal growth of the retinal blood vessels begins at 15 to 18 weeks of gestational age and is complete by about 44 weeks. Injury to the immature retinal blood vessels from factors such as hyperoxia, shock, asphyxia, hypothermia, and exposure to light can arrest the normal development of the retinal vasculature and result in ROP.

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Typically, ROP is visible in the retina only rarely before 4 to 6 weeks after birth. The rate of progression of the disease varies according to the gestational age. The peak incidence occurs at 34 to 42 weeks of gestational age. Therefore, a preterm infant whose birthweight was less than <1,500 g, who was born before 33 weeks of gestational age, and who is at risk for ROP, such as the infant described in the vignette, should be examined initially at 4 to 6 weeks after birth. The need for further examinations will depend on the degree of vascularization of the retina and the presence, severity, and rate of progression of retinopathy.

Birthweight and gestational age are strong predictors of ROP. The infants who are most premature at birth are most likely to develop ROP. The incidence of ROP is 47% in infants whose birthweights are 1,000 to 1,250 g, 78% in infants whose birthweights are 750 to 999 g, and 90% in infants whose birthweights are less than 750 g. Additional risk factors for ROP include Caucasian race, multiple births, need for transport after birth, and a complicated perinatal or neonatal course. The incidence of ROP is similar among boys and girls.

The rate of progression of ROP is highly variable. The worst prognosis is associated with early onset, rapid progression, and zone I disease (in close proximity to the optic nerve and the macula). The best prognosis is associated with delayed onset, slow progression, and zone III disease (in the periphery of the retina). Zone II disease in the part of the retina between zones I and III is the most common, and most infants who have Zone II disease experience resolution that varies from complete recovery to a residual retinal scar.

The visual outcome in infants who have ROP is apparent by 3 months after birth and varies according to the stage of the disease. Infants who have mild ROP and even those who heal without a residual retinal scar have a higher-than-expected incidence of myopia, strabismus, amblyopia, and other sequelae. Infants who have moderate ROP may show resolution of the disease but remain at high risk for developing slowly progressive retinal degeneration. Infants who have severe ROP have varying degrees of retinal detachment and are at high risk for partial or complete blindness.

References:

Palmer EA, Flynn JT, Hardy RJ, et al. Incidence and early course of retinopathy of prematurity. Ophthalmology. 1991;98:1628-1640

Phelps DL. Retinopathy of prematurity. Pediatr Clin North Am. 1993;40:705-714

Critique 159 Preferred Response: C

[View Question]

The most common presenting sign of retinoblastoma is a white pupillary reflex (leukokoria). This loss of the normal red reflex is noted most often by the parents and leads to the diagnosis in 60% of patients. Leukokoria becomes manifest when the tumor is large or when it has caused a total retinal detachment, resulting in a retrolental mass that is visible through the pupil. Strabismus is the second most common sign of retinoblastoma at the time of presentation, occurring in 20% of patients. Visual loss usually is not reported because the

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unilateral loss of vision in a young child rarely is detected. The average age of presentation is 13 to 18 months, and more than 90% of cases are diagnosed before 5 years of age.

The diagnosis of retinoblastoma is made by visual examination using indirect ophthalmoscopy, best performed by an ophthalmologist who has experience in examining children. This usually requires examination under general anesthesia. Other diagnoses that must be considered when the presentation includes a retinal detachment are retinopathy of prematurity and persistent hyperplastic primary vitreous. A cataract or uveitis can be distinguished easily from retinoblastoma on ophthalmoscopic examination. The age of the patient, unilateral presentation, and absence of prematurity allow the experienced examiner to distinguish among these disorders.

Retinoblastoma is an important example of a malignancy that occurs more frequently within certain families who have a genetic predisposition to this type of cancer. Most of these tumors appear sporadically, but an inherited form is transmitted as a mendelian autosomal dominant trait with nearly complete penetrance. This mutation is located on chromosome 13 and involves the RB1 locus. When classified by inheritance and location, 60% of retinoblastomas are nonhereditary and unilateral, 15% are hereditary and unilateral, and 25% are hereditary and bilateral. Accordingly, all patients who have bilateral retinoblastoma and approximately 15% of those who have unilateral disease will have a hereditary form of this malignancy. When a patient has an inherited form of the disease, 50% of his or her offspring also will be affected by the disease. Therefore, all families of a child who has retinoblastoma should be referred for appropriate genetic counseling and testing (ie, identification of an altered RB1 locus) to ensure that the parents understand the risk of having an affected child and to permit earlier detection of tumors in any affected children.

References:

Donaldson SS, Egbert PR, Newshaw I, Cavenee WK. Retinoblastoma. In: Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 3rd ed. Philadelphia, Pa: Lippincott-Raven; 1997:699-716

Mahoney DH Jr. Retinoblastoma. In: Oski FA, DeAngelis CD, Feigin RD, McMillan JA, Warshaw JB, eds. Principles and Practice of Pediatrics. 2nd ed. Philadelphia, Pa: JB Lippincott Co; 1994:1734-1736

Quesnel S, Malkin D. Genetic predisposition to cancer and familial cancer syndromes. Pediatr Clin North Am. 1997;44:791-808

Critique 232 Preferred Response: C

[View Question]

Common risk factors for thrush in an otherwise healthy infant younger than 6 months of age include prior antibiotic use, colonization of mother's nipples, and contamination of other items used in the care of infants, such as rubber nipples or medicine/vitamin droppers. Recurrences of thrush in such infants most often result from reinfection by contaminated

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nipples and bottles or, as is likely in the infant described in the vignette, a superficial infection of the mother's nipples. The recurrence of thrush in the infant should be treated with nystatin and steps taken to eliminate the source of reinfection. The best means of prevention in this instance is application of topical nystatin to the mother's nipples, which directly treats the source of reinfection.

Administration of oral nystatin prophylaxis to the mother will not treat the superficial infection of her nipples as effectively as topical therapy. Additionally, oral nystatin given to the mother would be transferred to a small degree to the infant through human milk, although the drug concentration should be so low as to pose little risk.

Administration of oral nystatin prophylaxis to the infant also would be inadequate intervention because there would be continued contact with the mother's infected nipples.

Rinsing the infant's mouth following feeding would not lessen the risk of reinfection substantially. There is no reason to discontinue breastfeeding in this infant once appropriate antifungal treatment has been instituted. Switching to bottle feeding with formula without proper education might result in another recurrence of thrush because artificial nipples also can become contaminated.

References:

Armstrong D, Schmitt HJ. Candida species infections. In: Rudolph AM, ed. Rudolph's Pediatrics. 20th ed. Stamford, Conn: Appleton & Lange; 1996:700-703

Committee on Infectious Diseases. Candidiasis. In: Report of the Committee on Infectious Diseases. 24th ed. Elk Grove Village, Ill: American Academy of Pediatrics; 1997:162-164

1998 Self-Assessment Exercise —

XXII. Disorders of the eye

[Return to Category List]

Questions [Print Directions]

Question 114. Answer.

A 13-year-old patient presents with a red, swollen area on the upper eyelid. The swollen area is in the anterior lid margin, which confirms your suspected diagnosis of an external hordeolum (stye).

Of the following, the BEST management of this lesion is

A. administration of oral oxacillin

B. application of topical corticosteroid

C. application of warm compresses twice daily

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D. intralesional injection of betamethasone

E. observation alone

Question 264. Answer.

Among the following, the MOST appropriate time for the initial examination of the retina of a 1,200 g infant born at 25 weeks' gestation and treated with supplemental oxygen is at

A. 24 hours

B. 2 weeks

C. 6 weeks

D. 8 weeks

E. 12 weeks

Answers

Critique 114 Preferred Response: C

[View Question]

The patient in the vignette has a stye (external hordeolum), which is an inflammation or an abscess of the ciliary follicle and its associated sebaceous gland along the anterior lid margin. Styes are the result of a pyogenic infection, usually caused by Staphylococcus aureus. At times, a stye can be confused with a chalazion (lipogranuloma of the meibomian gland), preorbital cellulitis, or dacryocystitis

Treatment for a stye consists of warm moist compresses applied for 20 minutes several times a day and application of an antibiotic opthalmic ointment such as erythromycin. If this treatment fails, incision and drainage are indicated. Administration of systemic antibiotic therapy such as oral oxacillin is not indicated because a stye is a local infection. Neither topical steroid nor intralesional injection of betamethasone is indicated as treatment for a stye. Finally, observation alone may result in spontaneous resolution of the stye, but the use of topical warm compresses and antibiotic ointment often will speed the recovery.

References:

Apt L, Miller KM. Hordeolum and chalazion. In: Rudolph AM, Hoffman JIE, Rudolph CD, eds. Rudolph's Pediatrics. 20th ed. Stamford, Conn: Appleton & Lange; 1996:2073-2074

Nelson LB. Abnormalities of the lids. In: Nelson WE, Behrman RE, Kliegman RM, Arvin AM, eds. Nelson Textbook of Pediatrics. 15th ed. Philadelphia, Pa: WB Saunders Co; 1996:1776-1778

Trobe JD. The red eye. In: The Physician's Guide to Eye Care. San Francisco, Calif: American Academy of Ophthalmology; 1993:44-45

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Critique 264 Preferred Response: C

[View Question]

Retinopathy of prematurity (ROP) is an ophthalmologic problem that occurs most frequently in low-birthweight preterm infants. Because cryotherapy has been proven to be of benefit for some infants who have retinal detachment due to ROP, it is important that all infants at risk be screened between 4 and 6 weeks of chronologic age and before 31 weeks post-conceptional age (ie, gestational age at birth plus chronologic age in weeks). Accordingly, the infant in the vignette should be examined at about 6 weeks. If the infant is to be discharged prior to this time, the screening should be performed at discharge. Further examinations are based on findings from the screening.

Vessels that supply the retina begin to grow from the optic disk at approximately the fourth month of gestation; growth is completed by 40 to 44 weeks' gestation. During this time of active growth, the vessels are vulnerable to injury that can result in obliteration. A neovascular response to this injury can be seen with indirect ophthalmoscopy. Because this response develops over time, a screening examination performed prior to 5 to 7 weeks after birth may miss the neovascularization process. Although milder stages of ROP can be seen only with the indirect ophthalmoscope, severe stages requiring surgery result in changes in the posterior pole of the eye. These changes can be seen with a direct ophthalmascope, and it has been shown that most clinicians can recognize this "plus disease" when present.

If the repair process is orderly, there are usually minimal sequelae. However, disordered neovascularization may lead to vitreal hemorrhage and scar formation with subsequent retinal traction or detachment. If the screening examination is delayed beyond 31 weeks post-conceptional age, the opportunity to save some of the infant's vision, if severe ROP develops, will be lost.

Review of the natural course of ROP has shown that the infant's post-conceptual or corrected gestational age markedly influences the timing of the disease progress. Severe ROP does not present until approximately 32 weeks' and peaks at 38 weeks' corrected age. It may be better to adjust the first screening examination in the most preterm infant by the corrected gestational age.

Infants of lowest gestational age are at greatest risk for developing ROP. Prolonged exposure to supplemental oxygen also greatly increases the incidence of ROP. Cases of ROP have been reported in term infants who have cyanotic heart disease. Accordingly, to identify infants at risk for ROP, screening should be performed on all neonates who have a birthweight less than 1,500 g and who were born earlier than 28 weeks' gestation, irrespective of oxygen use, and all older preterm infants who have had an unstable clinical course.

References:

Apt L, Miller KM. Retinal vascular diseases. In: Rudolph AM, Hoffman JIE, Rudolph CD, eds. Rudolph's Pediatrics. 20th ed. Stamford, Conn: Appleton & Lange; 1996:2103-2104

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Cryotherapy for Retinopathy of Prematurity Cooperative Group. Multicenter trial of cryotherapy for retinopathy of prematurity: 1-year outcome--structure and function. Arch Ophthalmol. 1990;108:1408-1416

Palmer EA, Flynn JT, Hardy RJ, et al. Incidence and early course of retinopathy of prematurity. Ophthalmology. 1991;98:1628-1640

Phelps DL. Retinopathy of prematurity. Pediatrics in Review. 1995;16:50-56

Screening examination of premature infants for retinopathy of prematurity: a joint statement of the American Academy of Pediatrics, the American Association for Pediatric Ophthalmology and Strabismus, and the American Academy of Ophthalmology. Pediatrics. 1997;100:273