1. What is a normal central endothelial cell count?

a. 250–350 cells/mm2
b. 500–1000 cells/mm2
c. 2000–3000 cells/mm2
d. 5000–6000 cells/mm2
2. What is the best noninvasive way to visualize the cysts pathognomonic of Acanthamoeba
keratitis?
a. anterior segment optical coherence tomography (AS- OCT)
b. confocal microscopy
c. sclerotic scatter
d. specular microscopy
3. Subtle abnormalities in posterior corneal contour are best evaluated by what diagnostic tool?
a. confocal microscopy
b. Placido- disk imaging
c. Scheimpflug tomography
d. slit- lamp microscope
4. A 70- year- old man pre sents with chronic unilateral crusting and discharge from the right
lateral canthus. Gram stain of the discharge reveals numerous gram- negative cocci in pairs.
What pathogen is the most likely cause of the patient’s symptoms?
a. Chlamydia trachomatis
b. Haemophilus influenzae
c. Moraxella lacunata
d. Pseudomonas aeruginosa
5. What organism is most commonly associated with phlyctenulosis in low- income countries?
a. Candida albicans
b. Chlamydia trachomatis
c. Mycobacterium tuberculosis
d. Staphylococcus aureus
6. Which type of topical glaucoma medi cation is most likely to cause aqueous tear deficiency?
a. α2- adrenergic agonists
b. β- blockers
c. carbonic anhydrase inhibitors
d. prostaglandin analogues
7. What is the primary cause of exposure keratopathy in patients with Parkinson disease?
a. cicatricial ectropion
b. decreased blink frequency
c. proptosis
d. upper- eyelid retraction
8. A 58- year- old obese man pre sents with chronic ocular irritation and inflammation. On
examination, the clinician notes significant eyelid laxity; in addition, the upper tarsus everts with
minimal upward force. What systemic workup is indicated?
a. buccal mucosal biopsy
 b. magnetic resonance imaging (MRI) of the brain
c. sleep study
d. thyroid function tests
9. After surgical removal of a pterygium, what is the best adjunctive treatment option to minimize
the risk of recurrence and avoid any late- term complications?
a. amniotic membrane transplant
b. bevacizumab injection
c. conjunctival autograft
d. mitomycin- C (MMC) injection
10. What is the most common complication of a conjunctival flap?
a. epithelial inclusion cysts
b. flap retraction
c. hemorrhage beneath the flap
d. ptosis
11. In addition to antibiotics, what is the most appropriate management for a 3-mm central corneal
perforation caused by Neisseria gonorrhoeae?
a. application of cyanoacrylate glue
b. corneal patch graft
c. multi- layered amniotic membrane graft
d. primary closure with 10-0 nylon sutures
12. In cases of untreated congenital syphilis, at what age does the onset of interstitial keratitis
typically occur?
a. 1–3 months
b. 1–2 years
c. 6–12 years
d. 20–40 years
13. How can posterior keratoconus be distinguished from Peters anomaly anatomically?
a. Descemet membrane is pre sent in posterior keratoconus but absent in Peters anomaly.
b. Descemet membrane is pre sent in Peters anomaly but absent in posterior keratoconus.
c. A thin layer of uveal tissue lining the posterior cornea is pre sent in posterior
keratoconus but absent in Peters anomaly.
d. A thin layer of uveal tissue lining the posterior cornea is absent in posterior keratoconus
but pre sent in Peters anomaly.
14. What is the treatment for symptomatic conjunctival concretions?
a. oral azithromycin
b. removal of lesions with a 25- gauge needle
c. topical ste roids
d. warm compresses
15. What is the composition of the deposits in spheroidal degeneration?
a. carbohydrate
b. glycosaminoglycan
c. lipid
 d. protein
16. A 73- year- old woman with hyperparathyroidism reports chronic ocular irritation and foreign
body sensation. There is a white, horizontal plaque in the interpalpebral zone of the cornea
bilaterally. Her corneal condition primarily affects what layer of the cornea?
a. epithelium
b. Bowman layer
c. anterior stroma
d. deep stroma
17. 17. What is the appropriate surgical therapy for visually significant Salzmann nodular
degeneration?
a. cauterization
b. cryotherapy
c. deep anterior lamellar keratoplasty (DALK)
d. superficial keratectomy
18. A 54- year- old man who has been taking amiodarone for cardiac arrhythmia has developed
deposits in a whorl- like pattern in the inferocentral corneal epithelium of both eyes. What is the
most appropriate management of the corneal deposits?
a. discontinuation of the amiodarone
b. observation
c. reduction of the dose of amiodarone
d. superficial keratectomy with excimer laser
19. A 55- year- old woman with a history of multiple episodes of severe eye pain upon awakening
reports that both eyes have been affected, although not always si mul ta neously. What corneal
findings are most likely to be pre sent on slit- lamp examination?
a. basement membrane fingerprint lines
b. epithelial filaments
c. punctate epithelial erosions
d. subepithelial infiltrates
20. What condition is characterized by bilateral dysfunctional corneal endothelial cells that
demonstrate epithelial- like be hav ior?
a. congenital hereditary endothelial dystrophy
b. Fuchs endothelial corneal dystrophy (FECD)
c. iridocorneal endothelial (ICE) syndrome
d. posterior polymorphous corneal dystrophy
21. A 57- year- old woman has confluent guttae in both eyes. She reports intermittent, transient
blurred vision in her left eye on waking. At 8 am, her best- corrected visual acuity is 20/25 OD
and 20/30 OS. What is the appropriate course of management?
a. ban dage soft contact lens
b. hypertonic sodium chloride ointment in the left eye at night
c. topical corticosteroids 4 times daily
d. penetrating keratoplasty (PK)
22. What behavioral modification can lower the risk of keratoconus progression?
 a. avoidance of eye rubbing
b. increased dietary consumption of omega-3 fatty acids
c. smoking cessation
d. reduction of alcohol use
23. Where does corneal thinning usually occur in eyes with pellucid marginal degeneration?
a. centrally
b. inferior periphery
c. nasal and temporal periphery
d. superior periphery
24. A 42- year- old man is referred for evaluation of red and irritated eyes. Examination reveals
severe punctate erosions on the cornea and conjunctiva. A foamy appearing patch is noted on
the temporal bulbar conjunctiva of the right eye. Tears bead along the conjunctival surface in
small, individual droplets. The patient’s medical history is significant for morbid obesity, for
which he underwent gastric bypass surgery 6 months prior. Abnormalities in what cell type are
responsible for the patient’s symptoms and examination findings?
a. goblet cells
b. keratocytes
c. lymphocytes
d. squamous epithelial cells
25. A young child with cognitive impairment and hyperkeratotic lesions of the palms and soles is
treated for recurrent episodes of corneal erosions and pseudodendrites that don’t stain well
with fluorescein or rose bengal dye. What treatment is indicated?
a. high- dose oral ascorbic acid
b. oral and/or topical cysteamine
c. oral penicillamine
d. restriction of tyrosine and phenylalanine intake
26. A 32- year- old man visits a clinic for a routine examination. He has a history of thyroid cancer
and uncontrolled hypertension. The anterior segment examination reveals enlarged corneal
nerves. What additional finding is most likely pre sent in this patient?
a. angioid streaks
b. marfanoid body habitus
c. microspherophakia
d. preauricular skin tags
27. A 15- year- old adolescent girl with a history of renal transplant reports experiencing aright-
sided headache for 2 days. Examination reveals redness of the right forehead, right upper eyelid,
and nose, with a few clear vesicles. Slit- lamp examination shows conjuncttival hyperemia and
superficial punctate keratitis without deeper corneal involvement or iritis. What is the preferred
management?
a. systemic antiviral medi cations
b. topical antiviral medi cations
c. oral corticosteroids
d. topical corticosteroids
28. A 34- year- old woman was treated for herpes simplex virus (HSV) epithelial keratitis with topical
trifluridine 8 times daily for 2 weeks. She reports that the ocular discomfort improved but that
shortly afterward, she developed a new onset of redness, tearing, and discomfort. What is the
most likely cause of her newer symptoms?
a. recurrent HSV keratitis
b. superimposed bacterial conjunctivitis
c. toxic epithelial keratopathy
d. trichiasis
29. Who should receive the recombinant zoster vaccine (Shingrix)?
a. immunocompetent adults aged 50 years and older
b. immunocompetent adults aged 65 years and older
c. immunocompetent adults who have not had chickenpox
d. immunosuppressed adults aged 18 and older
30. A 7- year- old boy pre sents with a 2- day history of unilateral purulent conjunctivitis with diffuse
subconjunctival hemorrhage. Gram stain shows numerous gram- negative bacilli. What is the
most likely causative pathogen?
a. Haemophilus influenzae
b. Moraxella catarrhalis
c. Neisseria gonorrhoeae
d. Streptococcus pneumoniae
31. A 27- year- old patient has 24- hour duration of severe, hyperpurulent conjunctival discharge
without a corneal ulcer. Conjunctival Gram stain reveals numerous neutrophils with gram
negative intracellular diplococci (GNID). What is the most appropriate treatment?
a. intramuscular ceftriaxone
b. intravenous penicillin G
c. oral azithromycin
d. topical gentamicin ointment
32. For acute Stevens- Johnson syndrome (SJS) with a conjunctival defect, what intervention has the
greatest long- term benefit?
a. amniotic membrane transplantation
b. placement of a symblepharon ring
c. repeated forniceal debridement with a glass rod
d. temporary tarsorrhaphy
33. A patient pre sents with bilateral stromal infiltrates without epithelial ulceration. During the
review of systems, the patient reports a recent viral upper respiratory tract infection and
difficulty with balance and tinnitus. Rapid plasma reagin (RPR) test and micro hemagglutination
assay (fluo rescent treponemal antibody absorption; FTA- ABS) results are negative. What
systemic treatment is indicated as part of the typical therapeutic regimen for this condition?
a. systemic antibiotic
b. systemic antiviral medi cation
c. systemic corticosteroid
d. systemic nonsteroidal anti- inflammatory drug
34. What systemic disease is most frequently associated with peripheral ulcerative keratitis?
a. granulomatosis with polyangiitis (Wegener granulomatosis)
b. rheumatoid arthritis
c. systemic lupus erythematosus
d. ulcerative colitis
35. Conjunctival melanoma in what location is associated with the best long- term prognosis?
a. bulbar conjunctiva
b. caruncle
c. fornix
d. palpebral conjunctiva
36. A 32- year- old man is diagnosed with ocular surface squamous neoplasia (OSSN). A serologic
test should be performed to look for what condition?
a. herpes simplex virus
b. HIV
c. human herpesvirus 8
d. varicella- zoster virus
37. In a patient with primary acquired melanosis (PAM), what clock- hour extent of conjunctival
involvement is an indication to remove the lesion?
a. more than 1 clock- hour
b. more than 2 clock- hours
c. more than 3 clock- hours
d. more than 6 clock- hours
38. What physical examination finding is the best predictor of late corneal neovascularization in a
patient with a chemical injury?
a. amount of scleral and limbal ischemia
b. degree of corneal edema
c. extent of skin and eyelid burns
d. presence of particulate chemical matter in the fornices
39. Why is tissue from donors younger than 2 years generally not used in corneal transplantation?
a. The corneal endothelial cells are not sufficiently developed.
b. The corneal stroma is too thin.
c. The tissue is steeply curved and flaccid.
d. There are ethical concerns about using tissue from infants and very young children.
40. In US eye banks, how long can corneal tissue remain viable once preserved in the storage
medium?
a. 24–48 hours
b. 3–5 days
c. 11–14 days
d. 25–35 days
41. What type of rejection is more common in DALK than PK?
a. endothelial rejection
b. epithelial rejection
 c. stromal rejection
d. subepithelial rejection

525

Answers

1. c. Normal central endothelial cell count is between 2000 and 3000 cell/mm2. Central endothelial cell
density decreases with age at an average rate of approximately 0.6% each year, diminishing from
approximately 3400 cells/mm2 at age 15 years to approximately 2300 cells/mm2 at age 85 years. Eyes
with an endothelial cell count below 500 cells/mm2 may be at risk for developing corneal edema.

2. b. Confocal microscopy has demonstrated efficacy in the early diagnosis of patients with
Acanthamoeba keratitis. It allows rapid identification of Acanthamoeba cysts with a relatively high
sensitivity and specificity, although it requires a skilled observer to interpret the images. Anterior
segment optical coherence tomography (AS- OCT) can providecross- sectional information about corneal
pathology but the resolution of the imagesobtained with AS- OCT is inadequate for detecting
microscopic structures the size of Acanthamoeba cysts. Sclerotic scatter is useful for demonstrating
subtle anterior corneal pathology such as mild central corneal edema but is not capable of resolving
pathology at the microscopic level. Specular microscopy allows imaging of the corneal endothelium but
is not considered useful in the diagnosis of Acanthamoeba keratitis.

3. c. The posterior contour is best evaluated using a Scheimpflug imaging system, a rotating scanning
slit- beam device. This technology is particularly useful for evaluating subtle changes in the posterior
corneal curvature and elevation that can indicate a higher risk for keratoconus or corneal ectasia in
patients undergoing refractive corneal surgery. Although confocal microscopy provides an in vivo highly
magnified view of the corneal epithelium, stroma, and endothelium, it does not provide contour
information. Placido- disk imaging employed in corneal topography only provides information about the
anterior corneal surface. A slit- lamp microscope is not sensitive enough to enable the clinician to
identify subtle corneal curvature changes.

4. c. The patient’s symptoms are consistent with those associated with angular blepharitis, a relatively
uncommon entity that may be mistaken for other forms of chronic eyelid margin inflammation.
Although Staphylococcus aureus and herpes simplex virus are possible etiologies, angular blepharitis is
classically caused by Moraxella lacunata (which produces the Gram stain result of gram- negative cocci
in pairs). Chlamydia trachomatis is apotential cause of chronic follicular conjunctivitis, but it is not
associated with angularblepharitis. Pseudomonas aeruginosa and Haemophilus influenzae can cause
acute conjunctivitis, but they are not known to cause angular blepharitis and do not demonstrate gram
negative cocci in pairs.

5. c. Phlyctenules are hyperemic, focal nodules consisting of chronic inflammatory cells. Phlyctenulosis is
believed to represent a T- cell– mediated, or delayed hypersensitiveity (type IV) response induced by
microbial antigens. Phlyctenulosis is most frequently associated with Staphlylococcus aureus in high-
income countries and is classically associated with Mycobacterium tuberculosis infection affecting
malnourished individuals in tuberculosis- endemic areas of the world. Phlyctenulosis is not an ocular
manifestation of Candida albicans or Chlamydia trachomatis infection.

6. b. Multiple medi cations have been associated with aqueous tear deficiency (ATD). Common systemic
medi cations implicated in ATD include diuretics, antihistamines, anticholinergics, and psychotropic
drugs. Among topical medications, β- blockers are most commonly associated with ATD. Although α2-
adrenergic agonists, such as brimonidine, may cause follicular conjunctivitis, they do not cause ATD.
Prostaglandin analogues can be associated with intraocular inflammation, and carbonic anhydrase
inhibitors may affect endothelial function, but neither causes ATD.

7. b. Reduced blink frequency is the major cause of exposure keratopathy in patients with Parkinson
disease. Certain medi cations used in the management of Parkinson disease may also reduce tear
production. Although progressive facial paralysis does occur in patients with Parkinson disease,
cicatricial ectropion is not the mechanism of exposure. Proptosis and upper- eyelid retraction are
associated with thyroid eye disease, not Parkinson disease.

8. c. Floppy eyelid syndrome is characterized by chronic ocular irritation, inflammation, and eyelid laxity.
Upper eyelid eversion occurs with minimal upward force. It is most frequently seen in obese patients
and is associated with obstructive sleep apnea, which can be diagnosed with a sleep study. Floppy eyelid
syndrome is not associated with abnormal results from brain imaging, abnormal results from a buccal
mucosa biopsy, or abnormalities in thyroid function.

9. c. The risk of recurrence after pterygium excision is lower if a conjunctival autograft, rather than an
amniotic membrane transplant, is used. If the defect created following dissection of the pterygium is
considerably larger than the area that can be covered with an autologous conjunctival graft, an amniotic
membrane graft or simple limbal epithelial transplantation (SLET) are viable techniques to cover the
entire area of resection. Although there is evidence that mitomycin C (MMC) applied topically in
conjunction with conjunctival autografting reduces the pterygium recurrence rate after surgical excision,
topical MMC can be toxic and may cause visually significant complications. Bevacizumab has no effect
on the recurrence rate after pterygium excision.

10. b. Retraction is the most common complication of conjunctival flaps, occurring in approximately 10%
of cases. Other, less common complications include hemorrhage beneath the flap and epithelial
inclusion cysts. Ptosis, usually due to levator dehiscence in el derly patients, may also occur
postoperatively and may or may not be related to the flap itself.

11. b. Corneal perforations of 3 mm typically require emergent surgical closure with a corneal patch
graft. Primary closure with nylon sutures is generally not feasible, particularly in eyes with acute
infectious keratitis in which the surrounding tissue is also likely necrotic. Amniotic membrane grafts and
cyanoacrylate glue are best suited for impending perforations or small perforations (≤1 mm). They are
unlikely to provide watertight closure of alarge perforation.
12. c. Congenital syphilis is acquired in utero and caused by infection with the spirochete Treponema
pallidum. In children with untreated congenital syphilis, onset of interstitial keratitis is typically between
6 and 12 years of age and is not pre sent at birth.

13. a. Posterior keratoconus may be a variant of Peters anomaly, but these conditions can be
differentiated by the presence of Descemet membrane, which is absent in Peters anomaly. Peters
anomaly is characterized by the presence, at birth, of a central or paracentral corneal opacity (leukoma),
which is due to the localized absence of the corneal endothelium and Descemet membrane beneath the
area of opacity. Posterior keratoconus is characterized by a typically localized central or paracentral
indentation of the posterior cornea without protrusion of the anterior corneal surface. Keratectasia
differs from congenital anterior staphyloma histologically by the absence of a thin layer of uveal tissue
lining the posterior cornea, which is pre sent in congenital anterior staphyloma.

14. b. Conjunctival concretions are epithelial inclusion cysts filled with epithelial and keratin debris, as
well as mucopolysaccharide and mucin. The majority of conjunctival concretions are asymptomatic. If
they erode the conjunctival epithelium and cause symptoms of per sis tent foreign body irritation, they
can be removed with a fine-gauge needle. Oral azithromycin, warm compresses, and topical ste roids
are not effective treatments for conjunctival concretions.

15. d. Spheroidal degeneration is characterized by the appearance of translucent, golden brown,

spheroidal deposits in the superficial stroma and Bowman layer. Despite its appearance, the brown
deposit is proteinaceous in nature, rather than lipid. In contrast to spheroidal degeneration, lipid
keratopathy most often occurs in association with stromal neovascularization. It can occur in the mid
and deep stroma and is creamy white in color. Glycosaminoglycan deposition is found in multiple
lysosomal storage diseases. Carbohydrate deposits are not associated with this corneal opacification.

16. b. Calcific band keratopathy is a degeneration of the superficial cornea that involves mainly Bowman
layer. The degeneration begins as fine, dustlike, basophilic deposits in at the level of Bowman layer,
usually first seen peripherally in the 3- and 9- o’clock positions. Eventually, the deposits may coalesce to
form a dense horizontal band of calcific plaques across the interpalpebral zone of the cornea. Numerous
systemic diseases, including hyperparathyroidism, chronic renal failure, sarcoidosis, and gout are risk
factors for band keratopathy.

17. d. Salzmann nodular degeneration refers to grayish subepithelial nodules that occur primarily or
secondary to previous inflammatory or mechanical insult to the cornea. These changes are typically
confined to the very superficial anterior stroma and are amenable to debridement with superficial
keratectomy. Deep anterior lamellar keratoplasty (DALK) is unnecessarily invasive for this superficial
condition. Cauterization and cryotherapy are not effective treatments for Salzmann nodular
degeneration.

18. b. Cornea verticillata, or vortex keratopathy, manifests as a whorl- like pattern of golden brown or
gray deposits in the inferior interpalpebral aspect of the cornea. The anti arrhythmic medi cation
amiodarone is the most common cause of corneal verticillata. Because it is unusual for these deposits to
result in reduction of vision or ocular symptoms, treatments such as superficial keratectomy with
excimer laser are not necessary. It is acceptable for the patient to continue taking the recommended
dose of systemic medication. The patient should be monitored by the ophthalmologist.

19. a. Recurrent corneal erosions typically occur in eyes that have suffered prior ocular surface trauma,
usually an abrading injury with shearing force, such as a fingernail injury. Approximately 50% of patients
with recurrent corneal erosions are believed to have underlying epithelial basement membrane
dystrophy (EBMD), although only 10% of patients with EBMD will develop recurrent corneal erosions.
Patterns of corneal epithelial findings evident on slit- lamp examination in eyes with EBMD include
fingerprint lines, maps, dots, and a bleb pattern. Filamentary keratitis is more characteristically
associated with dry eye disease and superior limbic keratoconjunctivitis than with recurrent corneal
erosions. Punctate epithelial erosions are associated with corneal surface dryness from dry eye disease,
but they are not a hallmark of recurrent corneal erosions. Subepithelial infiltrates are most typically
seen in epidemic keratoconjunctivitis.

20. d. Posterior polymorphous corneal dystrophy (PPCD) is characterized by bilateral (can be

asymmetric) vesicular or scalloped areas of abnormal endothelium that demonstrates epithelial
characteristics. The abnormal epithelium typically produces progressive or episodic corneal edema and
may produce peripheral anterior synechiae or iridocorneal adhesions that cause corectopia. Although
iridocorneal endothelial (ICE) syndrome can mimic all of these features, it is characteristically a unilateral
condition. Fuchs endothelial corneal dystrophy (FECD) and congenital hereditary endothelial dystrophy
(CHED) both cause bilateral endothelial dysfunction, but the endothelium in eyes with these dystro
phies does not have the epithelial properties seen in eyes with PPCD.

21. b. The signs and symptoms are typical of the early stages of FECD. The most commonly used
treatments for early FECD are hyperosmotic agents (5% sodium chloride); eyedrops used during the day
and ointment applied at night may reduce edema upon awakening. Another option is a hair dryer used
on the cool setting. Ban dage soft contact lenses may be used to treat painful erosions and ruptured
bullae but are not routinely used to treat early morning edema. In cases of more advanced disease,
surgical options can be considered; the first- line therapy is endothelial keratoplasty in patients without
significant stromal scarring. Penetrating keratoplasty (PK) is reserved for patients with endothelial
failure and significant corneal scarring.

22. a. Patients with keratoconus should be cautioned against eye rubbing because it can contribute to
the progression of keratoconus and increases the risk of hydrops in advanced cases. Smoking, alcohol
use, and reduced omega-3 fatty acid intake do not increase the risk of keratoconus progression.

23. b. Pellucid marginal degeneration is characterized by protrusion above a band of inferior peripheral
thinning in the absence of inflammation. In contrast, in eyes with keratoconus, typically there is inferior
paracentral thinning at the apex of the cone. Mooren ulcer is an inflammatory condition with associated
stromal thinning that starts in the nasal or tem- poral periphery. Terrien degeneration typically begins
with superior peripheral thinning associated with corneal neovascularization that crosses the thinned
area and lipid deposition at the central edge.
24. a. Vitamin A deficiency is associated with the loss of mucus production by goblet cells. This results in
xerosis, abnormal dryness of the conjunctival surface. In impoverished regions of the world, vitamin A
deficiency results from low dietary intake. Gastric bypass surgery can also result in vitamin A deficiency
due to malabsorption. Vitamin A deficiency can also result in reduced night vision. Abnormalities in
keratocytes, lymphocytes, and squamous cells are not associated with the conjunctival lesion or tear
findings.

25. d. Tyrosinemia type II (Richner- Hanhart syndrome) is an autosomal recessive disorder resulting from
defective tyrosine aminotransferase, which leads to excess tyrosine in the blood and urine. Signs and
symptoms include marked photophobia, tearing, conjunctival injection, and tarsal papillary hypertrophy.
Affected patients experience recurrent episodes of corneal erosions and pseudodendrites. Patients with
tyrosinemia type II may show hyperkeratotic lesions of the palms, soles, and elbows, as well as cognitive
impairment. Restriction of dietary intake of tyrosine and phenylalanine can reduce the severity of both
the corneal and systemic changes. High- dose ascorbic acid may reduce arthropathy in patients with
alkaptonuria, not tyrosinemia. Oral and/or topical cysteamine is the treatment for cystinosis, and oral
penicillamine is used to treat Wilson disease.

26. b. Enlarged corneal nerves are associated with multiple endocrine neoplasia type 2B (MEN 2B), a
condition that is also characterized by pheochromocytoma, medullary carcinoma of the thyroid, mucosal
neuromas, and a marfanoid body habitus. Angioid streaks may be seen in a number of conditions,
including pseudoxanthoma elasticum, Ehlers- Danlos syndrome, Paget disease of bone, and sickle cell
disease. Microspherophakia is seen in Weill- Marchesani syndrome, a condition also associated with
short stature, ectopia lentis, and brachydactyly. Preauricular skin tags may be seen in individuals with
Goldenhar Gorlin syndrome, also known as oculoauriculovertebral syndrome.

27. a. Herpes zoster virus infection occurs when dormant varicella- zoster virus in the dorsal root and
cranial nerve (CN) ganglia reactivates. Pain in a sensory nerve distribution may be the first sign of
reactivation. A unilateral vesicular rash that follows a dermatomal distribution is characteristic.
Postherpetic neuralgia may follow resolution. When CN V1 is involved, the condition is called herpes
zoster ophthalmicus (HZO). The current treatment recommendation for all patients with HZO is systemic
antiviral therapy. Topical antiviral medi cations are effective only in the treatment of epithelial mucoid
plaques or more chronic epithelial disease. Topical corticosteroids are indicated for treatment of
keratouveitis. Oral corticosteroids, a controversial treatment aimed at reducing postherpetic neuralgia,
are contraindicated in immunocompromised patients.

28. c. Although trifluridine is effective in treating herpes simplex virus (HSV) epithelial keratitis,
continued use of trifluridine may lead to toxicity of the ocular surface, which can manifest with
symptoms similar to those of the original HSV episode. The most common corneal finding associated
with toxicity is diffuse punctate epithelial erosions. Oral acyclovir has been reported to be as effective as
topical antiviral medi cations for the treatment of epithelial keratitis and does not cause ocular toxicity.
For this reason, oral therapyis preferred by an increasing number of ophthalmologists. Recurrent HSV
keratitis would be unlikely, given that the patient was still on topical antiviral treatment. Superimposed
bacterial conjunctivitis is quite unlikely, particularly in the absence of purulent discharge. Trichiasis,
while pos si ble, is far less likely than toxic keratopathy.

29. a. Although there are 2 vaccines for prevention of shingles, Zostavax and Shingrix, Zostavax is no
longer available in the United States. The recombinant zoster vaccine Shingrix, which has been available
since 2017, is recommended by the CDC as the preferred shingles vaccine, with 2 doses separated by 2
to 6 months for immunocompetent adults aged 50 year and older.

30. a. Acute purulent conjunctivitis in a child can be caused by a number of pathogens. In this case, the
associated subconjunctival hemorrhage and the result of the Gram stain make Haemophilus influenzae
the most likely etiology. Streptococcus pneumoniae can produce a purulent conjunctivitis, but the Gram
stain would demonstrate gram- positive cocci. Moraxella catarrhalis generally causes a chronic, low-
grade conjunctivitis, which may have associated angular blepharitis. Subconjunctival hemorrhages are
not typically pre sent, and the Gram stain would reveal gram- negative diplococci. Neisseria
gonorrhoeae typically pre sents as a hyperacute purulent conjunctivitis in sexually active adults. The
Gram stain would reveal gram- negative intracellular diplococci (GNID).

31. a. Hyperacute conjunctivitis with GNID is diagnostic of gonococcal conjunctivitis caused by Neisseria
gonorrhoeae. If corneal ulceration is pre sent, the patient should be admitted to the hospital to receive
intravenous ceftriaxone. In the absence of corneal involvement, outpatient therapy with intramuscular
ceftriaxone 1 g and close observation is appropriate. Intravenous penicillin G is indicated for treatment
of neurosyphilis. Oral azithromycin is indicated for the treatment of chlamydial conjunctivitis. Although
topical gentamicin ointment can be used as an adjunctive treatment for gonococcal conjunctivitis, the
main stay of treatment is systemic ceftriaxone.

32. a. Early amniotic membrane transplantation (within the first 3–7 days) has been shown to reduce the
formation of symblepharon and minimize long- term ocular surface complications for patients with
conjunctival inflammation from Stevens- Johnson syndrome (SJS). Symblepharon rings and forniceal
debridement have been largely supplanted by amniotic membrane grafting. Repeated forniceal
debridement may even worsen conjunctival scar ring due to mechanical trauma. Temporary
tarsorrhaphy may be indicated for nonhealing epithelial defects, but it is not directly beneficial to
preventing symblepharon formation.

33. c. Bilateral nonulcerative stromal inflammation is suggestive of interstitial keratitis, an inflammatory,

nonulcerative condition that primarily involves the corneal stroma. Herpes simplex and syphilis are the
most common etiologies of interstitial keratitis in the United States. In this case, the negative rapid
plasma reagin (RPR) and fluo rescent treponemal antibody absorption (FTA- ABS) test results and the
vestibuloauditory pathology suggested by the review of systems are consistent with Cogan syndrome, a
rare autoimmune disease often precipitated by a viral illness, with features that overlap with
polyarteritis nodosa. Cogan syndrome can be associated with vertigo and can lead to permanent
deafness if not treated promptly with systemic corticosteroids. A systemic antibiotic would not be
appropriate with a negative syphilis serology. A systemic antiviral medi cation could be considered if
herpes simplex was being considered. A systemic nonsteroidal anti inflammatory drug would not be the
best choice for a patient with Cogan syndrome.

34. b. Rheumatoid arthritis is the condition most frequently associated with peripheral ulcerative
keratitis. Testing for rheumatoid factor (RF) is warranted as part of the systemic evaluation. Systemic
lupus erythematosus, granulomatosis with polyangiitis (Wegener granulomatosis), and ulcerative colitis
are also associated with peripheral ulcerative keratitis but are less commonly pre sent.

35. a. Conjunctival melanoma– related mortality at 10 years ranges from 13% to 38%. Involvement of
the caruncle, palpebral conjunctiva, or conjunctival fornix are all associated with a twofold or greater
risk for mortality than is bulbar conjunctival involvement alone.

36. b. Risk factors associated with ocular surface squamous neoplasia (OSSN) include older age,
ultraviolet light exposure, prior skin cancer, male sex, smoking, fair complexion, human papillomavirus
(HPV) infection, HIV infection, and systemic immunosuppresssion. In a young adult, OSSN should prompt
consideration of a serologic test for HIV.Rapid growth of lesions may occur in a person with AIDS.
Neither herpes simplex virus nor varicella- zoster virus is considered a risk factor for OSSN. Human
herpesvirus 8 is the etiology of Kaposi sarcoma.

37. b. Primary acquired melanosis (PAM) is an acquired, noncystic, flat, patchy or diffuse, tanto brown
pigmentation of the conjunctival epithelium. PAM is usually unilateral or asymmetric if bilateral and is
most often seen in individuals with fair skin. Two clock- hours or less of conjunctival involvement is
associated with a lower risk of malignant transformation; involvement of more than 2 clock- hours is an
indication to remove the lesion for histologic diagnosis.

38. a. Although all these clinical features are pertinent when documenting the extent of ocu- lar surface
injury resulting from chemical trauma, the degree of limbal ischemia is the most impor tant predictor of
progression to limbal stem cell deficiency (which results in corneal neovascularization). Treatments to
improve prognosis include those intended tominimize ongoing exposure to the agent (eg, immediate
irrigation with water or saline,removal of any particulate matter), decrease inflammation (topical
corticosteroids, oral tetracyclines), and promote healing (oral vitamin C, amniotic membrane
transplantation).

39. c. Most eye banks accept corneal tissue from donors 2 to 75 years of age. Tissue from donors
younger than 2 years is typically not used because of the extremely steep curvature and flaccid nature of
the tissue, which makes the tissue difficult to handle. This poses challenges for the surgeon in creating a
watertight closure and achieving a predictable outcome.

40. c. In the United States, the most commonly used storage media used for corneal tissue are Optisol
GS (Bausch + Lomb) and Life4°C (Numedis). Both contain chondroitin sulfate, dextran, gentamycin, and
streptomycin; Life4°C also contains insulin. Tissue is stored at 2° to 8°C and can remain viable for up to
14 days. In Eu rope, donor corneal tissue is incubated in organ culture storage that allows tissue to be
stored for up to 35 days, but this requires a culture at the end of storage to confirm sterility, which can
be complex, expensive, and labor intensive.
41. c. Stromal rejection, characterized by significant haze and deep vascularization, can lead to corneal
opacification and is more common after DALK than PK. In DALK, the corneal endothelium is not
transplanted; therefore, endothelial rejection cannot take place. Epithelial rejection and subepithelial
infiltrates tend to be less visually significant and are possible in both DALK and PK.