Disorders of the Eye

Question 1.
Delayed removal of a congenital and complete unilateral cataract may lead to:
a)
Amblyopia
b)
Glaucoma
c)
Uveitis
d)
Strabismus
e)
Nyctalopia
a)Explanation: Amblyopia is a vision loss that is due not to a specific organic
lesion but rather to deprivation or disuse of the retina. Even after delayed removal of a
cataract or correction of strabismus, the previously unused retina "tunes out" the
image. (See Chapter 619 in Nelson Textbook of Pediatrics, 17th ed.)
-----------------------------------------------------------------------------Question 2.
Cataracts are noted in all of the following except:
a)
Rubella (congenital)
b)
Galactosemia
c)
Galactokinase deficiency
d)
Neonatal hypoglycemia
e)
Hypocalcemia
f)
Lowe syndrome
g)
Hyperoxygenation
h)
H. Steroid therapy
i)
I. Child abuse
g)Explanation: Hyperoxia in preterm neonates usually causes retinopathy of
prematurity, which occasionally is associated with cataracts. Oxygen does not directly
cause cataracts. (See Chapter 619 in Nelson Textbook of Pediatrics, 17th ed.)
-----------------------------------------------------------------------------Question 3.
An 18-mo-old manifests pendular nystagmus, head nodding, and torticollis. Findings
on a cranial MRI scan are normal. This child is most likely to have:
a)
Epilepsy
b)
Congenital blindness
c)
Neuroblastoma
d)
Dysmetria
e)
Spasmus nutans
e)Explanation: The acquired triad of nystagmus, head nodding, and torticollis, in
its classic form, is self-limited and benign. Nonetheless, children with brain tumors
may have signs resembling components of spasmus nutans. (See Chapter 614 in
Nelson Textbook of Pediatrics, 17th ed.)
----------------------------------------------------------------------------Question 4.

A 7-yr-old girl experiences fullness of the right upper eyelid and downward
displacement of the eye over a 2-mo period. The right eye also appears to be
proptotic. Which of the following is the most likely diagnosis?
a)
Myasthenia gravis
b)
Right superior oblique palsy
c)
Chalazion
d)
Rhabdomyosarcoma
e)
Hypothyroidism
d)Explanation: Rhabdomyosarcoma of the face, orbit, and sinus often presents
early because of the space-occupying and displacement effects of tumor growth. (See
Chapter 624 in Nelson Textbook of Pediatrics, 17th ed.)
-----------------------------------------------------------------------------Question 5.
The most common presenting sign of retinoblastoma is:
a)
Heterophoria
b)
Hypopyon
c)
Leukocoria
d)
Coloboma
e)
Red reflex
c)Explanation: The clinical manifestations of retinoblastoma vary, but the initial
sign in the majority of cases is leukocoria (white pupillary reflex, also known as "cat
eye") instead of the normal red pupillary reflex. (See Chapter 621 in Nelson Textbook
of Pediatrics, 17th ed.)
-----------------------------------------------------------------------------Question 6.
A 17-yr-old girl taking oral contraceptives presents with headache, nausea, and
vomiting. Physical examination reveals papilledema. The most appropriate action is:
a)
Discontinue the oral contraceptives and re-evaluate in 7-14 days
b)
Discontinue the oral contraceptives and administer promethazine (Phenergan)
as needed for nausea and vomiting
c)
Discontinue the oral contraceptives and administer oral corticosteroids
d)
Perform computed tomography (CT) or magnetic resonance imaging (MRI)
study of the head
e)
Perform lumbar puncture for determination of cerebrospinal fluid pressure
d)Explanation: Papilledema constitutes a neurologic emergency. Neuroimaging
should be performed, and if no intracranial masses are found, a lumbar puncture for
determination of cerebrospinal fluid pressure should be performed. This patient may
have pseudotumor cerebri. (See Chapter 622 in Nelson Textbook of Pediatrics, 17th
ed.)
----------------------------------------------------------------------------Question 7.
A 1-yr-old child presents with increased size of the cornea. Review of systems
reveals history of increased tearing and apparent sensitivity to light. The cornea
appears cloudy. The most likely diagnosis is:
a)
Retinoblastoma
b)
Glaucoma

c)
d)
e)

Chorioretinitis
Aniridia
Coloboma

b)Explanation: Symptoms of infantile glaucoma (glaucoma that begins in the

first 3 years of life) include the classic triad of epiphora (tearing), photophobia
(sensitivity to light), and blepharospasm (eyelid squeezing), which are usually
attributed to corneal irritation. An increase in intraocular pressure leads to expansion
of the globe, including the cornea. (See Chapter 623 in Nelson Textbook of Pediatrics,
17th ed.)
-----------------------------------------------------------------------------Question 8.
Which of the following is the most appropriate initial treatment for the child
described in Question 9?
a)
Topical glaucoma medications
b)
Oral glaucoma medications
c)
Corticosteroids
d)
Surgery
e)
Medications and surgery
d)Explanation: Unlike glaucoma in adults, in which medications are the first
line of therapy, the treatment of infantile glaucoma is primarily surgical. Procedures
used to treat glaucoma in children include surgery to establish a more normal anterior
chamber angle (goniotomy and trabeculotomy), to create a route for aqueous fluid to
exit the eye (trabeculectomy and seton surgery), or to reduce aqueous fluid production
(cyclocryotherapy and photocyclo-coagulation). In many children, several operations
are required to lower and maintain the intraocular pressure adequately. Long-term
medications may be necessary as well. (See Chapter 623 in Nelson Textbook of
Pediatrics, 17th ed.)
----------------------------------------------------------------------------Question 9.
A 12-yr-old boy has a small corneal abrasion detected by Wood lamp examination.
Which of the following is the most appropriate treatment?
a)
Topical antibiotic ointment
b)
Topical antibiotic ointment and a semipressure patch
c)
Topical antibiotic ointment, a semipressure patch, and a topical cycloplegic
agent
d)
Topical antibiotic ointment, a semipressure patch, and a topical anesthetic as
necessary for pain
e)
Topical antibiotic ointment, a topical cycloplegic agent, and a topical
anesthetic as necessary for pain
a)Explanation: Corneal abrasions are treated with frequent applications of a
topical antibiotic ointment until the epithelium is completely healed. The use of a
semi-pressure patch does not improve healing time or decrease pain. Furthermore, an
improperly applied patch may itself abrade the cornea. A topical cycloplegic agent
(cyclopentolate hydrochloride 1%) can relieve the pain from ciliary spasm in patients
with large abrasions. Topical anesthetics should not be given at home because they

retard epithelial healing and inhibit the natural blinking reflex. (See Chapter 625 in
Nelson Textbook of Pediatrics, 17th ed.)
----------------------------------------------------------------------------Question 10.
All of the following statements concerning the newborn eye are correct except:
a)
The cornea is smaller than that of an adult
b)
Tears are often not present with crying until 1-3 mo of age
c)
Ocular alignment may not be perfect in the first several months of life
d)
Most infants are myopic (nearsighted)
e)
Iris color may change in the first several months of life
d)Explanation: Most infants are hyperopic (farsighted). The cornea is smaller in
infants and grows to reach adult size near the age of 2 yr. Tears are not usually
produced with crying until a few months of age. It is common for children to have
strabismus in the first few months of life. (See Chapter 609 in Nelson Textbook of
Pediatrics, 17th ed.)
----------------------------------------------------------------------------Question 11.
Which of the following tests is not part of a routine eye examination?
a)
Visual acuity
b)
Pupil assessment
c)
Preferential looking test
d)
External examination
e)
Ocular motility
c)Explanation: Preferential looking tests are done by experienced examiners and
are not part of a routine eye examination. (See Chapter 610 in Nelson Textbook of
Pediatrics, 17th ed.)
----------------------------------------------------------------------------Question 12.
Which of the following statements concerning abnormalities of refraction is correct?
a)
Children with hyperopia will often squint to see better
b)
Astigmatism may be caused by an irregular shape of the cornea
c)
Myopia can be cured with the use of bifocal lenses
d)
Anisometropia occurs when one eye sees better than the other
e)
Myopia is always a benign condition
b)Explanation: Astigmatism is commonly caused by an irregular corneal shape.
Children with hyperopia will accommodate to see better. Children with myopia will
frequently squint to see better. Scientific studies have not proved that bifocals can
cure myopia. Anisometropia occurs when there is a difference in refractive error
between the two eyes. It may lead to amblyopia and decrease vision in one eye.
However, the term anisometropia does not indicate a difference in vision by itself.
High levels of myopia may lead to retinal detachment. (See Chapter 611 in Nelson
Textbook of Pediatrics, 17th ed.)
----------------------------------------------------------------------------Question 13.

A 4-yr-old girl presents with a large esotropia and poor vision in one eye. Both eyes
are normal on examination. What is the most likely reason for her poor vision?
a)
Need for glasses
b)
Malingering
c)
Cataract
d)
Suppression
e)
Amblyopia
e)Explanation: This child probably has amblyopia secondary to her strabismus.
Although the need for glasses in only one eye or a cataract in one eye could present in
the same way, these problems are far less common in this age group. (See Chapter
612 in Nelson Textbook of Pediatrics, 17th ed.)
----------------------------------------------------------------------------Question 14.
A newborn is presented with what appear to be large pupils that do not constrict to
light. There is no family history of similar ocular findings. An appropriate study to be
performed in the future is:
a)
CT scan of the head and orbit
b)
Visual evoked potentials
c)
Renal ultrasound study
d)
Lumbar puncture
e)
Cocaine test
c)Explanation: In aniridia, the pupils look large and nonreactive. Because
children so affected are at risk for Wilms tumor, renal ultrasound study is indicated.
(See Chapter 613 in Nelson Textbook of Pediatrics, 17th ed.)
----------------------------------------------------------------------------Question 15.
A 2-yr-old boy presents with pronounced crossing of his left eye of 2 months'
duration. On further examination, he is found to be excessively farsighted for his age.
The most likely diagnosis is:
a)
Congenital superior oblique palsy
b)
Accommodative esotropia
c)
Intermittent exotropia
d)
Duane syndrome
e)
Congenital esotropia
b)Explanation: Accommodative esotropia causes a crossing of the eyes
secondary to a high degree of farsightedness. Congenital esotropia does not develop at
this age. (See Chapter 614 in Nelson Textbook of Pediatrics, 17th ed.)
----------------------------------------------------------------------------Question 16.
Which of the following statements concerning congenital ptosis is true?
a)
Surgery should be performed shortly after birth in all cases
b)
Amblyopia will occur only if the lid covers the pupil
c)
Amblyopia cannot occur once the lid is surgically elevated
d)
Ptosis may occur in association with other ocular or systemic disorders

e)

Frontalis suspension surgery is used for mild cases of ptosis

d)Explanation: Ptosis is often associated with other systemic disorders. Surgery

does not need to be performed early if the vision is developing normally. Amblyopia
can occur if the ptosis causes a unilateral astigmatism. It can occur even if the pupil is
not completely occluded. Amblyopia can continue to occur even after successful
ptosis surgery. Frontalis suspension is used for cases of severe ptosis. (See Chapter
615 in Nelson Textbook of Pediatrics, 17th ed.)
----------------------------------------------------------------------------Question 17.
A 3-mo-old girl is presented with tearing and mucous discharge from her left eye that
has been present since near birth. Her external examination is otherwise normal.
Appropriate forms of treatment would include:
a)
Nasolacrimal massage
b)
Topical antibiotics for infections if they occur
c)
Cleansing of the lids
d)
Probing of the nasolacrimal duct if the symptoms do not resolve with time
e)
All of the above
e)Explanation: All of the above are correct. Many cases of nasolacrimal duct
obstruction will resolve within the first year of life. (See Chapter 616 in Nelson
Textbook of Pediatrics, 17th ed.)
----------------------------------------------------------------------------Question 18.
A 2-day-old infant is presented with bilateral conjunctival redness and purulent
discharge. The most likely diagnosis is:
a)
Nasolacrimal duct obstruction
b)
Ophthalmia neonatorum
c)
Congenital glaucoma
d)
Blepharitis
e)
Vernal conjunctivitis
b)Explanation: Ophthalmia neonatorum occurs shortly after birth. A
nasolacrimal duct obstruction does not usually cause significant conjunctival redness
this early. Congenital glaucoma is often associated with tearing but not with a
purulent discharge. Blepharitis and vernal conjunctivitis do not generally occur at this
age. (See Chapter 617 in Nelson Textbook of Pediatrics, 17th ed.)
----------------------------------------------------------------------------Question 19.
All of the following disorders may present as a congenital corneal opacity except:
a)
Sclerocornea
b)
Congenital cataract
c)
Infantile glaucoma
d)
Peters anomaly
e)
Epibulbar dermoid

b)Explanation: A cataract is opacity of the lens, not the cornea. (See Chapter 618
in Nelson Textbook of Pediatrics, 17th ed.)
-----------------------------------------------------------------------------Question 20.
A child presents with a dense unilateral cataract. All of the following are important
factors in the visual prognosis following the removal of the cataract except:
a)
Whether a contact lens or an intraocular lens implant is used
b)
The compliance with patching for amblyopia
c)
The length of time the cataract was present
d)
The age of the child at presentation
e)
The health status of the posterior segment of the eye
a)Explanation: The mode of visual rehabilitation is not the most important factor
in the outcome of pediatric cataract surgery. Early diagnosis, age at time of cataract
development, and success with occlusion therapy are the most important factors. (See
Chapter 619 in Nelson Textbook of Pediatrics, 17th ed.)
----------------------------------------------------------------------------Question 21.
A young girl is found to have bilateral iritis on routine examination. On further
questioning, it is found that she has been limping for the last several weeks. The most
likely diagnosis is:
a)
Sympathetic ophthalmia
b)
Juvenile rheumatoid arthritis
c)
Herpes simplex
d)
Toxoplasmosis
e)
Kawasaki disease
b)Explanation: Uveitis in patients with JRA commonly presents without pain or
redness. Sympathetic ophthalmia is unilateral and occurs in the uninjured eye
following a significant ocular injury. Herpes simplex may cause an anterior uveitis but
is usually unilateral and associated with pain. Toxoplasmosis causes a posterior
uveitis and is usually unilateral. Kawasaki disease is not associated with arthritis and
usually causes conjunctivitis. (See Chapter 620 in Nelson Textbook of Pediatrics, 17th
ed.)
-----------------------------------------------------------------------------Question 22.
A 20-mo-old girl is noted by her parents to have a unilateral white pupil. Funduscopic
examination reveals a very large white mass that fills most of the eye. All of the
following statements regarding this patient are correct except:
a)
Genetic counseling is indicated for this family
b)
Removal of the eye may be required
c)
A CT scan may demonstrate calcium within the lesion
d)
A biopsy should be performed as soon as possible
e)
Examination of the other eye should be performed with the patient under
anesthesia
d)Explanation: This patient is most likely to have a retinoblastoma. Because it
can be a hereditary tumor, genetic counseling is indicated. Treatment of a large
unilateral tumor may require enucleation. Appropriate work-up may include a CT

scan to demonstrate calcium within the eye. Examination of the other eye for smaller
tumors should always be performed. Biopsy of the suspected tumor is contraindicated,
as it may lead to spread of the tumor cells. (See Chapter 621 in Nelson Textbook of
Pediatrics, 17th ed.)
-----------------------------------------------------------------------------Question 23.
A 4-yr-old boy who is being followed by his primary care doctor for short stature is
found to have poor vision in one eye. On ophthalmic examination, he is noted to have
an afferent pupillary defect on the affected side. His anterior segment is normal, and
his vision cannot be improved with glasses. The most likely diagnosis is:
a)
Strabismus
b) Cataract
c)Optic nerve hypoplasia
d)Glaucoma
e)Retinal detachment
c)Explanation: Optic nerve hypoplasia may be associated with growth hormone
deficiency and poor vision. Patients with unilateral optic nerve abnormalities will
demonstrate an afferent pupillary defect. (See Chapter 622 in Nelson Textbook of
Pediatrics, 17th ed.)
----------------------------------------------------------------------------Question 24.
A 3-mo-old boy is brought in by his parents because he appears to be very light
sensitive. On examination, he is tearing, and his corneas appear large and cloudy. The
most likely diagnosis is:
a)Infantile glaucoma
b)Nasolacrimal tear duct obstruction
c)Peters anomaly
d)Infantile cataracts
e)Uveitis
a)Explanation: The classic findings in infantile glaucoma are enlarged corneas,
cloudy corneas, tearing, and light sensitivity. Although a tear duct obstruction also
causes tearing, it does not cause a change in the size or clarity of the cornea, and it is
not associated with photophobia. (See Chapter 623 in Nelson Textbook of Pediatrics,
17th ed.)
----------------------------------------------------------------------------Question 25.
A 2-yr-old child presents with a 1-day history of unilateral proptosis and fever.
Appropriate next steps in the diagnosis and treatment of this child could include all of
the following except:
a)
CT scan of the head, orbit, and sinuses
b)
Ophthalmologic consultation
c)
Administration of a broad-spectrum antibiotic
d)
Surgical drainage of any abscess
e)
Determination of TSH and TRH blood levels
e)Explanation: Although thyroid disease may manifest with unilateral proptosis,
it is not associated with fever and does not usually develop this rapidly. The other
answers are appropriate steps in the management of a child with a preseptal or orbital
cellulitis. (See Chapter 624 in Nelson Textbook of Pediatrics, 17th ed.)
-----------------------------------------------------------------------------

Question 26.
Which of the following statements regarding children with traumatic hyphemas is
most correct?
a)
This is generally a benign condition
b)
If secondary bleeding does not occur by the second day, there is little risk of
further complications
c)
Children with sickle cell disease are at increased risk for loss of vision
d)
Once the blood leaves the anterior chamber, there is no longer any risk from
the injury causing the hyphema
e)
The blood itself produces no ill effects
c)Explanation: Children with sickle cell disease are at increased risk for vision
loss following a traumatic hyphema. Traumatic hyphemas can be a significant cause
for vision loss. Rebleeding is most common 3 to 5 days after the initial injury. Blood
within the anterior chamber can lead to corneal blood staining and/or glaucoma.
Patients with a history of a hyphema are at risk for the development of glaucoma later
in life. (See Chapter 625 in Nelson Textbook of Pediatrics, 17th ed.)
-----------------------------------------------------------------------------