Classic Lattice Degeneration

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Classic Lattice Degeneration
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Perivascular Lattice
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Photocoagulation spots in moderate pattern
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Cryotherapy
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Vitreous Anomoly
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Attached with Silicone Oil
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Stickler GB, Hughes W, Houchin P. Clinical
features of hereditary progressive
arthro-ophthalmopathy (Sticklersyndrome) a
survey. Genet Med. 2001 May-Jun3(3)192-6.
Early diagnosis and intervention for retinal
detachments can preserve vision.
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Retinal Detachment and Prophylaxis in Type 1
Stickler Syndrome
Group Retina Detachment Bilateral RD Follow Up (years)
No Treatment 73 48 21
Bilateral Treatment 8 0 11.5
Unilateral Treatment 10 10 15.5
Ang A, Poulson AV, Goodburn SF, Richards AJ,
Scott JD, Snead MP.Ophthalmology. 2008
Jan115(1)164-8.
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Shapiro Recommendation

• Monitor Q 3 months to catch RD early
• EUAs as needed to get view of periphery
• Test each eye regularly
• Educate for Signs of breaks and RDs
• Educate of continued high risk (5-10)
• Treat with laser from ora to anterior equator or
  most posterior pathology.
• Consider randomizing to cryotherapy

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Choice of Intervention

• Peripheral Cryotherapy
• Peripheral Laser Photocoagulation
• Encircling Scleral Buckle
• Treatment of Pathologic
• PVD
• Monitoring

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Questions for Physician Education

• Why is detection and counseling important ?
• What are the triggers for suspicion?
• What diagnostic complexity is posed by
  COL2A1Exon2 disease ocular predominant
• When and How is Stickler outcome improved ?
• What are diagnostic pearls ?

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