Transcript Slide 1
Classic Lattice Degeneration Perivascular Lattice Photocoagulation spots in moderate pattern Cryotherapy Vitreous Anomoly Attached with Silicone Oil Stickler GB, Hughes W, Houchin P. Clinical features of hereditary progressive arthro-ophthalmopathy (Sticklersyndrome): a survey. Genet Med. 2001 May-Jun;3(3):192-6. Early diagnosis and intervention for retinal detachments can preserve vision. Retinal Detachment and Prophylaxis in Type 1 Stickler Syndrome Group Retina Bilateral RD Follow –Up Detachment (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 Jan;115(1):164-8. 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 Choice of Intervention • • • • • • Peripheral Cryotherapy Peripheral Laser Photocoagulation Encircling Scleral Buckle Treatment of Pathologic PVD Monitoring Questions for Physician Education 1 2 3 4 5 Why is detection and counseling important ? What are the triggers for suspicion? What diagnostic complexity is posed by COL2A1:Exon2 disease: ocular predominant When and How is Stickler outcome improved ? What are diagnostic pearls ? THE END (This power point was edited for the SIP Web Site)