Clinical Masqueraders: Pediatric and Binocular Vision Cases That Aren t What They Appear Katie S. Connolly, OD, FAAO Don W. Lyon, OD, MS, FAAO

Transcription

1 Clinical Masqueraders: Pediatric and Binocular Vision Cases That Aren t What They Appear Katie S. Connolly, OD, FAAO Don W. Lyon, OD, MS, FAAO Please silence all mobile devices and remove items from chairs so others can sit. Unauthorized recording of this session is prohibited.

2 Disclosure Statement: Nothing to disclose

3 Patient 1: 11 Year Old Caucasian Male Long Time Patient Seen Since Age 4 Cc Routine Exam Mother and patient so not have any complaints, no medications, no reported health problems VA 20/20 Distance and Near All BV testing normal Pupils normal APD Low hyperopic prescription External health normal OU Internal health..

4 Baseline OCT This is the previous patient, similar appearance with this patient

5 REFER Letter to Pediatrician indicating diagnosis and need for MRI and possible Lumbar Puncture Pediatrician wanted patient seen by ophthalmologist before heading down that route Patient saw OMD who agreed with diagnosis of Papilledema and sent off to neuro for appointment 3 months later received MRI, which was normal, and subsequent LP Opening pressure >580mm Dx Pseudo tumor Started Diamox Patient does not fit typical clinical picture: Male Overweight, 284 lbs. at Neuro appt

6 Repeat OCTs Baseline 2 months 3 months 5 months

7 Repeat Visual Fields

8 Patient is now 15 years old Still being followed by Neuro PCP wanted patient followed by OMD, mother refused stayed with us Continuous contact between Neuro and us on overall health and visual system Case complicated by additional health issues, Sleep Apnea, DM type 1.5, HTN, gaining weight Long term patient

9 Clinical Pearls Not all conditions follow the typical clinical picture Order the test(s) you are comfortable with, refer when needed Stand your ground on your concerns and Dx

10 Patient 2 10 year old Caucasian Male Patient here for routine comprehensive eye exam. LEE 1 year, Diagnosed with unspecified amblyopia. No treatment beyond habitual Rx, RTC 1 year. Short stature, hearing aid in left ear. No medications Results from previous exam VA 20/25 OD, 20/80 OS, Cover test orthophoric HAB Rx OD x025, OS x005 Ocular health normal Results from Current exam VA 20/25 OD, 20/200 OS, Cover test 15pd LXT Distance Stereo, -global, 400 local HAB Rx OD x025, OS x005 Ocular health

11 Patient 2 10 year old Caucasian Male 0.1/0.1 double ring sign mild vessel tortuosity 0.05/0.05 double ring, marked vessel tortuosity

12 Patient 2 10 year old Caucasian Male Diagnosis: NOT unspecified amblyopia If you do not know the reason for loss of visual acuity, diagnose Unspecified visual loss H54.7 To diagnose amblyopia, must have association, refractive, strabismic, form deprivation Bilateral optic nerve hypoplasia, (OS>OD) with reduced visual acuity. Order MRI, will call parents with the results.

13 Patient 2 10 year old Caucasian Male The patient was referred to his Pediatrician and to Pediatric Endocrinology. Patient was started on growth hormones. Since patient had constant exotropia patching was initiated to see if visual acuity could be improved in left eye patient lost to follow-up. Clinical Pearls Forget Unspecified Amblyopia is a diagnosis, DO NOT USE IT Cases with bilateral optic nerve hypoplasia imaging should be considered Cases with associated risk factor(s) for amblyopia and ocular health problem, visual acuity still may be improved through traditional amblyopic treatments.

14 Patient 2 10 year old Caucasian Male Results of MRI atrophy of the left and possibly right optic nerve along with a deficient septum pellucidum (SP) anteriorly. These results are suggestive of Septo-Optic Dysplasia, [DeMorsier s Syndrome] Axial flair image demonstrating absence of the septum pellucidum. uhrad.com-neurology Imaging Teaching Files

15 Septo-Optic Dysplasia A congenital malformation characterized by: optic nerve hypoplasia (ONH) Absence of the septum pellucidum Pituitary hormone deficiencies. 30% of patients will have all three findings Additional clinical signs may include Strabismus Nystagmus Decreased visual acuity

16 Patient 3: 6 Year Old Caucasian Female Referral for strabismus and amblyopia Previous visual acuity: 20/60 OD 20/20 OS Unremarkable systemic health, no allergies, no medications Normal birth and development

17 Patient 3: Entrance Evaluation Visual acuity (adhesive patch, single surround): 20/300 OD 20/20 OS No stereo, constant right eye suppression 30 PD right exotropia distance and near

18 Health/Refractive Error Anterior unremarkable Posterior unremarkable ONHs well perfused, symmetric in size Macula flat with foveal reflex Cycloplegic Retinoscopy: OD: x180 OS: x180

19 Assessment and Plan Assessment: Strabismic Amblyopia, right eye Plan Extensive education Rx patching OS 4-6 hours per day Referral to peds ophthalmology per parent request

20 Summary of Visits VA OD VA OS Plan 4 months 20/320 20/16 Up patching to 6 hrs/day 8.5 months 20/250 20/16 Cont patching 6 hrs/day. Consult with Ophthalmology. Order MRI.

21 MRI Results

22 Management Pearls Sit back and look at the big picture Don t forget about relative amblyopia Keep the pediatrician in the loop Be clear in your notes to ophthalmology

23 Patient 4: 5 year old African American Male Parents concerned with a white flash seen in photographs for the last eight months No medical conditions, normal birth, no medications Clinical exam VA 20/20 OD 20/150 OS, (with eccentric viewing) CT Ortho, but marked occlusion preference noted Bruckner Whiter, brighter reflex OS Stereo no measurable stereo Pupils, EOM normal Anterior Health normal OD, OS

24 Patient 4: Internal Assessment Internal Health OD normal 0.3 CD, well perfused OS Initial Dx Coat s Disease, refer to Riley for FA and Treatment

25 Coats FA

26 Coat s Clinical Pearl One of the most litigious diagnoses for OD, OMD, pediatricians Can be misdiagnosed as amblyopia Can be misdiagnosed as retinoblastoma Prompt treatment needed to hopefully achieve good visual prognosis

27 Patient 5: 9 Month Old Referral from pediatrician Abnormal esotropia OS, x 2 months, worsening White pupil in pictures Able to grab toys and spot people across the room

28 Patient 5: 9 Month Old Initial Visit VAs: 20/63 OU, strong occlusion preference 10 PD Intermittent left esotropia Anterior Chamber Angles: 4+ OD, 1+ OS

29 Left Eye

30 Right Eye

31 MRI

32 Prevalence/Genetics Most common eye cancer of childhood Requires mutation in both RB1 genes Bilateral 40% of cases Assumed hereditary/germline Unilateral 60% of cases Majority sporadic Detection 2/3 are diagnosed by 2 years of age

33 Treatment Chemotherapy OD: red diode and argon laser on new tumors and those not regressing OS: No anterior chamber infiltration, No optic nerve infiltration, eventual enucleation

34 Management Pearls Always dilate children, ensure you see the entire posterior pole If you want something done quickly, call Use these cases to educate those around you about what you do

35 Patient 6: 27 Year Old Caucasian Male This primary care exam Complaint of decreased vision and a shadow on his right side that seemed to be worsening or creeping into his central vision Was in for regular exam, the year before he had been diagnosed with Microtropia OS and resultant amblyopia 4 BO test was inconclusive at time of examination FDT had scattered defects Referred to BV/Peds for assessment of Microtrope with suggested 24-2 HVF due to the FDT findings.

36 Binocular Assessment VA 20/30 OD, 20/70 OS (was 20/50 previous year) CT 4 EP Dist. and Near NPC BON Pupils Grade 1 APD OS Color 10/10 OD, 9/10 OS Stereo 20 arc Ref X070 OD X105 OS External health Normal Internal health normal Versions smooth and full MEM OD, OS 4-Base Out inconclusive Worth Dot inconsistent results

37 Visual Field Results

38 Assessment and Plan 1. Vision loss unknown origin, results either inconclusive or inconsistent with diagnosis of amblyopia 2. Patient education that vision loss may be organic in nature. Recommended MRI. Patient does not have medical insurance and needs to discuss costs with wife prior to referral. Educated further on possible severity and not being imaged or further tested. Gave patient information on Volunteers in Medicine program. Patient called next day agreeing and MRI was scheduled.

39 MRI Results Pituitary prolactinoma, (18mmX23mmX18mm)with severe cisternal optic nerve and chiasmal compression. Subacute hemorrhagic components along left anterior border. Tumoral involvement left cavernous sinus. Referral to neurologist made

40 Neurologist Report 1. Neuro report indicated that patient detailed having problems with his left eye since childhood, told was a bad test taker for visual fields. 2. Discussed treatment options, decision was made to monitor and treat underlying condition by decreasing amount of prolactin in the body

41 MRI Comparison August 2011 November 2013

42 Follow-Up Patient has returned twice since the diagnosis of pituitary tumor. BVA 20/20 OD 20/25 OS Patient expressed this is the best vision he s had Still on medication to control levels to stop tumor from enlarging

43 Clinical Pearls 1. Functional Amblyopia vs. Organic Vision Loss 2. Children as poor test takers on Visual Fields

44 Case 7: 18 yo Caucasian Female Presents due to blurred vision OU, x 2 weeks, distance, when starting College for the 1 st time, worsens throughout the day as she transitions from distance to near more Test Visual Acuity Entrance Testing Cover Test Result 20/20 OD/OS PERRLA SAFE Matrix Clear Ortho at distance 2^ Esophoria at near NRA/PRA +1.25/-2.00 Amplitude of Accomm Vergence Ranges Ocular Health Age appropriate Reduced BO at near Unremarkable Macula flat, +FLR 0.20 C/D with good color

45 Case 7: Follow-up Now complaining of diplopia X 2 weeks, constant, worst in left gaze Associated droopy right upper eye lid x 1.5 weeks Associated mild headaches, behind right eye x 1 month Mild blur at distance now constant, worst in the left eye

46 Case 7: Follow-up Test Visual Acuity Pupils EOMS Result 20/20 OD, OS with contact lenses Intermittent anisocoria OD>OS, reactive pupils Restriction of the right eye in superior left gaze (up and in) Cover Test Red Lens Test Anterior Seg Posterior Seg 15^ RXT 7^ RHypoT at dist/near Exo and right hypo, worst in up and left gaze 5 mm right ptosis Macula flat with +FLR, Optic nerves well perfused with C/D of 0.20 OD, OS

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49 Giant Cell Tumor 1. Rare, benign primary bone tumor 2. Locally aggressive % of all benign bone tumors, usually in the epiphysis of long bones 4. 2% of cases present in the skull Ethmoid, temporal and sphenoid bones most commonly 5. Causes osteolytic bone destruction by formation of osteoclasts 6. Diagnosed via microscopic analysis after biopsy

50 Case 7: Resolution of Case 1. Underwent transphenoid approach resection 2 weeks after diagnosis treatments of proton beam radiation due to incomplete resection 3. 4 injections of Denosumab 4. Diplopia fully resolved, stable 2 years later, monitored every 3-6 months with MRI

51 Case 8: 58 yo Caucasian Male 1. Referred for diplopia Onset 5 days prior, horizontal, bilateral, worst when driving, worsening 2. Previous Evaluation: (3 days prior) 8^ LET distance/near Full EOMs, diplopia in right gaze Normal pupils/confrontations 0.25/0.25 C/Ds with good color and distinct marings 3. H/O high cholesterol. Taking Simvastatin

52 Case 8: Initial Visit Testing: Visual Acuity: 20/25 OD/OS EOMs: unable to abduct OD, full OS CT: 20^ RET distance and near Subjective prism eval: 20^ BO distance None for 20 cm and closer Red Lens Over OD IOP, Ocular Health unremarkable R Gaze

53 Case 8: Initial Evaluation 1. Assessment: 2. Plan: Cranial Nerve VI Palsy, Right side Fit pt with 20 PD Fresnel over right lens for driving Contact PCP via phone to discuss ordering lab work/imaging.

54 Case 8: Lab Work/MRI 1. Basic Metabolic Profile Sodium, Potassium, Chloride BUN, Creatinine, Calcium Anion Gap Osmolality, Calc GFR Hgb A1C 5. MRI? If less than 45 yo Between and no vasculopathic risk Pain, papilledema, bilateral or h/o cancer 2. CBC with platelets 3. Full neuro evaluation 4. Additional lab work? ESR/CRP RPR, Lyme Titer, FTA-ABS

55 Case 8: Resolution of Case 1. Lab work unremarkable 2. MRI unremarkable 3. Diagnosis?? Idiopathic viral?? 4. Slowly reduced prism over first year for driving. Now wearing 8^ BO for driving and none for other activities

56 Case 8: Management Pearls 1. Don t be afraid to assist the PCP with what to order 2. Their knowledge on the subject could be variable 3. Keep them informed on the health of the ONH Helps determine need for imaging and certain lab work 4. If unable to efficiently and effectively work with the PCP, order yourself. 5. Consider Fresnel prisms for specific tasks