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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

Failed Eye Exam

Rochelle Cochrane, M.D.

January 13, 2006

Tracy Crnic, M.D.


Case presentation
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


Case presentation1
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


Case presentation2
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


Case presentation3
Case Presentation

Cyclopegic Retinoscopy

  • OD –3.25 +1.75 x85

  • OS +1.00 +0.50 x93

  • Fundoscopic Exam


  • Differential diagnosis
    Differential Diagnosis

    • Optic Nerve Coloboma

    • Morning Glory Anomaly

    • Peripapillary Staphyloma

    • Optic Disc Drusen

    • Megalopapilla

    • Optic Disc Dysplasia

    • Glaucomatous Cupping

    • Optic Pits


    Differential diagnosis syndromes
    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


    Differential diagnosis syndromes1
    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



    Morning glory anomaly
    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


    Morning glory anomaly1
    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


    Morning glory anomaly2
    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


    Morning glory anomaly3
    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


    Work up treatment and follow up
    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


    Results
    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


    References
    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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