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CORNEA/EXTERNAL DISEASE SUMMARY BENCHMARKS FOR

PREFERRED PRACTICE PATTERN® GUIDELINES

Introduction Cochrane Library for articles in the English language


These are summary benchmarks for the Academy’s is conducted. The results are reviewed by an expert
Preferred Practice Pattern® (PPP) guidelines. The panel and used to prepare the recommendations,
Preferred Practice Pattern series of guidelines has which are then given a rating that shows the strength
been written on the basis of three principles. of evidence when sufficient evidence exists.
• Each Preferred Practice Pattern should be clinically To rate individual studies, a scale based on the
relevant and specific enough to provide useful Scottish Intercollegiate Guideline Network (SIGN) is
information to practitioners. used. The definitions and levels of evidence to rate
• Each recommendation that is made should be given individual studies are as follows:
an explicit rating that shows its importance to the • I++: High-quality meta-analyses, systematic reviews
care process. of randomized controlled trials (RCTs), or RCTs with
• Each recommendation should also be given an a very low risk of bias
explicit rating that shows the strength of evidence • I+: Well-conducted meta-analyses, systematic
that supports the recommendation and reflects the reviews of RCTs, or RCTs with a low risk of bias
best evidence available.
• I–: Meta-analyses, systematic reviews of RCTs, or
Preferred Practice Patterns provide guidance RCTs with a high risk of bias
for the pattern of practice, not for the care of a • II++: High-quality systematic reviews of case-control
particular individual. While they should generally or cohort studies; high-quality case-control or
meet the needs of most patients, they cannot possibly cohort studies with a very low risk of confounding
best meet the needs of all patients. Adherence to or bias and a high probability that the relationship is
these Preferred Practice Patterns will not ensure a causal
successful outcome in every situation. These practice • II+: Well-conducted case-control or cohort studies
patterns should not be deemed inclusive of all proper with a low risk of confounding or bias and a
methods of care or exclusive of other methods of moderate probability that the relationship is causal
care reasonably directed at obtaining the best results.
It may be necessary to approach different patients’ • II–: Case-control or cohort studies with a high risk of
needs in different ways. The physician must make the confounding or bias and a significant risk that the
ultimate judgment about the propriety of the care of relationship is not causal
a particular patient in light of all of the circumstances • III: Nonanalytic studies (e.g., case reports, case
presented by that patient. The American Academy series)
of Ophthalmology is available to assist members in
resolving ethical dilemmas that arise in the course of Recommendations for care are formed based on the
ophthalmic practice. body of the evidence. The body of evidence quality
ratings are defined by Grading of Recommendations
The Preferred Practice Pattern® guidelines are not Assessment, Development and Evaluation (GRADE)
medical standards to be adhered to in all individual as follows:
situations. The Academy specifically disclaims any • Good quality (GQ): Further research is very unlikely
and all liability for injury or other damages of any kind, to change our confidence in the estimate of effect
from negligence or otherwise, for any and all claims
that may arise out of the use of any recommendations • Moderate quality (MQ): Further research is likely to
or other information contained herein. have an important impact on our confidence in the
estimate of effect and may change the estimate
For each major disease condition, recommendations • Insufficient quality (IQ): Further research is
for the process of care, including the history, physical very likely to have an important impact on our
exam and ancillary tests, are summarized, along with confidence in the estimate of effect and is likely to
major recommendations for the care management, change the estimate; any estimate of effect is very
follow-up, and education of the patient. For each uncertain
PPP, a detailed literature search of PubMed and the

© 2017 American Academy of Ophthalmology November 2017 aao.org 1


CORNEA/EXTERNAL DISEASE SUMMARY BENCHMARKS FOR
PREFERRED PRACTICE PATTERN® GUIDELINES

Introduction (continued) • Level I includes evidence obtained from at least


Key recommendations for care are defined by GRADE one properly conducted, well-designed randomized
as follows: controlled trial. It could include meta-analyses of
randomized controlled trials.
• Strong recommendation (SR): Used when the
desirable effects of an intervention clearly outweigh • Level II includes evidence obtained from the following:
the undesirable effects or clearly do not •W
 ell-designed controlled trials without
• Discretionary recommendation (DR): Used when the randomization
trade-offs are less certain—either because of low- •W
 ell-designed cohort or case-control analytic
quality evidence or because evidence suggests studies, preferably from more than one center
that desirable and undesirable effects are closely •M
 ultiple-time series with or without the
balanced intervention

In PPPs prior to 2011, the panel rated recommendations • Level III includes evidence obtained from one of the
according to its importance to the care process. This following:
“importance to the care process” rating represents • Descriptive studies
care that the panel thought would improve the quality • Case reports
of the patient’s care in a meaningful way. The ratings
•R
 eports of expert committees/organizations (e.g.,
of importance are divided into three levels.
PPP panel consensus with external peer review)
• Level A, defined as most important
• Level B, defined as moderately important This former approach, however, will eventually be
• Level C, defined as relevant but not critical phased out as the AAO adopted the SIGN and
GRADE rating and grading systems.
The panel also rated each recommendation on the
strength of evidence in the available literature to The PPPs are intended to serve as guides in patient
support the recommendation made. The “ratings of care, with greatest emphasis on technical aspects. In
strength of evidence” also are divided into three levels. applying this knowledge, it is essential to recognize
that true medical excellence is achieved only when
skills are applied in a such a manner that the patients’
needs are the foremost consideration. The AAO
is available to assist members in resolving ethical
dilemmas that arise in the course of practice. (AAO
Code of Ethics)

© 2017 American Academy of Ophthalmology November 2017 aao.org 2


CORNEA
Bacterial Keratitis (Initial Evaluation)

Initial Exam History • Indications for smears and cultures:


• Ocular symptoms (e.g., degree of pain, redness, - Sight-threatening or severe keratitis of suspected
discharge, blurred vision, photophobia, duration of micro bial origin prior to initiating therapy. (III, IQ,
symptoms, circumstances surrounding the onset of DR)
symptoms) (III, GQ, SR) - A large central corneal infiltrate that extends to
• Contact lens history (e.g., wearing schedule, the middle to deep stroma. (III, IQ, DR)
overnight wear, type of contact lenses, contact lens - Chronic in nature. (III, IQ, DR)
solution, contact lens hygiene protocol, tap-water
rinse of contact lenses, swimming, using a hot tub, - Unresponsive to broad spectrum antibiotic
or showering while wearing contact lenses) (II+, GQ, therapy. (III, IQ, DR)
SR) - Clinical features suggestive of fungal, amœbic, or
• Review of other ocular history, including risk factors mycobacterial keratitis. (III, IQ, DR)
such as herpes simplex virus keratitis, varicella • The hypopyon that occurs in eyes with bacterial
zoster virus keratitis, previous bacterial keratitis, keratitis is usually sterile, and aqueous or vitreous
trauma, dry eye, and previous ocular surgery, taps should not be performed unless there is a high
including refractive surgery (III, GQ, SR) suspicion of microbial endophthalmitis. (III, IQ, DR)
• Review of other medical problems (III, GQ, SR) • Corneal scrapings for culture should be inoculated
• Current and recently used ocular medications (III, GQ, directly onto appropriate culture media to maximize
SR)
culture yield. (III, IQ, DR) If this is not feasible, place
specimens in transport media. (II+, MQ, DR) In
• Medication allergies (III, GQ, SR) either case, immediately incubate cultures or take
promptly to the laboratory. (III, GQ, SR)
Initial Physical Exam
• Visual acuity (III, GQ, SR) Care Management
• General appearance of patient, including skin • Topical antibiotic eye drops are preferred method in
conditions (III, GQ, SR) most cases. (III, GQ, SR)
• Facial examination (III, GQ, SR) • Use topical broad-spectrum antibiotics initially in
• Globe position (III, GQ, SR) the empiric treatment of presumed bacterial
keratitis. (III, IQ, DR)
• Eyelids and eyelid closure (III, GQ, SR)
• For central or severe keratitis (e.g., deep stromal
• Conjunctiva (III, GQ, SR) involvement or an infiltrate larger than 2 mm with
• Nasolacrimal apparatus (III, GQ, SR) extensive suppuration), use a loading dose (e.g.,
• Corneal sensation (III, GQ, SR) every 5 to 15 minutes for the first 30 to 60 minutes),
followed by frequent applications (e.g., every 30
• Slit-lamp biomicroscopy (III, GQ, SR) minutes to 1 hour around the clock). (III, IQ, DR) For
- Eyelid margins (III, GQ, SR) less severe keratitis, a regimen with less frequent
- Conjunctiva (III, GQ, SR) dosing is a
­ ppropriate. (III, IQ, DR)
- Sclera (III, GQ, SR) • Use systemic therapy for gonococcal keratitis. (III, IQ,
DR)
- Cornea (III, GQ, SR)
• For patients treated with ocular topical
- Anterior chamber for depth and the presence of
corticosteroids at time of presentation of suspected
inflammation, including cell and flare, hypopyon,
bacterial keratitis, reduce or eliminate corticosteroids
fibrin, hyphema (III, GQ, SR)
until infection has been controlled. (III, GQ, SR)
- Anterior vitreous (III, GQ, SR)
• When the corneal infiltrate compromises the
- Contralateral eye for clues to etiology as well as visual axis, may add topical corticosteroid therapy
possible similar underlying pathology (III, GQ, SR) following at least 2 to 3 days of progressive
improvement with treatment with topical antibiotics.
Diagnostic Tests (III, IQ, DR) Continue topical antibiotics at high levels
• Manage majority of community-acquired cases with gradual tapering. (III, IQ, DR)
with empiric therapy and without smears or • Examine patients within 1 to 2 days after initiation of
cultures. (III, IQ, DR) topical corticosteroid therapy. (III, IQ, DR)

© 2017 American Academy of Ophthalmology November 2017 aao.org 3


CORNEA
Bacterial Keratitis (Management Recommendations)

Patient Education •E
 ducate patients with contact lenses about
• Inform patients with risk factors predisposing them increased risk of infection associated with contact
to bacterial keratitis of their relative risk, the signs lens, overnight wear, and importance of adherence
and symptoms of infection, and to consult an to techniques to promote contact lens hygiene (II+,
ophthalmologist promptly if they experience such GQ, SR)
warning signs or symptoms (III, GQ, SR) •R
 efer patients with significant visual impairment
• Educate about the destructive nature of bacterial or blindness for vision rehabilitation if they are not
keratitis and need for strict compliance with therapy surgi­cal candidates (see www.aao.org/smart-sight-
(III, GQ, SR) low-vision)
• Discuss possibility of permanent visual loss and need
for future visual rehabilitation (III, GQ, SR)

Antibiotic Therapy of Bacterial Keratitis

Topical Subconjunctival
Organism Antibiotic Concentration Dose
No organism Cefazolin 50 mg/ml 100 mg in 0.5 ml
identified or with
multiple types Tobramycin or gentamicin 9–14 mg/ml 20 mg in 0.5 ml
of organisms or
Fluoroquinolones* Various†
Gram-positive Cefazolin 50 mg/ml 100 mg in 0.5 ml
Cocci Vancomycin‡ 15–50 mg/ml 25 mg in 0.5 ml
Bacitracin‡ 10,000 IU
Fluoroquinolones* Various†
Gram-negative Tobramycin or gentamicin 9–14 mg/ml 20 mg in 0.5 ml
Rods Ceftazidime 50 mg/ml 100 mg in 0.5 ml
Fluoroquinolones Various†
Gram-negative Ceftriaxone 50 mg/ml 100 mg in 0.5 ml
Cocci§ Ceftazidime 50 mg/ml 100 mg in 0.5 ml
Fluoroquinolones Various†
Nontuberculous Amikacin 20–40 mg/ml 20 mg in 0.5 ml
Mycobacteria Clarithromycin 10 mg/ml
Azithromycin|| 10 mg/ml
Fluoroquinolones Various†
Nocardia Sulfacetamide 100 mg/ml
Amikacin 20–40 mg/ml 20 mg in 0.5 ml
Trimethoprim/
Sulfamethoxazole:
Trimethoprim 16 mg/ml
Sulfamethoxazole 80mg/ml
* Fewer gram-positive cocci are resistant to gatifloxacin and moxifloxacin than other fluoroquinolones.
†B
 esifloxacin 6mg/ml; ciprofloxacin 3 mg/ml; gatifloxacin 3 mg/ml; levofloxacin 15 mg/ml; moxifloxacin 5 mg/ml; ofloxacin 3 mg/ml, all commercially available at
these concentrations
‡ For resistant Enterococcus and Staphylococcus species and penicillin allergy. Vancomycin and bacitracin have no gram-negative activity and should not be used as
a single agent in empirically treating bacterial keratitis.
§ Systemic therapy is necessary for suspected gonococcal infection.
|| Data from Chandra NS, Torres MF, Winthrop KL. Cluster of Mycobacterium chelonae keratitis cases following laser in-situ keratomileusis. Am J Ophthalmol 2001;
132:819–30.

© 2017 American Academy of Ophthalmology November 2017 aao.org 4


CORNEA
Blepharitis (Initial and Follow-up Evaluation)

Initial Exam History Diagnostic Tests


• Ocular symptoms and signs (e.g., redness, irritation, • Cultures may be indicated for patients with
burning, tearing, itching, crusting of eyelashes, eyelid recurrent anterior blepharitis with severe
sticking, contact lens intolerance, photophobia, inflammation as well as for patients who are not
increased frequency of blinking) (III, GQ, SR) responding to therapy. (III, IQ, DR)
• Time of day when symptoms are worse • Biopsy of the eyelid to exclude the possibility of
carcinoma may be indicated in cases of marked
• Duration of symptoms asymmetry, resistance to therapy or unifocal
• Unilateral or bilateral presentation recurrent chalazia that do not respond well to
• Exacerbating conditions (e.g., smoke, allergens, therapy. (III, IQ, DR)
wind, contact lenses, low humidity, retinoids, diet • Consult with the pathologist prior to obtaining the
and alcohol consumption, eye makeup) biopsy if sebaceous cell carcinoma is suspected.
• Symptoms related to systemic diseases (e.g., (III, GQ, SR)
rosacea, allergy) (III, IQ, DR)
Care Management
• Current and previous systemic and topical
medications (e.g., antihistamines or drugs with • Treat patients with blepharitis initially with a
anticholinergic effects, or drugs used in the past regimen of warm compresses and eyelid hygiene. (III,
that might have an effect on the ocular surface [e.g., IQ, DR)
isotretinoin]) (III, GQ, SR) • A topical antibiotic such as bacitracin or
• Recent exposure to an infected individual (e.g., erythromycin can be prescribed to be applied one
pediculosis palpebrarum [Pthirus pubis]) or more times daily or at bedtime on the eyelids for
one or more weeks. (III, IQ, DR)
• Ocular history (e.g., previous intraocular and eyelid
surgery, local trauma, including mechanical, thermal, • For patients with meibomian gland dysfunction,
chemical, and radiation injury, history of cosmetic whose chronic symptoms and signs are not
blepharoplasty, history of styes and/or chalazia) adequately controlled with eyelid hygiene, oral
(III, GQ, SR) tetracyclines and topical antibiotics can be
prescribed. (I–, MQ, DR)
Initial Physical Exam • A brief course of topical corticosteroids may be
• Visual acuity (III, GQ, SR) helpful for eyelid or ocular surface inflammation. The
minimal effective dose of corticosteroid should be
• External examination utilized and long-term corticosteroid therapy should
- Skin (III, GQ, SR) be avoided if possible. (III, GQ, SR)
- Eyelids (III, GQ, SR)
Follow-Up Evaluation
• Slit-lamp biomicroscopy
• Follow-up visits should include:
- Tear film (III, GQ, SR)
- Interval history (III, GQ, SR)
- Anterior eyelid margin (III, GQ, SR)
- Measurement of visual acuity (III, GQ, SR)
- Eyelashes (III, GQ, SR)
- External examination (III, GQ, SR)
- Posterior eyelid margin (III, GQ, SR)
- Slit-lamp biomicroscopy (III, GQ, SR)
- Tarsal conjunctiva (everting eyelids) (III, GQ, SR)
• If corticosteroid therapy is prescribed, re-evaluate
- Bulbar conjunctiva (III, GQ, SR)
patient within a few weeks to determine the
- Cornea (III, GQ, SR) response to therapy, measure intraocular pressure,
and assess treatment compliance (III, GQ, SR)

Patient Education
• Counsel patients about the chronicity and
recurrence of the disease process. (III, GQ, SR)
• Inform patients that symptoms can frequently be
improved but are rarely eliminated. (III, GQ, SR)
• Patients with an inflammatory eyelid lesion that
appears suspicious for malignancy should be referred
to an appropriate specialist. (III, GQ, SR)

© 2017 American Academy of Ophthalmology November 2017 aao.org 5


CORNEA
Conjunctivitis (Initial Evaluation)

Initial Exam History • Slit-lamp biomicroscopy (III, IQ, DR)


• Ocular symptoms and signs (e.g., itching, discharge, - Eyelid margins (inflammation, ulceration,
­irritation, pain, photophobia, blurred vision) discharge, nodules or vesicles, blood-tinged
• Duration of symptoms and time course debris, ­keratinization) (III, IQ, DR)
• Exacerbating factors - Eyelashes (loss of lashes, crusting, scurf, nits, lice,
trichiasis) (III, IQ, DR)
• Unilateral or bilateral presentation
- Lacrimal puncta and canaliculi (pouting,
• Character of discharge discharge) (III, IQ, DR)
• Recent exposure to an infected individual - Tarsal and forniceal conjunctiva (III, IQ, DR)
• Trauma (mechanical, chemical, ultraviolet) - Bulbar conjunctiva/limbus (follicles, edema,
• Mucus fishing nodules, chemosis, laxity, papillae, ulceration,
• Contact lens wear (lens type, hygiene and use scarring, phlyctenules, hemorrhages, foreign
regimen) material, keratinization) (III, IQ, DR)
• Symptoms and signs potentially related to systemic - Cornea (III, IQ, DR)
diseases (e.g., genitourinary discharge, dysuria, - Anterior chamber/iris (inflammation reaction,
dysphagia, upper respiratory infection, skin and ­synechiae, transillumination defects) (III, IQ, DR)
mucosal lesions) - Dye-staining pattern (conjunctiva and cornea)
• Allergy, asthma, eczema (III, IQ, DR)
• Use of topical and systemic medications
Diagnostic Tests
• Ocular history (e.g., previous episodes of
conjunctivitis and previous ophthalmic surgery) • Cultures, smears for cytology and special stains are
­indicated in cases of suspected infectious neonatal
• Compromised immune status
­conjunctivitis. (II–, IQ, DR)
• Current and prior systemic diseases
• Smears for cytology and special stains are
• Social history (e.g., smoking, occupation and recommended in cases of suspected gonococcal
hobbies, travel and sexual activity) conjunctivitis. (II–, IQ, DR)
• Confirm diagnosis of adult and neonate chlamydial
Initial Physical Exam conjunctivitis with immunodiagnostic test and/or
• Visual acuity (III, IQ, DR) culture.
• External examination (III, IQ, DR) • Biopsy the bulbur conjunctiva and take a sample
- Skin (signs of rosacea, eczema, seborrhea) (III, IQ, from an uninvolved area adjacent to the limbus in an
DR) eye with active inflammation when ocular mucous
membrane pemphigoid is suspected. (II–, IQ, DR)
- Abnormalities of the eyelids and adnexae
(swelling, discoloration, malposition, laxity, • A full-thickness lid biopsy is indicated in cases of
ulceration, nodules, ecchymosis, neoplasia) (III, IQ, suspected sebaceous carcinoma. (III, IQ, DR)
DR) • Confocal microscopy may be helpful to evaluate
- Conjunctiva (pattern of injection, subconjunctival some forms of conjunctivitis (e.g., atopic, SLK). (II–,
hemorrhage, chemosis, cicatricial change, MQ, DR)
symbleph­aron, masses, discharge) (III, IQ, DR) • Thyroid function tests are indicated for patients with
SLK who do not have known thyroid disease. (III, IQ,
DR)

© 2017 American Academy of Ophthalmology November 2017 aao.org 6


CORNEA
Conjunctivitis (Management Recommendations)

Care Management Follow-Up Evaluation


• Avoid indiscriminate use of topical antibiotics or • Follow-up visits should include
cor­ticosteroids because antibiotics can induce - Interval history (III, IQ, DR)
toxicity and corticosteroids can potentially prolong
adenoviral infections and worsen herpes simplex - Visual acuity (III, IQ, DR)
virus infections (III, GQ, SR) - Slit-lamp biomicroscopy (III, IQ, DR)

• Treat mild allergic conjunctivitis with an over-the- • If corticosteroids are used, perform periodic
counter antihistamine/vasoconstrictor agent or measurement of intraocular pressure and pupillary
second-generation topical histamine H1-receptor dilation to evaluate for cataract and glaucoma (III, IQ,
antagonists. If the condition is frequently recurrent DR)
or persistent, use mast-cell stabilizers (I++, GQ, SR)
• For contact lens-related keratoconjunctivitis, Patient Education
discontinue contact lens wear for 2 or more weeks • Counsel patients with contagious varieties to mini-
(III, IQ, DR) mize or prevent spread of diseases in the
• If corticosteroids are indicated, prescribe the lowest community (III, IQ, DR)
potency and frequency based on patient response • Inform patients who may require repeat short-term
and tolerance (III, IQ, DR) therapy with topical corticosteroid of potential
• If corticosteroids are used, perform baseline and complications of corticosteroid use
periodic measurement of intraocular pressure and • Advise patients with allergic conjunctivitis that
pupillary dilation (III, IQ, DR) frequent clothes washing and bathing/showering
• Use systemic antibiotic treatment for conjunctivitis before bedtime may be helpful (III, IQ, DR)
due to Neisseria gonorrhoeae or Chlamydia
trachomatis (III, IQ, DR)
• Treat sexual partners to minimize recurrence and
spread of disease when conjunctivitis is associated
with sexually transmitted diseases and refer patients
and their sexual partners to an appropiate medical
specialist (III, GQ, SR)
• Refer patients with manifestation of a systemic
disease to an appropriate medical specialist (III, GQ,
SR)

© 2017 American Academy of Ophthalmology November 2017 aao.org 7


CORNEA
Corneal Ectasia (Initial and Follow-up Evaluation)

Initial Exam History •C


 ollagen crosslinking can improve corneal rigidity by
• Disease onset and course increasing bonds between fibers.
• Vision impairment •L
 amellar keratoplasty using DALK techniques
can be considered for progressive keratoconus
• Ocular, medical, and family history without significant scarring or hydrops. (II++, MQ, DR)
Crescentic lamellar keratoplasty is an option when
Initial Physical Exam maximal thinning is in the cornea’s periphery. (III, IQ,
• Visual function assessment DR)
• External examination •P
 eripheral thinning and ectasia can be managed
- Corneal protrusion by a standard decentered lamellar procedure for
tectonic support, followed by a central penetrating
- Eyelids and periorbital skin keratoplasty later. (III, IQ, DR)
• Slit-lamp biomicroscopy •P
 enetrating keratoplasty is indicated when a
- Presence, extent, and location of the corneal patient can no longer achieve functional vision with
thinning or protrusion eyeglasses or contact lenses, or when persistent
- Indication of previous ocular surgery corneal edema occurs following hydrops. (III, IQ, DR)
Descemet stripping endothelial keratoplasty cannot
- Presence of Vogt striae, prominent corneal nerves, correct ectatic disorder. (III, IQ, DR)
Fleischer ring, or other iron deposition
•P
 enetrating keratoplasty is preferred over DALK in
- Evidence of corneal scarring or previous hydrops, cases of deep stromal scarring. (III, IQ, DR)
and presence of prominent corneal nerves
•A
 lamellar graft can be performed for tectonic
• IOP measurement (III, IQ, DR) support when ectasia occurs in the far periphery of
• Fundus examination: assessment of red reflex for the cornea. (III, IQ, DR)
dark area, and retina for tapetoretinal degenerations
(III, IQ, DR) Follow-Up Evaluation
•F
 ollow-up evaluation and visit intervals are dictated
Diagnostic Tests by treatment and disease progression. (III, IQ, DR)
• Keratometry (II+, MQ, DR) •A
 nnual follow up is recommended for cases of
• Corneal topography (II–, MQ, SR) ectasia unless the patient has significant changes in
• Topographic power map visual function. (III, IQ, DR)
• Topographic elevation map (II+, MQ, DR) •P
 atients should be made aware of the warning
signs of rejection and should seek medical
• Corneal pachymetry (II++, GQ, SR)
attention promptly if symptoms occur. (III, GQ, SR)
The practitioner should be aware of the slit-lamp
Care Management biomicroscopic findings of epithelial, stromal, and
• Therapy is tailored to the individual patient, endothelial rejection. (III, GQ, SR)
depending on the visual impairment and treatment
option(s). Counseling and Referral
• Vision can be corrected with eyeglasses, but contact •W
 hen medical therapy with eyeglasses and/or
lenses may be required as keratoconus progresses. contact lenses cannot improve visual function, a
• Rigid corneal gas permeable contact lenses can referral to an ophthalmologist trained in surgical
mask corneal irregularities. New hybrid contact treatments for corneal ectasia is indicated (III, GQ, SR)
lenses provide higher oxygen permeability and •P
 atients with a history of allergy and atopy may
greater RGP/hydrogel junction strength. Piggyback require a referral to a dermatologist or allergist (III,
contact lenses may be employed in cases of corneal GQ, SR)
scaring or decentered cones. Scleral lenses may be
•P
 atients with floppy eyelid disease may be best
indicated when RGP and/or hybrid contact lenses
managed by an oculoplastics specialist and referrals
fail.
to other medical specialists may also be needed (III,
• Intrastromal corneal ring segment implantation GQ, SR)
can improve contact lens tolerance and BCVA for
patients with corneal ectasia, a clear cornea, and
contact lens intolerance. (II–, MQ, DR)

© 2017 American Academy of Ophthalmology November 2017 aao.org 8


CORNEA
Corneal Edema and Opacification (Initial Evaluation)

Initial Exam History Diagnostic Tests


• Symptoms: blurred or variable vision; photophobia; • Potential acuity meter
redness; tearing; intermittent foreign body • Rigid contact lens overrefraction
sensation; pain
• Pachymetry (III, IQ, DR)
• Age of onset
• Scheimpflug imaging
• Rapidity of onset
• Specular and confocal microscopy (III, IQ, DR)
• Persistence
• Anterior segment optical coherence tomography
• Unilateral or bilateral presentation (III, IQ, DR)
• Moderating factors, like visual improvement related • Ultrasound biomicroscopy
to environmental factors
• Past ocular and medical history
• Topical and systemic medications
• Trauma
• Contact lens wear
• Family and social history

Initial Physical Exam


• Visual function assessment
• External examination
- Evidence of proptosis, ptosis, lagophthalmos, or
floppy eyelid syndrome
- Eyelid or facial asymmetry, scarring, and
malfunction
• Slit-lamp biomicroscopy (III, IQ, DR)
- Unilateral or bilateral signs
- Diffuse or localized edema
- Primarily epithelial or stromal edema
- Evidence of epithelial breakdown, stromal
infiltration, epithelial ingrowth, striae, focal
thickening, thinning, scarring, interface haze, striae
or inflammation, or stromal vascularization
- Evidence of guttae, Descemet’s membrane
tear or detachment, endothelial vesicles, keratic
precipitates (KP), pigment peripheral anterior
synechiae
- Involvement of host or donor tissue
- Evidence of sectoral corneal edema and KPs, or
an anterior chamber reaction
- Status, shape, and position of the pupil and iris
- Evidence of vitreous strands or pigment dusting
- Status and position of the lens
• IOP measurement
• Fundus examination
• Gonioscopy

© 2017 American Academy of Ophthalmology November 2017 aao.org 9


CORNEA
Corneal Edema and Opacification (Management
Recommendations)

Care Management • Corneal opacification: surgical management


• Therapeutic goal is to control the cause of corneal - Surgical strategy for managing corneal opacities
edema or opacity and enhance a patient’s quality of depends on the tissue layer(s) involved:
life by improving visual acuity and comfort ° E
 pithelial debridement is most helpful with
• Treatment starts with medical management, but lesions anterior to Bowman’s layer (III, IQ, DR)
surgery may be ultimately required ° E
 thylenediaminetetraacetic acid (EDTA) may be
• Corneal edema: medical management used to remove calcific band keratopathy (III, IQ,
- Lowering an elevated IOP is helpful DR)

- Topical carbonic anhydrase inhibitors should ° M


 itomycin-C for subepithelial, Bowman’s layer,
not be the first line of therapy when endothelial and anterior stromal scarring may help in cases
dysfunction is suspected (II–, MQ, SR) of possible recurrence (III, IQ, DR)
- Topical corticosteroid can control inflammation ° C
 orneal tattooing can mask cosmetically
once infection has been ruled out (III, GQ, SR) objectionable corneal leukomas
- Microcystic or bullous epithelial disease may ° A
 nterior corneal lesions, extending beyond
produce discomfort or pain necessitating the Bowman’s layer into the anterior and mid-
placement of a bandage contact. (III, GQ, SR) stroma, require more extensive treatment,
Periodic lens exchange is advised for longer-term such as superficial keratectomy, lamellar or
use. (III, IQ, DR) penetrating keratoplasty, and keratoprosthesis (III,
GQ, SR)
• Corneal edema: surgical management
- Patients with corneal edema and persistent Follow-Up Evaluation
discomfort, but limited or no visual potential,
are generally better candidates for the following • In the management of corneal edema, follow up is
procedures: essential to monitor endothelial dysfunction
° Phototherapeutic keratectomy (III, IQ, DR) • In the management of corneal opacification, follow
up to monitor corneal clarity and surface irregularity
° Conjunctival flap of Gunderson (III, IQ, DR) is necessary (III, GQ, SR)
° Corneal transplantation •C
 oexisting problems, particularly intraocular
° Endothelial keratoplasty inflammation and IOP, need regular reassessment (III,
° Penetrating keratoplasty (III, GQ, SR) GQ, SR)

• Corneal opacification: medical management


Counseling and Referral
- Corneal opacity treatment can be divided into
two phases: a) management of the principal, •D
 etailed discussion of the causes of edema or
initiating process (i.e., infection, trauma), and opacity, and various treatment options, is important.
b) management of the resulting problems (i.e., (III, GQ, SR)
surface erosions and irregularity, scarring, thinning, •R
 eferral to a corneal subspecialist is recommended
and vascularization) when sophisticated diagnostic or medical
- Conventional treatment involves an antibiotic drop management approaches are required (i.e., in cases
or ointment to protect against secondary bacterial exceeding the training of the treating physician).
infection (III, IQ, DR) (III, GQ, SR) Referrals to retina, glaucoma, or pediatric
ophthalmic subspecialists may also be needed. (III,
- Temporary glue, suture tarsorraphy, or lid splints
GQ, SR) Once the condition has been resolved, or
can be helpful when blinking or lid closure is
has stabilized, referral back to the comprehensive
inadequate (III, IQ, DR)
ophthalmologist is appropriate. (III, GQ, SR)
- A bandage contact lens may be useful in cases of
•W
 hen the disease process or management is
delayed healing (III, GQ, SR)
complex, every effort should be made to counsel
- A rigid gas permeable lens — or hybrid or scleral the patient regarding such challenges to allow for
lens when greater stability is needed — will often appropriate expectations and informed decision-
improve vision when surface irregularity is a making. (III, GQ, SR)
factor; such lenses may preclude the need for
more invasive procedures (III, IQ, DR)

© 2017 American Academy of Ophthalmology November 2017 aao.org 10


CORNEA
Dry Eye Syndrome (Initial Evaluation)

Initial Exam History - Nonocular surgery (e.g., bone marrow transplant,


• Ocular symptoms and signs (e.g., irritation, tearing, head and neck surgery, trigeminal neuralgia
burning, stinging, dry or foreign body sensation, surgery) (II++, GQ, SR)
mild itching, photophobia, blurry vision, contact - Radiation of orbit (II++, GQ, SR)
lens intolerance, redness, mucous discharge, - Neurological conditions (e.g., Parkinson disease,
increased frequency of blinking, eye fatigue, diurnal Bell palsy, Riley-Day syndrome, trigeminal neural-
fluctuation, symptoms that worsen later in the day) gia) (II++, GQ, SR)
(III, GQ, SR) - Dry mouth, dental cavities, oral ulcers (II++, GQ, SR)
- Fatigue (II++, GQ, SR)
• Exacerbating conditions (e.g., wind, air travel, - Joint pain, muscle aches (II++, GQ, SR)
decreased humidity, prolonged visual efforts
associated with decreased blink rate such as reading
Initial Physical Exam
and computer use) (III, GQ, SR)
• Visual acuity
• Duration of symptoms (III, GQ, SR)
• External examination
• Ocular history, including
- Skin (e.g., scleroderma, facial changes consistent
- Topical medications used and their effect on
with rosacea, seborrhea)
symptoms (e.g., artificial tears, “eyewash,”
- Eyelids (incomplete closure/malposition,
antihistamines, glaucoma medications,
incomplete or infrequent blink, eyelid lag,
vasoconstrictors, corticosteroids, homeopathic or
erythema of eyelid margins, abnormal deposits or
herbal preparations) (III, GQ, SR)
secretions, entropion, ectropion)
- Contact lens wear, schedule and care (III, GQ, SR)
- Adnexa (enlargement of the lacrimal glands)
- Allergic conjunctivitis (III, GQ, SR)
- Proptosis
- Ocular surgical history (e.g., prior keratoplasty,
- Cranial nerve function (e.g., cranial nerve V
cataract surgery, keratorefractive surgery) (III, GQ,
[trigeminal], cranial nerve VII [facial])
SR)
- Hands (joint deformities characteristic of
- Ocular surface disease (e.g., herpes simplex virus,
rheumatoid arthritis, Raynaud phenomenon,
varicella zoster virus, ocular mucous membrane
splinter hemorrhage underneath nails)
pemphigoid, Stevens-Johnson syndrome, aniridia,
graft-versus-host disease) (III, GQ, SR) • Slit-lamp biomicroscopy
- Punctal surgery (III, GQ, SR) - Tear film (height of the meniscus, debris, increased
- Eyelid surgery (e.g. prior ptosis repair, viscosity, mucus strands, and foam break-up time
blepharoplasty, entropion/ectropion repair) (III, GQ, and pattern)
SR) - Eyelashes (trichiasis, distichiasis, madarosis,
- Bell palsy (III, GQ, SR) deposits)
- Anterior and posterior eyelid margins
• Medical history, including
(abnormalities of meibomian glands [e.g., orifice
- Smoking or exposure to second-hand smoke
metaplasia, reduced expressible meibum, atrophy],
(II+, GQ, SR)
character of meibomian gland secretions [e.g.,
- Dermatological diseases (e.g., rosacea, psoriasis)
turbid, thickened, foamy, deficient], vascularization
(II++, GQ, SR)
crossing the mucocutaneous junction,
- Technique and frequency of facial washing
keratinization, scarring
including eyelid and eyelash hygiene (II++, GQ, SR)
- Puncta (patency, position, presence, and position
- Atopy (II++, GQ, SR)
of plugs)
- Menopause (II++, GQ, SR)
- Inferior fornix and tarsal conjunctiva (e.g., mucous
- Systemic inflammatory diseases (e.g., Sjögren
threads, scarring, erythema, papillary reaction,
syndrome, graft-versus-host disease, rheumatoid
follicle enlargement, keratinization, foreshortening,
arthritis, systemic lupus erythematosus,
symblepharon)
scleroderma) (II++, GQ, SR)
- Bulbar conjunctiva (e.g., punctate staining with
- Other systemic conditions (e.g., lymphoma,
rose bengal, lissamine green, or fluorescein
sarcoidosis) (II++, GQ, SR)
dyes; hyperemia; localized drying; keratinization,
- Systemic medications (e.g., antihistamines,
chemosis, chalosis, follicles)
diuretics, hormones and hormonal antagonists,
- Cornea (localized interpalpebral drying, punctate
antidepressants, cardiac antiarrhythmic drugs,
epithelial erosions, punctate staining with rose
isotretinoin, diphenoxylate/atropine, beta-
bengal or fluorescein dyes, filaments, epithelial
adrenergic antagonists, chemotherapy agents, any
defects, basement membrane irregularities,
other drug with anticholinergic effects) (II++, GQ, SR)
mucous plaques, keratinization, pannus
- Trauma (e.g., mechanical, chemical, thermal)
formation, thinning, infiltrates, ulceration, scarring,
(II++, GQ, SR)
neovascularization, evidence of corneal or
- Chronic viral infections (e.g., hepatitis C, human
refractive surgery)
immunodeficiency virus) (II++, GQ, SR)

© 2017 American Academy of Ophthalmology November 2017 aao.org 11


CORNEA
Dry Eye Syndrome (Management Recommendations)

Care Management Patient Education


• Treat any causative factors that are amenable to • Counsel patients about the chronic nature of dry
treatment as patients with dry eye symptoms often eye and its natural history. (III, GQ, SR)
have many contributory factors • Provide specific instructions for therapeutic regimens.
• Sequence and combination of therapies is (III, GQ, SR)
determined based on the patient’s needs and • Reassess periodically the patient’s compliance and
preferences and understanding of the disease, risks for associated
the treating ophthalmologist’s medical judgment structural changes and realistic expectations for
(III, GQ, SR) effective management, and reinforce education. (III,
• For mild dry eye, the following measures are GQ, SR)
appropriate: • Refer patients with manifestation of a systemic
- Education and environmental modifications disease to an appropriate medical specialist. (III, GQ,
(III, GQ, SR) SR)
- Elimination of offending topical or systemic • Caution patients with pre-existing dry eye that
medications (III, IQ, DR) kerato­refractive surgery, particularly LASIK, may
- Aqueous enhancement using artificial tear worsen their dry eye condition. (III, GQ, SR)
substitutes, gels/ointments (III, IQ, DR)
- Eyelid therapy (warm compresses and eyelid
hygiene) (III, IQ, DR)
- Treatment of contributing ocular factors such as
blepharitis or meibomianitis (II++, GQ, DR)
- Correction of eyelid abnormalities (II++, MQ, DR)
• For moderate dry eye, in addition to above
treatments, the following measures are appropriate:
- Anti-inflammatory agents (topical cyclosporine
and corticosteroids, systemic omega-3 fatty acids
supplements)
- Punctal plugs (I++, GQ, SR)
- Spectacle side shields and moisture chambers
(III, GQ, SR)
• For severe dry eye, in addition to above treatments,
the following measures are appropriate:
- Systemic cholinergic agonists
- Systemic anti-inflammatory agents
- Mucolytic agents (III, IQ, DR)
- Autologous serum tears
- Contact lenses
- Correction of eyelid abnormalities
- Permanent punctal occlusion (III, IQ, DR)
- Tarsorrhaphy (III, IQ, DR)
• Monitor patients prescribed corticosteroids for
adverse effects such as increased intraocular
pressure, corneal melting, and cataract formation (III,
GQ, SR)

© 2017 American Academy of Ophthalmology November 2017 aao.org 12