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Failed Eye Exam

Failed Eye Exam. Rochelle Cochrane, M.D. January 13, 2006 Tracy Crnic, M.D. Case Presentation. 5 y/o Caucasian Female presents to pediatric ophthalmology clinic after “failing” school eye exam Mother has not noticed any eye problems up to this point. Pt has had normal development.

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Failed Eye Exam

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  1. Failed Eye Exam Rochelle Cochrane, M.D. January 13, 2006 Tracy Crnic, M.D.

  2. Case Presentation • 5 y/o Caucasian Female presents to pediatric ophthalmology clinic after “failing” school eye exam • Mother has not noticed any eye problems up to this point. Pt has had normal development. • PMH, PSH, FHx – negative • Birth Hx – normal pregnancy and birth without complications

  3. Case Presentation • VA sc OD 20/400 OS 20/20 • Stereo Vision 0/3 Animals, 0/9 Circles • Motility Full OU • OrthoTropic and OrthoPhoric • Bruckner sc brighter reflex OS • Pupils equal, round, reactive to light and accommodation; No APD OU

  4. Case Presentation • PLE • Normal Facial Features • LLL WNL OU • Conj Clear and Quiet OU • Cornea Clear and Compact OU • A/C Deep and Quiet OU • Iris Normal OU • Lens Clear OU

  5. Case Presentation Cyclopegic Retinoscopy • OD –3.25 +1.75 x85 • OS +1.00 +0.50 x93 • Fundoscopic Exam

  6. Differential Diagnosis • Optic Nerve Coloboma • Morning Glory Anomaly • Peripapillary Staphyloma • Optic Disc Drusen • Megalopapilla • Optic Disc Dysplasia • Glaucomatous Cupping • Optic Pits

  7. Differential Diagnosis – Syndromes • CHARGE • Coloboma, Heart defects, choanal Atresia, Retarded growth and development, Genital and Ear anomalies • Aicardi – death usually occurs in first few years of life • Multiple depigmented chorioretinal lacunae clustered around disc, congenital disc anomalies • Epileptic seizures, agenesis of corpus callosum, psychomotor retardation • Alagille • Posterior Embryotoxon and retinal pigmentary changes, eccentric pupils • Jaundice, heart defects, poor school performance

  8. Differential Diagnosis – Syndromes • Edwards • Optic Atrophy, congenital glaucoma, corneal and lens opacities • Failure to thrive, heart defects, mental retardation • Lenz Microphthalmia • Coloboma, microcornea, microphthalmia • Severe renal dysgenesis, dental anomalies, severe speech impairment, short stature • Patau – fatal in first few months of life • Coloboma, microphthalmia, cataracts • Heart defects, cerebral hypoplasia, renal abnormalities, respiratory involvement, GI disease, urogenital involvement

  9. Fundus Exam

  10. Morning Glory Anomaly • Named after resemblance of optic nerve to the Morning Glory flower • Incidence is unknown, but very rare • Congenital abnormality • Sporadic, not inherited – colobomas run in families • Usually unilateral – colobomas bilateral • More common in females – equal in coloboma and staphyloma

  11. Morning Glory Anomaly • Pathogenesis unknown • Abnormal closure of embryonic fissure (like coloboma) • Abnormal development of distal optic stalk at its junction with the primitive optic vesicle • Disc enlarged with funnel-shaped excavation centrally – coloboma inferior temporal, staphyloma normal disc sunken and cup shaped • Central core of whitish glial tissue, either elevated or recessed, representing persistent hyaloid remnants - unique

  12. Morning Glory Anomaly • Surrounding elevated annulus of chorioretinal pigmentary disturbance – not seen in colobomas • Increased number of blood vessels, difficult to distinguish arteries from veins, which emerge in radial pattern from disc like spokes on a wheel - unique • Unusual phenomenon of contractile movements of the optic disc due to cuff of smooth muscle tissue in terminal optic nerve

  13. Morning Glory Anomaly • Visual potential from 20/20 to NLP, usually 20/100 to 20/200 • Serous retinal detachment develops in about 30% of patients • Systemic associations rare • Frontonasal dysplasia • Hypertelorism, depressed nasal bridge, hare lip, or cleft palate • Basal encephalocele, absent corpus callosum, or pituitary deficiency • Neurofibromatosis type 2

  14. Work-up, Treatmentand Follow-up • CT or MRI Head for possible Basal Encephalocele • Referral to pediatrician • Given full glasses prescription • Trial of patching, but should be discontinued if no improvement • Regular follow-up for retinal detachment • Spontaneous reattachment has been reported • Haik, et al. Ophthalmology. 1984 Dec;91(12):1638-1647

  15. Results • Obtained CT Head • normal orbits, symmetrical and normal optic nerves, no intracranial masses, fluid collections or cystic structures • Referred to pediatrician • Given Rx • OD -3.25 +1.75 x85 • OS plano +0.50 x93 • Patching OS 7 hours/day for 5 days/week • 5 weeks later VA cc OD 20/200 OS 20/20 • Follow up in 3 months

  16. References • Kanski, JJ. Clinical Ophthalmology: A Systemic Approach. 5th ed. Elsevier Science Limited; Philadelphia. 2003. • Dutton, GN. Congenital disorders of the optic nerve: excavations and hypoplasia. Eye. 2004;18(11):1038-1048. • Pollock, S. The morning glory disc anomaly: contractile movement, classification, and embryogenesis. Doc Ophthalmol. 1987 Apr;65(4):439-60. • Roy, FH. Ocular Syndromes and Systemic Diseases. 3rd ed. Lippincott Williams and Wilkins; Philadelphia. 2002. • Simon, JW, et al. Pediatric Ophthalmology and Strabismus. AAO; San Francisco. 2005; 339. • Chan, RT, et all. Morning glory syndrome. Clin Exp Optom. 2002;85(6):383-388. • Pictures www.atlasophthalmology.com

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