6/8/2016 • 5 y.o AM referred in for RXT eval and amblyopia – Parents interested in VT for XT – Parents noticed the eye turn 1.5 years ago Not So Atypical Pediatric Cases – Has worn glasses q1year – H/o patching 5-60min/day OS daily for 1.5 years • BHx: Unremarkable Steven Brooks MD, • Jenelle Mallios OD, FAAO DHx: Unremarkable Daniella Rutner OD, MS, FAAO, FCOVD • Ehx: Unremarkable Lauren Yeager MD • Meds: None • FHx: Unremarkable Lensometry: OD: +0.75-2.75x175 OS: plano-3.00x180 CTcc: Primary gaze: 40 XT, 8RHT @ D (comitant), VAcc: D: 20/50 OD, 20/50 OS with HOTV , poor attention except no hyper on L tilt Vacc: N: 20/50 OD,OS Pupils, EOMS – normal NPC: poor CVF- pt uncooperative Stereo: RDS: none Nystagmus: unilateral (OD), high frequency, low Dry ret: amplitude horizontal nystagmus; dampens in left gaze and convergence OD: +1.50-1.50x180 OS: plano-2.00x180 Color – pt uncooperative • SLE- unremarkable • IOPs: soft and equal : digital Cyclo ret: OD: +3.00-2.50x180 OS: +1.50-2.75x180 DFE: Retinoschisis OD>OS A: X linked juvenile retinoschisis P: Consult with Dr. Brooks, Pedi OMD Stat 1 6/8/2016 • Typically males • VA 20/60-20/120 • Requires Retinal consult • Low Vision Referral • Avoid Contact Sports • Tx: topical dorzolamide or oral azetazolamide • In the pipeline gene replacement therapy Anisometropic Amblyopia ? • 11 y-o male • BCVA 20/20 OD and 20/50 OS • Rx: +3.50 -3.50 X 175 OD -5.00 -2.00 X 180 OS 2 6/8/2016 OD OS Another Anisometropic Amblyope? • 12 year-old male • BCVA 20/40 OD and 20/20 OS • Rx: -6.00 OD, Plano OS OD OS 3 6/8/2016 Myelinated Nerve Fibers, Dysplastic Nystamgus Discs and Myopia • Nystagmus occurs in 0.4% of the clinical population. • frequency range of 2-5 Hertz (Hz) with an amplitude of 1-5 degrees. Developmental anomaly Usually horizontal in direction, although they may include a small vertical or rotary component. Affected macular integrity from birth • They cause sensory deficits such as reduced contrast sensitivity, visual acuity, and stereo acuity can have profound psychological effects resulting from the unusual cosmetic Resulting myopia? Degree of myelination directly proportional to poorer prognosis of visual improvement with therapy Kee C, Hwand JM. Visual prognosis of amblyopia associated with myelinated retinal nerve fibers. Am J Ophthalmol 2005;139:259-65. Case 1 Nystagmus • Nystagmus present at birth or prior to age • 7 month old 2 months is more likely to be idiopathic in • F&F nature or due to neurologic dysfunction. • OD +1.25-0.50x180 • Sensory deficit nystagmus most commonly • OS +1.25 presents at age 2-3 months. Further investigation of the visual system is • Pendular Nystagmus warranted in these cases. • CAXT (Right eye preferred fixation) • 3YO • 5yo • VA Cardiff OU 20/50 • 20/60 OD 20/32 OS • EOM full OA IO • EOM full • Intermittent LXT • NO tropia 4 6/8/2016 Infantile/Congenital • F – fixation THE 5 • U-upgaze • Aniridia • Achromatopsia • N-null point • Lebers Amarosis • B- bilateral • Albinism • L-latent • Optic Atrophy • O-OKN inverse response • C-convergence • S-symptomless Nystagmus Treatment options Rutner Treatment of Choice • less optical aberrations • Glasses • enlarged retinal image (in refractive myopes) • Prism • increased peripheral visual field. • Contact lenses • Any or all of the above would improve the quality • Biofeedback and/or extent of the retinal image, and hence – Visual provide a visual input of higher fidelity for fusion – Auditory and subsequent visual information processing. • Surgery Nystagmus Case 2 Nystagmus • An 18-year-old female with non-PAN, congenital jerk nystagmus, Condition Amplitude Frequency (Hz) High Contrast Visual Low Contrast (degrees Acuity Visual Acuity who had never worn contact lenses, was evaluated. Her refraction ) and best corrected visual acuity with spectacles was OD -4.00D/- 2.75D x 170 (20/120), and OS -4.00D/-3.00D x 025 (20/200). She had strabismus surgery two years earlier to correct a constant Test Session One: 9.00 1.25 20/120 OD 20/200 OS - - - - esotropia, with a residual 15 prism diopters of intermittent alternating Spectacles (baseline) esotropia. Ocular health examination revealed ocular albinism; all SCL 1.75 1.05 20/100 OD 20/125 20/125 OD unable else was within normal limits OS, 20/100 OU to perform OS at 1 meter • After a comprehensive eye examination to assess refractive, test distance binocular, and ocular health factors, the patient was fit with 20/125 OU Coopervision Preference Toric soft contact lenses (SCL). Lens parameters were: 8.7mm base curve, 14.4mm diameter, 0.09mm center thickness, OD -3.00D/-2.25Dx180, and OS -2.00D/- SCL with anesthetic 2.70 1.40 - - - - - - - - 2.25Dx010. Test Session Two: 0.72 1.80 20/80 OD 20/125 OS 20/100 OD 20/125 SCL one week later 20/80 OU OS 20/100 OU SCL with anesthetic 3.30 1.95 - - - - - - - - 1week later 5 6/8/2016 3rd Case Nystagmus • 4 month old with nystagmus • Healthy, FT • No family history of ocular disease • Parents not related • Mild photophobia • Nystagmus noted age 8 weeks Examination Findings Differential Diagnosis • Blinks to light OU • Congenital motor nystagmus/infantile • Tracking inconsistent nystagmus syndrome • Pupils equal, normal reactivity • Albinism • Conjugate, horizontal, pendular • Retinal dystrophy (eg. LCA, rod-done nystagmus with small amplitude and high dystrophy, CSNB) frequency • Optic nerve hypoplasia • Cycloplegic refraction: +4.50 sph OU • Coloboma 6 6/8/2016 New Onset Nystagmus in a 2 year Key Examination Points old • Assess visual function • Healthy 2 yo F • Iris transillumination • No prior illness • Check for paradoxical pupil response • Sudden onset of ataxia and nystagmus • Funduscopic examination • Lethargy, irritability • Myoclonic jerks Examination Findings Examination Findings • Rapid, conjugate, multi-directional jerky • Normal anterior segment and funduscopic eye movements examination • Central fixation, disrupted by jerky eye • Full range of EOM’s movements • Cycloplegic refraction +2.00sph OU • Normal pupils Diagnosis Opsoclonus-Myoclonus-Ataxia • OMA (opsoclonus-myoclonus-ataxia) • Rare – Post-encephalitic (viral) • Requires urgent neurological evaluation – Para-neoplastic (50%) • Neuroblastoma most frequently implicated – Idiopathic neoplasm • This is NOT nystagmus • Opsoclonus secondary to autoimmune attack of purkinje cells in the cerebellum • Recovery often incomplete 7 6/8/2016 5 year old with R face turn Examination Findings • Healthy 5 yo M • Va 20/60 OD, 20/70 OS, 20/30 OU open, • Noted by parents to turn habitually turn 20/25 OU open with face turn to R face to the right when watching TV • Conjugate, horizontal, nystagmus with fast • Nystagmus noted age 4 months phase to L in primary position • Good vision, asymptomatic • Normal pupils and EOM’s • Family history negative Examination Findings Differential Diagnosis • No iris transillumination • Congenital motor nystagmus/infantile • No photophobia nystagmus syndrome • No strabismus • Manifest-latent nystagmus/fusion • Nystagmus decreases in right gaze, maldevelopment nystagmus syndrome increases in left gaze • Periodic alternating nystagmus • Normal anterior segment and fundus exam • Cerebellar lesion • Cycloplegic refraction: plano OU • Vestibular lesion • Retinal dystrophy Interpretation of Examination Management • Congenital motor nystagmus with null • Surgery to shift null point point in left gaze (causing right face turn) • Surgery to reduce (dampen) nystagmus • Visual acuity improved with OU open • Prism glasses (latent component to nystagmus) • Imaging?? Neurology consultation?? • Visual acuity improved in left gaze (null • Follow-up point) 8 6/8/2016 6 month old with OS “shaking” Examination Findings • Healthy, FT • Normal appearing baby • Parents note that left eye seems to • Central, steady fixation with each eye intermittently shake or jiggle • Normal pupils • Normal development • OS intermittently shows very low • No FH of ocular disease amplitude, high frequency, pendular nystagmus • Child seems to tilt head to left and shake head intermittently Examination Findings Differential Diagnosis • Normal anterior segment OU • Spasmus nutans • Normal funduscopic examination OU • Sensory-loss nystagmus • Full EOM’s • Retinal dystrophy • Cycloplegic refraction • Idiopathic OD: +1.75+1.75x50 • Leukoencephalopathy/Leukomalacia OS: +1.75+1.50x130 Interpretation of Exam Management • Likely spasmus nutans • Neuro-imaging • Close observation • Correction of refractive error? • Patching to prevent amblyopia? 9 6/8/2016 Peds Ophth and Some Red 8 month old with esotropia Flags • Parents have noted ET “since birth” • Healthy, FT • Family history positive for ET in mother and a cousin • Slower to crawl than older sibling Examination Findings Examination Findings • Normal appearing baby • Normal pupils • Constant esotropia 40 PD by Krimsky • Normal anterior segment and fundus testing • No nystagmus • Holds fixation well with either eye • Normal versions • Mild limitation of abduction OU • Cycloplegic refraction OD: +1.75 sph OS: +1.75 sph Differential Diagnosis Interpretation of Examination • Congenital esotropia • Large angle constant esotropia • Infantile accommodative esotropia • No amblyopia • Sensory loss • Normal vision for age, no evidence of • EOM fibrosis syndrome ocular defects • Duane syndrome • Essentially full ductions • Nystagmus blockage syndrome • Minimal hyperopia, normal for age 10 6/8/2016 Interpretation of Examination Management • Likely congenital esotropia without • Glasses?? amblyopia • Patching?? • Accommodative component unlikely • Vision