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Kiran Turaka, Shepard Bryan, Alan Gordon, Henry Kwong, Clive Sell; Prophylactic Treatments for Retinal Tears/Detachment in Stickler Syndrome: Single Institution Experience. Invest. Ophthalmol. Vis. Sci. 2013;54(15):4958. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
To report various prophylactic treatment methods and outcomes for retinal tears and detachment in patients with Stickler Syndrome (SS).
A retrospective study of patients with SS from March 1991 to February 2012 was performed. Data analysis of 31 patients (62 eyes) included clinical features, systemic features, treatment (prophylactic scleral buckle, laser retinopexy and cryotherapy) and outcomes at the last follow-up (FU).
The median age of the patients was 12 years (range 0.2- 68). Of the 31 patients, there were 9 females and 22 males. Systemic features of SS were present in 23 patients, of which most common was cleft palate in 13, musculoskeletal abnormalities in 7. High myopia was present in 19 patients. Anterior segment examination showed cataract in 10 eyes, pseudophakia in 7 eyes and aphakia in 4 eyes. Vitreous syneresis was present in 25 eyes, membranes, veils and opacities in 13 eyes, posterior vitreous detachment in 3, and empty vitreous cavity in 7 eyes. Peripheral retina examination showed small retinal tears in 4 eyes, lattice degeneration in 12, giant retinal tear in 2, chorioretinal atrophic scars in 15, vitreoretinal tufts in 8, retinal detachment (RD) in 5. No retinal abnormalities were noted in 13 eyes. Treatment was by laser retinopexy and or cryotherapy in 15 eyes, and combination of vitrectomy, laser retinopexy, cryotherapy and or sclera buckle in 6 eyes and close observation in 40 eyes. Five fellow eyes with extensive lattice degeneration with or without associated retinal tears were treated by prophylactic scleral buckle. The median FU duration was 65.5 months (range 5-226). Retinal detachment developed in seven fellow eyes. The median time to develop the retinal detachment was 22.5 (range 5-123) months. Six eyes were treated by vitrectomy with sclera buckle and one eye with vitrectomy, silicon oil and laser retinopexy. Retinal tear in the fellow eyes occurred in 16 eyes and treated by laser retinopexy and or cryotherapy. None of the patients had RD at the last FU visit.
Prophylactic treatments such as scleral buckle, laser, and cryotherapy were effective in preventing the development of giant retinal tears or detachment not only in affected eyes but also in the fellow eyes of patients with Stickler syndrome.
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