Summary Benchmarks for Preferred Practice Pattern® Guidelines

SUMMARY BENCHMARKS FOR PREFERRED PRACTICE PATTERN® GUIDELINES

TABLE OF CONTENTS

Summary Benchmarks for Preferred Practice Pattern Guidelines Introduction ...... 1

Glaucoma Primary Open-Angle Glaucoma (Initial Evaluation) ...... 3 Primary Open-Angle Glaucoma (Follow-up Evaluation) ...... 5 Primary Open-Angle Glaucoma Suspect (Initial and Follow-up Evaluation) ...... 6 Primary Angle-Closure Disease (Initial Evaluation and Therapy) ...... 8

Retina Age-Related Macular Degeneration (Initial and Follow-up Evaluation) ...... 10 Age-Related Macular Degeneration (Management Recommendations) ...... 11 Diabetic Retinopathy (Initial and Follow-up Evaluation) ...... 12 Diabetic Retinopathy (Management Recommendations) ...... 13 Idiopathic Epiretinal Membrane and Vitreomacular Traction (Initial Evaluation and Therapy) ...... 14 Idiopathic Macular Hole (Initial Evaluation and Therapy) ...... 15 Posterior Vitreous Detachment, Retinal Breaks, and Lattice Degeneration (Initial and Follow-up Evaluation) ...... 17 Retinal and Ophthalmic Artery Occlusions (Initial Evaluation and Therapy) ...... 18 Retinal Vein Occlusions (Initial Evaluation and Therapy) ...... 19

Cataract/Anterior Segment Cataract (Initial and Follow-up Evaluation) ...... 20

Cornea/External Disease Bacterial Keratitis (Initial Evaluation) ...... 22 Bacterial Keratitis (Management Recommendations) ...... 23 Blepharitis (Initial and Follow-up Evaluation) ...... 24 Conjunctivitis (Initial Evaluation) ...... 25 Conjunctivitis (Management Recommendations) ...... 26 Corneal Ectasia (Initial Evaluation and Follow-up) ...... 27 Corneal Edema and Opacification (Initial Evaluation) ...... 28 Corneal Edema and Opacification (Management Recommendations) ...... 29 Dry Eye Syndrome (Initial Evaluation) ...... 30 Dry Eye Syndrome (Management Recommendations) ...... 31

Pediatric Ophthalmology/Strabismus Amblyopia (Initial and Follow-up Evaluation) ...... 32 Esotropia (Initial and Follow-up Evaluation) ...... 33 Exotropia (Initial and Follow-up Evaluation) ...... 34

Refractive Management/Intervention Keratorefractive Surgery (Initial and Follow-up Evaluation) ...... 35

Adult Strabismus Adult Strabismus with a History of Childhood Strabismus ...... 36

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

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 • Well-designed controlled trials without • Discretionary recommendation (DR): Used when the randomization trade-offs are less certain—either because of low- • Well-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 • Multiple-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 • Reports 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)

Initial Exam History (Key elements) • Assess the patient who is being treated with • Ocular history (e g. ,. refractive error, trauma, glaucoma medication for local ocular and systemic prior ocular surgery) side effects and toxicity • Race/ethnicity • Laser trabeculoplasty may be used as initial or • Family history adjunctive therapy in patients with POAG (see Table 5 of the POAG PPP) . Laser trabeculoplasty is • Systemic history effective in lowering IOP and may be performed to • Review of pertinent records 180 degrees or to 360 degrees of the angle . • Current medications • Prior glaucoma laser or incisional surgery Perioperative Care for Laser Trabeculoplasty Patients • The ophthalmologist who performs surgery has the Initial Physical Exam (Key elements) following responsibilities: • Visual acuity measurement - Obtain informed consent from the patient or • Pupil examination patient's surrogate decision maker after discussing • Confrontational visual fields the risk, benefits, and expected outcomes of surgery • Slit-lamp biomicroscopy - Ensure that the preoperative evaluation confirms • IOP measurement that surgery is indicated • Gonioscopy - At least one IOP check immediately prior to • Optic nerve head (ONH) and retinal nerve fiber layer surgery and within 30 minutes to 2 hours after (RNFL) examination surgery • Fundus examination - Follow-up examination within 6 weeks of surgery Diagnostic Testing (Key elements) or sooner if there is concern about IOP-related damage to the optic nerve • Central corneal thickness (CCT) measurement • Visual field evaluation Perioperative Care in Incisional Glaucoma Surgery • ONH, RNFL, and macular imaging Patients • The ophthalmologist who performs surgery has the Management Plan for Patients in Whom following responsibilities: Therapy is Indicated - Perform gonioscopy preoperatively, especially • The goal of treatment is to control the IOP in a when considering trabecular meshwork/Schlemm's target range and ensure the ONH/RNFL and visual canal-based MIGS (see Table 6 of the POAG PPP) fields are stable - Obtain informed consent from the patient or • Target IOP is an estimate and must be individualized patient's surrogate decision maker after discussing and/or adjusted during the course of the disease the risk, benefits, and expected outcomes of • Set an initial target pressure of at least 25% lower surgery than pretreatment IOP . Choosing a lower target IOP - Ensure that the preoperative evaluation accurately can be justified if there is more severe optic nerve documents findings and indications for surgery damage, if the damage is progressing rapidly, or if - Prescribe topical corticosteroids in the other risk factors are present (e g. ,. family history, age, postoperative period or disc hemorrhages) - Follow-up evaluation on the first postoperative • The IOP can be lowered by medical treatment, day and at least once during the first 1 to 2 weeks laser therapy, or incisional surgery (alone or in to evaluate visual acuity, IOP, and status of the combination) anterior segment • Medical therapy is presently the most common initial - In the absence of complications, perform additional intervention to lower IOP (see Table 4 of the POAG postoperative visits during a 3-month period PPP for an overview of options available); consider to evaluate visual acuity, IOP, and status of the balance between side effects and effectiveness in anterior segment choosing a regimen of maximal effectiveness and tolerance to achieve the desired IOP reduction for - Schedule more frequent follow-up visits, as each patient necessary, for patients with postoperative complications (flat or shallow anterior chamber, • If progression occurs at the target pressure, early bleb failure, increased inflammation, or undetected IOP fluctuations and adherence to the Tenon's cyst) therapeutic regimen and recommendations for therapeutic alternatives should be discussed before adjusting target IOP downward

- Undertake additional treatments as necessary to improve aqueous flow into the bleb and lower IOP if evidence of bleb failure develops, including injection of antifibrotic agents, bleb massage, suture adjustment, release or lysis, or bleb needling - Manage postoperative complications as they develop, such as repair of bleb leak or reformation of a flat anterior chamber - Explain that filtration surgery places the eye at risk for endophthalmitis for the duration of the patient's life, and that if the patient has symptoms of pain and decreased vision and the signs of redness and discharge he or she should notify the ophthalmologist immediately Patient Education for Patients with Medical Therapy • Discuss diagnosis, severity of the disease, prognosis and management plan, and likelihood of lifelong therapy • Educate about eyelid closure or nasolacrimal occlusion when applying topical medications to reduce systemic absorption • Encourage patients to alert their ophthalmologist to physical or emotional changes that occur when taking glaucoma medications

Follow-up Exam History • Contraindications to individual medications develop • Interval ocular history • Stable optic nerve status and low IOP occur for a • Interval systemic medical history prolonged period in a patient taking topical ocular hyopotensive agents . Under these circumstances, a • Side effects of ocular medications carefully monitored attempt to reduce the medical • Review of pertinent medication use, including regimen may be appropriate time of last administration • Downward adjustment of target pressure can be made in the face of progressive optic disc, imaging, Follow-up Physical Exam or visual field change • Visual acuity measurement • Upward adjustment of target pressure can be • Slit-lamp biomicroscopy considered if the patient has been stable and if the • IOP measurement patient either requires or desires less medication • Perform gonioscopy if there is a suspicion of angle- Patient Education closure component, anterior chamber shallowing or anterior chamber angle abnormalities, or if there is • Educate about the disease process, the rationale and an unexplained change in IOP . Perform gonioscopy goals of intervention, the status of their condition, periodically and relative benefits and risks of alternative interventions so that patients can participate • ONH and visual field evaluation meaningfully in developing an appropriate plan of action Adjustment of Therapy • Patients considering keratorefractive surgery should • Target IOP is not achieved and benefits of a change be informed about the possible impact laser vision in therapy outweigh the risks correction has on reducing contrast sensitivity and • Progressive optic nerve damage despite achieving decreasing the accuracy of IOP measurements the target IOP • Patients with substantial visual impairment or • Patient's intolerant of the prescribed medical blindness can be referred for and encouraged to use regimen appropriate vision rehabilitation and social services

Follow-Up:

Consensus-based Guidelines for Follow-up Glaucoma Status

Duration of Control Approximate Follow-up Interval Target IOP Achieved Progression of Damage (months) (months)* Yes No ≤6 6 Yes No >6 12 Yes Yes NA 1–2 No Yes NA 1–2 No No NA 3–6

IOP = intraocular pressure; NA = not applicable **Patients with more advanced damage or greater lifetime risk from primary open-angle glaucoma may require more frequent evaluations . These intervals are the maximum recommended time between evaluations .

Initial Exam History (Key elements) • Medical therapy is presently the most common • Ocular history (e g. ., refractive error, trauma, prior initial intervention to lower IOP (see Table 2 of the ocular surgery) POAG Suspect PPP for an overview of options available); consider balance between side effects • Race/ethnicity and effectiveness in choosing a regimen of maximal • Family history effectiveness and tolerance to achieve the desired • Systemic history IOP reduction for each patient • Review of pertinent records • If a medical therapy fails to reduce IOP sufficiently, then either switching to an alternative medication • Current and prior ocular and nonocular medications as monotherapy or adding additional medication is • Prior cataract surgery, LASIK and/or incisional appropriate until the desired IOP level is attained surgery Follow-up Exam History Initial Physical Exam (Key elements) • Interval ocular history • Visual acuity measurement • Interval systemic medical and medication history • Pupil examination • Side effects of ocular medications if the patient is • Confrontation visual field being treated • Slit-lamp biomicroscopy • Review of pertinent medication use if the patient • IOP measurement is being treated, including the time of the last administration • Gonioscopy • Optic nerve head (ONH) and retinal nerve fiber layer Follow-up Physical Exam (RNFL) examination • Visual acuity measurement • Fundus examination • Slit-lamp biomicroscopy Diagnostic Testing (Key elements) • IOP measurement • Central corneal thickness (CCT) measurement • Gonioscopy is indicated when there is a suspicion of development of an angle-closure component, • Visual field evaluation . If visual field glaucomatous anterior chamber shallowing, anterior chamber angle damage is newly detected in a glaucoma suspect abnormalities, or unexplained change in IOP patient, it is best to repeat testing • ONH, RNFL, and macular imaging . Clinicians Adjustment of Therapy should include all perimetric and other structural • Target IOP is not achieved and the benefits of a information in addition to digital imaging technology change in therapy outweigh the risks for the patient when formulating patient management decisions • The patient is intolerant of the prescribed medical Management Plan for Patients in Whom regimen Therapy is Indicated • The patient does not adhere to the prescribed • The goal of treatment is to monitor or lower IOP medical regimen due to costs or other factors through treatment if an eye is likely to progress to • New systemic conditions or treatments develop that POAG; monitor for structural changes in optic disc could be a contraindication to the current glaucoma and retina; and monitor for functional changes of the therapy optic nerve assessing the visual field • The patient under treatment has been stable for a • The decision to treat a glaucoma suspect patient prolonged period without progression to POAG, in may arise in various settings (see POAG Suspect which case cautious withdrawal of therapy may be PPP for detailed considerations) considered • Target IOP is an estimate and must be individualized • The patient has converted to POAG (see Primary and/or adjusted during the course of the disease Open-Angle Glaucoma PPP)

Patient Education • Discuss number and severity of risk factors, prognosis, life expectancy, management plan, and likelihood that therapy, once started, can be continued long term • Educate about their condition and its potential to lead to glaucoma, the status of their condition, the rationale and goals of intervention, and relative benefits and risks of alternative interventions • Educate about eyelid closure or nasolacrimal occlusion to reduce systemic absorption after medication instillation • Encourage patients to alert their ophthalmologist to physical or emotional changes that occur when taking glaucoma medications • Patients with substantial visual impairment or blindness can be referred for and encouraged to use appropriate vision rehabilitation and social services

Initial Exam History (Key elements) • Laser iridotomy is the preferred surgical treatment • Ocular history (e g. ., blurred vision, halos around for AACC because it has a favorable risk-benefit lights, eye pain, headache, eye redness) ratio; but patients with PACS who have not had an iridotomy should be warned of potential risk • Family history of acute angle-closure crisis (AACC) for AACC and certain medicines could cause pupil • Systemic history (e g. ., use of topical or systemic dilation and induce AACC medications) • Selection of cyclophotocoagulation over other procedures should be left to the discretion of the Initial Physical Exam (Key elements) treating ophthalmologist, in consultation with the • Refractive status individual patient • Pupil examination • The fellow eye should be scheduled for a • Slit-lamp biomicroscopy prophylactic iridotomy if the chamber angle is anatomically narrow, since approximately half of - Conjunctival hyperemia (in acute cases) fellow eyes can develop AACC within 5 years - Central and peripheral anterior chamber depth • Given the lack of convincing evidence for narrowing prophylactic use of iridoplasty in patients with - Anterior chamber inflammation suggestive of a plateau iris, and since iridoplasty can be painful and recent or current attack may cause inflammation, the decision of whether to - Corneal swelling . (Microcystic edema and stromal observe or treat these eyes is left to the judgment of edema are common in acute cases .) the testing ophthalmologist - Small corneal diameter (indicative of a smaller eye Perioperative Care for Iridotomy Patients at greater risk for PACD) • The ophthalmologist who performs surgery has the - Iris abnormalities, including diffuse or focal following responsibilities: atrophy, posterior synechiae, abnormal pupillary function, irregular pupil shape, and a mid-dilated - Obtain informed consent from the patient or pupil (suggestive of a recent or current attack) patient's surrogate decision maker after discussing the risk, benefits, and expected outcomes of - Lens changes, including cataract and surgery glaukomflecken - Ensure that preoperative evaluation confirms the - Corneal endothelial cell loss need for surgery • IOP measurement - Consider the preoperative use of a • Gonioscopy parasympathomimetic to facilitate LPI • Evaluation of fundus and optic nerve head using - Use topical ocular hypotensive agents direct ophthalmoscope or slit-lamp biomicroscope perioperatively to prevent sudden IOP elevation, with an indirect lens, the central portion of the particularly for patients who have severe disease gonioscopy lens, or by imaging the retina and - Ensure the patency of the iridotomy by directly optic nerve with photography using a nonmydriatic visualizing fluid flow of aqueous and pigment from camera through an undilated pupil the posterior to anterior chamber . Visualization of a red reflex alone is insufficient to confirm patency Diagnostic Testing (Key elements) - Enlarge the iridotomy as necessary to achieve • Anterior segment imaging diameter of at least 100 microns • Ocular biometry - Perform at least one IOP check immediately prior • Provocative testing to surgery and within 30 minutes to 2 hours following surgery Management Plan for Patients in Whom - Prescribe topical corticosteroids in the Iridotomy is Indicated postoperative period • In patients with Primary Angle-Closure Suspect - Ensure that the patient receives adequate (PACS), iridotomy may be considered to reduce the postoperative care risk of developing angle closure • In acute angle-closure crisis (AACC), use medical therapy first to lower the IOP to reduce pain and to clear corneal edema (see Table 4 of the POAG PPP) . Iridotomy should then be performed as soon as possible

• F ollow-up evaluations include: - Confirm patency of iridotomy by visualizing zonules, the anterior lens capsule, or ciliary processes -IOP measurement - Perform dark-room gonioscopy with compression/indentation to assess the extent of peripheral anterior synechiae (PAS) if it was not performed immediately following iridotomy - Examine the fundus as clinically indicated

Follow-up of Patients after Iridotomy • Patients (with or without glaucomatous optic neuropathy) with a residual open angle or a combination of open angle and some PAS should be followed at appropriate intervals to check for increasing PAS • If IOP remains elevated long term and patient develops PAC or PACG, then ongoing medical therapy to lower IOP may become necessary (see follow-up procedures and intervals in POAG PPP)

Patient Education • Patients with PAS who have not had an iridotomy should be warned of potential risk for AACC and that certain medicines could cause pupil dilation and induce AACC • P atients should be informed about the symptoms of AACC and instructed to notify their ophthalmologist immediately if symptoms occur • P atients with substantial visual impairment or blindness can be referred for and encouraged to use appropriate vision rehabilitation and social services

Initial Exam History (Key elements) Follow-up Exam History • Symptoms (metamorphopsia, decreased vision, • V isual symptoms, including decreased vision and scotoma, photopsia, difficulties in dark adaptation) metamorphopsia • Medica tions and nutritional supplement use • Changes in medications and nutritional supplements • Ocular history • Changes in ocular history and medical history • Medical history (any hypersensitivity reactions) • Changes in social history, especially smoking • F amily history, especially family history of AMD Follow-up Physical Exam • Social history, especially smoking • V isual acuity at distance with correction Initial Physical Exam (Key elements) • Amsler Grid • C omprehensive eye examination • S tereo biomicroscopic examination of the fundus • Amsler Grid Follow-up after Treatment for Neovascular AMD • S tereo biomicroscopic examination of the macula • Ex amine patients treated with intravitreal injections Diagnostic Tests of aflibercept, bevacizumab, or ranibizumab approximately at 4-week intervals Optical coherence tomography is important in diagnosing and managing AMD, particularly with • Subsequent examinations, OCT, and fluorescein respect to determining the presence of subretinal and angiography should be performed as indicated intraretinal fluid and in documenting the degree of depending on the clinical findings and the judgment retinal thickening . Optical coherence tomography of the treating ophthalmologist defines the cross sectional architecture of the Patient Education retina, which is not possible with any other imaging technology . It may reveal thepresence of fluid that • E ducate patients about the prognosis and potential is not apparent on biomicroscopy alone . It also value of treatment as appropriate for their visual assists in evaluating the response of the retina and and functional status RPE to therapy by allowing structural changes to be • Enc ourage patients with early AMD or a family followed accurately . Newer generation OCT modalities, history of AMD to assess their own visual acuity including SD-OCT, are preferred technologies . using monocular vision testing and to have regular dilated eye exams for early detection of Optical coherence tomography angiography (OCTA) intermediate AMD is a newer imaging modality that provides noninvasive • E ducate patients with a high-risk AMD phenotype evaluation of the retinal and choroidal vasculature and about methods of detecting new symptoms of CNV is being more commonly applied in the evaluation and and about the need for prompt notification to an management of AMD, but it has not replaced other ophthalmologist angiographic methods . • Ins truct patients with unilateral disease to monitor Intravenous fundus fluorescein angiography is their vision in their fellow eye and to return indicated: periodically even in absence of symptoms, but • when patient complains of new metamorphopsia promptly after onset of new or significant visual • when patient has unexplained blurred vision symptoms • when clinical exam reveals elevation of the RPE • Ins truct patients to report symptoms suggestive or retina, macular edema, subretinal blood, hard of endophthalmitis, including eye pain or increased exudates or subretinal fibrosis or the OCT shows discomfort, worsening eye redness, blurred or evidence of fluid . decreased vision, increased sensitivity to light, or increased number of floaters promptly • t o detect the presence of and determine the extent, type, size, and location of CNV • Enc ourage patients who are currently smoking to stop because there are observational data that • t o guide treatment (laser photocoagulation surgery support a causal relationship between smoking or verteporfin PDT) and AMD and other considerable health benefits of • t o detect persistent or recurrent CNV or other smoking cessation retinal diseases following treatment • R efer patients with reduced visual function for • t o assist in determining the cause of visual loss that vision rehabilitation (see www aao. or. g/low-vision- is not explained by clinical exam and-vision-rehab) and social services Each angiographic facility should have a care plan for an emergency and a clear protocol to minimize the risks and to manage complications .

Treatment Recommendations and Follow-up Plans for Age-Related Macular Degeneration

Recommended Treatment Diagnoses Eligible for Treatment Follow-up Recommendations Non-neovascular AMD Early AMD (AREDS category 2) Return exam at 6 to 24 months if asymptomatic or prompt Observation with no medical or exam for new symptoms suggestive of CNV surgical therapies Advanced AMD with bilateral subfoveal Return exam at 6 to 24 months if asymptomatic or prompt geographic atrophy or disciform scars exam for new symptoms suggestive of CNV Fundus photos, fluorescein angiography, OCT, or OCTA as appropriate Non-neovascular AMD Intermediate AMD (AREDS category 3) Return exam at 6 to 18 months if asymptomatic or prompt Antioxidant vitamin and mineral Advanced AMD in one eye (AREDS exam for new symptoms suggestive of CNV supplements as recommended in category 4) Monitoring of monocular near vision (reading/Amsler grid) the original AREDS and AREDS2 reports Fundus photography and/or fundus autofluorescence as appropriate Fluorescein angiography and/or OCT for suspicion of CNV Neovascular AMD Macular CNV Patients should be instructed to report promptly symptoms Aflibercept intravitreal injection suggestive of endophthalmitis, including eye pain or increased 2 0. mg as described in published discomfort, worsening eye redness, blurred or decreased vision, reports increased sensitivity to light, or increased number of floaters Return examination approximately 4 weeks after treatment initially; subsequent follow-up and treatment depends on the clinical findings and judgment of the treating ophthalmologist .

An every 8-week maintenance treatment regimen has been

shown to have comparable results to every 4 weeks in the first year of therapy .

Monitoring of monocular near vision (reading/Amsler grid) Neovascular AMD Macular CNV Patients should be instructed to report any symptoms Bevacizumab intravitreal injection suggestive of endophthalmitis promptly, including eye pain 1 .25 mg as described in published or increased discomfort, worsening eye redness, blurred or reports decreased vision, increased sensitivity to light, or increased The ophthalmologist should provide number of floaters

appropriate informed consent with Return exam approximately 4 weeks after treatment; respect to the off-label status subsequent follow-up depends on the clinical findings and judgment of the treating ophthalmologist Monitoring of monocular near vision (reading/Amsler grid) Neovascular AMD Macular CNV Patients should be instructed to report any symptoms Brolucizumab intravitreal injection suggestive of endophthalmitis promptly, including eye pain 6 0. mg as described in FDA or increased discomfort, worsening eye redness, blurred or labeling decreased vision, increased sensitivity to light, or increased number of floaters Return exam approximately 4 weeks after treatment; subsequent follow-up depends on the clinical findings and judgment of the treating ophthalmologist Monitoring of monocular near vision (reading/Amsler grid) Neovascular AMD Macular CNV Patients should be instructed to report any symptoms Ranibizumab intravitreal injection suggestive of endophthalmitis promptly, including eye pain 0 .5 mg as recommended in or increased discomfort, worsening eye redness, blurred or literature decreased vision, increased sensitivity to light, or increased number of floaters Return exam approximately 4 weeks after treatment; subsequent follow-up depends on the clinical findings and judgment of the treating ophthalmologist

Monitoring of monocular near vision (reading/Amsler grid) Less Commonly Used Treatments Macular CNV, new or recurrent, where the Return exam approximately every 3 months until stable, with for Neovascular AMD classic component is >50% of the lesion and retreatments as indicated PDT with verteporfin as the entire lesion is ≤5400 microns in greatest Monitoring of monocular near vision (reading/Amsler grid) recommended in the TAP and linear diameter VIP reports* Occult CNV may be considered for PDT with vision <20/50 or if the CNV is <4 MPS disc areas in size when the vision is >20/50 Juxtafoveal CNV is an off-label indication for PDT, but may be considered in select cases . Less Commonly Used Treatments May be considered for extrafoveal classic CNV, Return exam with fluorescein angiography approximately 2 for Neovascular AMD new or recurrent to 4 weeks after treatment, and then at 4 to 6 weeks and Thermal laser photocoagulation May be considered for juxtapapillary CNV thereafter depending on the clinical and angiographic findings surgery as recommended in the Retreatments as indicated MPS reports is rarely used Monitoring of monocular near vision (reading/Amsler grid)

AMD = age-related macular degeneration; AREDS = Age-Related Eye Disease Study; CNV = choroidal neovascularization; MPS = Macular Photocoagulation Study; OCT = optical coherence tomography; OCTA = optical coherence tomography angiography; PDT = photodynamic therapy; TAP = Treatment of Age-Related Macular Degeneration with Photodynamic Therapy; VIP = Verteporfin in Photodynamic Therapy *Contraindicated in patients with porphyria or known allergy

Initial Exam History (Key elements) • Optical coherence tomography can be used to • Duration of diabetes quantify retinal thickness, monitor macular edema, identify vitreomacular traction, and detect other • Past glycemic control (hemoglobin A1c) forms of macular disease in patients with diabetic • Medications macular edema . Decisions to treat with anti-VEGF • Medical history (e g. ., obesity, renal disease, systemic injections, change therapeutic agents (e g. ., use of hypertension, serum lipid levels, pregnancy) intraocular corticosteroids), initiate laser treatment, or even consider vitrectomy surgery are often based • Ocular history in part on OCT findings . Initial Physical Exam (Key elements) • Fluorescein angiography is not routinely indicated as a part of the examination of patients with • Visual acuity diabetes . Fluorescein angiography is used as a • Slit-lamp biomicroscopy guide for laser treatment of CSME and as a means • Measurement of IOP of evaluating the cause(s) of unexplained decreased visual acuity . Angiography can identify macular • Gonioscopy before dilation when indicated (for capillary nonperfusion as possible explanations for neovascularization of the iris or increased IOP) visual loss that is unresponsive to therapy . • Pupillary assessment for optic nerve dysfunction • Optical coherence tomography angiography offers a • Thorough funduscopy including stereoscopic noninvasive nature and the ability to visualize depth- examination of the posterior pole resolved, capillary-level abnormalities in the three • Examination of the peripheral retina and vitreous, retinal plexuses, offering a much more quantitative best performed with indirect ophthalmoscopy or assessment of macular ischemia . Although the with slit-lamp biomicroscopy technology is FDA approved, the guidelines and indications for use in diabetic retinopathy are evolving . Diagnosis • Ultrasonography enables assessment of the status of • Classify both eyes as to category and severity of the retina in the presence of a vitreous hemorrhage diabetic retinopathy and macular edema . (III, GQ, SR) or other media opacity, and may be helpful to define Each category has an inherent risk for progression the amount of vitreous hemorrhage, the extent and and is dependent on adherence to overall diabetes severity of vitreoretinal traction, and diagnose diabetic control . retinal detachments in the setting of media opacity .

Follow-up History Patient Education • Visual symptoms • Discuss results of exam and implications • Systemic status (pregnancy, blood pressure, serum • Encourage patients with diabetes but without dia- lipids, renal status) betic retinopathy to have annual dilated eye exams • Glycemic status (hemoglobin A1c) • Inform patients that effective treatment for diabetic retinopathy depends on timely intervention, despite • Other treatments (dialysis, fenofibrates) good vision and no ocular symptoms, and that current treatments often require multiple visits Follow-up Physical Exam and evaluations over time for adequate delivery of • Visual acuity therapeutic effect • Slit-lamp biomicroscopy with iris examination • Educate patients about the importance of maintaining • Measurement of IOP near-normal glucose levels and near-normal blood pressure and lowering serum lipid levels • Gonioscopy (preferably before dilation when iris neovascularization is suspected or if IOP is elevated) • Communicate with the attending physician, e g. ., family physician, internist, or endocrinologist, • Stereoscopic examination of the posterior pole after regarding eye examination findings dilation of the pupils • Provide patients whose conditions fail to respond • Examination of the peripheral retina and vitreous to surgery and for whom further treatment is when indicated unavailable with professional support and offer • OCT imaging when appropriate referral for counseling, rehabilitative, or social services as appropriate Ancillary Tests • Refer patients with functionally limiting • Color Fundus photography may be useful for postoperative visual impairment for vision documenting the severity of the diabetes presence rehabilitation (see www .aao org/low-vision-and-. of NVE and NVD, the response to treatment, and vision-rehab) and social services the need for additional treatment at future visits .

Management Recommendations for Patients with Diabetes

Severity of Retinopathy Presence of Follow-up Panretinal Focal and/or Intravitreal Anti- Macular (Months) Photocoagulation Grid Laser* VEGF Therapy Edema (Scatter) Laser Normal or minimal NPDR No 12 No No No Mild NPDR No 12 No No No NCI-DME 3-6 No Sometimes No CI-DME† 1* No Rarely Usually Moderate NPDR No 6-12‡ No No No NCI-DME 3–6 No Sometimes Rarely CI-DME† 1* No Rarely Usually Severe NPDR No 3-4 Sometimes No Sometimes NCI-DME 2–4 Sometimes Sometimes Sometimes CI-DME† 1* Sometimes Rarely Usually Non-high-risk PDR No 3-4 Sometimes No Sometimes NCI-DME 2–4 Sometimes Sometimes Sometimes CI-DME† 1* Sometimes Sometimes Usually High-risk PDR No 2-4 Recommended No Sometimes1,2 NCI-DME 2-4 Recommended Sometimes Sometimes CI-DME† 1* Recommended Sometimes Usually

Anti-VEGF = anti-vascular endothelial growth factor; CI-DME = center-involved diabetic macular edema; NCI-DME = noncenter-involved diabetic macular edema: NPDR = nonproliferative diabetic retinopathy; PDR = proliferative diabetic retinopathy * Adjunctive treatments that may be considered include intravitreal corticosteroids or anti-VEGF agents (off-label use, except aflibercept and ranibizumab) . Data from the Diabetic Retinopathy Clinical Research Network in 2011 demonstrated that, at two years of follow-up, intravitreal ranibizumab with prompt or deferred laser resulted in greater visual acuity gain and intravitreal triamcinolone acetonide plus laser also resulted in greater visual gain in pseudophakic eyes compared with laser alone . Individuals receiving the intravitreal injections of anti-VEGF agents may be re-examined as early as one month following injection . † Exceptions include hypertension or fluid retention associated with heart failure, renal failure, pregnancy, or any other causes that may aggravate macular edema . For patients with good visual acuity (20/25 or better) and CI-DME, there is no difference between observation plus aflibercept if visual acuity decreases, focal laser plus aflibercept if vissual acuity decreases, or anti-VEGF treatment . It is appropriate to defer treatment until visual acuity is worse than 20/25 . Deferral of photocoagulation for a brief period of medical treatment may be considered in these cases . Also, deferral of NCI-DME treatment is an option when the visual acuity is excellent (better than 20/32), close follow-up is possible, and the patient understands the risks . ‡ Or at shorter intervals if signs approaching those of severe NPDR appear .

References: 1 . Writing Committee for the Diabetic Retinopathy Clinical Research Network . Panretinal photocoagulation vs intravitreous ranibizumab for proliferative diabetic retinopathy: a randomized clinical trial . JAMA 2015;314:2137–46 . 2 . Olsen, TW . Anti-VEGF pharmacotherapy as an alternative to panretinal laser photocoagulation for proliferative diabetic retinopathy . JAMA 2015;314:2135–6 .

Initial Exam (Key elements) Surgery and Postoperative Care • Ocular history (e g. ., posterior vitreous detachment, • Vitrectomy surgery is often indicated in patients uveitis, retinal breaks, retinal vein occlusions, who are affected with a decrease in visual acuity, proliferative diabetic retinopathy, ocular metamorphopsia, and double vision or difficulty inflammatory diseases, recent wound healing) using their eyes together • Duration of symptoms (e g. ., metamorphopsia, • Patients should be examined postoperatively difficulty using both eyes together, and diplopia) day 1, and again 1 to 2 weeks following surgery, • Race/ethnicity or sooner depending upon the development of new symptoms or new findings during early • Systemic history postoperative examination

Physical Exam (Key elements) Follow-up Physical Exam: • Slit-lamp biomicroscopy the macula, vitreoretinal • Internal history interface, and optic disc • Measurement of IOP • An indirect peripheral retinal examination • Slit lamp biomicroscopy of anterior segment • Amsler grid test and/or Watzke-Allen test • Indirect binocular ophthalmoscopy of peripheral • OCT to diagnose and characterize VMA, ERM, VMT, retina and associated retinal changes • Counseling on use of post op medications • Fluorescein angiogram or OCTA may be helpful to evaluate ERMs and/or VMT • Counseling on signs and symptoms of retinal detachment Management Plan • Precautions on intraocular gas if used • The decision to intervene surgically in patients with ERM/VMT usually depends upon the severity of Patient Education and Follow-up symptoms, especially the impact on daily activities • Comparing OCT images in the abnormal versus • Patients should be informed that the majority of normal eye can aid patient understanding ERMs will remain stable and do not require therapy • Patients should be encouraged to periodically • Patients should be reassured that there is a very test their central vision monocularly to detect successful surgical procedure that could address changes that may occur over time, like increasing worsening symptoms or decreasing visual acuity metamorphopsia small central scotoma • Risks versus benefits of vitrectomy surgery should • Patients should be informed to notify their be discussed . Risks include decreased visual acuity, ophthalmologist promptly if they have symptoms cataract, retinal tears, retinal detachment, and such as an increase of floaters, loss of visual field, endophthalmitis metamorphopsia, or a decrease in visual acuity • Patients with functionally limiting postoperative visual impairment should be referred for vision rehabilitation (see www .aao org/low-vision-and-. vision-rehab) and social services

Initial Exam History (Key elements) Examination (Key elements) • Duration of symptoms • Slit-lamp biomicroscopic examination of the macula • Ocular history: glaucoma, retinal detachment or tear, and the vitreoretinal interface other eye diseases, eye or head or injuries, ocular • Indirect peripheral retinal examination surgery, or sun or eclipse gazing or use of a laser • Amsler grid test and/or Watzke-Allen test pointer or other laser • Medications that may be related to macular cystoid Ancillary Test edema (e g. ., systemic niacin, topical prostaglandin • OCT offers detailed information about the macular analogues, tamoxifen) anatomy size if an FTMH is present, and presence of any VMT or epiretinal membrane

Management Recommendations for Macular Hole Stage Management Follow-up 1-A and 1-B Observation • Follow-up at 2–4 month intervals in the absence of new symptoms • Recommend prompt return if new symptoms develop • Encourage monocular visual acuity testing with Amsler grid 2 Pneumatic Vitreolysis* • Performed usually within 1 to 2 weeks of diagnosis • Follow up at 1-2 days, then 1 week or sooner if new visual symptoms • Frequency and timing of subsequent visits varies depending on the outcome of surgery and the patient’s clinical course 2 Vitreoretinal surgery • Performed usually within 1 month of diagnosis to minimize risk of progression of macular hole and vision loss • Follow-up at 1–2 days postoperatively, then 1–2 weeks during which strict face down positioning is advised • Frequency and timing of subsequent visits varies depending on the outcome of surgery and the patient’s clinical course 2 Vitreopharmacolysis‡ • Performed usually within 1 to 2 weeks of diagnosis • Follow-up at 1 week and 4 weeks, or with new symptoms (i e. ., retinal detachment symptoms) 3 or 4 Vitreoretinal surgery • Performed usually within 1 month of diagnosis • Follow-up at 1–2 days postoperatively, then 1–2 weeks with strict face down positioning if advised • Frequency and timing of subsequent visits varies depending on the outcome of surgery and the patient’s clinical course

* Several small case series have shown promising results with this technique for smaller holes ‡ Ocriplasmin has been approved by the U .S . Food and Drug Administration for symptomatic vitreomacular adhesion . There is no evidence to support its use for treatment of idiopathic macular hole without vitreomacular traction or adhesion and this would be considered off-label use .

Surgical and Postoperative Care if Patient Receives • Examine postoperatively within 1 or 2 days and Treatment again 1 to 2 weeks after surgery • Patients should be informed about relative risks, • Components of follow-up visit should include benefits, and alternatives to surgery, and the need interval history, visual acuity measurement, for use of expansile intraocular gas or facedown measurement of IOP, slit-lamp biomicroscopy of the positioning postoperatively anterior chamber and central retina, and indirect • Formulate a postoperative care plan and inform the ophthalmoscopy of the peripheral retina, and OCT patient of these arrangements evaluation to document post-op macular anatomy when indicated • Patients should be informed of possible postoperative increase in IOP

Patient Education • Patients should be informed to notify their ophthalmologist promptly if they have symptoms such as an increase in floaters, a loss of visual field, metamorphopsia, or a decrease in visual acuity • Patients should be informed that air travel, travel to higher or lower altitudes, or general anesthesia with nitrous oxide should be avoided until the gas tamponade is nearly completely gone • Patients who have had a macular hole in one eye should be informed that they have a 10% to 15% chance of macular hole formation in the fellow eye, especially if the vitreous remains attached • Patients with functionally limiting postoperative visual impairment should be referred for vision rehabilitation (see www .aao org/low-vision-and-. vision-rehab) and social services

Initial Exam History (Key elements) • Formulate a postoperative care plan and inform • Symptoms of PVD patient of these arrangements • Family history of RD, related genetic disorders (e g. ., • Patients should be advised to contact Stickler syndrome) ophthalmologist promptly if they have a substantial • Prior eye trauma change in symptoms such as floaters, peripheral visual field loss, or decreased visual acuity • Myopia • History of ocular surgery including refractive lens Follow-up History exchange and cataract surgery • Visual symptoms • History of YAG laser capsulotomy • Interval history of eye trauma, intraocular injection, • History of intravitreal injection or intraocular surgery Ophthalmic Exam (Key elements) Follow-up Physical Exam • Confrontation visual field examination • Visual acuity • Visual acuity testing • Evaluation of the status of the vitreous, with • Pupillary assessment for the presence of a relative attention to the presence of pigment, hemorrhage, afferent pupillary defect or syneresis • Examination of the vitreous for hemorrhage, • Examination of the peripheral fundus with scleral detachment, and pigmented cells depression or a fundus contact or non-contact lens • Examination of the peripheral fundus using scleral using the slit-lamp biomicroscope depression . The preferred method of evaluating • Wide-field photography may help but does not peripheral vitreoretinal pathology is with indirect replace careful ophthalmoscopy ophthalmoscopy combined with scleral depression . • Optical coherence tomography if vitreomacular traction is present Diagnostic Tests • B-scan ultrasonography if the media are opaque • Optical coherence tomography may be helpful to evaluate and stage the PVD Patient Education • Perform B-scan ultrasonography if peripheral retina • Patients at high risk of developing retinal cannot be evaluated . detachment should be educated about the • If no abnormalities are found, frequent follow-up symptoms of PVD and retinal detachment and the examinations are recommended (i e. ., every 1-2 value of periodic follow-up exams weeks initially) . • Patients who undergo refractive surgery should be informed they remain at risk of RRD despite Management: reduction of their refractive error • Patients should be informed about the relative risks, benefits, and alternatives to surgery

Care Management Management Options Type of Lesion Treatment* Acute symptomatic horseshoe tears Treat promptly Acute symptomatic operculated holes Treatment may not be necessary Acute symptomatic dialyses Treat promptly Traumatic retinal breaks Usually treated Asymptomatic horseshoe tears (without subclinical RD) Consider treatment unless there are signs of chronicity Asymptomatic operculated tears Treatment is rarely recommended Asymptomatic atrophic round holes Treatment is rarely recommended Asymptomatic lattice degeneration without holes Not treated unless PVD causes a horseshoe tear Asymptomatic lattice degeneration with holes Usually does not require treatment Asymptomatic dialyses No consensus on treatment and insufficient evidence to guide management Eyes with atrophic holes, lattice degeneration No consensus on treatment and insufficient evidence to guide where the fellow eye has had an RD management

PVD = posterior vitreous detachment; RD = retinal detachment *There is insufficient evidence to recommend prophylaxis of asymptomatic retinal breaks for patients undergoing cataract surgery .

History (Key elements) Care Management • Duration of vision loss • Physicians should first consider GCA in patients 50 • Symptoms of GCA (e g. ., vision loss, headaches, years of age or older scalp tenderness, malaise, fatigue, temporal • In cases of GCA, physicians should consider tenderness, jaw claudication, weakness, fever, urgent systemic corticosteroid therapy to prevent myalgia, and diplopia) vision loss in the fellow eye or vascular occlusion • Medications elsewhere • Family history of cardiovascular disease, diabetes, • Diabetics with GCA should be carefully monitored systemic hypertension, or hyperlipidemia since systemic corticosteroid treatment may destabilize glucose control • Medical history (e g. ., systemic hypertension, diabetes, hyperlipidemia, cardiovascular disease, • Ophthalmologists should refer patients with hemoglobinopathy and polymyalgia rheumatica) or retinal vascular disease to the appropriate setting, drug history (e g. ., cocaine) depending on the nature of the retinal occlusion • Ocular history (e g. ., trauma, other eye diseases, • Acute, symptomatic OAO, CRAO, and BRAO from ocular injections, surgery) embolic etiologies should prompt an immediate referral to the nearest stroke center for prompt • Social history (e g. ., smoking) assessment for consideration of intervention

Physical Exam (Key elements) • When presented with an asymptomatic BRAO, clinicians should conduct a systemic evaluation • Visual acuity (careful medical history, assessment for systemic • Slit-lamp biomicroscopy disease), preferably in conjunction with the patient’s • IOP internist • Gonioscopy when IOP is elevated or when iris Patient Follow-up neovascularization risk is suspected (prior to dilation) • Follow-up should consider the extent of retinal or ocular ischemia neovascularization . Patients with • Relative afferent pupil defect assessment greater ischemia require more frequent follow-up • Slit-lamp biomicroscopy of the posterior pole • Many patients with retinal vascular disease will lose • Examination of the peripheral retina using indirect substantial vision despite various treatment options ophthalmoscopy through a dilated pupil to assess: and should be referred for appropriate social retinal hemorrhages, cotton-wool spots, retinal services and vision rehabilitation (see www .aao org/. emboli, retinal vascular “boxcarring,” and optic low-vision-and-vision-rehab) disc neovascularization and/or neovascularization • Follow-up evaluation includes a history (symptoms, elsewhere systemic conditions) and examination (visual acuity, slit-lamp biomicroscopy with iris examination, IOP, Diagnostic Tests undilated gonioscopy for iris neovascularization, • Color and red-free fundus photography biomicroscopic exam of posterior pole after dilation, • OCT peripheral retinal vitreous exam when indicated, OCT imaging when appropriate, fluorescein • Fluorescein angiography angiography) • Indocyanine green angiography • Patients with asymptomatic BRAO could be referred • Ultrasonography in the setting of significant media to a primary care physician opacity (to rule out other acute causes of vision loss)

Initial Exam (Key elements) Care Management • Location and duration of vision loss • Optimizing control of diabetes mellitus, • Current medications hypertension, hyperlipidemia, and IOP are important to manage risk factors • Medical history (e g. ., systemic hypertension, diabetes, hyperlipidemia, cardiovascular disease, • Systemic reviews have shown the efficacy of anti- sleep apnea, coagulopathies, thrombotic disorders, VEGF agents in treating macular edema associated pulmonary embolus) with RVO (I++, GQ, SR) • Ocular history (e g. ., glaucoma, other ophthalmologic • Laser treatment remains a viable treatment in eyes disorders, ocular injections, surgery, including retinal with BRVO, even if the duration of the disease is laser treatment, cataract surgery, refractive surgery) greater than 12 months • Sectoral pan retinal photocoagulation is still Physical Exam (Key elements) recommended for neovascularization when • Visual acuity complications such as vitreous hemorrhage or iris neovascularization occur • Pupillary assessment for relative afferent pupillary defect that corresponds to level of ischemia and • Ophthalmologists caring for patients with retinal predictive risk for neovascularization vascular occlusion should be familiar with specific recommendations of relevant clinical trials due to • Slit-lamp biomicroscopy for fine, abnormal, new iris the complexity of diagnosis and treatment vessels • Measurement of IOP Patient Follow-up • Gonioscopy prior to dilation; especially in cases of • Follow-up evaluation includes a history of changes an ischemic CRVO, when IOP is elevated, or when in symptoms and systemic status (pregnancy, blood iris neovascularization risk is high pressure, serum cholesterol, and blood glucose) • Binocular funduscopic evaluation of the posterior and examination (visual acuity, undilated slit-lamp pole biomicroscopy and gonioscopy) monthly for 6 months with CRVO and in eyes with ischemic • Examination of the peripheral retina and vitreous . CRVO after discontinuing anti-VEGF to detect Slit-lamp biomicroscopy with appropriate lenses neovascularization, pupillary assessment for a is recommended to evaluate retinopathy of the relative afferent pupillary defect, measurement posterior pole and midperipheral retina . Examination of IOP, stereoscopic exam of posterior pole after of the far peripheral retina is best performed using dilation, OCT imaging when appropriate, and indirect ophthalmoscopy peripheral retina and vitreous exam when indicated Diagnostic Tests • Ophthalmologist should refer patients with an RVO to a primary care physician for appropriate • Color red-free fundus photography for documenting management of their systemic condition and the severity of the retina findings, NVE, extent of communicate results to the physician managing the intravitreal hemorrhages and NVD patient’s ongoing care • Optical coherence tomography to detect the • Risk to the fellow eye should be communicated to presence and extent of macular edema, vitreoretinal both the primary care provider and the patient interface changes and subretinal fluid • Patients whose conditions fail to respond to therapy • Optical coherence tomography angiography to and when further treatment is unavailable should be detect capillary nonperfusion, enlarged foveal provided with professional support and offered a avascular zone, and vascular abnormalities referral for counseling, vision rehabilitation, or social • Fluorescein angiography to evaluate extent of services as appropriate (www .aao org/low-vision-. vascular occlusion, degree of ischemia, and extent of and-vision-rehab) macular edema • Ultrasonography (e g. ., when vitreous hemorrhage is present)

Initial Exam History (Key elements) Preoperative Care • Symptoms The ophthalmologist who is to perform the surgery • Ocular history has the following responsibilities: • Systemic history • Examine the patient preoperatively • Assessment of visual function status • Ensure that the evaluation accurately documents symptoms, findings, and indications for treatment • Medications currently used • Inform the patient about the risks, benefits, and expected outcomes of surgery, including Initial Physical Exam (Key elements) the anticipated refractive outcome or surgical • Visual acuity with current correction experience • Measurement of BCVA (with refraction when • Formulate surgical plan, including selection of IOL indicated) and anesthesia • External examination • Review results of presurgical and diagnostic • Ocular alignment and motility evaluations with the patient • Glare testing when indicated • Inform the patient about the possibility of visual • Pupil reactivity and function impairment continuing after cataract surgery, and the potential for rehabilitation (III, GQ, SR) • Measurement of IOP • Formulate postoperative plans and inform patient of • Slit-lamp biomicroscopy, including gonioscopy arrangements • Dilated examination of the lens, macula, peripheral • Answer patient’s questions regarding surgery, care, retina, optic nerve, and vitreous through a dilated pupil and cost • Assessment of relevant aspects of the patient’s • Routine preoperative laboratory testing in medical and physical status association with the history and physical examination is not indicated (I+, GQ, SR) Care Management • Treatment is indicated when visual function no Follow-up Evaluation longer meets the patient’s needs and cataract • High-risk patients should be seen within 24 hours of surgery provides a reasonable likelihood of quality- surgery of-life improvement • Routine patients should be seen within 48 hours of • Cataract removal is also indicated when there is surgery evidence of lens-induced disease or when it is • Frequency and timing of subsequent visits depend necessary to visualize the fundus in an eye that has on refraction, visual function, and medical condition the potential for sight of the eye • Surgery should not be performed under the • More frequent follow-up usually necessary for high following circumstances: risk patients - Tolerable refractive correction provides vision that • Components of each postoperative exam should meets the patient’s needs and desires; surgery is include: not expected to improve visual function, and no other indication for lens removal exists - Interval history, including new symptoms and use of postoperative medications - The patient cannot safely undergo surgery because of coexisting medical or ocular conditions - Patient’s assessment of visual function status - Appropriate postoperative care cannot be - Measurement of IOP arranged - Slit-lamp biomicroscopy - Patient or patient’s surrogate decision maker is - Operating ophthalmologist should provide unable to give informed consent for nonemergent postoperative care that is within the unique surgery competence of the ophthalmologist (III, GQ, SR) • Indications for second eye surgery are the same as for the first eye (with considerations given to needs for binocular function) • The standard of care in the United States is a small- incision phacoemulsification with foldable IOL implantation with either biaxial or coaxial approach (I+, GQ, SR)

Nd:YAG Laser Capsulotomy • Treatment is indicated when vision impaired by posterior capsular opacification does not meet the patient’s functional needs or when it critically interferes with visualization of the fundus • Educate about the symptoms of posterior vitreous detachment, retinal tears, and detachment and the need for immediate examination if these symptoms are noticed • The decision to perform capsulotomy should take into account the benefits and risks of the laser surgery . Laser posterior capsulotomy should not be performed prophylactically (i e. ., when the capsule remains clear) . The should be inflammatory-free and the IOL stable prior to performing Nd:YAG laser capsulotomy . (III, GQ, SR)

Initial Exam History - Chronic or unresponsive to broad spectrum • Ocular symptoms (e g. ., degree of pain, redness, antibiotic therapy . discharge, blurred vision, photophobia, duration of - History of corneal surgeries symptoms, circumstances surrounding the onset of - Atypical clinical features suggestive of fungal, symptoms) amœbic, or mycobacterial keratitis . • Contact lens history (e g. ., wearing schedule, - Infiltrates are in multiple locations on the cornea . overnight wear, type of contact lenses, contact lens solution, contact lens hygiene protocol, tap- • The hypopyon that occurs in eyes with bacterial water rinse of contact lenses, swimming, using a keratitis is usually sterile, and aqueous or vitreous hot tub, or showering while wearing contact lenses, taps should not be performed unless there is a high method of purchase, such as over the internet, and suspicion of microbial endophthalmitis, such as decorative contact lens use .) following an intraocular surgery, perforating trauma, or sepsis . • Review of other ocular history, including risk factors such as herpes simplex virus keratitis, varicella • Corneal scrapings for culture should be inoculated zoster virus keratitis, previous bacterial keratitis, directly onto appropriate culture media to maximize trauma, dry eye, and previous ocular surgery, culture yield . If this is not feasible, place specimens including refractive and facial (including laser in transport media . In either case, immediately cosmetic) surgery incubate cultures or take promptly to the laboratory . • Review of other medical problems, including Care Management immune status, systemic medications, and history of MRSA . • Topical antibiotic eye drops are capable of achieving high tissue levels, a preferred method of treatment • Current and recently used ocular medications in most cases . • Medication allergies • Single-drug therapy using a fluoroquinolone is as effective as combination therapy utilitizing fortified Initial Physical Exam antibiotics (I+, GQ, SR) There is no difference found in • Visual acuity corneal perforation rates across classes of topical • General appearance of patient, including skin antibiotics (I+, GQ, SR) conditions • Topical corticosteroid therapy may have a beneficial • Facial examination role, but much of the literature has not shown a difference in clinical outcome (I+, GQ, SR) • Globe position • Subconjunctival antibiotics may be helpful where • Eyelids and eyelid closure there is imminent scleral spread or perforation or • Conjunctiva where adherence is questionable . • Nasolacrimal apparatus • For central or severe keratitis (e g. ., deep stromal • Corneal sensation involvement or an infiltrate larger than 2 mm with extensive suppuration), use a loading dose • Slit-lamp biomicroscopy (e g. ., every 5 to 15 minutes), followed by frequent - Eyelid margins applications (e g. ., every hour is recommended .) - Conjunctiva Severe cases should be followed daily initially, at least until stable or improvement is confirmed . - Sclera • Systemic therapy may be useful in cases of scleral - Cornea or intraocular extension of infection of systemic - Anterior chamber for depth and the presence of infection such as gonorrhea . inflammation, including cell and flare, hypopyon, • For patients treated with ocular topical fibrin, hyphema corticosteroids at time of presentation of suspected - Anterior vitreous for the presence of inflammation bacterial keratitis, reduce or eliminate corticosteroids - Contralateral eye for clues to etiology as well as until infection has been controlled . possible similar underlying pathology • When the corneal infiltrate compromises the visual axis, may add topical corticosteroid therapy Diagnostic Tests following at least 2 to 3 days of progressive • Manage majority of community-acquired cases improvement with treatment with topical antibiotics with empiric therapy and without smears or typically after pathogen identification . cultures . • Examine patients within 1 to 2 days after initiation • Indications for smears and cultures: of topical corticosteroid therapy and monitor intraocular pressure . - Corneal infiltrate that is central, large, and/or associated with significant stromal involvement . • In general, modify the initial regimen if there is lack of improvement or stabilization within 48 hours .

Patient Education • Educate 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 ophthalmologist promptly if they experience such • Refer patients with significant visual impairment warning signs or symptoms or blindness for vision rehabilitation if they are not • Educate about the severe visual impairment from surgical candidates (see www .aao org/low-vision-. bacterial keratitis and need for strict adherence to and-vision-rehab) the therapeutic regimen • Discuss possibility of permanent visual loss and need for future visual rehabilitation

Antibiotic Therapy of Bacterial Keratitis

Topical Topical Subconjunctival Organism Antibiotic Concentration Dose No organism Cefazolin or Vancomycin 25-50 mg/ml 100 or 25 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‡ 10–50 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† Gram-positive Rods Amikacin 20–40 mg/ml 20 mg in 0 .5 ml (Nontuberculous Clarithromycin 10 mg/ml Mycobacteria) Azithromycin| 10 mg/ml Fluoroquinolones Various† Gram-positive Rods Sulfacetamide 100 mg/ml (Nocardia) Amikacin 20–40 mg/ml 20 mg in 0 .5 ml Trimethoprim/ Sulfamethoxazole: Trimethoprim 16 mg/ml Sulfamethoxazole 80mg/ml

Modified with permission from the American Academy of Ophthalmology Basic and Clinical Science Course Subcommittee . Basic Clinical and Science Course . External Disease and Cornea: Section 8, 2017-2018 . Table 10-6 . San Francisco: American Academy of Ophthalmology, 2017 . * Fewer gram-positive cocci are resistant to gatifloxacin, moxifloxacin, and besifloxacin than other fluoroquinolones . † Besifloxacin 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(6):819–30 .

Initial Exam History • Consult with the pathologist prior to obtaining the biopsy if sebaceous cell carcinoma is suspected . • Ocular symptoms and signs (e g. ., redness, irritation, burning, tearing, itching, crusting of eyelashes, loss Care Management of eyelashes, eyelid sticking, blurring or fluctuating vision, contact lens intolerance, photophobia, • Treat patients with blepharitis initially with a increased frequency of blinking and recurrent regimen of warm compresses and eyelid cleansing . hordeolum) • A topical antibiotic such as bacitracin or • Time of day when symptoms are worse erythromycin can be prescribed to be applied one or more times daily or at bedtime on the eyelid • Duration of symptoms margins for a few weeks . • Unilateral or bilateral presentation • For patients with meibomian gland dysfunction, • Exacerbating conditions (e g. ., smoke, allergens, whose chronic symptoms and signs are not wind, contact lenses, low humidity, retinoids, diet adequately controlled with eyelid cleansing or and alcohol consumption, eye makeup) meibomian gland expression, oral tetracyclines and • Symptoms related to systemic diseases (e g. ., topical antibiotics may be helpful . rosacea, atopy, psoriasis, and graft-versus-host • Topical azelaic acid, topical ivermectin, brimonidine, disease [GVDH]) doxycycline, and isoretinoin are effective treatments • Current and previous systemic and topical for patients with systemic rosacea . (I+, GQ, SR) medications (e g. ., antihistamines or drugs with • A brief course of topical corticosteroids may be anticholinergic effects, or drugs used in the past helpful for eyelid or ocular surface inflammation that might have an effect on the ocular surface [e g. ., such as severe conjunctival infection, marginal isotretinoin]) keratitis, or phlyclenules . The minimal effective dose • Recent exposure to an infected individual (e g. ., of corticosteroid should be utilized and long-term pediculosis palpebrarum [Pthirus pubis]) corticosteroid therapy should be avoided if possible . • Ocular history (e g. ., previous intraocular and eyelid • An eyelid tumor should be suspected in patients surgery, local trauma, including mechanical, thermal, with atypical eyelid-margin inflammation or disease chemical, and radiation injury, history of cosmetic not responsive to medical therapy, and these blepharoplasty, history of styes and/or chalazia) patients should be carefully re-evaluated .

Initial Physical Exam Follow-Up Evaluation • Visual acuity • Follow-up visits should include: • External examination - Interval history - Skin - Measurement of visual acuity - Eyelids - External examination • Slit-lamp biomicroscopy - Slit-lamp biomicroscopy - Tear film • If corticosteroid therapy is prescribed, re-evaluate patient within a few weeks to determine the - Anterior eyelid margin response to therapy, measure intraocular pressure, - Eyelashes and assess treatment compliance - Posterior eyelid margin - Tarsal conjunctiva (everting eyelids) Patient Education • Counsel patients about the chronicity and - Bulbar conjunctiva recurrence of the disease process . - Cornea • Inform patients that symptoms can frequently be improved but are rarely eliminated . Diagnostic Tests • Patients with an inflammatory eyelid lesion that • Cultures may be indicated for patients with appears suspicious for malignancy should be referred recurrent anterior blepharitis with severe to an appropriate specialist . inflammation as well as for patients who are not responding to therapy . • Biopsy of the eyelid to exclude the possibility of carcinoma may be indicated in cases of marked asymmetry, resistance to therapy or unifocal recurrent chalazia that do not respond well to therapy .

Initial Exam History masses, discharge) • Ocular symptoms and signs (e g. ., mattering and • Slit-lamp biomicroscopy adherence of eyelids, itching, tearing, discharge, - Eyelid margins (inflammation, edema, irritation, pain, photophobia, blurred vision) hyperpigmentation, meibomian gland dysfunction, • Duration of symptoms and time course ulceration, discharge, nodules or vesicles, blood- • Exacerbating factors tinged debris, keratinization) • Unilateral or bilateral presentation - Eyelashes (loss of lashes, crusting, scurf, mites, nits, lice, trichiasis) • Character of discharge - Lacrimal puncta and canaliculi (pouting, discharge, • Recent exposure to an infected individual edema) • Trauma (mechanical, chemical, ultraviolet) - Tarsal and forniceal conjunctiva • Recent surgery - Bulbar conjunctiva/limbus (follicles, edema, • Mucus fishing behavior (i e. ., repetitive manipulation nodules, chemosis, laxity, papillae, ulceration, and wiping of the conjunctiva leading to mechanical scarring, phlyctenules, hemorrhages, foreign irritation) material, keratinization) • Contact lens wear (lens type, hygiene and use - Cornea regimen) - Dye-staining pattern (conjunctiva and cornea) • Symptoms and signs potentially related to systemic - Anterior chamber/iris (inflammation reaction, diseases (e g. ., genitourinary discharge, dysuria, synechiae, transillumination defects) dysphagia, upper respiratory infection, skin and mucosal lesions) Diagnostic Tests • Allergy, asthma, eczema • Cultures, smears for cytology and special stains are • Use of topical and systemic medications indicated in cases of suspected infectious neonatal • Ocular history (e g. ., previous episodes of conjunctivitis. conjunctivitis and previous ophthalmic surgery) • Smears for cytology and special stains are • Compromised immune status (e g. ., HIV, recommended in cases of suspected infectious chemotherapy, immunosuppressents) neonatal conjunctivitis, chronic or recurrent conjunctivitis, and gonococcal conjunctivitis in any • Current and prior systemic diseases (e g. ., atopy, age group . SJS/TEN, carcinoma, leukemia, chickenpox, GVHD) • Confirm diagnosis of adult and neonate chlamydial • Social history (e g. ., smoking habits, exposure to conjunctivitis with laboratory testing . second hand smoke, occupation and hobbies, exposure to air pollutants, travel, exercise habits, • Biopsy the bulbur conjunctiva and take a sample diet, use of illicit drugs, and sexual activity) from an eye with active inflammation when ocular mucous membrane pemphigoid is suspected . Initial Physical Exam • A full-thickness lid biopsy is indicated in cases of • Visual acuity suspected sebaceous carcinoma . • External examination • Thyroid function tests are indicated for patients with SLK who do not have known thyroid disease . - Regional lymphadenopathy, particularly preauricular - Skin (signs of rosacea, eczema, seborrhea) - Abnormalities of the eyelids and adnexae (swelling, discoloration, malposition, laxity, ulceration, nodules, ecchymosis, neoplasia, lateral flare, lash loss) - Orbits: fullness, asymmetry - Conjunctiva (laterality, type of conjunctival reaction, distribution, subconjunctival hemorrhage, chemosis, cicatricial change, symblepharon,

Care Management Follow-Up Evaluation • The majority of cases in the adult popoulation • Follow-up visits should include are viral and self-limited, and do not require - Interval history antimicrobial treatment . There is no proven effective treatment for eradication of adenovial infection; - Visual acuity artificial tears, topical antihistamines, topical - Slit-lamp biomicroscopy steroids, oral analgesics or cold compresses may • If corticosteroids are used, perform periodic mitigate symptoms . The use of antibiotics should measurement of intraocular pressure and pupillary be avoided because of potential adverse treatment dilation to evaluate for cataract and glaucoma effects . • Allergen-specific immunotherapy is beneficial in Patient Education reducing allergic conjunctivitis, more in children than • Counsel patients with contagious varieties to mini- in adults (I+, GQ, SR) mize or prevent spread of disease and encourage • Treat mild allergic conjunctivitis with an over-the- minimization of contact with other people for 10 to counter antihistamine/vasoconstrictor agent or 14 days after onset of symptoms (I+, GQ, SR) in the second-generation topical histamine H1-receptor community antagonists . (I+, GQ, SR) If the condition is frequently • Inform patients who may require repeat short-term recurrent or persistent, use mast-cell stabilizers (I++, therapy with topical corticosteroid of potential GQ, SR) complications of corticosteroid use • Treatment for vernal/atopic conjunctivitis include • Advise patients with allergic conjunctivitis that modifying the environment and use of cold frequent clothes washing and bathing/showering compresses and ocular lubricants . For acute before bedtime may be helpful exacerbations, topical corticosteroids are usually needed . Topical cyclosporine is shown to be effective for severe cases . (I+, GQ, SR) • For contact lens-related keratoconjunctivitis, discontinue contact lens wear until the cornea returns to normal • In severe cases, topical cyclosporine or tacrolimus can be considered (I+GQ, DR) • Use systemic antibiotic treatment for conjunctivitis due to Neisseria gonorrhoeae or Chlamydia trachomatis • 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 • Refer patients with manifestation of a systemic disease to an appropriate medical specialist

Initial Exam History • Lamellar keratoplasty using DALK techniques • Disease onset and course can be considered for contact-lens-intolerant patients without significant scarring at Descemets • Vision impairment membrane or persistent hydrops . Crescentic lamellar • Ocular, medical, and family history keratoplasty is an option when maximal thinning is in the cornea’s periphery . Initial Physical Exam • Penetrating keratoplasty is indicated when a • Visual function assessment patient can no longer achieve functional vision • External examination with eyeglasses and contact lenses and CXL is contraindicated, or when persistent corneal edema - Eyelids and eyelid skin occurs following hydrops . Descemet stripping • Slit-lamp biomicroscopy endothelial keratoplasty cannot correct ectatic - Presence, extent, and location of corneal thinning disorder . or protrusion • Penetrating keratoplasty (PK) is preferred over - Indication of previous ocular surgery DALK in cases of deep stromal scarring . Overall, there is insufficient evidence to determine which - Presence of Vogt striae, prominent corneal nerves, tecnique offers better overall outcomes . (I+, GQ, DR) Fleischer ring, or other iron deposition • A lamellar graft can be performed for tectonic - Evidence of corneal scarring or previous hydrops, support when ectasia occurs in the far periphery of and presence of prominent corneal nerves the cornea and additional PK can be performed for • IOP measurement visual rehabilitation . • Fundus examination: assessment of red reflex for dark area, and retina for tapetoretinal degenerations Follow-Up Evaluation • Follow-up visits should include: Diagnostic Tests - Interval history • Keratometry - Visual acuity - External examination • Corneal topography and tomography - Slit-lamp biomicroscopy - Topographic power map - Assessment of corneal contour and thickness by - Topographic elevation map and tomography topography and tomography - Measurement of corneal thickness • Optical coherence tomography (OCT) • With the advent of CXL, more frequent follow-up Care Management (i e. ., 3-6 months) for progression is now indicated . • Therapy is tailored to the individual patient, Patient Education depending on the visual impairment and a risk/ benefit analysis of each treatment option(s) . • Counsel all patients to avoid eye rubbing . • Vision can be corrected with eyeglasses, but contact • Discuss the benefits and potential risks of early lenses may be required as keratoconus progresses crosslinking in patients at high risk for progression to correct vision and reduce distortion . or who historically have noted progressive loss of vision . • Rigid corneal gas permeable contact lenses can mask corneal irregularities . Hybrid contact lenses • Patients undergoing corneal transplantation should provide higher oxygen permeability and greater be made aware of the warning signs of rejection RGP/hydrogel junction strength . Piggyback contact and should seek medical attention promptly if lenses may be employed for greater comfort and less symptoms occur . The practitioner should be aware epithelial disruption . Scleral lenses may be indicated of the slit-lamp biomicroscopic findings of epithelial, when RGP and/or hybrid contact lenses fail . stromal, and endothelial rejection . • Intrastromal corneal ring segment implantation can improve contact lens tolerance and BCVA for patients with corneal ectasia, a clear cornea, and contact lens intolerance . • Cross-Linking (CXL) has long term data supporting its safety and stability and should be considered for patients with early Keratoconus and at risk of progression to arrest or slow progression in its earliest stage .

Initial Exam History • Slit-lamp biomicroscopy • Symptoms and signs: blurred or variable vision often - Unilateral or bilateral signs with a diurnial character; photophobia; redness; - Diffuse or localized edema tearing; intermittent foreign body sensation; intense, disabling, or task-disrupting pain - Primarily epithelial or stromal edema • Recent history of other ocular surgery - Evidence of epithelial breakdown, stromal infiltration, epithelial ingrowth, striae, focal • Age of onset thickening, thinning, scarring, interface haze, striae • Rapidity of onset: acute symptoms vs . gradual or or inflammation, or stromal vascularization or fluctuating deposits • Persistence: transient or permanent - Evidence of guttae, Descemet’s membrane • Unilateral or bilateral presentation tear or detachment, endothelial vesicles, keratic precipitates (KP), pigment, peripheral anterior • Moderating factors or situations synechiae • Past ocular and medical history - Involvement of host tissue, if there is a corneal • Topical and systemic medications transplant • Trauma: blunt or penetrating injury to eye or - Evidence of sectoral corneal edema and a cluster periocular region, forceps delivery, chemical injury line of KP, or an anterior chamber reaction • Contact lens wear: rationale, type of lens, wear time, - Status, shape, and position of the pupil and iris and cleaning routine - Status and position of the crystalline lens or IOL • Family and social history and any other intraocular device - Evidence of past keratorefractive procedures Initial Physical Exam - Healed or recent corneoscleral wounds, areas • Visual function assessment of scleral thinning associated with previous - Comparison of visual acuity measurement and surgery, surgical devices, and signs of intraocular functional status inflammation . - Glare testing • IOP measurement • External examination • Fundus examination - Evidence of proptosis, ptosis, lagophthalmos, or • Gonioscopy floppy eyelid syndrome - Eyelid or facial asymmetry, scarring, and Diagnostic Tests malfunction • Potential acuity meter - Miscellaneous (e g. ., pupil responses, corneal • Rigid contact lens over-refraction diameter, dry eye evaluation) • Pachymetry • Topography • Specular microscopy • Confocal microscopy • Anterior segment optical coherence tomography • Ultrasound biomicroscopy

Care Management - Pressure patching used to be standard treatment, • Therapeutic goal is to control the cause of corneal but a recent study found that this does not edema or opacity and enhance a patient’s quality of positively impact comfort or speed of healing (I+, life by improving visual acuity and comfort GQ, DR) • In most cases treatment starts with medical - Progressive thinning of cornea or a small management, when this is insufficient, surgery may perforation usually requires structural support be considered with application of a tissue adhesive . • Corneal edema: medical management - Topical corticosteroids are often used to reduce intraocular and corneal inflammation . IOP and - Lowering an elevated IOP is helpful cataract formation should be monitored with - Topical carbonic anhydrase inhibitors should long-term topical corticosteroid use . not be the first line of therapy when endothelial - A rigid gas permeable lens — or hybrid or scleral dysfunction is suspected lens when greater stability is needed — will often - Topical corticosteroid can control inflammation improve vision when surface irregularity is a once infection has been ruled out or controlled factor; such lenses may preclude the need for - Microcystic or bullous epithelial disease may more invasive procedures produce discomfort or pain necessitating the • Corneal opacification: surgical management placement of a bandage contact . Thin lenses with - Surgical strategy for managing corneal opacities high water content and high oxygen diffusion depends on the tissue layer(s) involved: coefficients may be the most advantageous . ° Superficial keratectomy may be indicated for - Supportive management should be initiated to removal of superficial deposits reduce inflammation and/or pain in cases of acute hydrops ° Lamellar keratoplasty may be indicated for removal of deeper deposits • Corneal edema: surgical management ° Penetrating keratoplasty may be indicated for - Patients with corneal edema and persistent removal of even deeper multilevel opacities discomfort, but limited or no visual potential, are generally better candidates for the following ° Ethylenediaminetetraacetic acid (EDTA) may be procedures: used to remove calcific band keratopathy (III, IQ, DR) ° Conjunctival flap ° Amniotic membrane transplantation Follow-Up Evaluation ° A number of scarification procedures • In the management of corneal edema, the goal of ° Corneal transplantation follow up is to monitor endothelial dysfunction ° Endothelial keratoplasty • In the management of corneal opacification, follow - For patients wtih persistent corneal edema, up to monitor corneal clarity and degree of surface a number of keratectomy and keratoplasty irregularity is necessary procedures can be considered . • Coexisting problems, particularly intraocular • Corneal opacification: medical management inflammation and IOP, need regular reassessment - Corneal opacity treatment can be divided into Patient Education two phases: a) management of the principal, initiating process (i e. ., infection, trauma), and • Provide an understanding of balanced expectations b) management of the resulting problems (i e. ., of the amount of visual function that can realistically surface erosions and irregularity, scarring, thinning, be preserved or recovered and risk of complications . and vascularization) • Detailed discussion of the causes of edema or - Conventional treatment involves an antibiotic drop opacity, and various treatment options, is important . or ointment to protect against secondary bacterial • When the disease process or management is infection complex, every effort should be made to counsel - Temporary tarsorraphy with botulinum toxin, or the patient regarding such challenges to allow for suture can be helpful when blinking or lid closure appropriate expectations and informed decision- is inadequate making . - A bandage contact lens or amniotic membrane • There is a commercially available point-of-care test may be useful in cases of delayed healing to identify Avellino dystrophy in keratorefractive surgery candidates if either family history or clinical findings are inconclusive for this condition .

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

Diagnostic Tests - Contact lenses • Tear Break-up Time - Correction of eyelid abnormalities • Ocular Surface Dye Staining - Permanent punctal occlusion • Schirmer Test - Tarsorrhaphy • Fluorescein Dye Disappearance Test/Tear Function • Monitor patients prescribed corticosteroids for Index adverse effects such as increased intraocular • Tear Osmolarity Test pressure, and cataract formation

Care Management Follow-Up Evaluation • Treat any causative factors that are amenable to • Purpose is to assess response to therapy as a basis treatment as patients with dry eye symptoms often for altering or adjusting treatment as necessary, to have many contributory factors monitor for ocular surface damage, and to provide reassurance . • Specific therapies may be chosen from any category (see Table) regardless of the level of • Frequency and extent will depend on the severity disease severity, depending on physician experience of disease, therapeutic approach and response to and patient preference therapy . • Artificial tears are safe and effective (I+, GQ, SR) Patient Education • Corticosteroids can decrease ocular irritation • Patient education is an important aspect of symptoms, decrease corneal fluorescein staining, successful management and improve filamentary keratitis (I+, GQ, SR) • Counsel patients about the chronic nature of dry • Silicone plugs may provide symptomatic relief in eye and its natural history . patients with severe dry eye (I+, GQ, DR) • Set and discuss realistic expectations for therapeutic • Autologous serum tears may improve ocular goals irritation symptoms compared with artificial tears in the short-term • Provide specific instructions for therapeutic regimens . • For mild dry eye, the following measures are • Reassess periodically the patient’s compliance and appropriate: understanding of the disease, risks for associated structural changes and realistic expectations for - Education and environmental modifications effective management, and reinforce education . - Elimination of offending topical or systemic • Refer patients with manifestation of a systemic medications disease to an appropriate medical specialist . - Aqueous enhancement using artificial tear • Caution patients with pre-existing dry eye that substitutes, gels/ointments keratorefractive surgery, particularly LASIK, may - Eyelid therapy (warm compresses and eyelid worsen their dry eye condition . hygiene) - Treatment of contributing ocular factors such as blepharitis or meibomianitis - Correction of eyelid abnormalities • 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 - Spectacle side shields and moisture chambers • For severe dry eye, in addition to above treatments, the following measures are appropriate: - Systemic cholinergic agonists - Systemic anti-inflammatory agents - Mucolytic agents - Autologous serum tears

Initial Exam History (Key elements) Follow-Up Evaluation • Ocular symptoms and signs • Follow-up visits should include: • Ocular history - Interval history • Systemic history, birth weight, gestational age, - Adherence to treatment plan prenatal and perinatal history, past hospitalizations - Side effects of treatment and operations, and general health and development - Visual acuity of each eye • Family history of eye conditions and relevant • Follow-up examination generally arranged 2 to systemic diseases 3 months after initiation of treatment • Timing varies according to intensity of treatment Initial Physical Exam (Key elements) and age of child • A Binocular red reflex (Brückner) test • Continued monitoring required because about one- fourth of children successfully treated experience a • Binocularity/stereoacuity testing recurrence within the first year after treatment has • Assessment of visual acuity and/or fixation pattern stopped • Binocular alignment and ocular motility Patient Education • Cycloplegic retinoscopy/refraction with subjective refinement when indicated • Discuss diagnosis, severity of disease, prognosis and treatment plan with patient, parents and/or • Funduscopic examination caregivers • Explain the disorder and recruit the family in a Care Management collaborative approach to therapy • All children with amblyopia should be offered an attempt at treatment regardless of age • Choose treatment based on patient’s age; visual acuity; adherence and response to previous treatment; and physical, social, and psychological status • Treatment goal is equal visual acuity between the two eyes • Once maximal visual acuity has been obtained, treatment should be tapered and eventually stopped

Initial Exam History (Key elements) Care Management • Ocular symptoms and signs • Consider all forms of esotropia for treatment and • Ocular history (date of onset and frequency of re-establish binocular alignment as soon as possible the deviation, presence or absence of diplopia, • Prescribe corrective lenses for any clinically squinting, closing one eye, or other visual significant refractive error as initial treatment symptoms) • If eyeglasses and amblyopia management are inef- • Systemic history, birth weight, gestational age, fective in aligning the eyes, then surgical correction prenatal and perinatal history, past hospitalizations is indicated and operations, and general health and development • Start amblyopia treatment before surgery because surgical treatment of esotropia in the presence of • Family history (strabismus, amblyopia, type of moderate to severe amblyopia has a lower success eyeglasses and history of wear, extraocular muscle rate than in the presence of mild or no amblyopia surgery, or other eye surgery, and genetic diseases) • Social history (e g. ., grade level in school, learning Follow-Up Evaluation difficulties, behavior problems, or issues with social • Periodic evaluations necessary because of risk of interactions) developing amblyopia losing binocular vision, and recurrence Initial Physical Exam (Key elements) • Children who are well-aligned and do not have • Verification of eyeglass correction with a lensometer amblyopia may be followed every 4 to 6 months • Binocular alignment at distance and near in • Frequency of follow-up visits can be reduced as the primary gaze, up and down gaze, and horizontal child matures gaze positions, if possible; if eyeglasses are worn, alignment testing should be performed with • New or changing findings may indicate need for correction more frequent follow-up examinations • Extraocular muscle function (ductions and versions, • Hyperopia should be assessed at least annually including incomitance such as found in some A and and more frequently if visual acuity decreases or V patterns) esotropia increases • Detection of latent or manifest nystagmus • Repeat cyclopegic refraction is indicated when esotropia does not respond to initial prescription • Sensory testing, including fusion and stereoacuity of hyperopic refraction or when esotropia recurs • Cycloplegic retinoscopy/refraction after surgery • Fundoscopic examination Patient Education • Monocular and binocular optokinetic nystagmus testing for nasal-temporal pursuit asymmetry • Discuss findings with the patient when appropriate and/or parents/caregivers to enhance understanding of disorder and to recruit them in a collaborative approach to therapy • Formulate treatment plans in consultation with the patient and/or family/caregivers

Initial Exam History (Key elements) Care Management • Ocular symptoms and signs • All forms of exotropia should be monitored and • Ocular history (date of onset and frequency of the some will require treatment deviation, presence or absence of diplopia, • Young children with intermittent exotropia and good squinting, closing one eye, or other visual fusional control can be followed without surgery symptoms) • Deviations that are present most or all of the time • Systemic history, birth weight, gestational age, require treatment prenatal and perinatal history, past hospitalizations • Prescribe corrective lenses for any clinically and operations, and general health and significant refractive error that caused reduced development vision in one or both eyes • Family history (strabismus, amblyopia, type of • Optimal therapy for exotropia, the long-term benefit eyeglasses and history of wear, extraocular muscle of early surgical correction, and the relative merits surgery, or other surgery, and genetic diseases) of bilateral versus unilateral surgery are not well • Social history (e g. ., grade level in school, learning established difficulties, behavior problems, or issues with social • Amblyopia is uncommon in patient with intermittent interactions) exotropia, but, if present, should be treated

Initial Physical Exam (Key elements) Follow-up Evaluation • Sensory testing, including fusion and stereoacuity • Frequency of follow-up evaluations is based on age • Verification of eyeglass correction with a lensometer of child, ability to obtain an accurate visual acuity, • Binocular alignment at distance and near in and control of the deviation primary gaze, up and down gaze, and horizontal • Children with good fusional control of intermittent gaze positions, if possible; if eyeglasses are worn, exotropia and without amblyopia are typically alignment testing should be performed with examined every 6 to 12 months correction • By age 7 to 10 years, the frequency of exams may be • Extraocular muscle function (ductions and versions, reduced including incomitance such as found in some A and • Includes frequency of deviation, adherence to V patterns) treatment (if any), and assessment of ocular motility • Detection of latent or manifest nystagmus and update of refractive correction, if needed • Cycloplegic retinoscopy/refraction Patient Education • Fundoscopic examination • Discuss findings with the patient when appropriate • Monocular and binocular optokinetic nystagmus and/or parents/caregivers to enhance understanding testing for nasal-temporal pursuit asymmetry of disorder and recruit them in a collaborative approach to therapy • Formulate treatment plans in consultation with the patient and/or family/caregivers

Initial Exam History Patient Education • Present status of visual function Discuss the risks and benefits of the planned [A:III] • Ocular history procedure with the patient . Elements of the discussion include the following: • Systemic history • Range of expected refractive outcomes • Medications • Residual refractive error Initial Physical Exam • Reading and/or distance correction postoperatively • Distance visual acuity with and without correction • The limitations of keratorefractive surgery with • Manifest, and when appropriate, cycloplegic respect to presbyopia and the potential loss of refraction uncorrected near visual function that accompanies myopia correction • Computerized corneal topography/tomography • Monovision advantages and disadvantages (for • Central corneal thickness measurement patients of presbyopic age) • Evaluation of tear film and ocular surface • Loss of best-corrected visual acuity • Evaluation of ocular motility and alignment • Side effects and complications (e g. ., microbial keratitis, sterile keratitis, keratectasia) Care Management • Changes in visual function not necessarily measured • Discontinue contact lenses before preoperative by visual acuity testing, including glare and function exam and procedure under low-light conditions • Inform patient of the potential risks, benefits, and • Night vision symptoms (e g. ., glare, haloes) alternatives to and among the different refractive developing or worsening; careful consideration procedures should be given to this issue for patients with high • Document informed consent process; patient should degrees of ametropia or for individuals who require be given an opportunity to have all questions a high level of visual function in low-light conditions answered before surgery • Effect on ocular alignment • Check and calibrate instrumentation before the • Development or exacerbation of dry eye symptoms procedure • Recurrent erosion syndrome • Surgeon confirms the identity of the patient, the operative eye, and that the parameters are correctly • Advantages and disadvantages of same-day entered into the laser’s computer bilateral keratorefractive surgery versus sequential surgery . Because vision might be poor for some time after bilateral same-day photorefractive Postoperative Care keratectomy, the patient should be informed that • Operating surgeon is responsible for postoperative activities such as driving might not be possible for management weeks . • For surface ablation techniques, examination on the • Possibility that it may influence predictive accuracy day following surgery is advisable and every 2 to of IOL calculations for subsequent cataract surgery 3 days thereafter until the epithelium is healed • Postoperative care plans (setting of care, providers • For uncomplicated LASIK, examine within 36 hours of care) following surgery, a second visit 1 to 4 weeks post • Loss of uncorrected near vision in myopic operatively, and further visits thereafter as presbyopes appropriate • Provide patients with a record or that the ophthalmologist maintains a record that lists the patient's eye condition, including preoperative keratometry readings and refraction, as well as stable postoperative refractions, so that it will be available if the patient requires cataract surgery or additional eye care

Initial Exam History Management Plan • Ocular misalignment • Patient should be monitored/observed if symptoms • Strabismus angle and direction are mild, occasional, and well tolerated or if patient is opposed to treatment • History of chronicity, review past clinical, surgical and imaging records • Consider if alignment might be improved with changing optical correction (e g. ., correction of Initial Physical Exam hyperopia and appropriate bifocal or progressive lenses for adults approaching presbyopia) • Optical corrections and presence of ground-in or overlay prism, and impact current correction has on • Reversal of monovision may be necessary and may alignment resolve symptoms • Manifest refraction to identify barriers to binocular • Prisms to address some forms of diplopia, and alignment or fusion orthoptic exercises to address some forms of diplopia and asthenopia can be considered • Assessment of alignment by light reflex testing (e g. ., Krimsky) to compare with cover test and Surgical and Postoperative Care identification of abnormal angle kappa • Correction of childhood strabismus in adults is • Dry manifest and cycloplegic refraction, providing generally surgical but, because a broad range of clues to original oculomotor disturbance conditions may be responsible, specifics of surgery • Complete motility examination, including cover- will vary uncover, alternate-cover testing, testing for binocular • Surgery is often challenging because of pre-existing fusion and stereopsis . surgical scarring, uncertainty about extraocular • Inspection of the ocular surface for conjunctival muscle attributes and location, possible limited scars (prior incision sites) and exposure of the fusional skills thinned sclera behind anatomical insertions • Sequelae of previous surgery should be addressed (evidence of likely muscle recession) to optimize postoperative alignment • Inspection of the interpalpebral fissures for evidence of prior vertical or horizontal rectus muscle Patient Education and Follow-up resection (smaller interpalpebral fissure) or recession (larger interpalpebral fissure) • Patients should be informed about the disorder and management options, as well as the adaption to the • Prism testing to simulate desired postsurgical new ocular alignment resulting from surgery alignment and range of overcorrection and undercorrection comfortably tolerated and unlikely • Inform the patient’s other health care providers to result in diplopia about the diagnosis and treatment plan • Assessment for ocular torsion by sensory testing or anatomic evidence of torsion noted during indirect ophthalmoscopy, particularly in patients with vertical strabismus • Imaging (e g. ., CT, MRI, orbital ultrasound) although nearly all cases can be managed without imaging

* Please refer to the Adult Strabismus Preferred Practice Patterns for care process of other forms of adult strabismus