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

OSC OSC/SS Ora Secunda Cerclage SS STL1 STL2 Stickler syndrome encircling laser prophylaxis giant retinal tear retinal detachment prevention

Journal

Clinical ophthalmology (Auckland, N.Z.)
ISSN: 1177-5467
Titre abrégé: Clin Ophthalmol
Pays: New Zealand
ID NLM: 101321512

Informations de publication

Date de publication:
2021
Historique:
received: 29 09 2020
accepted: 11 11 2020
entrez: 15 1 2021
pubmed: 16 1 2021
medline: 16 1 2021
Statut: epublish

Résumé

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. 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. 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. 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.

Identifiants

pubmed: 33447008
doi: 10.2147/OPTH.S284441
pii: 284441
pmc: PMC7802593
doi:

Types de publication

Case Reports

Langues

eng

Pagination

19-29

Informations de copyright

© 2021 Morris et al.

Déclaration de conflit d'intérêts

The authors report no conflicts of interest in this work financial or otherwise.

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Auteurs

Robert E 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.

Edward Scott Parma (ES)

Retina Specialists of Alabama Montgomery, Montgomery, AL, USA.

Nathaniel H Robin (NH)

UAB Department of Genetics, Birmingham, AL, USA.

Mathew R 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.

Matthew H 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.

Matthew R 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.

Donald C 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.

Ronald A Schuchard (RA)

Envision Research Institute, Wichita, KS, USA.

Ferenc 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.

Classifications MeSH