Pediatric Clinical Pearls. Valerie M. Kattouf O.D., FAAO, FCOVD Illinois College of Optometry Chicago, IL

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1 Pediatric Clinical Pearls Valerie M. Kattouf O.D., FAAO, FCOVD Illinois College of Optometry Chicago, IL

2 Pediatric Clinical Pearls Collecting exam data / Modification of exam techniques Determining the best treatment options Pediatric Refractive Error Amblyopia Strabismus

3 Collecting Exam Data How do you modify the tests you already know how to do?

4 Direct Observation

5 Visual Acuity Assessment

6 Fixate and Follow Target: Transilluminator / Face / Toy Evaluate: Response to light Note: Lid closure to bright light cortical function Tracking ability Comparison of ability of each eye When delayed Possible prematurity Possible generalized motor delay Possible Visual System Diagnosis

7 Fixate and Follow Procedure Cover one eye Examiner hand/thumb, Parent s hand Light directed at patient s face Obtain fixation with non-occluded eye May tap/flash/move light Recording: F&F = able to fixate and follow target CSM Central light reflex = central Steady eye maintains alignment with stationary and moving object Maintenance under binocular conditions does previously fixating eye maintain fixation

8 Forced Choice Visual Acuity Tests

9 Lea Symbols

10

11

12 Ocular Motility

13 Refractive Error Assessment

14 Pediatric Trial Frame Use

15 Administration of Cycloplegic Agent Cycloplegic Spray 0.5% Tropicamide 0.5% Cyclogel 2.5% Phenylephrine O BRIEN Pharmacy

16 Cycloplegic Spray Shelf life approved for 120 days (stability and potency) Stable past that FDA typically approves no more than 30 days Can vary percentages based on needs of practice

17 Prescribing for Children Issues to consider: Age Visual Function Refractive Error Norms Amblyogenic Risk Factors Birth History Family History Developmental History

18 Refractive Error Norms Highest rate of emmetropization 1 st months Hyperopia Average refractive error in infants = +2 D > 1.50 diopters hyperopia at 5 years old often remain hyperopic

19 Refractive Error Norms Myopia 25% of infants are myopic Myopic Newborns 7 years 54% still 7 years 46% 7 years no hyperopia

20 Refractive Error Norms Astigmatism Against the rule astigmatism more prevalent switches to with-the-rule with development At 3 1/2 years old astigmatism is at adult levels

21 Case Example 22 month old Hispanic Male

22 22 month old Hispanic Male 3 Visits/ First at 14 months Spina bifida w/ hydrocephalus Significant Developmental Delay (+) OT/PT/Speech/Developmental Therapy Asthma No visual complaints VA: F&F OD, OS NCT: Orthophoria

23 22 month old Hispanic Male Cycloplegic Retinoscopy : 14 months old x 180 OU 18 months old x 180 OU 22 months old x 180 OU

24 22 month old Hispanic Male Assessment/Plan Hyperopic Astigmatism OU Above age appropriate Significant developmental delays Rx given = x 180 OU Follow-up 3-4 months

25 Refractive Error and Strabismus

26 Strabismus Evaluation

27 Detection of Strabismus Bruckner Evaluation Kappa / Hirschberg infants/toddlers/deep amblyopes Cover Test preschool age

28 Bruckner Test Procedure use direct 1M from pt (scope plano) Room is dark / Pt looks at light Look through the scope as you shine the light at the bridge of the pts nose With the pt optically corrected look at the orange - red retinal reflexes Compare the color and brightness btw the 2 eyes WHITER & BRIGHTER REFLEX : STRABISMIC EYE

29 Hirschberg/ Kappa Evaluation

30 Hirschberg / Kappa Test Procedure Hirschberg Patient is binocular Shine light at bridge of nose, pt fixates penlight Evaluate placement of the corneal reflexes in relation to the center of the pupil (OD, OS) Follow with < Kappa - Patient monocular Evaluate placement of the corneal reflexes in relation to the center of the pupil (OD, OS)

31 HIRSHBERG / KAPPA TESTS Information gathered direction of strabismus = exotropia Laterality = alternating estimation of magnitude ~ 25 estimation of frequency = intermittent

32 HIRSHBERG / KAPPA TESTS Information gathered direction of strabismus = esotropia Laterality = alternating estimation of magnitude ~ 40 (variable) estimation of frequency = constant

33 Krimsky Test

34 Krimsky Test (quantifies Hirschberg) Procedure Perform Hirshberg Place neutralizing prism in front of fixating eye Add prism until the corneal reflex in the deviating eye looks symmetrical with that of the fixating eye The amount of prism necessary to achieve this = the Magnitude of the Deviation

35 Kappa/Hirschberg/Krimsky

36 Cover Test

37 Pseudoesotropia

38 Refractive Error and Strabismus

39 Case Example 4 y.o African American Male Twins

40 4 year old twins Case History Premature 2lbs, 2 ounces each Born at 24 weeks 2 weeks in NICU, no report of ROP No developmental delay is reported Mom notes difficulty with distance vision and a close working distance in both kids Mom notes eye turn out in Twin #1 only Have had previous exams and school screenings with no treatment recommendations

41 Twin #1 Visual acuity 20/60 OU, poor response to occlusion EOM FROM OD, OS Cover Test 20 RX(T), 20 RX(T) Lang Stereopsis = poor response Retinoscopy ~ x 180 OD x 180 OS Ocular Health evaluation = normal

42 Assessment / Plan Twin #1 Assessment Anisometropic Amblyopia likely OD Anisometropic Myopia OD>>OS Intermittent Exotropia OD Plan Rx given x 180 OD x 180 OS RTC 1 month after Rx dispense Repeat VA OD, OS, stereo, Re-evaluate CT cc, determine need for occlusion tx

43 Twin #2 Visual acuity 20/70 OU, poor response to occlusion EOM FROM OD, OS Cover Test ortho, no strabismus noted Lang Stereopsis = poor response Retinoscopy ~ D sphere D Sphere Ocular Health evaluation = normal

44 Assessment / Plan Twin #2 Assessment Isometropic Amblyopia possible High Myopia OU Plan Rx given D D RTC 1 month after Rx dispense Repeat VA OD, OS, stereo, Re-evaluate CT sc and cc

45 Case Example 11 month old Hispanic Male

46 Astigmatism Case Example Age = 11 month old hispanic male Systemic History Microcephaly Microcephaly is a medical condition in which the circumference of the head is smaller than normal because the brain has not developed properly or has stopped growing. Microcephaly can be present at birth or it may develop in the first few years of life. Seizure disorder Kepra d/c at 12 months Developmental delay (+)OT and (+)PT

47 Astigmatism Case Example Visit #1, 11 month old male Visual acuity fixate and follow OD, OS EOM FROM OD, OS Kappa / Hirschberg ortho, no X(T) noted with prolonged dissociation Bruckner = (+) Bifixation Cycloplegic Retinoscopy ~ plano x 180 OD, OS Ocular Health evaluation = normal

48 Astigmatism Case Example Visit #2, 14 month old male (3 month FU) Visual acuity fixate and follow OD, OS EOM FROM OD, OS Kappa / Hirschberg ortho, no X(T) noted with prolonged dissociation Bruckner = (+) Bifixation Cycloplegic Retinoscopy ~ plano x 180 OD, OS Ocular Health evaluation = normal

49 Astigmatism Case Example Visit #3, 17 month old male, 3 month FU Visual acuity fixate and follow OD, OS EOM FROM OD, OS Kappa / Hirschberg 30 X(T) Only noted with prolonged dissociation Low frequency, good fusion Cycloplegic Retinoscopy ~ plano x 180 OD, OS Rx given plano x 180 OD, OS

50 Astigmatism Case Example Visit #4, 19 month old male (2 month FU with RX) Per Mom: loves Rx, does not take them off, more alert and active Kappa / Hirschberg 20 X(T) Only noted with prolonged dissociation Very low frequency, good fusion RTC 4 months

51 Intermittent Exotropia

52 Studies evaluating IXT Treatment Options Treatment options in intermittent exotropia: a critical appraisal (Optom Vis Sci 1992 may;69 (5): ) Review of clinical literature Over minus Lens Therapy: 28% Prism Therapy: 28% Occlusion therapy: 37% EOM surgery: 46% Orthoptic vision therapy: 59%

53 Studies evaluating Overminus Lens Tx for IXT Overcorrecting minus lens therapy for treatment of intermittent exotropia (Ophthalmology 1983 Oct;90 (10):1160-5) Goal of tx: secure in quality of fusion 35 children -2D - - 4D x 18 mo. 46% improved quality of fusion during therapy 26% improved quality of fusion and quantitative decrease in angle of deviation 28% inadequate improvement in quality of fusion or decrease in angle size

54 Studies evaluating Overminus Lens Tx for IXT Refractive Error Changes in children with intermittent exotropia under overminus lens therapy (Arq Bras Oftalmol Nov-Dec;72(6):751-4) Goal of study: Does accommodation (used with accommodative convergence) increase myopia? Record review Conclusion: Treatment of IXT did not induce refractive error changes, even considering age, treatment period, initial spherical equivalent and overcorrection magnitude used

55 Amblyopia

56 DEFINITION OF FUNCTIONAL AMBLYOPIA Unilateral (infrequently bilateral) condition BVA < 20/20 No structural or pathologic anomalies 1 of the following occurring before age 6: Amblyogenic anisometropia Constant unilateral strabismus Amblyogenic bilateral isometropia Amblyogenic uni / bi astigmatism Image degradation

57 POTENTIALLY AMBLYOGENIC REFRACTIVE ERRORS ISOMETROPIA Astigmatism Hyperopia Myopia ANISOMETROPIA Astigmatism Hyperopia Myopia DIOPTERS > 2.50 D > D > D > 1.50 D > D > D 57

58 Determining the Best Treatment Options

59 Amblyopia Treatment PEDIG studies simplified

60 ATS Summary ATS 1-2B

61 ATS Summary ATS 3-6

62 ATS Summary ATS 7-10

63 ATS Summary ATS 1-2B

64 PEDIG Studies ATS 5 (3-7 y.o.)(18 week time course) RX correction (no occlusion tx) for anisometropic amblyopes Mean improvement = 3 lines Moderate and severe amblyopia (20/40-20/250) Rx correction (no occlusion tx) for strabismic amblyopes (or combined mechanism) 74% improved 2 lines, 54% 3 lines, 32% resolved Type of strabismus was irrelevant

65 PEDIG Studies Follow up treatment for Optical Treatment of Amblyopia 4-8 week intervals Some patients may not need occlusion Attempt one treatment at a time Allow for a total of weeks to monitor improvement

66 Case Example 7 y.o. African American male

67 Case 7 yo male Cc: blur at distance and near DVA: 20/60 OD 20/20 OS NVA: 20/50 OD 20/20 OS CT: ortho Cycloplegic retinoscopy x 180 OD x 180 OS Trial Frame x180 OU D:20/40 N:20/ x180 OU D:20/20 N:20/20 Stereo testing (cc) (+) RDS (+) Stereo Fly

68 Assessment / Plan

69 Assessment / Plan Assessment Anisometropic Amblyopia Hyperopia OU Astigmatism OD>OS (no previous Rx hx) Plan Rx given x 180 OD x 180 OS RTC 1 month after Rx dispense

70 1 month after wearing Rx FTW Rx, very comfortable x 180 OD x 180 OS DVA cc 20/20 OD 20/20 OS NVA cc 20/20 OD 20/20 OU Stereo (+) Forms NCT ortho, DCT ortho

71 PEDIG Studies Occlusion Dosage results 2 hours vs. 6 Hours = No difference (ATS 2B) moderate amblyopes 6 hours vs. Full time = No difference (ATS 2A) Severe amblyopes 6 hours occlusion vs. daily Atropine (ATS 1) Similar results 2-3 lines of VA improvement

72 Case Example 5 y.o. Caucasian male

73 Case 5 yo male 5 yo male, Failed school screening VA 20/25 20/32 20/400 20/200 ph 20/300 Cover Test 0rtho??? Stereo (-) Fly, (-) forms

74 Case 5 yo male Retinoscopy x x 045 Cycloplegic ret x /60 (20/30 w/ -1.00) x /300 DFE C/D 0.2 Rd, wnl

75 Case 5 yo male Assessment / Plan Likely strabismic amblyopia (vs. anisometropic) Rx given OD x 180 OS x 045 Occlusion therapy OD x 4 hours daily begin once Rx is received RTC 6 weeks

76 Case 5 yo male 3 month FU VA 20/30 20/30 20/60 20/60 Stereo (-) Fly, (-) RDS W 4 Dot Near = 4 dots Distance = LE Suppression Cover Test 6 CLET, 6 CLET

77 Case 5 yo male 4 month FU VA 20/20 20/20 20/40 20/40 Stereo (-) Fly, (-) RDS W 4 Dot Near = 4 dots Dist = LE Suppression Cover Test 6 CLET, 6 CLET

78 PEDIG Studies Atropine vs. Occlusion (3-7 y.o.) (ATS 1) Same results Tx effect similar to 2 and 6 hours of occlusion 80% reach max improvement by 4 months 50% 20/25 by 4 months may take up to 10 months Atropine Installation: Daily vs weekend (ATS 4) Same results Both revealed 2.3 lines of improved VA in moderate amblyopia Can Atropine be used for severe amblyopia (20/125-20/400) Atropine only = 21% mean VA 20/40, 4% mean VA 20/25 Atropine + plano lens =39% mean VA 20/40, 13% mean VA 20/25

79 Case Example 5 year old Caucasian male

80 Case 5 yo male 1 st exam April 2010, Referral from outside vision center Referral for vision therapy Diagnosis: Bilateral Isometropic/ Refractive Amblyopia BVA (Distance) 20/200 OD, OS, OU, NVA 20/80 OU (-) strabismus Trial Frame caused diplopia Trial framed full Rx NO Rx given secondary to pending consultation

81 Case 5 yo male IEI Peds Service Exam one month later Cc: holds things close to read, no developmental delays noted DVA: 20/125 OD, OS, OU NVA: 20/200 OD, OS, OU CT: ortho

82 Retinoscopy / Refraction Dry Ret x 180 OD 20/ x 180 OS 20/80 20/80 OU Trial Frame to determine plus acceptance: x180 OU D:20/80 N:20/ x180 OU D:20/50 N:20/80

83 Additional Testing Trial Frame : x180 OU Stereo testing (-) RDS (-) Stereo Fly Cycloplegic Ret x 180 OU

84 Assessment / Plan

85 Assessment / Plan Assessment Isometropic Amblyopia High Hyperopia / Astigmatism OU (no previous Rx hx) Plan Rx given x 180 OD x 180 OS Add D RTC 1 month after Rx dispense

86 5 year old male ONE month follow up / all testing with Rx wearing Rx FT with comfort Snellen VA Case findings Distance OD OS Near OD OS 20/50 20/50 20/40 20/40 Rx OD x 180 OS x Add Distance/Near Cover Test Stereopsis ortho (-) Forms, (-) Fly Ret over Rx VA with additional +2D +4 D NI

87 5 year old male TWO month follow up / all testing with Rx wearing Rx FT with comfort Snellen VA Case findings Distance OD OS Near OD OS 20/50 20/40 20/60 20/40 Rx OD x 180 OS x Add Distance/Near Cover Test Stereopsis ortho (-) Forms, (-) Fly Ret over Rx VA with additional +2D +4 D DVA 20/25 OU, NVA 20/30 OU What would you do next?

88 5 year old male THREE month follow up / all testing with Rx wearing Rx FT with comfort Snellen VA Case findings Distance OD OS Near OD OS 20/40 20/20 20/40 20/20 Rx OD x 180 OS x 180 Distance/Near Cover Test Stereopsis 6 CRET (-) Forms, (-) Fly W 4 Dot Bruckner Visuoscopy Distance 4 dots, Near RE Suppression RE whiter and brighter 2 nasal EF OD

89 Assessment / Plan Assessment Strabismic Amblyopia OD Small angle Esotropia / Microtropia High Hyperopia / Astigmatism OU) Plan Rx given x 180 OD x 180 OS

90 Cases

91 1 st clinic visit December, 2010 Pediatric Service 2 y.o. male c/o patient getting close to the TV Grandmother notes history of eye disease that causes blindness in males TAC VA OU 20/63 Poor cooperation/understanding with stereo tests Dry retinoscopy OD D OS D Cycloplegic retinoscopy OD D OS D DFE O.2 C/D OD, OS, noted clear macula and periphery with limited views

92 1 st clinic visit December 2010 Pediatric Service Assessment Anisometropic Amblyogenic Risk Factor OS Hyperopia OD >> OS Plan RTC 1 month after Rx dispense Rx OD Sph, OS sph At F/U do VA OS 1 st, attempt Lea symbols Determine need for occlusion at F/U Follow with yearly DFE due to pathology history

93 Age Visual Acuity Comments Patient History 2.5 none Cyclo Rx OD D OS D Rx given OD OS * OD 20/60 OS 20/200 No stereo fly, no RDS Stereo smile occlusion started 2-4 hrs/day OD 3.0* OD 20/60 OS 20/125 occlusion done 1-2 hrs/day OD Resume occlusion 2-4 hrs daily 3.1 OD 20/40 OS poor responses occlusion 2-4 hrs/day OD Poor compliance, was using pirate patch, stressed importance of adhesive occlusion 3.5 OD 20/60 OS 20/125 occlusion 2-4 hrs/day OD Poor compliance

94 Age Visual Acuity Comments 3.9 OD 20/60 OS 20/125 Dry Ret OD D, OS Patient History Occlusion d/c. Atropine started (1 gtt OD Saturday and Sunday ) 3.11 OD 20/70 OS 20/ OD 20/80 OS 20/ OD 20/60 OS 20/ OD 20/125 OS 20/125 Atropine Installation wrong eye Atropine Installation now appropriate VA not making sense, repeat DFE at FU, r/o pathology, repeat cyclo Cyclo Ret OD D, OS Cataract noticed on retinoscopy Poor dilation with spray, no detectable macula findings 4.9 OD 20/100 OS 20/125 Cyclo Ret OD OS Schedule posterior pole photos and OCT, suspect retinal disease

95 X Linked Juvenile Retinoschisis Ocular Findings Fundus exam shows areas of schisis (Splitting of NFL) in macula (Spoke wheel pattern) Most frequent finding = stellate maculopathy = foveal schisis Schisis of peripheral retina in 50% of cases Affected males have vision 20/60-20/120* Also associated with: axial hyperopia* posterior subcapsular cataract* strabismus

96

97

98 Assessment X- linked retinoschisis OU Hyperopia OU Plan ERG to confirm diagnosis Low Vision evaluation devices rxed Begin tx of topical dorzolamide (trusopt)

99 Low Vision Evaluation

100 Case Example Strabismus Pathologic vs non-pathologic

101 ER patient 15 year old AA Male Blurry vision OD, Left eye crossing in x 5 days (+) diplopia Horizontal Constant All distances No hx of trauma No pain

102 15 year old AA Male ER visit VA Distance = 20/50 OD, OS Near = 20/20 OD, OS PERRLA (-) APD SLE wnl CF = FTFC EOM s = -2 abduction deficit OD, OS (-) palpation of orbital mass IOP = OD 17, OS 15 CT Distance = 30 CLET Near = 25 CLET DFE wnl OD< OS Referral to peds/bv clinic

103 15 yo AA Male Pediatric service evaluation VA Distance = 20/50 OD, OS Near = 20/20 OD, OS EOMS (-)1 Abduction deficit OS Partially dilated (from cyclo x 2days??) CT Distance = 35 CAET, 6 RHT Near = 35 CAET, 6 RHT Retinoscopy OU (20/20 VA) Stereopsis (-) Fly (-) appreciation with 35 BO

104 15 yo AA Male 40 BO 40 BO 40 BO 40 BO 40 BO

105 Worth 4 dot Uncrossed Diplopia Fusion w/ 40 BO

106 Uncrossed Diplopia

107 Fusion with 40 Base OUT

108 15 yo AA Male Assessment Sudden Onset (Acquired), Large Angle Esotropia w/ diplopia Plan MRI scheduled

109 Review of data VA distance, adequate near = Myopia (-) amblyopia = recent onset Large angle, constant, comitant deviation with diplopia RECENT ONSET Fusion unstable motor fusion

110 MRI Summary The L SOV is enlarged as is the L ICA dx is carotid cavernous fistula His is slightly proptotic on the left as well. Is the conjunctiva injected, and can you hear his pulse in the left globe?

111 Carotid Cavernous Fistula (CCF) results from an abnormal communication between the arterial and venous systems within the cavernous sinus in the skull. It is a type of arteriovenous fistula. As arterial blood under high pressure enters the cavernous sinus, the normal venous return to the cavernous sinus is impeded and this causes engorgement of the draining veins, manifesting most dramatically as a sudden engorgement and redness of the eye of the same side. Radiologic techniques are used in embolization of carotidcavernous fistulas (CCFs). Angiography is invaluable for the guidance of catheter placement and delivery of the embolization materials. Angiography, computed tomography (CT) scanning, magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA) are also useful in assessing the effectiveness of treatment

112 Carotid Cavernous Fistula (CCF) Clinical Signs marked congestion of the eyelids, conjunctiva and orbit a red, congested eye that is often mistreated as an ocular infection or inflammation proptosis (which is often pulsatile) limitation of ocular movement diplopia ophthalmoplegia (often from CN VI palsy) tinnitus or orbital bruit Interventional radiologist

113 Pediatric Clinical Pearls Collecting exam data / Modification of exam techniques Determining the best treatment options Pediatric Refractive Error Amblyopia Strabismus

114 QUESTIONS? Contact: Valerie M. Kattouf O.D. Illinois College of Optometry (312)

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