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Tytuł pozycji:

Stickler Syndrome (SS): Laser Prophylaxis for Retinal Detachment (Modified Ora Secunda Cerclage, OSC/SS).

Tytuł:
Stickler Syndrome (SS): Laser Prophylaxis for Retinal Detachment (Modified Ora Secunda Cerclage, OSC/SS).
Autorzy:
Morris RE; Retina Specialists of Alabama, Birmingham, AL, USA.; Helen Keller Foundation for Research and Education, Birmingham, AL, USA.; University of Alabama at Birmingham (UAB), Department of Ophthalmology, Birmingham, AL, USA.; UAB Callahan Eye Hospital, Birmingham, AL, USA.
Parma ES; Retina Specialists of Alabama Montgomery, Montgomery, AL, USA.
Robin NH; UAB Department of Genetics, Birmingham, AL, USA.
Sapp MR; Retina Specialists of Alabama, Birmingham, AL, USA.; Helen Keller Foundation for Research and Education, Birmingham, AL, USA.; University of Alabama at Birmingham (UAB), Department of Ophthalmology, Birmingham, AL, USA.; UAB Callahan Eye Hospital, Birmingham, AL, USA.
Oltmanns MH; Retina Specialists of Alabama, Birmingham, AL, USA.; Helen Keller Foundation for Research and Education, Birmingham, AL, USA.; University of Alabama at Birmingham (UAB), Department of Ophthalmology, Birmingham, AL, USA.; UAB Callahan Eye Hospital, Birmingham, AL, USA.
West MR; Retina Specialists of Alabama, Birmingham, AL, USA.; Helen Keller Foundation for Research and Education, Birmingham, AL, USA.; University of Alabama at Birmingham (UAB), Department of Ophthalmology, Birmingham, AL, USA.; UAB Callahan Eye Hospital, Birmingham, AL, USA.
Fletcher DC; University of Kansas Medical Center, Department of Ophthalmology and KU Eye Center, Kansas City, KS, USA.; Retina Consultants of Southwest Florida, Ft. Myers, FL, USA.
Schuchard RA; Envision Research Institute, Wichita, KS, USA.
Kuhn F; Helen Keller Foundation for Research and Education, Birmingham, AL, USA.; Milos Eye Hospital, Belgrade, Serbia.; University of Pécs, Department of Ophthalmology, Pécs, Hungary.
Źródło:
Clinical ophthalmology (Auckland, N.Z.) [Clin Ophthalmol] 2021 Jan 06; Vol. 15, pp. 19-29. Date of Electronic Publication: 2021 Jan 06 (Print Publication: 2021).
Typ publikacji:
Case Reports
Język:
English
Imprint Name(s):
Original Publication: Auckland : Dove Medical Press, c2007-
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Contributed Indexing:
Keywords: OSC; OSC/SS; Ora Secunda Cerclage; SS; STL1; STL2; Stickler syndrome; encircling laser prophylaxis; giant retinal tear; retinal detachment prevention
Entry Date(s):
Date Created: 20210115 Latest Revision: 20220419
Update Code:
20240105
PubMed Central ID:
PMC7802593
DOI:
10.2147/OPTH.S284441
PMID:
33447008
Raport
Purpose: To introduce a novel technique of encircling laser prophylaxis (ora secunda cerclage Stickler syndrome, OSC/SS) to prevent rhegmatogenous retinal detachment (RRD) in Stickler syndrome eyes.
Patients and Methods: After first eye RRD at age 50 and at age 18, respectively, a 53-year-old father and his 22-year-old son with type 2 SS (STL2) gave informed consent and underwent OSC/SS prophylaxis, performed in each fellow eye. A 26-year-old STL2 daughter then suffered first eye retinal detachment and similarly chose fellow eye OSC/SS prophylaxis. A second son, 28 years of age with STL2, chose OSC/SS prophylaxis in both eyes.
Results: The three OSC/SS treated fellow eyes have gone 12 years, 11 years, and 8 years without RRD. STL1 and less common STL2 eyes are known to have a similar rate of RRD, and 80% of STL1 fellow eyes develop RRD at a median of 4 years in the absence of prophylaxis. Moreover, five of six (83%) known STL2 family members suffered RRD, only the STL2 son with bilateral OSC/SS remaining bilaterally attached. All five OSC/SS treated eyes (average 8.7 years post-prophylaxis) retained preoperative visual acuity of 20/20 to 20/30, with an average, asymptomatic reduction of meridional field in each eye to 50 degrees. In contrast, in the three eyes having suffered RRD prior to presentation, visual acuity ranged from 20/125 to 8/200 and average meridional field was 29 degrees.
Conclusion: Encircling grid laser (OSC) modified in Stickler eyes to encompass the ora serrata and extend posteriorly to and between the vortex vein ampullae (OSC/SS) is a reasonable RRD prophylaxis option to offer STL1 and STL2 patients as an alternative to no treatment or less effective prophylaxis. Because of rarity and severity, the ultimate proof of safety and efficacy will likely come not from randomized trials, but from a non-randomized, prospective, cohort comparison study of such individual efforts.
Competing Interests: The authors report no conflicts of interest in this work financial or otherwise.
(© 2021 Morris et al.)

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