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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
    • 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
  • 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
  • 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.
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