Failed Eye Exam

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
  Dec91(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.
  200418(11)1038-1048.
• Pollock, S. The morning glory disc anomaly
  contractile movement, classification, and
  embryogenesis. Doc Ophthalmol. 1987
  Apr65(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. 200285(6)383-388.
• Pictures www.atlasophthalmology.com